Clinical Algorithms in General Surgery PDF

Clinical Algorithms
in General Surgery
A Practical Guide
Salvatore Docimo Jr.
Eric M. Pauli
Editors
123
Clinical Algorithms in General Surgery
Salvatore Docimo Jr. • Eric M. Pauli
Editors
Clinical Algorithms
in General Surgery
A Practical Guide
Editors
Salvatore Docimo Jr. Eric M. Pauli
Department of Surgery Department of Surgery
Stony Brook Medicine Penn State Milton S. Hershey Medical
Stony Brook, NY Center
USA Hershey, PA
USA
ISBN 978-3-319-98496-4 ISBN 978-3-319-98497-1 (eBook)

https://doi.org/10.1007/978-3-319-98497-1
Library of Congress Control Number: 2019930648
© Springer Nature Switzerland AG 2019

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To my wife, Aisa, and our son, Massimo, thank you for the
unwavering support and patience. To my parents, I am forever
indebted to you. To my colleagues and friends throughout the
surgical world who contributed their expertise to these
algorithms, thanks. I will always be grateful to those who had a
part in my training and continue to offer their wisdom and
insight.
Salvatore Docimo, Jr., DO, MS
My surgical career has been the epitome of dumb luck. I have

had the good fortune to train under and with amazing
surgeons, I work with dynamic colleagues, and I get to train
eager students, residents, and fellows. I would like to thank all
those individuals who have contributed to my success,
especially those who were willing to share their expertise
through these algorithms. Thanks in particular to the three Jeffs
(you can rank yourselves in order), Yuri, Mike, Randy, Peter,
Ann, Evan, Abraham, Lou, and Chris.
Eric M. Pauli, MD
Preface
With the rapid expansion of surgical knowledge, it has never been more chal-
lenging to organize and articulate a simple and safe surgical plan. Traditional
textbooks, in an attempt to keep pace with the growth of surgical science,
have become encyclopedic reference books. Young surgeons, with a finite
amount of time to pore over such tomes and an increasing load of clinical
responsibilities, often search for educational materials that offer basic, safe
principles of general surgery. For the more seasoned surgeon, a brief review
of vital surgical topics does not require the comprehensive perspective offered
by traditional surgical textbooks.
We created this book of surgical algorithms in order to meet the needs of
both of these groups of surgeons. The goal of an algorithm is to create a set of
rules, permit data processing, and establish a solution in the most efficient
manner. An algorithmic approach to surgical scenarios allows for concise
organization of clinical information, application of basic and safe principles,
and, finally, formation of an unambiguous surgical solution. The algorithms
are also accompanied with a synopsis to provide a more comprehensive
review, if desired.
Students, residents, and surgeons will find the algorithms concise enough
to read to completion in moments of spare time. For the surgical trainee, they
should provide a foundation upon which future learning can be built. For the
more senior surgeon, they will provide an up-to-date overview of commonly
encountered topics from the “20,000 foot view.” For chief residents and
recent graduates, we hope this book enables crystallization of their knowl-
edge base as they prepare for the American Board of Surgery certifying
examination.
Stony Brook, NY, USA Salvatore Docimo Jr.

Hershey, PA, USA Eric M. Pauli
vii
Contents
Part I Skin and Soft Tissue
1 Management of Cutaneous Melanoma�� 3

Julie A. DiSano and Colette R. Pameijer
2 Basal Cell Carcinoma�� 5
3 Squamous Cell Carcinoma�� 7
4 Management of Soft Tissue Sarcoma �� 9
5 Necrotizing Soft Tissue Infection�� 11
Part II Head and Neck
6 Management of Squamous Cell Carcinoma

of the Oropharynx�� 17
Laila Siddique, Tom Shokri, and Neerav Goyal
7 Evaluation of Neck Mass �� 21
Tom Shokri, Laila Siddique, and Neerav Goyal
8 Evaluation of an Enlarged Cervical Lymph Node�� 25
9 Salivary Gland Tumors�� 29
Part III Thoracic
10 Massive Hemoptysis�� 35

Henry Tannous, Joanna Chikwe, and Maroun B. Yammine
11 Mediastinal Masses�� 39
12 Tracheal Stenosis�� 43
ix
x Contents
13 Incidental Lung Nodule�� 47

14 Management of Lung Cancer �� 51
Scott C. Tiedebohl and Matthew D. Taylor
15 Management of Empyema�� 55
Shannon R. Kotch and Matthew D. Taylor
16 Management of Spontaneous Pneumothorax�� 59
17 Thoracoabdominal Aortic Aneurysm�� 63
Albert G. Pavalonis and Anil Hingorani
Part IV Breast
18 Nipple Discharge�� 69

Anjali R. Thawani and Lillian M. Erdahl
19 Breast Mass Evaluation �� 73
20 Ductal Carcinoma In Situ�� 77
21 Lobular Carcinoma In Situ�� 81
22 Enlarged Axillary Lymph Node�� 85
Zeynep Bostanci and Laura Kruper
23 Metastatic Breast Cancer�� 87
24 Recurrent Breast Cancer�� 91
25 Paget’s Disease�� 95
26 Locoregional Recurrence of Breast Cancer�� 97
Jessica C. Gooch and Freya Schnabel
27 Metastatic Breast Cancer�� 101
28 Inflammatory Breast Cancer�� 105
29 Breast Reconstruction�� 109
30 Management of Male Breast Cancer�� 113
Contents xi
Part V Esophagus
31 Management of Esophageal Motility Disorders�� 119

Anthony R. Tascone and Caitlin A. Halbert
32 Management of Achalasia �� 123
33 Barrett’s Esophagitis �� 127
Caitlin A. Halbert and Anthony R. Tascone
34 Gastroesophageal Reflux Disease �� 129
35 Hiatal Hernia�� 133
Wanda Lam, Ruel Neupane, and Jeffrey M. Marks
36 Esophageal Carcinoma�� 135
Ruel Neupane, Wanda Lam, and Jeffrey M. Marks
37 Esophageal Perforation �� 139
38 Acidic and Basic Injuries�� 143
Part VI Stomach and Duodenum
39 Gastric Ulcer Management �� 149

Maria S. Altieri and Konstantinos Spaniolas
40 Duodenal Ulcer Management �� 153
41 Complications of Peptic Ulcer Disease �� 157
Carl J. Dickler and Konstantinos Spaniolas
42 Management of Recurrent Peptic Ulcer Disease�� 161
43 Management of Gastric Cancer�� 165
Christina L. Wolchok and Georgios V. Georgakis
44 Management of Gastrointestinal Stromal Tumors�� 169
Igor G. Elyash
45 Management of Upper Gastrointestinal Hemorrhage�� 171
Igor G. Elyash
Part VII Small Bowel
46 Small Bowel Obstruction�� 175

Ryan M. Juza and Vamsi V. Alli
47 Small Bowel Tumors�� 181
Vamsi V. Alli and Ryan M. Juza
xii Contents
48 Management of Small Bowel Neuroendocrine Tumors�� 185

Michele A. Riordon and Calvin H. L. Law
49 Management of Enterocutaneous Fistulas�� 191
Maria Michailidou
50 Management of Crohn’s Disease�� 195
Igor G. Elyash
51 Management of Postoperative Ileus �� 197
Igor G. Elyash
52 Management of Gallstone Ileus�� 199
Igor G. Elyash
53 Management of Short Bowel Syndrome�� 201
Igor G. Elyash
Part VIII Large Bowel
54 Management of Lower Gastrointestinal Bleeding�� 205

Audrey S. Kulaylat and David B. Stewart Jr.
55 Management of Diverticulitis�� 209
56 Management of Large Bowel Obstruction�� 213
57 Management of Colonic Pseudo-Obstruction �� 217
58 Management of Colonic Volvulus �� 221
59 Appendicitis�� 225
Kristen T. Crowell and Evangelos Messaris
60 Ulcerative Colitis�� 229
61 Crohn’s Colitis�� 233
Maria Michailidou and Evangelos Messaris
62 Ischemic Colitis�� 237
William Sangster and Evangelos Messaris
63 Clostridium difficile Colitis�� 241
64 Hereditary Colorectal Cancer Syndromes�� 243
Emily Huang and Michael F. McGee
65 Colorectal Polyps�� 251
66 Colon Cancer�� 255
Contents xiii
Part IX Rectum and Anus
67 Rectal Prolapse�� 263

Quinton Morrow Hatch and Eric K. Johnson
68 Solitary Rectal Ulcer Syndrome �� 269
John Kuckelman and Eric K. Johnson
69 Rectal Cancer �� 275
70 Rectovaginal Fistula�� 283
71 Management of Hemorrhoids�� 289
Matthew Z. Wilson and Joseph R. Notaro
72 Management of Anal Fissure�� 293
Matthew Z. Wilson and Kirsten Bass Wilkins
73 Management of Perianal Abscess and Fistula-in-Ano�� 297
Matthew Z. Wilson and Bertram T. Chinn
74 Management of Anal Cancer�� 301
75 Management of Fecal Incontinence�� 303
Matthew Z. Wilson and Suraj Alva
Part X Liver
76 Evaluation of Liver Nodule �� 307

Katelin A. Mirkin and Niraj J. Gusani
77 Cystic Diseases of the Liver�� 313
Laura M. Enomoto and Niraj J. Gusani
78 Management of Benign Liver Masses�� 319
79 Hepatic Abscess�� 323
Jasvinder Singh and Niraj J. Gusani
80 Malignant Liver Tumors (Metastatic Liver Disease)�� 327
Neal M. Mineyev, Karla M. Chaffee, and Joyce Wong
81 Diagnosis and Management of Hepatocellular Carcinoma�� 331
82 Diagnosis and Management of Primary Sclerosing Cholangitis 335
83 Portal Hypertension and Shunting�� 339
xiv Contents
Part XI Biliary
84 Acute Cholecystitis and Biliary Colic�� 345

Chanak J. Chantachote and Samer Sbayi
85 Acalculous Cholecystitis�� 349
86 Postcholecystectomy�� 351
87 Management of Postcholecystectomy Cholangitis�� 355
Joel VanderVelde and Ross F. Goldberg
88 Management of Choledocholithiasis�� 357
89 Acute Cholangitis �� 359
Joel Vandervelde and Ross F. Goldberg
90 Cholangiocarcinoma�� 361
Zachary J. Senders, John B. Ammori, and Jeffrey M. Hardacre
91 Diagnosis and Management of Gallbladder Cancer�� 365
Joshua L. Lyons, John B. Ammori, and Jeffrey M. Hardacre
92 Choledochal Cysts�� 369
Shreya Gupta, Jeffrey M. Hardacre, and John B. Ammori
93 Cholecystectomy of the Pregnant Patient�� 373
Avi Hameroff and Jaimey M. Pauli
Part XII Pancreas
94 Acute Pancreatitis�� 379

Kayla M. Hartz and Jennifer Maranki
95 Chronic Pancreatitis�� 383
96 Pancreas Divisum�� 387
97 Walled-Off Pancreatic Fluid Collections �� 391
98 Periampullary Carcinoma�� 395
Heidi N. Overton and Matthew J. Weiss
99 Management of Intraductal Papillary Mucinous Neoplasms�� 399
Jonathan G. Sham and Matthew J. Weiss
100 Pancreatic Necrosis�� 403
Ammar Asrar Javed and Matthew J. Weiss
Contents xv
Part XIII Spleen
101 Management of Splenic Abscess �� 409

Andrew T. Bates and Michael G. Svestka
102 Atraumatic Indications for Splenectomy�� 413
Maria S. Altieri and Andrew T. Bates
Part XIV Thyroid/Parathyroid
103 Hypothyroidism�� 419

Lukasz Czerwonka
104 Hyperthyroidism�� 423
Ewen Chao and Lukasz Czerwonka
105 Thyroiditis�� 427
Lukasz Czerwonka
106 Goiter�� 431
Lukasz Czerwonka
107 Thyroid Nodule�� 435
Melissa Boltz
108 Thyroid Cancer�� 439
Melissa Boltz
109 Hyperparathyroidism�� 443
Melissa Boltz
Part XV Endocrine
110 Cushing’s Syndrome and Disease �� 449

Edwina Moore and Vikram D. Krishnamurthy
111 Primary Hyperaldosteronism (Conn’s Syndrome) �� 453
Iuliana Bobanga, Cassandre Bénay, and Vikram D.
Krishnamurthy
112 Glucagonoma�� 457
Talia Burneikis and Vikram D. Krishnamurthy
113 Management of Pheochromocytoma�� 461
Hadley E. Ritter and Benjamin C. James
114 Management of Aldosteronoma�� 465
115 Management of Gastrinoma �� 469
Rachel E. Simpson and Benjamin C. James
116 Management of Insulinoma�� 473
xvi Contents
117 Management of Somatostatinoma�� 477

118 Management of VIPoma�� 481
Part XVI Pediatric
119 Congenital Diaphragmatic Hernia �� 487

Christopher J. McLaughlin, Rachel E. Hanke,
and Robert E. Cilley
120 Tracheoesophageal Fistula�� 491
Rachel E. Hanke, Morgan K. Moroi, and Robert E. Cilley
121 Other Diaphragmatic Hernias: Late-Presenting
Bochdalek Hernia, Morgagni Hernia, and Giant
Hiatal Hernia of Infancy �� 495
Morgan K. Moroi, Christopher J. McLaughlin,
122 Duodenal Obstruction in Newborns�� 499
Abdulraouf Y. Lamoshi, Sophia Abdulhai,
and Todd A. Ponsky
123 Small Intestinal Atresia �� 501
and Todd A. Ponsky
124 Management of Malrotation �� 505
Sophia Abdulhai, Abdulraouf Y. Lamoshi,
and Todd A. Ponsky
125 Management of Imperforate Anus �� 509
Sophia Abdulhai and Aaron Garrison
126 Hirschsprung Disease�� 513
Rachel E. Hanke, Morgan K. Moroi, and Kathryn Lynn
Martin
127 Pediatric Inguinal Hernia�� 517
Afif N. Kulaylat and Kathryn Lynn Martin
128 Meconium Ileus�� 521
Kathryn Lynn Martin and Afif N. Kulaylat
129 Pediatric Intussusception�� 525
130 Pyloric Stenosis�� 529
Dan W. Parrish, Jonathan H. DeAntonio,
and David A. Lanning
131 Necrotizing Enterocolitis �� 533
Jonathan H. DeAntonio, Dan W. Parrish,
Contents xvii
132 Omphalocele and Gastroschisis�� 537

133 Biliary Atresia�� 541
Part XVII Vascular
134 Carotid Artery Stenosis �� 547

Ian Bailey and Faisal Aziz
135 Abdominal Aortic Aneurysm�� 551
Erin K. Greenleaf and Faisal Aziz
136 Ruptured Abdominal Aortic Aneurysm�� 555
Faisal Aziz
137 Aortic Dissection�� 559
Katelynn Ferranti and Faisal Aziz
138 Acute Lower Extremity Ischemia�� 565
Afsha Aurshina and Anil Hingorani
139 Chronic Lower Extremity Ischemia �� 569
140 Intermittent Claudication �� 573
141 Acute Deep Venous Thrombosis�� 577
142 Management of Acute Mesenteric Ischemia�� 581
Josh Radtka
143 Management of Chronic Mesenteric Ischemia�� 585
Josh Radtka
144 Thoracic Outlet Syndrome�� 589
Tarik Z. Ali and Josh Radtka
145 AV Shunt Complications �� 593
Josh Radtka
Part XVIII Genitourinary
146 Management of the Renal Mass�� 597

J. Chris Riney, Neil J. Kocher, and Matthew Kaag
147 Prostate Cancer�� 601
Rosa Park and Matthew Kaag
148 Management of Scrotal/Testicular Mass�� 605
Brian M. Blair and Matthew Kaag
xviii Contents
149 Diagnosis and Management of Fournier’s Gangrene �� 609

Augustyna Gogoj and Matthew Kaag
Part XIX Trauma
150 Hypotension and Blunt Abdominal Trauma �� 615

Cheyenne C. Sonntag and Steven R. Allen
151 Traumatic Brain Injury�� 619
Shannon R. Kotch and Steven R. Allen
152 Penetrating Neck Trauma �� 623
Alexis Lauria and Steven R. Allen
153 Penetrating Chest Trauma�� 627
Melissa Linskey and Steven R. Allen
154 ED Thoracotomy�� 631
Nathan R. Manley and George O. Maish III
155 Blunt Chest Wall Trauma�� 633
156 Blunt Cardiac Injury �� 637
157 Deceleration Injury: Blunt Aortic Injury�� 641
158 Penetrating Abdominal Trauma �� 645
Michael Smith and Fausto Vinces
159 Blunt Abdominal Trauma�� 649
160 Management Algorithm for Acute and Chronic
Diaphragmatic Injuries �� 653
Elif Onursal and Fausto Vinces
161 Management of Traumatic Liver Injuries �� 657
Melissa Amberger and Fausto Vinces
162 Management of Pancreatic Trauma �� 661
Shreya Jammula and Eric H. Bradburn
163 Management of Traumatic Splenic Injuries�� 665
Eric H. Bradburn, Kameron Durante, and Shreya Jammula
164 Management of Kidney and Ureter Injuries �� 669
Eric H. Bradburn, Madison Morgan, and Danielle Von Nieda
165 Urethral Trauma�� 675
Cheyenne C. Sonntag and Susan MacDonald
166 Pelvic Fractures�� 679
Ryan M. Staszak and Lacee Jay Laufenberg
Contents xix
167 Bladder Injuries �� 683

168 Rectal Injuries�� 687
Amanda E. Lee, Karima Fitzgerald, and Lacee Jay Laufenberg
169 Extremity Compartment Syndrome�� 693
Karima Fitzgerald, Amanda E. Lee, and Lacee Jay Laufenberg
Part XX Critical Care
170 Management of Intracranial Hemorrhage�� 701

Ariel P. Santos
171 Airway Management �� 707
Robert S. Schoaps and Sprague W. Hazard III
172 Intubation and Extubation�� 711
Ariel P. Santos
173 Acute Respiratory Distress Syndrome (ARDS)�� 719
Dan A. Galvan
174 Management of Sepsis �� 723
Jacklyn Engelbart and Luis J. Garcia
175 Management of Shock �� 727
176 Blood Transfusion Indications�� 731
177 Abdominal Compartment Syndrome�� 735
178 Acute Renal Failure �� 739
Kathleen A. Iles and Richard J. King
179 Postoperative Pulmonary Emboli�� 743
180 Burns Management�� 747
181 Acid-Base Disorders�� 751
Part XXI Electrolytes
182 Hyponatremia�� 757

Kathryn W. Shaw and Andre A. S. Dick
183 Hypernatremia �� 761
xx Contents
184 Hypokalemia�� 765

185 Hyperkalemia �� 767
186 Management of Hypocalcemia�� 771
187 Management of Hypercalcemia�� 775
188 Paradoxical Aciduria �� 779
Part XXII Hernia
189 Inguinal Hernia�� 783

Q. Lina Hu and David C. Chen
190 Recurrent Inguinal Hernia�� 789
191 Femoral Hernia�� 793
192 Obturator Hernia�� 797
193 Ventral Hernia Repair �� 801
Justin A. Doble and Eric M. Pauli
194 Incarcerated and Strangulated Hernia�� 805
195 Management of Open Abdomen �� 809
196 Abdominal Wall Reconstruction�� 813
Part XXIII Bariatric Surgery
197 Indications for Bariatric Surgery �� 819

Jin Sun Kim and Ann M. Rogers
198 Work-Up of Abdominal Pain in the Bariatric Patient�� 821
Sarayna S. McGuire and Ann M. Rogers
199 Internal Hernia: Diagnosis and Treatment�� 825
Brandon LaBarge and Ann M. Rogers
200 Marginal Ulcer: Diagnosis and Treatment�� 827
Ye Tian and Ann M. Rogers
201 Ventral Hernia Repair in Bariatric Patients �� 831
Anish Shah and Salvatore Docimo Jr.
Contents xxi
202 Acute Leak Following Bariatric Surgery: Endoscopic Stent

Management �� 835
203 Vitamin and Micronutrient Deficiencies After
Bariatric Surgery �� 839
Part XXIV Pregnancy and General Surgery
204 Pregnancy and Cholelithiasis�� 845

Jaimey M. Pauli
205 Pregnancy and Appendicitis�� 849
Emily Smith and Jaimey M. Pauli
206 Pregnancy and Breast Cancer�� 853
James M. O’Brien and Jaimey M. Pauli
207 Pregnancy and Hernia �� 857
Jaimey M. Pauli
Index�� 861
Contributors
Sophia Abdulhai, MD Division of Pediatric Surgery, Akron Children’s

Hospital, Akron, OH, USA
Tarik Z. Ali, MD Division of Vascular Surgery, Penn State Milton S. Hershey
Medical Center, Hershey, PA, USA
Steven R. Allen, MD Department of Surgery, Penn State Health Milton
S. Hershey Medical Center, Hershey, PA, USA
Vamsi V. Alli, MD Department of Surgery, Division of Minimally Invasive
and Bariatric Surgery, Penn State Milton S. Hershey Medical Center, Hershey,
PA, USA
Maria S. Altieri, MD, MS Department of General Surgery, Stony Brook
University Hospital, Stony Brook, NY, USA
Suraj Alva, MD, FACD, FASCRS Department of Surgery, Rutgers Robert
Wood Johnson Medical School, Edison, NJ, USA
Melissa Amberger, DO Department of Surgery, St. Barnabas Health
System, Bronx, NY, USA
John B. Ammori, MD Department of Surgery, University Hospitals
Cleveland Medical Center, Cleveland, OH, USA
Afsha Aurshina, MBBS Department of Vascular Surgery, Vascular Institute
of New York, Brooklyn, NY, USA
Faisal Aziz, MD, FACS Division of Vascular Surgery, Penn State Heart and
Vascular Institute, Pennsylvania State University College of Medicine,
Hershey, PA, USA
Department of Surgery, Penn State Milton S. Hershey Medical Center,
Hershey, PA, USA
Ian Bailey, MD Division of Vascular Surgery, Penn State Heart and Vascular
Institute, Pennsylvania State University College of Medicine, Hershey, PA,
USA
Penn State Health Milton S. Hershey Medical Center, Hershey, PA, USA
Andrew T. Bates, MD Department of Surgery, Stony Brook University
Hospital, Stony Brook, NY, USA
xxiii
xxiv Contributors
Cassandre Bénay, MD, MSc Department of Endocrine Surgery, The

Cleveland Clinic, Cleveland, OH, USA
Brian M. Blair, MD Division of Urology, Penn State Milton S. Hershey
Iuliana Bobanga, MD Department of Endocrine Surgery, The Cleveland
Clinic, Cleveland, OH, USA
Melissa Boltz, DO, MBA Department of Surgery, Penn State Hershey
Zeynep Bostanci, MD Breast Surgical Oncology, Ironwood Cancer and
Research Centers, Avondale, AZ, USA
Eric H. Bradburn, DO, MS, FACS Department of Trauma and Acute Care
Surgery, Penn Medicine Lancaster General Health, Lancaster, PA, USA
Talia Burneikis, MD Department of Endocrine Surgery, The Cleveland
Karla M. Chaffee, MD Department of General Surgery, Lenox Hill
Hospital, New York, NY, USA
Chanak J. Chantachote, MD Department of Surgery, Stony Brook
Ewen Chao, MD Department of Surgery, Division of Otolaryngology –
Head and Neck Surgery, Stony Brook University Hospital, Stony Brook, NY,
USA
David C. Chen, MD Department of Surgery, Lichtenstein Amid Hernia
Clinic at University of California at Los Angeles, Los Angeles, CA, USA
Joanna Chikwe, MD Department of Surgery, Stony Brook University
Department of Cardiovascular Surgery, Icahn School of Medicine at Mount
Sinai, New York, NY, USA
Bertram T. Chinn, MD Department of Colon and Rectal Surgery, Rutgers
Robert Wood Johnson Medical School, Edison, NJ, USA
Robert E. Cilley, MD Department of Surgery, Penn State Milton S. Hershey
Kristen T. Crowell, MD Department of Surgery, Penn State Milton
Lukasz Czerwonka, MD Department of Surgery, Division of
Otolaryngology – Head and Neck Surgery, Stony Brook University Hospital,
Stony Brook, NY, USA
Jonathan H. DeAntonio, MD Division of Pediatric Surgery, Department of
General Surgery, Virginia Commonwealth University Health, Richmond, VA,
USA
Contributors xxv
Department of Surgery, Virginia Commonwealth University School of

Medicine, Richmond, VA, USA
Andre A. S. Dick, MD, MPH Department of Surgery, Section of Pediatric
Transplantation, Seattle Children’s Hospital and University of Washington,
Seattle, WA, USA
Carl J. Dickler, MD Department of General Surgery, SUNY Stony Brook
University Hospital, Health Sciences Center T19-030, Stony Brook, NY,
USA
Julie A. DiSano, MD Department of General Surgery, Penn State Milton
Justin A. Doble, MD Department of Surgery, Penn State Milton S. Hershey
Salvatore Docimo Jr., DO, MS Division of Bariatric, Foregut, and Advanced
Gastrointestinal Surgery, Stony Brook Medicine, Stony Brook, NY, USA
Kameron Durante Department of Trauma and Acute Care Surgery, Penn
Medicine Lancaster General Health, Lancaster, PA, USA
Igor G. Elyash, DO Morristown Surgical Associates, Morristown Medical
Center, Morristown, NJ, USA
Jacklyn Engelbart, BSE Department of Surgery, University of Iowa
Hospitals and Clinics, Iowa City, IA, USA
Laura M. Enomoto, MD Department of Surgery, Program for Liver,
Pancreas, and Foregut Tumors, Penn State College of Medicine, Penn State
Cancer Institute, Hershey, PA, USA
Lillian M. Erdahl, MD, FACS Department of Surgery, University of Iowa,
Iowa City, IA, USA
Katelynn Ferranti, MD Division of Vascular Surgery, Penn State Heart and
Vascular Institute, Pennsylvania State University College of Medicine,
Hershey, PA, USA
Department of Vascular Surgery, Penn State Health, Milton S. Hershey
Karima Fitzgerald, MD Division of Trauma, Acute Care, and Critical Care
Surgery, Penn State Milton S. Hershey Medical Center, Hershey, PA, USA
Dan A. Galvan, MD Geisinger Holy Spirit Hospital, Harrisburg, PA, USA
Luis J. Garcia, MD, FACS Department of Surgery, University of Iowa
Hospitals and Clinics, Iowa City, IA, USA
Aaron Garrison, MD Division of Pediatric Surgery, Akron Children’s
Georgios V. Georgakis, MD, PhD Department of Surgery, Division of
Surgical Oncology, Stony Brook University Hospital, Stony Brook, NY, USA
xxvi Contributors
Augustyna Gogoj, BS Division of Urology, Penn State Milton S. Hershey

Ross F. Goldberg, MD Creighton University School of Medicine, Phoenix,
AZ, USA
University of Arizona College of Medicine – Phoenix, Phoenix, AZ, USA
Jessica C. Gooch, MD Department of Surgery, NYU Langone Health, NYU
Perlmutter Cancer Center, New York, NY, USA
Neerav Goyal, MD, MPH Division of Otolaryngology-Head and Neck
Surgery, Department of Surgery, Penn State Milton S. Hershey Medical
Center, Hershey, PA, USA
Penn State Cancer Institute, Penn State Milton S. Hershey Medical Center,
Hershey, PA, USA
Erin K. Greenleaf, MD, MS Division of Vascular Surgery, Penn State Heart
and Vascular Institute, Pennsylvania State University College of Medicine,
Hershey, PA, USA
Penn State Milton S. Hershey Medical Center, Hershey, PA, USA
Shreya Gupta, MD Department of Surgery, University Hospitals Cleveland
Medical Center, Cleveland, OH, USA
Niraj J. Gusani, MD, MS, FACS Department of Surgery, Program for
Liver, Pancreas, and Foregut Tumors, Penn State College of Medicine, Penn
State Cancer Institute, Hershey, PA, USA
Caitlin A. Halbert, DO Advanced GI and Bariatric Surgery, Department of
General Surgery, Christiana Care Health System, Newark, DE, USA
Avi Hameroff, MD Maternal Fetal Medicine, Department of Obstetrics and
Gynecology, Penn State Health Milton S. Hershey Medical Center, Hershey,
PA, USA
Rachel E. Hanke, MD Department of Surgery, Penn State Milton S. Hershey
Jeffrey M. Hardacre, MD Department of Surgery, University Hospitals
Kayla M. Hartz, BA Edward Via College of Osteopathic Medicine,
Blacksburg, PA, USA
Quinton Morrow Hatch, MD Department of Surgery, Madigan Army
Medical Center, Tacoma, WA, USA
Sprague W. Hazard III, MD Department of Anesthesiology and
Perioperative Medicine, Penn State Hershey Medical Center, Hershey, PA,
USA
Anil Hingorani, MD, FACS Division of Vascular Services, NYU Langone
Hospital-Brooklyn, Brooklyn, NY, USA
Contributors xxvii
Q. Lina Hu, MD Department of Surgery, David Geffen School of Medicine

at University of California, Los Angeles, Los Angeles, CA, USA
Emily Huang, MD, MA Ed Department of Surgery, University of Chicago,
Chicago, IL, USA
Kathleen A. Iles, MD SUNY Upstate Medical University College of
Medicine, Syracuse, NY, USA
Department of Surgery, University of North Carolina Hospitals, Chapel Hill,
NC, USA
Benjamin C. James, MD, MS Department of Surgery, Harvard Medical
School, Boston, MA, USA
Department of Surgery, Beth Israel Deaconess Medical Center, Boston, MA,
USA
Shreya Jammula, BS Department of Trauma and Acute Care Surgery, Penn
Ammar Asrar Javed, MD Department of Surgery, Johns Hopkins Hospital,
Baltimore, MD, USA
Eric K. Johnson, MD Cleveland Clinic Foundation, Cleveland, OH, USA
Department of Surgery, Division of Colorectal Surgery, Hillcrest Hospital,
Mayfield Heights, OH, USA
Ryan M. Juza, MD Department of Surgery, Division of Minimally Invasive
and Bariatric Surgery, Penn State Milton S. Hershey Medical Center, Hershey,
PA, USA
Matthew Kaag, MD Division of Urology, Penn State Milton S. Hershey
Jin Sun Kim, BS, BA Pennsylvania State University College of Medicine,
Hershey, PA, USA
Richard J. King, MD, FACS Department of Surgery, SUNY Upstate
Medical University, Syracuse, NY, USA
Neil J. Kocher, MD Division of Urology, Penn State Milton S. Hershey
Shannon R. Kotch, MD Department of General Surgery, Penn State Health
Milton S. Hershey Medical Center, Hershey, PA, USA
Vikram D. Krishnamurthy, MD Department of Endocrine Surgery, The
Cleveland Clinic, Cleveland, OH, USA
Laura Kruper, MD, MSCE Breast Surgical Oncology, Department of
Surgery, City of Hope Hospital, Duarte, CA, USA
John Kuckelman, DO Department of General Surgery, Madigan Army
Uniformed Services University of the Health Sciences, Bethesda, MD, USA
xxviii Contributors
Afif N. Kulaylat, MD, MSc Department of Surgery, Division of Pediatric

Surgery, Penn State Milton S. Hershey Medical Center, Hershey, PA, USA
Audrey S. Kulaylat, MD Department of Surgery, Penn State Milton
Brandon LaBarge, BA Department of Surgery, Penn State College of
Medicine, Penn State Milton S. Hershey Medical Center, Hershey, PA, USA
Wanda Lam, MD Department of Surgery, University Hospitals Cleveland
Abdulraouf Y. Lamoshi, MD, MPH, CTS, ABPS, MS Division of Pediatric
Surgery, Akron Children’s Hospital, Akron, OH, USA
David A. Lanning, MD, PhD Division of Pediatric Surgery, Department of
General Surgery, Virginia Commonwealth University Health, Richmond, VA,
USA
Department of Surgery and Pediatrics, Children’s Hospital of Richmond,
Richmond, VA, USA
Department of Surgery, Virginia Commonwealth University School of
Medicine, Richmond, VA, USA
Alexis Lauria, MD Department of Surgery, Walter Reed National Military
Medical Center, Bethesda, MD, USA
Lacee Jay Laufenberg, MD Department of Surgery, Division of Trauma,
Acute Care, and Critical Care Surgery, Penn State Milton S. Hershey Medical
Calvin H. L. Law, MD, MPH, FRCSC Department of Surgical Oncology,
Sunnybrook Health Sciences Centre, Toronto, ON, Canada
Amanda E. Lee, MD Department of Surgery, Penn State Milton S. Hershey
Melissa Linskey, MD Department of Surgery, Penn State Health Milton
Joshua L. Lyons, MD Department of Surgery, University Hospitals
George O. Maish III, MD Department of Surgery, University of Tennessee
Health Science Center, Memphis, TN, USA
Nathan R. Manley, MD, MPH Department of Surgery, University of
Tennessee Health Science Center, Memphis, TN, USA
Jennifer Maranki, MD, MSc Department of Gastroenterology and
Hepatology, Penn State Milton S. Hershey Medical Center, Hershey, PA,
USA
Contributors xxix
Jeffrey M. Marks, MD, FACS, FASGE Department of Surgery, University

Hospitals Cleveland Medical Center, Cleveland, OH, USA
Kathryn Lynn Martin, MD, FRCSC Department of Surgery, Division of
Pediatric Surgery, Penn State Milton S. Hershey Medical Center, Hershey,
PA, USA
Michael F. McGee, MD, FACS, FASCRS Department of Surgery,
Northwestern Memorial Hospital, Chicago, IL, USA
Sarayna S. McGuire, BS Department of Surgery, Penn State Milton
Susan MacDonald, MD Division of Urology, Department of Surgery, Penn
State Milton S. Hershey Medical Center, Hershey, PA, USA
Christopher J. McLaughlin, MD Department of Surgery, Penn State
Evangelos Messaris, MD, PhD, FACS, FASCRS Department of Surgery,
Penn State Milton S. Hershey Medical Center, Hershey, PA, USA
Division of Colon and Rectal Surgery, Beth Israel Medical Center, Harvard
Medical Center, Boston, MA, USA
Maria Michailidou Department of Surgery, Penn State Milton S. Hershey
Neal M. Mineyev, MD Department of General Surgery, Lenox Hill Hospital,
New York, NY, USA
Katelin A. Mirkin, MD Department of Surgery, Program for Liver,
Pancreas, and Foregut Tumors, Penn State College of Medicine, Penn State
Cancer Institute, Hershey, PA, USA
Edwina Moore, MD Department of Endocrine Surgery, The Cleveland
Madison Morgan Department of Trauma and Acute Care Surgery, Penn
Morgan K. Moroi, BS Department of Surgery, Penn State Milton S. Hershey
Ruel Neupane, MD Department of Surgery, University Hospitals Cleveland
Joseph R. Notaro, MD, FACS, FASCRS Department of Surgery, Rutgers
Robert Wood Johnson Medical School, New Brunswick, NJ, USA
James M. O’Brien, MD Maternal Fetal Medicine, Department of Obstetrics
and Gynecology, Penn State Health, Milton S. Hershey Medical Center,
Hershey, PA, USA
xxx Contributors
Elif Onursal, DO, MS Department of General Surgery, St. Barnabas

Hospital Health System, Bronx, NY, USA
Heidi N. Overton, MD Department of Surgery, Johns Hopkins Hospital,
Baltimore, MD, USA
Colette R. Pameijer, MD Department of General Surgery, Penn State
Rosa Park, MD Division of Urology, Penn State Milton S. Hershey Medical
Dan W. Parrish, MD Department of Pediatric Surgery, Batson Children’s
Hospital, University of Mississippi Medical Center, Jackson, MS, USA
Albert G. Pavalonis, DO Department of Vascular Surgery, NYU Langone
Eric M. Pauli, MD, FACS, FASGE Department of Surgery, Penn State
Jaimey M. Pauli, MD Maternal-Fetal Medicine, Department of Obstetrics
and Gynecology, Penn State Health Milton S. Hershey Medical Center,
Hershey, PA, USA
Todd A. Ponsky, MD, FACS Division of Pediatric Surgery, Akron Children’s
Josh Radtka, MD Division of Vascular Surgery, Penn State Milton
J. Chris Riney, MD Division of Urology, Penn State Milton S. Hershey
Michele A. Riordon, MD, FRCSC Department of Surgery, Royal Victoria
Regional Health Centre, Barrie, ON, Canada
Hadley E. Ritter, MD Department of Surgery, Indiana University,
Indianapolis, IN, USA
Ann M. Rogers, MD Department of Surgery, Penn State Milton S. Hershey
William Sangster, MD Department of Surgery, Penn State Milton S. Hershey
Ariel P. Santos, MD, MPH, FRCSC, FACS Department of Surgery, Texas
Tech University Health Sciences Center, Lubbock, TX, USA
Samer Sbayi, MD, MBA, FACS Department of General Surgery, Stony
Brook University Hospital, Stony Brook, NY, USA
Freya Schnabel, MD Department of Surgery, NYU Langone Health, NYU
Robert S. Schoaps, MD Department of Anesthesiology and Perioperative
Medicine, Penn State Hershey Medical Center, Hershey, PA, USA
Contributors xxxi
Zachary J. Senders, MD Department of Surgery, University Hospitals

Anish Shah, MD Department of Surgery, Stony Brook University Hospital,
Jonathan G. Sham, MD Department of Surgery, Johns Hopkins Hospital,
Baltimore, MD, USA
Kathryn W. Shaw, MD Department of Surgery, Division of Transplant
Surgery, University of Washington Medical Center, Seattle, WA, USA
Tom Shokri, MD Division of Otolaryngology-Head and Neck Surgery,
Hershey, PA, USA
Laila Siddique, BA Division of Otolaryngology-Head and Neck Surgery,
Hershey, PA, USA
Department of Otolaryngology-Head and Neck Surgery, University of Miami
Miller School of Medicine, Miami, FL, USA
Rachel E. Simpson, MD Department of Surgery, Indiana University,
Jasvinder Singh, MD Department of Surgery, Program for Liver, Pancreas,
and Foregut Tumors, Penn State College of Medicine, Penn State Cancer
Institute, Hershey, PA, USA
Emily Smith, MD Maternal-Fetal Medicine, Department of Obstetrics and
Gynecology, Medical College of Wisconsin, Milwaukee, WI, USA
Michael Smith, DO Department of Surgery, Westchester Medical Center,
Valhalla, NY, USA
Cheyenne C. Sonntag, MD, MS Department of Surgery, Penn State Milton
Konstantinos Spaniolas, MD Department of Surgery, Stony Brook
Ryan M. Staszak, MD Department of Surgery, Division of Trauma, Acute
Care, and Critical Care Surgery, Penn State Milton S. Hershey Medical
David B. Stewart Jr., MD, FACS, FASCRS Department of Surgery, Banner
University Medical Center – Tucson, Tucson, AZ, USA
Michael G. Svestka, MD Department of Surgery, Stony Brook University
Henry Tannous, MD Department of Surgery, Stony Brook University
Department of Cardiovascular Surgery, Icahn School of Medicine at Mount
Sinai, New York, NY, USA
xxxii Contributors
Anthony R. Tascone, MD Department of General Surgery, Saint Luke’s

Health System, Kansas City, MO, USA
Matthew D. Taylor, MD Department of Surgery, Penn State Health Milton
Division of Thoracic Surgery, Penn State Health Milton S. Hershey Medical
Ye Tian, BA Penn State Health Milton S. Hershey Medical Center, Hershey,
PA, USA
Anjali R. Thawani, MD Division of Surgical Oncology, AMITA Health
System, Elk Grove Village, IL, USA
Scott C. Tiedebohl, MD Department of Surgery, Penn State Health Milton
Division of Thoracic Surgery, Penn State Health Milton S. Hershey Medical
Joel VanderVelde, MD Department of Surgery, Maricopa Integrated Health
System, Phoenix, AZ, USA
Fausto Vinces, DO, FACS, FICS Department of Surgery, Vassar Brothers
Medical Center, Poughkeepsie, NY, USA
Danielle Von Nieda Department of Trauma and Acute Care Surgery, Penn
Matthew J. Weiss, MD Department of Surgery, Johns Hopkins Hospital,
Baltimore, MD, USA
Kirsten Bass Wilkins, MD, FACS, FASCRS Department of Surgery,
Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA
Matthew Z. Wilson, MD, MSc Department of Surgery, Dartmouth
Hitchcock Medical Center, One Medical Center Drive, Lebanon, NH, USA
Christina L. Wolchok, DO Graduate Medical Education, Department of
General Surgery, Stony Brook University Hospital, Stony Brook, NY, USA
Joyce Wong, MD Department of General Surgery, Lenox Hill Hospital,
New York, NY, USA
Maroun B. Yammine, MD Department of Cardiovascular Surgery, Icahn
School of Medicine at Mount Sinai, New York, NY, USA
Part I
Skin and Soft Tissue
Management of Cutaneous
Melanoma 1
Algorithmic Approach and pelvis with IV contrast or full body PET/

CT, and consider brain MRI. If there is evi-
A. The history and physical examination should dence of metastasis, the patient should consider
include risk factors for melanoma and family systemic therapy or a clinical trial. If there is no
history. The lesion should be evaluated by the evidence of metastasis, the patient should be
ABCDE criteria: A, asymmetry; B, borders; offered a completion lymphadenectomy and
C, color; D, diameter; E, evolution. If the consider systemic therapy or a clinical trial.
lesion meets any one of these criteria, it E. If at the time of diagnosis there is suspicion for
should be biopsied. metastatic disease or palpable lymph nodes on
B. Key pathologic criteria that impact stage and exam, the patient should undergo staging
treatment include depth, ulceration, mitotic rate, imaging including either a CT chest, abdomen,
and satellitosis. Lesions ≤ 0.8 mm deep without pelvis with IV contrast or a full body PET/CT
ulceration or mitoses are considered low-risk as well as fine needle aspiration or core biopsy
lesions. These patients should undergo wide of suspicious nodes. If the disease is contained
local excision (WLE) with a 1 cm margin. There in the nodes, the patient should undergo wide
is no consensus regarding high-risk features in a local excision with lymph node dissection and
thin melanoma.* be considered for systemic therapy or a clinical
C. High-risk lesions should undergo WLE with a trial postoperatively. If the disease is meta-
1–2 cm margin and sentinel lymph node biopsy. static at the time of diagnosis, the patient
Sentinel lymph node biopsy should consist of should receive systemic therapy or enroll in a
preoperative lymphoscintigraphy and intraop- clinical trial. Limited metastatic disease may
erative use of gamma probe with or without be resected in carefully selected patients.
vital blue dye injection, followed by meticu- F. Follow-up care: Patient should have a com-
lous pathologic exam of sentinel lymph node. plete skin exam every 3–12 months for the
D. If the sentinel node is positive for malignancy, first 5 years and at least annually for life.
the patient should undergo staging workup, Imaging should be performed for signs or
including either a CT of the chest, abdomen, symptoms of metastasis in any patient and
considered every 3–12 months for 3 years for
stage IIB-IV. Patient should be educated about
J. A. DiSano · C. R. Pameijer (*)
Department of General Surgery, Penn State Milton
sun protection and self-examination.
S. Hershey Medical Center, Hershey, PA, USA *NCCN should be visited regularly for updated
e-mail: [email protected] guidelines.
© Springer Nature Switzerland AG 2019 3

S. Docimo Jr., E. M. Pauli (eds.), Clinical Algorithms in General Surgery,
https://doi.org/10.1007/978-3-319-98497-1_1
4 J. A. DiSano and C. R. Pameijer
• History and Physical Exam: pigmented cutaneous lesion

A • Complete skin exam and nodal exam
• Biopsy
Primary Melanoma with

melanoma suspicion of mets,
palpable LN E
B
• Staging imaging
Low High or • FNA/core biopsy of suspicious
WLE with low nodes or metastatic lesions
1 cm margin risk?
C High Metastatic Regional

disease disease
WLE with 1-2 cm margin and

sentinel lymph node biopsy Systemic chemotherapy WLE and
or clinical trial lymph node dissection
Consider systemic
Sentinel node Sentinel node chemotherapy or
negative for positive for
malignancy malignancy
D clinical trial
Staging imaging
Regional Metastatic
disease disease
Completion Consider systemic chemotherapy

lymphadenectomy or clinical trial
Follow-up care: Regular complete skin exam and sun protection education F
Algorithm 1.1
Suggested Reading
National Comprehensive Cancer Network. NCCN clinical
practice guidelines in oncology: melanoma (version
1.2017). https://www.nccn.org/professionals/physi-
cian_gls/pdf/melanoma.pdf. Accessed 17 July 2017.
Basal Cell Carcinoma
2
Algorithmic Approach D. The lesion should be risk stratified to deter-

mine if there is a high risk or low risk for recur-
A. The first step in the evaluation of a patient rence. High-risk lesions have any of the
with a suspicious skin lesion is the history following features: Location on mask areas of
and physical examination. The typical appear- the face, genitalia, hands, or feet; lesion greater
ance of basal cell carcinoma is a pearly, flesh- than 20 mm on the trunk or extremities; lesion
colored bump on sun-exposed skin. A greater than 10 mm on the cheeks, forehead,
complete skin exam should be performed as scalp, neck, and feet; poorly defined borders;
well as a nodal exam. The lesion should be recurrent disease; patient on immunosuppres-
biopsied; lymph nodes should only be biop- sion; lesion at the site of prior radiation ther-
sied if palpable on exam. apy; perineural involvement; and pathology
B. Once diagnosis of basal cell carcinoma is showing aggressive growth pattern.
established, surgical excision is the treatment E. Patients with high-risk lesions should be con-
of choice. The choice of Mohs micrographic sidered for further treatment with radiation
surgery or surgical excision is based on size, therapy. Addition of a hedgehog pathway
location, cosmesis, and patient comorbidities. inhibitor (vismodegib or sonidegib) should
Excision should achieve at least a 4 mm also be considered.
margin. F. Follow-up care: Patient should have a com-
C. If the lesion is not able to be excised, the plete skin exam every 6–12 months for the
patient should undergo radiation therapy. first 5 years and at least annually for life.
Treatment with a hedgehog pathway inhibitor Patient should be educated about sun protec-
(vismodegib or sonidegib) can be considered. tion and self-examination.

e-mail: [email protected]

History and Physical Exam:

Suspicious skin lesion
A
• Complete skin exam and nodal exam
• Biopsy
Basal Cell
Carcinoma
Candidate No Consider radiation therapy or

B for surgical hedgehog pathway inhibitor
excision?
Yes
Surgical excision
+/- Mohs
High or
D low High risk
risk?
Low risk Consider radiation therapy or

hedgehog pathway inhibitor
Follow-up care: Regular complete skin exam

F and sun protection education
Algorithm 2.1
Suggested Reading
practice guidelines in oncology: basal cell skin cancer
(version 1.2017). https://www.nccn.org/professionals/
physician_gls/pdf/nmsc.pdf. Accessed 17 July 2017.
Squamous Cell Carcinoma
3
Algorithmic Approach If excision is not possible, the patient should

undergo radiation therapy with or without
A. The typical appearance of squamous cell car- systemic chemotherapy.
cinoma is an ulcerated cutaneous lesion. A D. The lesion should be risk stratified to deter-
complete skin exam should be performed as mine risk of recurrence. High-risk lesions
well as a nodal exam. The lesion should be have any of the following features: Location
biopsied. on mask areas of the face, genitalia, hands, or
B. Any palpable lymph nodes should be further feet; lesion greater than 20 mm on trunk or
evaluated with fine needle aspiration or core extremities; lesion greater than 10 mm on the
needle biopsy. If the nodes are positive for cheeks, forehead, scalp, neck, and feet; poorly
malignancy, a staging CT of the chest, abdo- defined borders; recurrent disease; patient is
men, and pelvis should be done. If disease is immunosuppressed; lesion at site of prior
limited to the regional lymph nodes, the radiation therapy or chronic inflammation;
patient should have wide excision of the pri- perineural or lymphovascular involvement;
mary site and lymphadenectomy. and lesion with greater than 2 mm depth.
C. If there are no palpable nodes or the node E. Patients with high-risk lesions should be con-
biopsy shows no evidence of malignancy, sur- sidered for further treatment with radiation
gical excision should be performed. If the therapy.
lesion is amenable to excision (based on size, F. Follow-up care: Patients should have a com-
location, patient comorbidities, cosmesis, and plete skin exam every 3–12 months for the
need for reconstruction), it should be excised first 2 years, then every 6–12 months for
with 4–6 mm margins. If margins are posi- 3 years, and at least annually for life. Patient
tive, patient should undergo Mohs micro- should be educated about sun protection and
graphic surgery to achieve negative margins. self-examination.



Skin lesion that is ulcerated.
A
· Complete skin exam and nodal exam
· Biopsy
Squamous cell
carcinoma
Palpable Yes FNA or core needle

nodes? biopsy of node
No
Benign Malignant
C
No Candidate
Consider radiation Staging CT
for surgical
therapy Excision of primary and
excision?
lymph node dissection if
limited to regional disease.
Yes
High or
D low High risk
risk?
E
Consider radiation therapy
Low risk
Follow-up care: Regular complete skin exam

F and sun protection education
Algorithm 3.1
Suggested Reading
practice guidelines in oncology: squamous cell skin
cancer (version 1.2017). https://www.nccn.org/pro-
fessionals/physician_gls/pdf/squamous.pdf. Accessed
17 July 2017.
Management of Soft Tissue
Sarcoma 4
Algorithmic Approach should be reassessed for possible resection

based on their response to treatment.
A. Patients with sarcoma typically present with a D. Patients with low-grade or resectable tumors
painless mass that is increasing in size. The should undergo margin-negative resection.
history obtained from the patient should The biopsy site should be resected en bloc with
include duration of mass, changes over time, the surgical specimen. Postoperative radiation
symptoms related to the mass, and any his- therapy should be considered based on the
tory of radiation to the area. Patients often tumor grade, size, and margin status. Positive
report a history of trauma, which is not caus- margins should be re-excised if possible.
ative but likely draws attention to the area. E. Patients with high-grade or borderline resectable
B. Imaging of the lesion should be obtained with tumors should undergo neoadjuvant therapy
either MRI or CT scan. Biopsy should be prior to resection; this can consist of radiation,
obtained to establish histologic subtype and chemotherapy, or both. Following treatment,
grade. Percutaneous biopsy is acceptable. they should be reassessed with imaging, and a
Patients should undergo chest imaging for margin-negative resection should be performed
staging with either chest X-ray or CT. if possible. Attempts should be made to spare
C. Once the diagnosis of sarcoma is estab- limb function if possible. After resection, con-
lished, it should be determined if the lesion sider further therapy with radiation or chemo-
is low or high grade and if it is resectable. therapy, depending on preoperative treatment.
Patients with rhabdomyosarcoma should be F. Follow-up care: Patients should be followed
referred to a specialty center for further with complete history and physical every
management. Patients with unresectable 3–6 months for 3–5 years. They should
tumors, desmoid tumors, or Ewing’s sar- undergo surveillance with local imaging of
coma should be referred for chemotherapy the primary site as well as chest imaging for
and/or radiation. Following treatment, they evidence of metastasis.



A Painless mass, increasing in size.
B · Imaging with MRI or CT

· Image guided biopsy
C Refer to
Sarcoma Rhabdomyosarcoma
specialty center
D E
· Low grade · High grade · Unresectable

· Resectable · Borderline resectable · Desmoid
· Ewings
Margin negative Neoadjuvant

Systemic chemotherapy
resection therapy
and radiation
Consider radiation Reassess with Consider resection based

therapy imaging (MRI/CT) on response to treatment
Margin negative
resection
Follow-up Care:
F Local imaging of primary site
and chest imaging
Algorithm 4.1
Suggested Reading
National Comprehensive Cancer Network. NCCN clini-
cal practice guidelines in oncology: sarcoma (version
1.2017). https://www.nccn.org/professionals/physi-
cian_gls/pdf/sarcoma.pdf. Accessed 17 July 2017.
Necrotizing Soft Tissue Infection
5
Algorithmic Approach patient should be started on broad-spectrum

antibiotics, including coverage for gram-
A. Patients with necrotizing soft tissue infec- positive cocci, gram-negative rods, and anaer-
tions (NSTI) will often have a history of obes, including clostridial species (penicillin
obesity, diabetes, immunosuppression, or G, vancomycin, gentamicin, clindamycin).
recent surgery or trauma. A hallmark is NSTI is a surgical emergency, and nonopera-
pain out of proportion to the physical exam tive management is associated with almost
findings. They may have fever, tense edema, 100% mortality [4]. Excision should extend
bullae, ecchymosis or necrosis of the skin, to healthy, bleeding tissue at all margins and
cutaneous anesthesia, and evidence of sys- tissue should be sent for culture.
temic toxicity. NSTIs most commonly D. Patients typically require ongoing critical

involve the extremities, perineum, and gen- care support after surgery, including resusci-
italia [1, 2]. tation, broad-spectrum antibiotics, and man-
B. Laboratory analysis should include a CBC, agement of comorbidities. They should return
complete metabolic panel, and CRP. The to the operating room within 24 h for further
LRINEC (laboratory risk indicatory for nec- evaluation, and debridement if necessary.
rotizing fasciitis) can aid in decision-making. Patients with necrotizing soft tissue infec-
This score includes WBC (1 point for WBC tions will likely require serial debridement
15–25, 2 points for WBC >25), hemoglobin with most patients requiring 3–4 operative
(1 point for Hgb 11–13.5, 2 points for Hgb debridements.
<11), CRP (4 points for CRP ≥150), sodium E. Patients with lower index of suspicion can be
(2 points for Na <135), glucose (1 point for evaluated by bedside debridement. If the sur-
glucose >180), and creatinine (2 points for geon is easily able to slide a finger along the
Cre >1.6). If the LRINEC score is ≥6, there is fascial plane and finds evidence of necrotic
a high likelihood of NSTI [3]. tissue or “dish water fluid” on bedside I&D,
C. The patient should be appropriately resusci- the patient should be taken to the OR for sur-
tated. NSTIs are often polymicrobial, and the gical debridement.
F. Follow-up care: Patients will require a multi-
disciplinary team to aid in recovery. They will
J. A. DiSano · C. R. Pameijer (*) require rehabilitation, wound management,
Department of General Surgery, Penn State Milton and possible flap coverage.

History:
A Pain, cellulitis, history of obesity, diabetes, or
immunosuppression.
Obtain vital signs, blood work, and perform physical exam
Calculate LRINEC score

B
Yes
OR for debridement
C
High
suspicion
for NSTI?
D
Yes
No
Broad, spectrum antibiotics,

resuscitation, serial
debridement
E Bedside I&D
reveals
NSTI?
No
Broad, spectrum antibiotics,

IVF resuscitation, close
observation
Follow-up Care: Wound management, flap coverage,

F and rehabilitation
Algorithm 5.1
5 Necrotizing Soft Tissue Infection 13
References PubMed PMID: 25069713; PubMed Central PMCID:

PMCPMC4199388.
3. Wong CH, Khin LW, Heng KS, Tan KC, Low
1. Bonne SL, Kadri SS. Evaluation and management of
CO. The LRINEC (laboratory risk indicator for
necrotizing soft tissue infections. Infect Dis Clin N
necrotizing fasciitis) score: a tool for distinguishing
Am. 2017;31(3):497–511. https://doi.org/10.1016/j.
necrotizing fasciitis from other soft tissue infections.
idc.2017.05.011.
Crit Care Med. 2004;32(7):1535–41. PubMed PMID:
2. Hakkarainen TW, Kopari NM, Pham TN, Evans
15241098.
HL. Necrotizing soft tissue infections: review and
4. Anaya DA, Dellinger EP. Necrotizing soft-tissue
current concepts in treatment, systems of care, and
infection:diagnosis and management. Clin Infect Dis.
outcomes. Curr Probl Surg. 2014;51(8):344–62.
2007;44(5):705. Pubmed PMID: 17278065.
https://doi.org/10.1067/j.cpsurg.2014.06.001.
Part II
Head and Neck
Management of Squamous Cell
Carcinoma of the Oropharynx 6
Algorithmic Approach tongue, and posterior oropharyngeal wall.

Lesions may appear exophytic, flat, ulcerated,
A. The first step in the evaluation of a patient with verrucoid, or papillary. Fiberoptic endoscopy
suspected oropharyngeal squamous cell carci- should be performed in the office to visualize
noma (OPSCC) is the history and physical the extent of the lesion or to search for syn-
examination. Questions regarding the presence chronous lesions. Cranial nerve IX-XII func-
of dysphagia, odynophagia, oral bleeding, tion should be assessed for possible neurotropic
otalgia, change in speech, airway compromise, involvement and palpation of the neck should
B symptoms, and use of tobacco or alcohol be performed for irregular lymph nodes [1].
raise clinical suspicion for malignancy. A sex- C. Patients with clinical findings suspicious for
ual history should be obtained to assess the OPSCC should undergo a contrast-
likelihood of HPV-associated OPSCC [1]. enhanced CT or MRI of the head and neck
B. A thorough physical exam involves inspection for precise evaluation, staging, and treat-
and palpation of the soft palate, tonsils, base of ment planning [2]. Patients with lymph
node irregularities and an unknown primary
L. Siddique lesion should first undergo an ultrasound-
Division of Otolaryngology-Head and Neck Surgery, guided fine needle aspiration of the node,
Department of Surgery, Penn State Milton S. Hershey
followed by imaging if the results are posi-
tive for malignancy [3].
Department of Otolaryngology-Head and Neck
D. Definitive diagnosis requires histopathologi-
Surgery, University of Miami Miller School of
Medicine, Miami, FL, USA cal evaluation of a tissue sample. Biopsies of
e-mail: [email protected] the tonsil and soft palate can be performed in
T. Shokri the office, whereas difficult to reach regions
Division of Otolaryngology-Head and Neck Surgery, may require rigid endoscopy under general
Department of Surgery, Penn State Milton S. Hershey anesthesia for biopsy [1, 4].
E. Once the diagnosis of OPSCC is confirmed and
N. Goyal (*) staged, PET/CT or chest CT should be per-
Division of Otolaryngology-Head and Neck Surgery,
formed to evaluate for metastatic disease [5]
Medical Center, Hershey, PA, USA and stage-dependent treatment discussed with
a head and neck surgeon, including surgical
Penn State Cancer Institute, Penn State Milton
S. Hershey Medical Center, Hershey, PA, USA resection with possible reconstruction, radio-
e-mail: [email protected] therapy, and/or chemotherapy [1, 6].

18 L. Siddique et al.
F. Postoperative maintenance of the three major initiated. A temporary tracheotomy may

functions of the oropharynx (swallowing, rarely be required to maintain a patent air-
speech, and airway) is vital. Nasogastric way. All patients should maintain adequate
tubes can be used for feeding in smaller oral hygiene with regular suctioning and use
defects (with an expected temporary need), of antiseptic mouthwashes. Patients should
gastrostomy tube feeding with larger defects, regularly be monitored for detection of pos-
and those requiring adjuvant radiotherapy. sible recurrence and subsequent early inter-
Swallow and speech rehabilitation should be vention [1].
A History and Physical Exam:

Dysphagia, odynophagia, bleeding, otalgia, and change in speech
Social Hx: alcohol, tobacco, sexual history
Inspection and palpation of soft palate, tonsils, base of tongue, posterior oropharyngeal wall,
+/- Flexible fiberoptic endoscopy, cranial nerve exam IX-XII, bimanual palpation of neck for lymphadenopathy
Suspicious lesion Irregular lymph node with

+/- lymphadenopathy unknown primary
Ultrasound-guided FNA:
Suspicious for malignancy
C CT/MRI of the head and neck for evaluation, staging, and treatment planning
Does tumor
invade local
structures?
Do neck lymph
nodes enhance?
D Biopsy primary lesion in the office or with rigid endoscopy under anesthesia
Diagnosis of Oropharyngeal
SCC E
T1-T2 (cancer </= 4 cm) T3-T4a ( >4 cm-local invasion) Unresectable (T4b +)
or or or
1º resection, Radiotherapy 1º resection,

Chemoradiation Chemoradiation Palliation
selective ND +/- chemo bilateral ND
RT +/- chemo Salvage surgery RT +/- chemo

if high risk if high risk
Follow-up: Oral hygiene, NG /G-tube, speech rehab, +/-tracheostomy, and regular follow-up F
Algorithm 6.1
6 Management of Squamous Cell Carcinoma of the Oropharynx 19
References 4. Lewis JS, Thorstad WL, Chernock RD, et al. p16
positive oropharyngeal squamous cell carcinoma:
an entity with a favorable prognosis regard-
1. Flint PW, Cummings WC. Malignant neoplasms of
less of tumor HPV status. Am J Surg Pathol.
the oropharynx. In: Cummings otolaryngology: head
2010;34(8):1088–96.
and neck surgery. Philadelphia: Elsevier, Saunders;
5. Quon A, Fischbein NJ, McDougall IR, et al. Clinical
2015.
role of F-FDG PET/CT in the management of squa-
2. Wippold FJ. Head and neck imaging: the role of CT
mous cell carcinoma of the head and neck and thyroid
and MRI. J Magn Reson Imaging. 2007;25:453.
carcinoma. J Nucl Med. 2007;48(Suppl 1):58S–67S.
3. Righi PD, Kopecky KK, Caldemeyer KS, et al.
6. Yousem DM, Gad K, Tufano RP. Resectability
Comparison of the ultrasound- fine needle aspiration
issues with head and neck cancer. Am J Neuroradiol.
and computed tomography in patients undergoing elec-
2006;27(10):2024–36.
tive neck dissection. Head Neck. 1997;19(7):604–10.
Evaluation of Neck Mass
7
Algorithmic Approach symptoms of infection including symptoms

of fever, chills, fluctuance, erythema, tender-
A. A thorough history and physical exam remain ness, or warmth should prompt treatment
the mainstay of initial evaluation of a neck with antibiotics and re-evaluation.
mass. Key red flag symptoms to delineate B. A detailed head and neck examination is criti-
include rapid growth, dysphagia, weight loss, cal. Mobility, tenderness, location within the
otalgia, aural fullness, hearing loss, hemopty- neck, firmness, fluctuance, erythema, and
sis, epistaxis, paresthesia, and dyspnea [1, 2]. palpable bruits are some of the features that
Ninety percent of pediatric neck masses are should be initially noted. The aerodigestive
benign lesions, related to an inflammatory tract must be evaluated through palpation and
response or congenital anomaly. With the visualization of the floor of mouth, oral
exclusion of thyroid masses, 80% of adult tongue, palate, tonsils, base of tongue, buccal
neck masses are malignant [3]. Signs and mucosa, nasopharynx, oropharynx, larynx,
and nasal cavity [4].
C. Computed tomography (CT) is the most com-
T. Shokri monly utilized imaging modality. To avoid
Division of Otolaryngology-Head and Neck Surgery, ionizing radiation, ultrasonography may be
initially useful in the pediatric population as
they have a higher likelihood of an inflamma-
L. Siddique
tory, infectious, or congenital process [5].
Department of Surgery, Penn State Milton S. Hershey Ultrasonography should also be considered
Medical Center, Hershey, PA, USA for thyroid masses, as use of CT with iodine
Department of Otolaryngology-Head and Neck contrast can delay potential radioiodine treat-
Surgery, University of Miami Miller School of ment. MRI is useful in delineating soft tissue
Medicine, Miami, FL, USA anatomy, particularly if perineural disease is
suspected. CTA or MRA can be considered if
N. Goyal (*) there is suspicion for a vascular lesion.
D. Fine needle aspiration (FNA) or core needle
Medical Center, Hershey, PA, USA biopsy may be performed. If this does not
yield a diagnosis, an excisional biopsy may
S. Hershey Medical Center, Hershey, PA, USA be performed. However, the surgeon must be
e-mail: [email protected] cognizant of the risk of tumor seeding.

22 T. Shokri et al.
E. If the lesion is found to be infectious, conser- head and neck oncologic surgeon for further
vative therapy with antibiotic treatment and workup for staging and appropriate
possible steroids may be administered while management.
congenital lesions should undergo observa-
tion with reassessment for surgical excision.
Neoplastic lesions should be referred to a
A-B History and physical
Signs of Yes
Antibiotics with re-
infection evaluation
No
Diagnostic Imaging
CT: Standard
C CTA/MRA: Vascular lesion
MRI: Soft tissue concern
Ultrasound: Pediatric/thyroid
E
D No
Concern for
malignancy? Congenital or inflammatory
Yes
Trial of antibiotics ± steroids.
Biopsy and referral to head Refer to otolaryngologist.
and neck oncologic surgeon
Algorithm 7.1
7 Evaluation of Neck Mass 23
References Cummings otolaryngology: head and neck surgery.

5th ed. Philadelphia: Elsevier; 2010.
4. Kadom N, Lee EY. Neck masses in children: cur-
1. Goff CJ, Allred C, Glade RS. Current management
rent imaging guidelines and imaging findings. Semin
of congenital branchial cleft cysts, sinuses and fis-
Roentgenol. 2012;47:7–20.
tulae. Curr Opin Otolaryngol Head Neck Surg.
5. Layfield L. Fine-needle aspiration in the diagnosis
2012;20:533–9.
of head and neck lesions: a review and discussion of
2. Tracy TF, Muratore CS. Management of common head
problems in differential diagnosis. Diagn Cytopathol.
and neck masses. Semin Pediatr Surg. 2007;16:3–13.
2007;35:798–805.
3. Chen AY, Otto KJ. Differential diagnosis of neck
masses. In: Flint PW, Haughey BH, Lund VJ, editors.
Evaluation of an Enlarged Cervical
Lymph Node 8
Algorithmic Approach mass as well as its rate of growth are impor-

tant, as well as the presence of symptoms of
A. The differential diagnosis of a neck mass
infection and B symptoms. Information about
includes congenital anomalies, inflammatory/ travel and contact with sick individuals or
infectious lymphadenopathy, and malig- animals should be obtained [2].
nancy. Neck masses in children are more B. On physical examination, inspection and
commonly congenital or associated with bimanual palpation of the neck should be
inflammatory lymph nodes, whereas neck carefully performed to assess for location,
masses in adults raise a higher concern for size, tenderness, and symmetry [3]. Hard and
malignancy [1]. A thorough history and phys- tender lymph nodes are associated with infec-
ical exam are first required in evaluation. tions; hard nodes fixed to the skin or deeper
Questions regarding the onset of the neck structures can suggest malignancy; and fluc-
tuant masses can be seen with an abscess or
cyst [4]. A comprehensive physical exam
should also be performed to assess for other
L. Siddique signs of infection, peripheral lymphadenopa-
Division of Otolaryngology-Head and Neck Surgery, thy, or systemic inflammatory processes.
C. If the history and physical exam suggest an
infectious etiology, consider initiating a trial
Department of Otolaryngology-Head and Neck
of oral antibiotics. If symptoms do not begin
Surgery, University of Miami Miller School of
Medicine, Miami, FL, USA to improve within 2–3 days, obtain (1) a neck
e-mail: [email protected] ultrasound to rule out a neck abscess or an
T. Shokri infected congenital cyst [5]; (2) CBC, CRP,
Division of Otolaryngology-Head and Neck Surgery, LDH, and chest radiograph for systemic
Department of Surgery, Penn State Milton S. Hershey inflammatory processes; (3) PPD to rule out
tuberculosis lymphadenitis; (4) serology for
N. Goyal (*) HIV, EBV, CMV, and toxoplasmosis; and (5)
referral to a head and neck surgeon or infec-
Medical Center, Hershey, PA, USA tious disease physician as appropriate.
D. If the history and physical exam are suggestive
S. Hershey Medical Center, Hershey, PA, USA of a malignant process, or if infectious and
e-mail: [email protected] inflammatory etiologies have been ruled out, a

26 L. Siddique et al.
CBC, blood smear, and fine needle aspiration secondary to drug reactions, a developmental
should be performed to assess for malignancy mass such as a thyroglossal duct cyst or der-
and atypical mycobacterial infection, followed moid cyst, vascular malformation, or benign
by referral to a head and neck surgeon, oncolo- processes. In this situation, it is appropriate to
gist, or infectious disease internist [6]. observe and follow-up with the patient in
E. If a total diagnostic workup is negative and 4–6 weeks [5], with referral to a head and
the patient is otherwise asymptomatic, the neck surgeon if the mass enlarges during this
etiology may be cervical lymphadenopathy time or remains larger than 2 cm [7].
A History and Physical Exam: Rate of growth? Other infectious symptoms?

B symptoms? Social Hx: sick contacts, travel?
Inspection and bimanual palpation of neck: location, size, tenderness, hardness, fixation, cystic?
B Comprehensive physical exam with HEENT, cardiovascular, pulmonary, abdominal evaluation.
Signs of infection:
C YES
tenderness and
NO
erythema of node, D
chills, fatigue?
Trial of oral antibiotics Developmental mass

suspected?
E
NO
YES
CBC, blood smear, FNA/

Improvement in 2-3 days? excisional biopsy Developmental
Referral to ENT mass suspected?
YES NO
YES
• Ultrasound with reflex CT for
NO
abscess vs. cyst
Complete 10 • CBC, CRP, LDH, CXR for
daycourse inflammatory process Consider LAD
• HIV, EBV, CMV, toxo serology secondary to drug
Referral to ENT
reaction or benign
etiology
Positive results?
YES NO Observe for 4-6
weeks
Treat and follow up Evaluate for possible

with ENT and/or malignancy (see D) Refer to ENT if
infectious disease as node increases in
appropriate size or remains
>2 cm
Algorithm 8.1
8 Evaluation of an Enlarged Cervical Lymph Node 27
References 4. Acierno SP, Waldhausen JH. Congenital cervical

cysts, sinuses and fistulae. Otolaryngol Clin N Am.
2007;40(1):161–76. vii–viii.
1. Connolly AA, MacKenzie K. Paediatric neck
5. Leung AK, Robson WL. Childhood cervical lymph-
masses—a diagnostic dilemma. J Laryngol Otol.
adenopathy. J Pediatr Health Care. 2004;18(1):3–7.
1997;111(6):541–5.
6. Anne S, Teot LA, Mandell DL. Fine needle aspi-
2. Bauer PW, Lusk RP. Neck masses. In: Bluestone CD,
ration biopsy: role in diagnosis of pediatric head
Stool SE, Alper CM, et al., editors. Pediatric otolaryngol-
and neck masses. Int J Pediatr Otorhinolaryngol.
ogy. 4th ed. Philadelphia: Saunders; 2003. p. 1629–47.
2008;72(10):1547–53.
3. Torsiglieri AJ Jr, Tom LW, Ross AJ III, Wetmore RF,
7. Dickson PV, Davidoff AM. Malignant neo-
Handler SD, Potsic WP. Pediatric neck masses: guide-
plasms of the head and neck. Semin Pediatr Surg.
lines for evaluation. Int J Pediatr Otorhinolaryngol.
2006;15(2):92–8.
1988;16(3):199–210.
Salivary Gland Tumors
9
Algorithmic Approach C. Diagnostic imaging is warranted if there is a

concern for extragrandular extension.
A. The initial step in evaluation is a detailed history Ultrasonography is an appropriate initial step
and physical. The most common presenting in evaluation. Computed Tomography (CT)
symptom of a major salivary gland neoplasm is and Magnetic Resonance Imaging (MRI) are
asymptomatic swelling. Pain is more com- useful for further delineating anatomy [3].
monly reported in malignant lesions and may D. Fine needle aspiration cytology (FNAC) is rec-
suggest perineural invasion (PNI) [1]. ommended for major salivary gland lesions,
B. Physical findings that increase the index of allowing for tissue diagnosis. Core needle biopsy
suspicion for malignancy include facial pare- (CNB) yields greater diagnostic accuracy.
sis, cervical lymphadenopathy, and fixation However, CNB is more painful and for parotid
of mass to overlying skin or deep structures masses risks injury to the facial nerve [4, 5].
[2]. Lymphadenopathy is seen on presenta- E. Standard of care for benign lesions continues
tion in 10–30% of cases. to be removal, particularly if causing symp-
toms or growing in size. However, a subset of
T. Shokri patients may choose watchful waiting.
Division of Otolaryngology-Head and Neck Surgery, F. If positive for malignancy, staging, according
to AJCC guidelines [6], should be performed
for both prognostic measures and to guide
L. Siddique
management strategy.
Department of Surgery, Penn State Milton S. Hershey G. Surgery remains the gold standard for primary
Medical Center, Hershey, PA, USA and regionally metastatic cancer of the salivary
Department of Otolaryngology-Head and Neck Surgery, glands. Patients with clinically positive nodal
University of Miami Miller School of Medicine, disease, T3/T4 staging, or high-grade histol-
Miami, FL, USA ogy should undergo neck dissection (ND).
H. Adjuvant radiotherapy should be considered
N. Goyal (*) in cases of high grade, T3/T4, close (<2 mm)/
positive margins, PNI, recurrent disease, or
Medical Center, Hershey, PA, USA positive cervical lymph nodes following neck
dissection. Chemotherapy may be considered
S. Hershey Medical Center, Hershey, PA, USA as palliative treatment in unresectable or dis-
e-mail: [email protected] tantly metastatic disease.

30 T. Shokri et al.

A Vague asymptomatic swelling of the neck, pain, gland obstruction/sialadenitis
Palpable Mass
Assess for facial paresis, cervical lymphadenopathy, and fixation of

B mass to overlying skin or deep structures
Findings
concerning for
malignancy?
C Diagnostic Imaging: Ultrasonography, CT, or MRI
D Obtain Tissue Sample: FNAC or CNB
E/F Benign lesion Malignant lesion
· Surgical excision
G/H · Observant management
· Surgical excision · ND and adjuvant radiation if nodal
disease, T3/T4, high grade, and + margin
Algorithm 9.1
9 Salivary Gland Tumors 31
References a systematic review. J Oral Maxillofac Surg.

2010;68:2146–53.
5. Schmidt RL, Hall BJ, Wilson AR, et al. A system-
1. Spiro RH, Hajdu SI, Strong EW. Tumors of the sub-
atic review and meta-analysis of the diagnostic
maxillary gland. Am J Surg. 1976;132:463–8.
accuracy of ultrasoundguided core needle biopsy
2. Terhaard C, Lubsen H, Tan B, et al. Facial nerve func-
for salivary gland lesions. Am J Clin Pathol. 2011;
tion in carcinoma of the parotid gland. Eur J Cancer.
136:516–26.
2006;42:2744–50.
6. Edge SB, Byrd DR, Compton CC, Fritz AG, Greene
3. Lee YY, Wong KT, King AD, Ahuja AT. Imaging of
FL, Trotti A, editors. AJCC cancer staging manual.
salivary gland tumors. Eur J Radiol. 2008;66:300–4.
7th ed. New York: Springer; 2010.
4. Colella G, Cannavale R, Flamminio F, et al. Fine-
needle aspiration cytology of salivary gland lesions:
Part III
Thoracic
Massive Hemoptysis
10
Henry Tannous, Joanna Chikwe,
and Maroun B. Yammine
Algorithmic Approach C. After the initial stabilization, a bronchos-

copy is warranted. The patient should be
A. Other sources of bleeding like nasopharyngeal intubated with a large ET tube (size [>8])
and upper GI bleeding can present as hemop- to allow for an interventional bronchos-
tysis and have to be ruled out. A true massive copy. Flexible bronchoscopy is key to
hemoptysis will be defined in this chapter as evacuate clots, identify the source, and
life-threatening blood loss from the airways control the bleeding. Cold saline, epineph-
causing respiratory distress or hemodynamic rine wash, and direct pressure (with the
instability. Usually the rate of blood loss would scope or balloon) are commonly used
exceed 100 cc/h or 500 cc/24 h [1, 2]. effective modalities. Other possible modal-
B. The priority is to secure the airway to main- ities include cryoablation, laser therapy,
tain gas exchange and support the hemody- and procoagulants application. [Rigid
namics as needed. If a specific side is bronchoscopy can also be used; it provides
suspected to be the source of bleeding, then better suction but is reserved for proximal
the patient should be positioned in lateral airway bleeding [4, 5]].
decubitus with the suspected bleeding side D1. If the bronchoscopy was successful, further
down to prevent drowning of the unaffected workup and treatment would be directed
side. A more detailed history and physical based on the underlying etiology. Possible
exam should be taken and blood samples etiologies of massive hemoptysis include
sent to rule out anemia, thrombocytopenia, pulmonary infections (fungal, TB, etc.),
and coagulopathy [3]. vasculitis, AVMs, bronchiectasis, tumors,
airway inflammation or ecchymosis, iatro-
genic injuries, and fistulae.
H. Tannous (*) ∙ J. Chikwe D2. If bronchoscopy was unsuccessful, further
Department of Surgery, Stony Brook University actions should be taken to protect and venti-
late the unaffected lung, pending definite
Department of Cardiovascular Surgery, Icahn School treatment. This can be achieved by placing
of Medicine at Mount Sinai, New York, NY, USA
e-mail: [email protected] an endobronchial blocker on the affected
side, switching the ET tube to a double
M. B. Yammine
Department of Cardiovascular Surgery, Icahn School lumen tube, or advancing the ET tube into
of Medicine at Mount Sinai, New York, NY, USA the unaffected side. The latter option is con-

36 H. Tannous et al.
sidered as a last resort since it does not help Tracheo-innominate fistula is a potential cause
tamponade the bleed [3]. of massive hemoptysis in intubated patients or
E. Subsequently, the definitive treatment of air- patients with a tracheostomy. It requires
way (tracheal or bronchial) bleed includes immediate intervention with cuff overinfla-
angiography with embolization. However, a tion, digital compression of the artery against
parenchymal bleed is better treated with the sternum, and transfer to the operating room
lung resection [6–8]. for sternotomy and fistulae repair [9].
A
Massive Hemoptysis: Life-threatening blood loss from airways causing respiratory
distress or hemodynamic instability, >100cc/hr or >500cc/24hrs
Simultaneously
B
- Support hemodynamics
Secure the airway to maintain gas exchange
- Correct coagulopathy
- Position patient in lateral decubitus
(bleeding side down) - Transfuse as needed
- Intubate with large ET tube - Complete Hx and physical exam
(while bronchoscopy is being set up)
C
BRONCHOSCOPY
- Cold saline and epinephrine wash
- Direct pressure
- Cryotherapy/procoagulant application
Is the
bleeding
controlled?
D Yes No
Protect and ventilate unaffected lung

Address underlying etiology
- Endobronchial blocker in bleeding side
- Switch to a double lumen ET tube
- Advance ET tube to unaffected side
E What is the
source of
bleeding?
Airways Parenchyma Tracheoesophageal
Angiography with embolization Lung resection TE fistulae repair
Algorithm 10.1
10 Massive Hemoptysis 37
References choscopy before bronchial artery embolization

for massive hemoptysis. AJR Am J Roentgenol.
2001;177(4):861–7.
1. Ibrahim WH. Massive haemoptysis: the defini-
6. Shigemura N, Wan IY, Yu SC, Wong RH, Hsin MK,
tion should be revised. Eur Respir J. 2008;32.
Thung HK, et al. Multidisciplinary management of
England:1131–2.
life-threatening massive hemoptysis: a 10-year expe-
2. Fartoukh M, Khoshnood B, Parrot A, Khalil A,
rience. Ann Thorac Surg. 2009;87(3):849–53.
Carette MF, Stoclin A, et al. Early prediction of in-
7. Chun JY, Morgan R, Belli AM. Radiological manage-
hospital mortality of patients with hemoptysis: an
ment of hemoptysis: a comprehensive review of diag-
approach to defining severe hemoptysis. Respiration.
nostic imaging and bronchial arterial embolization.
2012;83(2):106–14.
Cardiovasc Intervent Radiol. 2010;33(2):240–50.
3. Jean-Baptiste E. Clinical assessment and man-
8. Andrejak C, Parrot A, Bazelly B, Ancel PY, Djibre
agement of massive hemoptysis. Crit Care Med.
M, Khalil A, et al. Surgical lung resection for severe
2000;28(5):1642–7.
hemoptysis. Ann Thorac Surg. 2009;88(5):1556–65.
4. Sakr L, Dutau H. Massive hemoptysis: an update on
9. Scalise P, Prunk SR, Healy D, Votto J. The incidence of
the role of bronchoscopy in diagnosis and manage-
tracheoarterial fistula in patients with chronic trache-
ment. Respiration. 2010;80(1):38–58.
ostomy tubes: a retrospective study of 544 patients in
5. Hsiao EI, Kirsch CM, Kagawa FT, Wehner JH,
a long-term care facility. Chest. 2005;128(6):3906–9.
Jensen WA, Baxter RB. Utility of fiberoptic bron-
Mediastinal Masses
11
Algorithmic Approach thenia gravis, Horner syndrome, paraneoplas-

tic syndromes, lymphadenopathy, neural
Mediastinal Mass deficit, stridor, dysphagia, or hoarseness [2].
B. A CT with IV contrast will narrow down the
The mediastinum is defined as the area between the differential based on the location of the mass
thoracic inlet, the diaphragm, and the pleural cavi- and evaluate the mass density, size, and inva-
ties laterally. A mediastinal mass is classified sion of surrounding structures.
according to its location [1, 2]. An anterior mass is C. For posterior mediastinal tumors, an MRI may
located between the sternum and the pericardium. be needed to fully assess the neurologic tumor.
The differential diagnosis includes thymomas, tera- A tissue biopsy may be possible to rule other
tomas [and other germ cell tumors], thyroid tumors, etiologies. Neurogenic masses warrant resec-
and lymphomas [3]. A mass in the middle mediasti- tion in conjunction with neurosurgery [4–6].
nal compartment, which houses the visceral organ D. Middle mediastinal tumors are usually cystic
and structures, is usually a cyst [cardiac, broncho- in nature and warrant resection if they are
genic, esophageal]. A posterior mediastinal mass is symptomatic. Bronchoscopic and esophageal
located around the spine and paraspinal structures cysts might need endoscopic studies as part
and is usually a neurogenic tumor. of their evaluation [7, 8].
E. Anterior mediastinal masses require more
A. A mediastinal mass can have local or systemic extensive workup:
symptoms or be completely asymptomatic. An (a) PET/CT to diagnose regional and sys-
extensive H&P should screen for previous Hx temic disease [9].
of cancer, superior vena cava syndrome, myas- (b) Technetium scan to locate ectopic thy-
roid tissue.
(c) Scrotal ultrasound for germ cell/gonadal
H. Tannous (*) ∙ J. Chikwe primary tumors.
Department of Surgery, Stony Brook University
(d) Tumor markers: AFP and B-HCG for

germ cell tumors [10, 11].
Department of Cardiovascular Surgery, Icahn School
(e) Serum anti-acetylcholine receptor
e-mail: [email protected] antibodies.
(f) Tissue sampling if lymphoma is sus-
M. B. Yammine
Department of Cardiovascular Surgery, Icahn School pected [percutaneous vs. endobronchial
of Medicine at Mount Sinai, New York, NY, USA vs. surgical].

Germ cell tumors are treated by chemother- Thymomas: Most common neoplasm of the ante-
apy, lymphomas with ChemoRad, and thyroid rior mediastinum [12, 13]. The decision to oper-
and thymic tumors with resection. ate should take into consideration the presence of
myasthenia gravis [2].
A
Complete H&P should screen for previous Hx of cancer, superior
vena cava syndrome, myasthenia gravis, Horner syndrome, signs
of tamponade, B symptoms, paraneoplastic syndromes, stridor,
dysphagia, and hoarseness
CT scan:
Assesses location of mass and
invasion of surrounding structures
E Anterior Middle Posterior

D C
Yes AFP/B- Surgical resection Tissue Bx may be helpful

Treatment of germ cell HCG recommended if
tumors [chemotherapy] +ve? symptomatic
No
Surgical resection
recommended in
conjunction with
Needle Bx followed by Yes Lymphoma neurosurgery
ChemoRad if confirmed suspected?
No
Vital organs invaded

Tissue biopsy needed prior Yes
(arch vessel, SVC,
to definitive ChemoRad pulmonary hilum)
No
Tumor resection with curative intent for thymic

tumor [thoracoscopic vs. median sternotomy]
Algorithm 11.1
11 Mediastinal Masses 41
References 7. Takeda S, Miyoshi S, Minami M, Ohta M, Masaoka

A, Matsuda H. Clinical spectrum of mediastinal cysts.
Chest. 2003;124(1):125–32.
1. Carter BW, Tomiyama N, Bhora FY, Rosado de
8. Esme H, Eren S, Sezer M, Solak O. Primary mediasti-
Christenson ML, Nakajima J, Boiselle PM, et al. A
nal cysts: clinical evaluation and surgical results of 32
modern definition of mediastinal compartments. J
cases. Tex Heart Inst J. 2011;38(4):371–4.
Thorac Oncol. 2014;9(9 Suppl 2):S97–101.
9. Schiepers C, Filmont JE, Czernin J. PET for stag-
2. Duwe BV, Sterman DH, Musani AI. Tumors of the
ing of Hodgkin’s disease and non-Hodgkin’s lym-
mediastinum. Chest. 2005;128(4):2893–909.
phoma. Eur J Nucl Med Mol Imaging. 2003;30(Suppl
3. Carter BW, Marom EM, Detterbeck FC. Approaching
1):S82–8.
the patient with an anterior mediastinal mass: a
10.
Javadpour N. Significance of elevated serum
guide for clinicians. J Thorac Oncol. 2014;9(9 Suppl
alphafetoprotein (AFP) in seminoma. Cancer.
2):S102–9.
1980;45(8):2166–8.
4. Reeder LB. Neurogenic tumors of the mediastinum.
11. Hori K, Uematsu K, Yasoshima H, Yamada A,

Semin Thorac Cardiovasc Surg. 2000;12(4):261–7.
Sakurai K, Ohya M. Testicular seminoma with
5. Mazel C, Grunenwald D, Laudrin P, Marmorat
human chorionic gonadotropin production. Pathol Int.
JL. Radical excision in the management of thoracic
1997;47(9):592–9.
and cervicothoracic tumors involving the spine:
12. Mullen B, Richardson JD. Primary anterior mediasti-
results in a series of 36 cases. Spine (Phila Pa 1976).
nal tumors in children and adults. Ann Thorac Surg.
2003;28(8):782–92. discussion 92.
1986;42(3):338–45.
6. Forsythe A, Volpe J, Muller R. Posterior mediastinal
13. Gerein AN, Srivastava SP, Burgess J. Thymoma: a ten
ganglioneuroma. Radiographics. 2004;24(2):594–7.
year review. Am J Surg. 1978;136(1):49–53.
Tracheal Stenosis
12
Algorithmic Approach ventilation and therapeutic coring or dila-

tion. Once the airway is secured, the severity,
A. Tracheal stenosis develops in patients with extent, and location of the stenosis should be
prolonged intubation due to scarring around fully assessed bronchoscopically [7].
the ET tube or tracheostomy. It could also C2. Subsequent workup includes a CXR and CT
be due to a tumor, injury, chest radiation, scan of the chest, which may show tracheal
autoimmune disorder, or infection [1–3]. deviation, foreign body, or mediastinal shift.
B. Initial evaluation should assess for respira- While CXR has low sensitivity and specific-
tory distress and include a complete history ity, a CT is more useful in diagnosing and
and physical exam. Suspicious symptoms assessing severity [3].
include SOB nonresponsive to bronchodila- D. Following airway establishment and for
tors and adult new-onset stridor. Other non-life-threatening stenosis, further man-
symptoms may include cough, dyspnea, agement should take into consideration the
hemoptysis, and wheezing [4–6]. following factors: Etiology of the lesion,
C1. For life-threatening stenosis, oxygen therapy prognosis, life expectancy, and ability to tol-
and airway management are paramount. erate planned procedures.
Bronchoscopy- guided intubation or emer- E1. For extrinsic compression of the trachea or
gent tracheostomy/cricothyroidotomy might systemic diseases causing stenosis, a
be needed. Rigid bronchoscopy intubation is disease- specific plan should be imple-
reserved for extreme cases. It allows mented. For example, ChemoRad for lym-
phoma, vascular intervention for aneurysms
and rings, and resection for substernal thy-
roid tumors and thymomas [3].
H. Tannous (*) ∙ J. Chikwe E2. For intrinsic pathologies, the following
treatment options are available [7–9]:
(a) Tracheal resection of the affected seg-
Department of Cardiovascular Surgery, Icahn School
mented and end-to-end reconstruction
e-mail: [email protected] provides excellent long-term relief [10].
(b) Tracheal stenting provides short- or

M. B. Yammine
Department of Cardiovascular Surgery, Icahn School long-
term relief and is done with a
of Medicine at Mount Sinai, New York, NY, USA metal or silicone stent [11].

(c) Tracheal dilation provides temporary

(d) Tracheal laser ablation and brachyther-
relief and is achieved with dilators or apy are used to excise scar tissue and
balloons [9]. provide temporary relief [6, 12].
A
History and Physical exam:
Adult with a history of recent hospitalization and prolonged intubation presenting
with new-onset stridor and SOB non responsive to bronchodilators
Is the
patient in
respiratory
distress?
Yes No
C
- O2 therapy Once stable - CXR: r/o foreign body and tracheal

- Bronchoscopy-guided intubation or
deviation and mediastinal shift
surgical airway
- CT Chest: Superior in diagnosis and
- Bronchoscopic assessment of
assessing severity, extent, and
severity, extent, and location of
location of stenosis
stenosis
D
- Etiology
- Prognosis/life expectancy
- Ability to tolerate therapeutic
intervention
Intrinsic compression:
Extrinsic compression:
Implement a disease-specific plan, for - Tracheal resection
example, ChemoRad for lymphoma - Tracheal stenting/dilation
- Tracheal laser ablation
Algorithm 12.1
12 Tracheal Stenosis 45
References 7. Li WT, Xiao YB, Liu GN, Huang SM, Ling Y,

Zhang JQ, et al. Management of benign tracheal
stenosis by intubation dilatation under flexible
1. Zias N, Chroneou A, Tabba MK, Gonzalez AV, Gray
bronchoscopic guidance. Zhonghua Yi Xue Za Zhi.
AW, Lamb CR, et al. Post tracheostomy and post intu-
2011;91(42):2995–8.
bation tracheal stenosis: report of 31 cases and review
8. Ciccone AM, De Giacomo T, Venuta F, Ibrahim M,
of the literature. BMC Pulm Med. 2008;8:18.
Diso D, Coloni GF, et al. Operative and non-operative
2. Koshkareva Y, Gaughan JP, Soliman AM. Risk factors
treatment of benign subglottic laryngotracheal steno-
for adult laryngotracheal stenosis: a review of 74 cases.
sis. Eur J Cardiothorac Surg. 2004;26(4):818–22.
Ann Otol Rhinol Laryngol. 2007;116(3):206–10.
9. Dalar L, Karasulu L, Abul Y, Ozdemir C, Sokucu SN,
3. Bacon JL, Patterson CM, Madden BP. Indications and
Tarhan M, et al. Bronchoscopic treatment in the man-
interventional options for non-resectable tracheal ste-
agement of benign tracheal stenosis: choices for sim-
nosis. J Thorac Dis. 2014;6:258–70.
ple and complex tracheal stenosis. Ann Thorac Surg.
4. Majid A, Guerrero J, Gangadharan S, Feller-Kopman
2016;101(4):1310–7.
D, Boiselle P, DeCamp M, et al. Tracheobronchoplasty
10. Yamamoto K, Kojima F, Tomiyama K, Nakamura T,
for severe tracheobronchomalacia: a prospective out-
Hayashino Y. Meta-analysis of therapeutic procedures
come analysis. Chest. 2008;134(4):801–7.
for acquired subglottic stenosis in adults. Ann Thorac
5. Papla B, Dyduch G, Frasik W, Olechnowicz H. Post-
Surg. 2011;91(6):1747–53.
intubation tracheal stenosis – morphological-clinical
11. Chin CS, Litle V, Yun J, Weiser T, Swanson SJ. Airway
investigations. Pol J Pathol. 2003;54(4):261–6.
stents. Ann Thorac Surg. 2008;85(2):S792–6.
6. Allen AM, Abdelrahman N, Silvern D, Fenig E,
12. Leventhal DD, Krebs E, Rosen MR. Flexible

Fruchter O, Kramer MR. Endobronchial brachy-
laser bronchoscopy for subglottic stenosis in the
therapy provides excellent long-term control of
awake patient. Arch Otolaryngol Head Neck Surg.
recurrent granulation tissue after tracheal stenosis.
2009;135(5):467–71.
Brachytherapy. 2012;11(4):322–6.
Incidental Lung Nodule
13
Algorithmic Approach C. If the nodule is >8 mm, a PET/CT is indi-

cated to help categorize it. A hypometa-
Incidental lung nodule is a solid parenchymal lung bolic nodule can undergo a repeat CT
lesion picked up incidentally by CXR, CT scan, or scan at 6 months, whereas a hypermeta-
MRI done during a screening or workup for other bolic one should undergo tissue biopsy
pathologies. When larger than 3 cm, the nodule is (CT guided, endobronchial, or excisional)
considered a “mass.” For the purpose of this discus- [4, 6, 7].
sion, all solid or part-solid parenchymal lesions less D1. If primary lung cancer is confirmed, stag-
than 3 cm will be referred to as lung nodules [1, 2]. ing workup and preoperative evaluation
are initiated. For stage 1 or 2 lung cancer,
A. History and physical exam are key: Smoking surgery is the mainstay of treatment with
history, exposure to carcinogens (asbestos or lobectomy being the gold standard of ther-
silica, etc.), recent travel history, previous apy. For stage 3 or 4, chemoradiation is the
cancer history, or a family history of malig- most common treatment. For patients who
nancy. The differential diagnosis includes are poor candidates for surgery, alternative
infectious and inflammatory processes as local therapies include cryoablation, ste-
well as benign and malignant tumors [3]. reotactic radiation, and radiofrequency
B. For patients with no previous history of can- ablation [8].
cer and no prior imaging for comparison, a D2. For a metastatic lung nodule, a complete
nodule ≤8 mm can be followed up in metastatectomy is reserved for controlled
6 months with a CT scan [4, 5]. primary malignancies with favorable prog-
nosis and no other metastatic sites.
E. If the mass is not cancerous, treatment
H. Tannous (*) ∙ J. Chikwe should target the underlying pathology:
Department of Surgery, Stony Brook University Fungal infection, TB, and vasculitis [1].
NB: Ground glass opacifications should
Department of Cardiovascular Surgery, Icahn School be followed up through regular serial imag-
e-mail: [email protected] ing unless they develop an enlarging solid
component, become >2.5 cm, or increase in
M. B. Yammine
Department of Cardiovascular Surgery, Icahn School size by 25%. In this case, it would require a
of Medicine at Mount Sinai, New York, NY, USA tissue biopsy.

History and Physical exam:

Smoking history, exposure to carcinogens (asbestos, silica, etc.), recent travel,
A previous malignancy, or family Hx of malignancy
Follow up CT in 6 months only if there

No is low index of suspicion:
Is the
B - No previous Hx of cancer
nodule
- Prior imaging comparison
> 8 mm?
Yes
PET/CT
No
Active imaging surveillance
Is the lesion (documenting interval growth
hypermetabolic? warrants repeat PET/CT)
Yes
Tissue biopsy (CT guided, endobronchial, 1- Primary lung CA: Staging

or excisional) workup, preoperative
evaluation, definitive resection
2- Metastatic nodule: Consider
metastasectomy
Yes
D
Is the lesion
malignant?
No Address underlying etiology

E (autoimmune, infectious, etc.)
Algorithm 13.1
13 Incidental Lung Nodule 49
References images: from the Fleischner Society 2017. Radiology.

2017;284(1):228–43.
6. Jaklitsch MT, Jacobson FL, Austin JH, Field JK, Jett
1. Ost D, Fein AM, Feinsilver SH. Clinical practice.
JR, Keshavjee S, et al. The American Association for
The solitary pulmonary nodule. N Engl J Med.
Thoracic Surgery guidelines for lung cancer screening
2003;348(25):2535–42.
using low-dose computed tomography scans for lung
2. Gould MK, Donington J, Lynch WR, Mazzone PJ,
cancer survivors and other high-risk groups. J Thorac
Midthun DE, Naidich DP, et al. Evaluation of indi-
Cardiovasc Surg. 2012;144(1):33–8.
viduals with pulmonary nodules: when is it lung can-
7. Berghmans T, Dusart M, Paesmans M, Hossein-
cer? Diagnosis and management of lung cancer, 3rd
Foucher C, Buvat I, Castaigne C, et al. Primary tumor
ed: American College of Chest Physicians evidence-
standardized uptake value (SUVmax) measured on
based clinical practice guidelines. Chest. 2013;143(5
fluorodeoxyglucose positron emission tomography
Suppl):e93S–e120S.
(FDG-PET) is of prognostic value for survival in non-
3. McWilliams A, Tammemagi MC, Mayo JR, Roberts
small cell lung cancer (NSCLC): a systematic review
H, Liu G, Soghrati K, et al. Probability of cancer in
and meta-analysis (MA) by the European Lung
pulmonary nodules detected on first screening CT. N
Cancer Working Party for the IASLC Lung Cancer
Engl J Med. 2013;369(10):910–9.
Staging Project. J Thorac Oncol. 2008;3(1):6–12.
4. Wood DE. National Comprehensive Cancer Network
8. Rivera MP, Mehta AC, Wahidi MM. Establishing the
(NCCN) clinical practice guidelines for lung cancer
diagnosis of lung cancer: diagnosis and management
screening. Thorac Surg Clin. 2015;25(2):185–97.
of lung cancer, 3rd ed: American College of Chest
5. MacMahon H, Naidich DP, Goo JM, Lee KS, Leung
Physicians evidence-based clinical practice guide-
ANC, Mayo JR, et al. Guidelines for management
lines. Chest. 2013;143(5 Suppl):e142S–e65S.
of incidental pulmonary nodules detected on CT
Management of Lung Cancer
14
Scott C. Tiedebohl and Matthew D. Taylor
Algorithmic Approach D. If the nodule is suspicious for malignancy, a

PET/CT is used to evaluate for nodal involve-
A. Lung cancer should be included in the differ- ment and metastatic disease [2].
ential diagnosis of any pulmonary nodule dis- E. If there is no evidence of lymph node involve-
covered on imaging. A complete history and ment or metastatic disease, the determination
physical exam should be completed, noting of the patient’s ability to tolerate surgery
the patient’s prior exposures to smoking and must be made. Patients should have a recent
other environmental risk factors along with EKG and pulmonary function tests.
pulmonary symptoms [1]. Postoperative predictive values of FEV1 and
B. A CT scan should then be obtained to better DLCO of >60% indicate that the patient should
characterize the lesion. A lesion that is spicu- be able to tolerate a major lung resection.
lated and not calcified is concerning for Values between 30% and 60% require cardio-
malignancy. It is important to compare new pulmonary exercise testing. If the values are
studies to all previous studies to see if the <30%, then the patient is high risk, and
lesion has grown or changed in character [2]. VO2max should be obtained. If this is less than
C. If after reviewing a patient’s history, physical 10 mL/kg/min, the patient is not a surgical
examination, and CT findings the suspicion candidate [3].
for a malignancy remains low, no further F. For those patients who are not surgical candi-
workup is necessary, and the nodule can be dates, definitive chemoradiation or SBRT
observed with repeat imaging based on the should be offered [1].
Fleischner society guidelines. G. If the patient is an operative candidate based
on preoperative assessment and the lesion is
deemed resectable, the patient can proceed to
surgery. Ideally, patients should undergo
lobectomy or pneumonectomy with additional
mediastinal lymph node assessment. If a
S. C. Tiedebohl ∙ M. D. Taylor (*) patient cannot tolerate these lobectomy, then a
Department of Surgery, Penn State Health Milton
more limited resection should be performed.
Regardless of the resection, it should be per-
Division of Thoracic Surgery, Penn State Health
formed minimally invasively, if possible [1].
Milton S. Hershey Medical Center,
Hershey, PA, USA H. If there are suspicious lymph nodes on PET
e-mail: [email protected] imaging, biopsy of the suspicious nodes

52 S. C. Tiedebohl and M. D. Taylor
should be undertaken via endobronchial should include the surgical team, medical
ultrasound (EBUS) or mediastinoscopy in oncology, and radiation oncology. The
order to appropriately stage the patient and patient can then proceed directly to surgery
direct further management [1]. after preoperative evaluation and receive
I. If after biopsy, there is no evidence of lymph induction chemotherapy, or definitive che-
node disease or only N1 disease, then the motherapy or SBRT [1].
patient can undergo preoperative evaluation K. If there is evidence of unresectable disease
and proceed to surgery [1]. or metastatic disease, the patient is not a
J. If there is evidence of N2 disease on biopsy, surgical candidate, and referral to medical
then the patient’s case should be discussed and radiation oncology should be under-
in a multidisciplinary conference which taken [1].
History:
Elderly male, current smoker
Pulmonary nodule found on
CXR for cough
A
Full history/physical exam
B CT
C Low suspicion of malignancy High suspicion of malignancy
D
Surveillance PET/CT
Suspicious
K
lymph nodes
E No evidence of lymph node Unresectable disease or obvious
involvement or metastatic disease metastatic disease
EBUS or
H mediastinoscopy
Operative Definitive
candidate? chemoradiation
J or SBRT
No Yes
Negative or locally +Mediastinal lymph nodes
positive lymph nodes (N1) (N2)
Definitive
radiation or
I
SBRT
Single-level N2
disease Multilevel N2
F disease
Surgery
G Multidisciplinary
evaluation
Algorithm 14.1
14 Management of Lung Cancer 53
References Johns Hopkins textbook of cardiothoracic surgery.

2nd ed. New York: McGraw Hill Publishing; 2014.
3. Arndt A. Pulmonary physiology and pulmonary func-
1. University of Texas. MD Anderson Cancer Center.
tion tests. In: DM LP, Mery CM, Turek JW, editors.
Thoracic Center Faculty. Non-small cell lung cancer. MD
TSRA review of cardiothoracic surgery. 2nd ed.
Anderson: Department of Clinical Effectiveness; 2017.
Chicago: Thoracic Surgery Residents Association;
2. Kim MP, Vaporciyan AA. Primary lung cancer. In:
2015.
Yuh DD, Yang SC, Vricella LA, Doty JR, editors.
Management of Empyema
15
Algorithmic Approach collection or a dense fibrous peel, important for

planning the appropriate treatment [1].
A. In a patient with a history of current or recent D. After demonstration of a fluid collection on
pneumonia with ongoing symptoms, parapneu- imaging, thoracentesis with pleural fluid anal-
monic effusion should be suspected. Pneumonia ysis is the next step in management. This can
can be associated with pleural effusions around be done with image-guided technique, either
60% of the time; however, less than 5% typi- ultrasonographically or by CT guidance. Fluid
cally progress to empyema. Causes of empy- can then be analyzed by Light’s criteria. A pH
ema other than infection include trauma and less than 7.2 or glucose less than 60 is diagnos-
iatrogenic instrumentation [1, 2]. tic of infection. Pleural fluid can be cultured;
B. Vital signs consistent with infection (i.e., however, sensitivity has been reported to be
tachycardia, fever), leukocytosis, and physi- between 40% and 60% [1, 2].
cal exam findings consistent with a pulmo- E. After identification of an infected pleural col-
nary process should lead the clinician to lection (empyema), tube thoracostomy should
suspect a parapneumonic effusion [1]. then be performed. Fluid should be sent for
C. Physical exam findings and a history sugges- gram stain and culture. Empiric antibiotics
tive of a parapneumonic effusion warrant imag- should be started after fluid has been sent [1, 2].
ing. This usually begins with a chest radiograph, F. If, after adequate drainage for 24 h, there is
which often demonstrates findings of a pleural evidence of a persistent fluid collection, CT
effusion. CT scan is the gold standard for imag- should be repeated.
ing a pleural abnormality as it can differentiate G. Fibrinolytics, consisting of tPA and DNase,
pleural fluid from a parenchymal abnormality. can be instilled into the collection if it is a
CT can also demonstrate loculations within a simple collection and is still in the early
phase, typically less than 3 days. Fibrinolytics
S. R. Kotch have been shown to decrease the need for sur-
Department of General Surgery, Penn State Health gical intervention. This benefit is seen when
the two agents are used together; when used
M. D. Taylor (*) alone, they are ineffective [3].
H. If there is evidence of pleural thickening, the col-
lection is multiloculated, or the collection is sim-
ple, but has been present for more than 3 days,
Hershey, PA, USA then the patient should undergo video-assisted
e-mail: [email protected] thoracoscopic surgery (VATS) with decortica-
56 S. R. Kotch and M. D. Taylor
tion. If VATS is felt to be unsafe due to the poten- I. If a simple collection fails fibrinolytic ther-
tial for parenchymal injury due to a dense fibrous apy, surgical treatment is indicated by VATS
peel, open thoracotomy is indicated [2]. or thoracotomy [2].
History:
Productive cough, fever, dyspnea, pleuritic chest pain
A
Current or recent history of pneumonia
Obtain vital signs and blood work, and perform physical exam
38.6, BP 115/70, HR 90, RR 20, WBC 20,000

B Diminished breath sounds, dullness to percussion, fremitus, and egophony
Obtain chest radiograph or CT scan
Chest radiograph shows unilateral pleural effusion

C CT scan shows pleural fluid collection consistent with parapneumonic effusion
Sample pleural fluid (thoracentesis) with pleural

D fluid analysis
pH <7.2 or glucose <60
Tube thoracostomy with Gram

E
stain and fluid cultures. Begin
antibiotics
F Obtain CT if collection persists >24 hr
Simple collection, early (<3 days) Multiloculated, pleural thickening

Simple collection, late (>3 days)
G
H
Fibrinolytics
VATS/thoracotomy
Failure to improve at 24 hr
I
Algorithm 15.1
15 Management of Empyema 57
References 3. Rahman NM, Maskell NA, West A, Teoh R, Arnold A,

Mackinlay C, Peckham D, Davies CW, Ali N, Kinnear
W, Bentley A, Kahan BC, Wrightson JM, Davies HE,
1. Raymond D. Surgical intervention for thoracic infec-
Hooper CE, Lee YC, Hedley EL, Crosthwaite N, Choo L,
tions. Surg Clin North Am. 2014;94(6):1283–303.
Helm EJ, Gleeson FV, Nunn AJ, Davies RJ. Intrapleural
https://doi.org/10.1016/j.suc.2014.08.004.
use of tissue plasminogen activator and DNase in
2. Bhatnagar R, Maskell NA. Treatment of complicated pleu-
pleural infection. N Engl J Med. 2011;365(6):518–26.
ral effusions in 2013. Clin Chest Med. 2013;34(1):47–62.
https://doi.org/10.1056/NEJMoa1012740.
https://doi.org/10.1016/j.ccm.2012.11.004.
Management of Spontaneous
Pneumothorax 16
Algorithmic Approach C. Imaging consists of a simple upright chest

radiograph. CT may also be obtained to
A. In a patient who presents with a chief com- assess for underlying lung disease; however,
plaint of sudden-onset dyspnea and pleuritic- in the acute setting, it is not necessary [1].
type chest pain, pneumothorax must be D. Determining the patient’s stability is key to
included in the differential diagnosis. providing the best treatment [1].
Pneumothorax may be asymptomatic in 10% E. In patients who are hemodynamically stable and
of patients. Primary spontaneous pneumotho- have a small pneumothorax, typically less than
rax is typically seen in young male smokers 2 cm, observation is appropriate. Intervention is
who are otherwise healthy and tend to be tall not necessary unless the pneumothorax enlarges
and thin. These patients have apical subpleu- or the patient’s condition declines [1, 2].
ral blebs which rupture. Secondary spontane- F. Patients who are hemodynamically unstable
ous pneumothorax occurs in patients with at presentation or who have a large pneumo-
known lung disease including COPD, infec- thorax on chest radiography should undergo
tion, cystic fibrosis, and catamenial [1]. tube thoracostomy. This can be accomplished
B. The physical exam findings are many and vary by means of a small-bore catheter (14F or
between stable and unstable patients. In patients less) or a 16F to 22F chest tube [2].
experiencing significant respiratory distress, G. Once there is radiographic resolution of the
are tachypniec, have distended neck veins and pneumothorax, the clinician should consider
tracheal deviation, or are otherwise hemody- removal of the chest tube. If there is no air leak
namically unstable, the clinician should be con- after suction has been discontinued, a second
cerned about a tension pneumothorax [1]. chest radiograph may be obtained to confirm
that there has not been recurrence of the pneu-
S. R. Kotch mothorax. The timing of the radiograph varies
Department of General Surgery, Penn State Health with most (67%) of clinicians waiting 5–12 h
after the last documented air leak [2].
M. D. Taylor (*) H. However, in patients with radiographic reso-
lution of the pneumothorax, but who have a
persistent air leak after 4–5 days, evaluation
for surgical management is indicated.
Hershey, PA, USA Thoracoscopy with bullectomy and mechani-
e-mail: [email protected] cal pleurodesis can be performed in these

60 S. R. Kotch and M. D. Taylor
patients. In patients who are poor surgical does not typically occur until after the sec-
candidates or those who decline surgery, ond recurrence unless the patient is involved
chemical pleurodesis with talc or doxycy- in high-risk activities such as flying or
cline can be instilled via the chest tube to scuba diving. Patients with secondary
achieve pleural symphysis [2]. pneumothoraces typically undergo inter-
I. Thoracoscopy with pleurodesis is also per- vention after the first occurrence due to the
formed to prevent recurrence. For those potential morbidity and mortality of a
patients with primary pneumothorax, this recurrence [2].
History:
A Sudden onset of dyspnea, and chest pain
Obtain vital signs, and perform physical exam
• 37.6, BP 110/65, HR 95, RR 25

• Unilateral decreased breath sounds, hyper resonance, decreased chest excursion, and
B subcutaneous emphysema
• Significant respiratory distress, tachypnea, pulsus paradoxus, distended neck veins, and
tracheal deviation
C Obtain chest radiograph confirming pneumothorax
D Hemodynamically stable Hemodynamically unstable
F
Tube thoracostomy
Small pneumothorax Large pneumothorax
E
Observation Radiographic resolution of Radiographic resolution of
pneumothorax, no air leak pneumothorax, air leak present
H
G Pull chest tube Thoracoscopy or
chemical pleurodesis
Prevention of recurrence
I
Algorithm 16.1
16 Management of Spontaneous Pneumothorax 61
References 2. Baumann MH, Strange C, Heffner JE, Light R, Kirby

TJ, Klein J, Luketich JD, Panacek EA, Sahn SA, AACP
Pneumothorax Consensus Group. Management of
1. van Berkel V, Kuo E, Meyers BF. Pneumothorax,
spontaneous pneumothorax: an American College of
bullous disease, and emphysema. Surg Clin North
Chest Physicians Delphi consensus statement. Chest.
Am. 2010;90(5):935–53. https://doi.org/10.1016/j.
2001;119(2):590–602.
suc.2010.06.008.
Thoracoabdominal Aortic
Aneurysm 17
Albert G. Pavalonis and Anil Hingorani
Algorithmic Approach Type I: distal to left subclavian and proximal

to the renal arteries.
A. The diagnostic imaging for a patient with a Type II: distal to left subclavian and inferior
thoracoabdominal aneurysm is usually done to the renal arteries.
by the time he/she is seen by the surgeon. In a Type III: 6th intercostal space and inferior to
majority of cases, a TAAA is found inciden- the renal arteries.
tally on imaging done for other reasons. The Type IV: inferior to the diaphragm extending
two preferred imaging modalities for TAAA to the aortic bifurcation.
are CT angiography and MR angiography (if Type V: 6th intercostal space and proximal to
contrast allergy or significant concern for the renal arteries.
renal failure). High-quality imaging of the C. After the aneurysm has been anatomically
thoracoabdominal aorta is essential for accu- delineated based on the Crawford classifica-
rate classification of the aneurysm because tion, then the next important thing is the risk
this determines treatment options [1]. of rupture. A TAAA with a size greater than
B. Once high-quality imaging is available, the 6 cm carries a yearly risk of rupture of 14.1%
next step is classifying the specific TAAA [4]. Aneurysm growth of more than 2 mm per
subtype. The Crawford classification is the year also carries an increased risk of rupture.
standardized identification system. This sys- Patients with the following characteristics
tem is based on anatomic location of the exhibit higher propensity for rupture: Female,
aneurysm and not size [2]. The Crawford COPD, and uncontrolled hypertension (espe-
classification is: cially diastolic >100 mmHg) [6].
A. G. Pavalonis
Department of Vascular Surgery, NYU Langone
A. Hingorani (*)
Division of Vascular Services, NYU Langone

64 A. G. Pavalonis and A. Hingorani
D. The perioperative management/workup of

considered when the risk of rupture overshad-
TAAA patients is important for optimal out- ows potential complications.
come. Ideally, management and extended F. Repair options involve open repair, endovascular
workup should occur at a center that special- repair, and hybrid repair. Open repair is the origi-
izes in the treatment of TAAA (high-volume nal modality of TAAA repair and is still a viable
center). It is important to consider the follow- option in the setting of anatomy that may not be
ing management [3, 6]: adequate for endovascular repair. Endovascular
1. Cardiac risk assessment repair involves intraluminal insertion of a cus-
2. Strict control of hypertension tom-made stent graft; however, this is not always
3. Initiation of statin therapy (found to
an option in cases of rupture as it requires
decrease risk of rupture) 4–6 weeks to manufacture a patient-specific
4. Pulmonary assessment (FEV1 > 1.4
stent graft [7]. That being said, endovascular
required for single lung ventilation during repair has been successfully performed in cases
open repair) of rupture with the utilization of chimney grafts
E. All repair options carry considerable risk for in conjunction with off-the-shelf stent grafts [1].
complications. The most significant compli- Finally, a hybrid repair could be considered. This
cations are the following: Paralysis, renal involves open debranching of visceral and aortic
failure, visceral ischemia, and myocardial arch vessels to maintain perfusion with subse-
infarction [3]. Given the high risk of opera- quent placement of a stent graft to exclude the
tion, the repair of a TAAA should only be aneurysmal aortic segment [5].
17 Thoracoabdominal Aortic Aneurysm 65

Thoracoabdominal aortic aneurysms usually found incidentally during the evaluation
and work up of other medical conditions
A
Obtain high-quality imaging (CT angiography or MR angiography)
Classification of TAAA based on the Crawford system

B
Type I: Distal to left subclavian and proximal to the renal arteries
Type II: Distal to left subclavian and inferior to the renal arteries
Type III: Sixth intercostal space and inferior to the renal arteries
Type IV: Inferior to the diaphragm extending to the aortic bifurcation
Type V: Sixth intercostal space and proximal to the renal arteries
Identify risk of rupture

C
> 6 cm carries a yearly risk of rupture of 14.1%.
Aneurysm growth of more than 2 mm
Patients with the following characteristics exhibit higher

propensity for rupture: female, COPD, uncontrolled hypertension
(especially diastolic >100 mmHg).
Peri operative Management
D 1. Cardiac risk sssessment

2. Strict control of hypertension
3. Initiation of statin therapy
4. Pulmonary assessment
E
Discussion of Risks and Complications Related to Therapeutic Intervention
F Referral to High-Volume Center for Repair
Algorithm 17.1
66 A. G. Pavalonis and A. Hingorani
Suggested Reading Fredric JR, Woo YJ. Thoracoabdominal aortic aneurysm.

Ann Cardiovasc Surg. 2012;1(3):227–85.
Kabbani LS, Criado E, Upchurch GR Jr, et al. Hybrid
Black SA, Wolfe JH, Clark M, et al. Complex thora-
repair of aortic aneurysms involving the visceral and
coabdominal aortic aneurysms: endovascular exclu-
renal vessels. Ann Vasc Surg. 2010;24:219–24.
sion with visceral revascularization. J Vasc Surg.
Stein LH, Berger J, Tranquilli M, et al. Effect of statin
2006;43:1081–9.
drugs in thoracic aortic aneurysms. Am J Cardiol.
Davies RR, Goldstein LJ, Coady MA, et al. Yearly rup-
2010;55:841–57.
ture or dissection rates for thoracic aortic aneurysms:
Zinganshin BA, Elefteriades JA. Surgical manage-
simple prediction based on size. Ann Thorac Surg.
ment of thoracoabdominal aneurysms. Heart.
2006;81:169–77.
2014;100:1577–82.
Escobar GA, Upchurch GR Jr. Management of thora-
coabdominal aortic aneurysms. Curr Probl Surg.
2011;48:70–133.
Part IV
Breast
Nipple Discharge
18
Algorithmic Approach cancer, and therefore generally is not helpful

in diagnosing malignancy [5].
A. The first step in the evaluation of a patient D. Physical exam should focus on any skin or
with nipple discharge is a history to differenti- nipple changes, nipple retraction, masses, or
ate pathologic from physiologic discharge. nodules. An attempt at expressing the dis-
Questions regarding the timing (spontaneous charge should be made, within reason.
vs. expressed), side, color/character, single vs. Regional lymphadenopathy should also be
multiple ducts, history of pregnancy and nurs- assessed [1, 2].
ing, nipple trauma, smoking, signs of mastitis, E. In the case of physiologic discharge, appropri-
prior history of nipple discharge, as well as ate work-up may include mammogram, ultra-
breast cancer risk assessment will help to sound, and hormone levels to include prolactin
determine the type of discharge present [1]. and TSH. Galactorrhea can be caused by ele-
B. Physiologic nipple discharge is generally vated prolactin levels, which in turn can be
bilateral, involves multiple ducts, and is often caused by many medications which increase
nonspontaneous. The consistency of physio- prolactin levels, including psychotropics, anti-
logic discharge may be serous, milky (galac- hypertensives, gastrointestinal drugs, anesthet-
torrhea), green, or brown [1, 2]. ics, amphetamines, marijuana, and estrogens [1,
C. Pathologic nipple discharge is generally uni- 2]. If prolactin levels are elevated in the absence
lateral, spontaneous, and serous or bloody, but of drugs/medications, an MRI of the brain may
can also be brown. It is often from a single be required to r/o prolactinoma. It is important
duct but can involve multiple ducts [2, 3]. It is to note that lactation can last up to 2 years after
more often associated with carcinoma, espe- pregnancy and occasionally longer.
cially when serous or bloody [4]. Cytology of F. Green or brown discharge is typically associ-
nipple aspirate has a very low sensitivity for ated with periductal mastitis and generally
resolves on its own, as long as no active infec-
tion is present [2].
A. R. Thawani G. In the case of pathologic nipple discharge,
Division of Surgical Oncology, AMITA Health
diagnostic mammogram and subareolar ultra-
sound are the imaging modalities of choice.
L. M. Erdahl (*)
Although historically the sensitivity and
Department of Surgery, University of Iowa,
Iowa City, IA, USA specificity of mammogram and US was
e-mail: [email protected] considered low for diagnosis of malignancy

70 A. R. Thawani and L. M. Erdahl
[3, 5], newer data suggests otherwise [6, 7]. If mance of ductography is dependent on a phy-
no abnormalities (defined as a mass, indeter- sician who is experienced with this technique,
minate/suspicious calcifications, or architec- but is often poorly correlated with the surgi-
tural distortion) are seen on mammogram and cal diagnosis [2, 8]. In some cases, MRI may
subareolar US every 6 months for 2 years or be considered for suspicious bloody dis-
until the discharge is resolved, the upstage charge with no imaging abnormality seen on
risk to carcinoma is <3%. Surgical therapy to mammogram and ultrasound, especially in
excise the duct is typically unnecessary. In those women at high risk for breast cancer
the presence of an imaging abnormality, based on family history, age, or both.
biopsy is recommended to evaluate the cause. I. Surgical options include ductoscopy, image/
Most often, surgery is also indicated to discharge-guided single duct excision, and
remove the affected duct and underlying major subareolar duct excision (the last of
lesion in order to rule out an associated carci- these is defined as excision of all the central
noma [6, 7]. nipple ducts) [9]. It is important to counsel
. Some institutions also utilize ductography for
H women of child-bearing age that surgery may
imaging of the affected duct. The perfor- affect future lactation.
18 Nipple Discharge 71
A Nipple discharge
History and physical
Spontaneous Spontaneous
B/C Non Spontaneous
Unilateral Bilateral
Milky/white
D Green/gray/tan
Bloody Copious
Straw colored Milky
Mammogram and
Observe Endocrine work up
subareolar
E/F Routine screening including TSH and
ultrasound
and exams prolactin
G Abnormal
Negative
mammogram
mammogram
and/or
/US
subareolar US
Image guide
Counsel low
percutaneous biopsy
risk of
and/or subareolar duct
carcinoma
excision
(<3%)
Major or localized Q6-month follow-up

*Subareolar ducts can be
imaging and exams
I subareolar duct
until resolved or for
localized using a lacrimal
excision * probe, methylene blue dye,
1–2 years
ductography, or ductoscopy.
Algorithm 18.1
References 5. Simmons R, Adamovich T, Brennan M, et al.

Nonsurgical evaluation of pathologic nipple dis-
charge. Ann Surg Oncol. 2003;10(2):113–6.
1. Laronga C, Tollin S, Turanga K. History, physi-
6. Gray RJ, Pockaj BA, Karstaedt PJ. Navigating murky
cal examination, and staging. In: Keurer H, editor.
waters: a modern treatment algorithm for nipple
Keurer’s breast surgical oncology. New York: The
discharge. Am J Surg. 2007;194:850–4; discussion,
Mc-Graw Hill Companies; 2010. p. 126–8.
854–5.
2. Kato M, Simmons R. The evaluation and treatment of
7. Ashfaq A, Senior D, Pockaj B, et al. Validation of a
nipple discharge. In: Scott-Conner C, Dirbas F, edi-
modern treatment algorithm for nipple discharge. Am
tors. Breast surgical techniques and interdisciplinary
J Surg. 2014;208:222–7.
management. New York: Springer; 2011. p. 179–86.
8. Cabioglu N, Hunt K, Singletary SE, et al. Surgical
3. Adepoju LJ, Chun J, El-Tamer M, et al. The value of
decision making and factors determining a diagnosis
clinical characteristics and breast imaging studies in
of breast carcinoma in women presenting with nipple
predicting a histopathologic diagnosis of cancer or
discharge. J Am Coll Surg. 2003;196(3):354–64.
high-risk lesion in patients with spontaneous nipple
9. Sharma R, Dietz J, Wirght H, et al. Comparative anal-
discharge. Am J Surg. 2005;190:644–6.
ysis of minimally invasive microductectomy versus
4. Neslon RS, Hoehn JL. Twenty year outcome follow-
major duct excision in patients with pathologic nip-
ing central duct resection for bloody nipple discharge.
ple discharge. Surgery. 2005;138:591–6; discussion,
Ann Surg. 2006;243:522–4.
596–7.
Breast Mass Evaluation
19
Algorithmic Approach between clinical and radiographic findings

should always be evaluated.
A. Workup of any breast mass starts with taking C. Mammography is typically the preferred
a focused history and performing a physical imaging modality for palpable breast lesions.
exam. Key components of the history include For young women and women with dense
onset, duration, size, change in size, associ- breast tissue, mammogram may miss lesions.
ated pain, cyclical changes, nipple discharge, If a woman is under 40, we recommend first
and personal history of breast disease. In discussing the optimal imaging with the radi-
addition, the history should include a gyneco- ologist performing the study. Ultrasound is
logic and personal family history of cancer the study of choice in the evaluation of a pal-
including but not limited to breast cancer. The pable breast mass in women under the age of
physical exam should include examination of 30 or who are lactating or pregnant. Women
the regional lymph nodes in the neck, chest, aged 30–39 may benefit from starting with
and axilla. Breast exam components include ultrasound according to the American College
inspection, palpation seated and supine, and of Radiology Appropriateness Criteria© [2].
examination for nipple discharge. There are D. After mammogram, ultrasound of a palpable
limited data regarding the most effective mass is usually recommended to provide fur-
technique for breast exam. Based on research ther detail on the characteristics of the mass.
using sensor-based breast models, it appears Diagnostic ultrasound can also be used to
the rubbing movement is the most effective determine the best method of biopsy if a
technique for detecting masses [1]. biopsy is recommended [2].
B. Once the history and exam are complete, the E. It is rarely necessary to perform MRI for
practitioner can determine the best type of diagnostic evaluation of a palpable mass.
imaging for further evaluation. Correlation However, it may be indicated for further eval-
uation after initial imaging and biopsy [2].
F. Suspicious masses on imaging, BIRADS cat-
A. R. Thawani egory 4 or 5 should be biopsied with core
biopsy and placement of a radiologic marker
for future identification. Medical or surgical
L. M. Erdahl (*)
treatment will then be based on both patho-
Iowa City, IA, USA logic findings and radiologic-pathologic
e-mail: [email protected] concordance. Discordant biopsy results


should be reviewed with the performing radi- have a 2.6–6.9% rate of progressing to appear
ologist and pathologist to determine next more suspicious and require biopsy [3, 4].
steps and surgical excisional biopsy should The majority of lesions that progress will do
be considered [2]. so in the first 6 months [3].
G. Solid masses interpreted as probably benign H. Benign or negative imaging findings of

on imaging, BIRADS 3, should be reviewed BIRADS 2 and 1, respectively, should be
and core biopsy considered if they are new in reviewed. If physical exam is concerning, dis-
a patient with prior imaging within the past cuss with a radiologist possible additional
year. Lesions that are not biopsied should be imaging to evaluate the finding. If no addi-
followed with short interval exam and imag- tional imaging will be beneficial, consider
ing [2]. Perform repeat history and physical fine needle aspiration of palpable mass. Treat
exam in 3 months to evaluate for change in the patient based on pathology results [2].
the mass that might require additional evalua- I. Cysts presenting as palpable masses should
tion. Continue to follow every 6 months until not be routinely aspirated. Aspiration of a
the lesion is stable for 2 years. These lesions painful cyst may be considered [2].
19 Breast Mass Evaluation 75
A B Breast mass
Age <30
Pregnant Age 30–39 Age ≥ 40
Lactating
Diagnostic
Diagnostic targeted Diagnostic
mammogram or
ultrasound mammogram
targeted ultrasound
Review imaging findings, if BIRADS 0-incomplete

Order additional testing to complete
BIRADS 1 : Negative BIRADS 4 : Suspicious

BIRADS 3 : Probably Benign
BIRADS 2 : Benign BIRADS 5 : Highly Suspicious
G New on imaging
Further workup only on Follow-up in 3 months Image-guided percutaneous

H suspicious masses Monitor on physical exam core needle biopsy with F
Consider FNA in select cases Repeat imaging 3-6 months radiologic clip placement
Are radiology and

Follow solid lesions 3-6 pathology concordant?
months until stable 2 years
NO
YES
Consider excisional
Treat tissue diagnosis
biopsy
Algorithm 19.1
References 3. Buch K, Qureshi M, Bloch B, et al. Surveillance of

probably benign (BI- RADS 3) lesions in mammog-
raphy: what is the right follow-up protocol? Breast J.
1. Laufer S, D’Angelo AD, Kwan C, Ray RD, Yudkowsky
2015;21(2):168–74.
R, Boulet JR, McGaghie WC, Pugh CM. Rescuing the
4. Harvey JA, Nicholson BT, Lorusso AP, Cohen MA,
clinical breast examination: advances in classifying
Bovbjerg VE. Shortterm follow-up of palpable breast
technique and assessing physician competency. Ann
lesions with benign imaging features: evaluation of
Surg. 2017;266(6):1069–74.
375 lesions in 320 women. AJR Am J Roentgenol.
2. Harvey JA, et al. ACR appropriateness criteria palpable
2009;193:1723–30.
breast masses. J Am Coll Radiol. 2016;13(11):e31–42.
Ductal Carcinoma In Situ
20
Algorithmic Approach tory and physical exam. Key aspects of the

history include family history of breast can-
A. Ductal carcinoma in situ (DCIS) of the breast cer, time of unopposed estrogen (i.e., early
is a heterogenous group of lesions with menarche, late parity, late menopause), pres-
diverse malignant potential and several treat- ence of a mass, nipple discharge or retraction,
ment options. It is generally considered a history of hormone replacement therapy, his-
“preinvasive” lesion, meaning that it has not tory of previous chest or neck radiation ther-
crossed the basement membrane of the cell apy, BMI, and presence of previous breast
into adjacent tissue. It is a nonobligate pre- biopsies [6]. A strong family history should
cursor to invasive cancer. Overall, if left warrant a consultation with a genetic counsel-
alone, 25–40% of patients will develop an lor for genetic testing. * Refer to NCCN
invasive cancer, which constitutes the ratio- guidelines on hereditary testing [7]. Physical
nale for treatment. When appropriately exam should include a detailed breast exam,
treated, the mortality from DCIS remains expression of nipple discharge if present,
low, at 0.5–1.0% [1–4]. breast size, and the presence of lymphade-
B. Most DCIS is found on mammography. nopathy [2].
Eighty percent are associated with calcifica- D. DCIS is classified based on nuclear grade and
tions seen on mammogram. These calcifica- the presence of necrosis in many systems.
tions are generally a good guide, but do not This classification allows for prediction of
always predict the extent of DCIS. The final local recurrence risk when combined with
pathology may be larger, smaller, or the same tumor size, age, and margin status. The high-
as the mammographic imaging [1, 5]. est local recurrence risk is for high-grade
C. The first step in the evaluation of the patient lesions. Non-high-grade lesions with necrosis
with biopsy-proven DCIS is a complete his- recur more often than those without necrosis
[2, 8, 9].
E. Treatment for DCIS includes excision alone,
A. R. Thawani excision and radiation therapy (RT) +/− hor-
mone therapy (HT), and mastectomy. Choice
of operation does not alter overall survival,
L. M. Erdahl (*)
although local recurrence is higher after
Iowa City, IA, USA breast-conserving surgery. The addition of RT
e-mail: [email protected] will generally decrease the local recurrence

risk by 50%. The addition of HT will also Microinvasion is defined as the extension of
reduce the local recurrence risk by at least cancer beyond the basement membrane into
30% [10–16]. adjacent tissues with no focus greater than
F. The choice of treatment is based upon the 1 mm in dimension. In general, the incidence
extent of disease, patient preference, and esti- of occult invasion is less than 10%. A delayed
mation of local recurrence. The van Nuys pre- sentinel lymph node biopsy when invasion is
diction Index incorporates size, age, margin found at final pathology is safe, accurate, and
status, and pathology and is a useful tool to esti- prevents unnecessary axillary surgery, since
mate local recurrence and to determine future the majority of patients will not have invasive
therapy. Genomic testing (currently Oncotype cancer on final excision [1, 2].
DCIS) can be performed on the lumpectomy H. The most recent consensus statement on mar-
specimens to better quantify recurrence risk gins recommends a 2 mm margin for DCIS,
and need for adjuvant therapies [9, 17, 18]. which has been thought to decrease the local
G. Sentinel lymph node biopsy is not indicated recurrence risk while minimizing unneces-
for pure DCIS on core biopsy, unless the sary surgery in the form of re-excisions or
patient has to undergo mastectomy. mastectomy [19, 20].
20 Ductal Carcinoma In Situ 79
DCIS diagnosed on
A percutaneous core biopsy
Review imaging and obtain

B/C additional imaging if needed
to confirm extent of disease
Favorable breast size to tumor

size ratio or
E favorable for oncoplastic
Multicentric disease
Diffuse, malignant, calcifications
technique
Early to mid-pregnancy
History of previous breast radiation
*History of scleroderma, active lupus
*Unfavorable tumor to breast size ratio
Lumpectomy
Image localization with marker
F
Invasion present Mastectomy
with sentinel lymph node biopsy
No invasion present
Invasive cancer algorithm G

Margins < Margins > or
H 2 mm = 2 mm
Mastectomy Re-excision
*Denotes relative contraindication

**Radiation therapy
**May be avoided in select patients
**Hormonal therapy
in ER + PR + patients
Algorithm 20.1
References New Zealand: randomized controlled trial. Lancet.

2003;362:95–102.
12. Viani GA, Stefano EJ, Alfonso SL, et al. Breast-

1. Silverstein M, Lagios M. Ductal carcinoma in situ. In:
conserving surgery with or without radiotherapy in
Keurer H, editor. Keurer’s breast surgical oncology.
women with ductal carcinoma in situ: a metaanalysis
New York: The McGraw Hill Companies; 2010.
of randomized trials. Radiat Oncol. 2007;2:28–39.
p. 189–207.
13.
Fisher B, Constantino J, Redmond C, et al.
2. Lagios M. Duct carcinoma in situ: a Gordian knot
Lumpectomy compared with lumpectomy and radia-
untied. In: Scott-Conner C, Dirbas F, editors. Breast
tion therapy for the treatment of intraductal breast
surgical techniques and interdisciplinary manage-
cancer. N Engl J Med. 1993;328:1581–6.
ment. New York: Springer; 2011. p. 623–31.
14. Fisher B, Dignam J, Wolmark N, et al. Lumpectomy
3. Sanders M, Schuyler P, Dupont W, Page D. The natu-
and radiation therapy for the treatment of intraductal
ral history of low grade ductal carcinoma in suit of the
breast cancer: findings from the National Surgical
breast in women treated by biopsy only revealed over 30
Adjuvant Breast and Bowel Project B-17. J Clin
years of long term follow-up. Cancer. 2005;103:2481–4.
Oncol. 1998;16:441–52.
4. Rosen P, Senie R, Schottenfeld D, Ashikari
15. Julien J, Bijker N, Fentiman I, et al. Radiotherapy in
R. Noninvasive breast carcinoma: frequency of unsus-
breast conserving treatment for ducal carcinoma in
pected invasion and implications for treatment. Ann
situ: first results of EORTC randomized phase III trial
Surg. 1979;1989:377–82.
10853. Lancet. 2000;355:528–33.
5. Holland R, Hendriks J, Verbeek A, et al. Extend,
16. Fisher B, Dignam J, Wolmark N, et al. Tamoxifen
distribution, and mammographic/histological cor-
in treatment of intraductal breast cancer: National
reclations of breast ductal carcinoma in situ. Lancet.
Surgical Adjuvant Breast and Bowel Project B-24 ran-
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domised controlled trial. Lancet. 1999;353:1993–2000.
6. Eva SS. Rating the risk factors for breast cancer. Ann
17. Solin LJ, Gray R, Bahner FL, et al. A multigene
Surg. 2003;237(4):474–82.
expression assay to predict local recurrence risk for
7. National Comprehensive Cancer Network. Breast
ductal carcinoma in situ of the breast. J Natl Cancer
cancer. Genetic/familial high-risk assessment: breast
Inst. 2013;105(10):701–10.
and ovarian. Version 3.2017. https://www.nccn.org/
18. Rackovich E, Nofech-Mozes S, Henna W, et al. A
professionals/physician_gls/pdf/genetics_screening.
population-based validation study of the DCIS score
pdf. Accessed 2/2018.
predicting recurrence risk in individuals treated by
8. Holland R, Peterse J, Millis R, et al. Ductal carcinoma
breast-conserving surgery alone. Breast Cancer Res
in situ: a proposal for a new classification. Semin
Treat. 2015;152(2):389–98.
Diagn Pathol. 1994;11:167–80.
19. Moran MS, Schnitt SJ, Giuliano AE, et al. Society of
9. Silverstein MJ, Poller D, Waisman J, et al. Prognostic
Surgical Oncology-American Society for Radiation
classification of breast ductal carcinoma-in-situ.
Oncology consensus guideline on margins for breast-
Lancet. 1995;345:1154–7.
conserving surgery with whole-breast irradiation in
10. Fisher B, Land S, Mamounas E, et al. Prevention
stages I and II invasive breast cancer. Ann Surg Oncol.
of invasive breast cancer in women with ductal car-
2014;21(3):704–16.
cinoma in situ: an update of the National Surgical
20. Rosenberger LH, Mamtani A, Fuzesi S, et al. Early
Adjuvant Breast and Bowel Project experience.
adoption of the SSO-ASTRO consensus guide-
Semin Oncol. 2001;28:400–18.
lines on margins for breast-conserving surgery with
11. UK Coordinating Committee on Cancer Research
whole-breast irradiation in stage I and II invasive
(UKCCR), Ductal Carcinoma in Situ (DCIS)
breast cancer: initial experience from Memorial
Working Party. Radiotherapy and tamoxifen in
Sloan Kettering Cancer Center. Ann Surg Oncol.
women with completely excised ductal carcinoma
2016;23(10):3239–46.
in situe of the breast in the UK, Australia, and
Lobular Carcinoma In Situ
21
Algorithmic Approach performed if radiologic and pathologic find-

ings are discordant, as this carries a risk of
A. LCIS is a high-risk atypical breast lesion.
upgrade to cancer at excisional biopsy as high
Women diagnosed with classic type LCIS as 38% in modern series [2, 3]. Excisional
have a 20–25% 10-year risk of developing biopsy is performed with localization of the
invasive breast cancer [1]. Studies show that biopsy site and any residual imaging findings.
this risk exists for both breasts. Historically, it It is not necessary to achieve a negative mar-
had been shown that the risk of developing gin for classic LCIS if specimen radiograph
invasive cancer was equal in the ipsilateral and pathology confirm removal of the suspi-
and contralateral breasts [1]. More recent cious area without additional findings. If can-
studies suggest that the risk is somewhat cer is found, margins should be assessed
higher in the ipsilateral breast although both following the margin guidelines outlined in
breasts are still at risk. the corresponding chapters.
B. The first step in evaluation of a patient with D. While some studies suggest classic LCIS

LCIS is to determine how and why the biopsy diagnosed on core biopsy in normal risk
was performed and whether other disease was patients diagnosed on screening mammogra-
associated with the LCIS. Most often, LCIS is phy with confirmed radiology-pathology con-
thought of as an incidental finding on biopsy cordance can be monitored on imaging and
of an imaging abnormality such as mass, cal- do not require excisional biopsy, this has not
cifications, or MRI enhancement [2]. been widely adopted as practice. The most
C. Excisional biopsy should be performed if a recent NCCN guidelines for breast cancer
mass was present or there is another finding, recommend not excising LCIS with concor-
such as radial scar, for which excision is rec- dance (biopsy otherwise benign) [4].
ommended. Excisional biopsy should also be Confirming concordance is the responsibility
of the surgeon and requires discussion with
the radiologist and the pathologist to ensure
A. R. Thawani agreement [5]. Pursuing this option requires
monitoring the biopsied area with imaging
[5]. Evidence demonstrating a higher upstage
L. M. Erdahl (*)
rate at excisional biopsy in women who had
Iowa City, IA, USA LCIS found on high-risk screening protocol
e-mail: [email protected] had the following: imaging for a clinically

palpable mass, >4 foci of atypia at biopsy, reduce the risk of breast cancer by more than
LCIS with associated atypical ductal hyper- 50% [9, 10]. Modifiable risk factors for breast
plasia, or LCIS found with a history of partial cancer should also be discussed including
mastectomy for breast cancer [3]. obesity, sedentary lifestyle, and alcohol
E. The risk of upstage to cancer in a woman intake. Another strategy that is sometimes
undergoing surgical excision of LCIS is 3% considered for risk reduction is bilateral pro-
overall with higher risk if excision is per- phylactic mastectomies. Comorbidities that
formed for high-risk features listed in point D impact risk and alternatives of high-risk
up to 38% [3, 6]. Women should be counseled screening, hormonal therapy, and lifestyle
prior to excision that additional therapy may modification should be discussed with a
be recommended depending on the final woman considering prophylactic mastecto-
excision. mies for LCIS.
F. Women with a personal history of LCIS are at H. Pleomorphic LCIS is a special type of LCIS
increased risk of developing cancer of and there is little research with long-term
approximately 1–2% per year [1, 7]. Due to follow-up looking at the outcomes of PLCIS
the increased risk, high-risk screening should [11]. Excision to negative margins may
be recommended. Screening should include require mastectomy and the impact of clear
clinical breast exam every 6 months and margins on long-term outcome is not clear
yearly mammogram with consideration of [11, 12]. Multidisciplinary discussion of
yearly MRI [5, 8]. cases can aid in developing a treatment plan
G. After excision or biopsy of classic LCIS,
as the role of radiation therapy is unclear.
women should be offered risk-reducing Hormonal therapy should be offered as in
hormonal therapy which has been shown to all LCIS.
21 Lobular Carcinoma In Situ 83
B LCIS diagnosed on core biopsy
Pleiomorphic LCIS Classic LCIS
Radiologic-pathologic
Review pathology, discuss Discordant or
high risk
concordance no D
excision to negative other lesion
margins
C
High risk screening F
Consider radiation Excisional biopsy with and
Consider hormonal therapy marker localization* discuss risk
reduction G
DCIS or invasive
cancer Classic LCIS
Use algorithms for

High risk screening F
and
DCIS or invasive
discuss risk
cancer
reduction
G
Algorithm 21.1
5. Landercasper J, Linebarger JH. Contemporary breast

References imaging and concordance assessment: a surgical per-
spective. Surg Clin North Am. 2011;91(1):33–58.
1. Haagensen CD, Lane N, Lattes R, et al. Lobular neo- 6. Murray MP, Luedtke C, Liberman L, et al. Classic
plasia (so-called lobular carcinoma in situ) of the lobular carcinoma in situ and atypical lobular
breast. Cancer. 1978;42(2):737–69. hyperplasia at percutaneous breast core biopsy:
2. Degnim AC, King TA. Surgical management of outcomes of prospective excision. Cancer.
high-risk breast lesions. Surg Clin North Am. 2012;119(5):1073–9.
2013;93(2):329–40. 7. King TA, Pilewskie M, Muhsen S, et al. Lobular
3. Rendi MH, Dintzis SM, Lehman CD, et al. Lobular carcinoma in situ: a 29-year longitudinal experience
in-situ neoplasia on breast core needle biopsy: imag- evaluating clinicopathologic features and breast can-
ing indication and pathologic extent can identify cer risk. J Clin Oncol. 2015;33(33):3945–52.
which patients require excisional biopsy. Ann Surg 8. National Comprehensive Cancer Network. Clinical
Oncol. 2012;19(3):914–21. practice guidelines in oncology. Breast cancer.
4. National Comprehensive Cancer Network. Clinical Genetic/familial high-risk assessment: breast and
practice guidelines in oncology. Breast cancer. ovarian. Version 3.2017. https://www.nccn.org/pro-
Version 4.2017. https://www.nccn.org/professionals/ fessionals/physician_gls/pdf/genetics_screening.pdf.
physician_gls/pdf/breast.pdf. Accessed 2/2018. Accessed 2/2018.
9. Fisher B, Costantino JP, Wickerham DL, et al. 11. De Brot M, Mautner SK, Muhsen S, et al. Pleomorphic
Tamoxifen for prevention of breast cancer: report of the lobular carcinoma in situ of the breast: a single insti-
National Surgical Adjuvant Breast and Bowel Project tution experience with clinical follow-up and cen-
P-1 Study. J Natl Cancer Inst. 1998;90(18):1371–88. tralized pathology review. Breast Cancer Res Treat.
10. Vogel VG, Costantino JP, Wickerham DL, et al. Effects 2017;165:411–20.
of tamoxifen vs raloxifene on the risk of developing 12. Flanagan MR, Rendi MH, Calhoun KE, Anderson
invasive breast cancer and other disease outcomes: the BO, Javid SH. Pleomorphic lobular carcinoma in situ:
NSABP study of tamoxifen and raloxifene (STAR) radiologic-pathologic features and clinical manage-
P-2 trial. JAMA. 2006;295(23):2727–41. ment. Ann Surg Oncol. 2015;22(13):4263–9.
Enlarged Axillary Lymph Node
22
Algorithmic Approach cancers, melanomas, thyroid cancers, skin

cancers, sarcomas, lung cancers, and gastric
A. The differential diagnosis of an enlarged axil- cancers [1]. Tumor markers can help narrow
lary lymph node includes reactive lymphade- the diagnosis and include carcinoembryonic
nopathy, lymphoma, and metastatic antigen (CEA), estrogen receptor (ER) and
carcinoma. When a patient presents with an progesterone receptor (PR), mammaglobin,
enlarged axillary node, a detailed history and cytokeratins 7 and 20, CA 125, and thyroid
physical are essential, focusing on details transcription factor (TTF-1). If the biopsy
such as tobacco use, recent travel, recent ill- demonstrates metastatic breast cancer and
ness, history of prior cancers (particularly mammography and ultrasound is unable to
breast cancer), and symptoms such as cough, demonstrate a primary breast lesion, MRI is
night sweats, or weight loss. A thorough recommended as it can detect 50% of occult
breast examination should be performed, and breast cancers [2]. In addition, staging should
imaging including mammography and ultra- be completed with CT chest, abdomen, pelvis,
sound should be ordered. and bone scan, or PET CT if indicated. If a
B. One of the first steps in a patient presenting primary lesion is identified on MRI, it should
with new onset axillary adenopathy is a biopsy be biopsied under MRI guidance. The patient
of the lymph node; core needle is preferred should then be managed depending on the
over FNA. Once a malignancy is identified, clinical stage and receptors.
tumor markers are evaluated to identify the C. If the biopsy is consistent with a breast pri-
primary site of disease and to guide manage- mary and no primary lesion is identified, this
ment. Neoplasms presenting with axillary is referred to as “occult breast cancer,” repre-
nodal involvement include lymphomas, breast senting 0.1–0.8% of all newly diagnosed
breast cancers [3, 4]. In this patient popula-
tion, in addition to systemic therapy, the
options include mastectomy with axillary dis-
section or, alternatively, axillary node dissec-
Z. Bostanci (*)
tion and radiation to the breast with equivalent
Breast Surgical Oncology, Ironwood Cancer and
Research Centers, Avondale, AZ, USA survival outcomes [5]. Adjuvant systemic
therapies are given, depending on the tumor
L. Kruper
Breast Surgical Oncology, Department of Surgery, markers (ER, PR, Her2neu). Neoadjuvant
City of Hope Hospital, Duarte, CA, USA chemotherapy may be considered.

86 Z. Bostanci and L. Kruper
A 55-year-old woman palpated an axillary mass. She has no other complaints. On physical
A eamination she has a mobile, solitary lymph node at the right axilla. Examination of
bilateral breasts is within normal limits. She has no supraclavicular or cervical adenopathy
Obtain bilateral mammogram and ultrasound and right axillary ultrasound with biopsy of the
lymph node, if suspicious appearing on ultrasound
B Bilateral mammogram shows scattered fibroglandular breasts.

No suspicious lesions on mammography or ultrasound.
Axillary ultrasound shows a solitary, enlarged lymph node
with cortical thickening and loss of fatty hilum which was
biopsied. Biopsy shows adenocarcinoma consistent with
breast primary
Obtain bilateral breast MRI with contrast (also obtain CT chest

abdomen, pelvis, and bone scan to rule out metastatic disease)
Primary lesion
identified on
MRI?
C Yes D No
MRI-guided biopsy and Discussion with the patient about right

management directed by mastectomy and axillary node dissection vs
biopsy results axillary node dissection and radiation
Algorithm 22.1
4. Walker GV, Smith GL, Perkins GH, Oh JL, Woodward

References W, Yu T-K, et al. Population-based analysis of occult
primary breast cancer with axillary lymph node
1. NCCN clinical practice guidelines in oncology occult metastasis. Cancer. 2010;116(17):4000–6.
primary version 2.2017 [Internet]. 2016 [cited 2017 5. He M, Tang L-C, Yu K-D, Cao A-Y, Shen Z-Z, Shao
Sep 4.]. Available from: https://www.nccn.org/profes- Z-M, et al. Treatment outcomes and unfavorable prog-
sionals/physician_gls/pdf/occult.pdf. nostic factors in patients with occult breast cancer.
2. Buchanan CL, Morris EA, Dorn PL, Borgen PI, Van Eur J Surg Oncol J Eur Soc Surg Oncol Br Assoc Surg
Zee KJ. Utility of breast magnetic resonance imag- Oncol. 2012;38(11):1022–8.
ing in patients with occult primary breast cancer. Ann
Surg Oncol. 2005;12(12):1045–53.
3. Foroudi F, Tiver KW. Occult breast carcinoma pre-
senting as axillary metastases. Int J Radiat Oncol Biol
Phys. 2000;47(1):143–7.
Metastatic Breast Cancer
23
Algorithmic Approach PET CT may be used if the CT scan or bone

scan is equivocal or suspicious. PET CT is not
A. When a patient presents with history and
indicated for early stage breast cancer or oper-
physical examination findings concerning able stage III disease [2]. In addition to the
metastatic breast cancer, initial workup staging work up, any patient (<60 years old)
should include breast imaging and tissue diagnosed with triple negative breast cancer
biopsy. This should include bilateral diagnos- should be referred for genetic testing [3].
tic mammography, ultrasound(s), and biop- C. Depending on the practice setting, patient
sies of any breast masses and suspicious should be referred to medical oncology once
lymph nodes for characterization of the tumor there is a suspicion for metastatic disease. If
and confirming metastatic disease in the staging scans are concerning for metastatic
axilla, infraclavicular, or supraclavicular disease, biopsy should be performed to con-
areas. firm the diagnosis. The tumor markers ER,
B. Metastatic breast cancer at the time of initial PR, and Her2neu should be tested from the
diagnosis (de novo stage IV breast cancer) metastatic focus as these will guide treat-
compromises 5–6% of women with newly ment. It is important to involve medical
diagnosed breast cancer [1]. According to oncologists early as systemic therapy should
NCCN guidelines, patients who present with be started urgently after diagnosis.
clinical stage IIIA disease or higher or lower D. The role of surgery is limited in metastatic
stage with symptoms concerning metastatic breast cancer. When there is an excellent
disease will need systemic staging with CT response to systemic therapy, resection of the
chest, abdomen, and pelvis and bone scan. metastatic focus (lung, liver) may be consid-
ered if there is no prohibitive risk to surgery
[4, 5]. Surgery on the primary breast tumor is
controversial. Surgery should not be recom-
mended if the distant disease is progressing;
however, breast surgery may be considered in
Z. Bostanci (*)
selected patients with well-controlled oligo-
Research Centers, Avondale, AZ, USA metastatic distant disease with multidisci-
plinary input [6]. Ultimately, some patients
L. Kruper
Breast Surgical Oncology, Department of Surgery, might require a palliative mastectomy to con-
City of Hope Hospital, Duarte, CA, USA trol infection, bleeding, or pain.

A
A 55-year-old woman felt a mass in her right breast a couple months ago. She never had screening
mammography. Recently, she noticed that the mass was getting larger and she also felt a lump at
her armpit. She has no other complaints. On physical examination, she has a 6 cm mass taking up
most of the right breast with nipple retraction. She also has bulky lymphadenopathy at right axilla
Obtain bilateral mammogram and right breast and axillary ultrasound with biopsy
of the mass and lymph nodes
Mammogram shows a 6 cm spiculated mass at the central right

breast. Ultrasound shows a 5.8 cm hypodense mass with irregular
borders and posterior shadowing at retroareolar position. There are
several suspicious lymph nodes with cortical thickening and loss of
fatty hilum. Biopsy of the mass shows invasive ductal carcinoma,
grade 3, triple negative. Biopsy of a representative lymph node
confirms metastatic adenocarcinoma consistent with breast primary
B Obtain CBC, CMP, CT chest, abdomen, pelvis, bone scan or PET CT, and genetic testing
C CT scan shows a solitary liver mass; biopsy was performed and

shows metastatic triple negative breast cancer. Patient referred to
medical oncology
Chemotherapy
Response?
Stable or regression Worsening disease
E Consider metastetectomy
Management per medical
oncology (change chemotherapy
and reassess)
Algorithm 23.1
23 Metastatic Breast Cancer 89
References 3]. Available from: https://www.nccn.org/profession-

als/physician_gls/pdf/genetics_screening.pdf.
4. Sadot E, Lee SY, Sofocleous CT, Solomon SB, Gönen
1. Breast cancer facts & figures. American Cancer
M, Kingham TP, et al. Hepatic resection or ablation
Society [Internet]. [cited 2017 Sept 3]. Available
for isolated breast cancer liver metastasis: a case-
from: https://www.cancer.org/research/cancer-facts-
control study with comparison to medically treated
statistics/breast-cancer-facts-figures.html.
patients. Ann Surg. 2016;264(1):147–54.
2. NCCN clinical practice guidelines in oncology breast
5. Macherey S, Mallmann P, Malter W, Doerr F,
cancer, version 2.2017 [Internet]. 2017 [cited 2017
Heldwein M, Wahlers T, et al. Lung metastasec-
Sept 3]. Available from: https://www.nccn.org/profes-
tomy for pulmonary metastatic breast carcinoma.
sionals/physician_gls/pdf/breast.pdf.
Geburtshilfe Frauenheilkd. 2017;77(6):645–50.
3. NCCN clinical practice guidelines in oncology
6. Khan SA. Surgical management of de novo stage IV
genetic/familial high risk assessment: breast and ovar-
breast cancer. Semin Radiat Oncol. 2016;26(1):79–86.
ian version 2.2017 [Internet]. 2016 [cited 2017 Sept
Recurrent Breast Cancer
24
Algorithmic Approach diation (APBI), and the presence of a genetic

mutation, all factors into how the patient
A. Patients with history of breast cancer are rou- should be followed and what findings raise
tinely followed to monitor for recurrence. clinical suspicion. Other important details
Most breast cancer recurrences occur within include initial stage of disease, response to
the first 5 years of diagnosis [1]. For this rea- neoadjuvant chemotherapy, and tumor type
son, National Comprehensive Cancer (i.e., hormone receptor positive, Her2neu
Network Guidelines recommend that patients positive, triple negative) since the risk of
treated for breast cancer undergo a history recurrence is dependent on such factors. A
and physical examination 1–4 times per year careful review of systems should be per-
(as clinically indicated) for 5 years and then formed at each visit, assessing symptoms
annually thereafter [2]. For patients who have such as abdominal pain, chest pain, cough,
undergone breast conserving therapy (BCT), bone pain, weakness, recent weight loss, per-
mammography is performed every 12 months. sistent headache, and blurred vision.
Ultrasound can be utilized to complement B. Once a breast cancer recurrence is suspected,
mammography when specified. In following whether by examination or imaging, a tissue
the patient with prior breast cancer, it is diagnosis is needed. It is important to know
important to note the details of treatment the status of estrogen, progesterone, and
including surgery, adjuvant therapies, and, if Her2neu receptors as these will guide sys-
a component of prior therapy, response to temic therapies.
neoadjuvant chemotherapy and type of radia- C. After confirming breast cancer recurrence,
tion. Whether the patient had a lumpectomy metastatic disease should be ruled out with
or mastectomy, sentinel lymph node biopsy staging scans and labs (CBC and CMP). A
or axillary node dissection, whole breast radi- CT scan of the chest, abdomen, and pelvis
ation (WBI) or accelerated partial breast irra- along with a bone scan are indicated. PET/CT
is most helpful in situations where standard
imaging studies are equivocal [2]. Brain MRI
Z. Bostanci (*)
is indicated if central nervous system symp-
Research Centers, Avondale, AZ, USA toms present.
D. If there is no evidence of distant metastasis on
L. Kruper
Breast Surgical Oncology, Department of Surgery, imaging, the case should be discussed in a
City of Hope Hospital, Duarte, CA, USA multidisciplinary fashion including surgery,

medical oncology, and radiation oncology Table 24.1 Management of local recurrence
with plastic surgery and genetics as indicated. Primary breast cancer Recurrent breast cancer
Systemic therapy is indicated for patients treatment treatment
with recurrent disease. If the recurrence is Lumpectomy + WBI Mastectomy
Lumpectomy + Mastectomy or lumpectomy +
unresectable upon presentation, systemic
APBI WBI
therapy should be the first step of treatment. Mastectomy without Excision of chest wall
Otherwise, there is no consensus on whether reconstruction mass + radiation
systemic therapies should be given before or Mastectomy + Excision of chest wall mass
after surgery. In cases where the recurrence implant-based with possible removal of the
reconstruction implant + radiation
has a more aggressive phenotype such as tri-
Mastectomy + Excision of chest wall
ple negative or Her2neu positive, chemother- tissue-based mass + radiation
apy may be recommended prior to surgery. If reconstruction
metastatic disease is identified, patient should
be referred to medical oncology and radiation
oncology. (Please see metastatic breast can- If patient had sentinel node biopsy at the time
cer topic.) of lumpectomy, repeat sentinel node biopsy at the
E. The patient’s previous treatment history is time of surgery may be performed.
taken into account when deciding surgical Regional recurrence is treated with surgery and
management. Table 24.1 shows the most radiation (axillary recurrence) or radiation alone
common treatment option in each circum- (supraclavicular or internal mammary nodes) in
stance, and alterations to these may be neces- addition to systemic therapy. There are multiple
sary depending on patient preferences and layers of complexity with each patient, and multi-
input from medical oncology, radiation disciplinary decision-making is fundamental when
oncology, and plastic surgery. treating a patient with recurrent breast cancer.
24 Recurrent Breast Cancer 93
A 55-year-old woman with a history of Stage I left breast cancer treated with breast
A conservation therapy (lumpectomy and radiation) presents with a new density
adjacent to the surgical bed on surveillance mammography
Perform physical examination, left breast ultrasound, ultrasound guided vs.

stereotactic biopsy of the area of concern
B
No distinct mass palpated at the area of concern. No palpable axillary lymphadenopathy.
1.1 × 1.2 × 1.5 cm mass was seen on ultrasound and biopsied which shows invasive
ductal carcinoma
C Obtain CBC, CMP, CT chest, abdomen, pelvis and bone scan or PET CT
Evidence of
distant
metastasis?
CT scan shows bone lesions at L2,

No evidence of distant metastasis on imaging
L3, L4, and femur
Multidisciplinary Refer the patient to medical oncology and

discussion for upfront radiation oncology
surgery or surgery
following chemotherapy
Systemic therapies are recommended

Surgery and systemic depending on receptor status. Radiation
therapies depending on therapy can be offered to treat bone pain
E receptor status
Algorithm 24.1
References tomy, and total mastectomy followed by irradiation. N

Engl J Med. 2002;347(8):567–75.
1. Fisher B, Jeong J-H, Anderson S, Bryant J, Fisher ER, cancer, version 2.2017 [Internet]. 2017 [cited 2017
Wolmark N. Twenty-five-year follow-up of a random- Sept 3]. Available from: https://www.nccn.org/profes-
ized trial comparing radical mastectomy, total mastec- sionals/physician_gls/pdf/breast.pdf.
Paget’s Disease
25
Algorithmic Approach should be obtained [2]. Of note, even when a

mass is identified with mammography or
A. A concern for Paget’s disease is raised in any ultrasound and diagnosis confirmed with
patient presenting with eczema of the nipple biopsy, MRI may still be useful in evaluating
or areola, bleeding, yellowish exudate, and the extent of disease (i.e., continuity between
itching or ulceration of the nipple. Paget’s the mass and NAC) which can aid in surgical
disease of the breast is rare, accounting for planning (central lumpectomy vs.
1–3% of newly diagnosed breast cancers. mastectomy).
With Paget’s, greater than 90% are associated D. If MRI demonstrates a mass not visualized on
with intraductal or invasive breast cancer [1]. mammography or ultrasound, an MRI-guided
The presentation may be confused by derma- biopsy should be performed. Depending on
tologic conditions which may delay the the size of the mass and the relationship
diagnosis. between the mass and NAC, mastectomy
B. When Paget’s disease is suspected, a thor- with sentinel lymph node biopsy (SLNB) or
ough physical examination of the breast lumpectomy including NAC (central lumpec-
should be performed. If there is an underlying tomy) ± SLNB may be considered and dis-
cancer in the breast, the cancer may be remote cussed with the patient [3]. Patients treated
from the nipple-areolar complex (NAC). A with breast conservation will need radiation
punch biopsy of the affected nipple skin therapy.
should be obtained for diagnosis. E. If no underlying cancer is identified within
C. Initial evaluation includes mammography the breast and the patient has isolated Paget’s
and ultrasound. If the nipple biopsy demon- disease of the nipple, options include central
strates Paget’s and initial imaging fails to lumpectomy ± SLNB and radiation or mas-
detect a cancer within the breast, breast MRI tectomy with SLNB [3].
Z. Bostanci (*)
Research Centers, Avondale, AZ, USA
L. Kruper
Breast Surgical Oncology, Department of Surgery,
City of Hope Hospital, Duarte, CA, USA

A 55-year-old woman is complaining of itching and burning sensation of the right nipple. She
A also noticed that it is red and scaly. She does not have nipple discharge. On examination,
right nipple has eczematous changes. There are no distinct masses or skin changes at the rest
of the breast, no lymphadenopathy. Her last mammogram was a year ago and normal.
Obtain bilateral mammogram and right breast

B
ultrasound, punch biopsy of the nipple
Bilateral mammogram shows heterogeneously dense breasts.

No suspicious lesions on mammography or ultrasound. Punch
abiopsy shows tumor cells in the epidermis consistent with
Paget’s disease
Obtain breast MRI with contrast
Concomitant
malignancy
identified on MRI?
D Yes E No
MRI-guided biopsy Discuss with the patient about right

mastectomy and sentinel node biopsy or right
breast central lumpectomy + radiation
Depending on the biopsy results and the

relationship between the mass and the
nipple-areolar complex (NAC),
mastectomy with SLNB, excision of NAC
and lumpectomy, ± SLNB, or central
lumpectomy including the mass and nipple
± SLNB may be considered
Algorithm 25.1
disease in select patients with Paget disease of the

References nipple. J Am Coll Surg. 2008;206(2):316–21.
1. Yim JH, Wick MR, Philpott GW, Norton JA, Doherty cancer, version 2.2017 [Internet]. 2017 [cited 2017
GM. Underlying pathology in mammary Paget’s dis- Sept 3]. Available from: https://www.nccn.org/profes-
ease. Ann Surg Oncol. 1997;4(4):287–92. sionals/physician_gls/pdf/breast.pdf.
2. Morrogh M, Morris EA, Liberman L, Van Zee K,
Cody HS, King TA. MRI identifies otherwise occult
Locoregional Recurrence of Breast
Cancer 26
Algorithmic Approach rent disease on the chest wall or reconstructed

breast. Chest wall recurrence after mastec-
A. Patients who have had breast cancer should tomy is associated with the development of
undergo close follow-up after management of metastatic disease. Most LRR occurs within a
their primary disease. A patient with a per- median of 4 years of the original cancer diag-
sonal history of breast cancer is at risk for the nosis [2]. Triple negative and HER-2/neu-
development of recurrence in the ipsilateral positive tumors are more likely to develop
breast after breast-conserving surgery (BCT) LRR than tumors that are hormone receptor
and for the development of a contralateral positive [8–10].
second primary [1, 2]. The most significant B. Surveillance recommendations include peri-
risk factor for in-breast recurrence after BCT odic history and physical examination by the
is a positive lumpectomy margin [2]. Other surgeon every 3–6 months for the first 3 years,
risk factors for in-breast recurrence include every 6–12 months for the next 2 years, and
young age, larger tumor size, lymphovascular annually after 5 years of follow-up is com-
invasion, multifocal disease, high nuclear pleted [11]. Patients who have undergone
grade, lymph node positivity, LCIS, triple BCT are at risk for ipsilateral breast tumor
negative or HER-2/neu positive disease, and recurrence, along with the development of a
the lack of radiation therapy to the breast after contralateral second primary breast cancer.
a lumpectomy [2, 3]. Isolated regional lymph Mammography of the affected breast every
node recurrences are uncommon, with vari- 6 months for the first 2 years after treatment is
ous studies citing rates of 0.2–1.6% in clini- recommended, followed by annual exams.
cally node-negative patients who undergo Other imaging may also be incorporated as
sentinel node biopsy [4–6]. In-breast recur- indicated, based on breast density and other
rence after BCT has recently been shown to factors. After mastectomy, LRR may present
be 2% at 5 years and 5% at 10 years in a large as a palpable superficial mass on the chest
cohort from the Netherlands [7]. After mas- wall (or reconstructed breast) or in the axilla.
tectomy surgery, patients may develop recur- Most patients will also be followed by their
medical oncologists, who may recommend
blood work and other imaging as per proto-
J. C. Gooch · F. Schnabel (*)
col. The workup for a suspected locoregional
Department of Surgery, NYU Langone Health, NYU
Perlmutter Cancer Center, New York, NY, USA recurrence starts with a full history and physi-
e-mail: [email protected] cal exam. The history should include the

98 J. C. Gooch and F. Schnabel
relevant details of the patient’s previous recurrence should be widely excised, remap-
breast cancer, including the clinicopathologic ping sentinel nodes should be done if possi-
features of the tumor, the hormone, and ble, and the patient referred for radiation
HER-2/neu status as well as the type of breast treatment (RT) as well as systemic therapy
and axillary surgery, radiation treatment (RT), [3]. Patients with isolated axillary recurrence
and systemic therapy. after sentinel node biopsy should undergo a
C. The patient should undergo a thorough workup completion axillary dissection followed by
to evaluate the extent of the recurrent disease. radiation treatment and systemic therapy as
When LRR is suspected after breast- appropriate. In cases of axillary recurrence
conserving surgery, patients should undergo after prior axillary dissection, surgical resec-
diagnostic mammography with ultrasound tion should be performed for local control of
and/or MRI as clinically indicated. Tissue disease and followed by radiation treatment
sampling of all suspect areas is required to and systemic therapy as appropriate.
confirm diagnosis and establish the biomarker Recurrences involving the internal mammary
profile. Extensive or high-risk local disease or supraclavicular nodes should be referred
may also necessitate imaging to rule out dis- for radiation and systemic treatment [3].
tant metastases. Chest wall recurrences after E. After local management is complete, the
mastectomy are associated with the develop- patient should be referred to a medical oncol-
ment of distant metastases, and these patients ogist for a discussion of their systemic treat-
should be evaluated to rule out distant disease. ment options. The purpose of systemic therapy
PET/CT or CT of the chest, abdomen, and pel- is to reduce the risk for the development of
vis and bone scan are useful in this regard [3]. metastatic disease. Recommendations for sys-
D. Local recurrences may be categorized as
temic treatment will be based on the patient’s
either an in-breast recurrence after breast prior disease and treatment along with the
conservation, axillary recurrence, or a chest details of the recurrence, including the histo-
wall recurrence after mastectomy. In patients pathologic factors and biomarker profile [12].
who had previous lumpectomy without radia- F. The patient with LRR will require careful
tion, it may be possible to perform a second follow-up. Regular breast imaging is impor-
breast-conserving surgery with the addition tant to monitor any remaining breast tissue.
of post-lumpectomy radiation. In the case of Annual mammography and physical exam
the patient who has had prior lumpectomy every 6–12 months with the surgeon is war-
with radiation treatment, a completion total ranted. Blood work and other imaging may be
mastectomy is the standard approach [3]. If directed by the medical oncologist, with the
the patient had a prior sentinel node biopsy focus on potential sites of disease. Any new
and is clinically node negative, it is appropri- symptoms should have further evaluation.
ate to attempt to map the axilla and perform a The following algorithm for management of
second sentinel node biopsy. In patients who locoregional recurrence is adapted from the
have had previous mastectomy, a chest wall NCCN guidelines [11].
26 Locoregional Recurrence of Breast Cancer 99
Patient presents with palpable mass

A or
a new lesion is seen on routine
imaging/surveillance
B History and Physical Exam

-Previous cancer treatment: lumpectomy vs. mastectomy?
-Previous radiation and what dose
-Previous chemotherapy
-Previous endocrine therapy
-Palpable mass vs. imaging finding?
-Perform full physical exam, including bilateral breasts and lymph node basins
Imaging and Labs

C -Diagnostic mammogram if previous BCT and/or intact contralateral breast
-Full staging workup–consider US/CT/MRI for extent of breast/chest wall involvement
-Consider CT chest/abdomen/pelvis, PET/CT, bone scan, and CT/MRI of the brain to
evaluate for distant disease
-Biopsy of disease sites and evaluation of receptor status
-Check labs: CBC, CMP, LFTs, serum tumor markers
D In-breast recurrence Chest wall recurrence Nodal recurrence
-If previous BCT -If skin/chest wall -If axillary, resect if

with RT, perform recurrence, resect if possibleand refer for
mastectomy possible and radiation
consider radiation -If supraclavicular or
treatment internal mammary,
refer for radiation
treatment
E Referral to medical oncology for systemic chemotherapy and endocrine therapy

if hormone receptor positive
-Anti-Her2 therapy for Her2 positive disease
F
Follow-up
Algorithm 26.1
References B-32 randomised phase 3 trial. Lancet Oncol.

2010;11(10):927–33.
7. Bosma SC, et al. Very low local recurrence rates after
1. Lin NU, et al. International guidelines for manage-
breast-conserving therapy: analysis of 8485 patients
ment of metastatic breast cancer (MBC) from the
treated over a 28-year period. Breast Cancer Res
European School of Oncology (ESO)-MBC Task
Treat. 2016;156(2):391–400.
Force: surveillance, staging, and evaluation of patients
8. Lowery AJ, et al. Locoregional recurrence after breast
with early-stage and metastatic breast cancer. Breast.
cancer surger: a systematic review by receptor pheno-
2013;22(3):203–10.
type. Breast Cancer Res Treat. 2012;133:831–41.
2. Grotz TE, Boughey JC. Recurrent breast cancer. In: Wilke
9. Hattangadi-Gluth JA, et al. Basal subtype of inva-
LG, Chagpar AB, editors. American Society of Breast
sive breast cancer is associated with a higher
Surgeons breast surgery manual: American Society of
risk of true recurrence after conventional breast-
Breast Surgeons; 2012. www.breastsurgeons.org.
conserving therapy. Int J Radiat Oncol Biol Phys.
3. Shikama N, Sekiguchi K, Nakamura N. Management
2012;82(3):1185–91.
of locoregional recurrence of breast cancer. Breast
10. Gillon P, et al. Factors predictive of locoregional

Cancer. 2011;18(4):252–8.
recurrence following neoadjuvant chemotherapy in
4. Navarro-Rodriguez E, et al. Factors associated with
patients with large operable or locally advanced breast
disease recurrence in breast cancer patients with nega-
cancer: an analysis of the EORTC 10994/BIG 1-00
tive sentinel lymph node biopsy. Clin Breast Cancer.
study. Eur J Cancer. 2017;79:226–34.
2016;16(6):e181–6.
11.
Network, N.C.C. NCCN-evidence blocks–recur-
5. de Boniface J, et al. Ten-year report on axillary recur-
rent or stage IV disease. In: NCCN guidelines ver-
rence after negative sentinel node biopsy for breast
sion 2.2016–invasive breast cancer. Fort Washington.
cancer from the Swedish Multicentre Cohort Study.
www.nccn.org: National Comprehensive Cancer
Br J Surg. 2017;104(3):238–47.
Network; 2016. p. 17–23.
6. Krag DN, et al. Sentinel-lymph-node resection com-
12. Aebi S, et al. Chemotherapy for isolated locoregional
pared with conventional axillary-lymph-node dissec-
recurrence of breast cancer (CALOR): a randomised
tion in clinically node-negative patients with breast
trial. Lancet Oncol. 2014;15(2):156–63.
cancer: overall survival findings from the NSABP
Metastatic Breast Cancer
27
Algorithmic Approach nodes) should be evaluated with PET/CT (or

CT chest, abdomen, and pelvis and bone
A. Metastatic breast cancer accounts for about scan) to rule out metastatic disease. The most
6% of all newly diagnosed breast cancers, an common first site of metastasis in breast can-
incidence which has not changed despite cer is bone, with ribs and spine frequently
improvements in prognosis in recent years [1, involved. Other common sites for breast can-
2]. Once breast cancer is metastatic, it is no cer metastasis include the liver and lungs.
longer considered curable; however, a variety Biopsy of a suspected metastatic site is often
of treatment options to extend survival and required to confirm the diagnosis and provide
palliate symptoms are available. Five-year sur- information on the biomarker profile which
vival in stage IV disease is approximately 26% may be different from the primary.
although recent studies suggest this may be C. Systemic therapy is the initial treatment for
increasing to as much as 36% [2–4]. The algo- patients with metastatic disease at the time of
rithm is adapted from the NCCN guidelines presentation. The biomarker profile of the
for management of recurrent and metastatic tumor will dictate some of the appropriate
disease. This is a highly complex topic, the full therapeutic options. If the disease is ER/PR
scope of which is beyond this brief review [5]. positive, hormonal therapy (tamoxifen in pre-
B. Patients presenting with a new breast cancer menopausal patients, tamoxifen or aromatase
diagnosis should undergo a full history, phys- inhibitors in postmenopausal patients) is the
ical exam, and complete breast imaging. recommended first treatment. If the disease is
Biopsy of the primary site should be done to not hormone sensitive, chemotherapy is gen-
confirm the tumor histology and for bio- erally given. Anti-HER-2/neu therapy is rec-
marker analysis. Most commonly, this is done ommended for patients with HER-2/
via percutaneous or image-guided core nee- neu-positive disease. These patients should
dle biopsy. Patients presenting with locally be monitored closely to assess for response of
advanced breast cancer or those with high- the primary tumor. Although some studies
risk disease (large primaries, positive axillary have suggested a benefit of surgical excision
of the primary tumor, the mainstay of treat-
ment for metastatic disease is systemic
J. C. Gooch · F. Schnabel (*) therapy, with surgery or radiation treatment
Department of Surgery, NYU Langone Health, NYU for local control and palliation of symptoms
Perlmutter Cancer Center, New York, NY, USA [6–9]. A multidisciplinary approach is key.

D. Patients may present with metastatic disease at to other endocrine options. In this manner,
some time after their initial breast cancer treat- patients may cycle through tamoxifen and all
ment. Important factors to ascertain at the time three aromatase inhibitors. Other endocrine
of diagnosis of metastatic disease include treatments may include fulvestrant, which is
patient performance status, menopausal status, approved for treatment of metastatic breast
current symptoms, and history of prior treat- cancer in postmenopausal women [3]. In the
ments. The physical exam should include exam- case of disease that is resistant to endocrine
ination of the breasts and axillary, supraclavicular treatment, studies have shown that the addi-
and cervical lymph node basins, auscultation of tion of mTOR or CDK inhibitors such as
the chest, and palpation of the abdomen, at a everolimus or palbociclib in combination
minimum. Diagnostic breast imaging should be with endocrine treatment may improve pro-
performed to rule out a new primary breast can- gression-free survival [3].
cer. A full set of labs including CBC, CMP, F. Patients may progress through several lines of
LFTs, and serum tumor markers should also be therapy during the course of their disease. All
ordered. The full staging workup includes imag- recurrent or metastatic patients should be
ing of the chest, abdomen, pelvis, skeletal sys- offered the option of enrollment in clinical
tem, and brain using CT, MRI, PET/CT, or bone trials. Patients may benefit from surgical
scan. Any sites suspicious for metastatic disease resection or radiation therapy for symptom-
should be biopsied to confirm the diagnosis and atic control of selected lesions [6–9].
to evaluate the biomarker profile of the tumor, Radiation to bony metastasis may improve
which may differ from that of the original pri- pain control and reduce risk of pathologic
mary tumor [10, 11]. fractures, while surgery and radiation in con-
E. For patients with hormone-receptor-positive junction with systemic therapy has been
metastatic breast cancer, endocrine therapy is shown to increase median survival in some
appropriate as a first step. Targeted anti- studies of patients with brain metastasis [9].
HER-2/neu therapy may be used for patients The initial follow-up for patients with meta-
whose tumors overexpress HER-2/neu. For static disease will occur primarily in the med-
hormone-receptor-negative patients, the ical oncologist’s office. Labs should be
mainstay of treatment is chemotherapy. repeated as needed while actively on systemic
While multidrug regimens have been demon- treatment. Bone density testing should be
strated to provide a survival benefit when ordered every 2 years while on an aromatase
given as adjuvant therapy for local disease, inhibitor and echocardiogram every 3 months
the role of these regimens in metastatic dis- while on Herceptin. Imaging and follow-up
ease is still open to discussion [12]. However, with the surgeon should be considered on an
some studies have advocated polychemo- as-needed basis. Any new symptoms should
therapy for metastatic disease [13], citing prompt a return to the physician’s office for
longer survival and better overall quality of an exam and imaging to rule out progression
life. For premenopausal patients, tamoxifen of disease.
is the mainstay of hormonal treatment, with G. Palliative care referrals for assistance with
the option of ovarian suppression and an aro- symptoms such as pain or nausea may be
matase inhibitor in selected cases. In post- appropriate. Hospice referral is appropriate
menopausal patients, an aromatase inhibitor when patients have progression of disease
is the most common first-line hormonal ther- despite multiple lines of therapy and are
apy. Patients with metastatic ER/PR positive approaching the end of life. Maintenance of
breast cancer are treated with a hormonal qualify of life and attention to the patient’s
agent until the time of progression of dis- goals of care is key throughout the manage-
ease, at which time the treatment is changed ment of metastatic breast cancer.
27 Metastatic Breast Cancer 103
A Patient presents for evaluation of

breast cancer
New breast cancer diagnosis; Previous breast cancer diagnosis;

concern for advanced disease concern for recurrence or metastasis
B D
History and Physical Exam History and Physical Exam

-Perform full physical exam, including bilateral -Evaluate performance status, menopausal status,
breasts and lymph node basins current symptoms and previous treatment regimens
-Diagnostic mammogram and ultrasound -Perform full physical exam including bilateral breasts
-Full staging workup–consider US/CT/MRI for extent and lymph node basins, auscultation of chest and
of breast/chest wall involvement examination of abdomen
-Consider CT chest/abdomen/pelvis, PET/CT, bone
scan, and CT/MRI of the brain to evaluate for distant -Diagnostic mammogram if previous BCT and/or intact
disease contralateral breast
-Biopsy of disease sites and evaluation of receptor
status -Full staging workup – consider US/CT/MRI for extent
of breast/chest wall involvement
-Check labs: CBC, CMP, LFTs,
and serum tumor markers -Consider CT Chest/Abdomen/Pelvis, PET/CT, bone
scan and CT/MRI of the brain to evaluate for distant
disease
-Biopsy of disease sites and evaluation of receptor

For ER/PR-positive disease:
C -Tamoxifen (premenopausal) or aromatase inhibitor
status
(postmenopausal) -Check labs: CBC, CMP, LFTs, serum tumor markers
-Evaluation for systemic chemotherapy
E
For Her-2/neu-positive disease:
Add Trastuzumab/Pertuzumab
For ER/PR-positive disease:
-Tamoxifen (premenopausal) or aromatase inhibitor
For hormone-receptor-negative disease: (postmenopausal)
-Systemic chemotherapy -Consider ovarian ablation/suppression
-Evaluation for systemic chemotherapy
Local treatments:
-RT or surgery for symptom control as needed
For Her-2/neu-positive disease:
Stable disease Disease progression Add Trastuzumab/Pertuzumab
For hormone-receptor-negative disease:

F Follow-up -Chemotherapy -Systemic chemotherapy
-Consider second-
or third-line endocrine
Local treatments;
regimens
-RT or surgery for symptom control as needed
Stable
G Consider second- disease
or third-line
Disease Stable
chemotherapy
progression disease
Hospice/palliative regimens and/or
Follow-up
care referral clinical trial
enrollment
Algorithm 27.1
References 7. Ruiterkamp J, et al. Surgical resection of the pri-

mary tumour is associated with improved survival in
patients with distant metastatic breast cancer at diag-
1. Barinoff J, et al. Primary metastatic breast cancer
nosis. Eur J Surg Oncol. 2009;35(11):1146–51.
in the era of targeted therapy – prognostic impact
8. Ruiterkamp J, Ernst MF. The role of surgery in meta-
and the role of breast tumour surgery. Eur J Cancer.
static breast cancer. Eur J Cancer. 2011;47:S6–S22.
2017;83:116–24.
9. Budach W. Radiotherapy in patients with metastatic
2. Surveillance, E.a.E.R.P. Cancer stat facts: female
breast cancer. Eur J Cancer. 2011;47:S23–7.
breast cancer. In: Cancer stat facts. National Cancer
10. Van Poznak C, et al. Use of biomarkers to guide
Institute Surveillance, Epidemiology and End Results
decisions on systemic therapy for women with meta-
Program (SEER). seer.cancer.gov. 1975–2014.
static breast cancer: American Society of Clinical
3. Kaklamani V, Gradishar WJ. Endocrine therapy in
Oncology clinical practice guideline. J Clin Oncol.
the current management of postmenopausal estrogen
2015;33(24):2695–704.
receptor positive metastatic breast cancer. Oncologist.
11. Lin NU, et al. International guidelines for manage-
2017;22:507–17.
ment of metastatic breast cancer (MBC) from the
4. Mariotto AB, et al. Estimation of the number of
European School of Oncology (ESO)-MBC Task
women living with metastatic breast cancer in the
Force: surveillance, staging, and evaluation of patients
United States. Cancer Epidemiol Biomarkers Prev.
with early-stage and metastatic breast cancer. Breast.
2017;26(6):809–15.
2013;22(3):203–10.
5. Network, N.C.C. NCCN- evidence blocks – recur-
12. Cardoso F, et al. International guidelines for man-
rent or stage IV disease. In: NCCN guidelines ver-
agement of metastatic breast cancer: combination vs
sion 2.2016 – invasive breast cancer. Fort Washington.
sequential single-agent chemotherapy. J Natl Cancer
www.nccn.org: National Comprehensive Cancer
Inst. 2009;101(17):1174–81.
Network; 2016. p. 17–23.
13. Stockler M, et al. Systematic reviews of chemother-
6. Leung AM, et al. Effects of surgical excision on sur-
apy and endocrine therapy in metastatic breast cancer.
vival of patients with stage IV breast cancer. J Surg
Cancer Treat Rev. 2000;26(3):151–68.
Res. 2010;161(1):83–8.
Inflammatory Breast Cancer
28
Algorithmic Approach common, presenting in nearly 80% of

patients, according to one study [6]. The dif-
A. Inflammatory breast cancer is a rare presenta- ferential diagnosis at presentation may
tion of invasive breast cancer that is associ- include mastitis, and an initial course of anti-
ated with a poor prognosis. Between 1% and biotics may be a reasonable strategy.
5% of all newly diagnosed breast cancers in However, failure to respond promptly to
the United States each year present in this treatment should be the cause for concern and
manner [1–3]. The diagnosis is suggested by should trigger additional investigation with-
the clinical features at presentation and con- out delay [1–3].
firmed on tissue sampling. According to the C. Patients with suspected inflammatory breast
most recent AJCC staging criteria, inflamma- cancer should undergo diagnostic breast
tory cancer is by definition a T4d lesion. As a imaging, including bilateral mammography
result, these patients are at a minimum stage and additional imaging as appropriate.
IIIB at diagnosis [4, 5]. Imaging should include an evaluation of the
B. The first step in management is a full and axillary nodes. MRI may also be helpful and
thorough history and physical exam. The will demonstrate over 90% of cancers as well
diagnosis of inflammatory breast cancer as skin thickening and enhancement [1, 3].
relies on several clinical criteria [1]. These Core biopsies of any suspicious breast masses
include the rapid onset of breast erythema, should be done to confirm the diagnosis and
edema, and/or peau d’orange, with or without for biomarker analysis. In addition, a punch
the presence of an underlying mass. The ery- biopsy of involved skin should be done, as the
thema should occupy at least 1/3 of the breast. histologic hallmark of inflammatory breast
There may be nipple involvement such as cancer is tumor involvement of the dermal
flattening, crusting, or retraction. The symp- lymphatics, which produces the characteristic
toms should have been present for no longer presentation.
than 6 months. Involved lymph nodes are D. Once there is tissue confirmation of the dis-
ease, the patient should proceed expeditiously
to a workup to exclude metastatic disease,
including labs, bone scan, chest X-ray, CT, or
J. C. Gooch · F. Schnabel (*)
PET/CT. Regional nodal disease is common
Perlmutter Cancer Center, New York, NY, USA in inflammatory cancer, and 30% of patients
e-mail: [email protected] are found to have metastatic disease at

diagnosis [1, 3]. Suspicious axillary nodes radiation. Comprehensive nodal irradiation
should be biopsied to confirm the extent of (including the ipsilateral axilla, infraclavicu-
disease. The treatment of patients with meta- lar and supraclavicular nodes) is recom-
static breast cancer is discussed elsewhere in mended for patients with extensive nodal
this text. disease [2, 4]. Breast reconstruction may be
E. The contemporary approach to treatment for offered to patients with good response to neo-
patients with inflammatory breast cancer adjuvant treatment; however, the impact of
includes neoadjuvant chemotherapy, fol- post-mastectomy radiation should be taken
lowed by surgery and other treatment modali- into consideration (see Chap. 29, Breast
ties (including radiation treatment and Reconstruction).
endocrine therapy) as appropriate. The typi- G. After surgery, patients with HER-2/neu-

cal chemotherapy regimens include anthracy- positive disease will continue anti-HER-2
clines and taxanes [2–4]. Those patients treatment for 1 year. As noted above, post-
whose tumors overexpress HER-2/neu should mastectomy radiation is commonly recom-
also receive trastuzumab, and dual blockade mended for patients with inflammatory breast
with pertuzumab is also approved for neoad- cancer. Although up to 83% of inflammatory
juvant treatment of HER-2/neu-positive breast cancers lack hormone receptor expres-
patients [3, 4]. Patients should be followed sion, endocrine therapy is appropriate for
clinically during neoadjuvant treatment, with patients with ER/PR-positive disease [2].
repeat MRI at the completion of therapy to Following treatment, the patient should con-
evaluate for objective response. tinue close clinical follow-up including phys-
F. Patients who respond to chemotherapy and ical examination every 3–6 months and
have resectable disease are candidates for sur- regular imaging of the contralateral breast.
gical resection. The most common procedure These patients should also be monitored for
is a total mastectomy with evaluation of the the possibility of chest wall recurrence. As
axillary nodes as appropriate. However, a these patients are at increased risk for the
patient with excellent response to neoadjuvant development of metastatic disease, additional
treatment may be a candidate for less exten- follow-up with lab work and body imaging is
sive procedures [2]. Axillary nodal involve- appropriate and may be directed by the
ment is noted in up to 85% of patients, making patient’s medical oncologist. Any new symp-
those patients ineligible for sentinel node tom referable to the common sites for metas-
procedures [3]. Following surgery, most tasis from breast cancer (bone, lung, liver)
patients are candidates for post-mastectomy should be evaluated promptly.
28 Inflammatory Breast Cancer 107
History and physical exam

A Patient presents with painful erythematous
breast with rapid onset and progression of
symptoms
B Assess for presence of diagnostic criteria
–Rapid onset of breast erythema, edema, peau d’orange, with or without an underlying mass
–Flattening, crusting or retraction of the nipple may be present
–Duration of no more than 6 months
–History of mastitis not responding to antibiotics
–Examination reveals erythema occupying at least 1/3 of the breast and may reveal an underlying
mass or palpable lymph nodes
Imaging: Diagnostic mammography and ultrasound; consider MRI

–Evaluate axilla
C
–Biopsy of mass
–Skin punch biopsy
D Metastatic Workup
–Labs
–Bone Scan
–CT Yes Biopsy No
Course of
–PET/CT consistent with
antibiotics
E –biopsy of suspicious carcinoma
nodes
Metastatic disease absent Metastatic disease present

Close follow
–Definitive systemic chemotherapy up for
–Primary systemic chemotherapy improvement;
–Endocrine therapy for HR+ Insufficient –Endocrine therapy for HR+ tumors
–Trastuzumab/Pertuzumab for continue
tumors response workup
–Trastuzumab/Pertuzumab for HER2+ tumors
HER2+ tumors
Response to treatment
F Monitor for response to
therapy
–Modified Radical Mastectomy
–Axillary Lymph Node Dissection
G
Postmastectomy radiation
therapy –Continue systemic therapy Follow up: physical exam q3–6mo
–Consider delayed –imaging for contralateral breast
reconstruction –symptom directed workup for
suspicion of recurrence
Algorithm 28.1
References 3. Yamauchi H, et al. Inflammatory breast cancer: what

we know and what we need to learn. Oncologist.
2012;17(7):891–9.
1. Dawood S, et al. International expert panel on
4. Network NCC. NCCN – evidence blocks – inflam-
inflammatory breast cancer: consensus statement for
matory breast cancer, in NCCN guidelines version
standardized diagnosis and treatment. Ann Oncol.
2.2016 – Invasive breast cancer. 2016. p. 1–2.
2011;22(3):515–23.
5. Cancer AJCO. Breast cancer staging. 8th ed: Chicago,
2. van Uden DJ, et al. Inflammatory breast cancer: an over-
Illinois: A. C. Society; 2017.
view. Crit Rev Oncol Hematol. 2015;93(2):116–26.
6. Wecsler JS, et al. Lymph node status in inflam-
matory breast cancer. Breast Cancer Res Treat.
2015;151(1):113–20.
Breast Reconstruction
29
Algorithmic Approach ties as well as obesity are risk factors that may
increase the likelihood of postoperative
A. The initial stage in planning for breast recon- wound-healing complications, and these
struction is evaluation by the surgeon who will should be identified in the preoperative evalua-
perform the oncologic operation. The initial tion [1, 4]. Inflammatory breast cancer may be
evaluation focuses on the disease being treated a relative contraindication to immediate breast
and the appropriate options for the surgical reconstruction due to the need to resect
phase of treatment. Referral to a plastic surgeon involved skin, the risk of recurrence, and the
for discussion of applicable reconstructive need to move directly to postoperative radio-
options may follow. Women should be edu- therapy as soon as possible [1]. However, in
cated about the option for breast reconstruction current practice, the majority of these patients
as it has significant benefits for quality of life undergo neoadjuvant chemotherapy, and the
and emotional outcomes for the patient [1–3]. response to treatment may have an effect on
By federal law (Women’s Health and Cancer the posttreatment surgical options.
Rights Act), the cost of breast reconstruction C. The role of breast reconstruction varies with
after mastectomy is covered by medical insur- the type of oncologic surgery planned for
ance, and in New York State, a discussion of all treatment of the patient’s cancer. Patients
of the various breast reconstruction options, undergoing breast-conserving therapy should
including contralateral symmetry procedures, be evaluated for the likely magnitude of cos-
is also mandated by state law. metic defect after lumpectomy and the poten-
B. Plastic surgery consultation should include a tial benefit of oncoplastic techniques as part
full history and physical exam, including a of the procedure. Oncoplastic surgery aims to
focus on the cancer treatment plan, the patient’s improve the cosmetic results of breast cancer
desires and concerns as related to breast sur- surgery by mitigating the effects of resection
gery, the body habitus, smoking history, and of a volume of breast tissue and reducing the
general medical condition and comorbidities. deformities that may result. These techniques
Smoking and significant medical comorbidi- make use of advancement flaps of glandular
tissue and skin to fill the lumpectomy cavity
and may allow patients with unfavorable fea-
J. C. Gooch · F. Schnabel (*) tures to undergo a breast-conserving approach
Department of Surgery, NYU Langone Health, NYU with an acceptable cosmetic outcome [5].
Perlmutter Cancer Center, New York, NY, USA These patients often require a contralateral
reduction and/or mastopexy for symmetry.
D. As part of the preoperative evaluation, it is this approach does require multiple outpa-
important to determine whether or not the tient visits for expansion, and requires a sec-
patient is likely to require post-mastectomy ond surgical procedure. In addition, a breast
radiation therapy (PMRT) as this impacts on implant for reconstruction may not provide
the reconstructive decision-making. PMRT is an acceptable match for a contralateral native
felt to adversely affect autologous tissue breast, especially in women with large and
reconstruction and increases the rate of cap- pendulous breasts [2–4].
sular contracture of implant reconstructions Autologous reconstruction may be per-
[6]. Patients who are likely to undergo PMRT formed via any number of different flaps,
may be best served by placement of a tissue including the TRAM, DIEP, SIEA, and GAP
expander and expansion prior to beginning flaps [7], depending on patient body habitus
radiation treatments. This preserves the skin and availability of sufficient tissue. These
envelope and allows for the options of either flaps may be transferred on a vascular pedicle
continued implant-based reconstruction or or may utilize free tissue transfer techniques
conversion to autologous tissue reconstruc- with microvascular anastomoses to place the
tion after the completion of radiation treat- flap into position. Autologous reconstruction
ment [1]. PMRT increases the risk of capsular with free flaps and microsurgical anastomosis
contracture, poor cosmetic outcome, poor requires significantly more technical exper-
wound healing, and implant loss compared to tise than implant-based reconstruction, and
the non-radiated scenario. the procedures are time and resource inten-
E. Most patients undergoing mastectomy proce- sive. However, the patient has the benefit of
dures are able to undergo immediate recon- the natural feel of their own tissues and fre-
struction. In patients undergoing mastectomy quently a better match of the native contralat-
procedures, the two reconstructive options eral breast. Autologous reconstruction has the
are implant-based reconstruction and recon- added advantage that the reconstructed breast
struction using autologous tissue. Implant- will change with changes in the patient’s
based reconstruction is the most widely body habitus over time. The additional donor
performed procedure in the United States for site morbidity and significantly higher num-
post-mastectomy reconstruction. Most often, bers of overall postoperative complications
a tissue expander is placed at the time of the including DVT/PE, blood transfusion require-
mastectomy. The expander is filled over the ments and surgical site infections when com-
course of several months after the patient has pared to implant-based reconstruction are
healed from the initial operation. The drawbacks to this procedure [4]. Most studies
expander is exchanged for a permanent cite an overall complication rate for free tis-
implant at a follow-up procedure. Some sue transfer techniques in the range of
patients may be able to undergo one-step 23–30% [4]. In addition, a small percentage
implant-based reconstruction, with a perma- of patients, ranging from 1% to 5% will expe-
nent implant placed at the time of mastec- rience complete flap loss, depending on the
tomy. Typically, this approach requires them type of flap and whether it relies on a pedicle
to accept a smaller implant than the size of or a microvascular anastomosis [4]. However,
their breast before surgery. Currently, most patient satisfaction with autologous recon-
patients will opt to have silicone implants struction is quite high over time [1–4].
placed [2]. Implant-based reconstruction has F. All patients may find that their reconstruc-
the advantage of being relatively simple and tion requires revision after the initial
straightforward with fewer surgical morbidi- operation(s) are completed. Scar revision,
ties than autologous reconstruction. Implant- fat grafting, and, in the case of implant
based reconstruction does not involve the reconstruction, capsulotomy or capsulec-
added morbidity of the donor site required in tomy may all be required at some point.
autologous tissue reconstructions. However, Patients who have had total mastectomies
29 Breast Reconstruction 111
may also choose to undergo nipple recon- choose to have contralateral mastopexy or
struction with any one of a variety of local other surgery for symmetry [2].
flap techniques to recreate a nipple-areola G. Following the conclusion of reconstruction,
complex. The flap can subsequently be tat- patients should continue screening with their
tooed to simulate the natural pigmentation breast surgeon or oncologist. MRI may be
of the nipple and areola. Patients may also useful in evaluating implant integrity.
Initial Evaluation by Breast/General Surgeon

A and Referral to Plastic Surgeon
History and Physical Exam Considerations

B Focus:Cancer treatment plan, body habitus, smoking Significant comorbidities
history, medical comorbidities, patient desires/concerns -Body Habitus
-Active smoking
Contraindications
-Inflammatory Cancer
C Mastectomy -Patient does not desire
Breast Conserving
Therapy
Post-Mastectomy
D Radiation Therapy
planned? Oncoplastic
reconstruction if
appropriate with
Yes No consideration for
contralateral symmetry
procedures if necessary
E “Delayed” vs.
Immediate Immediate
Reconstruction Reconstruction
Tissue Expander
during Radiation Autologous Implant
Therapy Reconstruction Reconstruction
Expander exchange for

Implant after
Radiation Therapy
F
-Revision as needed
-Contralateral symmetry procedures
-Nipple Reconstruction
G Follow up
Algorithm 29.1
References 4. Gart MS, et al. Autologous options for postmastec-

tomy breast reconstruction: a comparison of outcomes
based on the American College of Surgeons National
1. Network NCC NCCN – Evidence blocks – Principles
Surgical Quality Improvement Program. J Am Coll
of breast reconstruction following surgery, in NCCN
Surg. 2013;216(2):229–38.
guidelines version 2.2016 – Invasive Breast Cancer.
5. Savalia NB, Silverstein MJ. Oncoplastic breast recon-
2016. p. 1–2.
struction: patient selection and surgical techniques. J
2. Djohan R, Gage E, Bernard S. Breast reconstruction
Surg Oncol. 2016;113(8):875–82.
options following mastectomy. Cleve Clin J Med.
6. Clemens MW, Kronowitz SJ. Current perspectives on
2008;75(Supplement 1):S17–23.
radiation therapy in autologous and prosthetic breast
3. Alderman AK, et al. Does patient satisfaction with
reconstruction. Gland Surg. 2015;4(3):222–31.
breast reconstruction change over time? Two-year
7. Reconstruction options: a comparison chart. 2013,
results of the Michigan Breast Reconstruction
BreastCancer.org: www.breastcancer.org.
Outcomes Study. J Am Coll Surg. 2007;204(1):7–12.
Management of Male Breast
Cancer 30
Algorithmic Approach breast and other related cancers. Male

BRCA2 mutation carriers are known to be at
A. Male breast cancer accounts for only 1% of increased risk to develop breast cancer [2].
all breast cancers and less than 1% of all All men affected with breast cancer should
malignancies in men [1, 2]. In 2017, it is undergo genetic counselling and be recom-
estimated that there will be 2470 new cases mended for genetic testing [6]. Past medical
of breast cancer in men compared to 252,710 history and medication history are important
new cases in women, with a projected 460 as increased exposure to estrogens is a risk
deaths [3]. The mean age at diagnosis is typ- factor for breast cancer development.
ically quoted as 60–65 years [4, 5]. As male Klinefelter syndrome, cryptorchidism, gyne-
breast cancer is a relatively rare clinical comastia, liver disease, and prostate cancer
entity, there are no established screening treated with hormonal therapy have been
protocols. As a result, male breast cancers associated with an increased risk for male
tend to present with painless palpable breast cancer, although some studies dispute
masses rather than radiographic findings. this fact [5, 7]. The patient should be ques-
Some studies suggest a delay between onset tioned as to the location of the mass and
of symptoms and a final diagnosis of breast length of time it has been present, as well as
cancer in males of over 10 months [5]. While any other associated symptoms. As in
stage-for-stage they have similar outcomes females, physical exam of the male patient
to female breast cancers, male patients tend should focus on identification of any palpable
to present at a more advanced stage at diag- mass and whether it is fixed or involving the
nosis [1, 4, 5]. skin. In males, due to the general paucity of
B. The workup of a male with a breast mass breast tissue, most breast cancers are located
begins with a full history and physical exam. in the subareolar aspect of the breast, where
In a male patient, breast cancer risk factors the majority of the ductal tissue is present [5].
are predominantly related to family history of The contralateral breast should also be exam-
ined. The regional lymph nodes (including
axillary, cervical, and supraclavicular nodes)
should be evaluated for the presence of any
J. C. Gooch · F. Schnabel (*) clinically positive nodes.

C. A male patient with a palpable breast mass E. Adjuvant therapy after surgery for male breast
should be referred for diagnostic breast cancer should be recommended based on clini-
imaging. Mammography is generally feasi- copathologic characteristics, following similar
ble, particularly in the presence of gyneco- protocol as for female breast cancer. Biomarker
mastia. Ultrasound may be useful for the analysis of the tumor (ER/PR, HER-2/neu sta-
evaluation of any clinically suspicious axil- tus, and Ki-67) is standard, and genomic
lary nodes and may also guide the perfor- assays may also be employed to determine the
mance of a biopsy. Biopsy samples should be patient’s risk for recurrence and shed light on
sent for histologic diagnosis and biomarker the potential benefit for cytotoxic chemother-
analysis. The majority of male breast cancers apy. As in females, anthracyclines and taxanes
are hormone receptor positive (over 90%) and form the basis for most standard chemothera-
mostly HER-2/neu negative, corresponding peutic regimens. Patients with hormone recep-
to a Luminal A or Luminal B phenotype [5]. tor (ER/PR)-positive tumors should receive
Preoperative assessment of the axilla should hormonal therapy with tamoxifen or aroma-
follow the protocols for female patients tase inhibitors. There is discussion in the litera-
addressed elsewhere in the text. ture about the combination of an aromatase
D. The general approach to a male breast cancer inhibitor and an androgen suppressor for hor-
patient is similar to that of an affected female. monal treatment of breast cancer, but this is not
Patients who present with locally advanced the standard of care [1, 2]. Post-lumpectomy
disease should be offered neoadjuvant che- radiation should be delivered to patients under-
motherapy prior to surgery. The most com- going breast conservation surgery. The guide-
mon surgical procedure for male breast lines for post-mastectomy radiation in males
cancer is a total mastectomy with evaluation follow those for females, with radiation rec-
of the axillary nodes. The axilla should be ommended after mastectomy in cases of pri-
evaluated with sentinel node biopsy and com- mary tumors greater than 5 cm in size and/or
pletion axillary dissection as appropriate [2, lymph node involvement.
5]. Male breast cancer patients are more F. After treatment is completed, patients should
likely to have axillary nodal involvement than continue close clinical follow-up with their
their female counterparts [5]. Breast recon- healthcare team. Annual imaging of the contra-
struction is not often performed after male lateral breast is appropriate as well. As noted
breast cancer treatment. There is little experi- above, male breast cancer is associated with
ence with breast-conserving surgery in males, BRCA2 mutations. If a man tests positive for a
and the typical subareolar primary location mutation, close relatives should consider genetic
decreases the cosmetic value of that approach. counselling and testing to clarify their status.
30 Management of Male Breast Cancer 115

A
Patient presents with palpable breast mass
B
History
Mass: onset, location, symptoms
Past medical history/medications
Family history: breast or ovarian cancer in female relatives? Known genetic mutations?
Risks: Klinefelter syndrome, gynecomastia, BRCA 2, liver disease, DM, prostate cancer,
exogenous estrogen, and androgens
Physical exam
Mass: mobile vs. fixed location?
Axilla: palpable nodes, fixed/matted nodes?
C Imaging: mammography and ultrasound

Core biopsy – assess receptor status
D Workup demonstrates malignancy
Clinical T1, N0 Clinical T2 or above

E Clinical N1 or above
-Breast surgery – mastectomy vs. lumpectomy

-Consider neoadjuvant chemotherapy
-Axillary surgery – sentinel node biopsy, possible
-Axillary US and FNA
axillary dissection
-Consider chemotherapy
-Consider tamoxifen for ER+ tumors
-Consider PMRT
F Follow up
Algorithm 30.1
References to race: a SEER population-based study. Oncotarget.

2017:1–11.
5. Javidiparsijani S, Rosen L, Gattuso P. Male breast car-
1. Kiluk JV, et al. Male breast cancer: manage-
cinoma: a clinical and pathological review. Int J Surg
ment and follow-up recommendations. Breast J.
Pathol. 2017;25(3):200–5.
2011;17(5):503–9.
6. Network NCC. Breast and/or ovarian cancer genetic
2. Losurdo A, et al. Controversies in clinicopathological
assessment, in NCCN guidelines Version 2. 2017.
characteristics and treatment strategies of male breast
7. Hagberg KW, et al. Impact of 5-alpha reductase inhib-
cancer: a review of the literature. Crit Rev Oncol
itors for treatment of benign prostatic hyperplasia on
Hematol. 2017;113:283–91.
erectile dysfunction, treated depression, gynecomas-
3. Society AC. Cancer facts and figures 2017. Cancer.
tia and breast cancer: a real world 20 year observa-
2017:1–74.
tional study. Clin Epidemiol. 2017;9:83–91.
4. Sun H, et al. Clinicopathological characteristics and
survival outcomes of male breast cancer according
Part V
Esophagus
Management of Esophageal
Motility Disorders 31
Algorithmic Approach the “major disorders of peristalsis” into three

categories: distal esophageal spasm; hyper-
A. The first step in the evaluation of a patient contractile, or jackhammer, esophagus; and
with a suspected esophageal motility disorder absent contractility. Distal esophageal spasm
is the history and physical examination. is defined by premature contractions in at
Patients classically present with dysphagia least 20% of swallows with normal lower
and chest pain. It is also important to inquire esophageal sphincter (LES) relaxation.
about reflux symptoms, as gastroesophageal Jackhammer esophagus is characterized by
reflux disease is the cause of most esophageal hypercontractile peristalsis in at least 20% of
motility disorders [1]. swallows. Absent contractility is character-
B. The next step in the workup of esophageal ized by aperistalsis in the setting of normal
motility disorders is to rule out mechanical LES relaxation [4].
obstruction and esophageal cancer with esoph- D. It is important to note that there is a paucity of
agogastroduodenoscopy (EGD) [2]. Barium data on treatment and outcomes for some of
esophagram is an additional imaging modality, the newly defined major disorders of peristal-
which can help to rule out mechanical obstruc- sis as described by the Chicago Classification
tion and give additional information about [4]. Treatment for each of the disorders begins
esophageal and gastric anatomy. pH monitor- with avoiding trigger factors that cause symp-
ing may also be done to rule out reflux in toms, eating soft foods and chewing thor-
patients without classic symptoms [3]. oughly, and reducing reflux with a proton
C. After mechanical obstruction has been ruled pump inhibitor (PPI) [2]. Distal esophageal
out, proceed with high-resolution manome- spasm is one of the new terms, and data is
try. The most recent version of the Chicago scarce on the best treatment options.
Classification, released in May 2014, divides Botulinum injections have shown promise in
reducing dysphagia symptoms in some small
A. R. Tascone studies [5]. Based on results from patients
Department of General Surgery, Saint Luke’s Health with diffuse esophageal spasm, surgical myot-
System, Kansas City, MO, USA
omy with an extended myotomy showed good
C. A. Halbert (*) results and can be used to treat patients who
Advanced GI and Bariatric Surgery, Department
fail botulinum injection therapy [6]. There is
of General Surgery, Christiana Care Health System,
Newark, DE, USA also minimal data supporting the best treat-
e-mail: [email protected] ment of jackhammer esophagus. A trial of cal-

120 A. R. Tascone and C. A. Halbert
cium channel blockers, nitrates, sildenafil, and as there is no specific treatment to restore or
pain modulators (trazodone) may help with improve peristalsis. Patients should be advised
pain [2]. Limited research suggests that botu- to favor liquid and semisolid nutrition over
linum injections and surgical myotomy may solids, consume meals in the upright position,
be of some benefit in patients with continued chew thoroughly, and drink during meals to
symptoms after pharmacologic treatment [2, facilitate esophageal clearance. Reduction of
3]. Treatment of absent esophageal contractil- reflux symptoms with a PPI is also a target of
ity entails dietary and lifestyle modifications, therapy [2].
A History and Physical:

Chest pain, dysphagia, reflux
B Esophagogastroduodenoscopy (EGD)
+/- Barium esophagram
+/- pH monitoring
No mechanical
obstruction?
Obtain high-resolution manometry

C
Findings consistent Absent Findings consistent

with distal esophageal with jackhammer
esophageal spasm contractility esophagus
Treatment: Treatment: Treatment:

1. Avoid trigger factors Dietary and lifestyle 1. Avoid trigger factors
and reduce reflux modifications, reflux and reduce reflux
2. Botulinum injections treatment 2. Pharmacologic
3. Extended myotomy smooth muscle
relaxants
3. Botulinum injections
or surgical myotomy
Algorithm 31.1
31 Management of Esophageal Motility Disorders 121
References 4. Kahrilas PJ, Bredenoord AJ, Fox M, Gyawali

CP, Roman S, Smout AJPM, et al. The Chicago
classification of esophageal motility disorders, v3.0.
1. Diener U, Patti MG, Molena D, Fisichella PM, Way
Neurogastroenterol Motil. 2015;27(2):160–74.
LW. Esophageal dysmotility and gastroesophageal
5. Bashashati M, Andrews C, Ghosh S, Storr
reflux disease. J Gastrointest Surg. 2001;5(3):260–5.
M. Botulinum toxin in the treatment of diffuse
2. Zerbib F, Roman S. Current therapeutic options for
esophageal spasm. Dis Esophagus [Internet].
esophageal motor disorders as defined by the Chicago
2010;23(7):554–60. Available from: http://www.ncbi.
classification. J Clin Gastroenterol. 2015;49(6):1–10.
nlm.nih.gov/pubmed/20459446.
3. Schlottmann F, Patti M. Primary esophageal motility
6. Leconte M, Douard R, Gaudric M, Dumontier I,
disorders: beyond achalasia. Int J Mol Sci [Internet].
Chaussade S, Dousset B. Functional results after
2017;18(7):1399. Available from: http://www.mdpi.
extended myotomy for diffuse oesophageal spasm. Br
com/1422-0067/18/7/1399.
J Surg. 2007;94(9):1113–8.
Management of Achalasia
32
Algorithmic Approach junction, and candidiasis. Barium esopha-

gram is an additional imaging modality,
A. The first step in the evaluation of a patient which can help to rule out mechanical
with achalasia is the history and physical obstruction and, additionally, aid in diagnosis
examination. Patients classically present with of achalasia. It classically shows a dilated
dysphagia to solids and liquids associated esophagus with a narrow gastroesophageal
with regurgitation of undigested food or junction, otherwise known as a “bird’s beak”
saliva. Other common symptoms include appearance. Other suggestive features include
heartburn, noncardiac chest pain, epigastric contrast filling the esophagus with poor emp-
pain, cough or asthma, chronic aspiration, tying, a “corkscrew appearance,” and aperi-
and hoarseness [1]. An important part of the stalsis [2].
examination of patients with potential achala- C. If there is no mechanical obstruction or other
sia is to rule out oropharyngeal dysphagia, cause for the patient’s symptoms found on
which can be done by watching the patient EGD or barium esophagram, proceed with
drink water. high-resolution manometry to secure the
B. The next step in the workup of achalasia is to diagnosis. Manometric findings of aperistal-
rule out mechanical obstruction and esopha- sis and incomplete lower esophageal sphinc-
geal cancer, or pseudoachalasia, with esopha- ter relaxation diagnose achalasia. The most
gogastroduodenoscopy (EGD). Findings recent version of the Chicago Classification
suggestive of achalasia on EGD include a of esophageal motility disorders divides
dilated or tortuous esophagus, food impac- achalasia into three distinct phenotypes,
tions, fluid pooling in the esophagus, resis- which have prognostic and potential thera-
tance to intubation of the gastroesophageal peutic implications [3, 4]. Type I achalasia
has the above findings with a normal esopha-
geal pressure. Type II achalasia is character-
A. R. Tascone ized by increased pan-esophageal pressure,
Department of General Surgery, Saint Luke’s Health and Type III achalasia shows distal esopha-
System, Kansas City, MO, USA
geal spastic contractions [3].
C. A. Halbert (*) D. The recommended first-line treatment of

Advanced GI and Bariatric Surgery, Department of
achalasia depends on the specific phenotype.
General Surgery, Christiana Care Health System,
Newark, DE, USA Type I and Type II achalasia are treated with
e-mail: [email protected] either graded pneumatic dilation or laparo-

124 A. R. Tascone and C. A. Halbert
scopic surgical myotomy with partial or unwilling to undergo definitive therapy as

fundoplication, ideally performed at high-
described above, botulinum toxin therapy is
volume centers of excellence. First-line treat- recommended. Pharmacologic therapy with
ment of Type III achalasia is laparoscopic calcium channel blockers, long-acting
myotomy with partial fundoplication [5]. nitrates, or the phosphodiesterase-5 inhibitor,
Peroral endoscopic myotomy (POEM) has sildenafil, is reserved for patients who have
also emerged as a safe and reliable treatment failed botulinum toxin therapy. Laparoscopic
option when performed in expert centers but surgical myotomy can be used for pneumatic
carries a high prevalence of GERD due to the dilation failures and vice versa. Patients with
fact that no antireflux procedure is performed refractory achalasia can be offered repeat
[6]. If the patient is a poor surgical candidate myotomy or in rare cases esophagectomy [7].
A History and Exam:

Common symptoms: Progressive dysphagia, heartburn, regurgitation or vomiting,
noncardiac chest pain, epigastric pain, cough or asthma, chronic aspiration,
hoarseness
Rule out oropharyngeal dysphagia
B
Obtain imaging studies to rule out mechanical obstruction:
Esophagogastroduodenoscopy (EGD)
Barium esophagram
Obtain high-resolution manometry

C
Findings Pursue other

consistent with diagnosis
Achalasia?
High surgical risk
D Low surgical risk
Botulinum toxin
therapy
Type I-II Type III
Nitrates
Pneumatic Calcium channel blockers
Myotomy Sildenafil
dilation
Repeat myotomy or
dilation Esophagectomy
Algorithm 32.1
32 Management of Achalasia 125
References evant classification by high-resolution manometry.

Gastroenterology. 2008;135(5):1526–33.
5. Zerbib F, Roman S. Current therapeutic options for
1. Tsuboi K, Hoshino M, Srinivasan A, Yano F,
esophageal motor disorders as defined by the Chicago
Hinder RA, Demeester TR, et al. Insights gained
classification. J Clin Gastroenterol. 2015;49(6):1–10.
from symptom evaluation of esophageal motil-
6. Talukdar R, Inoue H, Reddy DN. Efficacy of
ity disorders: a review of 4,215 patients. Digestion.
peroral endoscopic myotomy (POEM) in the
2012;85(3):236–42.
treatment of achalasia: a systematic review and meta-
2. Pandolfino JE, Gawron AJ. Achalasia. JAMA
analysis. Surg Endosc Other Interv Tech [Internet]
[Internet]. 2015;313(18):1841. Available from: http://
2015;29(11):3030–46. Available from: https://doi.
jama.jamanetwork.com/article.aspx?doi=10.1001/
org/10.1007/s00464-014-4040-6.
jama.2015.2996.
7. Vaezi MF, Pandolfino JE, Vela MF. ACG clinical
3. Kahrilas PJ, Bredenoord AJ, Fox M, Gyawali CP,
guideline: diagnosis and management of achalasia.
Roman S, Smout AJPM, et al. The Chicago clas-
Am J Gastroenterol [Internet] 2013;108(8):1238–
sification of esophageal motility disorders, v3.0.
49. Available from: http://www.nature.com/
Neurogastroenterol Motil. 2015;27(2):160–74.
doifinder/10.1038/ajg.2013.196.
4. Pandolfino JE, Kwiatek MA, Nealis T, Bulsiewicz W,
Post J, Kahrilas PJ. Achalasia: a new clinically rel-
Barrett’s Esophagitis
33
Algorithmic Approach therapy is not indicated for patients with

Barrett’s esophagitis. Endoscopic eradications
A. Patients with multiple risk factors for Barrett’s in the form of radiofrequency ablation (RFA),
esophagitis should undergo screening endos- photodynamic therapy (PDT), or endoscopic
copy. Risk factors include age greater than 50, mucosal resection (EMR) are recommended
presence of symptoms for greater than 5 years, for patients with high-grade dysplasia [1].
presence of a hiatal hernia, obesity, white race, Eradication therapy should also be considered
and male sex. Screening for low-risk patients in patients with low-grade dysplasia, especially
with GERD is not routinely recommended [1]. if persistent pathology is seen on repeat biopsy
B. Barrett’s esophagitis is a condition defined by [3]. EMR is preferred in patients with irregu-
metaplasia of the distal esophageal mucosa. larities of the mucosa on endoscopic visualiza-
On endoscopic biopsy, it consists of meta- tion. While endoscopic eradication therapy for
plastic columnar epithelium extending above high-grade dysplasia is successful for 70–80%
the gastroesophageal junction into the tubular of patients, esophagectomy is an alternative
esophagus. White light endoscopy is recom- form of therapy. Esophagectomy should be
mended with 4-quadrant mucosal biopsies of considered in patients with high-grade long-
the Barrett’s epithelium every 2 cm. The segment or multifocal disease [1].
interval is shortened to 1 cm for patients with D. There are several indications for surgical

a history of dysplasia [1]. consultation for patients with Barrett’s
C. Treatment of patients with Barrett’s esophagus, esophagus and GERD. Patients who are par-
with or without GERD symptoms, consists of tial responders to PPI, have severe regurgita-
long-term therapy with proton pump inhibitors tion, or cannot tolerate therapy with PPI. The
(PPI) [2]. Greater than standard dosing of PPI latter includes patients who elect surgical
intervention over lifelong need for medical
therapy [4]. While some experts recommend
C. A. Halbert (*) antireflux surgery in any patient with
Advanced GI and Bariatric Surgery, Department of Barrett’s esophagus, there is no evidence to
prove that it is superior to medical manage-
Newark, DE, USA
e-mail: [email protected] ment [1, 5].
E. Surveillance endoscopy timing is dependent
A. R. Tascone
Department of General Surgery, Saint Luke’s Health on pathologic findings if no eradication ther-
System, Kansas City, MO, USA apy has been performed [1].

128 C. A. Halbert and A. R. Tascone
Risk factors for Barrett’s esophagitis:

A Age greater than 50, presence of symptoms for greater than 5 years,
presence of a hiatal hernia, obesity, white race and male sex
Upper endoscopy
B Non-dysplastic Low-grade dysplasia High-grade dysplasia
Medical therapy for Medical therapy

Endoscopic eradication
C treatment of GERD Versus
therapy
symptoms and reflux Endoscopic eradication
esophagitis therapy
Consideration for Consideration for

D antireflux surgery antireflux surgery
Consideration for
antireflux surgery
Surveillance endoscopy: Surveillance endoscopy: Surveillance endoscopy:

E every 3-5 years every 6-12 months every 3 months
Algorithm 33.1
3. Kestens C, Offerhaus GJA, van Baal JWPM, Siersema

References PD. Patients with Barrett’s esophagus and persis-
tent low-grade dysplasia have an increased risk for
1. Spechler SJ, Sharma P, Souza RF, Inadomi JM, high-grade dysplasia and cancer. Clin Gastroenterol
Shaheen NJ. American Gastroenterological Hepatol. 2016;14(7):956–62.
Association medical position statement on the man- 4. Stefanidis D, Hope WW, Kohn GP, Reardon PR,
agement of Barrett’s esophagus. Gastroenterology. Richardson WS, Fanelli RD. Guidelines for surgi-
2011;140(3):1084–91. cal treatment of gastroesophageal reflux disease.
2. Freedberg DE, Kim LS, Yang Y-X. The risks and Surg Endosc [Internet]. 2010;24(11):2647–69.
benefits of long-term use of proton pump inhibi- Available from: http://www.ncbi.nlm.nih.gov/
tors: expert review and best practice advice from pubmed/20725747.
the American Gastroenterological Association. 5. Wassenaar E, Oelschlager B. Effect of medical and
Gastroenterology [Internet]. 2017;152(4):706–15. surgical treatment of Barrett’s metaplasia. World J
Available from: http://linkinghub.elsevier.com/ Gastroenterol. 2010;16(30):3773–9.
retrieve/pii/S0016508517300914.
Gastroesophageal Reflux Disease
34
Algorithmic Approach nal GERD. Avoidance of tobacco, alcohol,

chocolate, caffeine, spicy foods, citrus, and
A. Gastroesophageal reflux disease can present carbonated beverages have limited effects in
with a varying display of symptoms. They are GERD symptoms except in the case where
categorized into esophageal and extra- patient intake correlates with exacerbation
esophageal symptoms, according to the of clinical symptoms [2].
Montreal Classification [1]. Important ques- C. Patients presenting with chest pain must have
tions for patients include but are not limited a cardiac etiology ruled out before consider-
to timing, duration, and association of reflux ing GERD. Dysphagia is not classically asso-
symptoms, sleeping habits, frequency of ciated with GERD and can be an alarming
upper respiratory infections, and over-the- symptom representing something more
counter medication use. severe. Early workup is imperative and should
B. Patients presenting with classic symptoms include upper endoscopy and barium radio-
of heartburn and/or regurgitation may graphs to rule out intraluminal pathology and
achieve relief with lifestyle modifications. mechanical obstruction. If the diagnosis is
Weight loss is strongly recommended for unclear, pH manometry may be necessary.
patients with a BMI greater than 25. Patients with refractory GERD also require
Elevating the head of the bed can improve further workup as described above [3].
symptoms, particularly for patients with D. Medical management includes proton pump
nocturnal GERD. Avoidance of meals, espe- inhibitors (PPIs), histamine-receptor (H2)
cially those with high-fat content, within antagonists, and antacids. PPIs are the most
2–3 h of reclining can also improve noctur- powerful and commonly prescribed acid sup-
pression medication. Patients should be
started on once-daily PPI therapy for an ini-
tial course of 8 weeks. If patients are only
C. A. Halbert (*) partially responsive at this dosing, the PPI
Advanced GI and Bariatric Surgery, Department of therapy can be titrated to twice daily. H2
antagonists can be prescribed for symptom
Newark, DE, USA
e-mail: [email protected] relief in patients without erosive esophagitis,
especially for use with nocturnal GERD [3].
A. R. Tascone
Department of General Surgery, Saint Luke’s Health Patients in whom PPI therapy has success-
System, Kansas City, MO, USA fully treated their symptoms should be trialed

off of PPIs. The exception would be patients ence of abnormal esophageal acid exposure
with known Barrett’s esophagus and severe and symptom correlation, if not diagnosed
esophagitis. If patients are unable to tolerate a on previous testing. Telemetry capsule pH
trial off PPI therapy, further workup is indi- and catheter-based pH monitoring are avail-
cated [3]. able, with the latter providing additional
E. There are several indications for surgical con- information on weakly acidic or nonacid
sultation for patients suffering from GERD: reflux [3].
1. Failure of medical management: Patients G. Traditionally, the Nissen fundoplication has
who are partial responders to PPI, have been the gold standard for the surgical treat-
severe regurgitation, or cannot tolerate ment of GERD. There is evidence demon-
therapy with PPI. strating that the Toupet fundoplication has
2. Patients who elect surgical intervention
equivalent heartburn control with a lower
over lifelong need for medical therapy. dysphagia rate. Some experts advocate for
3. Severe, complicated GERD: Patients with Toupet fundoplication over Nissen fundopli-
Barrett’s esophagus or peptic strictures. cation, thereby also rendering the manome-
4. Patients with extra-esophageal symp-
try unnecessary in the preoperative workup
toms [4]. [4]. This remains a debated topic. The mag-
F. Many patients will already have some testing netic sphincter augmentation device was
completed by the time they are referred for approved by the US Food and Drug
surgical consideration. If not already com- Administration in 2012. Clinical trials in the
pleted, they will need upper endoscopy. USA have shown significant reflux control
Barium swallow is a consideration preoper- and minimal side effects at 5 years from
atively to further delineate anatomy. implantation [5]. In patients meeting indica-
Esophageal manometry is important to rule tions for bariatric surgery, the roux-en-Y
out achalasia or esophageal dysmotility. gastric bypass should be considered. It has
This study can also help to tailor surgery been shown to have significant improvement
according to the functionality of the esopha- in typical and atypical GERD symptoms, as
gus [4]. Impedance-pH ambulatory reflux well as additional obesity-related comorbid-
monitoring provides evidence of the pres- ities [6].
34 Gastroesophageal Reflux Disease 131

Esophageal and Extraesophageal Symptoms
Classic symptoms: Atypical symptoms:

Regurgitation, Chest pain, dysphagia,
heartburn weight loss
B Lifestyle Modification Upper endoscopy,

C Barium esophagram
Findings
Symptom
c/w GERD
relief?
D No
Medical
Management
Meets
indications
for surgical
referral?
E
Upper endoscopy,
Barium esophagram,
Esophageal manometry,
F Impedance pH
Normal Esophageal motility Impaired Esophageal motility
G BMI >35
Magnetic
Nissen/Toupet Roux-en-Y Toupet
Sphincter
Fundoplication Gastric Bypass Fundoplication
Augmentation
Algorithm 34.1
References 4. Stefanidis D, Hope WW, Kohn GP, Reardon PR,

Richardson WS, Fanelli RD. Guidelines for surgi-
cal treatment of gastroesophageal reflux disease.
1. Vakil N, Van Zanten SV, Kahrilas P, Dent J, Jones R,
Surg Endosc [Internet]. 2010;24(11):2647–69.
Bianchi LK, et al. The Montreal definition and clas-
Available from: http://www.ncbi.nlm.nih.gov/
sification of gastroesophageal reflux disease: a global
pubmed/20725747.
evidence-based consensus. Am J Gastroenterol.
5. Ganz RA, Edmundowicz SA, Taiganides PA, Lipham
2006;101:1900–20.
JC, Smith CD, DeVault KR, et al. Long-term out-
2. Kahrilas PJ, Shaheen NJ, Vaezi MF, Hiltz SW, Black
comes of patients receiving a magnetic sphincter aug-
E, Modlin IM, et al. American Gastroenterological
mentation device for gastroesophageal reflux. Clin
Association medical position statement on the
Gastroenterol Hepatol. 2016;14(5):671–7.
management of gastroesophageal reflux disease.
6. Frezza EE, Ikramuddin S, Gourash W, Rakitt T,
Gastroenterology [Internet]. 2008;135(4):1383–91,
Kingston A, Luketich J, et al. Symptomatic improve-
1391-e5. Available from: http://www.ncbi.nlm.nih.
ment in gastroesophageal reflux disease (GERD) fol-
gov/pubmed/18789939.
lowing laparoscopic Roux-en-Y gastric bypass. Surg
3. Katz PO, Gerson LB, Vela MF. Guidelines for the
Endosc. 2002;16(7):1027–31.
diagnosis and management of gastroesophageal reflux
disease. Am J Gastroenterol. 2013;108(10):1672.
Hiatal Hernia
35
Algorithmic Approach most of these cases, reflux of gastric con-

tents is uncommon.
A. Patients with hiatal hernia can be asymptom- (c) Type 3: mixed hernias involve herniation
atic or present to outpatient clinic with reflux of the stomach with the gastroesophageal
symptomatic, such as heartburn, abdominal junction into the mediastinum.
pain, anemia, and regurgitation, as well as (d) Type 4: giant paraesophageal hernia

respiratory symptoms, such as dyspnea, chest characterized by intrathoracic stomach
pain, or cough. There is a 1% risk per year for along with associated viscera such as the
these patients to develop acute complications spleen, colon, small bowel, or pancreas.
(hemorrhage, incarceration, gastric volvulus, C. Barium swallow and upper endoscopy are
obstruction, and strangulation) that require useful in the diagnosis of hiatal hernia. In the
urgent surgical intervention [1]. Physical setting of acute symptoms, CT might be con-
exam findings in hiatal hernia lack specific- sidered, while EGD provides a therapeutic
ity; further imaging is warranted in the diag- means to decompress and reduce the volvu-
nosis of hiatal hernia. lized stomach and to assess the viability of
B. There are four types of hiatal hernias [2]: the stomach [3]. In planning for elective
(a) Type 1: sliding hernia, where the GE repair of hiatal hernia, additional pH, manom-
junction moves above the diaphragm etry study, and gastric emptying studies can
together with some or the entire stomach, be helpful in determining the surgical repair
associated with esophageal shortening. of choice and if there is concomitant
(b) Type 2: rolling or paraesophageal hernia, achalasia.
where the gastric fundus herniates into D. Urgent intervention is indicated in the setting
the mediastinum alongside the esopha- of acute hemorrhage, incarceration, gastric
gus, with the gastroesophageal junction volvulus, obstruction, and strangulation.
remaining in an intra-abdominal posi- Hiatal hernia reduction and gastropexy may
tion. Since the gastroesophageal sphinc- be warranted in certain emergent situations.
teric mechanism functions normally in Elective repair is offered to patients with
symptomatic hiatal hernias refractory on
medical therapy (PPI or H2 blocker) who are
W. Lam · R. Neupane · J. M. Marks (*)
suitable surgical candidates [3].
Department of Surgery, University Hospitals
Cleveland Medical Center, Cleveland, OH, USA E. In an elective repair of hiatal hernia, laparo-
e-mail: [email protected] scopic approach has become the standard of

134 W. Lam et al.
care. Transthoracic approach can be consid- repair, there is no existing guideline on tailor-
ered if esophageal lengthening is required. ing the degree of fundoplication, which
The use of mesh has not been shown to include Nissen, a 360 degree wrapping of the
improve or worsen long-term outcome. While anterior and posterior walls of the stomach
a fundoplication is recommended as a step in around the esophagus, or partial anterior or
repairing a sliding or paraesophageal hernia posterior wrapping [4, 5].
Presentation with dysphagia, heart burn, regurgitation.

A B Atypical symptoms can include dyspnea, chest or abdominal pain.
Barium swallow
C Endoscopy can be diagnostic and therapeutic
Consider pH and manometry studies if differential
diagnosis remains broad
Elective surgical intervention Unstable, requiring urgent

• Medical treatment failure,intolerance or non- intervention
compliance • Hemorrhage
D E • Prospect of lifelong medical therapy • Incarceration
• Severe esophagitis, Barrett’s • Volvulus
• Benign stricture • Obstruction
• Quality of life improvement • Strangulation
Surgical repair
Transabdominal
• Laparoscopic –standard of care, vs open
Transthoracic-failed/not candidate for transabdominal
approach
Fundoplication
• Nissen–complete
• Partial –anterior or
posterior
Algorithm 35.1
management of hiatal hernia. Surg Endosc [Internet].

References 2013;27(12):4409–28. Available from: http://www.
ncbi.nlm.nih.gov/pubmed/24018762.
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hernias: operation or observation? Ann Surg [Internet]. ment of hiatal hernia. Surg Endosc [Internet].
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of modern surgical practice. 20th ed. Philadelphia: Surg [Internet]. 2005;241(1):185–93. Available from:
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3. Kohn GP, Price RR, DeMeester SR, Zehetner J,
Muensterer OJ, Awad Z, et al. Guidelines for the
Esophageal Carcinoma
36
Algorithmic Approach the lesion. A barium swallow is a functional

study that provides the characteristic finding
A. Esophageal cancer is the sixth leading cause of of apple core lesion that is seen in esophageal
cancer death in the world and carries a 5-year cancer. A chest and abdomen CT is recom-
survival rate of only 15–25%. In the USA and mended in detecting metastatic lesions to the
other developed countries, such as Western liver and lungs for locally advanced disease.
Europe and Australia, esophageal adenocarci- PET/CT/MRI is also employed to improve
noma is the most common histological type, the accuracy in staging. Endoscopic ultra-
whereas in the developing world, squamous cell sound is essential in the staging of resectable
carcinoma is the predominate type. Esophageal esophageal cancer by providing information
adenocarcinoma is associated with long-stand- on depth of invasion, as well as lymph node
ing reflux disease, obesity, and Barrett’s esopha- involvement. Data on the use of staging tho-
gus, whereas squamous cell carcinoma is racoscopy or laparoscopy is lacking [3, 4].
associated with smoking and alcohol use [1, 2]. D. In the TNM staging for esophageal cancer
B. Unintentional weight loss and dysphagia (Table 36.1), T0 represents no evidence of dis-
likely represent late symptoms of esophageal ease; Tis high-grade dysplasia, defined as
cancer since the esophagus can accommodate malignant cells confined by the basement
distention that slowly progresses over a membrane; T1 submucosa; T2 muscularis pro-
period of time, evident by the fact that 50% of pria; T3 adventitia; and T4 adjacent structures.
patients have unresectable or metastatic dis- N0 represents no regional lymph node involve-
ease at the time of diagnosis. Nodal spread is ment; N1 up to 3 regional nodes; N2 up to 6
also rapid, with 14–21% present in T1 lesions nodes, and N3 7 or more nodes [5]. For patients
(submucosa) and 38–60% in T2 lesions (mus- with Barrett’s esophagus with high-grade dys-
cularis propria) [2]. plasia, endoscopic intervention is recom-
C. Endoscopy is the gold standard for diagnos- mended [6]. In patients with T1–T3 lesions,
ing esophageal cancer with tissue biopsy, as esophagectomy is offered to patients who are
well as characterizing the size and location of good surgical candidates. This is followed by
adjuvant therapy for advanced disease to
improve survival [2]. A Cochrane review of 13
R. Neupane · W. Lam · J. M. Marks (*)
studies has suggested survival benefits of neo-
Cleveland Medical Center, Cleveland, OH, USA adjuvant chemotherapy for resectable thoracic
e-mail: [email protected] esophageal cancer [7]. The type of surgical

136 R. Neupane et al.
Table 36.1 American Joint Committee on Cancer approach, namely the transhiatal, Ivor-Lewis,
(AJCC) tumor/node/metastasis (TNM) classification
or three-field, has not been shown to differ in
T – Primary tumor survival benefits or operative mortality, though
T0 No evidence of primary tumor the transhiatal approach is associated with a
Tis High grade dysplasia
lower postoperative morbidity [2]. Preoperative
T1 Lamina propria, muscularis mucosa, or
submucosa esophageal stenting is sometimes used for
T2 Muscularis propria patients with dysphagia as a bridge to surgery
T3 Adventitia while undergoing neoadjuvant therapy [8].
T4 Adjacent structures Some surgeons opt to establish enteral access
N – Regional lymph node for patients with significant weight loss by
N0 No lymph node involvement either gastrostomy or jejunostomy prior to sur-
N1 Metastasis in 1–2 regional lymph nodes gical resection for nutrition optimization.
N2 Metastasis in 3–6 regional lymph nodes
E. For unresectable lesions, palliative options
N3 Metastasis in 7+ regional lymph nodes
are offered, including chemoradiation, esoph-
M – Distant metastasis
M0 No distant metastasis
ageal stenting, feeding gastrostomy or jeju-
M1 Distant metastasis nostomy, and esophagectomy [3].
Presentation of GERD, dysphagia or asymptomatic/constitutional symptoms

Anatomic symptoms
A B • Fistula to airway,
• recurrent laryngeal nerve involvement - hoarseness
• metastases to liver bone and lung
Work up with barium swallow and endoscopy

C D Staging with CT, PET, MRI, EUS
Additional staging with bronchoscopy, mediastinocopy, thoracoscopy, laparoscopy
Chemoradiation Surgery
• Lymph node involvement • En bloc resection with T1-T2 disease
E • T3 and greater disease • After neoadjuvant chemoradiation for
• Significant co-morbidities or distant organ metastases lymph node involvement or T3 disease
• Squamous cell carcinoma may respond completely • Palliation
Palliation Options
• Surgery
• Chemoradiation
• Stenting
• Lasar ablation
• Feeding gastrostomy, jejunostomy
Algorithm 36.1
36 Esophageal Carcinoma 137
References 5. Rice TW, Patil DT, Blackstone EH. 8th edition AJCC/
UICC staging of cancers of the esophagus and esoph-
agogastric junction: application to clinical practice.
1. José M, Arnal D, Arenas ÁF, Arbeloa ÁL. Esophageal
Ann Cardiothorac Surg. 2017;6(2):119–30.
cancer: risk factors, screening and endoscopic treat-
6. Spechler SJ. Barrett esophagus and risk of esopha-
ment in Western and Eastern countries. World J
geal cancer: a clinical review. JAMA [Internet].
Gastroenterol. 2015;21(26):7933–43.
2013;310(6):627–36. Available from: http://www.
2. Enzinger PC, Mayer RJ. Esophageal cancer. N Engl
ncbi.nlm.nih.gov/pubmed/23942681.
J Med [Internet]. 2003;349(23):2241–52. Available
7. Kidane B, Coughlin S, Vogt K, Malthaner
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R. Preoperative chemotherapy for resectable tho-
3. Townsend C, Bouchamp DB, Evers MB, Mattox
racic esophageal cancer. Cochrane Database Syst
K. Sabiston textbook of surgery: the biological basis
Rev [Internet]. 2015;(5):CD001556. Available from:
of modern surgical practice. 20th ed. Philadelphia:
http://www.ncbi.nlm.nih.gov/pubmed/25988291.
Elsevier; 2016.
8. Martin RCG, Cannon RM, Brown RE, Ellis SF,
4. Varghese TK, Hofstetter WL, Rizk NP, Low DE,
Williams S, Scoggins CR, et al. Evaluation of quality
Darling GE, Watson TJ, et al. The society of thoracic
of life following placement of self-expanding plastic
surgeons guidelines on the diagnosis and staging of
stents as a bridge to surgery in patients receiving neo-
patients with esophageal cancer. Ann Thorac Surg
adjuvant therapy for esophageal cancer. Oncologist
[Internet]. 2013;96(1):346–56. Available from: http://
[Internet]. 2014;19(3):259–65. Available from: http://
www.ncbi.nlm.nih.gov/pubmed/23752201.
www.ncbi.nlm.nih.gov/pmc/articles/PMC3958458/.
Esophageal Perforation
37
Algorithmic Approach tenderness might be present on physical exam.

Hemodynamic instability occurs as a result of
A. Esophageal perforation is a medical and surgi- mediastinitis or pleuritis [4].
cal emergency. One meta-analysis of 75 stud- C. Once suspected, workup for esophageal per-
ies reported a pooled mortality of 11.9% [1]; a foration should be promptly undertaken.
12-year national study from England reported Leukocytosis and elevated amylase level is
a 30-day mortality of 30% among 2564 consistent with esophageal perforation.
patients [2]. Mortality rate is doubled if the Electrolytes, coagulation panel, and type and
diagnosis is delayed for more than 24 h [3]. screen should be obtained in anticipation of
The etiologies of esophageal perforation need for surgical or endoscopic intervention.
include iatrogenic instrumentation, foreign Extravasation of contrast in an esophagram is
body ingestion, trauma, malignancy, or force- diagnostic; the contrast of choice depends on
ful emesis, also known as Boerhaave’s location of suspected perforation to minimize
Syndrome, when a transmural esophageal tear inflammatory response from contrast extrava-
occurs after retching. This is different from sation. Gastrografin swallow evaluation
Mallory-Weiss tear, which is a mucosal tear should be utilized first as the most useful ini-
that does not result in esophageal perforation. tial test, as it will not cause mediastinitis,
B. Due to its high mortality, esophageal perfora- unlike barium [5]. Mediastinal air and/or
tion must be considered in patients presenting fluid on CT scan helps localize the site of per-
with neck, substernal, or epigastric pain, with a foration [4].
history of instrumentation, trauma, foreign D. Supportive measures, namely, fluid resuscita-
body ingestion, malignancy, or vomiting. Fever, tion, broad-spectrum antimicrobials, NPO
tachycardia, tachypnea, subcutaneous emphy- status, close hemodynamic monitoring, and
sema, diminished breath sounds, and abdominal support in an ICU setting, are critical in the
initial management of esophageal perfora-
tion. A 12-year retrospective study in England
advocated for centralization of care at high-
volume centers where multidisciplinary
approach led to improved outcomes [2]. In a
R. Neupane · W. Lam · J. M. Marks (*)
hemodynamically stable patient with
Cleveland Medical Center, Cleveland, OH, USA contained perforation, conservative manage-
e-mail: [email protected] ment is acceptable. Esophageal stenting with

140 R. Neupane et al.
mediastinal and pleural drainage is a treat- 2/3 perforation. The possibility of primary
ment option for stable patients with limited repair is dictated by the degree of tissue
mediastinal or pleural contamination, though inflammatory which often depends on timing
its efficacy has not been compared with surgi- from onset of symptoms. Muscle flap has
cal intervention [6]. been established as a safe approach for intra-
E. Surgical intervention is indicated for patients thoracic or cervical esophageal perforation
who are hemodynamically unstable or show when primary repair is impossible or risky
no improvement on nonoperative manage- [7]. In the face of severe inflammation, devi-
ment. The operative approach is guided by talized tissue should be debrided and esopha-
the location of perforation, whether it is con- geal diversion should be considered if primary
tained or not, and underlying pathology. A repair is deemed high risk for anastomotic
cervical incision is made for a high esopha- leak. Temporary feeding access, such as gas-
geal perforation: a right thoracotomy for the trostomy or jejunostomy, and wide drainage
upper 2/3 and a left thoracotomy for the lower should be established.
Presentation with forceful emesis, foreign body ingestion, or trauma

A B Vitals, blood work, and physical exam
C D Resuscitation, foley, NG, NPO, antibiotics, +/– ICU
Discern cervical vs thoracic versus

abdominal symptoms? Confirm
with imaging or endoscopy
E Hemodynamically stable Hemodynamically unstable
Contained perforation–continue Uncontained Non/pre-perforation –

Uncontained perforation
conservative therapy. Consider perforation – debridement, diversion,
– emergent surgical
stenting if limited mediastinal, operating room for drainage, gastrostomy/
exploration
pleural contamination exploration jejunostomy
Operative intervention
• Cervical approach – amenable to drainage and primary repair =/– muscle flap
• Thoracic and abdominal perforation – possible primary repair, muscle flap, and drainage. Jejunostomy
• Distal obstruction – resection, reconstruction, drainage, jejunostomy
• Possible esophagectomy and exclusion
Algorithm 37.1
37 Esophageal Perforation 141
References center 10-year experience. World J Gastroenterol.

2014;20(30):10613–9.
4. Townsend C, Bouchamp DB, Evers MB, Mattox
1. Biancari F, D’Andrea V, Paone R, Di Marco C, Savino
K. Sabiston textbook of surgery: the biological basis
G, Koivukangas V, et al. Current treatment and out-
of modern surgical practice. 20th ed. Philadelphia:
come of esophageal perforations in adults: systematic
Elsevier; 2016.
review and meta-analysis of 75 studies. World J Surg.
5. Michel L, Grillo HC, Malt RA. Esophageal perfora-
2013;37(5):1051–9.
tion. Ann Thorac Surg. 1982;33(2):203–10.
2. Markar SR, Mackenzie H, Wiggins T, Askari A,
6. Dasari BVM, Neely D, Kennedy A, Spence G, Rice
Faiz O, Zaninotto G, et al. Management and out-
P, Mackle E, et al. The role of esophageal stents in
comes of esophageal perforation: a national study
the management of esophageal anastomotic leaks
of 2,564 patients in England. Am J Gastroenterol.
and benign esophageal perforations. Ann Surg.
2015;110(11):1559–66.
2014;259(5):852–60.
3. Persson S, Elbe P, Rouvelas I, Lindblad M, Kumagai
7. Richardson JD, Tobin GR. Closure of esopha-
K, Lundell L, et al. Predictors for failure of stent treat-
geal defects with muscle flaps. Arch Surg.
ment for benign esophageal perforations – a single
1994;129(5):541–7. discussion 547–8.
Acidic and Basic Injuries
38
Algorithmic Approach of ingested substance, timing of ingestion,

and duration of tissue contact. History taking
A. Caustic injuries are uncommon in the United can be challenging in this patient population,
States with 5000 cases reported every year, commonly of pediatrics and mental illnesses.
most of which occur in the pediatric popula-
C. Clinical and radiographic examination of
tion. Household disinfectants such as bleach caustic injury alone is inadequate. Early inter-
are common offending agents. Whereas acids vention with flexible fiber-optic endoscopy up
results in coagulation necrosis and eschar for- to 36 h was shown to be safe and accurate in
mation that limits depth of penetration and characterizing the location, extent, and sever-
injury, alkalis leads to liquefactive necrosis ity of injury [4]. Zargar et al. developed a
and saponification that promotes deeper tis- grading system to classify the caustic injuries
sue penetration [1, 2]. and predict prognosis: grade 0, normal exam;
B. In the initial evaluation of a patient with caus- grade 1, edema and mucosal hyperemia; grade
tic injuries, the management of airway, 2a, superficial and noncircumferential ulcer-
breathing, and circulation is crucial. For ation; grade 2b, deep and circumferential
patients with rapidly progressing upper air- ulceration; grade 3a scatter necrosis; and
way edema, stridors, emesis, respiratory dis- grade 3b, extensive necrosis with high risk of
tress, endotracheal intubation should be stricture formation [4]. There is evidence to
considered; anesthesia and otolaryngology support the safety in completely evaluating
teams should be involved early if there is con- the esophagus and stomach for additional
cern for difficult airway [3]. Intravenous lesions beyond circumferential burns [5]. A
access should be established and hemody- nasogastric tube should be placed under direct
namic stability monitored closely. A thorough visualization at this time for grade 2b and 3
history should be taken to determine the form injuries without significant gastric involve-
ment [6]. Emesis should be minimized and
neutralization is contraindicated due to the
risk of thermal injury.
D. Patients with grades 1–2a injuries can be

safely managed on the regular nursing floor
W. Lam · R. Neupane · J. M. Marks (*)
and discharged home within 1–2 days with
Cleveland Medical Center, Cleveland, OH, USA good oral intake and antacids therapy. Patients
e-mail: [email protected] with grades 2b–3 injuries should be admitted

144 W. Lam et al.
to an intensive care unit with nutritional sup- F. Serial endoscopy is performed to monitor
port and monitored for perforation for up to progression of injuries, iatrogenic perfora-
1–2 weeks. The use of corticosteroids is con- tion, as well as long-term stricture develop-
troversial and has not been shown to reduce ment, which can take place 3 weeks to 1 year
the formation of strictures in grade 3 patients. after the initial exposure [2]. Barium esopha-
Antibiotics should be used if there is evidence gram is an alternative diagnostic modality to
of perforation with peritonitis or mediastini- evaluate for strictures [5]. Local injection and
tis, but has not been shown to reduce the for- topical application of mitomycin C to esoph-
mation of strictures [7]. ageal mucosa has been described with good
E. Surgical intervention is indicated in the acute outcome in preventing structure formation in
setting of peritonitis and perforation, as well high-grade injuries, though further prospec-
as long-term stricture formation that is not tive studies are required to validate its effi-
amenable to endoscopic dilation or stenting. cacy [8]. Serial dilation is commonly
In a hemodynamically stable patient, laparo- performed every 1–3 weeks for strictures [2].
scopic exploration can be considered as ini- Nutrition should be optimized to support
tial operative approach [1]. Refer to healing, which might require establishing
“Esophageal Perforation” chapter for details enteral access with either gastrostomy or jeju-
of surgical management. nostomy early in the management plan.
History and physical: vitals, substance, duration

A B C Labs, CXR, AXR, or CT
Endoscopy
NPO, IVF, +/– ICU monitoring

D Antibiotics E
1st-degree burn 2nd-degree burn 3rd-degree burn
48 h ICU, monitor for sepsis

observation. PO ICU, IV antibiotics Airway involvement –
challenge. Repeat Swallow evaluation at 24 h Consider fiber-optic
endoscopy and Possible second look if intubation
esophagram with concernfor perforation/ Diagnostic laparoscopy if
follow-up necrosis question of perforation
Exploratory laparotomy in
unstable patient, full
thickness involvement
Surgical options include

debridement of devitalized
Stricture formation
tissue
and treatment • +/– esophagectomy
F options include • +/– gastric resection
serial dilation, stent, • cervical
and jejunostomy esophagostomy
feeding gastrostomy
or jejunostomy
Algorithm 38.1
38 Acidic and Basic Injuries 145
References of burns. Gastrointest Endosc [Internet]. 37(2):165–

9. Available from: http://www.ncbi.nlm.nih.gov/
pubmed/2032601.
1. Kluger Y, Ishay O Ben, Sartelli M, Katz A, Ansaloni
5. Temiz A, Oguzkurt P, Ezer SS, Ince E, Hicsonmez
L, Gomez CA, et al. Caustic ingestion management:
A. Predictability of outcome of caustic ingestion by
world society of emergency surgery preliminary sur-
esophagogastroduodenoscopy in children. World
vey of expert opinion. World J Emerg Surg [Internet].
J Gastroenterol [Internet]. 2012;18(10):1098–103.
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Available from: http://www.ncbi.nlm.nih.gov/pmc/
nih.gov/pmc/articles/PMC4609064/.
articles/PMC3296984/.
2. Contini S, Scarpignato C. Caustic injury of the upper
6. Shub MD. Therapy of caustic ingestion: new treat-
gastrointestinal tract: a comprehensive review. World J
ment considerations. Curr Opin Pediatr [Internet].
Gastroenterol [Internet]. 2013;19(25):3918–30. Available
2015;27(5):609–13. Available from: http://www.ncbi.
from: http://www.ncbi.nlm.nih.gov/pubmed/23840136.
nlm.nih.gov/pubmed/26196260.
3. Struck MF, Beilicke A, Hoffmeister A, Gockel I,
7. Park KS. Evaluation and management of caustic inju-
Gries A, Wrigge H, et al. Acute emergency care and
ries from ingestion of Acid or alkaline substances. Clin
airway management of caustic ingestion in adults:
Endosc [Internet]. 2014;47(4):301–7. Available from:
single center observational study. Scand J Trauma
http://www.ncbi.nlm.nih.gov/pubmed/25133115.
Resusc Emerg Med [Internet]. 2016;24:45. Available
8. Berger M, Ure B, Lacher M. Mitomycin C in the
from: http://www.ncbi.nlm.nih.gov/pmc/articles/
therapy of recurrent esophageal strictures: hype or
PMC4827211/.
hope? Eur J Pediatr Surg [Internet]. 2012;22(2):109–
4. Zargar SA, Kochhar R, Mehta S, Mehta SK. The role
16. Available from: http://www.ncbi.nlm.nih.gov/
of fiberoptic endoscopy in the management of corro-
pubmed/22517516.
sive ingestion and modified endoscopic classification
Part VI
Stomach and Duodenum
Gastric Ulcer Management
39
Algorithmic Approach: Introduction lesser curvature of the stomach high along

cardia; and type V, an ulcer due to NSAID use.
Gastric ulcer is one of the most common gastro- Types II and III are due to high acid secretion,
intestinal diagnoses in the USA with a preva- which is an important consideration in terms of
lence of 2%. Gastric ulcers are divided into five surgical management. Gastric ulcer manage-
types depending on the location and based on ment primarily depends on the acute or chronic
the Modified Johnson classification: type I, presentation.
located on the lower lesser curvature of the Common acute presentations of gastric ulcer
stomach; type II, two ulcers – one on the lower complications that may require surgical inter-
curvature and the other a duodenal ulcer; type vention include bleeding, perforation, and
III, a prepyloric ulcer; type IV, located on the obstruction.
M. S. Altieri
Department of General Surgery, Stony Brook
K. Spaniolas (*)
e-mail: Konstantinos.Spaniolas@
stonybrookmedicine.edu

150 M. S. Altieri and K. Spaniolas
Bleeding
Bleeding
Resuscitate
A Stable?
No IV fluids
PPI drip
Transfuse
Yes
Endoscopic
B assessment and
control
Yes No Yes
Successful? Stable? Angioembolization Successful?
No Yes No
Wedge resection Wedge resection

Monitor Monitor
or (include TV +
Serial H/H Serial H/H
Primary control via Pyloroplasty for
PPIs PPIs
gastrotomy II/III)
Algorithm 39.1
A. Initial treatment in the case of bleeding from a units of blood, recurrent hemorrhage after
gastric ulcer is based on resuscitation by one or more EGD attempts, repeated hospi-
obtaining intravenous access, fluid resuscita- talization for bleeding ulcer, and concurrent
tion, and placement of a nasogastric tube. indications for surgery such as perforation or
Nasogastric aspiration of blood is suggestive obstruction, surgical intervention is
of an upper GI bleed. any coagulopathy should indicated.
be corrected and transfusion should be initi-
C. Surgery depends on the stability of the
ated in case of instability or in case of signifi- patient. If unstable, gastric ulcer can be
cant blood loss. Proton pump inhibitor (PPI) oversewn for bleeding control. in a stable
therapy is particularly important, and should patient, resection of the gastric ulcer is the
be initiated early, while awaiting esophago- preferred approach. For types I, IV, and V,
gastroduodenoscopy (EGD), which, besides wedge resection alone is adequate (provided
establishing the diagnosis, is often the defini- that closure will not cause obstruction at the
tive treatment for most ulcers either through GE junction or the pylorus). For types II and
cautery, clipping, or epinephrine injection. III in a stable patient, the optimal surgical
B. If endoscopic control fails, angioemboliza- options are pyloroplasty with oversewning
tion can be attempted. endoscopic marking of the ulcer or antrectomy; both options should
the ulcer with a clip can facilitate endovascu- include a vagotomy, especially in patients
lar control, if such expertise and institutional with history of chronic PPI use. If a formal
experience is available. In the case of massive resection is not performed, a biopsy is
hemorrhage with failed endoscopic control, required as gastric ulcers are associated with
transfusion requirement of more than four risk of malignancy.
39 Gastric Ulcer Management 151
Perforation
Perforation
Resuscitate
No IV fluids
A Stable?
PPI
Antibiotics
Yes
Primary closure
B Minimal No and/or Omental
contamination? Patch
H pylori treatment
Yes
Wedge resection
C (add TV +
Pyloroplasty for
II/III)
Algorithm 39.2
A. In case of an obstruction or perforation, initial B. If tissue quality will not allow for primary
management should include ensuring ade- closure, formal resection and reconstruction
quate intravenous access and fluid resuscita- (simple wedge resection for types I, IV, and
tion, PPI therapy, and antibiotics (in case of V; antrectomy with reconstruction – See
perforation). For perforated gastric ulcers in above) may be performed if the patient is
an unstable patient, the ulcer should be biop- stable.
sied and a simple primary or omental patch C. For patients with extensive tissue damage and
closure can be performed. if the location and large perforations in type II and III ulcers,
extent of the perforation is such that primary with instability or severe duodenal scarring,
closure would lead to an obstruction (overall wide drainage including a duodenostomy
not common, but can be seen with type II or tube or pyloric exclusion with gastrojejunos-
III ulcers), inclusion of the perforation within tomy should be considered.
a pyloroplasty field may be advantageous.
Obstruction duodenal stump. When duodenal scarring is

minimal (commonly the case for gastric
ulcers), definitive treatment should consist of
Obstruction antrectomy. Vagotomy should be considered
for all patients, especially if they have a his-
tory of chronic PPI use (PPI therapy failure).
Reconstruction can consist of Bilroth I,
Resuscitate Bilroth II, or Roux-en-Y gastrojejunostomy,
No IV fluids depending on tissue mobility and surgeon
A Stable? NGT
PPI experience.
Yes
Intractable Ulcer
EGD with bx
B UGI after 1 week The initial management for intractable chronic
ulcers includes EGD with biopsy to rule out malig-
Persistent Obstruction nancy, in addition to removing any risk factors,
Antrectomy including smoking cessation, NSAID use, and
or assuring compliance with PPIs. For patients with
C Gastrojejunostomy
family history or multiple ulcers in different loca-
(consider addition
of TV) tions, Zollinger Ellison syndrome should be ruled
out. Surgical treatment should be considered for
Algorithm 39.3 patients who have had multiple recurrences or
failed two trials of PPI therapy of 12 weeks each.
It is paramount to perform biopsies with each
A. This is almost an exclusive complication of EGD. Surgery approach in this case depends on
type II and III ulcers. Patients who present the tissue quality of the duodenum. If the duode-
with an acute obstruction may benefit from a num demonstrates minimal scarring, antrectomy
course of nasogastric decompression and PPI and reconstruction (Bilroth I if there is enough
therapy, as a mode to rehabilitate the gastric mobility; Bilroth II or Roux-en-Y gastrojejunos-
body for longer term functional recovery, and tomy if there is not enough mobility for gastroduo-
to improve quality of gastric tissues in antici- denostomy) are the treatments of choice. If the
pation of resection. duodenal tissues are profoundly scarred, and there
B. In patients with obstruction, endoscopic dila- is a major concern for duodenal stump leak, repeat
tion can relieve the symptoms. Biopsy is par- biopsies and diversion with gastrojejunostomy
amount to assess for underlying malignancy. without resection remain as options. If resection is
The addition of PPI therapy, eradication of unavoidable, the “difficult duodenum” can be
Helicobacter pylori, nutritional support, and managed with Nissen stump closure (using the
smoking cessation will decrease the chance pancreatic capsule) or with primary closure and
of obstruction recurrence following endo- lateral duodenostomy tube drainage. Truncal
scopic or surgical therapy. vagotomy may be added for type II and III ulcers.
C. Surgical treatment is indicated if endoscopic
dilation fails or is not feasible, despite medi-
cal optimization. In this case, surgical Suggested Reading
approach will depend on the quality of the
duodenum. Patients with marked duodenal Cameron JL, Cameron AM. Stomach. In: Current surgical
therapy. 11th ed. Philadelphia, PA: Saunders; 2014.
scarring should undergo biopsy of the ulcer p. 69–107.
to rule out malignancy, and if negative, pro- Kitagawa Y, Dempsey DT. Stomach. In: Schwartz’s prin-
ceed with gastrojejunostomy (loop or Roux- ciples of surgery. New York City, NY: McGraw-Hill
en-Y) to avoid the risk of the “difficult” Education\Medical; 2015. p. 1035–95.
Duodenal Ulcer Management
40
Algorithmic Approach B. Bleeding is the most common complication

of duodenal ulcer. EGD is the best test for
Duodenal ulcers are more common compared both diagnosis and treatment. Biopsies can be
to gastric ulcers. These ulcers tend to be asso- performed to test for H. pylori and anti-H.
ciated with Helicobacter pylori infection and pylori therapy initiated. Similar to bleeding
are most commonly found in the first portion of gastric ulcers, endoscopic approach can be
the duodenum. Management of duodenal ulcer highly successful in bleeding control (see
begins with the assessment of hemodynamic Bleeding Gastric Ulcer section).
instability (see Chap. 39, Gastric Ulcer C. Surgical intervention is suggested for failure
Management). of or inability to provide endoscopic therapy.
A. Resuscitating the patient should be the initial This involves anterior duodenotomy with
goal. Surgery, although at this time it is rarely ligation of the gastroduodenal artery and
indicated, is needed in case of perforation, oversewing of the ulcer. For stable patients,
protracted bleeding despite endoscopic ther- especially with a history of chronic PPI use
apy, obstruction, intractability despite medi- (failure of PPI therapy), vagotomy and pylo-
cal treatment, or inability to rule out cancer. roplasty should be considered in this setting.
M. S. Altieri
K. Spaniolas (*)
e-mail: Konstantinos.Spaniolas@
stonybrookmedicine.edu

Bleeding
Resuscitate
No IV fluids
Stable? PPI drip
A
Transfuse
Yes
Endoscopic
assessment and
B control
No Duodenotomy and
Successful? suture ligation
C or pyloroplasty+TV
Yes
Monitor
Serial H/H
PPIs
H. pylori treatment
Algorithm 40.1
40 Duodenal Ulcer Management 155
A. Resuscitating the patient should be the initial C. If endoscopic therapy is unsuccessful or not
goal. indicated (see above—gastric ulcer obstruc-
B. In case of obstruction, initial treatment should tion) or if obstruction recurs, surgical therapy
concentrate on a trial of gastric decompression, is indicated (same as obstruction due to gas-
PPI therapy, and endoscopic assessment for pos- tric ulcer).
sible dilation (as well as biopsy for H. pylori).
Obstruction
No Resuscitate
Stable? IV fluids
A
NGT
PPI
Yes
EGD with bx
B UGI after 1 week
Persistent obstruction
Antrectomy
or
C Gastrojejunostomy
(consider addition
of TV)
Algorithm 40.2
A. Contained perforation in a stable patient can omental patch (Graham patch) is the most
be treated conservatively. The diagnosis of a commonly performed surgery. The addition
contained perforation is established on a of vagotomy should be considered for patients
patient with minimal or improving pain and on chronic PPI use (failure of PPI therapy).
hemodynamic stability and illustrated on con- For patients with extensive tissue damage and
trast imaging. This commonly occurs in cases large perforations, with instability or severe
where the ulcer is in the retroperitoneal loca- duodenal scarring, wide drainage including a
tion (segment III or IV of the duodenum). duodenostomy tube or pyloric exclusion with
B. In case of free perforation, peritonitis, or gastrojejunostomy should be considered
instability, surgery is required. Placement of along with distal feeding jejunostomy.
Perforation
A No Resuscitate
Stable? Antibiotics
PPI
Yes
Antibiotics
PPI
H.Pylori Yes Contained on
treatment imaging?
Close monitor
No B
Primary closure
and/or Omental
Patch
(Antrectomy+TV
if PPI failure)
(Lateral
duodenostomy or
pyloric exclusion
for severe tissue
damage)
Algorithm 40.3
Suggested Reading
Gilliam AD, Speake WJ, Lobo DN, et al. Current prac-
tice of emergency vagotomy and Helicobacter pylori
eradication for complicated peptic ulcer in the United
Kingdom. Br J Surg. 2003;90:88–90.
Complications of Peptic Ulcer
Disease 41
Algorithmic Approach diarrhea, gastric stasis, bile reflux, and Roux syn-
drome (Algorithm 41.1).
Surgical treatment of peptic ulcer disease (PUD) Dumping syndrome is more common after
traditionally involved one of the three operations: pyloroplasty or distal gastrectomy, affecting
highly selective vagotomy (HSV), vagotomy and 5–10% of patients, but can occur after vagotomy
drainage (V + D), or vagotomy and antrectomy without an emptying procedure as well. Dumping
(V + A). The choice of which procedure to per- syndrome is further characterized into early or
form depended on the patient’s condition and sur- late. Early dumping results in diaphoresis, weak-
geon’s preference/experience. All are considered ness, tachycardia, and lightheadedness that
acceptable treatments for PUD. Currently, with develop 15–30 min postprandially. Late dumping
the use of proton pump inhibitors, surgery for results in reactive hypoglycemia and hyperinsu-
PUD is uncommon. However, occasionally the linemia that occur 2–3 h after a meal. These
practicing surgeon will encounter patients with symptoms are thought to be related to the rapid
intractable disease or emergent manifestations of influx of hyperosmolar contents into the small
PUD, requiring surgical intervention. The afore- bowel due to lack of pylorus or pyloric function.
mentioned operations are not without complica-
tions and risks. Complications related to the A. The primary treatments are dietary modifica-
procedures are commonly the consequence of tions and avoiding fluids during meals and
different alterations they incur on the gastrointes- adding fibers.
tinal tract. These include dumping syndrome, B. Acarbose can be an effective medication for
late dumping syndrome.
C. Octreotide is the first line medication, which
can be started at 100 ug BID and up-titrated
to 500 ug TID.
C. J. Dickler D. If medical treatment fails, then the last resort
Department of General Surgery, SUNY Stony Brook is surgical revision. Options include reversed/
University Hospital, Health Sciences Center T19-030, antiperistaltic intestinal interpositions (which
have risks of obstruction) or conversion to
K. Spaniolas (*) Roux-En-Y gastrojejunostomy with or with-
out a vagotomy (goals should be to minimize
e-mail: Konstantinos.Spaniolas@ size of gastric remnant to lower risks of stasis
stonybrookmedicine.edu and ulcers).

158 C. J. Dickler and K. Spaniolas
Prior surgery for peptic

ulcer disease
Dumping
Roux Syndrome Diarrhea
Syndrome
A
Positive
Early Late
Hydrogen
Antibiotics breath
UGI or gastric test
emptying scan
Dietary Modifications A
A Negative
Positive
Acarbose B B
Promotility agents,
anti-emetics, low Acid Fecal
Octreotide dose opioids suppression fat test
C B Negative
Surgical
D Medical management:
revision
Revise gastric cholestyramine
C octreotide
remnant or
subtotal/total codeine/loperamide
gastrectomy
Fails
C
Jejunal
D interposition or
anti-peristaltic
limb
Algorithm 41.1
A portion of patients (25–30%) who have had A. UGI or gastric emptying scans will show

Roux-En-Y reconstruction will experience Roux delayed gastric emptying. The syndrome is
or Afferent Limb Syndrome. It is more predomi- thought to be due to disordered motility in the
nant with longer Roux limbs (>40 cm). Patients limb as the reconstruction displaces the jeju-
can experience vomiting, epigastric pain, early num away from pacemaker cells in the duo-
satiety, and weight loss. There can be dilation of denum. This leads to ectopic pacemaker
the Roux limb, gastric pouch, or distal efferent activity in the Roux limb that results in food
limb on imaging. peristalsis going toward the gastric remnant
instead of away from it.
41 Complications of Peptic Ulcer Disease 159
B. Medical management starts with promotility Gastric Stasis

agents, antiemetics, and low-dose opioids. A. The initial evaluation of gastric stasis is with
C. Persistent symptoms can require surgical endoscopy to rule out anastomotic strictures.
revision with either partial resection of the Patients with gastric stasis resulting from
gastric remnant or subtotal or total obstruction maybe relieved with endoscopic
gastrectomy. dilation as a first step. If recurrence of PUD
coexists with anastomotic stricture and gas-
Diarrhea is a common symptom after PUD tric stasis, aggressive management of the
surgery. Symptoms usually develop early postop- PUD (maximal PPI therapy, smoking cessa-
eratively. It is thought to be caused by dysmotil- tion, and eradication of Helicobacter pylori),
ity or accelerated transit through the GI tract, or concurrently with serial dilations, is of para-
related to bile acid malabsorption or bacterial mount importance.
overgrowth. B. If there is no evidence of stricture, the next diag-
nostic test would be a gastric emptying studies
A. The first step involves ruling out bacterial or upper GI series to check for any delay.
overgrowth (hydrogen breath test, or trial of C. Gastric stasis that results from motility issues
empiric antibiotic therapy, when testing is not can usually be treated medically (diet modifi-
available). cations and promotility agents).
B. The next step is to rule out fat malabsorption D. If treatment is refractory, then surgery (subto-
(fecal fat testing) which can be treated with tal gastrectomy) is an option as a last resort.
acid suppression.
C. If these tests are negative, the next medical
treatment is with cholestyramine, octreotide, Biliary Reflux
or antisecretory/antimotility agents (loper- A. The initial evaluation is with endoscopy,

amide, opioids, etc.). which can show bile during upper
endoscopy.
If medical treatment fails, the surgical treat- B. If there is no bile seen on upper endoscopy, the
ment involves revision with a possible jejunal diagnosis can be confirmed with a HIDA scan
interposition segment vs. antiperistaltic intestinal which will show prolonged imaging (tracer
limb (distal ileal graft). Both surgeries have risks appears to pool into the residual stomach).
of obstruction or bacterial overgrowth. C. Patients with biliary reflux can be managed
Gastric stasis and biliary reflux can also first with cholestyramine and sucralfate.
develop after these surgeries with similar symp- Efferent loop obstruction may also cause bili-
toms of nausea/vomiting, epigastric pain, bloat- ary reflux and can often be diagnosed, and
ing, and/or weight loss. They can both result even managed, endoscopically.
from disordered motility of the remaining stom- D. Patients with biliary reflux and failure of

ach, and gastric stasis can also result from medical therapy are best managed with con-
mechanical (anastomotic stricture) or functional version to Roux-en-Y gastrojejunostomy,
(retrograde peristalsis) obstruction. Gastric stasis with or without subtotal gastrectomy. If bili-
is usually suggested by emesis of undigested ary reflux occurs in patients with Roux-en-Y
food, while biliary reflux has biliary emesis configuration, elongation of the Roux limb
(Algorithm 41.2). will often alleviate the pathology.
Prior surgery for peptic

ulcer disease
Gastric stasis Biliary reflux Roux Syndrome
A A EGD Roux Syndrome

EGD
No
B HIDA Bile UGI or Gastric

Anastomotic Emptying Scan
stricture Dilate
Yes
C
Reflux Cholestyramine Promotility
Persists + Sucralfate Agents
No Yes
Persists
Revise Revise Gastric
B anastomosis Subtotal
Remnant or
D Subtotal/Total
gastrectomy Gastrectomy
Nuclear gastric Normal
emptying variant
study
Delayed No delay
Promotility C
agents
Persists
Subtotal D
gastrectomy
Algorithm 41.2
Kitagawa Y, Dempsey DT. Stomach. In: Schwartz’s prin-

Suggested Reading ciples of surgery. New York City, NY: McGraw-Hill
Education\Medical; 2015. p. 1035–95.
Savas JF, Miller TA. Postgastrectomy syndromes.
Bolton JS, Charles Conway W. Postgastrectomy syn- In: Shackelford’s surgery of the alimentary tract.
dromes. Surg Clin N Am. 2011;91(5):1105–22. Philadelphia, PA: Elsevier/Saunders; 2013. p. 757–66.
https://doi.org/10.1016/j.suc.2011.07.001. Teitelbaum, Ezra N, et al. Stomach. In: Sabiston textbook
Cameron JL, Cameron AM. Stomach. In: Current surgical of surgery: the biological basis of modern surgical
therapy. 11th ed. Philadelphia, PA: Saunders; 2014. practice. 12th ed. Philadephia, PA: Elsevier; 2017.
p. 69–107. p. 1188–236.
Management of Recurrent Peptic
Ulcer Disease 42
Algorithmic Approach tions (NSAIDs and smoking) can help patients

manage their disease. Clinicians must also be
Peptic ulcer disease (PUD) is a common cause of vigilant to other possible causes. All patients
epigastric pain and gastrointestinal symptoms with recurrent disease should undergo esoph-
throughout the world. The most common causes agogastroduodenoscopy (EGD) with biopsies
of PUD are Helicobacter pylori infection and the of any ulcers to rule out cancer and biopsy for
use of nonsteroidal anti-inflammatory drugs recurrent H. pylori infections.
(NSAIDs). Treatment of the disease has advanced B. Clinicians should have a high index of suspi-
significantly with the advent of proton pump cion for cancer if symptoms worsen despite
inhibitors (PPIs) and histamine-2 (H2) blockers. initial treatments. Patients with prior cancer
They have shifted the disease from being man- resections can have cancer manifest as new
aged surgically to medically in most cases. ulcer at or near the anastomosis, which can be
However, a small subset (5–10%) of patients will ruled out with biopsy during esophagogastro-
be refractory to standard treatment with PPIs. duodenoscopy (EGD).
When presented with such a patient, one must C. Having a thorough knowledge of a patient’s
thoroughly investigate the possible causes of prior surgical procedures can help rule out post-
recurrent or inadequately treated disease. surgical causes of recurrent disease. In the liter-
ature, this is most commonly the result of
A. The most common causes of recurrent PUD retained antrum from an incomplete antrectomy
are medication noncompliance, smoking, H. (proximal margins should be at the incisura of
pylori infection, and NSAID use. Ensuring the lesser curvature and the third branch of the
patient medication compliance and counsel- right gastroepiploic artery along the greater cur-
ing patients to adhere to lifestyle modifica- vature). This can be confirmed by performing a
sodium pertechnetate Tc 99 m scan.
C. J. Dickler D. If the prior history is not enlightening, basic
Department of General Surgery, SUNY Stony Brook laboratory testing can help point one in the
University Hospital, Health Sciences Center T19-030, right direction. Preliminary studies should
include evaluation for H. pylori and checking
K. Spaniolas (*) of gastrin levels, with a plan to follow up with
a secretin test if the results are equivocal.
e-mail: Konstantinos.Spaniolas@ These will help rule out a gastrinoma
stonybrookmedicine.edu (Zollinger–Ellison syndrome, ZES).

E. If elevated gastrin levels are seen, but there is meal test, which will show a 3× fold increase
a poor response to a secretion test (less than in gastrin levels in pZES, but only about 40%
200 pg/ml in gastrin levels), then pseudo- in ZES.
Zollinger–Ellison syndrome or antral G-cell F. Ulcers that do not respond to maximal medical
hyperplasia must be suspected. This can be therapy will require surgical revision with par-
distinguished from ZES by using a standard tial gastrectomy with or without vagotomy.
Recurrent peptic ulcer

disease
Endoscopy with biopsy (Cancer vs. H. pylori A

Medical Management (PPI BID, Smoking cessation, Treat H. pylori
Rule out cancer Re-evaluation

(Repeat endoscopy with Biopsy of symptoms
or screen with CT Scan)
Equivalent
Negative
C
Prior surgical Tc-99 scan

history? (Retained antrum)
Yes
Surgical Positive
resection No
D
Completion antrectomy
Octreotide Zollinger–Ellis Gastrin levels with redo anastomosis or
scan Syndrome (Secretin test) conversion to Roux En Y
Elevated
Normal
E
Pseudo-Zollinger– Standard meal
Ellison Syndrome test
Elevated
Normal
Nonhypergastrinemia related ulcer
Revision gastrectomy
Medical management
+/- vagotomy
Fails
Algorithm 42.1
42 Management of Recurrent Peptic Ulcer Disease 163
Suggested Reading Keuppens F, et al. Antral gastrin cell hyperplasia in

patients with peptic ulcer. Ann Surg. U.S. National
Library of Medicine, 1980. www.ncbi.nlm.nih.gov/
Cameron JL, Cameron AM. Stomach. In: Current surgical
pubmed/7362294.
therapy. 11th ed. Philadelphia, PA: Saunders; 2014.
Kitagawa Y, Dempsey DT. Stomach. In: Schwartz’s prin-
p. 69–107.
ciples of surgery. New York City, NY: McGraw-Hill
Dacha S, et al. Hypergastrinemia. Gastroenterol Rep.
Education\Medical; 2015. p. 1035–95.
2015;3(3):201–8. https://doi.org/10.1093/gastro/
Teitelbaum EN, et al. Stomach. In: Sabiston textbook
gov004.
of surgery: the biological basis of modern surgical
Fisher WE, et al. Pancreas. In: Schwartz’s principles of
practice. 12th ed. Philadephia, PA: Elsevier; 2017.
surgery. New York City, NY: McGraw-Hill Education\
p. 1188–236.
Medical; 2015. p. 1392–3.
Vakil N. Approach to refractory or recurrent peptic ulcer
Gray GR, et al. Extragastric gastrinoma or G-cell hyper-
disease. Approach to refractory or recurrent peptic
plasia of the antrum? The preoperative diagnosis in a
ulcer disease. UpToDate5, 5 July 2017, www.upto-
case of hypergastrinaemia. Br J Surg. 1976;63(8):596–
date.com/contents/approach-to-refractory-or-recur-
8. https://doi.org/10.1002/bjs.1800630809.
rent-peptic-ulcer-disease.
Management of Gastric Cancer
43
Christina L. Wolchok and Georgios V. Georgakis
Algorithmic Approach C. Upper gastrointestinal endoscopy and biopsy

is important for confirmation of the diagno-
A. Patients with gastric cancer present most
sis. Endoscopic ultrasound can give addi-
commonly with nonspecific abdominal pain. tional information about the T stage, the
It is important to identify the presenting presence of regional lymphadenopathy,
symptom or sign (heart burn, dysphagia, nau- which permits nodal biopsy via fine needle
sea, pain, obstruction, GI bleed, weight loss, aspiration. An imaging study like CT scan of
and anemia) as the presence of such symp- the chest, abdomen and pelvis or a PET/CT
toms signifies cancer at later stages. Signs evaluation (skull base to mid-thigh) is also
that are associated with advanced disease used for staging [1].
include a palpable abdominal mass, Virchow’s D. Clinical staging is done by reviewing all the
node (left supraclavicular), Sister Mary clinical data (endoscopy, EUS, CT, or PET
Joseph’s node (umbilical), Irish node (left CT) according to the AJCC guidelines, con-
axillary), Krukenberg’s tumor (adnexa/ sidering the tumor depth of invasion (T), the
ovary), Blumer’s shelf (pelvic cul-de-sac presence or absence of regional lymph nodes
mass), ascites, and jaundice. (N), and the presence or absence of distant
B. Some patients present in extremis as a result metastases (M). A multidisciplinary approach
of dehydration from gastric outlet obstruc- should be taken at all disease stages [2].
tion, malnutrition and weight loss from dys- E. Gastric cancer is deemed unresectable if there
phagia, and/or severe anemia from suspicious or biopsy proven positive lymph
malabsorption. In these cases, restoring the nodes at the root of the mesentery or para-aortic
patient’s homeostasis precedes any workup. nodes, distant metastases, or peritoneal seeding
(including positive peritoneal cytology).
F. In the case of unresectable disease, or in the
C. L. Wolchok event the patient is unfit for surgery, chemo-
Graduate Medical Education, Department of General therapy, chemoradiation, and/or palliative pro-
Surgery, Stony Brook University Hospital, cedures (stenting, resection for obstruction or
uncontrollable bleeding, and gastrostomy tube
G. V. Georgakis (*) placement for decompression) are the pre-
Department of Surgery, Division of Surgical
ferred modes of treatment. Once the treatment
Oncology, Stony Brook University Hospital,
Stony Brook, NY, USA is concluded, restaging may be performed to
e-mail: [email protected] re-evaluate the mass for possible resection.

166 C. L. Wolchok and G. V. Georgakis
G. When resectable, an initial diagnostic lapa- concluded, restaging may be performed to re-
roscopy with washings and cytology is evaluate the mass for resection [4].
strongly recommended [3, 4]. I. If cytology is negative, surgery with periop-
H. If cytology is positive, then the patient should erative chemotherapy or chemoradiation is
receive chemotherapy according to the multi- warranted [5, 6].
disciplinary approach. Once the treatment is
Vague abdominal pain or more specific symptoms

A Sings of obstruction, GI bleed, weight loss, anemia
In the event of a patient presenting in extremis (obstruction, dehydration,

B severe malnutrition), immediate measures to restore homeostasis (e.g., NG
decompression, IV hydration, and TPN initiation) are warranted.
· Esophagogastroduodenoscopy
· Endoscopic Ultrasound
C · CT Chest/abdomen/pelvis with IV contrast
· PET-CT for intestinal type and nonmucinous tumors
· CBC and comprehensive chemistry
D
Staging
Absence of metastatic disease Presence of metastatic disease

E Surgically resectable Surgically unresectable
Surgical candidate Nonsurgical candidate
Chemotherapy or Chemoradiation
Consider diagnostic laparoscopy
G with cytology
Palliative procedures for obstruction, F
uncontrollable bleeding, etc.
I Cytology negative Cytology positive H
Surgery
Perioperative chemotherapy Chemotherapy
Perioperative chemoradiation
Algorithm 43.1 Treatment algorithm for gastric cancer

43 Management of Gastric Cancer 167
References carcinoma for laparoscopic staging. Am J Surg.

2006;191(1):134–8.
4. Mezhir JJ, Shah MA, Jacks LM, Brennan MF, Coit DG,
1. Park SR, Lee JS, Kim CG, Kim HK, Kook MC,
Strong VE. Positive peritoneal cytology in patients
Kim YW, et al. Endoscopic ultrasound and com-
with gastric cancer: natural history and outcome of
puted tomography in restaging and predicting prog-
291 patients. Ann Surg Oncol. 2010;17(12):3173–80.
nosis after neoadjuvant chemotherapy in patients
5. Cunningham D, Allum WH, Stenning SP, Thompson
with locally advanced gastric cancer. Cancer.
JN, Van de Velde CJ, Nicolson M, et al. Perioperative
2008;112(11):2368–76.
chemotherapy versus surgery alone for resect-
2. Amin MB, Edge S, Greene F, Byrd DR, Brookland
able gastroesophageal cancer. N Engl J Med.
RK, Washington MK, Gershenwald JE, Compton
2006;355(1):11–20.
CC, Hess KR, Sullivan DC, Jessup JM, Brierley JD,
6. Smalley SR, Benedetti JK, Haller DG, Hundahl
Gaspar LE, Schilsky RL, Balch CM, Winchester DP,
SA, Estes NC, Ajani JA, et al. Updated analysis of
Asare EA, Madera M, Gress DM, Meyer LR, edi-
SWOG-directed intergroup study 0116: a phase III
tors. AJCC Cancer staging manual. 8th ed. Chicago:
trial of adjuvant radiochemotherapy versus obser-
Springer; 2017. p. 1032.
vation after curative gastric cancer resection. J Clin
3. Sarela AI, Lefkowitz R, Brennan MF, Karpeh
Oncol. 2012;30(19):2327–33.
MS. Selection of patients with gastric adeno-
Management of Gastrointestinal
Stromal Tumors 44
Igor G. Elyash
Algorithmic Approach tends to occur in the intraperitoneal cavity).

An esophagogastroduodenoscopy (EGD) is
A. Gastrointestinal stromal tumors (GISTs) are also helpful in characterizing the mass and
the most common nonepithelial tumors of the often shows a smooth mass covered by nor-
GI tract. They are characterized by genes mal mucosa. If there are any diagnostic dis-
encoding receptor protein tyrosine kinases, crepancies, magnetic resonance imaging
such as KIT and platelet-derived growth fac- (MRI) can also be completed as part of the
tor receptor alpha (PDGFRA). They can be workup.
seen in any part of the gastrointestinal tract C. Once a radiologic or clinical diagnosis of a
but are usually found in the stomach. GISTs GIST has been made, further management is
typically present in middle age, and only a dependent on resectability and tumor spread.
small percentage of them present in a famil- If the primary tumor demonstrates no signs of
ial/genetic pattern; most are sporadic. They metastasis and is highly suspicious of a GIST
are found in the wall of the gastrointestinal on imaging and endoscopy, there is no need for
tract and can have various types of presenta- a preoperative biopsy. Endoscopic ultrasound-
tion. Symptomatology includes early satiety, guided biopsy may assist in establishing a
abdominal discomfort, and bleeding. Other diagnosis in metastatic disease or if any clini-
times, they are asymptomatic and are inci- cal ambiguity exists. Resectable primary
dentally found on imaging and endoscopy. tumors are managed with surgical resection
B. Initial examination of patients begins with a with negative margins. If tumors have a high
history and physical characterizing symp- risk of recurrence (high mitotic rate, increased
toms and their natural course. Basic blood size), they are postoperatively treated with the
work can be ordered as part of the workup. tyrosine kinase inhibitor, imatinib. Large/unre-
The diagnosis is often made by CT of the sectable tumors are treated with neoadjuvant
abdomen with contrast. GISTs are often imatinib and then surgery if downsized.
smooth in appearance and enhance with con- D. Resected tumors are followed with exams

trast. CT scans can also identify any potential and CT scans every 3 to 6 months for 3 to
sites of metastasis (majority of metastasis 5 years and then on a yearly basis.
I. G. Elyash (*)
Morristown Surgical Associates, Morristown Medical

170 I. G. Elyash
A
History: Patient presenting with early satiety, vague
abdominal discomfort, GI bleed. Can be
asymptomatic with incidental finding.
B History and Physical exam: Symptoms and their natural

course.
Basic blood work and then evaluate symptoms with CT

abdomen/ EGD. MRI if any diagnostic discrepancies.
C
Resectable lesion?
Yes No
Surgical resection with EUS guided biopsy for

negative margins. confirmation.
Imatinib treatment
High risk of Yes
reoccurrence?
No
Surgical resection if
D resectable tumor
Surveillance
Algorithm 44.1
Suggested Reading
Brunicardi FC. Schwartz’s principles of surgery. 10th ed.
New York: McGraw Hill; 2015.
Cameron JL, Cameron AM. Current surgical therapy. 11th
ed. Philadelphia: Elsevier; 2014.
Keung EZ, Raut CP. Management of gastrointestinal stro-
mal tumors. Surg Clin North Am. 2017;97(2):437–52.
Management of Upper
Gastrointestinal Hemorrhage 45
Igor G. Elyash
Algorithmic Approach B. Baseline hemoglobin levels, coagulation pro-

files, basic chemistries, and liver functions
A. Upper gastrointestinal hemorrhage can present are helpful with management. After the
as hematemesis, melena, and also hematoche- patient is resuscitated and hemodynamically
zia in the setting of significant hemorrhage. stable, the initial diagnostic study is an upper
The initial approach with all patients starts endoscopy. Upper endoscopy allows for
with ensuring the maintenance/support of the localization of the source of bleeding and
airway and proper resuscitation with intrave- intervention at the same time.
nous (IV) fluids and blood products as needed. C. If the source is identified (i.e., peptic ulcers,
Patients should be in a monitored setting with varicosities, Mallory-Weiss tears), the goal is
strict monitoring of outputs and hemodynam- to control bleeding during endoscopy and
ics. The source of bleeding is further investi- treat it accordingly. If no source is identified,
gated with a thorough history and physical the next step is to proceed with either a tagged
examination. History should focus on previous red blood cell (RBC) scan for slow bleeding
episodes of bleeding, comorbidities, current or angiography for significant bleeding.
medications, previous surgical history, as well D. If unstable bleeding cannot be controlled with
as other symptoms. History of abdominal pain, endoscopy/angiography, the next step is sur-
dysphagia, retching, and jaundice/ascites can gical intervention. Bleeding may be con-
shed light on the cause of bleeding. Nasogastric trolled with oversewing and may occasionally
tube (NGT) and lavage are occasionally used if require resection. Recurrent bleeding often
source of bleeding is in question. requires surgical intervention as well.
I. G. Elyash (*)

172 I. G. Elyash
A History: Hematemesis, melena, hematochezia
Resuscitation with IVF and blood products if needed,

strict hemodynamic monitoring. History and physical:
Previous episodes of bleeding, comorbidities,
medications, other symptoms.
B
Order blood work (CBC, chemistries, LFTs, coagulation
profile). After proper resuscitation and suspicion of
upper GI bleed, endoscopy is the first test of choice.
Source of
bleeding is
identified?
Yes No
Slow Bleeding
Endoscopic control of or Massive
bleeding followed by
observation
Slow Massive
Consider RBC scan. If Angiography/surgical

D intervention
negative, observe or
If re-bleed, repeat
consider other source.
endoscopy. Additional
bleeding requires surgery.
Algorithm 45.1
Suggested Reading
Barkun AN, et al. International Consensus recommen-
dations on the management of patients with nonvari-
ceal upper gastrointestinal bleeding. Ann Intern Med.
2010;152(2):101–13.
Part VII
Small Bowel
Small Bowel Obstruction
46
Ryan M. Juza and Vamsi V. Alli
Algorithmic Approach which can rapidly become ischemic. A high

level of suspicion is necessary to accurately
A. Symptoms: Symptoms of small bowel diagnose and appropriately treat a small
obstruction include progressive abdominal bowel obstruction.
pain and distention with accompanying nau- B. Workup: The first step in evaluating a patient
sea and vomiting. Symptom development, with small bowel obstruction includes
however, can be variable depending on the obtaining a thorough history and physical
degree of obstruction. Partial bowel obstruc- examination. Pertinent history includes
tion results in delayed intestinal transit from past surgeries and endoscopies, previous
incomplete blockage of the bowel. Symptoms episodes, bowel habits, and symptom pro-
may develop gradually over several days, and gression. Physical exam should evaluate for
patients can continue to pass flatus or bowel signs of dehydration, evidence of hernias,
movements despite the obstruction. Complete presence and character of bowel sounds,
or high-grade bowel obstructions develop and signs of peritonitis. Digital rectal exam
more rapidly as a result of complete intestinal is necessary to rule out an obstructing rec-
blockage. Patients decompress proximally tal mass. The first diagnostic test typically
through emesis, and bowel function is absent. obtained is a plain abdominal X-ray, fol-
Closed-loop obstructions present rapidly lowed shortly after with a computed tomog-
without distention but with significant raphy (CT) of the abdomen, preferably with
abdominal pain and nausea. They result in an oral contrast. Typical findings include con-
isolated segment of bowel with both inflow tinuously dilated loops of proximal small
and outflow obstructions as a result of adhe- bowel with air-fluid levels, with distal
sive disease, volvulus, or internal hernias, decompression after a so-called transition
point. A fluid-filled stomach on imaging is
common but may be absent in the setting of
recent emesis. In addition to history, physi-
cal exam, and imaging, laboratory studies
are critical to the clinical care of the patient
R. M. Juza (*) ∙ V. V. Alli and will help determine whether a patient
Department of Surgery, Division of Minimally
requires an operation. A complete blood
Invasive and Bariatric Surgery, Penn State Milton S.
Hershey Medical Center, Hershey, PA, USA count, lactic acid, electrolyte and basic
e-mail: [email protected] metabolic panel, and international normal-

176 R. M. Juza and V. V. Alli
ized ratio are required. Depending on the vention. It should, however, be regarded
duration and severity of symptoms, signifi- carefully as a normal lactic acid level
cant electrolyte abnormalities and acute does not rule out intestinal ischemia in
kidney injury are possible and require the setting of venous outflow
aggressive fluid resuscitation with intrave- obstruction.
nous crystalloid solution and electrolyte C. Special groups: Certain patient subgroups
replacement. In a patient without preexist- require special attention.
ing kidney disease, endpoints of resuscita- (a) Virgin abdomen: Patients who present
tion include achieving adequate urine with obstructive symptoms without prior
output [1]. abdominal surgery warrant close surgical
(a) Concerning findings: Abdominal com- scrutiny. Previous dogma stated that all
plaints should be limited to mild general- patients with a “virgin” abdomen and
ized abdominal distention and discomfort. obstruction warrant operative exploration
Evidence of fevers, chills, or peritoneal because of the risk of malignant obstruc-
signs requires operative intervention. tion [2]. Recent studies, however, suggest
Small bowel obstruction secondary to that adhesive disease is still the most
adhesive disease should not typically likely culprit in patients without prior
cause significant hemodynamic, hemato- surgical history [3]. Regardless, patients
logic, or electrolyte abnormalities. The who present with obstructive symptoms
presence of these findings should prompt without prior abdominal surgery com-
closer evaluation. Depending on the mand a lower threshold for operation.
duration of symptoms, patients may dem- (b) Altered gastrointestinal (GI) tract anat-
onstrate tachycardia associated with omy, such as roux-en-Y gastric bypass:
hypovolemia, which should respond to Patients with prior gastric bypass surgery
intravenous fluid resuscitation. are at risk of internal hernia formation, in
Additionally, hypotension that is unre- addition to adhesive small bowel disease
sponsive to a weight-based fluid bolus is as the source of bowel obstruction. Internal
abnormal and should prompt further hernias form through the mesenteric
workup. defects created when performing a roux-
(b) Imaging: Findings on imaging include: en-Y reconstruction. Bowel herniated
(i) Isolated segments of dilated bowel. through these defects is at a high risk of
(ii) Nonphysiologic free fluid (i.e., incarceration and strangulation. Any
male/amenorrheic females). patient with prior gastric bypass warrants
(iii) Dilation greater than 3 cm in
additional workup, including an upper
diameter. gastrointestinal (UGI) or CT scan with
(iv) Pneumotosis intestinalis. oral and intravenous contrast to evaluate
(v) Free abdominal air. for internal hernia. Typical radiographic
(vi) Mesenteric edema. findings include swirling in the mesentery
(c) Laboratory: Significant leukocytosis is and loops of dilated bowel with interven-
suggestive of compromised bowel and ing segments of decompressed bowel.
should lower the threshold for operative Some surgeons advocate diagnostic lapa-
intervention. A mild leukocytosis can be roscopy in all gastric bypass patients who
seen with obstructive symptoms due to present with obstructive symptoms
bowel edema and hypovolemic state. because of the risk of strangulated internal
This should begin to normalize with hernia. The risk of internal hernia is not
appropriate nasogastric decompression unique to roux-en-Y reconstructions but
and resuscitation. Likewise, lactic acido- can occur anywhere a bowel resection and
sis should prompt earlier surgical inter- anastomosis create a mesenteric defect.
46 Small Bowel Obstruction 177
(c) Hernia: Abdominal wall hernias are a exam. Patients who are elderly, obtunded,
common cause of mechanical small or cognitively impaired may not have a
bowel obstruction. Herniated bowel is at reliable abdominal examination.
risk of incarceration and strangulation. Additionally, patients who are on steroids
CT evidence of a hernia with a narrow or are immune suppressed may not mani-
neck is more concerning for strangula- fest the typical signs and symptoms of
tion than a hernia with a wide neck where bowel ischemia. A surgeon should have a
the bowel can easily self-reduce. lower threshold for operative exploration
Attempts to manually reduce the hernia in this patient population.
can be performed and, if successful, often . Treatment: Initial management with nasogas-
D
alleviates the obstruction. If the patient tric decompression, fluid resuscitation, and
has exam findings suggestive of strangu- close monitoring is indicated. Patients who
lation, attempts to manually reduce the do not have signs of peritonitis or concerning
hernia should be avoided, and the patient lab and imaging findings can be managed
should be taken to the operating room for with electrolyte replacement and often with a
exploration as this is a sign of an underly- provocative upper GI series utilizing hyperos-
ing ischemic bowel. motic water-soluble contrast dye, which may
(d) Impaired patient: Nonoperative manage- decrease the time to return of bowel function
ment of patients with small bowel obstruc- [4]. Patients who fail to resolve their symp-
tion is contingent on the ability to serially toms after a 24–48-h trial of nonoperative
examine the patient for changes in clinical management require surgical exploration.
178 R. M. Juza and V. V. Alli
Patient with obstructive symptoms

A
Nasogastric tube
decompression, IV fluid
resuscitation
Thorough and focused history and physical

examination
Plan films – supine/upright Abdominal X-ray

CT abdomen/pelvis with oral and intravenous contrast
Laboratory studies
Workup consistent with small bowel obstruction
Yes
High-risk group OR
C
No
Yes
High-risk OR
features
No
24–48 hour trial nonoperative

management
Algorithm 46.1
46 Small Bowel Obstruction 179
Return of
bowel function
Partial
Consider small
bowel follow-
though study
Yes No
Contrast
reaches colon
No
Yes
Remove NG and OR
slow diet
advancement
Algorithm 46.1 (continued)
tion in the virgin abdomen: the need for a mandatory

References laparotomy explored. Am J Surg. 2014;208(2):243–8.
3. Tavangari FR, Batech M, Collins JC, Tejirian T. Small
1. Maung AA, Johnson DC, Piper GL, Barbosa RR, bowel obstructions in a virgin abdomen: is an opera-
Rowell SE, Bokhari F, Collins JN, Gordon JR, Ra JH, tion mandatory? Am Surg. 2016;82(10):1038–42.
Kerwin AJ, Eastern Association for the Surgery of 4. Di Saverio S, Catena F, Ansaloni L, Gavioli M,
Trauma. Evaluation and management of small-bowel Valentino M, Pinna AD. Water-soluble contrast
obstruction: an Eastern Association for the Surgery medium (gastrografin) value in adhesive small
of Trauma practice management guideline. J Trauma intestine obstruction (ASIO): a prospective, ran-
Acute Care Surg. 2012;73(5 Suppl 4):S362–9. domized, controlled, clinical trial. World J Surg.
2. Beardsley C, Furtado R, Mosse C, Gananadha S, 2008;32(10):2293–304.
Fergusson J, Jeans P, Beenen E. Small bowel obstruc-
Small Bowel Tumors
47
Vamsi V. Alli and Ryan M. Juza
Algorithmic Approach A. Clinical suspicion for small bowel mass.

The most common presentation of small
Small bowel tumors are a rare clinical entity but bowel tumors is abdominal pain, which occurs
one that the general surgeon should be prepared in 44–90% of patients, followed by weight
to address. They comprise 3–5% of all GI tumors loss in 24–44% and bleeding in 23–41% of
and 0.5% of cancer in the United States [1]. cases. Some patients remain asymptomatic
Symptoms are often vague and nonspecific, with until tumors reach a critical mass, resulting in
frequent delays in diagnosis, reportedly up to obstruction from obliteration of the lumen or
3 years for benign tumors and 18 months for intussusception. Obstructive symptoms such
malignant tumors [2]. As a result, small bowel as nausea and vomiting occur in 17–64% of
tumors may be encountered as either an inciden- patients with small bowel tumors, while
tal finding or as an unexpected source of nonspe- 22–26% of tumors present with bowel
cific abdominal pain, weight loss, or anemia. In obstruction and 6–9% with perforation and
the case of carcinoid tumors, diagnosis is made jaundice in 6% of patients [4].
following manifestation of systemic effects of B. For patients with suspected gastrointestinal
metastatic disease, i.e., carcinoid syndrome. malignancy, a thorough history should be
obtained, specifically inquiring regarding
abdominal pain, weight loss, fatigue, nausea,
Types of Small Bowel Tumors vomiting, anorexia, early satiety, abdominal
distension, change in bowel habits, melena.
Primary: adenocarcinoma, neuroendocrine Past medical history should inquire about
tumors (including carcinoid), sarcoma prior oncologic history, specifically lung,
(including gastrointestinal stromal tumors breast, cervical, colon cancers, as well as
(GIST)), metastatic [3] melanoma and sarcomas, all of which may
metastasize to the bowel.
Personal and family history of conditions
predisposing to small bowel malignancy
should be elicited, including Peutz–Jeghers,
V. V. Alli (*) ∙ R. M. Juza hereditary nonpolyposis colorectal cancer,
Department of Surgery, Division of Minimally
familial adenomatous polyposis syndrome,
Invasive and Bariatric Surgery, Penn State Milton
S. Hershey Medical Center, Hershey, PA, USA Crohn’s disease, neurofibromatosis type 1,
e-mail: [email protected] and celiac disease [5].

182 V. V. Alli and R. M. Juza
Review of symptoms relevant to meta- Additional endoscopic evaluation may

static disease should cover chest pain, short- identify the tumor. This includes capsule
ness of breath, cough, wheezing, hemoptysis, endoscopy (passive study with purely diagnos-
jaundice, acholic stool, neurologic symp- tic capability, sensitivity of 1.5–9%) [2] and
toms, dermatitis, diarrhea, and mental status either push (valuable until proximal jejunum)
changes. or double-balloon enteroscopy which allow
A focused abdominal exam should evalu- biopsy and potentially endoscopic resection.
ate for distension, masses, organomegaly, Once the mass is identified, metastatic
tenderness, and shifting dullness. Additional workup should be conducted as indicated.
attention should be paid to digital rectal . Treatment Based on Size, Location, and Tumor
E
exam and fecal occult blood testing. Type
Examination of lymphatic basins may reveal If size is <2 cm, assessment using endo-
evidence of metastasis from visceral sources, scopic ultrasound (EUS) may reveal high-risk
particularly the eponymous Sister Mary characteristics. If no high-risk features are
Joseph node (umbilical) and Virchow’s node noted, asymptomatic lesions found incidentally
(supraclavicular). may be surveilled, though there is a chance of
Laboratory examinations lack specificity. missed diagnosis of malignancy. *Lesions
However, complete blood count (CBC) with <1 cm in size found to be carcinoid tumors may
differential basic metabolic panel (BMP), be amenable to endoscopic resection alone.
liver function tests (LFTs), and coagulation If size is >2 cm, assess location and suitabil-
profile should be obtained to screen for ane- ity for resection. If the lesion is unresectable,
mia and abnormal differential (may indicate locally advanced, or workup reveals metastatic
lymphoma) and assess nutrition and gross disease, systemic chemotherapy (biopsy-
abnormalities in liver function or electrolytes guided therapy) is indicated. Treatment of met-
prior to potential procedures. astatic disease is largely palliative.
Tumor specific markers – Chromogranin
A in neuroendocrine tumors and 5-hydroxy- Surgery
indoleacetic acid (5-HIAA) in carcinoid
syndrome. When oncologically indicated and anatomically
C. Testing feasible, surgical resection with primary anas-
Initial options for radiographic imaging tomosis is preferred. Segmental resections
include small bowel follow-through (50–60% may be performed, with adjacent lymphade-
sensitivity) or axial imaging in the form of nectomy. Care should be taken to assess the
computed tomography (CT) or magnetic res- remainder of the GI tract, particularly in the
onance imaging (MRI) (both have >80% case of carcinoid (~30% are multiple) or meta-
sensitivity). static lesions, where multiple sites of disease
Endoscopic evaluation: Upper/lower are possible [3].
endoscopy for direct endoscopic identifica- • Endoscopic resection: Biopsy-proven benign
tion and potential biopsy. Twenty-five percent lesions may be resected endoscopically.
of small bowel masses occur in the • Difficult location: Tumors involving the
duodenum. first and second portion of the duodenum
. Additional Diagnostic Modalities
D are treated with a Whipple procedure.
Additional contrast-enhanced imaging of Distal ileal tumors require ileocolectomy.
the small bowel may be required if initial stud- • Metastatic disease may require palliative
ies fail to localize the tumor. These options resections for bleeding, or bypass for
include CT enterography (85% sensitivity) and malignant obstructions, and chemotherapy
MR enterography (91–97% sensitivity). for lymphoma.
47 Small Bowel Tumors 183
Concern for small bowelmalignancy A
Thorough and appropriate history & physical,

laboratory workup,evaluationfor signs of metastatic B
disease
Symptoms; Existing imaging
without any
imaging
C CT or MRI
Symptoms of
metastatic
disease
Endoscopic evaluation with EGD &/orcolonoscopy
Tumor
identified
D Yes
Metastatic
No Yes workup
Small bowel contrast studies (UGI, CTE, or MRE),

capsule studyor enteroscopy to identify small
bowel mass
No Metastatic disease
Positive for
Metastatic disease
E No Resectable
disease
Consider palliative
resection, bypass,
Yes
chemotherapyin cases of
lymphoma or carcinoid
Resection
Adjuvant
chemotherapy
Followup: Surveillence
& labs
Algorithm 47.1
184 V. V. Alli and R. M. Juza
References 4. Talamonti MS, Goetz LH, Rao S, Joehl RJ. Primary

cancers of the small bowel: analysis of prognostic fac-
tors and results of surgical management. Arch Surg.
1. Siegel RL, Miller KD, Jemal A. Cancer statistics,
2002;137(5):564–70.
2018. CA Cancer J Clin. 2018;68:7.
5. Kim JS, Park SH, Hansel S, Fletcher JG. Imaging and
2. Emanuele R, Anastasios K, Diana Y, Reddy SN, Julius
screening of cancer of the small bowel. Radiol Clin
G, Marco P. Neoplastic diseases of the small bowel.
North Am. 2017;55(6):1273–91.
Gastrointest Endosc Clin N Am. 2017;27:93–112.
3. Ashley SW, editor. Scientific American surgery.
Hamilton/Ontario/Philadelphia: Decker Intellectual
Properties; 2018. ISSN 1547-1616.
Management of Small Bowel
Neuroendocrine Tumors 48
Michele A. Riordon and Calvin H. L. Law
Algorithmic Approach sured by Ki-67. Most recent classification by

Ki-67 includes G1 (<3%), G2 (3–20%), and
A. Small bowel neuroendocrine tumors (NETs) G3 (>20%) [1–5].
include both foregut (duodenal) and midgut B. A multislice, triple phase computed tomogra-
(jejunal and ileal) types. Most foregut NETs phy (CT) of the chest, abdomen, and pelvis
do not produce a biologically active hormone (including an enterogram protocol) is the first
and are often classified as nonfunctioning. choice of imaging for both the detection and
Functioning NETs are usually from the midgut staging of small bowel neuroendocrine
and typically produce and secret serotonin, tumors. Magnetic resonance imaging (MRI)
tachykinin, and prostaglandins. Both groups of can be used if there is diagnostic uncertainty
NETs most often present with nonspecific after CT, there is a contraindication to the con-
symptoms, which often leads to delayed diag- trast medium, or to further characterize liver
nosis. A careful history and physical exam can involvement. Functional imaging such as
help provide clues that will determine the next 111
In-pentetreotide (OctreoScan) or positron
steps in the investigation and which patients emission tomography (PET)/CT imaging with
are at risk of having functional tumors and 68Ga-labeled somatostatin analog are useful
metastatic disease. Carcinoid syndrome, clas- in the localization of well-differentiated small
sically characterized by flushing, diarrhea, and bowel NETs, whereas a flurodeoxyglucose
valvular heart disease, occurs most often in (18FDG) PET/CT is more useful for poorly
metastatic NETs of the small bowel, but the differentiated tumor localization [5–7].
overall incidence is less than 13%. Biological C. Biochemical workup with a serum chromo-
behavior and response to therapy are linked to granin a (CgA) level and a 24-h
differentiation and the grade of tumor as mea- 5-hydroxyindole acetic acid (5-HIAA) urine
collection are not considered first-line diag-
nostic tests. The sensitivity of serum CgA is
in the range of 67–93%, but the specificity is
M. A. Riordon less than 50% [8, 9]. A 24-h urine 5-HIAA
Department of Surgery, Royal Victoria Regional
level has a sensitivity that approaches 100%
Health Centre, Barrie, ON, Canada
but a specificity of only 35%, and it is falsely
C. H. L. Law (*)
elevated by serotonin-rich foods [9, 10].
Department of Surgical Oncology, Sunnybrook
Health Sciences Centre, Toronto, ON, Canada However, carcinoid syndrome is a result of
e-mail: [email protected] metastases outside the portal circulation and

186 M. A. Riordon and C. H. L. Law
thus elevates serum serotonin by avoiding Metastatic treatment: Tissue diagnosis for
first-pass effect. In this situation, 24-h urine differentiation and Ki-67 index are key to
5-HIAA may confirm elevated serotonin and choosing the optimal treatment. Options
identify this population of patients at risk of include observation; somatostatin analogs;
carcinoid crisis. palliative surgery to prevent symptoms (small

D. Carcinoid crisis is associated with a rapid bowel resection to prevent obstruction and
release of hormones from metastatic tumor cholecystectomy to decrease somatostatin
deposits that can result in sudden fluctuations in side effects); surgical cytoreduction (particu-
blood pressure (most commonly hypotension), larly for functional tumors); liver-directed
tachycardia, bronchospasm, and hyperthermia. therapy, including embolization options; and
If a patient has features of carcinoid syndrome, chemotherapy for high Ki-67 or poorly dif-
periprocedural prophylaxis with octreotide is ferentiated tumors.
used in attempts to prevent a carcinoid crisis, F. Disease progression:
but recent data suggest that prophylactic octreo- –– G1 and G2 (Ki-67 < 20%) – Targeted
tide may not be beneficial. Shortening the dura- molecular therapy: Tyrosine kinase inhibi-
tion of hemodynamic instability with prompt tors (i.E., everolimus), peptide receptor
usage of vasopressors does seem to decrease radionuclide therapy (177-lutetium).
complications from the crisis [11, 12]. –– G3 and/or poorly differentiated (Ki-
E. Localized treatment: Curative intent surgery 67 > 20%): Platinum-based chemotherapy.
includes exam for synchronous, multifocal –– For all tumors – Additional symptom
lesions and regional lymphadenectomy. control may also be achieved with telo-
Avoidance of short-gut syndrome is an impor- tristat in patients with functional midgut
tant consideration. tumors.
48 Management of Small Bowel Neuroendocrine Tumors 187
History
-Local symptoms: abdominalpain, mass, obstructive symptoms, bloating,
gastrointestinal bleeding
-Systemic symptoms: weight loss,
-Functional neuroendocrine symptoms from small bowel tumors
-Carcinoid syndrome: flushing, diarrhea, valvular heart disease
-Bronchospasm: (wheezing, chestpain, cough)
A
Physical exam
Cardiac: elevated JVP, cardiomegaly, murmurs, edema
Respiratory: wheezing
Abdominal: mass, signs of bowel obstruction, hepatomegaly, stigmata
of liver disease
General: evidence of flushing, signs of weight loss
CT scan (enterogram protocol)

showing small bowel primary,
mesenteric mass or clinical features
suggestive of a small bowel neuro
endocine tumor?
B
Workup
1. Multiphase CT abdomen and pelvis for primary tumor evaluation and to rule out
metastatic liver disease
2. MRI if CT nondiagnostic or further liver evaluation required
3. Octreotide scan – (111-In-labelled pentetreotide) for primary tumor evaluation
and metastatic disease (or preferably 68GA scan if available)
4. PET/CTscan – especially if there is negative octreotide/68GA scan
5. Cardiac consult and ECHO if abnormal cardiac exam
Algorithm 48.1
Evidence of carcinoid syndrome

on history, exam or
metastatic disease on imaging?
Yes
1. Chromogran in A level
No 2. 24-hour urine 5-HIAA
Elevat
Yes
No
Perioperative
octreotide
prophylaxis prior to
any biopsy or surgery
Localized, advanced,
or metastatic disease?
E, F
Localized and resectable disease Locally advanced disease Metastatic disease

1. Inspect for synchronous lesions 1. Resection of primary and 1. Obtain tissue for diagnosis and
2. En-bloc margin-negative resection metastatic disease with differentiation with Ki-67 index
3. Adequate lymphadenectomy curative intent 2. Observation
2. Avoid short guy syndrome 3. Somatostatin analogues
4. Surgical cytoreduction
5. Palliative surgery for symptoms
6. Liver directed therapy
7. Chemotherapy for high Ki-
67/poorly differentiated tumors
48 Management of Small Bowel Neuroendocrine Tumors 189
Disease progression
G (consider re-biopsy to
ensure no change in Ki-67)
G1, G2 (KI-67<20%) G3 (KI-67>20%)
Targeted molecular therapy

1. Tyrosine kinase inhibitors (i.e.,
everolimus) Platinum-based chemotherapy
2. Peptide receptor radionuclide therapy
(i.e.,177-Lutetium)
Functional Functional
tumor? tumor?
Consider telotristat for

additional symptom control
metastatic presentation, and outcomes. Cancer.

References 2015;121:589–97.
4. Pape UF, Berndt U, Muller-Nordhorn J. Prognostic
1. Strosberg JR, Weber JM, Feldman M, et al. Prognostic factors of long-term outcome in gastroenteropancre-
validity of the American Joint Committee on can- atic neuroendocrine tumors. Endocr Relat Cancer.
cer staging classification for midgut neuroendocrine 2008;15:183–97.
tumors. J Clin Oncol. 2013;31:420–5. 5. Raphael MJ, Chan DL, Law C, et al. Principles of
2. Modlin IM, Oberg K, Chung DC, et al. diagnosis and management of neuroendocrine tumors.
Gastroenteropancreatic neuroendocrine tumors. CMAJ. 2017;189:E398–404.
Lancet Oncol. 2008;9:61–72. 6. Onaitis MW, Kirshbom PM, Hayward TZ, et al.
3. Hallet J, Law CHL, Cukier M, et al. Exploring Gastrointestinal carcinoids: characteristics by
the risking incidence of neuroendocrine tumors: site of origin and hormone production. Ann Surg.
a population- based analysis of epidemiology, 2000;232:549–56.
7. Grimaldi F, Fazio N, Attanasio R, et al. Italian 10. Bajetta E, Ferrari L, Martinetti A, et al. Chromogranin
Association of Clinical endocrinologists (AME) A, neuron specific enolase, carinoembryonic anti-
position statement: a stepwise clinical approach gen, and hydroxyindole acetic acid evaluation
to the diagnosis of gastroenteropancreatic neu- in patients with neuroendocrine tumors. Cancer.
roendocrine neoplasms. J Endocrinol Investig. 1999;86:858–65.
2014;37:875–909. 11. Woltering EA, Wright AE, Stevens MA, et al.

8. Stridsberg M, Eriksson B, Oberg K, et al. A compari- Development of effective prophylaxis against
son between three commercial kits for chromogranin intraoperative carcinoid crisis. J Clin Anesth.
a measurements. J Endocinol. 2003;177:337–41. 2016;32:189–93.
9. Singh S, Law C. Chromogranin a: a sensitive bio- 12. Condron ME, Pommier SJ, Pommier RF. Continuous
marker for the detection and post-treatment monitor- infusion of octreotide combined with perioperative
ing of gastroenteropancreatic neuroendocrine tumors. octreotide bolus does not prevent intraoperative car-
Expert Rev Gastroenterol Hepatol. 2012;6:313–34. cinoid crisis. Surgery. 2016;159:358–65.
Management of Enterocutaneous
Fistulas 49
Maria Michailidou
Algorithmic Approach initially made nil per os (NPO), undergo fluid

resuscitation, and have any electrolyte
A. Patients with EC fistulas typically present
derangements intravenously corrected. Sepsis
with drainage of enteric contents through the accounts for the majority of morbidity related
abdominal wall or with occult findings of to EC fistulas, and therefore any sign of sep-
abdominal pain, ileus, fevers, malaise, and a sis should mandate early administration of
CT consistent with an intraabdominal broad spectrum antibiotics. In addition, cross-
abscess. Past medical and surgical history sectional imaging with CT of the abdomen
should be obtained, including any history of and pelvis may identify any intraabdominal
abdominal operations, trauma, malignancy, fluid collections that require image-guided
radiation, or inflammatory bowel disease. EC drainage, as well as sites of distal obstruction.
fistulas are categorized based on etiology (iat- Patients with peritonitis or persistent septic
rogenic, mesh related, because of inflamma- shock should be taken to the operating room
tory bowel disease, diverticulitis, radiation for wide drainage and enteric diversion.
effects, trauma, neoplastic process), location Many patients are nutritionally depleted upon
(proximal or distal small bowel), and daily presentation and suffer from severe protein
output (low < 200 ml/day, intermediate 200– losses from the EC fistula and therefore
500 ml/day, high > 500 ml). require supplemental nutrition, mainly in the
B. EC fistulas carry high morbidity and mortal- form of total parenteral nutrition (TPN).
ity and therefore require a multidisciplinary Nutritional goals should include an average
approach. Principal management involves caloric and protein intake of 30 kcal/kg/day
fluid resuscitation with electrolyte correction and 1.5–2.5 g/kg/day, respectively. High-
and replacement of fluid losses, characteriza- output fistulas may be controlled with TPN,
tion and control of sepsis, nutritional support, proton pump inhibitors (PPI), antidiarrheals,
and local wound care [1]. Patients should be and octreotide injections [2]. The goal of
enteric output should be <1.5 L/day. In
patients with no evidence of ileus or no
increase in the daily fistula output after intro-
duction of oral intake, enteral feeds are pre-
ferred over TPN or should be used
M. Michailidou (*)
Department of Surgery, Penn State Milton S. Hershey supplementarily to TPN. Local wound care
Medical Center, Hershey, PA, USA should involve a wound care specialist and/or

192 M. Michailidou
enterostomal therapist and aim at controlling D. Surgical management should be offered no

the effluent and protect the adjacent skin site, sooner than 12 weeks (ideally 6 months)
with either local wound barriers or negative from the onset of the EC fistula in patients
pressure vacuum dressings [3]. Special situa- whose nutrition has been optimized, sepsis
tions include patients with spontaneous fistu- has resolved, and comorbidities managed
las secondary to inflammatory bowel disease [5, 6]. Preoperatively, cross-sectional imag-
(IBD). Percutaneous drainage, along with the ing should be obtained to rule out occult
use of a biologic agent, can aid in the sponta- fluid collections, as well as a fistulogram to
neous closure of the fistula [4]. rule out distal obstruction. Surgical princi-
C. About one third of fistulas will spontaneously ples include safe entry to the abdomen away
close by 4–6 weeks. Fistulas that remain open from the EC fistula, lysis of adhesions,
after 12 weeks will require surgical manage- inspection of potential distal obstruction,
ment. Factors associated with failure of sponta- avoidance of enterotomies, removal of
neous closure include retained foreign body retained foreign body, and takedown of EC
(most commonly permanent suture or mesh), fistula with resection of involved segment(s)
high-output (often proximal) fistulas such as and primary anastomosis. Often, full thick-
jejunal or ileal fistulas, presence of distal obstruc- ness resection of the EC fistula and the
tion, spontaneous fistulas secondary to radiation, abdominal wall is required, which may
IBD or malignancy, as well as short (<2 cm) fis- necessitate complex abdominal wall recon-
tulas and those with epithelialized tracts. struction [7].
49 Management of Enterocutaneous Fistulas 193
Drainage of enteric contents through surgical site /abdominal wall

Ileus, abdominal pain, fevers, leukocytosis s/p abdominal operation
A Obtain past medical and surgical history

- Prior abdominal operations
- Radiation
- Malignancy
- Inflammatory bowel disease
Yes Operative
Early (<7 days) exploration,
postoperative, bowel
sepsis or resection,
peritonitis washout,
ostomy
No
B 1. NPO, IV fluid resuscitation, Electrolyte repletion

2. Sepsis Control / Define fistula
CT Abdomen/Pelvis to rule out abscess, distal
obstruction
Broad Spectrum Antibiotics
CT guided drainage of intra-abdominal abscess
3. Nutritional support with TPN
4. Local wound care
5. IBD related –CT Enterography/Biologic therapy
Yes No
Oral nutritional Proton Pump Inhibitors
Output <1.5 Octreotide
supplementation L/day?
Wean TPN if tolerated Loperamide
Yes
C Fistula Expectant Observation

Resolves?
No
Repeat CT Abdomen/Pelvis and Fistulogram
D Optimize nutrition
OR in 6 months for LOA, ECF takedown with bowel
resection, abdominal wall reconstruction
Algorithm 49.1
194 M. Michailidou
References neous fistula in patients with Crohn’s disease treated

with anti-TNF therapy: a cohort study from the
GETAID. Am J Gastroenterol. 2014;109(9):1443–9.
1. Gribovskaja-Rupp I, Melton GB. Enterocutaneous
5. Brenner M, Clayton JL, Tillou A, Hiatt JR, Cryer
fistula: proven strategies and updates. Clin Colon
HG. Risk factors for recurrence after repair of entero-
Rectal Surg. 2016;29(2):130–7.
cutaneous fistula. Arch Surg (Chicago, Ill: 1960).
2. Rahbour G, Siddiqui MR, Ullah MR, Gabe SM,
2009;144(6):500–5.
Warusavitarne J, Vaizey CJ. A meta-analysis of
6. Visschers RG, van Gemert WG, Winkens B, Soeters
outcomes following use of somatostatin and its ana-
PB, Olde Damink SW. Guided treatment improves
logues for the management of enterocutaneous fistu-
outcome of patients with enterocutaneous fistulas.
las. Ann Surg. 2012;256(6):946–54.
World J Surg. 2012;36(10):2341–8.
3. Misky A, Hotouras A, Ribas Y, Ramar S, Bhan C. A
7. Krpata DM, Stein SL, Eston M, Ermlich B, Blatnik
systematic literature review on the use of vacuum
JA, Novitsky YW, et al. Outcomes of simultane-
assisted closure for enterocutaneous fistula. Color
ous large complex abdominal wall reconstruction
Dis. 2016;18(9):846–51.
and enterocutaneous fistula takedown. Am J Surg.
4. Amiot A, Setakhr V, Seksik P, Allez M, Treton X,
2013;205(3):354–8; discussion 8–9.
De Vos M, et al. Long-term outcome of enterocuta-
Management of Crohn’s Disease
50
Igor G. Elyash
Algorithmic Approach C. If the patient is having active disease, medical

therapy is initiated. Treatment consists of ste-
A. Crohn’s disease usually presents in the third roids and biologic medications. If medical
and sixth decades of life as a focal inflamma- therapy is successful, the patient is followed
tion that can occur at any location along the closely and considered for remission therapy.
GI tract from the mouth to anus, usually spar- If medical therapy fails in treating active
ing the rectum. Symptoms include intermit- inflammation and/or the patient’s symptoms
tent abdominal pain, diarrhea, and weight worsen due to medical treatment (i.e., ste-
loss. Patients can also present with extraintes- roids), surgical intervention should be consid-
tinal manifestations such as ocular disease, ered. Patients should also be considered for
skin lesions, and joint disease. Initial evalua- surgery if they demonstrate signs of peritoni-
tion includes a history and physical examina- tis, bowel obstruction, signs of perforation,
tion and should assess for previous episodes, toxic megacolon, concern for malignancy,
family history of inflammatory bowel dis- and/or fistulas.
ease, and any prior treatments. D. In the operating room, the goal is disease
B. A thorough workup of Crohn’s disease should resection with gross margins. Patients with
include blood work and endoscopic evalua- Crohn’s disease will oftentimes require mul-
tion to assess the level of inflammation and tiple surgeries in their lifetime. It is impor-
also to rule out possible malignancy. A com- tant to be mindful of short-gut syndrome,
puted tomography (CT) scan can be helpful which can be a devastating complication of
to rule out specific abdominal pathology and multiple bowel resections. If large segments
may identify fistulas and abscess formation. of bowel are involved or a patient has mul-
Testing for perinuclear antineutrophil cyto- tiple previous bowel resections, stricturo-
plasmic antibody (p-ANCA) may be consid- plasty should be considered over segmental
ered when the diagnosis is unclear. resection.
I. G. Elyash (*)

196 I. G. Elyash
A History: Diffuse abdominal pain, diarrhea,

weight loss with intermittent symptoms.
Perform a history and physical exam. Ask about

previous episodes, family history of inflammatory
bowel disease, previous skin or ophthalmic issues.
B Order blood work (CBC, chemistries, LFTs),

Endoscopy, CT scan with contrast. May consider
antibody testing.
C If signs of active inflammatory disease confirmed by

studies, start Medical Therapy (5-ASA, Steroids,
biologic medications)
Response to medical Poor response to Peritoneal signs, bowel

Therapy medication or complications obstruction, signs of perforation,
due to medications toxic megacolon, signs of mass,
fistulas.
D Close follow up-

consider
medication for Operative Intervention
remission
Resection of gross
disease only
Yes Risk of short
Consider Stricturoplasty gut syndrome? No
Algorithm 50.1
Suggested Reading
Management of Postoperative
Ileus 51
Igor G. Elyash
Algorithmic Approach C. Initial treatment for postoperative ileus is

supportive. Treat any infectious process with

A. Postoperative ileus typically presents with appropriate antibiotics if needed. Provide
symptoms of nausea, vomiting, abdominal proper fluid and electrolyte replacement.
pain, and abdominal distention postopera- Medications that slow gastrointestinal (GI)
tively. Evaluation begins with a comprehen- motility, such as opiates, should be limited.
sive history and physical examination. History Consider nasogastric tube (NGT) placement
should include assessment of a patient’s past and supplemental nutrition. Patients should
medical history, events during the surgery, and also be on bowel rest and have serial abdomi-
current medications. nal examinations.

B. After performing a history and physical D. If disease is prolonged without any clinical
exam, blood work should be obtained to look improvement, a computed tomography (CT)
for elevations in white blood cells and scan may be required to look for certain treat-
changes in electrolytes. An abdominal X-ray able causes such as fluid collections or
with supine and upright images should be abscess formation. Specific disease processes
ordered to look for signs of bowel distention. should be treated accordingly. If there is no
This will confirm the diagnosis. If a patient resolution of symptoms, surgical exploration
develops fevers, tachycardia, peritoneal signs, may be necessary.
and/or clinically deteriorates, this is an indi-
cation for operative exploration.
I. G. Elyash ()
Morristown Surgical Associates,
Morristown Medical Center, Morristown, NJ, USA

198 I. G. Elyash
A History: Distended abdomen with diffuse tenderness,

nausea/vomiting a few days after abdominal surgery
Perform a history and physical exam. Review medical

history, medications patient is taking and operative events.
B Order blood work and supine/upright plain abdominal film.
Signs of fever,
X-ray shows dilated bowel tachycardia, generalized
loops in continuous pattern peritonitis.
C Supportive care: Treat infections, Operative Intervention

fluid/electrolyte replacement, limit
opiates, bowel rest, serial abdominal
exams. Consider NGT, may need
supplemental nutrition
D
Prolonged disease despite
conservative care
Order CT abdomen Treat specific findings. May

with contrast need surgical intervention.
Algorithm 51.1
Suggested Reading
Management of Gallstone Ileus
52
Igor G. Elyash
Algorithmic Approach signs of small bowel obstruction, and pres-

ence of a radiolucent gallstone. If no signs of

A. Gallstone ileus classically presents with gallstone ileus are present, appropriate care is
symptoms of episodic bowel obstruction. continued.
Usually, this involves nausea, vomiting, dif- C. When signs of gallstone ileus are seen on CT
fuse abdominal pain, and constipation. or if patient is having diffuse peritoneal signs,
Patients are often female and are elderly. A surgical exploration after resuscitation is nec-
detailed history and physical examination essary. During laparotomy, the entire bowel
should be taken, with a focus on any history of should be inspected as multiple stones in vari-
biliary disease and prior episodes of small ous locations are possible. Once the area of
bowel obstruction. Following a history and obstruction is localized, a longitudinal enter-
physical exam, supportive care such as intra- otomy should be made proximal to the area of
venous (IV) hydration and nasogastric inser- obstruction and the stone removed. Any
tion should be started. Any diffuse peritoneal necrotic bowel should be resected if necessary.
signs should prompt emergency surgical If bowel is viable, the enterotomy should be
exploration. Blood work should look for closed in two layers in a transverse fashion.
changes in white blood cell count and any D. Once enterolithotomy is performed, a deci-
electrolyte abnormalities. Liver function tests sion should be made regarding a cholecystec-
will usually be nonspecific. If patient is hemo- tomy and fistula take down. Patients that have
dynamically stable, proceed with a computed minimal comorbidities, are hemodynamically
tomography (CT) of the abdomen and pelvis. stable, and with limited inflammatory changes
B. CT scan is the test of choice for gallstone can undergo cholecystectomy and bilioen-
ileus. It will show signs that are suspicious teric fistula closure at the time of initial oper-
for the diagnosis such as bowel obstruction or ation. All other patients (hemodynamically
gallbladder thickening. The most characteris- unstable, severe comorbidities) should
tic and pathognomonic finding is Rigler’s undergo a cholecystectomy and fistula take-
triad. It is characterized by pneumobilia, down electively once medically optimized.
I. G. Elyash (*)

200 I. G. Elyash
A
History: Episodes of nausea/vomiting with diffuse
abdominal pain and constipation that are
intermittent in nature.
B
Perform a physical exam. Start IVF and consider NGT
placement. Order labs and CT scan with contrast.
Any signs of
Rigler’s triad on
CT?
Yes No
Appropriate
C Resuscitation followed by laparotomy: Inspect care
entire bowel, identify site of obstruction and
perform longitudinal enterolithotomy to
obstruction. Resect necrotic bowel
D
Is the patient
high risk?
No
Yes
Can perform cholecystectomy and fistula take

Finish operation. Patient can have an elective
down at same time as laparotomy
cholecystectomy if recurrent symptoms and if
medically optimized.
Algorithm 52.1
Suggested Reading
Nuno-Guzman CM, et al. Gallstone ileus, clinical pre-
sentation, diagnostic and treatment approach. World J
Gastrointest Surg. 2016;8(1):65–76.
Ravikumar R, Williams JG. The operative manage-
ment of gallstone ileus. Ann R Coll Surg Engl.
2010;92:279–81.
Management of Short Bowel
Syndrome 53
Igor G. Elyash
Algorithmic Approach C. The first step in the management of short

bowel syndrome is supportive. Treat any
A. Short bowel syndrome is characterized by
underlying disorders, replete fluids and elec-
compromised intestinal absorption leading to trolytes, consider antimotility agents, and
symptoms of malabsorption. These include provide parenteral nutrition as needed. In
diarrhea, dehydration, electrolyte abnormali- many cases, intestines will undergo adapta-
ties, and/or malnutrition. Evaluation begins tion, and bowel absorption will be restored.
with a thorough history and physical exami- When medical therapy fails or patients
nation. History should include prior medical develop complications related to total paren-
history with a focus on symptoms of Crohn’s teral nutrition (TPN) or chronic malabsorp-
disease and any prior bowel surgery. Increased tion, surgical options are considered.
risk of short bowel syndrome occurs with a D. If medical therapy fails and the patient has
shortened small bowel length, previous not developed complications, such as sepsis,
colonic surgery, and absence of a functional organ failure from TPN or malabsorption,
ileocecal valve. certain surgical options are available. If the
B. After a thorough history and physical exam, patient has an ostomy, attempts should be
the next step in evaluation is to obtain blood made to restore bowel continuity. Patients can
work. Electrolyte abnormalities will need to also undergo surgery to slow bowel transit
be corrected, as with any vitamin deficien- such as segmental reversal of small bowel or
cies. An albumin level can be helpful in interpositioning of colon between small
assessing overall nutrition status. It is also bowel. If bowels are dilated, patients can
important to rule out other potential causes of undergo the longitudinal intestinal lengthen-
malabsorption and malnutrition such as pan- ing and tailoring (LILT) or serial transverse
creatitis, celiac disease, and infectious causes. enteroplasty procedure (STEP) to increase
Stool studies may be helpful. Endoscopy can intestinal length. Intestinal transplantation
be considered to assess the quality of bowel should be considered if a patient develops
as well. severe or life-threatening complications.
I. G. Elyash (*)
Morristown Surgical Associates,
Morristown Medical Center, Morristown, NJ, USA

202 I. G. Elyash
A
History: Symptoms of diarrhea, steatorrhea,
dehydration, malnutrition.
Perform a history and physical: History should look for

previous bowel surgery, surgery for malignancy, history
of Crohn’s, pediatric intestinal disorders.
B
Order Lab work. May consider endoscopy and
additional studies as needed.
C Treat primary condition, fluid/electrolyte

replacement/parenteral nutrition, anti-motility
agents.
Symptoms not improving despite medical

treatment
Major Complications related to intestinal

failure/Long term artificial nutrition?
No Yes
Consider
Consider surgical option such as
Intestinal
LILT, STEP.
Transplantation.
Algorithm 53.1
Suggested Reading
Iyer KR. Surgical management of short bowel syndrome.
J Parenter Enter Nutr. 2014;38:53–9.
Part VIII
Large Bowel
Management of Lower
Gastrointestinal Bleeding 54
Algorithmic Approach source masquerading as an LGIB (10–15% of

cases) [1]. If either of these is positive, appro-
A. Lower gastrointestinal bleeding (LGIB) can priate interventions to address the source
range from low-volume bleeding episodes of should be pursued.
no hemodynamic consequence to life- D. For patients with a self-limited bleeding epi-
threatening hemorrhage. A thorough history sode who respond to resuscitative efforts, an
eliciting the onset, frequency, duration, and early colonoscopy may be performed after
character (hematochezia vs. melena) of bleed- mechanical bowel preparation [3]. In this
ing is important, as is identifying associated instance, colonoscopy can be diagnostic (in
symptoms that help indicate the underlying over 75% of cases), as well as therapeutic [4].
pathology (e.g., weight loss and cancer) [1]. Endoscopic clipping, epinephrine injection,
B. For patients with massive LGIB, early resus- electrocautery, or a combination thereof can
citation with isotonic crystalloid and, if nec- be employed to address the source of bleed-
essary, blood products is critical prior to ing [3].
further diagnostic efforts. Repeat exams E. For patients who are hemodynamically stable
monitoring for the development or recurrence but who have ongoing bleeding, attempts at
of hypovolemic shock are necessary. localization should be made prior to further
Laboratory studies, including serial complete intervention. Depending upon the patient’s
blood counts and coagulation panels, can comorbidities (such as renal failure) and
help guide resuscitative efforts [2]. institutional availability, either CT angiogra-
C. A focused examination should be performed phy (CTA) or nuclear scintigraphy with
to rule out two common causes of GI bleed- 99m
Tc-labeled RBCs can be used. CTA
ing: anoscopy/proctoscopy to evaluate for requires higher rates of bleeding (>0.3 mL/
anorectal causes (5–20% of cases) and naso- min) to localize a source, but this study is able
gastric lavage to evaluate for an upper GI to localize the site of bleeding more effec-
tively than nuclear medicine studies, while
A. S. Kulaylat scintigraphy is able to detect lower rates of
Department of Surgery, Penn State Milton S. Hershey bleeding (>0.05 mL/min), and repeat scans
Medical Center, Hershey, PA, USA can be performed for up to 24 h if initial scans
D. B. Stewart Jr. (*) are negative. Scintigraphy is not precise
Department of Surgery, Banner University Medical enough to allow planning for a segmental
Center – Tucson, Tucson, AZ, USA colectomy [1].

206 A. S. Kulaylat and D. B. Stewart Jr.
F. For patients with negative CTA or repeatedly citative efforts, and for whom other sources
negative scintigraphy, supportive care should (upper GI and anorectal) have been ruled out,
be continued while continually reassessing emergent exploratory laparotomy should be
the patient for signs of rebleeding. For performed [1]. In the absence of an obvious
patients with a positive CTA or scintigraphy, small bowel source during exploration, such
super selective embolization with mesenteric as a Meckel’s diverticulum, a total abdominal
angiography may successfully address the colectomy with end ileostomy is the proce-
bleed, particularly for patients with an active dure of choice, as this surgery has the lowest
“blush” on scintigraphy or a short time inter- incidence of postoperative bleeding. For
val between scintigraphy/CTA and mesen- patients who rebleed after multiple therapeu-
teric angiography, which predicts the success tic attempts but who on the basis of angiogra-
of this intervention [5, 6]. phy have had the source of their bleeding
G. For patients who initially present with hemo- successfully localized, a segmental colec-
dynamic instability that is refractory to resus- tomy can be performed.
History and Physical Examination:

A Elicit onset, duration, frequency, and character of bleeding
Assess for signs of hemodynamic instability
Symptoms for 36 hours
B Resuscitation with IV fluids +/– blood products, obtain laboratory parameters
C Rule out upper GI source (nasogastric lavage) or anorectal source (anoscopy/proctoscopy)
Response to
resuscitation?
Not responding to
Self-limited bleeding, resuscitation,
hemodynamically stable: G
D Mechanical bowel prep
hemodynamically
Ongoing bleeding, unstable: exploratory
followed by colonoscopy laparotomy
hemodynamically stable:
CTA or nuclear
scintigraphy
Positive
scan?
Negative: Continued Positive: Consider

supportive care, F intervention with
assess for rebleeding mesenteric angiography
Algorithm 54.1
54 Management of Lower Gastrointestinal Bleeding 207
References acute lower intestinal bleeding. Clin Gastroenterol

Hepatol Off Clin Pract J Am Gastroenterol Assoc.
2010;8(4):333–43; quiz e344.
1. Steele SR, Hull T, Read TE, Saclarides TJ, Senagore
5. Koh FH, Soong J, Lieske B, Cheong WK, Tan
AJ, Whitelow CB, editors. The ASCRS textbook of
KK. Does the timing of an invasive mesenteric
colon and rectal surgery. 3rd ed. Arlington Heights:
angiography following a positive CT mesenteric
Springer; 2016.
angiography make a difference? Int J Color Dis.
2. Strate LL, Gralnek IM. Management of patients
2015;30(1):57–61.
with acute lower gastrointestinal bleeding. Am J
6. Ng DA, Opelka FG, Beck DE, et al. Predictive value
Gastroenterol. 2016;111(4):459–74.
of technetium Tc 99m-labeled red blood cell scin-
3. Pasha SF, Shergill A, Acosta RD, et al. The role of
tigraphy for positive angiogram in massive lower
endoscopy in the patient with lower GI bleeding.
gastrointestinal hemorrhage. Dis Colon Rectum.
Gastrointest Endosc. 2014;79(6):875–85.
1997;40(4):471–7.
4. Strate LL, Naumann CR. The role of colonoscopy
and radiological procedures in the management of
Management of Diverticulitis
55
Algorithmic Approach sion for intravenous antibiotics and bowel

rest is indicated [2].
A. Abdominal pain, usually centered in the
D.
Uncomplicated disease is recurrent in
lower abdomen, is a frequent symptom. 13–47% of patients [3]. In general, recurrent
Pneumaturia, fecaluria, or the passage of fla- episodes of disease tend to be of similar
tus or stool per vagina in patients who have severity to prior episodes. Therefore, the
undergone a hysterectomy is indicative of decision to offer surgery to these patients
colovesical and colovaginal fistulas, respec- should be tailored to a patient’s number of
tively [1]. On physical examination, patients episodes, their history of dietary modification
should be evaluated for lower abdominal ten- to prevent recurrent disease, and their opera-
derness and, in the acute setting, for signs of tive risk. Prior to elective resection, evalua-
peritonitis. tion of the large intestine with colonoscopy is
B. Especially for acute diverticulitis, laboratory advised no sooner than 6 weeks following an
evaluation should include a CBC, and CT acute episode, if no recent evaluation has
scanning is recommended to evaluate for been performed, in order to ensure the
pericolic fat stranding, abscess formation, absence of alternative diagnoses such as can-
and pneumoperitoneum [2]. cer or Crohn’s disease [1].
C. Uncomplicated disease represents the most E. Complicated disease, including the formation
common manifestation of diverticulitis and of an abscess (10–25%), a fistula (2%, most
can typically be managed with antibiotics commonly colovesicular), or a free perfora-
with Gram-negative and anaerobic coverage. tion resulting in diffuse peritonitis (1%),
In hemodynamically stable, immunocompe- occurs in a minority of patients (~1%) but is
tent and socially reliable patients who can associated with a high incidence of morbidity
tolerate a diet, outpatient treatment with oral and even mortality [1]. For disease compli-
antibiotics can be pursued; for others, admis- cated by an abscess 3 cm or larger, percutane-
ous drainage with antibiotic therapy is
A. S. Kulaylat recommended [2].
Department of Surgery, Penn State Milton S. Hershey F. While prior recommendations to provide an
Medical Center, Hershey, PA, USA elective resection after the first episode of
D. B. Stewart Jr. (*) complicated disease have been questioned,
Department of Surgery, Banner University Medical certain disease-related complications such as
Center – Tucson, Tucson, AZ, USA fistulas and strictures should prompt surgical

intervention. Elective resection should tion of a stapled rectosigmoid stump and an

include removing the entire sigmoid and rec- end descending colostomy) is the safest sur-
tosigmoid colon, creating a true colorectal gical option in the short term due to the
anastomosis as opposed to a colocolostomy. absence of an anastomosis. However, consid-
Failure to remove the rectosigmoid colon is ering the historically high complication rates
associated with a higher incidence of anasto- associated with a subsequent colostomy clo-
motic leaks and recurrent diverticulitis. If the sure, the option of an on-table colonic lavage
surgeon is well versed in advanced laparo- with the construction of a colorectal anasto-
scopic techniques, then a minimally invasive mosis, with a possible diverting loop ileos-
approach is preferable [4]. tomy, can be considered based on the patient’s
G. For patients with peritonitis due to free perfo- physiologic status as well as the degree of
ration, urgent sigmoid colectomy is recom- contamination [5]. Construction of an anasto-
mended. In many respects, a Hartmann’s mosis is generally contraindicated in the set-
procedure (sigmoidectomy with the construc- ting of widespread feculent contamination.
55 Management of Diverticulitis 211
History and physical examination:

LLQ pain, fevers, altered bowel habits, abdominal distension
A LLQ tenderness +/- peritoneal signs
Obtain laboratory studies and CT scan

B
Generalized
peritonitis on exam
or free
perforation on CT?
No Yes: Urgent
surgical G
intervention
Complicated
disease?
Yes: Initial
No: Medical medical
C E
management management
Colonoscopy in 6-8 weeks Colonoscopy in six to eight weeks (if

(if no recent endoscopic exam), and no recent endoscopic exam), and F
D
then consider dietary modification then definitive surgical resection
versus elective resection
Algorithm 55.1
References 3. Buchs NC, Mortensen NJ, Ris F, Morel P, Gervaz

P. Natural history of uncomplicated sigmoid divertic-
ulitis. World J Gastrointest Surg. 2015;7(11):313–8.
4. Bachmann K, Krause G, Rawnaq T, et al. Impact of
early or delayed elective resection in complicated diver-
ticulitis. World J Gastroenterol. 2011;17(48):5274–9.
Springer; 2016.
5. Salem L, Flum DR. Primary anastomosis or
2. Feingold D, Steele SR, Lee S, et al. Practice param-
Hartmann's procedure for patients with diverticular
eters for the treatment of sigmoid diverticulitis. Dis
peritonitis? A systematic review. Dis Colon Rectum.
Colon Rectum. 2014;57(3):284–94.
2004;47(11):1953–64.
Management of Large Bowel
Obstruction 56
Algorithmic Approach Initial radiographic evaluation with an acute

abdominal series can be obtained, in order to
A. Since large bowel obstructions (LBO) can
(1) evaluate for signs of perforation, (2)
result from a variety of both benign (e.g., assess the degree of colonic distention, and
diverticular disease, ischemic colitis) and (3) potentially identify an etiology, such as a
malignant (e.g., colorectal cancer, extrinsic volvulus [1].
compression from ovarian cancer) diseases, a C. In patients with systemic toxicity or signs of
detailed history is essential for determining free perforation, intravenous broad-spectrum
the diagnosis. Important factors to consider antibiotics and emergent exploratory laparot-
include the onset and duration of obstructive omy are indicated. If unresectable disease
symptoms, as well as any associated symp- (e.g., carcinomatosis) or disease that requires
toms. Patients will report complaints of initial medical treatment (e.g., neoadjuvant
abdominal pain and distention, as well as pro- therapy for obstructing rectal cancer) is
gressively worsening obstipation. encountered, then proximal diversion is an
Competency of the ileocecal valve can impact appropriate procedure. If resectable disease is
patient presentation: patients with competent found, resection of the diseased intestine is
valves are at risk for a closed-loop obstruc- indicated, along with careful inspection of the
tion and are less likely to have nausea/vomit- remaining large intestine for either ischemia
ing, which is commonly seen in large bowel or synchronous lesions.
obstructions [1]. D. In hemodynamically stable patients with a

B. A thorough physical exam evaluating for clear diagnosis, such as colonic volvulus (10–
signs of peritonitis and systemic toxicity 15% of LBO), acute colonic pseudoobstruc-
should be performed, in additional to obtain- tion, or foreign body impaction, further
ing laboratory studies to assess for electrolyte management should proceed according to the
derangements or signs of bowel ischemia. underlying etiology of bowel obstruction
[1–3].
A. S. Kulaylat E. In hemodynamically stable patients without
Department of Surgery, Penn State Milton S. Hershey signs of perforation, but for whom the diag-
Medical Center, Hershey, PA, USA nosis remains unclear, further imaging should
D. B. Stewart Jr. (*) be obtained. Either contrast enema (particu-
Department of Surgery, Banner University Medical larly for left-sided lesions) or CT scans can
Center – Tucson, Tucson, AZ, USA be helpful in determining the etiology of the

obstruction. Colonoscopy, preferably with surgical resection, with the possibility of

CO2 insufflation, can also be used to obtain a converting a more urgent surgery to an elec-
tissue diagnosis in patients with suspected tive procedure with a lower likelihood of
intraluminal disease, such as colorectal can- requiring a stoma [4]. However, stents are
cer (~50% of LBO) [1]. often less effective at relieving the initial
F. Further management of LBO in the none- obstruction (53% vs. 99%) and have high
mergent setting is dependent upon etiology. rates of reobstruction [5]. Since stents are
In general, the two main options are surgical safest when used as a bridge to elective sur-
(either resection or diversion) or endoscopic gery within several weeks, careful consider-
stenting, the latter of which can be used as a ation of the goals of care is necessary in
bridge to surgery or as definitive palliation. choosing how to relieve the patient’s
Stents offer lower initial morbidity than obstructive symptoms.
Detailed History:
A Abdominal pain and distention, obstipation, +/–nausea/vomiting
Thorough physical examination

B Obtain laboratory studies and abdominal plain films
Peritonitis?
Clear
diagnosis?
Hemodynamically
Hemodynamically
unstable or signs of
stable, no clear E
C Hemodynamically diagnosis
free perforation:
stable, clear
Exploratory surgery D diagnosis (e.g.
colonic volvulus,
foreign body, etc) Abdominal/pelvis CT
or contrast enema
Resectable
Definitive
disease?
management Consider definitive
depending upon
surgical resection or F
etiology
stenting, depending
upon etiology
Yes: Resect
with proximal No: Proximal
diversion diversion
Algorithm 56.1
56 Management of Large Bowel Obstruction 215
References 3. Chudzinski AP, Thompson EV, Ayscue JM. Acute

colonic pseudoobstruction. Clin Colon Rectal Surg.
2015;28(2):112–7.
4. van Hooft JE, van Halsema EE, Vanbiervliet G,
et al. Self-expandable metal stents for obstructing
colonic and extracolonic cancer: European Society of
Springer; 2016.
Gastrointestinal Endoscopy (ESGE) clinical guide-
2. Vogel JD, Feingold DL, Stewart DB, et al. Clinical
line. Endoscopy. 2014;46(11):990–1053.
practice guidelines for colon volvulus and acute
5. Sagar J. Colorectal stents for the management of
colonic pseudo-obstruction. Dis Colon Rectum.
malignant colonic obstructions. Cochrane Database
2016;59(7):589–600.
Syst Rev. 2011(11):CD007378.
Management of Colonic Pseudo-
Obstruction 57
Algorithmic Approach dilatation of the cecum (>12 cm), endoscopic

decompression or surgical intervention is
A. The primary goals in the evaluation of
required.
patients with suspected colonic pseudo- D. For patients without the above indications,
obstruction (CPO) are (1) to exclude other conservative interventions for 48–72 h are the
etiologies of obstruction, particularly recommended first treatment and result in
mechanical etiologies, and (2) to determine if resolution in 70–90% of cases [3, 4].
signs of perforation or ischemia are present. • Bowel rest, IV fluid resuscitation, electro-
Patients will report absent or decreased bowel lyte correction, and discontinuation of
function, with increasing abdominal disten- instigating agents (e.g., opioids) are main-
tion and discomfort [1]. Depending on the stays of treatment.
competency of the ileocecal valve, nausea • Serial abdominal exams and plain films to
and vomiting may not be present in the acute assess for worsening colonic dilation and/
setting. Physical examination should include or signs of perforation are indicated.
a digital anorectal examination to ensure the • Nasogastric and/or rectal tube decompres-
absence of an obstructing anorectal mass. sion can be considered depending on the
B. Laboratory studies are essential to identify patient’s symptoms and radiographic
contributory electrolyte abnormalities. findings.
Although colonic dilation can be seen on E. For patients who do not respond to these con-
plain films, either CT or contrast enemas servative measures, intravenous neostigmine
should be used to rule out a mechanical is the next therapeutic option for patients
obstruction in these patients [2]. without contraindications to its use [5].
C. If a patient presents with or develops signs of • Prior to administration, patients should be
perforation or ischemia, surgical intervention in a monitored setting with reversal agents
is mandated [3]. In the scenario of significant (atropine and glycopyrrolate) available in
case hypotension or bradycardia resulting
A. S. Kulaylat in hemodynamic instability, or severe
Department of Surgery, Penn State Milton S. Hershey abdominal cramps, develops.
Medical Center, Hershey, PA, USA • A dose of 2–2.5 mg IV administered over
D. B. Stewart Jr. (*) 2–5 min is effective in ~90% of cases [1].
Department of Surgery, Banner University Medical • If ineffective or bowel dilation recurs (17–
Center – Tucson, Tucson, AZ, USA 38% of cases) [6], a provider may administer

repeat doses after waiting for at least 80 min • Decompression to the hepatic flexure with
for some of the drugs to be eliminated. the placement of a colonic tube is the goal
F. Endoscopic decompression can be performed of the intervention, which can be repeated
in patients for whom the above measures are multiple times if dilation recurs.
ineffective and results in sustained resolution G. In cases that are refractory to the above

in 70–90% of cases [3]. measures, surgical intervention may be
• No bowel preps or laxatives should be given required, which can range from an ileoco-
prior to endoscopy, and sedation with ben- lectomy to a subtotal colectomy, depending
zodiazepines (not opioids) is preferred [6]. on the distribution of nonviable colon. In
• CO2 insufflation should be used when this setting, an ileostomy should be
available, given its faster resorption. constructed.
History and Physical Examination (Including a Digital Anorectal Exam):

A Increasing abdominal distention and pain, with absence of bowel movements
Distended abdomen +/– peritonitis
Obtain laboratory studies and plain films, +/– CT scan or

B contrast enema: rule out mechanical obstruction
Signs of
ischemia or perforation?
Yes: Surgical
No: Bowel rest, IVF, electrolyte correction, C
D discontinue narcotics, +/– NG or rectal tube intervention
E Trial of neostigmine
F Trial of endoscopic decompression
G Surgical intervention
Algorithm 57.1
57 Management of Colonic Pseudo-Obstruction 219
References colonic pseudo-obstruction. Dis Colon Rectum.

2016;59(7):589–600.
4. Trevisani GT, Hyman NH, Church JM. Neostigmine:
1. Chudzinski AP, Thompson EV, Ayscue JM. Acute
safe and effective treatment for acute colonic pseudo-
colonic pseudoobstruction. Clin Colon Rectal Surg.
obstruction. Dis Colon Rectum. 2000;43(5):599–603.
2015;28(2):112–7.
2. Harrison ME, Anderson MA, Appalaneni V, et al. The
role of endoscopy in the management of patients with
known and suspected colonic obstruction and pseudo-
Springer; 2016.
obstruction. Gastrointest Endosc. 2010;71(4):669–79.
6. Jain A, Vargas HD. Advances and challenges in the man-
3. Vogel JD, Feingold DL, Stewart DB, et al. Clinical
agement of acute colonic pseudo-obstruction (ogilvie
practice guidelines for colon volvulus and acute
syndrome). Clin Colon Rectal Surg. 2012;25(1):37–45.
Management of Colonic Volvulus
58
Algorithmic Approach cases) by revealing a “bent inner tube” or

“omega loop” pointing toward the right
A. In suspected colonic volvulus, a full history upper quadrant. In the case of a cecal vol-
and physical exam should be expeditiously vulus, a “coffee bean” pointing toward the
performed. Unlike malignant obstruction, left upper quadrant is sometimes evident
symptoms from a volvulus evolve more (20%) [2].
acutely. Patients may report abdominal pain, D. CT scans provide more details, and in most
increasing abdominal distention, and a lack cases of volvulus they demonstrate a mesen-
of bowel function; in the case of sigmoid vol- teric swirl, as well as distinguish the laterality
vulus, a history of being institutionalized, of the volvulus and assess for intestinal perfo-
either due to mental health issues or due to ration. Water-soluble contrast enemas may
senescence, is a frequent historical element. also be used to establish the diagnosis of a
While chronic constipation is a common fea- volvulus and to distinguish between sigmoid
ture of sigmoid volvulus, cecal volvulus can and cecal volvuluses, though this study is
be associated with prior surgeries which often difficult to obtain during off-hours, and
mobilized the cecum resulting in a pathologic it can delay intervention [2].
degree of mobility as a risk factor for volvu- E. For sigmoid volvulus, if peritonitis or perfo-
lus. On physical examination, a distended and ration is present, an emergent exploratory
tympanitic abdomen will be encountered, laparotomy with resection should be per-
with or without tenderness to palpation [1]. formed. Depending upon patient status and
B. Laboratory studies, including a CBC, electro- degree of peritoneal contamination, a
lytes, and a renal function panel, should be Hartmann’s procedure (sigmoidectomy with
obtained. the construction of an end descending colos-
C. If plain films are obtained, they may be diag- tomy and a stapled rectosigmoid stump) may
nostic for a sigmoid volvulus (50–70% of be indicated rather than a sigmoidectomy
with the construction of a colorectal anasto-
A. S. Kulaylat mosis [3].
Department of Surgery, Penn State Milton S. Hershey F. For sigmoid volvulus (50–75% of cases), in
Medical Center, Hershey, PA, USA the absence of peritonitis or radiographic evi-
D. B. Stewart Jr. (*) dence of perforation, detorsion with flexible
Department of Surgery, Banner University Medical endoscopy is the first-line treatment for sig-
Center – Tucson, Tucson, AZ, USA moid volvulus, and this intervention is

s uccessful in 60–95% of cases. If nonviable dant colon should be performed to reduce the
colon is encountered during this procedure, risk of recurrence [1].
then immediate surgical resection is indi- H. Because of low success rates, attempts at

cated, usually in the form of a Hartmann’s endoscopic decompression should be avoided
procedure [4]. in the setting of cecal volvulus, and instead
G. In the setting of successful endoscopic
immediate surgical resection should be per-
decompression of a volvulus, elective sig- formed. If no significant contamination or
moid resection prior to discharge is recom- gangrene is present, and if the patient is sta-
mended, given high recurrence rates ble, primary anastomosis can be performed.
(20–90%) and the significant incidence of Otherwise, resection with ileostomy and
complications associated with recurrent epi- mucus fistula, or the construction of an anas-
sodes. A full evaluation of the colon with tomosis with a diverting loop ileostomy,
colonoscopy should be performed prior to should be considered. Detorsion alone, ceco-
resection. Unless contraindicated by the pexy or cecostomy tube insertion should not
patient’s overall status, primary anastomosis be performed due to high recurrence and
should be attempted. Resection of all redun- mortality rates [5].
58 Management of Colonic Volvulus 223
History and physical examination:

A Abdominal pain, increasing distention, lack of bowel movement
Distended, tympanitic abdomen +/– peritonitis
B Obtain vital signs, laboratory studies, and plain films
Does plain film

C distinguish sigmoid from cecal
volvulus?
D No: obtain CT
SIGMOID or contrast CECAL
enema
Peritoneal Emergent right

H colectomy
signs?
F
No: endoscopic
intervention
Yes: immediate
E surgical Elective resection
intervention G prior to discharge
Algorithm 58.1
3. Akcan A, Akyildiz H, Artis T, Yilmaz N, Sozuer

References E. Feasibility of single-stage resection and primary
anastomosis in patients with acute noncomplicated
1. Vogel JD, Feingold DL, Stewart DB, et al. Clinical practice sigmoid volvulus. Am J Surg. 2007;193(4):421–6.
guidelines for colon volvulus and acute colonic pseudo- 4. Atamanalp SS. Treatment of sigmoid volvulus: a
obstruction. Dis Colon Rectum. 2016;59(7):589–600. single-center experience of 952 patients over 46.5
2. Steele SR, Hull T, Read TE, Saclarides TJ, Senagore years. Tech Coloproctol. 2013;17(5):561–9.
AJ, Whitelow CB, editors. The ASCRS textbook of 5. Kapadia MR. Volvulus of the small bowel and Colon.
Colon and Rectal surgery. 3rd ed. Arlington Heights: Clin Colon Rectal Surg. 2017;30(1):40–5.
Springer; 2016.
Appendicitis
59
Algorithmic Approach count (WBC). An elevated WBC >10 sup-

ports acute appendicitis; however, when it is
A. Acute appendicitis is suspected in patients very high (>15), this suggests complicated,
with acute abdominal pain that begins at the perforated, or gangrenous appendicitis. Vital
umbilicus and is associated with anorexia, signs should be obtained, and tachycardia
nausea, or vomiting. The pain migrates to the could represent dehydration that would
right lower quadrant (RLQ) as the inflamma- require rehydration in the ED.
tory process progresses to involve the overly- C. Patients with a history consistent with the
ing peritoneum. Duration of pain in acute above, a mild elevation of the WBC, fever,
uncomplicated appendicitis is less than 48 h, and rebound tenderness in the RLQ, are
whereas there is increased risk of perforated highly suspicious for acute uncomplicated
or complicated appendicitis when pain is appendicitis. Male patients do not require any
present for >48–72 h [1]. Physical exam is a imaging, and a laparoscopic appendectomy
reliable indicator of appendicitis when there should be performed [2]. Imaging, CT scan,
is rebound tenderness in the RLQ; other exam or ultrasound should be considered in female
signs include Rovsing sign and RLQ pain patients to rule out other sources of RLQ or
when the LLQ is palpated. Diffuse peritonitis pelvic pain, including pelvic inflammatory
should be evaluated for perforated appendici- disease, tuboovarian abscess, ovarian cyst or
tis or other causes of an acute abdomen. torsion, or other gynecologic causes.
B. In addition to the exam, laboratories should D. In patients with a suspicious history, but the
be obtained focusing on the white blood cell signs are not as clear, such as the WBC is less
than 15, they do not have fever, and the exam
is only mildly concerning for rebound in the
K. T. Crowell RLQ, imaging should be performed to delin-
Medical Center, Hershey, PA, USA eate the diagnosis [2]. A CT scan should be
obtained on adults as it can be cost saving
E. Messaris (*)
Department of Surgery, Penn State Milton S. Hershey compared to ultrasound, but ultrasound or
Medical Center, Hershey, PA, USA MRI should be obtained in the pediatric
Division of Colon and Rectal Surgery, Beth Israel population.
Medical Center, Harvard Medical Center, E. Imaging that shows an inflamed appendix
Boston, MA, USA (>10 mm), wall thickening, and periappendi-
e-mail: [email protected], ceal fluid with no sign of perforation or
[email protected]

226 K. T. Crowell and E. Messaris
gangrene is suggestive of acute uncompli- Nonoperative treatment is associated with a

cated appendicitis [3]. If imaging suggests lower morbidity profile than immediate
acute appendicitis, then the patient should appendectomy. An interval appendectomy
undergo laparoscopic appendectomy as lapa- 6–8 weeks later should be considered for pre-
roscopy decreases length of stay and postop- vention of recurrence (risk >10%) and identi-
erative pain. Complications of appendectomy fication of cancer especially in adults or
are surgical site infection, small bowel Crohn’s disease. Patients over the age of 40
obstruction, and abscess [4]. should undergo a screening colonoscopy
F. If the imaging shows a normal appendix or prior to an interval appendectomy [5].
the appendix is not visualized, then the patient H. Immediate surgery for complicated appendi-
can either be discharged to home or observed citis is indicated when free perforation is
based upon the clinical suspicion [2]. found on imaging, surgical exploration is
G. Imaging demonstrating a contained fluid col- warranted, and the use of laparoscopy is
lection indicating an abscess raises suspicion appropriate depending on the comfort of the
for perforation. A contained perforation with surgeon. Complicated appendicitis carries the
an abscess may be treated with a nonopera- same postoperative morbidity, although with
tive modality. Intravenous antibiotics, bowel increased risk of wound infection, 20%, and
rest, and CT-guided percutaneous drainage potential for ileocecal resection or right hemi-
are the hallmarks of nonoperative treatment. colectomy [6].
59 Appendicitis 227
History and Physical

A Acute onset, Migratory abdominal pain: periumbilical to RLQ,
Anorexia, Fever, Tenderness in RLQ
B Obtain vital signs, laboratory studies
High suspicion Lowersuspicion

C WBC > 15, Temp > 38.5°C, WBC < 14.9, afebrile, light
Rebound/Guarding in RLQ tenderness in RLQ
Laparoscopic Imaging:
appendectomy Ultrasound vs CT scan
Does imaging
Laparoscopic Yes suggest acute No Discharge vs.
E F
appendectomy uncomplicated observe
appendicitis?
CT Scan: Fluid collection in RLQ signifying a contained abscess
Start antibiotics, NPO, IVF, CT-guided drainage
Fever, tenderness, leukocytosis Sepsis, persistent fever, increasing

improve leukocytosis
Immediate H
G • Discharge home on antibiotics. Appendectomy
• Interval appendectomy is
recommended, preceded by
colonoscopy if >50
Algorithm 59.1
References appendix on treatment of patients and use of hospital

resources. N Engl J Med. 1998;338(3):141–6.
4. Long KH, Bannon MP, Zietlow SP, Helgeson ER,
1. Atema JJ, van Rossem CC, Leeuwenburgh MM,
Harmsen WS, Smith CD, et al. A prospective random-
Stoker J, Boermeester MA. Scoring system to distin-
ized comparison of laparoscopic appendectomy with
guish uncomplicated from complicated acute appen-
open appendectomy: clinical and economic analyses.
dicitis. Br J Surg. 2015;102(8):979–90.
Surgery. 2001;129(4):390–400.
2. Scott AJ, Mason SE, Arunakirinathan M, Reissis
5. Andersson RE, Petzold MG. Nonsurgical treatment of
Y, Kinross JM, Smith JJ. Risk stratification by the
appendiceal abscess or phlegmon: a systematic review
appendicitis inflammatory response score to guide
and meta-analysis. Ann Surg. 2007;246(5):741–8.
decision-making in patients with suspected appendi-
6. Mentula P, Sammalkorpi H, Leppaniemi
citis. Br J Surg. 2015;102(5):563–72.
A. Laparoscopic surgery or conservative treatment
3. Rao PM, Rhea JT, Novelline RA, Mostafavi AA,
for appendiceal abscess in adults? A randomized con-
McCabe CJ. Effect of computed tomography of the
trolled trial. Ann Surg. 2015;262(2):237–42.
Ulcerative Colitis
60
Algorithmic Approach fever, tachycardia, hypotension, abdominal

tenderness, or end organ failure and should
A. Patients with suspected ulcerative colitis
warrant an inpatient admission with support-
(UC) have a history of abdominal pain and ive care and a CT scan to evaluate the degree
bloody diarrhea, rectal urgency, or tenesmus. of colonic inflammation and colonic dilata-
There is a bimodal presentation in adolescent tion and the presence of perforation.
and middle-age men and women, more com- D. If CT scan shows colonic inflammation cor-
monly in white and black individuals [1]. relating with UC with no additional compli-
B. In the evaluation of the patient with UC, mul- cations, a flexible sigmoidoscopy can be
tiple serum markers should be obtained, performed to establish the diagnosis.
including CBC, electrolytes, renal function, Flexible sigmoidoscopy is safe in acute
albumin, ESR/CRP, and LFTs to evaluate leu- colonic inflammation, and biopsies help
kocytosis, anemia, electrolyte abnormality determine the extent of inflammation and
related to diarrhea, nutritional status, and the presence of CMV infection. IV steroids
inflammatory markers. Stool cultures are are started immediately, and if there is no
obtained to evaluate for secondary infection, improvement in 3 days, then IV cyclospo-
especially cytomegalovirus (CMV) and rine or infliximab should be initiated [2].
Clostridium difficile [2]. E. Patients who do not improve in 3–5 days
C. Based upon exam, vitals, and labs, patients after rescue therapy or worsen clinically
should be categorized according to signs of (fever, leukocytosis, megacolon >6 cm)
systemic toxicity. Toxicity signs include likely will not respond to medical therapy,
and total abdominal colectomy with end ile-
ostomy is indicated. Ileal pouch-anal anasto-
K. T. Crowell mosis (IPAA) is the recommended operation
Medical Center, Hershey, PA, USA for UC patients, either in a one-, two-, or
three-stage procedure depending on the
E. Messaris (*)
Department of Surgery, Penn State Milton S. Hershey severity of illness [3].
Medical Center, Hershey, PA, USA F. Patients who improve with IV steroids and
Division of Colon and Rectal Surgery, Beth Israel cyclosporine or infliximab can be fed orally,
Medical Center, Harvard Medical Center, can be transitioned to PO medications, and
Boston, MA, USA should continue on maintenance cyclosporine
e-mail: [email protected], or infliximab. Of the patients with acute
[email protected]

severe UC, around 86% do not require sur- seen in Crohn’s disease (CD), and if found
gery for UC in the following 1 year after dis- then UC should be ruled out. Biopsies are
charge [4]. obtained to verify histology, e.g., crypt dis-
G. Patients with acute severe colitis with signs of tortion, decreased crypt density, and trans-
fulminant colitis including perforation or mucosal inflammation. In patients with
colonic ischemia should undergo emergent severe colitis, flexible sigmoidoscopy is
colectomy. Unstable patients should undergo safer and preferred over colonoscopy [5].
subtotal colectomy with ileostomy, and after Medical management depends on the sever-
the patient is stable plans to undergo IPAA ity of illness. 5-ASA or mesalamine for
should be made [3]. mild disease, induction therapy can include
H. Stable patients should undergo elective
steroids and/or thiopurines. For more
colonoscopy with biopsy, as it is the diag- severe diseases, biologic therapy is indi-
nostic study of choice. Endoscopic features cated including anti-TNF agents or vedoli-
include vascular congestion, loss of vascu- zumab. Patients should be monitored for
lar pattern, erythema, mucosal friability, response to medical therapy and indica-
and ulceration which occur in a continuous tions for colectomy: medically refractory
manner from the rectum proximally in the disease, concern for carcinoma, perfora-
colon. The terminal ileum is only involved tion, toxic megacolon, and uncontrolled
in backwash ileitis. “Skip” lesions are only colonic hemorrhage [2].
60 Ulcerative Colitis 231

A
Chronic abdominal pain, bloody diarrhea, tenesmus
Vitals
B CBC, CMP, stool cultures, C diff assay
Systemic Yes
C CT scan of
toxicity? abdomen/pelvis
D G
No
Colitis, no perforation Perforation
H Colonoscopy with
biopsy
Flex sigmoidoscopy Emergent total
confirms UC abdominal
colectomy, end
Medical management: ileostomy
5-ASA/mesalamine
Steroids
IV steroids and rescue
Thiopurine
infliximab or CSA
Biologic therapy
F
Clinical Discharge home

Yes on steroids and
improvement
? continue TNF
antagonist tx
No
Emergent total
E
abdominal
colectomy, end
ileostomy
Algorithm 60.1
References proctocolectomy for ulcerative colitis: preoperative

status and long-term results. Inflamm Bowel Dis.
2007;13(10):1228–35.
1. Rao SS, Holdsworth CD, Read NW. Symptoms and
4. Aratari A, Papi C, Clemente V, Moretti A, Luchetti
stool patterns in patients with ulcerative colitis. Gut.
R, Koch M, et al. Colectomy rate in acute severe
1988;29(3):342–5.
ulcerative colitis in the infliximab era. Dig Liver Dis.
2. Kornbluth A, Sachar DB, Practice Parameters
2008;40(10):821–6.
Committee of the American College of Gastro
5. Dignass A, Eliakim R, Magro F, Maaser C, Chowers
enterology. Ulcerative colitis practice guidelines
Y, Geboes K, et al. Second European evidence-based
in adults: American College of Gastroenterology,
consensus on the diagnosis and management of ulcer-
Practice Parameters Committee. Am J Gastroenterol.
ative colitis part 1: definitions and diagnosis. J Crohns
2010;105(3):501–23; quiz 524.
Colitis. 2012;6(10):965–90.
3. Tariverdian M, Leowardi C, Hinz U, Welsch T,
Schmidt J, Kienle P. Quality of life after restorative
Crohn’s Colitis
61
Maria Michailidou and Evangelos Messaris
Algorithmic Approach has been associated with increased risk of

developing Crohn’s disease, as well as
A. Clinical manifestations vary based on the
increased severity, need for surgical interven-
behavior of the disease (nonstricturing and tion, and recurrence of the disease after sur-
nonpenetrating, stricturing or penetrating, gery [2].
associated perianal disease) [1]. In general, B. B. Diagnosis of Crohn’s colitis is based on a
patients present with crampy abdominal pain, combination of clinical, endoscopic, and
bloody diarrhea, fevers, weight loss, or pathologic findings as well as laboratory
obstructive symptoms. Extraintestinal mani- markers. Endoscopic features of Crohn’s
festations include pyoderma gangrenosum, colitis include segmental inflammation with
erythema nodosum apththous stomatitis, pri- skip lesions, apthoid ulcers, cobblestone
mary sclerosing cholangitis, and peripheral appearance of mucosa, and presence of fistu-
arthritis. Belonging to the Ashkenazi Jew las or strictures. In about 50% of patients, the
population, living in urban areas, and positive rectum is spared. Microscopically they pres-
family history of inflammatory bowel disease ent with focal transmural chronic inflamma-
(IBD) are all risk factors for developing the tion, lymphoid aggregates, and granulomas.
disease. The current theory of pathogenesis Presence of anti-Saccharomyces cerevisiae
suggests that an environmental trigger in a antibodies (ASCA), although not specific,
predisposed individual with an altered host along with absence of perinuclear antineutro-
defense leads to mucosal inflammation and phil cytoplasmic antibodies (P-ANCA) is
the development of IBD. Tobacco smoking highly suggestive of Crohn’s disease [3].
Nevertheless, 10–15% of patients are diag-
nosed with indeterminate colitis.
M. Michailidou C. Small bowel follow through, computed tomog-
Medical Center, Hershey, PA, USA raphy (CT), and magnetic resonance imaging
(MRI) enterography are useful adjuncts that
E. Messaris (*)
Department of Surgery, Penn State Milton S. Hershey should be used to assess the presence and
Medical Center, Hershey, PA, USA extent of associated small bowel disease.
Division of Colon and Rectal Surgery, Beth Israel D. Medical treatment remains the mainstay ther-
Medical Center, Harvard Medical Center, apy for Crohn’s colitis. High-dose systemic
Boston, MA, USA steroids or budesonide, an enteric coated
e-mail: [email protected], oral steroid, is an effective medication for
[email protected]

234 M. Michailidou and E. Messaris
induction of remission of disease. Azathioprine Patients with medically refractory toxic coli-
and 6-mercaptopurine are immunomodulators tis should undergo total abdominal colectomy
being used in moderate disease as monother- with end ileostomy [7].
apy or in conjunction with biologic agents to F. Patients with severe colitis typically present
achieve remission. Biologic agents, which with six or more bloody bowel movements
mainly act as monoclonal antibodies against per day, plus one systemic sign of toxicity
TNF-a, are highly effective in inducing and that includes anemia (<10.5 g/dL), ESR
maintaining remission in steroid-dependent, (>30 mm/h), fever (>37.5 °C), or tachycardia
steroid refractory, or fistulizing disease. Early (>90 beats per minute). Patients with total or
administration of biologic agents, often with segmental dilation of the colon with associ-
conjunction of immunomodulators, after ated systemic toxicity have toxic megacolon
diagnosis of Crohn’s disease is linked with [7]. Treatment involves intravenous hydra-
better outcomes [4]. In addition, they are tion and antibiotics with high-dose systemic
being used as an effective medical prophy- corticosteroids. CMV and Clostridium diffi-
laxis after surgery. cile colitis need to be ruled out since they can
E. Indications for surgery include failure of or present parallel to severe colitis. Although
intolerance to medical therapy, chronic most data originate from fulminant ulcer-
obstruction, fistula formation, local perfora- ative colitis cases [8], patients who fail to
tion with abscess formation, toxic colitis, fail- respond to systemic steroids within 2–3 days
ure to thrive, and presence of dysplasia or of initiation should be offered either rescue
malignancy [5]. Patients with rectal involve- therapy with infliximab (5 mg/kg) or cyclo-
ment benefit from total proctocolectomy with sporine (2–4 mg/kg) [9] or total abdominal
end ileostomy. Patients with single segment colectomy with end ileostomy. Patients who
or multiple segment colonic disease and fail medical management with rescue ther-
spared rectum should undergo segmental col- apy, typically within 5–7 days, should be
ectomy and total abdominal colectomy with offered total abdominal colectomy with end
ileorectal anastomosis, respectively [6]. ileostomy.
61 Crohn’s Colitis 235
A Clinical presentation suspicious for inflammatory bowel disease

(abdominal pain, diarrhea, weight loss, obstructive symptoms)
B Rule out other causes of colitis (ischemic, infectious)

C. difficile toxin, CMV, stool cultures for ova and parasites
CBC, ESR, CRP, P-ANCA, ASCA, rule out concomitant celiac disease
CT A/P
Colonoscopy with biopsies and intubation of terminal ileum
Severe colitis (six or more bloody stools per day plus one sign of systemic
toxicity) which includes anemia (<10.5 g/dL), ESR (>30 mm/h), fever (>37.5°C),
and tachycardia (>90 beats per minute) +/-megacolon
Yes No
F NPO, IV fluids, IV antibiotics Colonoscopy with intubation of C

Rule out CMV/C.diff colitis terminal ileum
IV steroids +/-rescue anti-TNF-a CT or MRI enterography
Improvement Diagnosis consistent

No improvement
with Crohn’s Colitis
E Steroids D
Total abdominal colectomy
Immunomodulators
with end ileostomy
Biologic agents
• Failed medical management • Multiple areas of

• Failure to thrive low-grade dysplasia
• Stricture • High-grade dysplasia
• Perforation • Colon adenocarcinoma
E Total proctocolectomy with end

ileostomy
– Single segment colonic disease

Segmental colectomy
– Multiple segment colonic disease
with no rectal involvement
TAC with ileorectal anastomosis
– Colonic disease with rectal
involvement
Total proctocolectomy with end
ileostomy
Algorithm 61.1
236 M. Michailidou and E. Messaris
References (REACT): a cluster randomised controlled trial.

Lancet. 2015;386(10006):1825–34.
5. Toh JW, Stewart P, Rickard MJ, Leong R, Wang N,
1. Silverberg MS, Satsangi J, Ahmad T, Arnott ID,
Young CJ. Indications and surgical options for small
Bernstein CN, Brant SR, et al. Toward an integrated
bowel, large bowel and perianal Crohn’s disease.
clinical, molecular and serological classification of
World J Gastroenterol. 2016;22(40):8892–904.
inflammatory bowel disease: report of a Working
6. Strong S, Steele SR, Boutrous M, Bordineau L, Chun
Party of the 2005 Montreal World Congress of
J, Stewart DB, et al. Clinical practice guideline for the
Gastroenterology. Can J Gastroenterol = Journal cana-
surgical management of Crohn’s disease. Dis Colon
dien de gastroenterologie. 2005;19(Suppl A):5a–36a.
Rectum. 2015;58(11):1021–36.
2. Parkes GC, Whelan K, Lindsay JO. Smoking in
7. Strong SA. Management of acute colitis and
inflammatory bowel disease: impact on disease course
toxic megacolon. Clin Colon Rectal Surg.
and insights into the aetiology of its effect. J Crohns
2010;23(4):274–84.
Colitis. 2014;8(8):717–25.
8. Andrew RE, Messaris E. Update on medical and
3. Smids C, Horjus Talabur Horje CS, Groenen MJM,
surgical options for patients with acute severe ulcer-
van Koolwijk EHM, Wahab PJ, van Lochem EG. The
ative colitis: what is new? World J Gastrointest Surg.
value of serum antibodies in differentiating inflam-
2016;8(9):598–605.
matory bowel disease, predicting disease activity and
9. Narula N, Marshall JK, Colombel JF, Leontiadis GI,
disease course in the newly diagnosed patient. Scand
Williams JG, Muqtadir Z, et al. Systematic review
J Gastroenterol. 2017;52(10):1104–12.
and meta-analysis: infliximab or cyclosporine as
4. Khanna R, Bressler B, Levesque BG, Zou G, Stitt
rescue therapy in patients with severe ulcerative
LW, Greenberg GR, et al. Early combined immuno-
colitis refractory to steroids. Am J Gastroenterol.
suppression for the management of Crohn’s disease
2016;111(4):477–91.
Ischemic Colitis
62
William Sangster and Evangelos Messaris
Algorithmic Approach 2. Inflammatory colitis such as Crohn’s dis-

ease or ulcerative colitis
A. Ischemic colitis can develop secondary to: 3. Traumatic etiology other than ischemia
1. Acute arterial occlusion (embolic or
D. Laboratory assessment, though not diagnos-
thrombotic) tic, may aid in determining severity. Increased
2. Venous thrombosis white blood cell count, serum lactate, lactate
3. Hypoperfusion of the colonic vasculature dehydrogenase (LDH), creatine phosphoki-
causing nonocclusive ischemia nase (CPK), or blood urea nitrogen (BUN)
B. Typically patients with ischemic colitis will may indicate advanced tissue damage [2].
present with sudden, crampy abdominal pain Stool culture, ova and parasite testing, and
with an urgent desire to defecate and subse- Clostridium difficile toxin assay should be
quent passage of bloody diarrhea within 24 h. ordered to rule out infectious etiologies of
Abdominal tenderness is usually mild to bloody diarrhea.
moderate with focal peritonitis which is
E. Computed tomography (CT) scan of the
located in the distribution of the segment of abdomen with intravenous contrast is the
colon involved [1]. study of choice to initially diagnose ischemic
C. The differential diagnosis for ischemic colitis colitis. CT findings are nonspecific and in
includes: early cases of ischemic colitis can be normal.
1.
Infectious etiologies such as Typical findings of ischemic colitis include
Cytomegalovirus or Clostridium difficile abnormal wall enhancement, wall thickening,
colitis, Shigella, or Salmonella and mesenteric fat stranding [3]. Pneumatosis
coli, gas in the mesenteric or portal veins, or
pneumoperitoneum is also not specific to
W. Sangster colonic ischemia but may be seen in more
Medical Center, Hershey, PA, USA advanced stages and indicate colonic necro-
sis/perforation [4].
E. Messaris (*)
Department of Surgery, Penn State Milton S. Hershey F. If the patient is not improving or the diagno-
Medical Center, Hershey, PA, USA sis is in question, colonoscopy can be per-
Division of Colon and Rectal Surgery, Beth Israel formed as the diagnostic test of choice and
Medical Center, Harvard Medical Center, should be completed within 48 h of symptom
Boston, MA, USA onset. Although nonspecific, features on
e-mail: [email protected], colonoscopy suggestive of ischemic colitis
[email protected]

238 W. Sangster and E. Messaris
include segmental erythema, edema, mucosal cal and radiologic resolution of symptoms within
friability, ulcerations, and blue-black nodules 1–2 weeks [7].
with dark-dusky backgrounds suggestive of For embolic or thrombotic colonic ischemia,
gangrene [5]. anticoagulant therapy should be instituted. Unlike
G. Treatment of colonic ischemia depends upon in cases of mesenteric ischemia, embolectomy,
its etiology and severity. Supportive care with bypass graft, or endarterectomy is generally not
bowel rest and observation is appropriate pro- performed in cases of primary colonic ischemia.
vided there is no evidence of colonic perfora- If findings of severe colonic ischemia exist, such
tion, necrosis, or gangrene. Intravenous fluids as diffuse peritoneal signs on physical exami-
should be given to ensure adequate colonic nation, pneumatosis or pneumoperitoneum
perfusion. Though there is no strong evidence on radiographic examination, or gangrene on
supporting the routine use of antibiotics for colonoscopic examination, surgical explora-
the treatment of all patients with colonic isch- tion is indicated. The specific operation depends
emia, current guidelines suggest starting upon the location of the affected colon and may
empiric broad-spectrum antibiotics for most require a segmental colectomy or total colec-
patients with colonic ischemia, except those tomy. In cases where a segmental resection is
with mild disease [6]. performed without the creation of a diverting
ostomy, a “second-look” operation within 12
After instituting supportive care, most patients to 24 h may be appropriate to assess the viabil-
with nonocclusive colonic ischemia will improve ity of the remaining colon and integrity of any
within one or two days and have complete clini- anastomoses.
62 Ischemic Colitis 239
Clinical presentation concerning for ischemic colitis? (acute abdominal

pain with hematochezia)
Evidence of diffuse (nonfocal) peritonitis on physical examination?
No Yes
Perform laboratory assessment (white blood

cell count, lactate, LDH, CPK, BUN, stool
cultures)
Obtain CT scan with IV contrast (assess for

abnormal wall enhancement, wall thickening,
mesenteric fat stranding, pneumatosis coli,
mesenteric gas or pneumoperitoneum)
Evidence of severe colonic ischemia

or colonic perforation?
(WBC>15,000/mm3, BUN>20
mg/dL, LDH>350 units/L,
pneumatosis coli, pneumoperitoneum)
No Yes
1.Supportive care with Consider surgical exploration

IV fluids and IV
antibiotics and reassess
2. Consider
colonoscopic evaluation
within 48 hours if
symptoms persist to
confirm diagnosis
Stricture present
If patient develops chronic symptomatology,

obtain water-soluble enema study or
endoscopic evaluation to assess for stricture
No stricture present
Observe
Algorithm 62.1
240 W. Sangster and E. Messaris
References findings of pneumatosis and portomesenteric venous

gas in acute bowel ischemia. World J Gastroenterol.
2013;19(39):6579–84.
1. Feuerstadt P, Brandt LJ. Colon ischemia: recent
5. Zou X, Cao J, Yao Y, Liu W, Chen L. Endoscopic
insights and advances. Curr Gastroenterol Rep.
findings and clinicopathologic characteristics of
2010;12(5):383–90.
ischemic colitis: a report of 85 cases. Dig Dis Sci.
2. Mosele M, Cardin F, Inelmen EM, Coin A,
2009;54(9):2009–15.
Perissinotto E, Sergi G, et al. Ischemic colitis in
6. Brandt LJ, Feuerstadt P, Longstreth GF, Boley
the elderly: predictors of the disease and prognostic
SJ. ACG clinical guideline: epidemiology, risk fac-
factors to negative outcome. Scand J Gastroenterol.
tors, patterns of presentation, diagnosis, and manage-
2010;45(4):428–33.
ment of colon ischemia (CI). Am J Gastroenterol.
3. Cruz C, Abujudeh HH, Nazarian RM, Thrall JH. Ischemic
2015;110(1):18–44.
colitis: spectrum of CT findings, sites of involvement
7. O'Neill S, Yalamarthi S. Systematic review of
and severity. Emerg Radiol. 2015;22(4):357–65.
the management of ischaemic colitis. Color Dis.
4. Milone M, Di Minno MN, Musella M, Maietta P,
2012;14(11):e751–63.
Iaccarino V, Barone G, et al. Computed tomography
Clostridium difficile Colitis
63
Algorithmic Approach icity, hypotension, oliguria, tachycardia, or

perforation [3]. A plain abdominal X-ray may
A. In the setting of new onset diarrhea, history be obtained to evaluate for toxic megacolon.
and physical examination are the first step With a concerning physical exam, a CT scan
with specific inquiry about risk factors for C. can assist in determining the extent of colonic
difficile colitis. Usually, the diarrhea is inflammation and can further characterize
watery. The strongest risk factor is recent signs of perforation or impending perforation
antibiotic use, and patients who are immuno- with pneumatosis.
suppressed or diagnosed with inflammatory E. If fulminant disease or perforation is identi-
bowel disease are at higher risk [1]. Physical fied, total abdominal colectomy with end ile-
examination should evaluate for abdominal ostomy and a stapled rectal stump is the safest
distention and peritoneal signs. surgical option [4].
B. A diarrheal specimen should be evaluated for F. Without peritoneal signs or perforation, antibi-
C. difficile toxin [2]. otic therapy in addition to supportive care is
C. With a positive assay, the severity of colitis is indicated. The antibiotic regimen chosen is
determined next. Vital signs and laboratory based upon disease severity as listed below [5]:
studies including complete blood count (a) Mild disease (WBC <15,000 and creatinine
(CBC), electrolytes, renal function, and albu- (Cr) less than 1.5 times baseline) is treated
min should be obtained. with oral or IV antibiotic therapy with met-
D. Patients should be evaluated for fulminant C. ronidazole 500 mg tid for 10–14 days.
difficile colitis, which includes systemic tox- (b) Severe disease (WBC >15,000 and/or
Cr >1.5 times baseline Cr) is treated with
125 mg vancomycin PO qid for 10–14 days.
K. T. Crowell (c) Severe-complicated disease is character-
Medical Center, Hershey, PA, USA ized by additional complicating factors,
including ileus, shock requiring vaso-
E. Messaris (*)
Department of Surgery, Penn State Milton S. Hershey pressors, and megacolon, or worsening
Medical Center, Hershey, PA, USA symptoms or lack of improvement after
Division of Colon and Rectal Surgery, Beth Israel 5 days of antibiotic treatment. CT scans
Medical Center, Harvard Medical Center, of the abdomen/pelvis should be
Boston, MA, USA obtained. Treatment: IV metronidazole
e-mail: [email protected], 500 mg tid, PO vancomycin 125 mg qid,
[email protected]

and if ileus: add vancomycin enema apy should be evaluated for surgical inter-
500 mg qid. vention. Patients with refractory or recurrent
G. Continue to monitor patient for signs of C. disease should be considered for fecal
difficile colitis. Patients who clinically microbiota transplant prior to surgical inter-
worsen or do not respond to medical ther- vention [6].
History:
A Duration of diarrhea, nausea, vomiting, abdominal pain
Recent antibiotic use, IBD, immunosuppression
Positive
B
Clostridium
difficile toxin
assay?
C Physical exam, vital signs, laboratories
Fulminant C.
D difficile colitis or
colonic G
perforation?
Continue
to
Yes No reassess
Initiate antibiotic
Immediate F
E therapy
surgical
intervention
Mild: Severe: Severe-
metronidazole vancomycin PO complicated:
PO vanco + IV
metro, ± vanco
enema
Algorithm 63.1
4. Kaiser AM, Hogen R, Bordeianou L, Alavi K, Wise

References PE, Sudan R, et al. Clostridium difficile infection
from a surgical perspective. J Gastrointest Surg.
1. Ananthakrishnan AN. Detecting and treating 2015;19(7):1363–77.
Clostridium difficile infections in patients with 5. Katzman M. Antibiotic therapy for Clostridium
inflammatory bowel disease. Gastroenterol Clin N difficile infection. Semin Colon Rectal Surg.
Am. 2012;41(2):339–53. 2014;25(3):143–9.
2. Surawicz CM, Brandt LJ, Binion DG, 6. Lee CH, Steiner T, Petrof EO, Smieja M, Roscoe
Ananthakrishnan AN, Curry SR, Gilligan PH, et al. D, Nematallah A, et al. Frozen vs fresh fecal micro-
Guidelines for diagnosis, treatment, and prevention of biota transplantation and clinical resolution of
Clostridium difficile infections. Am J Gastroenterol. diarrhea in patients with recurrent Clostridium dif-
2013;108(4):478–98. quiz 99 ficile infection: a randomized clinical trial. JAMA.
3. Butala P, Divino CM. Surgical aspects of ful- 2016;315(2):142–9.
minant Clostridium difficile colitis. Am J Surg.
2010;200(1):131–5.
Hereditary Colorectal Cancer
Syndromes 64
Algorithmic Approach The surgeon typically encounters two clinical

situations which are outlined in this chapter’s
Approximately 30% of colorectal cancer cases are flow charts: [1] management of a patient or kin-
associated with a family history of colorectal pol- dred with recently diagnosed colorectal cancer
yps or cancer, but only 3–5% of cases are associ- with suspicion of Lynch syndrome and [2] man-
ated with a specifically identifiable inheritable agement of a patient with personal or family his-
colorectal cancer syndrome. The most commonly tory of significant colonic polyposis.
identifiable cause of inheritable colorectal cancer is
Lynch syndrome, characterized by a specific muta- A. A personal or family history of young (< age
tion in one of the DNA mismatch repair (MMR) or 50) colorectal, endometrial, or Lynch-
EPCAM genes. Although the terms Lynch syn- associated cancers should trigger suspicion of
drome and hereditary nonpolyposis colorectal can- Lynch syndrome. A review of systems may
cer (HNPCC) are often used interchangeably, the reveal symptoms such as gastritis or abnor-
term Lynch syndrome refers to patients with a spe- mal vaginal bleeding that might provide clues
cifically identifiable germline MMR defect, to concurrent malignancies in a patient who
whereas HNPCC refers to a patient or kindred who presents with a known diagnosis of colon
meets the Amsterdam criteria (Table 64.1). cancer. Additionally, thorough personal and
Hereditary polyposis syndromes are a group family histories must be obtained evaluating
of hereditary disorders characterized by a pleth- for colorectal cancers, polyps, and Lynch-
ora of polyps throughout the gastrointestinal tract associated cancers (endometrial, gastric,
that predispose the patient to cancer. It is impor- ovarian, small bowel, and urinary tract can-
tant to distinguish Lynch syndrome from polypo- cers). The surgeon should be familiar with the
sis syndromes, as the former typically causes Amsterdam criteria to assess the suspected
isolated cancers with minimal adenomatous kindred’s risk for hereditary nonpolyposis
polyp burden, rather than extensive polyposis. colorectal cancer (Table 64.1).
B. Recent recommendations encourage routine
testing of all colorectal cancers for MMR
E. Huang
Department of Surgery, University of Chicago,
gene defects. Some institutions may limit
Chicago, IL, USA tumor MMR testing to patients using the
M. F. McGee (*)
revised Bethesda criteria (see Table 64.2).
Department of Surgery, Northwestern Memorial Most commonly, immunohistochemistry
Hospital, Chicago, IL, USA (IHC) for the four most common defective

244 E. Huang and M. F. McGee
Table 64.1 Amsterdam II criteria for hereditary nonpolyposis colorectal cancer [1]
1 At least three family members affected, one of whom is a first-degree relative of the other two, with HNPCC-
related cancers (colorectal, endometrial, small bowel, ureter, renal pelvis)
2 Two successively related generations
3 At least one of the HNPCC-related cancers diagnosed before age 50
4 Familial adenomatous polyposis excluded
Table 64.2 Bethesda guidelines to guide tumor testing for MMR gene defects [2]
1 Colorectal cancer diagnosed in patient under 50 years of age
2 Synchronous or metachronous colorectal cancer or other HNPCC-associated tumors, regardless of age
3 Colorectal cancer with high levels of microsatellite instability (MSI-H) histology in a patient younger than
60 years old
4 Colorectal cancer in one or more first-degree relatives with an HNPCC-related tumor, with one of the cancers
diagnosed under 50 years of age
5 Colorectal cancer diagnosed in two or more first- or second-degree relatives with HNPCC-related tumors,
regardless of age
MMR genes (MLH1, MSH2, MSH6, PMS2) have “Lynch-like syndrome” arising from a
is performed on tumor biopsies, although somatic (i.e., not germline) defect of the
some institutions additionally test for the MMR genes. There is no consensus on man-
EPCAM gene as well. It should be noted that agement and surveillance of Lynch-like
nearly all testing can be done with endoscopic patients; however, many advocate total colec-
biopsies alone. tomy in lieu of segmental colectomy in
C. Patients with abnormal tumor MLH1 testing appropriate patients. For this reason, germ-
merit further tumor testing. The most com- line testing typically should not delay surgi-
mon cause of abnormal MLH1 testing is cal therapy for recently diagnosed Lynch-like
somatic promoter methylation caused by a patients, since confirmation of a germline
BRAF gene defect, a condition which is typi- MMR defect is more pertinent to the kindred
cally sporadic and not inheritable. Any patient than to the patient in question. Patients who
with a defective or missing MLH1 gene screen positively for Amsterdam II criteria
should undergo reflexive BRAF gene testing and fail to possess a germline MMR defect
of the tumor biopsy. If a mutated BRAF gene are considered to have familial colorectal
is found, the cancer is considered to be spo- cancer type X, which is an entity distinct
radic (not Lynch) and treated accordingly. If from Lynch syndrome. There is no consensus
BRAF testing is normal (i.e., wild type), then on management of colorectal cancer type X
the MLH1 defect is considered to arise from patients; however, many advocate total colec-
Lynch or Lynch-like syndrome and should be tomy in appropriate patients and intensive
further evaluated with germline testing. postoperative surveillance programs.
D. MLH1 and PMS2 are often coexpressed, so an
MLH1 defect may also cause a PMS2 defect. Polyposis disorders are characterized by a
A purely isolated PMS2 defect is likely to be known germline mutation, and new clinical data
related to Lynch or Lynch-like syndrome. on the genetics of polyposis syndromes continue
E. Patients with other MMR defects not attrib- to arise with ongoing research. Whether establish-
uted to a BRAF mutation are likely to have ing a new diagnosis, performing surgery for cancer
Lynch or Lynch-like syndrome and merit or prophylaxis, or counseling members from a pol-
confirmation through germline (blood) test- yposis kindred, the surgeon must be aware of pol-
ing with a genetic counselor. Patients with a yposis syndromes and their workup, diagnosis,
tumor MMR defect who fail to demonstrate a and treatments. Table 64.3 details the heritability
germline genetic defect are considered to and characteristics of polyposis syndromes.
64 Hereditary Colorectal Cancer Syndromes 245
F. The most common presentations to the surgeon I. Depending on the specific syndrome, surveil-
in the context of hereditary polyposis syn- lance of the colon, the entire GI tract, or the GI
dromes will be (1) the patient with a new colo- tract and other organs may be warranted.
noscopic finding of polyposis or diagnosis of Recommendations for modalities and inter-
colorectal cancer who requires workup for a vals are outlined in Table 64.3 by syndrome
hereditary syndrome and (2) the patient with a [5–16]. For familial adenomatous polyposis
known hereditary syndrome presenting for risk (FAP), recommendations are generally con-
reduction surgery or a new diagnosis of cancer. current between the American College of
G. The first encounter should begin with a detailed Gastroenterology (ACG), American Society
and tailored history and physical exam. The of Gastrointestinal Endoscopists (ASGE), and
personal history focuses on (gastrointestinal) various European guidelines [5–9]. Juvenile
GI symptoms and personal history of cancer. polyposis syndrome is rare and thus without a
The family history must be taken in an orga- large burden of collective experience to guide
nized fashion and include any relatives (focus- management [8]. In addition to the modalities
ing on first- or second-degree relatives) with mentioned, capsule endoscopy, push enteros-
cancer, the type and location of the cancer copy, and magnetic resonance imaging (MRI)
(colorectal vs. extraintestinal), and age at diag- enterography are also used as screening
nosis. Using a pedigree to organize this infor- modalities for selected FAP patients at higher
mation can be helpful. Furthermore, because risk of developing small bowel adenomas
phenotypes for the various polyposis and non- (those with duodenal adenomas) and patients
polyposis syndromes can overlap, the clinician with Peutz-Jeghers syndrome [17].
should be alert and wary of any distinguishing Recommendations for surveillance in Peutz-
traits. The physical exam includes a complete Jeghers syndrome are taken from the work of
evaluation for surgical fitness and thorough a Dutch multidisciplinary group and concur
abdominal evaluation, as usual, but here we closely with those from other European and
point out a number of important and unusual American societies.
findings that are characteristic of polyposis J. Recommendations for segmental, total
syndromes. A thorough clinical exam may abdominal colectomy, and proctocolectomy
detect supernumerary teeth, epidermoid cysts, vary depending on the specific polyposis syn-
thyroid nodules, and desmoid tumors, for drome. In classic FAP, where the colorectal
instance, which are characteristic of familial cancer risk reaches a lifetime risk of 100% by
adenomatous polyposis (FAP) [3]. around age 40, recommendations for procto-
H. Patients who present with a new finding of colectomy are very strong, although proce-
polyposis on colonoscopy or who raise clini- dures can be staged to be tailored to patients’
cal suspicion of a hereditary polyposis syn- individual needs, such as fecundity preserva-
drome (e.g., the young patient with newly tion or rectal polyp burden. In Peutz-Jeghers
diagnosed colon cancer with a duodenal ade- syndrome, recommendations are targeted
noma) should be referred for genetic counsel- toward symptomatic and nonmalignant com-
ing and testing. Apart from determining the plications of the hamartomatous polyps,
risk for cancer for an individual patient, including intussusception and bleeding. The
genetic testing has significant implications exact mechanism of carcinogenesis in Peutz-
for the entire family and may inform subse- Jeghers syndrome is yet unknown, so surveil-
quent decisions about surveillance or risk lance aims to detect malignancy early, with
reduction, as most of the hereditary polyposis polypectomy having the potential benefit of
syndromes are autosomal dominant. Genetic removing premalignant lesions. Regardless
counseling guides patients through the often of treatment, the cycle of surveillance and
complex decisions about treatment and pre- appropriate treatment continues throughout
vention that arise from a new genetic diagno- the rest of life in these patients and begins
sis and can adjust misperceptions [4]. anew with subsequent generations.
Table 64.3 Hereditary polyposis syndromes, their characteristics, and recommended surveillance and treatment regimens [5–16]
246
Approximate
Polyp type Syndrome Genes Inheritance Clinical findings risk of CRC Recommended surveillance Treatment
Adenoma Familial APC Autosomal Profuse: over 1000 polyps Classic: Annual flexible sigmoidoscopy starting from Total proctocolectomy
adenomatous dominant Classic: hundreds of polyps 100% puberty, with colonoscopy if high risk/numerous by age 25 (usually late
polyposis Attenuated: fewer than 100 Profuse: polyps found teens); eliminates
(FAP) polyps 100% Annual proctoscopy for patients with ileorectal cancer risk
Duodenal adenomas and Attenuated: anastomosis Total colectomy with
carcinomas, gastric fundic gland 70–80% Upper endoscopy every 3 years from age 30 ileorectal anastomosis
polyps; desmoid tumors, Screening can start slightly later/be spaced to every is an option for
epidermoid cysts, supernumerary 2 years for attenuated FAP individuals with few
teeth, osteomas polyps in the rectum
MUTYH- MYH Autosomal 0–1000 adenomas, CRC <50 years; 75% Colonoscopy beginning at age 25–30, and repeated Endoscopic
associated recessive gastric fundic gland polyps, every 3–5 years if no neoplasia found; repeated polypectomy where
polyposis duodenal adenomas, and every 1–2 years if polyps found, depending on possible. Surgery
(MAP) carcinomas histology indicated for
EGD to evaluate for duodenal adenomatous colorectal cancer,
neoplasia beginning at age 30, and repeated every high-grade dysplasia
3–5 years if exam is normal not amenable to
Annual thyroid ultrasound beginning at age 25–30 endoscopic
polypectomy, or high
polyp burden. Surgery
should be with total
colectomy (colon
cancer) and ileorectal
anastomosis, or total
proctocolectomy
(rectal cancer), as the
entire colon is at risk
E. Huang and M. F. McGee
Hamartoma Juvenile BMPR1A Autosomal Working definition by Jass et al. 40% EGD and colonoscopy, starting in the later teen No strong evidence
polyposis SMAD4, dominant [13]: years, every years regarding the role of
syndrome heterogeneous At least 5 juvenile polyps preventative
Juvenile polyps throughout GI proctocolectomy;
tract however, the decision
Any number of juvenile polyp should be based on
with JPS family history number and size of
Typically, 50–200 polyps; 20–50% polyps
have JPS family history; presenting
by age 20 with rectal bleeding
Peutz-Jeghers STK11 Autosomal Orocutaneous pigmentation; 40% Annual physical exam, hemoglobin, starting at age Polypectomy for
syndrome dominant family history of PJP; cancer of 10 polyps over 1–1.5 cm
small bowel, colon, stomach, Video capsule endoscopy and/or MRI enterography both to prevent
pancreas, breast, ovary, testis every 2–3 years starting at age 10 nonmalignant
Upper endoscopy every 2–5 years starting at age 20 complications such as
Colonoscopy every 2–5 years starting at age 25–30 intussusception or
Annual breast exam and mammography/breast MRI, bleeding, and to
64 Hereditary Colorectal Cancer Syndromes
starting at age 25 (mammography at age 30) remove possible

Annual pelvic exam, Pap smear, transvaginal precursor to
ultrasonography, and CA-125, starting at age 25–30 malignancy
Treatment of
malignancy as
indicated
PTEN PTEN Autosomal Colorectal adenomas, lipomas, 10% As advocated by NCCN 2007 [14]: Colectomy or
hamartoma dominant fibromas, ganglioneuromas, (Cowden Annual or biannual colonoscopy starting at age 35 proctocolectomy
tumor juvenile hamartomas; colorectal syndrome); Breast self exam starting at age 18 based on polyp
syndrome cancer; macrocephaly, most Annual clinical breast examinations starting at age burden, if endoscopic
trichilemmomas malignant 25 (or 5–10 years before earliest known breast management
potential is cancer in the family) inadequate. No good
extracolonic Annual mammography and breast MRI starting at estimate of colorectal
age 35 (or 5–10 years before earliest known breast cancer risk given rarity
cancer in the family) of this syndrome
Blind endometrial biopsy
Annual physical exam with focus on breast and
thyroid starting at age 18 (or 5 years before
youngest age of cancer diagnosis in the family)
Baseline thyroid ultrasound at age 18; consider
annual ultrasound depending on individual factors
(continued)
247
Table 64.3 (contiuned)
248
Approximate
Polyp type Syndrome Genes Inheritance Clinical findings risk of CRC Recommended surveillance Treatment
Serrated Serrated Unknown Unknown WHO criteria one of the following 25–40% Annual colonoscopy with removal of polyps larger Endoscopic
(hyperplastic) [16]: than 5 mm and biopsies of smaller clusters (likely polypectomy where
polyposis At least 20 serrated polyps of hyperplastic); usually starting at age of diagnosis possible. Surgery
syndrome any size throughout the colon Colonoscopy interval can be lengthened in patients indicated for
Any number of serrated polyps without polyps on subsequent colonoscopy colorectal cancer or
proximal to the sigmoid in an high polyp burden.
individual with family history of Surgery should be
SPS with total colectomy
At least 5 serrated polyps and ileorectal
proximal to the sigmoid, 2 of anastomosis, with
which are over 1 cm diameter ongoing surveillance
of any remaining
sigmoid and/or rectum
E. Huang and M. F. McGee
64 Hereditary Colorectal Cancer Syndromes 249
Young patient diagnosed with colorectal,

A or HNPCC-related cancer; or strong
family history of HNPCC-related cancer
Tumor specimen microsatellite instability

B (MSI) determination and mismatch repair
protein (MMR) testing
MLH1defect or Yes
BRAF C
MLH1+PMS2
mutation?
defect?
No Yes No
Germline
PMS2 defect
D MMR
(isolated)?
Yes defect?
No
Yes No
Yes
MSH2 or
MSH6
defect?
Lynch syndrome: Strongly
Lynch-like syndrome: No
consider total colectomy
consensus on treatment,
No and TAH/BSO, family
consider total colectomy,
evaluation, surveillance
surveillance program
program
Amsterdam
criteria
positive? Yes
E
No
Familial colorectal cancer type X, no consensus

Traditional treatment and surveillance for
on treatment, consider total colectomy,
sporadic colorectal cancer
surveillance program
Patient with significant Patient with known family

polyposis on colonoscopy, no history of hereditary
F
known mutation polyposis syndrome
Thorough and appropriate personal history,

G family history, physical exam, endoscopic
staging
H Genetic counseling and testing; diagnosis
I Surveillance (See Table 64.1)
J
Treatment (See Table 64.1)
Algorithm 64.1 Management of Patients with Suspected Inheritable Colorectal Cancer Syndromes
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familial adenomatous polypolis, juvenile polyposis,
syndromes. Am J Gastroenterol. 2005;100(1):27.
Colorectal Polyps
65
Algorithmic Approach tions. Narrow-band imaging, colonoscopic

microscopy, and chromoendoscopy may
A. Adenomatous colon polyps are precursors to yield additional information about the polyp
colon cancer, and colonoscopic polypectomy surface features. Nongranular surface fea-
reduces both the incidence and mortality of tures and irregular nonstructured pits (Kudo
colorectal cancer [1]. Adequate bowel prepa- pit pattern type V) are both features that
ration, endoscopic irrigation, and meticulous should raise suspicion of submucosal inva-
suctioning permit thorough and complete sion and invasive adenocarcinoma [5].
mucosal evaluation to detect and treat all C. Many suggest routinely tattooing all polyps
colonic neoplasia. larger than 1–2 cm to facilitate endoscopic
B. Following polyp identification, thorough irri- surveillance or future surgical resection
gation and suctioning are performed to ade- should the lesion prove to be malignant.
quately assess polyp-specific features to D. Nearly all benign polyps are amenable to

guide management and enable treatment. endoscopic excision. Cold snare polypec-
Determination of polyp size, morphology, tomy is the workhorse for most sessile polyps
and location is essential, and use of Paris smaller than 1 cm. Cold forceps can be used
classification to characterize polyp morphol- to excise the smallest (1–2 mm) polyps; how-
ogy is encouraged [2]. Malignant tumors are ever, this technique is associated with high
differentiated from benign adenomas by size, rates of residual adenomatous tissue [6]. Hot
firmness, central depression, ulceration, and biopsy forceps polypectomy techniques have
fixation to the deeper bowel wall [3, 4]. fallen out of favor due to high rates of delayed
Inability to expand peri-polyp submucosa bleeding and perforation. Hot snare polypec-
during saline lift (i.e., “non-lifting sign of tomy is typically used for pedunculated and
Uno”) is associated with invasive cancer or larger sessile (>1 cm) polyps. Saline-lift
scarring from prior polypectomy interven- endoscopic mucosal resection (EMR) is a
useful technique in which the submucosal
E. Huang layer is first injected with saline to “lift” the
Department of Surgery, University of Chicago, polyp, facilitating en bloc or piecemeal resec-
Chicago, IL, USA tion with a hot snare. EMR is helpful for large
M. F. McGee (*) polyps, those spanning many folds, and for
Department of Surgery, Northwestern Memorial large right-sided polyps where the bowel wall
Hospital, Chicago, IL, USA may be more susceptible to thermal injury.

Large postpolypectomy defects may benefit of cancerous invasion of the muscularis

from prophylactic clip closure to decrease the mucosa. These lesions are premalignant (i.e.,
risk of postpolypectomy hemorrhage. Tis or T0), and colonoscopic resection alone
E. Malignant appearing lesions should be biop- may be adequate. Histology, margins, and
sied and tattooed, and not removed, since depth of malignant invasion determine the
malignancy merits oncologic surgical resec- adequacy of colonoscopic polypectomy for
tion. Indeterminately malignant lesions with malignant pedunculated polyps. Haggitt’s
benign biopsy pathology may be referred to classification dictates that polypectomy alone
expert endoscopists for consideration of is sufficient for a favorable- histology tumor
advanced polypectomy. The endoscopist confined to the polyp stalk with a 2 mm mar-
must be aware of his or her limitations prior gin from the cut polyp edge [8]. The analo-
to attempting polypectomy because an gous Kikuchi classification for sessile polyps
incomplete polypectomy may cause submu- has shown polypectomy to be sufficient for
cosal scarring and prohibit later EMR favorable-histology tumor penetration lim-
attempts by an expert. Special situations may ited to the upper third (<1 mm) of submucosa
mandate surgical resection regardless of [9]. Sessile and pedunculated polyps with
polyp histology. For example, polyps grow- deeper submucosal cancerous penetration
ing into the appendiceal orifice or ileocecal (>1 mm) should be considered for oncologic
valve are frequently not amenable to endo- surgical resection given the high frequency of
scopic resection due to the difficulty of lymph node metastases. Regardless of polyp
obtaining a negative margin, as well as risk of morphology, high-risk pathologic features
perforation or appendicitis. In these special such as poor differentiation, lymphovascular
cases, patients should be referred for advanced invasion, and extensive budding increase the
expert colonoscopic polypectomy or consid- risk of lymphatic metastasis and typically
eration of surgical resection. mandate oncologic surgical resection [10].
F. If polyp pathology demonstrates no evidence H. Occasionally, polyp margins, histology, and
of cancer, surveillance colonoscopy should the endoscopist’s assessment of polypectomy
continue based on the number, size, histol- completeness may be unclear. In these situa-
ogy, completeness of polypectomy, bowel tions, multidisciplinary review with the
preparation quality, and patient and family endoscopist, surgeon, and pathologist can
history. Periodically updated guidelines dic- guide decision-making. In this meeting, the
tate the frequency of postpolypectomy sur- risks of local cancer recurrence and lymph
veillance for commonly resected polyps in node metastasis should be balanced against
average-risk individuals [7]. the risk of surgical resection, using the
G. “Carcinoma in situ” or “intramucosal carci- patient’s wishes and operative risk to deter-
noma” are confusing terms that describe lack mine the course of subsequent care.
65 Colorectal Polyps 253
A Polyp identified
Evaluate polyp features: size, morphology,

firmness, villous pattern, ulceration,central
depression
Malignant Stop: Tattoo polyp site

features? and biopsy extensively
Yes
No
Consider tattooing all E

C polyps > 1–2 cm
D
Await pathology and

consider referral to
Any advanced endoscopist or
Sessile polyp
pedunculated oncologic surgical
Sessile polyp < >10 mm: hot Technically
polyp: hot snare, resection
10 mm: cold snare or EMR, infeasible
snare consider clipping consider clipping
base base Multi-disciplinary review:
consider oncologic
surgical resection, EMR,
endoscopic surveillance
Pathology: no
H
malignancy, OR Pathology:
no neoplasia malignancy
at margins G
Carcinoma in Pedunculated <1 mm Uncertain

situ, negative T1 cancer, margin, margins or
margins, no >2 mm margins, >1 mm SM uncertain
F high risk no high risk depth, high histologic
features features risk features features
Endoscopic surveillance Oncologic surgical resection
Algorithm 65.1
References Byth K. Endoscopic mucosal resection outcomes

and prediction of submucosal cancer from advanced
colonic mucosal neoplasia. Gastroenterology.
1. Zauber AG, Winawer SJ, O'Brien MJ, Lansdorp-
2011;140(7):1909–18.
Vogelaar I, van Ballegooijen M, Hankey BF, Shi
6. Efthymiou M, Taylor A, Desmond P, Allen B, Chen
W, Bond JH, Schapiro M, Panish JF, Stewart
R. Biopsy forceps is inadequate for the resection of
ET. Colonoscopic polypectomy and long-term pre-
diminutive polyps. Endoscopy. 2011;43(4):312–6.
vention of colorectal-cancer deaths. N Engl J Med.
7. Lieberman D, Rex D, Winawer S, Giardiello F, Johnson
2012;366(8):687–96.
D, Levin T. Guidelines for colonoscopy surveillance
2. Inoue H, Kashida H, Kudo S, Sasako M, Shimoda T,
after screening and polypectomy: a consensus update
Watanabe H, Yoshida S, Guelrud M, Lightdale CJ,
by the US Multi-Society Task Force on colorectal
Wang K, Riddell RH. The Paris endoscopic classi-
cancer. Gastroenterology. 2012;143(3):844–57.
fication of superficial neoplastic lesions: esophagus,
8. Haggitt R, Glotzbach R, Soffer E, Wruble
stomach, and colon: November 30 to December 1,
L. Prognostic factors in colorectal carcinomas aris-
2002. Gastrointest Endosc. 2003;58(6 Suppl):S3–43.
ing in adenomas: implications for lesions removed
3. Galandiuk S, Fazio VW, Jagelman DG, Lavery IC,
by endoscopic polypectomy. Gastroenterology.
Weakley FA, Petras RE, Badhwar K, McGonagle
1985;89:328–36.
B, Eastin K, Sutton T. Villous and tubulovillous
9. Kikuchi R, Takano M, Takagi K, Fujimoto R, Nozaki
adenomas of the colon and rectum: a retrospective
T, Fujiyoshi T, Uchida Y. Management of early inva-
review, 1964–1985. Am J Surg. 1987;153(1):41–7.
sive colorectal cancer. Risk of recurrence and clinical
4. Doniec JM, Löhnert MS, Schniewind B, Bokelmann
guidelines. Dis Colon Rectum. 1995;38(12):1286–95.
F, Kremer B, Grimm H. Endoscopic removal
10. Resch A, Langner C. Risk assessment in early

of large colorectal polyps. Dis Colon Rectum.
colorectal cancer: histological and molecular markers.
2003;46(3):340–8.
Dig Dis. 2015;33(1):77–85.
5. Moss A, Bourke MJ, Williams SJ, Hourigan LF,
Brown G, Tam W, Singh R, Zanati S, Chen RY,
Colon Cancer
66
Algorithmic Approach
B. For patients with suspected colon cancer,
history-taking should explore abdominal
The surgeon may first encounter a patient at any symptoms (nausea, emesis, early satiety, pain,
point along the timeline of the diagnosis, workup, changes in bowel habits, hematochezia) that
or treatment of colon adenocarcinoma. From an might suggest obstruction or help localize
emergent consultation for perforation to an elec- pathology. Anorexia and weight loss should
tive clinic referral for newly diagnosed tumor to a be assessed. Extraabdominal symptoms such
multidisciplinary tumor board evaluation for as chest pain, cough, dyspnea, and jaun-
complex multivisceral resection, surgeons must dice or anicteric stools may suggest pulmo-
be familiar with the clinical approach for many nary or liver metastases. The patient’s fecal
scenarios [1]. continence status should be explored and
may occasionally impact surgical decision-
A. Up to 30% of colorectal adenocarcinomas
making. As noted in the separate chapter on
may be asymptomatic at the time of diagno- hereditary colorectal cancer, a family history
sis; however, the remainder of patients will of colon cancer or polyposis may prompt
experience symptoms. The most common referral for genetic counseling and testing
symptoms of colon cancer are “alarm” symp- [2, 3]. Physical examination should focus on
toms such as changes in bowel habits or the abdomen, noting any scars from previous
bloody bowel movements. Abdominal pain, surgery that might affect operative planning,
which is infrequently associated with cancer, areas of tenderness, or masses, as well as
is a worrisome sign associated with obstruc- hepatosplenomegaly or ascites. A thorough
tion, local tumor ingrowth, or perforation and lymph node exam may rarely discover meta-
merits expedited workup when cancer is static disease to the inguinal, supraclavicular,
suspected. or cervical lymph node chains. A digital rec-
tal exam may reveal a low-laying tumor and
allows qualitative assessment of sphincter
E. Huang tone. Lower extremity edema may be seen
Department of Surgery, University of Chicago, with hypoalbuminemia and may indicate a
Chicago, IL, USA malnourished state. The initial evaluation
M. F. McGee (*) should also assess baseline functional status,
Department of Surgery, Northwestern Memorial frailty, and other comorbidities, which may
Hospital, Chicago, IL, USA affect future surgical decision-making.

C. The first diagnostic procedure, if not already colorectal), medical oncologists, patholo-
performed, is colonoscopy with tumor local- gists, radiologists, and radiation oncologists
ization, diagnostic biopsy, and location tat- and considered for neoadjuvant chemother-
tooing. As noted in the chapter on colon apy versus primary surgery.
polyps, endoscopic evaluation of the lesion is F. Stage IV colon cancer patients presenting
very important, as certain features are sug- with significantly obstructing, bleeding, or
gestive of malignancy. Tattooing lesions is perforated tumors may be candidates for pal-
essential and guides surgical resection, liative interventions in conjunction with input
whereas complete and thorough evaluation of from a multidisciplinary management team
the remaining colon excludes synchronous and the patient. Obstructing lesions may be
cancers or polyps and other conditions which evaluated for self-expanding metal stents,
may impact surgical decision-making. Rarely, proximal diverting loop stoma, or palliative
occult adenocarcinoma may be discovered in resection. Similarly, tumor-related hemor-
an initially benign-appearing adenomatous rhage may prompt palliative surgical resec-
polyp. Management of such malignant polyps tion or targeted palliative radiotherapy in
is discussed in the colon polyps section of select patients. Perforated lesions should be
this text. managed on clinical context and acuity, bal-
D. Following confirmation of colon cancer, stag- anced carefully on the desires of the patient,
ing computed tomography (CT) of the chest, surgical risks, and likelihood of meaningful
abdomen, and pelvis is performed. survival. Treatment of perforated cancers
Complementary ultrasound, magnetic reso- ranges from nonoperative management with
nance imaging (MRI), or positron emission antibiotics, bowel rest, and percutaneous
tomography (PET) imaging and image- drain placement to emergent proximal divert-
guided biopsy may help characterize equivo- ing stoma or colectomy. The surgeon must be
cal findings found on initial staging studies. A particularly conscientious when considering
complete blood count (CBC), serum chemis- palliative surgery and predicate all decision-
try, liver function tests, and carcinoembry- making to be commensurate with patient’s
onic antigen (CEA) should be drawn at this long-term wishes since comorbidities and
initial encounter. Unexplained anemia may advanced disease may subject patients to
suggest a chronically bleeding lesion, abnor- unacceptably high surgical risks.
mal LFTs may raise suspicion for metastatic G. In the absence of metastatic disease, the sur-
disease and provide a nutritional evaluation, geon should assess appropriateness of cura-
and a baseline CEA level serves as a refer- tive attempt surgery. Imaging typically guides
ence to guide postoperative survivorship [4]. the surgeon to determine resectability of the
E. Approximately one quarter of newly diag- cancer while ensuring adequate margins and
nosed colon cancer patients will present with lymphadenectomy without collateral damage
metastatic disease. Patients with metastatic of adjacent structures. Moreover, the patient
disease absent significant symptoms benefit should prove to be a good candidate for the
from multidisciplinary evaluation in conjunc- proposed intervention. Principles of onco-
tion with a medical oncologist. Curative- logic colectomy include 5 cm margins proxi-
attempt multivisceral resection may confer mally and distally along the colon wall,
survival benefits in select situations with or grossly negative circumferential margins for
without neoadjuvant chemotherapy in T4b cancers (where tumor directly invades
patients with isolated liver and/or lung metas- other structures), high ligation of the arterial
tases [5, 6]. Patients with potentially resect- supply to the affected colon segment, and
able metastatic disease should be discussed complete en bloc lymphadenectomy to assure
with a multidisciplinary team consisting of a minimum of 12 lymph nodes for pathology
surgeons (thoracic, hepatobiliary, and review. Patients with completely resected
66 Colon Cancer 257
tumors who are found to have nodal metasta- imaging, physical exams, CEA, and colonos-
ses (stage III) should be referred to a medical copy determined by periodically updated
oncologist for adjuvant chemotherapy. Stage guidelines.
II (T3 or T4 N0) cancer patients should be
H. Occasionally, local resectability may be in
referred to a medical oncologist to discuss the question, and multidisciplinary consultation
role of adjuvant chemotherapy; however, sur- assists with treatment planning. Tumors found
vival advantages conferred by adjuvant che- to be invading or threatening surrounding
motherapy for stage II colorectal cancers are structures may require multivisceral resection
modest and merit balance of chemotherapy or neoadjuvant treatments aimed to enable
risks and side effects with expected survival future resection with negative margins. The
benefits. Regardless of the prescribed adju- National Comprehensive Cancer Network
vant treatments, all curative intent colectomy (NCCN) guidelines lay out the recommended
patients follow surveillance protocols with treatment regimens for such situations.
Patient with “alarm” symptoms worrisome

A for colorectal cancer, or abnormal imaging
B Thorough and appropriate history and physical
Symptoms; no
colonoscopy yet
Colonoscopy already
Colonoscopy, completed (for symptoms
biopsy, tattoo or screening)
C
Endoscopic confirmation of adenocarcinoma
Metastatic workup: CT chest, abdomen, and

pelvis; CBC, chemistry, LFTs, CEA H
D
Multidisciplinary
discussion
Locally resectable
Special discussion of
Metastatic disease in
neoadjuvant
disease? medically
chemotherapy,
No operable patient? No
intraoperative
radiation therapy
Yes
Yes G
Significantly
Adjuvant
obstructing, Surgical chemotherapy,
bleeding, or resection surveillance
perforated tumor?
Yes
No
Multidisciplinary discussion Consider palliative

E Special cases: isolated liver and/or resection,
diversion, stenting,
F
lung metastases
or radiotherapy
Algorithm 66.1
66 Colon Cancer 259
References lines for hereditary nonpolyposis colorectal cancer

(Lynch syndrome) and microsatellite instability. J
Natl Cancer Inst. 2004;96(4):261–8.
1. National Comprehensive Cancer Network (NCCN).
4. Slentz K, Senagore A, Hibbert J, Mazier WP, Talbott
NCCN clinical practice guidelines in oncology: colon
TM. Can preoperative and postoperative CEA pre-
cancer. Version 2.2017. Accessed at www.nccn.org/
dict survival after colon cancer resection? Am Surg.
professionals/physician_gls/PDF/colon.pdf on 7 Aug
1994;60(7):528–31.
2017.
5. Rosen SA, Buell JF, Yoshida A, Kazsuba S, Hurst R,
2. Vasen HF, Watson P, Mecklin JP, Lynch HT. New
Michelassi F, Millis JM, Posner MC. Initial presenta-
clinical criteria for hereditary nonpolyposis
tion with stage IV colorectal cancer: how aggressive
colorectal cancer (HNPCC, Lynch syndrome) pro-
should we be? Arch Surg. 2000;135(5):530–4.
posed by the International Collaborative group on
6. Pfannschmidt J, Dienemann H, Hoffmann H. Surgical
HNPCC. Gastroenterology. 1999;116(6):1453–6.
resection of pulmonary metastases from colorectal
3. Umar A, Boland CR, Terdiman JP, Syngal S, Chapelle
cancer: a systematic review of published series. Ann
AD, Rüschoff J, Fishel R, Lindor NM, Burgart LJ,
Thorac Surg. 2007;84(1):324–38.
Hamelin R, Hamilton SR. Revised Bethesda guide-
Part IX
Rectum and Anus
Rectal Prolapse
67
Algorithmic Approach maximum conservative measures are undertaken.

Surgeons, therefore, are often called upon to take
Rectal prolapse (procidentia) is defined as full- care of these patients.
thickness intussusception of the rectum through Unfortunately, no consensus exists as to the
the anus [1]. It is a secondary disorder in adults, optimal management of rectal prolapse. This lack
generally arising from chronic pelvic dysfunction of unanimity has resulted in the development and
due to laxity in the muscles of the pelvic floor and adoption of a number of surgical endeavors, none
anal canal, an abnormally deep pouch of Douglas, of which has proven absolute superiority over the
and a lack of normal rectal fixation [2–7]. At others. This very fact has led to discordant prac-
times, these abnormalities are of congenital deri- tice patterns and some degree of confusion, par-
vation; however, in most cases, the weakened ticularly for providers who do not routinely see
pelvic floor is caused by old age, multiparity, these patients. This chapter will attempt to guide
large birth weight of vaginally delivered babies, the surgeon through the appropriate rectal pro-
prior pelvic surgery, increased body mass index, lapse workup and provide clarity in operative
chronic straining, chronic diarrhea, or chronic decision-making (Fig. 1).
constipation [2, 8–11]. Unfortunately, the pro-
lapse itself results in a self-perpetuating cycle of A. Patients with prolapse usually describe a rub-
worsening pelvic floor weakening as the chronic bery mass that protrudes from the anus. Some
stretch on the sphincters and nerves continuously have described it as feeling like a “hard-boiled
exacerbates the problem [11]. As a result of this egg.” It is more pronounced with Valsalva and
vicious cycle, full-thickness rectal prolapse rarely in fact may spontaneously reduce between
resolves spontaneously, even in cases where bowel movements. Most patients will describe
some level of fecal soiling, bleeding, pelvic
discomfort, and altered bowel habits (consti-
Q. M. Hatch pation, diarrhea, incomplete evacuation) [11–
Department of Surgery, Madigan Army Medical
Center, Tacoma, WA, USA 15]. On physical exam, full-thickness rectal
prolapse must be differentiated from internal
E. K. Johnson (*)
Cleveland Clinic Foundation, Cleveland, hemorrhoids, which may also prolapse to an
OH, USA impressive degree. Prolapse will display either
Department of Surgery, Division of Colorectal concentric folds or will be completely smooth
Surgery, Hillcrest Hospital, Mayfield Heights, circumferentially. Hemorrhoids, in contrast,
OH, USA will always appear as radially oriented col-

264 Q. M. Hatch and E. K. Johnson
umns. An additional point of differentiation is majority of patients present with a chronic,

that hemorrhoids rarely prolapse greater than reducible, prolapse with healthy-appearing
5 cm, whereas rectal prolapses routinely do so mucosa. In such cases, sufficient time exists
[8]. While it is possible to have a patient dem- for additional workup and patient counsel-
onstrate rectal prolapse in the lateral decubitus ing. Patients should be reassured that pro-
position, the most consistent way of evaluat- lapse is not dangerous, and people with
ing the degree of rectal prolapse is to have small, minimally symptomatic prolapse do
a patient sit on a toilet or squat over a towel not require an operation.
and then perform a Valsalva maneuver. A
digital rectal exam is important, as it will rule Rectal prolapse may be thought of as a pelvic
out any masses that may be acting as a lead hernia. As with hernias of the abdominal wall, the
point. It will also give a general sense as to the natural tendency is to progress with time. Surgery
function of the pelvic muscles of defecation is therefore indicated (though not mandatory)
(sphincter, puborectalis). In-office testing of even in relatively mild cases. An assessment of
the anal wink and/or bulbocavernosus reflex the patient’s overall health and ability to tolerate
is not required but can help the surgeon esti- an operation must be taken into account when
mate the likelihood of restoring continence making a decision as to whether or not to offer
after surgical repair. Those with impaired surgery. While there is nuance and subjective
reflexes signifying nerve dysfunction may be interpretation involved in this assessment, it
less likely to recover continence fully after stands to reason that patients with exceedingly
surgery. It is important to inspect the visible poor performance status who have mild proci-
rectal mucosa and establish whether or not the dentia may not benefit from an operation of any
rectum is reducible. type. Such patients should be advised to optimize
B. Tissue prolapsing through the anus is often their bowel habits with fiber and hydration.
quite concerning to both patients and clini- Pelvic floor physical therapy and biofeedback
cians. In most cases, however, it is not an may treat the underlying etiology and in doing so
acute matter, as the lax sphincter complex improve defecatory function [16–18].
and weak pelvic floor inherently provide
some degree of protection against acute incar- D. Patients who are deemed operative candi-
ceration. Nevertheless, incarceration and dates should undergo further evaluation to
strangulation may occur, and delay in surgi- guide operative decision-making. The ratio-
cal intervention will result in grave conse- nale behind this is that rectal prolapse is
quences for the patient [8]. rarely an isolated disorder but rather a mani-
festation of global pelvic floor herniation.
If acutely incarcerated or strangulated rectum Cystoceles, enteroceles, rectoceles, sig-
is encountered, the appropriate surgical interven- moidoceles, or vaginal vault prolapses are
tion is a perineal proctectomy or Altemeier pro- frequent cooccurrences and may necessitate
cedure. This procedure involves the full-thickness complex pelvic floor reconstruction in com-
excision of the rectum and, at times, a portion of bination with urogynecology. Dynamic pel-
the sigmoid colon followed by a coloanal or low vic floor imaging evaluates for such anatomic
colorectal anastomosis. In some cases of appar- abnormalities. Both cinedefecography and
ent incarceration, application of sugar to the pro- dynamic pelvic magnetic resonance imaging
lapse may result in osmotic effect, reduction of (MRI) are adequate radiographic studies.
swelling, and the ability to reduce the prolapse— The benefit of MRI is that it is noninvasive;
Turning an acute scenario into an elective one. however, it does not allow the patient to
assume the normal sitting position (unless
C. While a large, strangulated rectal prolapse is the institution has a sitting MRI) that pro-
likely the most dramatic presentation, the motes complete evacuation of stool. These
67 Rectal Prolapse 265
studies will affect the management strategy the sacrum and/or resection or plication of
in up to 40% of cases [19]. redundant bowel [2, 5, 7, 11].
Prior to pursuing an operative procedure to Two broad categories of approach and repair
correct a prolapse in a patient with chronic con- exist: abdominal and perineal. Despite multiple
stipation, it is also important to rule out func- studies, including several randomized controlled
tional obstruction as the underlying etiology. A trials, no approach or procedure has shown supe-
sitz marker study will assess the function of the riority, with recurrence rates ranging anywhere
colon and rule out colonic inertia, while dynamic from 0 to 50%, depending on the series [2, 11, 21,
pelvic floor imaging and anorectal manometry 22]. The recently published PROSPER trial,
will assess for obstructed defecation. A positive which randomized patients to either suture recto-
finding on any of these studies will provide some pexy, resection rectopexy, Delorme, or Altemeier,
understanding of the ultimate causation of the suggested a 10-year recurrence rate of approxi-
procidentia, but as already suggested, nonopera- mately 40%, with no difference between proce-
tive optimization of these abnormalities likely dures [22].
will not correct the prolapse itself. In fact, the
prolapse will only serve to exacerbate the diffi- F. Despite a lack of evidence, dogma holds that
culty with any of these processes and often must perineal procedures have a higher recurrence
be corrected prior to engaging in effective pelvic rate. The benefit, however, is that they spare
floor physical therapy. the patient an abdominal operation. Perineal
If desired, the anatomic construct and function proctosigmoidectomy (Altemeier) has there-
of the anal sphincter itself may be assessed with fore become the procedure of choice for frail,
anal ultrasound or electromyographic studies. elderly patients suffering from rectal pro-
These tests are rarely utilized in practice, as clini- lapse. This procedure involves a circumferen-
cally relevant sphincter dysfunction is likely tial, full-thickness incision just above the
exacerbated by the prolapse itself. The prolapse dentate line, pulling through of redundant
must therefore be surgically corrected before the rectum and sigmoid colon, resection of the
true sphincter function may be accurately redundant segment, and a coloanal anastomo-
assessed or effectively treated. sis. Unfortunately, some of what is saved by
Complete prolapse is associated with a four- sparing abdominal surgery is lost in func-
fold increase in relative risk of colorectal malig- tional morbidity, as proctectomy often results
nancy [20]. It is therefore wise practice to evaluate in high rates of incontinence, soilage, and
for luminal lesions prior to planning any colorec- urgency due to loss of the normal rectal resor-
tal operation in a patient who has not been recently vior [2, 11, 21, 23, 24]. Alternative perineal
screened. It is unlikely that there is a causal rela- options are the mucosal sleeve resection
tionship between the two disease processes (Delorme) and prosthetic anal encirclement
beyond the fact that they both occur in older (Thiersch). These latter two procedures have
patients; however, the correlation highlights the limited use in true full-thickness rectal pro-
necessity of thorough and thoughtful evaluation. lapse in the modern era.
G. Abdominal procedures are more often

E. As a general rule in treating difficult disor- selected in young, fit patients who are bet-
ders, the number of described treatments is ter able to tolerate an abdominal operation.
inversely proportional to the efficacy of any While this approach has not produced a
one. This certainly holds true in cases of definitive benefit in terms of recurrence or
procidentia, as multiple operations have functional outcomes in randomized con-
been described. In most cases, the proce- trolled trials, the prevailing sentiment is
dures rely on the fundamental principles of that the abdominal approach is superior in
prolapse repair, which are rectal fixation to both regards.
Abdominal approaches to rectal prolapse shown significant improvement from baseline

repair include suture rectopexy, resection recto- constipation (48–16%) [25]. Whether or not this
pexy, posterior mesh rectopexy, and ventral mesh holds true as our experience with ventral mesh
rectopexy. There is considerable variation in the rectopexy increases is yet to be determined.
technical aspects of each. Furthermore, any one There does not appear to be a long-term differ-
of them can be performed using laparoscopic or ence in outcomes between minimally invasive
robotic-assisted platforms. Suffice it to say that and open approaches, although avoidance of an
the premise of each is to reduce the prolapse and abdominal incision results in faster overall recov-
anchor it to the sacral promontory (except per- ery and earlier discharge [26].
haps in ventral mesh rectopexy, where the mech-
anism providing success is debated). In the case H. There is no specific need for any follow-up
of resection rectopexy, the fixation is combined beyond routine postoperative care.
with resection of the sigmoid colon. A common Nevertheless, it is critical to reemphasize the
morbidity among these procedures is worsening importance of maintaining optimal bowel
constipation [2, 11]. Advocates of resection rec- habits. This includes a high-fiber diet (25–35
topexy argue that constipation is reduced by grams of soluble fiber a day) and 64 oz of
resecting the sigmoid colon. This assertion has water a day. Fiber supplements such as psyl-
not consistently held true in the literature, lium often help to achieve the goal of soft,
although most series do show some postoperative bulky stools that do not require straining.
improvement in constipation from baseline [2, Patients with obstructed defecation should be
11]. There is an increasing trend toward ventral referred for pelvic floor physical therapy and
mesh rectopexy, as a recent large series has biofeedback [16–18].
67 Rectal Prolapse 267
A History and Physical:

prolapsing tissue with
concentric folds on valsalva
Incarcerated
Reducible
or
strangulated
B C
Functional
evaluation:
Perineal Table –Defecography

–Consider Sitz
D
proctectomy sugar
(Altmeier) markers
–Consider
manometry
–Consider EMG
High-risk Low-risk
patient patient
F G
Observation Prosthetic Perineal Abdominal

with PRN anal prolapse prolapse repair:
manual encirclement repair:
reduction (Thiersch) –Suture
–Altemeier rectopexy
–Delorme –Resection
rectopexy
–Posterior mesh
rectopexy
–Ventral mesh
rectopexy
Algorithm 67.1
References 15. Kim D, Tsang C, Wong W, et al. Complete rectal

prolapse: evolution of management and results. Dis
Colon Rectum. 1999;42(4):460–6.
1. Jacobs L, Lin Y, Orkin B. The best operation for rectal
16. Ternent C, Bastawrous A, Morin N, et al. Practice
prolapse. Surg Clin North Am. 1997;77:49–70.
parameters for the evaluation and management of
2. Madiba T, Baig M, Wexner S. Surgical management
constipation. Dis Colon Rectum. 2007;50:2013.
of rectal prolapse. Arch Surg. 2005;140:63–73.
17. Jorge J, Habr-Gama A, Wexner SD. Biofeedback

3. Broden B, Snellman B. Procidentia of the rectum
therapy in the colon and rectal practice. Appl
studied with cineradiography: a contribution to
Psychophysiol Biofeedback. 2003;28:47–61.
the discussion of causative mechanism. Dis Colon
18. Khaikin M, Wexner S. Treatment strategies in

Rectum. 1968;11:330–47.
obstructed defecation and fecal incontinence. World
4. Roig J, Buch E, Alo´s R, et al. Anorectal function in
J Gastroenterol. 2006;12(20):3168–73.
patients with complete rectal prolapse: differences
19. Harvey C, Halligan S, Bartram C, et al. Evacuation
between continent and incontinent individuals. Rev
proctography: a prospective study of diagnostic and
Esp Enferm Dig. 1998;90:794–805.
therapeutic effects. Radiology. 1999;211:223–37.
5. Kuijpers H. Treatment of complete rectal prolapse: to
20. Rashid Z, Basson M. Association of rectal pro-

narrow, to wrap, to suspend, to fix, to encircle, to pli-
lapse with colorectal cancer surgery. Surgery.
cate or to resect? World J Surg. 1992;16:826–30.
1996;119:51–5.
6. Nicholls R. Rectal prolapse and the solitary ulcer syn-
21. Deen K, Grant E, Billingham C, et al. Abdominal
drome. Ann Ital Chir. 1994;65:157–62.
resection rectopexy with pelvic floor repair ver-
7. Yakut M, Kaymakciioglu N, Simsek A, et al. Surgical
sus perineal rectosigmoidectomy and pelvic floor
treatment of rectal prolapse: a retrospective analysis
repair for full thickness rectal prolapse. Br J Surg.
of 94 cases. Int Surg. 1998;83:53–5.
1994;81(2):302–4.
8. Stein E, Stein DE. Rectal procidentia: diagnosis
22. Senapati A, Gray RG, Middleton LJ, et al. PROSPER:
and management. Gastrointest Endosc Clin N Am.
a randomised comparison of surgical treatments for
2006;16:189–201.
rectal prolapse. Color Dis. 2013;15(7):858–68.
9. Marceau C, Parc Y, Debroux E, et al. Complete rectal
23. Johansen O, Wexner S, Daniel L, et al. Perineal rec-
prolapse in young patients: psychiatric disease a risk
tosigmoidectomy in the elderly. Dis Colon Rectum.
factor of poor outcome. Color Dis. 2005;7:360.
1993;36:767–72.
10. Mellgren A, Bremmer S, Johansson C, et al.

24. Yoshioka K, Ogunbiyi O, Keighley M. Pouch peri-
Defecography. Results of investigations in 2,816
neal rectosigmoidectomy gives better functional
patients. Dis Colon Rectum. 1994;37:1133.
results than conventional rectosigmoidectomy in
11. Hatch Q, Steele S. Rectal prolapse and intussuscep-
elderly patients with rectal prolapse. Br J Surg.
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12. Hiltunen K, Matikainen M, Auvinen O, et al. Clinical
25. Consten EC, van Iersel JJ, Verheijen PM, et al. Long-
and manometric evaluation of anal sphincter func-
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Keighley M, Fielding J, Alexander-Williams
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14. Madoff R, Mellgren A. One hundred years of rectal
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Solitary Rectal Ulcer Syndrome
68
Algorithmic Approach rectal pressures resulting in a relative ischemia

of the mucosa while creating shearing of the rec-
Solitary rectal ulcer syndrome (SRUS) often tum against the pelvic floor muscles, namely the
confused with colitis cystica profunda, rectal puborectalis muscle. This phenomenon ulti-
neoplasia, or inflammatory bowel disease/procti- mately results in repeated mucosal damage and
tis is a rare benign disorder of defecation that can the formation of one or multiple (polypoid)
cause significant distress for those affected. ulcers [2, 3].
There is a relative paucity of literature associated In this chapter, we will present key findings
with SRUS with the majority of the published that will aid in correctly identifying SRUS and
articles being limited to descriptive analysis and provide a stepwise approach on how best to con-
case reports. Approximately 1 in 100,000 indi- servatively manage these patients. Unfortunately,
viduals per year will suffer from SRUS with conservative management fails in approximately
similar incidences in both female and male pop- one third of patients who require surgical inter-
ulations with a slight dominance in women. vention for resolution. There are no official
Diagnosis is usually in the third or fourth decade guidelines regarding the surgical management of
of life although certain pediatric and geriatric these patients. As such, there have been many
populations are also at higher risk [1]. The surgical options developed overtime [4]. We
pathophysiology of SRUS is poorly understood, review the benefits and drawbacks to these vari-
although there are several prevailing factors that ous approaches in hopes of providing a helpful
seem to be consistently associated. Most patients reference for surgeons who are called on to man-
display a dyssynergia between contraction of the age SRUS.
pelvic floor muscles and defecation causing high
A. A complete history and physical exam in

the office is perhaps the single most impor-
J. Kuckelman tant step in determining if a patient has
Department of General Surgery, Madigan Army
SRUS. Rectal ulcers can be due to vari-
ous etiologies (see B). The clinician should
Uniformed Services University of the Health
attempt to elicit any and all underlying eti-
Sciences, Bethesda, MD, USA
ologies at the initial patient encounter. The
E. K. Johnson (*)
focus of treatment should be on rectifying or
Cleveland Clinic Foundation, Cleveland, OH, USA
optimizing the primary issue. Resolution of
Department of Surgery, Division of Colorectal Surgery,
the rectal ulcer will often follow.
Hillcrest Hospital, Mayfield Heights, OH, USA

270 J. Kuckelman and E. K. Johnson
The most common complaint is rectal bleed- risk factors for rectal cancer. These examples
ing and/or a mucous discharge associated with a are independent ailments to SRUS and should
feeling of incomplete evacuation. However, it is be managed separately from the algorithm
important to realize that a quarter of patients will shown in Fig. 1. Several have devoted chap-
present without any symptoms at all with rectal ters in this book.
ulcer being discovered on a screening exam or C. The diagnosis of SRUS is often delayed due
colonoscopy or during the workup for rectal to the large differential diagnosis for rectal
bleeding or tenesmus [1, 4]. On physical exam, bleeding, as well as the need to rule out other
the surgeon may see blood or mucous discharge primary causes such as rectal cancer.
at the anus. Digital rectal exam may reveal muco- Performing a colonoscopy with biopsies of
sal disruption. Most SRUSs have been reported normal and abnormal mucosa is crucial to
to be found on the anterior wall at various lengths making the diagnosis of SRUS, as histologi-
from the anal margin up to 10 cm [5, 6]. A rectal cal analysis can confirm the diagnosis.
ulcer may also be directly visualized in the office Solitary rectal ulcer syndrome may be dis-
using anoscopy or proctosigmoidoscopy. If a rec- covered to be a misleading name on endo-
tal ulcer is identified, it should be evaluated with scopic analysis, as there is a high variability
further studies to ensure a diagnosis of SRUS. in the appearance of SRUS ranging from
simple mucosal erythema to a chronic-
B. Patients with rectal ulcer should be screened appearing ulcer with nodular edges and a
for history of inflammatory bowel disease— white or sloughing base [1, 10]. Further, up
Specifically ulcerative colitis and immuno- to one third of patients may have a polypoid
suppressive disorders such as HIV/AIDS as lesion with multiple ulcers present [1]. Once
opportunistic viral infections may cause rec- biopsies are obtained, histological analysis
tal ulceration. These causes should be man- showing no evidence of malignancy with
aged with primary treatment being targeted at obliterated lamina propria and hypertrophy
the underlying cause [7]. The relationship of of the muscular layer with regenerative
rectal ulcer to rectal prolapse is a matter of changes of the crypts is diagnostic of SRUS
some debate as some view them as synony- [1, 10, 11].
mous and related diagnoses, while others
view them as two separate entities. Dynamic imaging may play a vital role in
Intussusception of the rectum may create an understanding the cause of SRUS, and it should
ischemic environment that predisposes the be performed prior to operative intervention to
rectal mucosa to ulceration. In the presence of help aid in preoperative planning. Defecography
prolapse, patients should be questioned about either using fluoroscopy or magnetic resonance
or asked to demonstrate how they reduce their imaging (MRI) may show a lack of coordination
prolapsed rectum as digital reduction may between the involuntary pelvic floor muscles and
cause ulceration through repeated trauma to the external anal sphincter [12, 13]. Often, an
the area [8, 9]. Rectal prolapse identified in internal rectal intussusception will be seen with-
the setting of SRUS should be repaired out evidence of full-thickness rectal prolapse.
(options discussed elsewhere). Circumstances These studies can aid both in supporting the diag-
causing repeated trauma to the rectum (such nosis and in determining which intervention is
as anal-receptive intercourse) should be needed. Finally, endorectal ultrasound (ERUS)
avoided. Patients with history of vascular dis- has been shown to evaluate both ulcer depth and
ease may be predisposed to ulceration at the external and internal sphincter muscles. There
Sudeck’s point, and treatment should be is some evidence that sphincter thickness on
aimed at optimizing blood flow to this area ERUS correlates to internal rectal intussusception
[7]. Finally, all patients with a visualized rec- leading to SRUS; and this information may guide
tal ulcer should be questioned for any signs or operative planning as well [14].
68 Solitary Rectal Ulcer Syndrome 271
D. Once benign SRUS has been confirmed, con- F. As this is a benign condition, the primary
servative management should be the first line goals of surgery are to manage the patient’s
of treatment. On rare occasions, symptoms symptoms while keeping the bowel in conti-
may be so severe that early operative inter- nuity. For patients without any evidence of
vention may be warranted; however, every intussusception, a local repair should be
attempt at conservative management should attempted. Depending on size and location of
be made. The mainstay of conservative man- the ulcer, transanal excision may be attempted
agement includes patient education focusing down to the muscular layer. Ulcers located
on behavioral modification. Patients should higher in the rectum may be amendable to
understand the benefits of healthy stooling local excision using a transanal minimally
habits including high-fiber diets, regular toi- invasive approach (TAMIS) [19]. Other strat-
leting, and avoidance of straining. Bulking egies for local therapy have been described
agents and stool softeners may be added to using sclerotherapy. Direct therapies such as
accomplish these goals. These modifications serial application of argon beam coagulation
are highly effective in asymptomatic or as well as fibrin glue have also been cited in
mildly symptomatic patients. the literature [20]. Primary repair with suture
closure using healthy surrounding redundant
Medical therapy can be used to aid in healing mucosa has been described, but these local
and quell symptomatic patients. Topical agents therapies usually provide short-term symp-
such as sucralfate or mesalamine may be adminis- tomatic relief without significant long-term
tered via enema and in some cases have been found benefits [4, 5]. These local therapies, although
beneficial for acute management. Little evidence described, typically have poor outcomes and
exists to support long-term efficacy [1, 15, 16]. may even worsen the size and depth of the
ulcer; thus, they are generally not recom-
E. In patients who continue to experience symp- mended. If there is evidence of a hypertonic
toms without resolution of SRUS, the next or thickened sphincter muscle, injection of
step should be biofeedback and pelvic floor botulinum toxin may relax the sphincter com-
physical therapy. These methods target pelvic plex and allow healing of the ulcer [21].
floor behaviors to specifically reprogram Although it has been described, division of
autonomic pathways associated with defeca- the puborectalis muscle is a particularly mor-
tion. This effectively corrects dyssynergy and bid operation resulting in high rates of incon-
prevents straining in the majority of patients. tinence and is not generally recommended
Objective evidence suggests that biofeedback [22]. Overall, these surgical approaches may
therapy provides the best chance for patients provide some short-term relief, but they are
with SRUS with 50–75% of patients having often not durable, and recurrence is common
complete resolution of their ulcer and associ- [23, 24].
ated symptoms [3, 17, 18]. G. When there is either clinical or radiographic
evidence of full thickness or internal rectal
If conservative management fails, surgical prolapse, then surgical repair of this disorder
intervention remains an option. In fact, up to one should be considered first. Rectopexy with or
third of patients will require surgical interven- without the use of mesh using either an open
tion. [1] There are multiple approaches that have or laparoscopic abdominal approach is the
described that we will review. However, due to procedure of choice for these patients, as it
the variability in location, size, and potential directly addresses the most likely attributed
pathophysiology coupled with the relative rarity pathophysiology. This approach will be effec-
of the disease, there is no one favored surgical tive and possible in either scenario and is sup-
approach to SRUS, and each patient should be ported by the largest amount of evidence,
treated based on findings discussed in A and C. with 55–83% of patients having symptomatic
improvement [22, 24, 25]. The decision to fecal diversion should be discussed with the
perform a perineal proctectomy (Altemeier patient. This may be done as a temporizing
procedure) will depend on the severity of the measure or as a permanent solution in will-
prolapse (not applicable to internal intussus- ing patients. This is typically accomplished
ception), the fitness of the patient for surgery, with the formation of an end colostomy
as well as the depth of the ulcer. Ulcers which may only be required for a matter of
extending into muscular layers on ERUS months. Endoscopic evidence of complete
should be resected with proctectomy or trans- healing should be obtained prior to consider-
anal excision if possible [26]. In some severe, ation of a restorative procedure. It is impor-
persistent, or recurrent cases, a low anterior tant to consider the most likely etiology for
resection with coloanal anastomosis/recon- the disorder and to employ a strategy to cor-
struction can be effective. It should be noted rect it as a cornerstone in the patient’s treat-
that this is a radical option associated with ment plan. As mentioned earlier, any plan
significant morbidity and should be used only that addresses the ulcer without attention to
as a last resort. the underlying cause will typically result in
. In the case that all other treatments, conser-
H recurrence and frustration for both the
vative and surgical, have been exhausted, patient and surgeon.
68 Solitary Rectal Ulcer Syndrome 273

Bleeding/Mucous
Discharge
Constipation
A Tenesmus/Straining
Digital rectal exam
Anoscopy
Proctosigmoidoscopy
-Colonoscopy with biopsies -

C Barium enema -Defecography
-
Manometry/Electromyograph
y -Rectal ultrasound
B -Cancer
-Prolapse -
Infectious -
Separate Algorithms Ulcerative colitis
-Ischemic colitis -
Retained foreign
body
Benign solitary
Conservative management
Rectal ulcer
D
-Patient education -
Behavior modification
Severe symptoms -High fiber, bulking -
Failure
Sulcralfate enema -
Topical steroids -
E Bio
Sulfasalazine
feedback
Failure
No
intussusception Intussusception
G
F
Surgical repair of
Local repair
prolapse
Failure
Sclerotherapy Botulinum toxin Local excision

Fecal diversion H
TAMIS Transanal
End colostomy
Algorithm 68.1
References 15. Zargar SA, Khuroo MS, Mahajan R. Sucralfate

retention enemas in solitary rectal ulcer. Dis Colon
Rectum. 1991;34(6):455–7.
1. Zhu QC, et al. Solitary rectal ulcer syndrome: clinical
16. Aad G, et al. Combined measurement of the Higgs
features, pathophysiology, diagnosis and treatment
boson mass in pp collisions at sqrt[s]=7 and 8 TeV
strategies. World J Gastroenterol. 2014;20(3):738–44.
with the ATLAS and CMS experiments. Phys Rev
2. Morio O, et al. Anorectal physiology in solitary ulcer
Lett. 2015;114(19):191803.
syndrome: a case-matched series. Dis Colon Rectum.
17. Rao SS, et al. ANMS-ESNM position paper and

2005;48(10):1917–22.
consensus guidelines on biofeedback therapy for
3. Rao SS, et al. Pathophysiology and role of biofeed-
anorectal disorders. Neurogastroenterol Motil.
back therapy in solitary rectal ulcer syndrome. Am J
2015;27(5):594–609.
Gastroenterol. 2006;101(3):613–8.
18. Vaizey CJ, Roy AJ, Kamm MA. Prospective evalua-
4. Bulut T, et al. Solitary rectal ulcer syndrome:
tion of the treatment of solitary rectal ulcer syndrome
exploring possible management options. Int Surg.
with biofeedback. Gut. 1997;41(6):817–20.
2011;96(1):45–50.
19. Walega P, Kenig J, Richter P. Transanal endoscopic
5. Niv Y, Bat L. Solitary rectal ulcer syndrome--clin-
microsurgery combined with endoscopic posterior
ical, endoscopic, and histological spectrum. Am J
mesorectum resection in the treatment of patients with
Gastroenterol. 1986;81(6):486–91.
T1 rectal cancer - 3-year results. Wideochir Inne Tech
6. Vaizey CJ, et al. Solitary rectal ulcer syndrome. Br J
Maloinwazyjne. 2014;9(1):40–5.
Surg. 1998;85(12):1617–23.
20. Somani SK, et al. Healing of a bleeding solitary

7. Tjandra JJ, et al. Clinical and pathologic factors asso-
rectal ulcer with multiple sessions of argon plasma.
ciated with delayed diagnosis in solitary rectal ulcer
syndrome. Dis Colon Rectum. 1993;36(2):146–53.
21. Keshtgar AS, et al. Botulinum toxin, a new treatment
8. Tjandra JJ, et al. Clinical conundrum of solitary rectal
modality for chronic idiopathic constipation in chil-
ulcer. Dis Colon Rectum. 1992;35(3):227–34.
dren: long-term follow-up of a double-blind random-
9. Mackle EJ, Parks TG. The pathogenesis and patho-
ized trial. J Pediatr Surg. 2007;42(4):672–80.
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22. Sitzler PJ, et al. Long-term clinical outcome of sur-
syndrome. Clin Gastroenterol. 1986;15(4):985–1002.
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10. Chiang JM, Changchien CR, Chen JR. Solitary rec-
1998;85(9):1246–50.
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23. Choi HJ, et al. Clinical presentation and surgical out-
presentation and literature review. Int J Color Dis.
come in patients with solitary rectal ulcer syndrome.
2006;21(4):348–56.
Surg Innov. 2005;12(4):307–13.
11. Madigan MR, Morson BC. Solitary ulcer of the rec-
24. Ihnat P, et al. Novel combined approach in the

tum. Gut. 1969;10(11):871–81.
management of non-healing solitary rectal ulcer
12. Mahieu PH. Barium enema and defaecography in the
syndrome - laparoscopic resection rectopexy and
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transanal endoscopic microsurgery. Wideochir Inne
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syndrome: findings at barium enema study and defe-
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Rectal Cancer
69
Algorithmic Approach ment of rectal cancer has become a complex mul-

tidisciplinary endeavor for which a simple linear
Rectal cancer has historically been associated algorithm does not exist. This chapter will
with substantial morbidity and mortality [1, 2]. attempt to distill the nuances and complexity of
This applies not only to the primary disease pro- rectal cancer into a straightforward flow.
cess but to the treatment as well. An abdomino-
perineal excision may result in a number of A. There is no “classic” presentation of rectal
perineal wound issues in addition to the obligate cancer. This is in part due to relatively poor
permanent stoma. Sphincter-sparing low anterior screening rates, reluctance of patients to seek
resection routinely results in a significant change early care for anorectal complaints, and the
in defecatory function that can even be disabling lack of specificity regarding visceral pain and
[3]. Additionally, either operation or the adminis- gastrointestinal hemorrhage [9, 10]. Despite
tration of radiotherapy may result in sexual dys- the nonspecific nature of presenting com-
function, urinary dysfunction, or chronic pelvic plaints, it is important to maintain a high
pain [4]. Fortunately, these risks are at least in index of suspicion for malignancy as up to
part counterbalanced by vastly improved onco- two thirds of colorectal cancers will manifest
logic outcomes over the past century [1, 5–8]. vague abdominal complaints or anemia prior
Key breakthroughs allowing for these improve- to diagnosis [10].
ments include surgical innovation (abdomino-
perineal excision, total mesorectal excision) in Because of the myriad presentations, we may
addition to significant advances in chemotherapy come in contact with rectal cancer patients at any
and radiation therapy. Consequently, the manage- given point in the diagnostic evaluation. As such,
we must be able to pick up efficiently at any point
in the algorithm. Regardless of the point at which
Q. M. Hatch we as surgeons encounter the patient, the history
Department of Surgery, Madigan Army Medical and physical examination is a requisite first step
Center, Tacoma, WA, USA before further discussion can ensue. We must
E. K. Johnson (*) rapidly decide if this is an acute presentation that
Cleveland Clinic Foundation, needs an immediate intervention (such as severe
Cleveland, OH, USA bleeding or complete obstruction) or if there is
Department of Surgery, Division of Colorectal time for a thorough evaluation before initiating
Surgery, Hillcrest Hospital, Mayfield Heights, treatment. A complete large bowel obstruction
OH, USA

secondary to a rectal cancer is a surgical emer- any rectal cancer or left-sided colon cancer (as
gency necessitating colonic decompression. In flexible endoscopy is inaccurate when assessing
most cases, this may be accomplished with a loop distance from the anus). This procedure allows
sigmoid colostomy or perhaps a rectosigmoid for precise tumor localization in relation to the
stent. It is important to bear in mind that while an anus and thereby helps predict our ability to per-
endoluminal stent is an attractive tool as a bridge form a sphincter-sparing surgery.
to surgery, it must be proximal enough that the
distal extension does not impinge on the anorec- B. In cases where a minimally symptomatic or
tal ring [11]. In rare instances, the colon proximal asymptomatic rectal polyp is identified on
to the tumor may be compromised or perforated, endoscopy, it is essential that the staging
in which case a subtotal colectomy with end ile- workup not be compromised by poor diag-
ostomy and a mucous fistula may be warranted. nostic decision-making. Small sessile polyps
Bleeding may be corrected by resuscitation and or pedunculated polyps may undergo polyp-
stabilization, followed by radiation therapy [12]. ectomy with near impunity, insofar as the
An emergent proctectomy is rarely required, endoscopist feels confident of the ability to
although for hemodynamically compromising perform complete polypectomy. However, in
bleeding refractory to endovascular treatments it cases where a complete endoscopic excision
may be necessary. cannot be ensured (as in large, sessile pol-
The majority of rectal cancer referrals will yps), it is best to proceed with staging prior to
exhibit subacute or chronic symptoms such as tissue biopsy. Unfortunately, it is all too com-
changes in bowel habits (74%), vague abdominal mon that a large rectal polyp is “excised” and
pain (67%), or anemia (41%) [10]. These patients returns with adenocarcinoma and a positive
are afforded the luxury of a complete evaluation. margin. This sequence of events severely
A thorough assessment of preoperative sphincter compromises the ability of the surgeon to
function must be performed. This is a critical step adequately stage the cancer, as the tissue
as a restorative proctectomy will be particularly planes are edematous and inflamed. This
morbid for a patient with questionable continence error in judgment diminishes our ability to
at baseline. It is also essential to identify the accurately differentiate the T-stage. This is a
patient’s preoperative sexual function (erections, critical mistake as preoperative staging deter-
ejaculation in men). The abdominal exam should mines which patients will derive benefit from
assess for distention or organomegaly which neoadjuvant therapy. Regardless of the spe-
would suggest obstruction or metastatic disease, cific management of the polyp, endoscopic
respectively. A digital rectal examination should “tattooing” adjacent to the lesion is essential,
be performed, with particular attention paid to as it allows for accurate identification later in
the distance between the anal sphincter complex the workup.
and the distal aspect of the tumor, evidence of C. Once the diagnosis of adenocarcinoma is
tumor fixation, and anterior-posterior location. confirmed, the depth of invasion must be
Patients with tumor abutting the anal sphincters assessed. In the case of cancer limited to a
should be counseled that an abdominoperineal polyp with a clear (>2 mm) resection margin,
excision will likely be required. Anterior tumors endoscopic surveillance is appropriate [13,
are inherently more likely to abut or invade adja- 14]. Notable exceptions are cases in which
cent structures such as the posterior vagina, the cancer is poorly differentiated, there is
uterus, prostate, urethra, and bladder (in men or lymphovascular invasion, or if the cells are
women post-hysterectomy) as all reside within mucinous or signet-ring cell type [15]. If the
millimeters of the anterior rectum. margin is in question or there is invasion into
An essential adjunct to the physical exam is the rectal wall proper, a complete staging
rigid proctoscopy in the office. This critical step workup is required. If the diagnosis remains
should be performed during surgical workup of in question after biopsies of a large polyp, a
69 Rectal Cancer 277
transanal excision or submucosal lift and Table 69.1 American Joint Committee on cancer TNM
definitions for rectal cancer
snare polypectomy may be performed [16].
D. Further evaluation after diagnosis of inva- Primary Tumor
sive cancer necessitates assessment of Tx Primary tumor cannot be assessed
T0 No evidence of primary tumor
tumor markers (carcinoembryonic antigen
Tis Carcinoma in situ: intraepithelial or invasion of
(CEA) and complete blood count (CBC)). lamina propria
Liver function tests are routinely obtained T1 Tumor invades submucosa
but are not essential. Clinical tumor, node, T2 Tumor invades muscularis propria
metastasis (TNM) staging is entirely reliant T3 Tumor invades through the muscularis propria
on imaging modalities. Depth of invasion into pericolorectal tissues
(T-stage) and regional nodal involvement T4a Tumor penetrates to the surface of the visceral
peritoneum
may be assessed using either transrectal
T4b Tumor directly invades or is adherent to other
ultrasound (TRUS) or magnetic resonance organs or structures
imaging (MRI) using a specific rectal cancer Regional Nodes
protocol. Neither modality has proven supe- Nx Regional lymph nodes cannot be assessed
riority, although TRUS may more accurately N0 No regional lymph node metastasis
stage early (T1 vs T2) tumors, while MR N1a Metastasis in one regional lymph node
may more accurately determine the tumor’s N1b Metastasis in 2–3 regional lymph nodes
distance from the sphincters and the distance N1c Tumor deposit(s) in the subserosa, mesentery,
or nonperitonealized pericolic or perirectal
to the mesorectal fascia (threatened circum- tissues without regional nodal metastasis
ferential resection margin) [17]. Computed N2a Metastasis in 4–6 regional lymph nodes
tomography of the chest, abdomen, and pel- N2b Metastasis in 7 or more regional lymph nodes
vis with oral and intravenous contrast is used Distant Metastases
to rule out distant metastases. In cases where M0 No distant metastasis
axial imaging shows suspicious lesions M1a Metastasis confined to one organ or site (for
example, liver, lung, ovary,
without definitive evidence of metastasis,
nonregional node)
positron emission tomography (PET) scan- M1b Metastases in more than one organ/site or the
ning may be helpful. peritoneum
E. The staging of rectal cancer can be seen
in Tables 69.1 and 69.2 [18]. Stage 1 dis-
ease encompasses T1 and T2 lesions with- final pathology returns with these adverse
out nodal involvement. For small (<3 cm, risk factors or if the pathologic T-stage is
<40% luminal circumference) T1 cancers, higher than was previously expected, fur-
a full-thickness transanal excision is likely ther treatment is needed. While a number
appropriate [19]. This approach is limited of approved algorithms exist, we recom-
by the technical ability to perform the pro- mend completion proctectomy with total
cedure without compromising the rectal mesorectal excision (low anterior resection
lumen. Additionally, patients must be thor- versus abdominoperineal excision, depend-
oughly counseled that transanal excision ing on the distance from the sphincter), fol-
does not include lymphadenectomy, which lowed by observation (if pathologic stage
is essential for pathologic staging. This is a returns T1-2, N0) or a combination of sys-
critical point as up to 12% of T1 lesions may temic chemotherapy and chemoradiation
have regional nodal involvement that would (if pathologic stage returns T3-4 or N1-2)
upstage them to stage III [20]. Risk factors (Table 69.3). An alternative algorithm is to
for regional node involvement include poor proceed to chemoradiation (Table 69.3) fol-
differentiation, lymphovascular invasion, or lowed by either observation (if complete
adverse histologic subsets such as “muci- response), completion proctectomy, or com-
nous” or signet-ring cell type [15, 20]. If the bination chemotherapy [21].
Table 69.2 American Joint Committee on cancer TNM Table 69.3 Definitions and types of combination che-
stages for rectal cancer motherapy and chemoradiation within NCCN guidelines
Stage T N M Chemoradiation: 50·4 Gy in 28 fractions of 1·8 Gy
0 Tis N0 M0 plus radiosensitizing radiation and interval surgery
1 T1 N0 M0 Capecitabine plus radiation
T2 N0 M0 Infusional 5-FU plus radiation
IIA T3 N0 M0 Bolus 5-FU/Leucovorin plus radiation
IIB T4a N0 M0 Short-course radiation: 25 Gy in five fractions of 5 Gy
IIC T4b N0 M0 and surgery within 7 days
IIIA T1–T2 N1/N1c M0 Combination chemotherapy
T1 N2a M0 FOLFOX
IIIB T3–T4a N1/N1c M0 CAPEOX
T2–T3 N2a M0 5-FU/Leucovorin or capecitabine
T1–T2 N2b M0
IIIC T4a N2a M0 Cancer Network (NCCN) recommends
T3–T4a N2b M0
against short-course radiation in cases of T4
T4b N1–N2 M0
disease. Definitive proctectomy should follow
IVA Any T Any N M1a
IVB Any T Any N M1b radiation therapy by 4–12 weeks, though most
are waiting 8–12 weeks prior to undertaking
resection [24].
T2 lesions have a 22% risk of regional nodal
involvement [20]. Accordingly, T2 lesions should Interestingly, the benefit of neoadjuvant radia-
be managed with proctectomy/total mesorectal tion/chemoradiation in terms of local control has
excision (TME) (depending on sphincter involve- not definitively resulted in improved mortality in
ment and preoperative sphincter function. most cases [7, 8]. This is likely explained by our
Assuming complete excision and an adequate ongoing relative inability to control distant recur-
mesorectal dissection, additional chemotherapy rence, which remains roughly 30% for stage II
or radiation is not required. and III rectal cancer [8, 22]. These high rates of
metastatic recurrence have led many to adopt
F. Stage II rectal cancer includes T3 and T4
neoadjuvant combination chemotherapy fol-
tumors. It represents the formal stage at lowed by chemoradiation and ultimately defini-
which neoadjuvant therapy is recommended. tive resection. This algorithm front-loads
A number of studies have observed a benefit systemic therapy to treat subclinical metastases.
in local recurrence when surgery is preceded Further studies are needed to determine if this
by radiation therapy. The debate between the treatment strategy will yield improved survival.
benefit of short-course radiation (Table 69.3) It is important to understand that the current
and long-course radiation (in conjunction standard is to recommend preoperative chemora-
with radiosensitizing chemotherapy) rages diotherapy in extraperitoneal rectal adenocarci-
on, as randomized controlled trials have not nomas that are T3 or greater or node positive
shown oncologic superiority of either strategy. based on preoperative staging. This recommen-
However, the secondary outcome of negative dation may be called into question in the future
circumferential resection margin (CRM) seems based on outcomes as they relate to tumors with
to be improved with long-course chemora- and without threatened circumferential resection
diation, and this endpoint has been associated margin on MRI pending the results of ongoing
with lower local recurrence rates [22, 23]. It is studies [25].
this fact which has led many centers to pref-
erentially select chemoradiation in cases with G. Stage III rectal cancer is defined as regional
a threatened CRM by MRI. In keeping with node-positive disease. Treatment algorithms
this concept, the National Comprehensive are the same as those for stage II disease, with
neoadjuvant radiation conferring a 52% (9% ation where a patient presents with oligometa-
versus 19%) decrease in local recurrence static disease. Most would not recommend
when compared to resection alone. Long- chemoradiation followed by resection up front
term follow-up to the landmark Dutch ran- but would instead recommend a strategy of
domized controlled trial also suggests a employing palliative chemotherapy followed by
survival benefit in patients with stage III rec- restaging after an appropriate period of time—
tal cancer treated with neoadjuvant radiation typically 3 months. If the metastatic disease is
(50% versus 40% at 10 years) [26]. stable or improved, then many would encourage
H. The definition of resectable disease is in flux standard chemoradiation followed by palliative
and may change depending on tumor response resection after an appropriate wait (8–12 weeks
to neoadjuvant chemotherapy. Suffice it to after completion of radiotherapy). While resec-
say that the old paradigm suggesting M1 dis- tion is not curative, it will prevent the suffering
ease is unresectable is no longer valid. associated with a locally invasive tumor in the
Patients with resectable liver metastases and pelvis. It is the authors’ preference to perform
potentially extrahepatic metastases may be an abdominoperineal resection in this setting.
considered resectable for cure [27]. In fact, This offers a single stage procedure allowing
patients undergoing R0 resection of liver the patient to resume life-lengthening chemo-
metastases have 5-year survival as high as therapy as soon as possible.
71% in some series [28]. Clearly, this deci-
sion requires multidisciplinary discussion I. Surveillance begins after treatment for cura-
with surgeons who routinely evaluate these tive intent. This should include office history
organ systems. The decision-making strategy and physical every 3–6 months for 2 years,
regarding the order in which to proceed with then every 6 months for a total surveillance
chemoradiation, combination chemotherapy, period of 5 years. In cases of stage II–IV dis-
and surgery in cases of metastatic disease is ease treated only with transanal excision, the
beyond the scope of this chapter, but all are office exam should be accompanied by rigid
valid first options depending on the specific proctoscopy and either transrectal ultrasound
clinical scenario. or rectal MRI. CEA levels should be obtained
in conjunction with all clinic visits. Computed
In the case of unresectable disease (either tomography of the chest, abdomen, and pelvis
locally unresectable or metastases), palliative with oral and intravenous (IV) contrast should
chemotherapy is an option in appropriately fit be obtained every 6 to 12 months for a total of
patients who desire to extend life. In rare cir- 5 years. Colonoscopy should be performed
cumstances, a robust response to chemotherapy 1 year after surgery. If an advanced adenoma
may actually convert the disease burden from is found, another colonoscopy should be per-
unresectable to resectable. Hepatic artery infu- formed a year later. If an advanced adenoma
sion of chemotherapy may act as an adjunct is not found, colonoscopy should be per-
[29]. In these cases, additional multidisciplinary formed 3 years after the first and every 5 years
discussion is warranted. There is also the situ- thereafter [21].
History and physical:

A rectal polyp or mass
digital rectal exam
rigid proctoscopy
Minimally
Obstructed?
symptomatic
Pedunculated or Site marking (tattoo)

Fecal diversion Sessile ³ 2cm
sessile < 2cm TRUS or MRI rectal protocol
Stent
Polypectomy Rigid proctoscopy

site marking biopsy
Adenocarcinoma
C
Margin Margin positive Pathology uncertain
negative Adenocarcinoma
or unclear
CEA
CBC
LFT EMR
transanal
Surveillance excision
D Stage?
• TRUS
• MRI Rectum
• CT Chest, Abdomen,
Pelvis
Algorithm 69.1
E I
F II III
G IV
H
T1 £1/3 luminal T2, large T1, high

circumference risk T1 Short course
Chemoradiation Chemotherapy
radiation (not T4) Resectable
metastases?
• Liver
• Lung
TME Transanal TME • Inguinal
excision nodes
Chemoradiation
Chemotherapy TME TME

High risk? T1 Sort course
Chemoradiation Chemotherapy
LVI radiation (not T4)
Poor differentiation
Mucinous
Signet ring
³T2
Chemotherapy
Chemoradiation TME Chemoradiation
I
Chemotherapy TME
Surveillance
Interview Survey. Cancer Epidemiol Biomark Prev.

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Rectovaginal Fistula
70
Algorithmic Approach ment of these fistulas can be a significant

challenge for the consulted surgeon. There is a
Rectovaginal fistula is an inappropriate commu- relatively limited body of evidence that guides
nication between the rectum and the vaginal workup and treatment of these patients; however,
vault, most commonly caused by obstetrical the data that has been accumulated can effec-
trauma suffered during childbirth. Approximately tively guide clinically sound judgment and ulti-
1% of women who experience third- or fourth- mately successful resolution. The Clinical
degree perineal lacerations during vaginal birth Practice Guideline Committee of the American
will develop a rectovaginal fistula [1]. Midline Society of Colon and Rectal Surgeons has pro-
episiotomy has also been associated with rela- vided guidance based on the available evidence
tively high rates of fistula formation [2]. Incidence to help guide the physician in the surgical man-
is notably higher in developing countries with agement of rectovaginal fistulas [6]. This chapter
limited access to professional obstetric care and will attempt to provide a systematic approach to
sterility being contributing factors [3]. the patient with a rectovaginal fistula.
Inflammatory bowel disease, specifically Crohn’s
disease, is associated with rectovaginal fistula A. History and physical exam should be focused
with an incidence of 9–10% being reported [4, 5]. on symptoms as well as past medical and sur-
Infections after perineal repairs, complications gical history that would place the patient at
from low anterior resection, pelvic radiation, higher risk for fistula development. Symptoms
perianal or rectal abscess, and malignancies of can be influenced by the size and underlying
pelvic organs all carry a risk of rectovaginal fis- etiology. Patients may present with com-
tula development [6]. The discovery and treat- plaints of recurrent vaginal irritation, feculent
vaginal malodor, and passage of gas through-
J. Kuckelman out the vaginal canal. Passage of stool through
Department of General Surgery, Madigan Army the vagina may be present when the patient is
Medical Center, Tacoma, WA, USA experiencing loose or softer stools. Issues
Uniformed Services University of the Health with stooling urgency and symptoms of
Sciences, Bethesda, MD, USA incontinence may also be present if there is
E. K. Johnson (*) concomitant sphincter dysfunction. Personal
Cleveland Clinic Foundation, Cleveland, OH, USA history should focus on obstetrical history
Department of Surgery, Division of Colorectal elucidating number of vaginal births that
Surgery, Hillcrest Hospital, Mayfield Heights, required episiotomy and/or repair of
OH, USA

lacerations. History of inflammatory bowel to decrease inflammation in this subset of

disease, need for pelvic radiation, and any patients [7]. When malignancy is suspected,
other pelvic surgery histories should be eluci- appropriate workup and treatment should be
dated during the interview. addressed first.
Physical exam that accurately locates recto- Management in the acute setting is not differ-
vaginal fistulae can be very challenging but is ent from other types of fistulae. Conservative
crucial to the decision-making process. Digital measures with a period of observation, sitz baths
rectal exam should focus on sphincter function and diet, and placement of a draining seton is
and may identify low fistulas with simple palpa- appropriate. Seton placement may aid in source
tion. Speculum exam may identify stool in the control, but importantly it will aid in the develop-
vagina. Direct visualization of a dimple or open- ment of a defined fibrotic tract, necessary for
ing may be seen on anoscopy and speculum eventual surgical repair.
exam. Acute inflammation or vaginitis may be
present. Alternatively, placement of a tampon C. Fistulae that are established, less than 5 mm
with injection of methylene blue into the rectum in width, low (just above dentate line or vagi-
has also been described. After 1 h, the tampon is nal fourchette), and simple (not involving
removed, and blue discoloration confirms a rec- sphincter and not associated with Crohn’s)
tovaginal fistula. may see spontaneous closure with conserva-
An exam under anesthesia may be neces- tive management [8]. There is no defined
sary to make the diagnosis in more challenging period of observation that is appropriate, but
cases. Many methods of discovery have been up to 50% may have complete resolution after
described. Beginning with positioning in the 6–9 months—Though it may be difficult to
lithotomy position, the vagina may be filled with get many patients to wait this long [9]. The
warm water and the rectum insufflated after the authors would recommend a waiting period
placement of a proctoscope. Dual visualization of at least 12 weeks, ensuring adequate source
with a speculum exam may reveal the fistula control, prior to attempting any surgical
tract with evidence bubbles in the vaginal vault. repair. As mentioned above, watchful waiting
Other methods include a similar technique using should also include patient education on the
hydrogen peroxide. These methods provide the benefits of a high fiber diet and sitz baths with
benefit of discovering exact location of the fis- or without use of bulking agents.
tula which may ultimately be necessary if surgi-
cal repair is required. When watchful waiting is unsuccessful, surgi-
cal treatment should be discussed. Further char-
B. Given the variable causes and presentations acterization of the fistula is crucial to surgical
of rectovaginal fistulae, the first step should planning. If needed, this may be accomplished
be determining whether the clinician is deal- using ancillary studies such as contrast enemas
ing with an acute or an established fistula. In and fistulography. Just as in the imaging of other
the acute setting, definitive repair should be fistulas, pelvic MRI can help characterize the
postponed and the focus should be on optimal location, tract, and evidence of additional fistulas
management of the underlying cause. When otherwise missed on physical exam [10].
abscess or frank pelvic sepsis is present, Endorectal ultrasound with or without the instil-
source control/drainage, resuscitation, and ment of hydrogen peroxide can characterize the
antibiotic courses should be completed pri- fistula tract in addition to measuring the width of
marily. In the case of Crohn’s disease, opti- the perineal body as well as the state of the anal
mal medical management should be the focus. sphincter muscles [11, 12]. It is the authors’
Metronidazole is the antibiotic of choice in experience that most fistulas can be localized by
Crohn’s patients as it has been reliably shown simple exam under anesthesia allowing
70 Rectovaginal Fistula 285
differentiation between low and high fistulas mation is in danger of being created too low,
which are managed quite differently. a reverse of the classic operation should be
performed cranially using the anoderm as the
D. As previously mentioned, in patients who
flap [21].
have low, simple rectovaginal fistulas, con- E. If sphincter damage is extensive strictly due
servative management may be attempted to obstetrical trauma or prior abscess, then a
prior to surgical intervention. However, when episioproctotomy may be beneficial. This
operative repair is necessary, an endorectal operation involves transperineal takedown of
advancement flap is the preferred first surgi- the fistula tract and complete reconstruction
cal step. A repair on the rectal side is sup- of the rectovaginal septum. Studies have
ported by the fact that the rectum is the found that this operation results in high rates
higher-pressure cavity and thus closure and of fistula closure as well as excellent func-
protection of the closure on the rectal side tional outcomes with appropriately selected
have the best chance for long-term success. patients [22–25]. This approach is analogous
This operation is accomplished through rais- to a classically described sphincteroplasty for
ing a partial thickness flap on the rectal side fecal incontinence resulting from an anterior
of the fistula, closing the fistula primarily on sphincter defect. This approach is useful only
the rectal side and advancing the flap cau- in low-lying rectovaginal fistulae. Anterior
dally to the extent that healthy mucosa is cov- sphincter disruption should be confirmed
ering the now closed fistula tract. The excess based on physical exam and an imaging
distal mucosa is then excised. This operation modality—Typically endoanal ultrasound—
has the added advantage of anal sphincter Prior to undertaking this procedure.
repair, when necessary with a sphinctero- F. With recurrent rectovaginal fistulas or those
plasty. Success rates range from 78% to 100% in complex situations such as those in patients
in patients who have simple fistulas due to with prior radiation or Crohn’s disease, the
obstetrical injury [13–16]. These success next best option is using tissue interposition
rates may drop to just below 50% in patients of either the gracilis muscle or the bulbocav-
who have Crohn’s disease or other complex ernosus (Martius flap). Studies of these meth-
etiologies [14, 17–19]. Advancement flaps ods are relatively limited to small retrospective
have also been shown to have relatively high studies; however, the largest series available
rates of success in recurrence as well with evaluating 24 patients found the gracilis flap
some studies reporting over 90% success in to be successful in nearly 80% of patients
cases where prior attempts have failed [15, overall [26–28]. Similar results have been
18, 20]. No studies have shown improved out- shown for patients undergoing bulbocavern-
comes with fecal diversion in conjunction ousus flaps [29–31]. Studies completing these
with endorectal advancement flaps [18, 20]. operations in combination with fecal diver-
When fecal incontinence or sphincter dys- sion have shown the highest rates of success.
function secondary to an anterior sphincter Unfortunately, for patients with Crohn’s dis-
defect is associated with rectovaginal fistula, ease, there is a wide range of reported success
Tsang and colleagues showed that sphincter- from 30% to 100%. In Crohn’s disease related
oplasty improves success rate with 84% of fistulae, it is essential to have adequate medi-
women having successful repairs with sphinc- cal control of disease locally. Any repair
teroplasty versus 33% in women who have undertaken in the setting of active Crohn’s-
flaps created without sphincteroplasty [16]. related inflammation will fail. Patients with
Finally, continuous anal mucosal discharge ongoing active Crohn’s disease related procti-
due to a flap that has been brought too low tis should be managed either nonoperatively
over the anoderm is an avoidable complica- with a draining seton or via fecal diversion or
tion of this operation. In cases when flap for- proctectomy based on severity of symptoms
and patient desire. If a gracilis or Martius flap to debride the involved structures back to
repair is being undertaken in the setting of a healthy tissue prior to closure.
recurrent fistula, it would be wise to perform
proximal fecal diversion prior to or as a part In some cases, repair of high fistulae may
of this procedure. require proctectomy with coloanal reconstruction.
G. Patients are considered to have high recto- If this is undertaken, the anastomosis should be
vaginal fistulas if the vaginal opening of the protected with a proximal stoma—typically a loop
tract is near the cervix or vaginal cuff in the ileostomy. These may be required for a patient
setting of prior hysterectomy. High fistulas with complex causes for their rectovaginal fistula
can be very difficult to diagnose, but if they to include prior radiation. Success in approxi-
are due to a benign etiology, it is typically mately 75% of patients has been reported for proc-
from a prior operation such as a hysterectomy tectomy with coloanal anastamosis [33–35].
or proctectomy. In the setting of fistula for-
mation that is not associated with a colorectal H. Rectovaginal fistulization resulting from

anastomosis, the first option is to perform a anastomotic leak may occur in up to 10% of
fistula takedown through an abdominal women who have a proctectomy with recon-
approach either using open or laparoscopic struction for any reason. This should be
technique. When the tissue planes are sepa- treated with fecal diversion primarily as over
rated and the tracts closed, interposition of one third of patients may see resolution at
vascularized tissue, such as the omentum or 6 months [36]. If fecal diversion fails to
other biologic materials, can be placed resolve the fistula, then previously discussed
between the two tissues [32]. It is important options can be explored.
70 Rectovaginal Fistula 287

-Vaginal malodor
-Fecal soiling
-Incontinence
-Vaginal passage of gas or stool
-Digital rectal exam
-Anoscopy
A. -Vaginal exam
-Exam under anesthesia
Observe
Small (5mm)
B. Acute Chronic
low
sitz baths
high fiber diet
C. simple
Ancillary studies
-Fistulography
Observe -Transrectal ultrasound
sitz baths -Pelvic MRI
High fiber diet
Treat underlying
cause
G.
Low
Draining seton High
No Abdominal approach
Sphincter sphincter
involved involvement
From colorectal
anastomosis
D. Fistula takedown
H.
E. Episioproctotomy Endorectal advancement
Observe Failure
flap w/wo sphincteroplasty
Sitz baths
High fiber diet
Treat underlying
cause Proctectomy
Recurrent
or
F. Complex
Seton
Reconstruction Fecal diversion

with muscle flap
Algorithm 70.1
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las that has failed previous repair attempts. Dis Colon
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Rectum. 1995;38(9):921–5.
inflammatory bowel disease. World J Gastroenterol.
26. Lefevre JH, et al. Operative results and qual-

2016;22(3):1078–87.
ity of life after gracilis muscle transposition for
8. Rothenberger DA, Goldberg SM. The manage-
recurrent rectovaginal fistula. Dis Colon Rectum.
ment of rectovaginal fistulae. Surg Clin North Am.
2009;52(7):1290–5.
1983;63(1):61–79.
27. Wexner SD, et al. Gracilis muscle interposition for the
9. Rahman MS, et al. Surgical treatment of rectovagi-
treatment of rectourethral, rectovaginal, and pouch-
nal fistula of obstetric origin: a review of 15 years’
vaginal fistulas: results in 53 patients. Ann Surg.
experience in a teaching hospital. J Obstet Gynaecol.
2008;248(1):39–43.
2003;23(6):607–10.
28. Hotouras A, et al. Gracilis muscle interposition for
10. Sheedy SP, et al. MR imaging of perianal Crohn dis-
rectovaginal and anovaginal fistula repair: a system-
ease. Radiology. 2017;282(3):628–45.
atic literature review. Color Dis. 2015;17(2):104–10.
11. Sudol-Szopinska I, Jakubowski W, Szczepkowski

29. Pitel S, et al. Martius advancement flap for low rec-
M. Contrast-enhanced endosonography for the
tovaginal fistula: short- and long-term results. Color
diagnosis of anal and anovaginal fistulas. J Clin
Dis. 2011;13(6):e112–5.
Ultrasound. 2002;30(3):145–50.
30. Songne K, et al. Treatment of anovaginal or rectovag-
1 2. Dwarkasing S, et al. Anovaginal fistulas: evalu-
inal fistulas with modified Martius graft. Color Dis.
ation with endoanal MR imaging. Radiology.
2007;9(7):653–6.
2004;231(1):123–8.
31. Kniery KR, Johnson EK, Steele SR. Martius flap for
13. Lowry AC, et al. Repair of simple rectovaginal fistu-
repair of recurrent rectovaginal fistulas. Dis Colon
las. Influence of previous repairs. Dis Colon Rectum.
Rectum. 2015;58(12):1210.
1988;31(9):676–8.
32. van der Hagen SJ, et al. Laparoscopic fistula exci-
14. Mazier WP, Senagore AJ, Schiesel EC. Operative

sion and omentoplasty for high rectovaginal fistulas:
repair of anovaginal and rectovaginal fistulas. Dis
a prospective study of 40 patients. Int J Color Dis.
Colon Rectum. 1995;38(1):4–6.
2011;26(11):1463–7.
15. Sonoda LA, et al. Novel application of a fecal manage-
33. Nowacki MP. Ten years of experience with Parks’
ment system for vaginal use in radiotherapy-associated
coloanal sleeve anastomosis for the treatment of post-
rectovaginal fistula. Palliat Med. 2013;27(1):91–3.
irradiation rectovaginal fistula. Eur J Surg Oncol.
16. Tsang CB, et al. Anal sphincter integrity and function
1991;17(6):563–6.
influences outcome in rectovaginal fistula repair. Dis
34. Patsouras D, Yassin NA, Phillips RK. Clinical out-
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36. Kosugi C, et al. Rectovaginal fistulas after rectal can-
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19. El-Gazzaz G, et al. Analysis of function and predic-
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tors of failure in women undergoing repair of Crohn's
Management of Hemorrhoids
71
Matthew Z. Wilson and Joseph R. Notaro
Algorithmic Approach colorectal malignancy may undergo office

flexible sigmoidoscopy and hemoccult test-
A. “Hemorrhoids” are a very common com-
ing. Patients at higher risk (age, family his-
plaint. Patients will relate varying symptoms tory, genetic predisposition) should undergo
including rectal pain or pressure, prolapsing full colonoscopy.
tissue that reduces spontaneously or manu- After a trial of medical management,
ally, difficulty maintaining hygiene (“seep- patients should be reevaluated to assess for
age”), or general perineal discomfort. symptomatic improvement. Patients who do
Examination of the perineum includes a not improve should be considered for office-
visual inspection, digital rectal examination, based treatments. Banding is the most effec-
and anoscopy/rigid proctoscopy. Hemorrhoids tive and can be repeated. It is best to avoid
should be graded (I–IV), and other disease banding multiple internal hemorrhoids at the
processes such as abscess, fistula, or condy- same time, and patients should not be taking
loma should be ruled out. aspirin or blood thinners at the time of the
B. Classify hemorrhoids according to location. procedure or for the following 7 days.
Internal hemorrhoids are insensate and above Sclerotherapy with 5% phenol in vegetable
the anal verge. External hemorrhoids are dis- oil and infrared coagulation can be useful in
tal to the anal verge and covered by squamous patients who cannot be without anticoagula-
epithelium. The former are responsible for tion, though the efficacy is inferior to
painless bleeding, whereas the latter for pain banding.
and/or irritation. D. External hemorrhoids may present with

C. Bleeding per rectum should not be primarily thrombosis. This is exquisitely painful but
ascribed to hemorrhoids without ruling out will resolve with time and nonsurgical man-
other potential causes. Patients at low risk of agement. In the early acute phase (24–72 h),
the clot may be excised under local anesthe-
M. Z. Wilson (*) sia in the office setting. After 96 h, the pain
Department of Surgery, Dartmouth Hitchcock has generally begun to reduce, and drainage
Medical Center, One Medical Center Drive,
will be less effective. Erosion of clot through
Lebanon, NH, USA
e-mail: [email protected] the epithelium can occur in later stages, and
this should be excised under local anesthesia.
J. R. Notaro
Department of Surgery, Rutgers Robert Wood In the absence of thrombosis, excision of
Johnson Medical School, New Brunswick, NJ, USA external hemorrhoids should be avoided;

290 M. Z. Wilson and J. R. Notaro
hygiene or local irritation or excoriation along with complications such as stricture,

concerns can be addressed with topical
stenosis, fissure, and even incontinence.
hydrocortisone, zinc-oxide-based cream, and Grade IV hemorrhoids are a surgical emer-
barrier gel or powder. Minimization of cof- gency; use of hyaluronidase combined with
fee, spicy foods, hot showers, and harsh soaps local anesthetic agents will allow reduction of
will help reduce the potential for irritation. the prolapsed tissue; any external hemorrhoid
E. Surgical hemorrhoidectomy should only be thrombosis can then be excised. Prolapsing
entertained when all other attempts at treat- internal components can be banded.
ment have failed due to the potentially severe Otherwise, excisional hemorrhoidectomy can
discomfort and protracted recovery period be performed in the operating room.
71 Management of Hemorrhoids 291
History: symptoms vary but frequently include (alone or in

A combination) bleeding, prolapsing tissue, pain, difficulty
maintaining hygiene, or general discomfort.
Exam: visual inspection, digital rectal examination, anoscopy and/or

proctoscopy looking for thrombosis or other disease processes
mistakenly ascribed to
“hemorrhoids”
B External
Internal or Internal
external
C
D Thrombosis? Bleeding
symptoms?
Yes
No
Endoscopy to
24–72 hrs, may Dietary modification
Excoriation and rule out
excise under local with increased fiber
induration may alternative
anesthesia to uptake, and avoidance
be treated with pathology
relieve symptoms. of straining with
topical therapy
defecation. May
>96 h, Abnormal Normal consider emollient or
supportive hydrocortisone
therapy suppositories for
Treat Symptoms symptomatic relief.
accordingly resolve
No improvement
Continue
conservative
therapy Consider office-based
Surgical hemorrhoidectomy should be reserved banding, sclerotherapy,
for patients with disease refractory to office or infrared coagulation
procedures, large external hemorrhoids, for grade I, II, or III
Continued symptoms
combined internal, and external hemorrhoids hemorrhoids
with grades III–IV prolapse. E
Doppler-guided
Excisional Stapled hemorrhoidopexy (PPH) hemorrhoidectomy/pexy,
hemorrhoidectomy useful for multiple quadrant particularly useful for
(open or closed) disease or circumferential patients with predominant
useful for single mucosal prolapse with minimal symptoms of bleeding with
quadrant disease external disease prolapsing hemorrhoids
Algorithm 71.1
Suggested Reading
Rivadeniera DE, et al. Practice parameters for the man-
agement of hemorrhoids (Revised 2010). Dis Colon
Rectum. 2011;54:1059–64.
Management of Anal Fissure
72
Algorithmic Approach Emollient suppositories can help to soothe

inflamed tissue. Warm sitz baths and topical
A. Patients with acute anal fissure present with lidocaine may also relieve pain.
pain during and after defecation. They may Acetaminophen/nonsteroidal antiinflamma-
notice bleeding onto the toilet tissue. tory drugs (NSAIDS) are helpful.
Questions regarding bowel habits, specifi- B. Continue nonoperative treatment for 4 weeks
cally constipation or straining with defeca- and reassess the patient. Patients with
tion, will help to confirm the diagnosis. As improved symptoms may continue topical
fissures can be exquisitely painful, it is therapy with fiber supplementation for an
acceptable to do a limited perianal exam to additional 4 weeks with good results.
evaluate for other causes of anal pain such as Persistent discomfort is an indication for
external thrombosed hemorrhoid or abscess. examination under anesthesia.
Persistent pain despite medical therapy is a C. The purpose of an examination under anesthe-
reason for examination under anesthesia. sia is twofold: to determine whether the fis-
Treatment is targeted toward controlling sure is typical or atypical and to render
constipation, alleviating pain, and healing the appropriate therapy. Typical fissures are
fissure. Fiber supplements and stool softeners located in the posterior midline in the majority
are crucial. Topical calcium channel com- of cases. However, anterior midline fissures
pounds or nitrates reduce spasm of the inter- and simultaneous anterior and posterior mid-
nal sphincter and encourage healing. Calcium line fissures do occur. Atypical fissures are
channel blockers (diltiazem or nifedipine) are located laterally in the anal canal. Multiple
preferred due to lower incidence of head- simultaneous fissures are also considered
aches associated with their use. Systemic atypical. The presence of an atypical fissure
absorption is minimal with topical use. should prompt careful evaluation for Crohn’s,
tuberculosis, syphilis, human immunodefi-
ciency virus (HIV), or hematologic malig-
M. Z. Wilson (*)
Department of Surgery, Dartmouth Hitchcock nancy. Regardless of location, the edges of the
Medical Center, One Medical Center Drive, fissure should be biopsied for pathological
Lebanon, NH, USA evaluation.
e-mail: [email protected] Surgical treatment of fissure consists of
K. B. Wilkins two approaches: botulinum toxin injection
Department of Surgery, Rutgers Robert Wood and sphincterotomy. Botulinum toxin injec-
Johnson Medical School, New Brunswick, NJ, USA

294 M. Z. Wilson and K. B. Wilkins
tion into the internal sphincter has similar is divided into the proximal extent of the fis-
rates of healing compared to topical therapy sure rather than into the dentate line as with
as a first line and shows slightly increased traditional sphincterotomy, has been advo-
rates of healing as a second line after a cated. Healing rates with this approach are
course of topical therapy. Lateral internal slightly less than traditional sphincterotomy,
sphincterotomy is the standard treatment for but the risk of incontinence is also less.
chronic fissures and is associated with higher Patients with significant anal stenosis are
healing rates compared to any other therapy good candidates for sphincterotomy. Patients
but carries a risk of incontinence. Recently with patulous anal tone may benefit from
“tailored” sphincterotomy, where the muscle flap procedures.
72 Management of Anal Fissure 295
A History: Primary symptom is anal pain with defecation. Pain

may last for several hours, +/– bleeding. The patient will often
relate a history of constipation and straining to defecate.
Exam: Acute fissure manifests as a linear tear in the anal mucosa, often just inside the anal verge.
Chronic fissures may have a hypertrophied papilla proximally, exposed internal sphincter at the
base and a skin tag distally. Frequently acute fissure will be exquisitely painful, and it is acceptable
to defer DRE until pain has improved. Persistent pain is an indication for exam under anesthesia.
First line therapy: Sitz baths (10 min in warm water) two times or more daily, psyllium fiber
supplementation (with plenty of water), and topical nitrates or calcium channel blockers. Emollient
suppositories may provide relief.
Continue topical Symptoms Symptoms

therapy for additional 4 RE-exam Examination under
weeks, long-term fiber in 4 weeks anesthesia
supplementation/diet
improve persist
modification
No Yes Treat internal sphincter

Typical
Biopsy with botox vs lateral
Fissure?
internal sphincterotomy
after assessing anal
tone. Debride edges of
lesion and biopsy.
Treat underlying cause

of fissure
Long-term fiber
supplementation/diet
modification
Algorithm 72.1
Suggested Reading
Stewart DB, et al. Clinical practice guideline for the
management of anal fissures. Dis Colon and Rectum.
2017;60:7–14.
Management of Perianal Abscess
and Fistula-in-Ano 73
Matthew Z. Wilson and Bertram T. Chinn
Algorithmic Approach intersphincteric abscess or a supralevator

abscess due to a proximal extension of an
A. Perianal and ischiorectal abscesses are sig- intersphincteric abscess. Drainage of these
nificantly more common than intersphincteric abscesses through the ischiorectal fossa may
or supralevator abscesses. They are generally result in a transsphincteric or suprasphinc-
amenable to drainage under local anesthesia teric fistula.
with a generous elliptical incision or catheter A supralevator abscess due to a proximal
drainage with a stab incision and a 10–14fr extension of a transsphincteric abscess should
Pezzer catheter. Incisions should be made as be drained from the ischiorectal fossa as tran-
close to the anal verge as possible without srectal drainage may result in an extrasphinc-
injuring the sphincter complex; this will pro- teric fistula.
vide the shortest fistula tract possible should Drainage of a supralevator abscess not due
one develop. Packing is generally unneces- to a crypto-glandular source (e.g., abdominal
sary as it may impede drainage. Packing for or pelvic) generally requires treatment of the
hemostasis is rarely required as a topical underlying source and possible operative or
hemostatic agent such as ferric subsulfate is interventional radiology (IR) drainage.
usually adequate to control raw surface bleed-
B. Following treatment of the acute abscess,
ing. Sitz baths twice daily will provide symp- some patients will have persistent drainage
tomatic relief. from the site of the previous abscess. The
Due to pain, intersphincteric and suprale- acute inflammation will be resolved, but a
vator abscesses may best be addressed in the small amount of granulation tissue may be
operating room, likely with general anesthe- present in the area, likely representing the
sia. Transrectal drainage is advised for an external opening of a fistula-in-ano. Probing
of these openings in the office setting is dis-
couraged due to significant patient discom-
M. Z. Wilson (*) fort and the possibility of disrupting the tract.
Department of Surgery, Dartmouth Hitchcock
Medical Center, One Medical Center Drive, If evidence of abscess is present, this should
Lebanon, NH, USA be treated again with drainage.
e-mail: [email protected] Examination under anesthesia is the best
B. T. Chinn way to delineate the course of a fistula tract
Department of Colon and Rectal Surgery, Rutgers and determine appropriate treatment. Inability
Robert Wood Johnson Medical School, to identify the internal opening in any fi stula
Edison, NJ, USA

298 M. Z. Wilson and B. T. Chinn
is reason to obtain a pelvic MRI to identify come more superficial, allowing for subse-
the tract. Although most causes of an abscess/ quent fistulotomy. If fistulotomy is not possi-
fistula are due to a crypto-glandular source, ble, then consider more advanced procedures
a full examination of the perianal skin, anal with the understanding that none are as effec-
canal, and rectum should be performed to tive as fistulotomy. These procedures include
evaluate for alternative causes of abscess and ligation of intersphincteric fistula tract
fistula such as malignancy or Crohn’s disease. (LIFT), plug procedures, and endorectal
C. Superficial fistulae, particularly those in the advancement flaps. Setons can remain in
posterior midline, are amenable to fistulot- place indefinitely to control sepsis if a defini-
omy; this is the most effective treatment for tive procedure cannot be performed or the
fistulae. Complex fistulae are generally best fistula is due to Crohn’s disease. Fistulotomy
approached in a staged manner, primarily to in Crohn’s-associated fistulae can result in
avoid major sphincter disruption. Placement prolonged or nonhealing wounds. In these
of a seton will allow the tract to drain and cases, evaluation by a surgeon with experi-
reduce local inflammation. Frequently, the ence in treating inflammatory bowel disease
presence of a seton will allow the fistula to be (IBD) is warranted.
73 Management of Perianal Abscess and Fistula-in-Ano 299
H&P: Varies, but may include fever, pain, tenderness,

erythema, draining sinus, a perianal mass or relatively normal
appearance but with deep seated pelvic pain.
Perianal or ischiorectal abscess will almost Intersphincteric and supralevator abscesses

always have a tender, fluctuant mass. A may have few external findings. It will
frequently have fluctuance on digital rectal
examination but pain may preclude DRE.
Incise and drain under local anesthesia with DRE, anoscopy or rigid sigmoidoscopy may
elliptical incision or stab incision with Pezzer be confirmatory and reveal internal opening.
(10–14Fr) catheter. Consider CT scan if diagnosis is unclear or
supralevator abscess suspected.
Antibiotics should be limited to patients with Exam under anesthesia (EUA) and
severe cellulitis, immune suppression, or transrectal drainage for intersphincteric
concomitant systemic illness (e.g., DM) abscess. Drainage of supralevator abscess
based upon etiology.
Fistula-in-ano
B Will often present as

persistent drainage
after treatment of
acute abscess
If fistula is intersphincteric
Simple Complex with diminished sphincter
Perform fistulotomy for EUA integrity, extrasphincteric,
superficial or
transsphincteric, proceed
intersphincteric fistula with
with a staged procedure.
minimal sphincter involved.
If tract is clearly identified with both internal and external openings,

If tract is unclear, consider place a non-cutting seton (silastic vessel loop or large (#1)
MRI fistulagram to polyester suture) to promote drainage and avoid recurrent abscess.
identify the tract.
After period of 6–16 weeks of seton in place, repeat EUA. If a

candidate, may perform a fistulotomy. Otherwise consider LIFT
D procedure, endorectal advancement flap, porcine or PTFE
plug procedures.
Algorithm 73.1
Suggested Reading
Steele SR, et al. Practice parameters for the management
of perianal abscess and Fistula-in-Ano. Dis Colon
Rectum. 2011;54:1465–74.
Management of Anal Cancer
74
Algorithmic Approach sarily indicated at this time as continued

response to treatment is frequently observed.
A. Patients with anal cancer present with various D. If a complete response is observed, perform
anorectal complaints. Symptoms vary, and digital rectal examination (DRE), anoscopy,
there are multiple risk factors. Detailed peri- and inguinal node palpation at the prescribed
anal exam is necessary to delineate the extent intervals. Annual imaging with computed
of the disease. Examination under anesthesia tomography (CT) of the chest, abdomen,
may be required to obtain adequate tissue and pelvis is recommended to survey for
biopsy and determine the location of the lesion. occult metastasis. Evidence of persistent
B. The location of the lesion is imperative with disease should be followed at a shorter inter-
regard to treatment. T1N0M0 well- val (1 month). If the lesion appears stable,
differentiated anal margin lesions may be then continue to observe and reevaluate at
locally excised with 1 cm margins. All other 3 months. At 6 months, any suspicious lesion
stages of both anal canal and anal margin can- should be biopsied. If disease progresses
cer should be evaluated for treatment with despite therapy, then biopsy the lesion and
chemoradiotherapy. Human immunodefi- repeat the staging workup.
ciency virus (HIV) testing is important, as E. After confirmation of persistent or progres-
confirmation of appropriate CD4 counts is sive disease after primary treatment, local
necessary prior to initiation of therapy. CD4 disease (including inguinal nodes) can be
counts <200 cells/mL are associated with treated with APR (with groin dissection if
higher treatment toxicity. necessary). Patients with metastatic disease
C. Approximately 12 weeks after completion of should be considered for systemic chemo-
therapy, the patient should be reexamined to therapy. Patients who develop metastatic dis-
determine the response. Biopsy is not neces- ease after salvage abdominoperineal resection
(APR) for recurrent or persistent local disease
should also be considered for systemic che-
M. Z. Wilson (*)
Department of Surgery, Dartmouth Hitchcock motherapy. Inguinal node recurrence (with-
Medical Center, One Medical Center Drive, out evidence of anal disease) can be treated
Lebanon, NH, USA with groin dissection and radiation if previ-
e-mail: [email protected] ous radiation field did not include the groin.
K. B. Wilkins Following surgery, surveillance continues as
Department of Surgery, Rutgers Robert Wood prescribed above.
Johnson Medical School, New Brunswick, NJ, USA

302 M. Z. Wilson and K. B. Wilkins
A
History: Patients will complain of a slow growing perianal mass, frequently mistaken for a “hemorrhoid”,
may be in the anal canal itself. Pain, puritis, and/or bleeding are common. Inguinal lymphadenopathy
may be present. Risk factors include history of other HPV-related disease, previous STD or HIV,
cigarette smoking, anoreceptive intercourse, history of solid organ transplant or
other immunosuppression.
Office exam: Anorectal examination and evaluation of inguinal nodes. Determine size of primary lesion
and location in relation to anal verge. May biopsy in office setting if patient will tolerate.
Gynecologic exam needed for female patients. Exam under anesthesia (if required): Biopsy of suspicious
lesions through anoscope or sigmoidoscope. Determine size of primary lesion.
Anal margin (anal verge

Anal canal B to 5cm circumference of
Location perianal skin)
of lesion
Obtain CT chest,
T2 or greater, any N, any M
abdomen and pelvis,
T/N/M
and pelvic MRI. PET-CT
stage
may be obtained. HIV
testing Primary treatment is T1N0M0
should be done. chemotherapy with
Local Well differentiated
radiation
Wide local excision

Evaluate 12 weeks after
completion of therapy Complete response
with exam + DRE and
anoscopy DRE and anoscopy q3-6mos
for 2yrs then q6mo up to 5
C years then annually. Annual
imaging for 5 years.
APR + groin dissection if
Persistent disease positive inguinal nodes
Continue to follow regularly,

Response biopsy suspicious lesions at
to 6mo E
therapy?
Local
Progressive disease disease
D
Rebiopsy, Restage Systemic chemotherapy
should be considered
for metastasis or
Metastatic disease
recurrence
following salvage
surgery
Algorithm 74.1
Suggested Reading
NCCN clinical practice guidelines in oncology anal carci-
noma version 2. 2017.
Steele SR, et al. Practice parameters for anal squamous
neoplasms. Dis Colon Rectum. 2012;55:735–49.
Management of Fecal
Incontinence 75
Matthew Z. Wilson and Suraj Alva
Algorithmic Approach atropine and opioids, while effective in slow-

ing intestinal transport, are associated with
A. Management of incontinence is multifactorial dependency and can cause constipation.
and should be individualized. Severity of fecal C. Anorectal physiology and endoanal ultrasound
incontinence can be measured with scoring testing will help to define the anatomic factors
instruments such as the Fecal Incontinence contributing to incontinence such as squeeze
Severity Index, St. Mark’s incontinence score, pressure and resting sphincter tone, rectal sensa-
and the Cleveland Clinic Florida Fecal tion, compliance, and capacity and evidence of
Incontinence Score. All are based on patient’s sphincter defect. Endoscopy should be performed
subjective experience, and none correlate to in patients who are presenting with specific
objective findings, and none are predictive of symptoms (diarrhea, bleeding, or obstruction) or
outcomes of the various treatment options. who meet general screening criteria. Magnetic
Given these limitations, the instruments are use- resonance imaging (MRI) defecography or fluo-
ful to measure response to treatment over time. roscopic examination is valuable to assess the
B. Initial management of incontinence begins coordination of pelvic floor musculature and elu-
with increasing fiber intake and reduction of cidate conditions such as paradoxical contraction
caffeine, sugar substitutes, lactose, and other of the puborectalis or pelvic descent.
bowel irritants. Skin care including barrier Biofeedback therapy is not invasive and should be
ointments and gentle soap can increase considered for failure of medical management
patient comfort. Medications may be useful in patients with voluntary sphincter contrac-
in instances of increased motility. Kaopectate tion. Sphincter reconstruction, performed by a
will absorb excess fluid, and cholestyramine colorectal surgeon, can improve continence in
will bind bile acids. Antidiarrheals such as the short term for patients with sphincter dis-
loperamide and diphenoxylate-atropine will ruption, but long-term improvement is not
slow intestinal motility. Diphenoxylate- assured. Sacral neuromodulation can be used in
patients with and without sphincter defects as
well as those with concomitant urinary and
M. Z. Wilson (*) fecal incontinence with good results. Patients
Department of Surgery, Dartmouth Hitchcock Medical
who have failed the above therapies or cannot
Center, One Medical Center Drive, Lebanon, NH, USA
e-mail: [email protected] pursue them due to personal preference or med-
ical comorbidities can have a stoma created.
S. Alva
Department of Surgery, Rutgers Robert Wood Patients who elect this option report improved
Johnson Medical School, Edison, NJ, USA quality of life in the majority of cases.
304 M. Z. Wilson and S. Alva
A History: Incontinence is defined as uncontrolled passage of feces or gas over

at least a 1-month duration in an individual at least 4 years of age who
previously achieved control. Common risk factors include: multiple
pregnancies, chronic diarrhea, previous anorectal surgery, neurologic disease,
obesity, rectal prolapse, and sphincter disruption due to birth or other trauma
Exam: External inspection and digital rectal exam; observe for signs of
patulous anus, fistulous opening or mucosal or full-thickness
prolapse. Assess rectovaginal septum for atrophy. Digital
examination may provide estimation of resting, squeeze pressure,
and/or pelvis floor coordination. Rule out impaction and overflow
incontinence. Use grading scale to determine severity of symptoms.
Dietary and medication

modification, Psyllium fiber
supplementation, and Kegel Improvement
exercises. Consider antimotility
agents.
No improvement
Worsening of Continue dietary

Anorectal physiology testing and modification and
ERUS based on risk factors and medication(s). No further
C clinical suspicion. +/– MRI or
symptoms
intervention
fluoro defecogram
No improvement
Refer to pelvic floor Improvement

physical therapy if dis-
coordination of pelvic
floor musculature
No improvement
Consider referral to colorectal

surgeon for evaluation for surgical Colostomy
intervention.
Algorithm 75.1
Suggested Reading
Paquette IM, et al. The American Society of Colon and
Rectal Surgeons’ clinical practice guideline for
treatment of fecal incontinence. Dis Colon Rectum.
2015;58:623–36.
Part X
Liver
Evaluation of Liver Nodule
76
Algorithmic Approach C. Labs: In addition to liver function tests,

patients with a liver mass should have serum
Diagnosis alpha-fetoprotein drawn. If elevated, con-
cern for hepatocellular carcinoma grows. To
A. History: In addition to a thorough history, evaluate for common metastases to the liver,
focus should be placed on symptoms such as serum carcinoembryonic antigen (CEA),
abdominal fullness or pain, early satiety, and cancer antigen 19-9 (CA19-9), and chromo-
weight loss. The patient should be evaluated granin levels should be drawn. Additionally,
for a history of anemia, rectal bleeding, serologic testing for hepatitis B virus (HBV)
malignancy, or liver disease, including jaun- surface antigen and hepatitis C virus (HCV)
dice, hepatitis, or cirrhosis. Patients should be antibody should be drawn to evaluate for
asked if they have used oral contraceptive hepatitis B virus and hepatitis C virus,
pills (OCPs) or anabolic steroids in the past respectively.
and how often they drink alcohol. Risk fac- D. Imaging: Typically, a tri-phasic abdominal
tors for viral hepatitis, including history of computed tomography (CT) scan with con-
blood transfusions, unprotected sex, and trast is obtained to evaluate a solid nodule.
intravenous drug use, should be assessed. It is However, a patient may present with a mass
also important to inquire about family history found incidentally on imaging for an unre-
of liver diseases. lated workup. In the event that the CT is non-
B. Physical exam: A full physical exam should diagnostic, a magnetic resonance imaging
be performed with focused attention on the (MRI) with gadolinium- based contrast is
abdominal exam. Particular attention should recommended.
be placed on stigmata of liver disease, such as
jaundice, a palpable liver mass, palmar ery-
thema, spider angiomata and other varices, Etiology
and fluid wave or other signs of ascites.
E. Hepatocellular carcinoma (HCC): HCC is
primary liver cancer that typically occurs in
K. A. Mirkin · N. J. Gusani (*) the setting of chronic liver disease. Patients
Department of Surgery, Program for Liver, Pancreas, typically present with symptoms of chronic
and Foregut Tumors, Penn State College of Medicine, liver disease such as ascites, jaundice,
Penn State Cancer Institute, Hershey, PA, USA encephalopathy, and/or a history of variceal

308 K. A. Mirkin and N. J. Gusani
bleeds. The American Association for the as it is difficult to distinguish the classic cen-
Study of Liver Diseases (AASLD) has pub- tral stellate scar and lesion itself can appear
lished an algorithm for evaluating liver nod- hyper, hypo, or isoechoic [3]. On noncontrast
ules based on underlying liver disease and CT, FNH appears hypodense relative to the
lesion size. A mass found in the setting of surrounding tissue. The central stellate scar
hepatitis B or cirrhosis is more likely HCC is only identified in one third of patients [4].
[1]. Nodules under 1 cm should be followed Once contrast is injected, there is rapid tran-
by ultrasound (US) every 3–6 months. If, sient enhancement in the arterial phase, and
after 2 years, there has been no growth, rou- then the lesion is isodense during the portal
tine surveillance can commence. For nodules phase. FNH appears as an isodense lesion on
>1 cm, MRI or contrast CT is needed for fur- T1-weighted images and can be hyperintense
ther characterization. Tissue biopsy should on T2-weighted images. The central stellate
only be pursued when two diagnostic imag- scar enhances on delayed images. Recently,
ing studies are inconclusive. On US, HCC MR imaging using gadobentate dimeglu-
typically appears as a hyperechoic mass with mine – eliminated via renal and hepatobiliary
poorly defined, irregular margins. On CT, excretion – has been used to differentiate FNH
HCC tumors demonstrate increased vascular- from hepatic adenomas. FNH lesions, com-
ity during the hepatic arterial phase and prised of hepatic cells, appear isointense with
washout during the delayed phases. HCC this substance. Because FNH derives from a
appears as a low-intensity lesion on polyclonal proliferation of hepatic cells, FNH
T1-weighted images and a high-intensity lesions show equal or greater uptake of the
lesion on T2-weighted images. LI-RADS tracer on Tc-99 sulfur colloid scans.
imaging criteria can help determine whether a H. Hepatic adenoma: Hepatic adenomas are
lesion is consistent with or suspicious for classically found in the right lobe of young
HCC by CT or MRI. women (20–40) and are associated with the
F. Metastatic disease: Several malignancies use of steroids and oral contraceptives
have a propensity to metastasize to the liver. (OCPs). They tend to present with epigastric
In fact, metastases are far more common in or right upper quadrant abdominal pain.
Western countries than primary liver tumors Hepatic adenomas derive from monoclonal
[2]. Metastatic disease from the colon, stom- proliferations of hepatocytes. Thus, they
ach, and pancreas should be ruled out when carry the potential for malignant transforma-
evaluating a liver nodule. On CT, metastatic tion in addition to a risk of bleeding and rup-
lesions typically demonstrate lower attenua- ture. Hepatic adenomas findings on ultrasound
tion relative to the liver parenchyma. Much are relatively nonspecific and can appear as
like HCC, metastatic lesions appear as low- hyperechoic and well circumcised or hetero-
intensity lesions on T1-weighted images and geneous. Hepatic adenomas appear as well-
high-intensity lesions on T2- weighted demarcated lesions on CT and may have
images. areas of intratumoral hemorrhage. On con-
G. Focal nodular hyperplasia (FNH): FNH, trast CT, adenomas show peripheral enhance-
the second most common benign hepatic ment during early phase and centripetal flow
mass, typically occurs in women and pres- during the portal venous phase. It should be
ents asymptomatically. It carries no malig- noted that hepatic adenomas share many
nant potential and is often found incidentally. diagnostic features, and it is important to dif-
FNH derives from a polyclonal proliferation ferentiate the two as they are managed differ-
of all liver components, including Kupffer ently. On MRI, adenomas appear well
cells. Classically, this lesion demonstrates demarcated and are usually hyperintense on
the pathognomonic central stellate scar. US is T1-weighted images. Adenomas have a small
often of limited use in the diagnosis of FNH, number of nonfunctional Kupffer cells. Thus,
76 Evaluation of Liver Nodule 309
adenomas do not take up technetium Tc-99 m appear similar. Hepatic hemangiomas appear
sulfur colloid and produce a cold spot on the as a well-demarcated hypodense lesion on
liver during this scan. CT. Contrast CT classically demonstrates
I. Hepatic hemangioma: Hepatic hemangiomas peripheral to central enhancement. On MRI,
are the most common benign hepatic mass. hepatic hemangiomas typically appear as a
Most are asymptomatic and present inciden- well-demarcated hypodense mass with a low
tally on imaging or laparoscopy for an unre- signal intensity on T1 and a high signal inten-
lated condition. When they do produce sity on T2. On tagged red blood cell (RBC)
symptoms, they typically present with upper studies, hepatic hemangiomas show an initial
abdominal pain and fullness. On ultrasound, hypoperfusion during arterial flow, followed
hepatic hemangiomas usually appear as well- by retention of the tracer on delayed images.
demarcated homogenous lesions. They are Tagged RBC scans offer the greatest specific-
hyperechoic and blood flow can be demon- ity for diagnosing hepatic hemangiomas
strated with color Doppler, though this has (~100%) [6]. Fine-needle aspiration biopsy
not been shown to improve diagnostic accu- (FNAB) is not currently recommended for
racy [5]. It is important to note that hepatocel- suspected hemangioma, as it poses severe
lular carcinoma and hepatic metastases can bleeding risks.
A
History: history of fullness/pain, early satiety, weight loss,history of malignancy or hepatitis, anemia,
rectal bleeding, jaundice, cirrhosis, use of OCPs, alcohol use
B Physical exam: palpable liver mass, palmar erythema, spider angiomata, jaundice, and as cites
C Laboratory studies: CBC, chem, LFTs, serum AFP, CEA, CA19-9, chromogranin A, HBV, HCV
D Imaging: triphasic abdominal CT scan with contrast. If non-diagnostic, obtain MRI with gadolinium-
based contrast.
Risk factors Yes E

Concern for HCC
for HCC?
No
Elevated tumor
Yes Concern for metastatic F
makers, weight loss,
disease
history of
malignancy?
No
G
Central stellate Yes

FNH
scar on CT?
H
Hepatic adenoma:
peripheral enhancement
No during early phase,
centripetal flow during
portal venous phase
Enhancement
on CT?
I
Hepatic hemangioma:
peripheral to central
enhancement
Algorithm 76.1
76 Evaluation of Liver Nodule 311
References 4. Shamsi K, De Schepper A, Degryse H, Deckers

F. Focal nodular hyperplasia of the liver: radiologic
findings. Abdom Imaging. 1993;18(1):32–8.
1. Bruix J, Sherman M. Management of hepatocellular
5. Perkins AB, Imam K, Smith WJ, Cronan JJ. Color
carcinoma: an update. Hepatology (Baltimore Md).
and power Doppler sonography of liver hemangio-
2011;53(3):1020–2.
mas: a dream unfulfilled? J Clin Ultrasound : JCU.
2. Paley MR, Ros PR. Hepatic metastases. Radiol Clin N
2000;28(4):159–65.
Am. 1998;36(2):349–63.
6. Tamm EP, Rabushka LS, Fishman EK, Hruban
3. Cherqui D, Rahmouni A, Charlotte F, et al.
RH, Diehl AM, Klein A. Intrahepatic, extramed-
Management of focal nodular hyperplasia and hepa-
ullary hematopoiesis mimicking hemangioma on
tocellular adenoma in young women: a series of
technetium-99m red blood cell SPECT examination.
41 patients with clinical, radiological, and patho-
Clin Imaging. 1995;19(2):88–91.
logical correlations. Hepatology (Baltimore Md).
1995;22(6):1674–81.
Cystic Diseases of the Liver
77
Laura M. Enomoto and Niraj J. Gusani
Algorithmic Approach contact with infected dogs. When the para-

site reaches human liver parenchyma, it
A progresses to a cystic larval phase, which
can develop into a hydatid cyst within weeks
Clinical Presentation to months.
Cysts of the liver include hydatid cysts, simple 2. Simple cysts: Unknown. An association
cysts, polycystic liver disease (PCLD), von between simple hepatic and renal cysts has
Meyenburg complexes, Caroli disease, cystade- been reported but not well explained.
nomas, and cystadenocarcinomas [1, 2]. Most 3. PCLD: Can be associated with autosomal
patients with cystic liver disease are asymptom- dominant polycystic kidney disease (ADPKD)
atic. Large cysts may cause abdominal pain, dis- or occurs alone. Both are autosomal domi-
comfort, and distension, but this must be nant, however, and their clinical course is
diagnosed with caution only after other possible identical [3].
causes of abdominal pain have been excluded. 4. Caroli disease: Dilation of the intrahepatic
Acute pain has been reported, largely related to bile ducts due to a long common pancreatico-
biliary obstruction due to large cysts. Patients biliary channel that causes reflux of pancreatic
may also present with jaundice and pruritus, but juice into the biliary tree leading to inflamma-
cholangitis is rare. Caroli disease may progress to tion, ectasia, and dilation.
recurrent cholangitis and portal hypertension 5. von Meyenburg complexes: Also known as
with variceal bleeding or liver failure. biliary hamartomas, they are due to abnor-
mal development of small intrahepatic bile
Etiology ducts.
1. Hydatid cysts: Form due to infection caused 6. Cystadenoma: Hypothesized that tumors form
by Echinococcus, a parasite carried by due to ectopic ovarian cells migrating to the
sheep and dogs. Humans become infected liver during embryonic development, releas-
after eating contaminated food or by close ing hormones and growth factors causing
endodermally derived epithelium to prolifer-
ate to form a tumor.
L. M. Enomoto · N. J. Gusani (*) 7. Cystadenocarcinoma: Unknown. It is usually
Department of Surgery, Program for Liver, Pancreas,
and Foregut Tumors, Penn State College of Medicine, assumed to represent the malignant degenera-
Penn State Cancer Institute, Hershey, PA, USA tion of a cystadenoma. Risk of malignant
e-mail: [email protected] transformation is variable.

314 L. M. Enomoto and N. J. Gusani
Diagnosis simple cysts, cystadenomas, and cystadenocarci-

Demographics Simple cysts are uncommon nomas. Cyst aspiration should be performed with
before age 40, and the incidence is higher in caution as cystadenocarcinomas have a high pro-
women. von Meyenburg complexes are associ- pensity for peritoneal seeding.
ated with PCLD, Caroli disease, and congenital
hepatic fibrosis but can be seen in 6% of normal
livers. Cystadenomas with an ovarian stromal D
(OS) component are found almost exclusively in
women. bdominal Imaging (table in algorithm 77.1)
A
1. Hydatid cysts:
(a). US: first-line imaging in endemic regions.
B Cysts can be classified into six types
depending on the stage of growth. Early
Labs Liver function tests are generally normal stages are unilocular with thin cyst walls,
in all cystic disease. Alkaline phosphatase is ele- but as they progress through the lifecycle
vated in cystadenoma, helping to differentiate they become heterogenous with thick-
from simple cysts. Increase in serum carcinoem- ened walls and daughter cysts.
bryonic antigen (CEA) and CA 19-9 in cystade- (b). CT: well circumscribed lesions with clear
nocarcinoma is rare. membrane that do not invade surround-
ing liver tissue.
(c). MRI: low signal intensity rim on
Serologic testing If the patient is in a region T2-weighted images due to the collagen-
where echinococcal disease is endemic, serologic rich outer-laminated membrane of the
testing for parasitic infection should be per- cyst. Daughter cysts are hypointense rel-
formed, with antibody testing being the method ative to the parent cyst on T1-weighted
of choice. images and hyperintense on T2-weighted
images.
2. Simple cysts:
Genetic testing PCLD associated with (a). US: best diagnostic modality; anechoic
ADPKD is linked to mutations in PKD1 or circular or oval lesion with sharp, smooth
PKD2. Isolated PCLD is associated with muta- borders and posterior wall echoes, no
tions in PRKCSH, SEC63, or LRP5 genes. All septations
mutations have not yet been identified, however, (b). CT: water-dense lesions without
so genetic testing is not required for diagnosis. septations
There are no clinical guidelines to distinguish (c). MRI: hypointense on T1-weighted
PCLD from multiple simple cysts, but previous images and hyperintense on T2-weighted
case series have used five or six cysts or greater images with homogenous cystic content
than 50% involvement of the parenchyma, as 3. PCLD:
well as family history. (a). US: multiple fluid-filled round or oval
cysts with sharp margins
(b). CT: cysts with −5 to +20 Houndsfield
C units with distinct margins
(c). MRI: hypointense on T1-weighted
Cyst aspiration Cystic fluid does not discrimi- images and hyperintense on T2-weighted
nate between cystadenoma and cystadenocarci- images with homogenous cystic content
noma, and thus aspiration is usually not indicated. 4. Caroli disease:
Malignant or atypical cells are infrequently (a). US: hypoechoic intrahepatic biliary dila-
retrieved. CEA and CA 19-9 are increased in tions without septations
77 Cystic Diseases of the Liver 315
(b). CT: combine with intravenous (IV) chol- location, size, and number of cysts. In all cases,
angiography to identify contrast within spillage of cyst content should be avoided due to
the cyst and identify a communication the risk of anaphylaxis.
with the bile ducts
(c). MRI: multiple unilobar or cysts arising 1. Radical surgery: Cysts are injected with an
from segmental intrahepatic bile ducts. ethanol and hypertonic saline solution, which
Magnetic resonance cholangiopancrea- should remain in contact with the germinal
tography (MRCP) is the gold standard layer for at least 15 min. Cysts are then aspi-
for imaging. rated, and partial hepatectomy is performed.
5. von Meyenburg complexes: 2. Cystectomy: It does not require transection of
(a). US: range from hypoechoic to hyper- liver parenchyma. All cysts are surrounded
echoic or heterogenous based on size, with hypertonic saline-soaked packs and then
associated biliary dilation, and fibrous carefully aspirated, avoiding spillage.
stroma Hypertonic saline is then injected into the
(b). CT: hypodense with no enhancement cysts, carefully monitoring for hypernatremia.
(c). MRI: hypointense on T1-weighted This is aspirated after several minutes, and the
images, hyperintense on T2-weighted process is repeated. After the second aspira-
images. Best seen on MRCP tion, the cyst wall is resected and the entire
6. Cystadenoma: cavity washed with hypertonic saline.
(a). US: anechoic mass with echogenic inter- 3. Puncture, aspiration of cyst, injection of pro-
nal septations and papillary projections toscolicidal solution, reaspiration of fluid
into the cyst (PAIR): Fine aspiration needle is inserted into
(b). CT: multi-loculated hypodense mass cysts under image guidance. As much fluid as
with well-defined wall, fine septal calcifi- possible is aspirated, followed by injection of
cations. CT is less accurate than US and a protoscolicidal solution. After 15 min of
MRI as it may not visualize internal dwell time, the fluid is aspirated and the nee-
septa. dle withdrawn.
(c). MRI: multi-loculated cyst with high sig- 4. Medical management: For inoperable patients
nal intensity on T2-weighted images and with multiple cysts in two or more organs or
low signal intensity on T1-weighted peritoneal cysts. Mebendazole and albenda-
images. Contrast enhancement of thin zole are antihelminthic drugs that impair the
internal septa. parasite’s glucose uptake. Albendazole is the
7. Cystadenocarcinoma: drug of choice due to its superior gastrointes-
(a). CT: larger septa, cystic debris, bile duct tinal (GI) absorption. A 74% success rate has
dilation, coarse calcifications along the been shown in patients with single cysts
wall or septa, and enhancement of mural treated 3–6 months.
nodules
(b). MRI: hypointense cysts on T1-weighted Simple Cyst
images and hyperintense on T2-weighted 1. Asymptomatic simple cysts: no treatment or
images with cystic debris, calcifications, further surveillance
and bile duct dilation 2. Symptomatic cysts [4]:
(a). Sclerotherapy: destroys the epithelial lin-
Management ing of the cyst by US-guided injection of
95% ethanol
Hydatid Cyst (b). Fenestration: excision of the roof of the
Treatment is aimed at removing or destroying the cyst to establish a communication with
entire parasite and cavity as well as identifying the peritoneal cavity causing cyst cavity
and treating any biliary fistula, depending on the collapse
(c). Marsupialization: incision of the cyst Caroli Disease

wall and suturing the wall open so the Treatment depends on the distribution of cysts.
cyst remains open and draining If they are localized to one hepatic lobe or seg-
ment, hepatic resection with hepaticojejunos-
PCLD tomy is appropriate. If disease is diffuse, hepatic
transplantation offers the best chance of
1. Medical Management survival.
(a). Somatostatin analogs: There is limited
decrease in liver volume, and liver reverts von Meyenburg Complexes
to baseline when treatment is stopped. No treatment is required. These are often found
(b). Mammalian target of Rapamycin incidentally during surgery for carcinoma of
Inhibitors: longterm efficacy and safety another organ, causing the surgeon to biopsy for
studies still required. frozen section. Pathologists must be aware of the
(c). Arterial embolization: Cysts are largely existence of these complexes to avoid mistaking
supplied by hepatic arteries and emboli- them for metastases.
zation target segments replaced by cysts.
2. Surgical Management Cystadenoma
(a). Sclerotherapy: often ineffective because rigid Complete resection to prevent malignant trans-
architecture of the cysts prevents collapse. formation or recurrence, with consideration of
(b). Fenestration: excision of the roof of as intraoperative cholangiogram to exclude commu-
many cysts as possible. nication with the biliary tree.
(c). Partial hepatectomy: partial liver resec-
tion is possible if cysts are asymmetri- Cystadenocarcinoma
cally distributed. The remnant liver is Complete resection with wide margins, with
widely fenestrated. consideration of intraoperative cholangiogram
(d). Liver transplantation: only curative treat- as some tumors communicate with the biliary
ment. May require simultaneous renal tree. Hepaticojejunostomy may be required if
transplant in patients with ADPKD and the resection of the biliary confluence is
requires special exceptions in (MELD) required. Care should be taken not to rupture
scoring for those patients who do not the cyst as peritoneal carcinomatosis has
have concomitant liver failure. been reported.
77 Cystic Diseases of the Liver 317
History:
• Most patients with cystic liver disease are asymptomatic.

• Large cysts may cause abdominal pain, discomfort and distension, but this must be
diagnosed with caution only after other possible causes of abdominal pain have
been excluded.
• Acute pain has been reported, largely related to biliary obstruction due to large
cysts. Patients may also present with jaundice and pruritis, but cholangitis is rare.
• Caroli disease may progress to recurrent cholangitis and portal hypertension with
variceal bleeding or liver failure.
Laboratory:
• Liver function tests are generally normal in all cystic disease.

• Alkaline phosphatase is elevated in cystadenoma, helping to differentiate from simple cysts.
• Increase in serum CEA and CA 19-9 in cystadenocarcinoma is rare.
• If the patient is in a region where ecchinococcal disease is endemic, serologic testing for parasitic infection
should be performed, with antibody testing being the method of choice.
Cyst Aspiration:
• Cystic fluid does not discriminate between cystadenoma and cystadenocarcinoma and thus aspiration is
usually not indicated.
• Malignant or atypical cells are infrequently retrieved.
• CEA and CA 19-9 are increased in simple cysts, cystadenomas, and cystadenocarcinomas.
• Cyst aspiration should be performed with caution as cystadenocarcinomas have a high propensity for
peritoneal seeding.
Algorithm 77.1
D
Table: Abdominal imaging
Ultrasound CT MRI
Hydatid cyst Early stages are unilocular Well circumscribed lesions Low signal intensity rim on
with thin cyst walls, but as with clear membrane that do T2-weighted images due to
they progress through the not invade surrounding liver the collagen-rich outer
lifecycle they become tissue laminated membrane of the
heterogenous with thickened cyst. Daughter cysts are
walls and daughter cysts hypointense relative to the
parent cyst on T1-weighted
images and hyperintense on
T2-weightedimages
Simple cyst Best diagnostic modality; water dense lesions without hypointense on T1-weighted
anechoic circular or oval septations images and hyperintense on
lesion with sharp, smooth T2-weighted images with
borders and posterior wall homogenous cystic content
echoes, no septations
PCLD multiple fluid filled round or cysts with –5 to +20 hypointense on T1-weighted
oval cysts with sharp margins Houndsfield units with images and hyperintense on
distinct margins T2-weighted images with
homogenous cystic content
Caroli disease hypoechoic intrahepatic combine with IV multiple unilobar or cysts
biliary dilations without cholangiography to identify arising from segmental
septations contrast within the cyst and intrahepatic bile ducts.
identify a communication with MRCP is the gold standard
the bile ducts for imaging
von Meyenburg complexes range from hypechoic to hypodense with no hypointense on T1-weighted
hyperechoic or heterogenous enhancement images, hyperintense on T2-
based on size, associated weighted images. Best seen
biliary dilation, and fibrous on MRCP
stroma
Cystadenoma anechoic mass with multi-loculated hypodense multi-loculated cyst with high
echogenic internal septations mass with well-defined wall, signal intensity on T2-
and papillary projections into fine septal calcifications. CT weighted images and low
the cyst is less accurate than US and signal intensity on T1-
MRI as it may not visualize weighted images. Contrast
internal septa enhancement of thin internal
septa
Cystadenocarcinoma larger septa, cystic debris, hypointense cysts on T1-
bile duct dilation, coarse weighted images and
calcifications along thewall hyperintense on T2-weighted
or septa, and enhancement images with cystic debris,
of mural nodules calcifications, and bile duct
dilation
3. Everson GT, Taylor MR, Doctor RB. Polycystic dis-

References ease of the liver. Hepatology. 2004;40(4):774–82.
Epub 2004/09/24.
1. Jarnagin W, editor. Blumgart’s surgery of the liver, 4. Furuta T, Yoshida Y, Saku M, Honda H, Muranaka
biliary tract, and pancreas. 6th ed. Philadelphia: T, Oshiumi Y, et al. Treatment of symptomatic non-
Elsevier, Inc. parasitic liver cysts–surgical treatment versus alcohol
2. Hai S, Hirohashi K, Uenishi T, Yamamoto T, Shuto injection therapy. HPB Surg. 1990;2(4):269–77. dis-
T, Tanaka H, et al. Surgical management of cystic cussion 77-9. Epub 1990/10/01.
hepatic neoplasms. J Gastroenterol. 2003;38(8):759–
64. Epub 2003/09/25.
Management of Benign Liver
Masses 78
Algorithmic Approach B. Hepatic adenoma: Hepatic adenomas harbor

a malignant potential, in addition to a poten-
A. Hepatic hemangioma: Hemangiomas are tial for hemorrhagic rupture. Hemorrhagic
common in the general population, and the events most commonly occur in lesions ≥
use of medical imaging has further illumi- 5 cm. Thus, surgical resection should be con-
nated their prevalence. Hepatic hemangiomas sidered in large lesions.
follow a benign course, and conservative non- Symptomatic: Symptomatic patients should
operative management should be followed in undergo surgical resection.
most cases [1]. Arterial embolization: There is some evidence
Asymptomatic: Per the American College of that preoperative arterial embolization may
Gastroenterology clinical guidelines, there control hemorrhage and reduce mortality
is no intervention required for asymptom- [3]. Alternatively, in high-risk patients
atic hepatic hemangiomas regardless of unable to tolerate resection, arterial embo-
size [2]. lization may offer a viable option [2].
Symptomatic: Symptomatic patients with Surgical resection: Surgical management
impaired quality of life should be offered ranges from enucleation to resection to
surgical resection once other potential causes liver transplantation.
of symptoms have been excluded [1]. Asymptomatic: Oral contraceptive pills
Arterial embolization: In the event of acute (OCPs), anabolic steroids, and hormone
bleeding, arterial embolization is a viable containing intrauterine devices (IUDs)
treatment option. It can also be used to should be avoided per the American
reduce the size of the lesion preoperatively College of Gastroenterology [2].
or control symptoms. Pregnancy is not contraindicated. For
Surgical resection: Surgical management small adenomas <5 cm, discontinuation of
ranges from enucleation to liver OCPs or steroid use can be considered,
transplantation. with a follow-up with repeat imaging (CT
or MRI) in 6–12 months. If the lesion does
not resolve or increases in size after medi-
K. A. Mirkin · N. J. Gusani (*) cation changes have been made, surgical
Department of Surgery, Program for Liver, Pancreas, resection should be considered. For
and Foregut Tumors, Penn State College of Medicine; asymptomatic lesions ≥5 cm, surgical
Penn State Cancer Institute, Hershey, PA, USA resection should be considered to reduce

the risk of hemorrhage and malignant Observation: Because FNH does not carry a
degeneration [2, 4, 5]. malignant potential, this lesion should be
C. Focal nodular hyperplasia: FNH is the sec- managed nonoperatively. For women with
ond most common benign hepatic lesion. It FNH who continue to use OCPs, they
tends to follow a benign course, and nonop- should undergo annual ultrasound for
erative management should be followed in 2–3 years [5]. Follow-up imaging is not
most lesions [2]. necessary in patients not using OCPs.
A B C
Focal nodular
Hepatic adenoma Hepatic hemangioma hyperplasia
Yes
Yes Consider surgical Use of
Sympto
resection or Annual ultrasound OCPs?
matic?
arterial for 2–3 years
embolization
No
No
Yes
Yes Consider surgical

Sympto-
resection or arterial
≥5cm? matic?
embolization
No No
Discontinuation of OCPs and

steroids No intervention or follow-up No intervention or follow-up
Repeat imaging (CT or MRI) in necessary necessary
6-12 months
Algorithm 78.1
78 Management of Benign Liver Masses 321
References 3. Ault GT, Wren SM, Ralls PW, Reynolds TB, Stain
SC. Selective management of hepatic adenomas. Am
Surg. 1996;62(10):825–9.
1. Deneve JL, Pawlik TM, Cunningham S, et al. Liver
4. Terkivatan T, de Wilt JH, de Man RA, et al.
cell adenoma: a multicenter analysis of risk fac-
Indications and long-term outcome of treatment for
tors for rupture and malignancy. Ann Surg Oncol.
benign hepatic tumors: a critical appraisal. Arch Surg
2009;16(3):640–8.
(Chicago, Ill : 1960). 2001;136(9):1033–8.
2. Marrero JA, Ahn J, Rajender RK. ACG clinical guide-
5. Dokmak S, Paradis V, Vilgrain V, et al. A single-center
line: the diagnosis and management of focal liver
surgical experience of 122 patients with single and
lesions. Am J Gastroenterol. 2014;109(9):1328–47.
multiple hepatocellular adenomas. Gastroenterology.
quiz 1348.
2009;137(5):1698–705.
Hepatic Abscess
79
Jasvinder Singh and Niraj J. Gusani
Algorithmic Approach Patients may have prodromal symptoms of

weight loss, fatigue, malaise, fever, and anorexia
Epidemiology for many days before more specific symptoms
like right upper quadrant pain localize the pro-
The overall incidence of liver abscess in the cess. Patient may even present with bacteremia of
United States is estimated to be 3.6 per 100,000 unclear etiology.
population per year. Although the incidence and
mortality from liver abscess have gone down sig-
nificantly over the last century, its incidence Etiology
appears to be increasing in recent years [1, 2].
Amoebic liver abscess is the most common type 1. Pyogenic: Biliary, portal, arterial, or traumatic
of liver abscess worldwide, whereas in western origin. The etiology has changed over the last
series, pyogenic liver abscess predominates. 100 years, with the biliary route replacing
portal route as the most common cause of
pyogenic liver abscess [3]. Patients usually
A present with multiple abscesses, especially
when biliary in origin.
(a). Biliary: Result of biliary obstruction and
cholangitis from gallstones, biliary stric-
Clinical Presentation and Diagnosis ture or malignancy.
(b). Portal: Spread of infection from the gas-
The most common symptoms include fever trointestinal tract to the liver via the por-
(90%) and chills (50%), respectively, right upper tal vein. Examples include appendicitis,
quadrant pain and hepatomegaly with or without diverticulitis, perforated ulcers, perfo-
jaundice [2, 3]. Diagnosis is usually made after rated cancers, etc.
radiologic studies, e.g., ultrasound (US) abdo- (c). Arterial: Hematogenous spread of infec-
men or computed tomography (CT) scan. tion from a distant site, e.g., infectious
endocarditis.
J. Singh · N. J. Gusani (*) (d). Traumatic: Secondary infection of a trau-
Department of Surgery, Program for Liver, Pancreas, matic liver hematoma.
and Foregut Tumors, Penn State College of Medicine, 2. Amoebic: Usually solitary and in the right
Penn State Cancer Institute, Hershey, PA, USA lobe but can be multiple. It is uncommon in

324 J. Singh and N. J. Gusani
the United States but is important to consider

if there is history of travel to the tropical D
region, immunosuppression, human immuno-
deficiency virus (HIV), and corticosteroids.
3 . Malignant: Historically, malignant tumors
Serologic diagnosis Serologic testing for anti-
accounted for approximately 3% of liver
bodies to Entamoeba histolytica using indirect
abscess cases [4]. But with the increased use
hemagglutination and gel diffusion precipitation is
hepatic arterial interventions like transarterial
the best way to confirm diagnosis of amoebic liver
chemoembolization (TACE) and transarterial
abscess (85–95% sensitivity and specificity) [6].
radioembolization (TARE) with yttrium mic-
roparticles, it is not uncommon to see hepatic
abscess in patients with malignant tumors in
Culture The most commonly isolated organ-
the present era.
isms in those with positive blood culture include
Streptococcus species (29.5%) and E. coli
(18.1%) with polymicrobial cultures noted in
Diagnosis 16.3% [1]. Presence of Streptococcus faecalis
may indicate underlying colon cancer and should
B prompt evaluation for this [7].
Stool for ova and parasites The cysts of

Right upper quadrant US It can identify liver Entamoeba histolytica are found in the stool in
abscess, gallstones, biliary dilation from com- about 1/4th of the patients [6].
mon bile duct (CBD) stones.
CT scan Compared to ultrasound, CT scan has Management

slightly increased sensitivity (95% vs 90%) and
better anatomic localization for complex biopsies
and drainage procedures. The characteristics sug-
E
gestive of pyogenic liver abscess include contrast
enhancement in the periphery as opposed to non- moebic Liver Abscess
A
enhancement of the central portion during the (a). Medical Treatment
portal venous phase [5]. –– Metronidazole is the mainstay of treat-
ment. Dosage is 750 mg three times daily
for 7–10 days.
MRI Magnetic resonance imaging (MRI) is a –– Metronidazole treatment needs to be fol-
reasonable cross-sectional imaging modality but lowed with paromomycin (25–35 mg/kg/
does not have any advantage over CT for diagno- day in three divided doses for 7 days) or
sis or management of liver abscess. diloxanide furoate (500 mg three times
daily for 10 days) to eradicate amoeba in
the intestine.
–– Alternatives for metronidazole include
C chloroquine (600 mg daily for 2 days,
followed by 300 mg daily for 3 weeks)
with or without dehydroemetine.
–– Role of antibiotics: Antibiotics may be
Labs Elevated white blood cell (WBC) count,
needed in case of secondary infection of
anemia, elevated liver enzymes, hyperbilirubine-
amoebic liver abscess. Secondary infec-
mia, hypoalbuminemia
79 Hepatic Abscess 325
tion should be suspected if patient doesn’t otic therapy is needed, but duration of anti-
respond to metronidazole within a few biotic treatment should be individualized
days; usually aspiration of abscess would based on clinical response, etiology, and
be required to confirm this. number/extent of abscesses [3]. If there is
( b). Drainage significant clinical improvement, patients
–– Abscess drainage: Routine drainage of can be transitioned to oral antibiotics after
abscess is not needed. When needed, per- 2–3 weeks of intravenous antibiotics with
cutaneous needle aspiration (PNA) usu- equivalent results [9].
ally provides relief, and catheter (b). Drainage (percutaneous or internal):
placement may be avoided. Indications Drainage is essential to control sepsis and
for drainage include left-lobe abscess, may be needed to confirm diagnosis. Usually
impending rupture, multiple abscesses, percutaneous needle aspiration (PNA) alone
or abscess that does not respond to medi- is sufficient especially when the size of the
cal therapy within three to 5 days, unclear abscess is <5 cm. Percutaneous catheter
diagnosis [8]. drainage (PCD) should be done in abscesses
>5 cm or those that fail to resolve with PNA
alone [10]. PCD generally is more effective
than PNA for large abscesses, with a higher
F success rate and reduced time to achieve
clinical relief [11]. Occasionally, operative
intervention including drainage or resection
yogenic Liver Abscess
P of the affected liver is required.
(a). Antibiotics: Initially start with parenteral (c). Primary source control: Treatment of the
broad spectrum antibiotics (polymicrobial primary cause (e.g., appendicitis, diverticu-
infection is common), with adjustments per litis, biliary obstruction, etc.) is essential.
culture reports. Usually 4–6 weeks of antibi-
326 J. Singh and N. J. Gusani
Typical symptoms: fever, chills, right upper quadrant pain, and hepatomegaly with or without jaundice
Prodromal symptoms: Weight loss, fatigue, malaise, fever, and anorexia (may be the only symptoms)
Imaging findings: Fluid collection in liver (Single or multiple)
A
Clinical history
Pyogenic abscess: h/o gallstones/CBD stones, septic focus in GI tract (appendicitis,
diverticulitis etc.), h/o liver trauma, h/o HPB malignancy/ liver metastasis
Amoebic liver abscess: recent travel to the tropical region, immunosuppressed
B C
Imaging: US abdomen, CT scan (more sensitive), MRI
Labs: elevated WBC count, deranged liver function tests,
Findings: fluid collection (single or multiple)
hypoalbuminemia, amoebic serology
Associated pathology: gallstones, CBD stones, malignantlesion in liver
D Amoebic serology
Positive
Negative
F Pyogenic liver abscess

Amoebic liver abscess E
Send blood cultures - Metronidazole (750 mg thrice daily for 7-10 days).
- Followed with paromomycin (25-35 mg/kg/day in 3
divided doses for 7 days) or diloxanide furoate (500 mg
thrice daily for 10 days) to eradicate amoeba in intestine.
Start broad-spectrum antibiotics early – Antibiotics: may be needed if secondary infection.
Change later per antibiotic sensitivity
Drainage of abscess Drainage: No need for routine drainage.

<5 cm: PNA Indications: left-lobe abscess, impending rupture, multiple
>5 cm: PCD abscesses, or abscess that does not respond to medical therapy
Send for culture and sensitivity within 3–5days, unclear diagnosis
Treatment of primary cause

- Relieve biliary obstruction: ERCP or PTC, surgical drainage
- Appendicitis, diverticulitis, other infectious
intra-abdominal pathology
Algorithm 79.1
6. Haque R, Huston CD, Hughes M, Houpt E, Petri WA,

References Amebiasis J. N Engl J Med. 2003;348(16):1565–73.
7. Teitz S, Guidetti-Sharon A, Manor H, Halevy
1. Meddings L, Myers RP, Hubbard J, Shaheen AA, A. Pyogenic liver abscess: warning indicator of silent
Laupland KB, Dixon E, et al. A population-based colonic cancer. Report of a case and review of the lit-
study of pyogenic liver abscesses in the United States: erature. Dis Colon Rectum. 1995;38(11):1220–3.
incidence, mortality, and temporal trends. Am J 8. Sharma MP, Ahuja V. Amebiasis. N Engl J
Gastroenterol. 2010;105(1):117–24. Med. 2003;349:307–8. https://doi.org/10.1056/
2. Huang CJ, Pitt HA, Lipsett PA, Osterman FA Jr, NEJM200307173490323.
Lillemoe KD, Cameron JL, et al. Pyogenic hepatic 9. Ng FH, Wong WM, Wong BC, Kng C, Wong SY, Lai KC,
abscess. Changing trends over 42 years. Ann Surg. et al. Sequential intravenous/oral antibiotic vs. continuous
1996;223(5):600–7. discussion 7–9. intravenous antibiotic in the treatment of pyogenic liver
3. Pitt HA. Surgical management of hepatic abscesses. abscess. Aliment Pharmacol Ther. 2002;16(6):1083–90.
World J Surg. 1990;14(4):498–504. 10. Zerem E, Hadzic A. Sonographically guided percu-
4. Trump DL, Fahnestock R, Cloutier CT, Dickman MD. taneous catheter drainage versus needle aspiration in
Anaerobic liver abscess and intrahepatic metastases: the management of pyogenic liver abscess. AJR Am J
a case report and review of the literature. Cancer. Roentgenol. 2007;189(3):W138–42.
1978;41(2):682–6. 11. Cai YL, Xiong XZ, Lu J, Cheng Y, Yang C, Lin YX, et al.
5. Benedetti NJ, Desser TS, Jeffrey RB. Imaging of Percutaneous needle aspiration versus catheter drainage
hepatic infections. Ultrasound Q. 2008;24(4): in the management of liver abscess: a systematic review
267–78. and meta-analysis. HPB (Oxford). 2015;17(3):195–201.
Malignant Liver Tumors
(Metastatic Liver Disease) 80
Neal M. Mineyev, Karla M. Chaffee,
and Joyce Wong
Algorithmic Approach If the anticipated functional liver remnant is

inadequate, portal vein embolization can be
A. Many patients with metastatic liver disease offered to allow for hypertrophy of the rem-
are identified because of staging imaging or nant [1].
imaging obtained to evaluate specific con- C. If the primary lesion is not symptomatic, sys-
cerns, such as elevated liver function enzymes temic chemotherapy should be offered. In
or elevated tumor markers. Staging imaging colorectal cancer with an intact colorectal pri-
typically involves computed tomography mary cancer, for example, the majority of
(CT) scan, magnetic resonance imaging patients will not require palliative resection
(MRI), or positron emission tomography of the primary tumor during chemotherapy
(PET)/CT, depending on the primary tumor. [2]. These patients can be offered resection,
Hepatic disease should be noted, whether either in a combined or staged manner, if they
identified during initial staging of a cancer do not demonstrate disease progression fol-
(i.e., synchronous hepatic disease) or during lowing short-course systemic therapy. If the
surveillance for a cancer (metachronous). patient has resectable hepatic metastases and
Dedicated liver imaging should be obtained primary lesion, other options also include
to determine hepatic burden of disease, i.e., combined resection of the primary tumor
triple phase CT of the liver or MRI of the with hepatic metastasectomy, followed by
abdomen. The presence of extrahepatic dis- adjuvant systemic treatment, or even staged
ease must also be considered. resection with resection of the primary tumor
B. The tumor burden within the liver should be followed by hepatic metastasectomy [3]. This
defined: solitary metastasis vs. multiple approach is generally reserved for patients
metastases, single lobe vs. bilobar distribu- with oligometastatic hepatic disease or single
tion, and proximity to the hepatic veins, main hepatic metastasis. Resection of the hepatic
portal veins, or biliary tree. Resectability metastasis(es) is considered the treatment of
should be determined based on the functional choice and offers improved outcome vs. abla-
liver remnant and quality of liver remaining. tion [4]. In patients with unresectable liver
metastases, systemic chemotherapy should
be offered as initial treatment, followed by
N. M. Mineyev · K. M. Chaffee · J. Wong (*) restaging imaging. In patients with extrahe-
Department of General Surgery, Lenox Hill Hospital, patic disease in addition to the primary tumor,
New York, NY, USA systemic therapy is typically offered. Further

328 N. M. Mineyev et al.
treatment would be predicated upon disease be offered [3]. Interval follow-up with restag-
response to chemotherapy. ing imaging should be performed.
D. In those with persistent unresectable hepatic F. If patients are rendered disease free, they should
disease, without extrahepatic disease, liver complete the recommended systemic chemo-
directed therapies should be considered, therapy and be followed in surveillance with
including radioembolization (i.e., yittrium- interval physical examination, bloodwork and
90), transarterial chemoembolization, or
cross-sectional imaging. If disease progression
hepatic artery infusion pump [5, 6]. is demonstrated, third- line chemotherapy or
E. If the disease burden progresses following clinical trial should be considered. Surveillance
systemic chemotherapy, change to an alter- should be continued for 5 years [3].
nate systemic chemotherapy regimen should
80 Malignant Liver Tumors (Metastatic Liver Disease) 329
Hepatic mass identified on routine surveillance imaging

A Detected on workup for symptoms such as abdominal pain
Detected during staging for primary cancer, i.e., colon cancer
Obtain vital signs, and blood work and perform physical examination
Review imaging, obtain dedicated imaging, i.e. MRI, as needed
Status of primary malignancy must be defined
Solitary or mulitple
hepatic lesions?
B
Status of primary
cancer?
Resectable hepatic disease Resectable hepatic disease

Primary tumor intact, Multiple hepatic lesions,
Primary tumor intact,
symptomatic not resectable
asymptomatic
Consider resecting primary

tumor. Systemic therapy (i.e.,
If solitary liver lesion, may chemotherapy)
C perform metastasectomy in
same setting.
Restage with cross-sectional imaging
Multiple hepatic lesions, still

Resectable hepatic disease, unresectable
Progressive disease
stable or regressed
If primary tumor controlled,

E Surgical Resection of hepatic
Systemic therapy
consider liver-directed
metastases therapy:
(second line) D
Resect primary tumor if intact Y-90 radioembolization
TACE
Hepatic artery infusion pump
Algorithm 80.1
References 4. Abdalla EK, Vauthey JN, Ellis LM, et al. Recurrence

and outcomes following hepatic resection, radio-
frequency ablation, and combined resection/abla-
1. Covey AM, Brown KT, Jarnagin WR, et al. Combined
tion for colorectal liver metastases. Ann Surg.
portal vein embolization and neoadjuvant chemo-
2004;239:818–25.
therapy as a treatment strategy for resectable hepatic
5. Van Hazel GA, Heinemann V, Sharma NK, et al.
colorectal metastases. Ann Surg. 2008;247:451–5.
SIRFLOX: randomized phase III trial comparing first-
2. Poultsides GA, Servais EL, Saltz LB, et al. Outcome
line mFOLFOX6 (plus or minus bevacizumab) versus
of primary tumor in patients with synchronous stage
mFOLFOX6 (plus or minus bevacizumab) plus selec-
IV colorectal cancer receiving combination chemo-
tive internal radiation therapy in patients with meta-
therapy without surgery as initial treatment. JCO.
static colorectal cancer. JCO. 2016;34:1723–31.
2009;27(20):3379–84.
6. Zacharias AJ, Jayakrishnan TT, Rajeev R, et al.
3. NCCN Clinical Practice Guidelines in Oncology,
Comparative effectiveness of hepatic artery based
Colon Cancer. Version 2.2017 – March 13, 2017.
therapies for unresectable colorectal liver metastases:
NCCN.org.
a meta-analysis. PLoS One. 2015;10:e0139940.
Diagnosis and Management
of Hepatocellular Carcinoma 81
and Joyce Wong
Algorithmic Approach veins, or biliary tree. Resectability is deter-

mined based on the primary tumor character-
A.
Patients with hepatocellular carcinoma istics, functional liver remnant and quality of
(HCC) are typically male, between 50 and liver remaining as well as tumor location, lack
60 years of age, with a liver mass identified of portal hypertension, and bilirubin level [2].
on routine imaging for surveillance in hepati- C. If extrahepatic metastasis is identified, patients
tis B/C, with evaluation of cirrhosis, or found will generally receive palliative treatment with
incidentally. Patients may present with unex- sorafenib or other systemic therapy, regardless
plained weight loss, nausea, lethargy, or right of liver function or Child-Pugh class. If no
upper quadrant pain with/without a palpable extrahepatic metastasis is identified, then the
mass. Evaluation of these symptoms includes treatment of choice is complete excision of
routine laboratory studies including a liver HCC by resection or liver transplantation.
function panel and alpha-fetoprotein (AFP). Selection of the appropriate patients for resec-
Although AFP has a low sensitivity and spec- tion is crucial as the mortality after liver resec-
ificity for HCC, it is useful in monitoring tion can be as high as 20%, depending on the
treated patients for possible recurrence. patient’s preoperative liver function [3].
Dedicated liver imaging should be obtained D. Liver volumetry and Child-Pugh classification
to determine hepatic burden of disease with a are key elements to determine if the patient
triple phase CT of the liver or MRI of the will tolerate resection. Patients with Child-
abdomen [1]. Pugh class B or C cirrhosis or those with portal
B. Once radiologic evidence of HCC is con- hypertension typically do not tolerate resec-
firmed by arterial hypervascularity and venous tion. Taking location of tumor into account,
or delayed phase washout, the presence of there is no general consensus on tumor size for
extrahepatic metastasis must be carefully selection of patients for resection, although
evaluated. Additionally, the extent of tumor generally eligibility for resection is restricted
burden within the liver should be defined: to tumors that are ≤5 cm in diameter.
number of tumors, lobar distribution, and Locoregional therapies can be used for a vari-
proximity to the hepatic veins, main portal ety of patients with complex disease and those
who are not eligible for resection or transplan-
tation. Radiofrequency ablation (RFA) is typi-
N. M. Mineyev · K. M. Chaffee · J. Wong (*)
Department of General Surgery, Lenox Hill Hospital, cally used for unresectable Child-Pugh class A
New York, NY, USA or B, for tumors <4 cm, and as a bridge to

transplant. According to current National treated with orthotopic liver transplantation

Comprehensive Cancer Network (NCCN) (OLTx). An established guideline for OLTx
guidelines, RFA plus TACE (transarterial che- evaluation is the Milan criteria. However,
moembolization) has been recommended for some intuitions will apply the University of
intermediate-sized HCC from 3 to 5 cm. California San Francisco (UCSF) criteria
Radioembolization with yttrium-90 (Y-90) (single tumor ≤6.5 cm, or 2–3 lesions, none
particles has shown great success in recent tri- exceeding 4.5 cm, with total tumor diame-
als; however, its efficacy compared to other ter ≤8 cm, and no vascular invasion) as an
ablative therapies has yet to be thoroughly extended version of Milan criteria. For those
studied. Although controversial, small tumors patients whose disease falls within the crite-
in Child-Pugh class B patients with no portal ria, OLTx is the only treatment with or with-
hypertension, favorable peripheral location, out adjuvant “bridging” ablation therapy.
and adequate functional status may be able to Patients whose disease extends beyond the
tolerate resection or ablative therapy (RFA/ confines of the guidelines are treated with
TACE/Y-90/etc.) [4]. palliative care and sorafenib. In recent stud-
E. Patients who are unresectable due to underly- ies, a new systemic agent, regorafenib, has
ing severe liver dysfunction (Child-Pugh shown survival benefit in HCC patients who
class B/C) or with portal hypertension are have previously received sorafenib [5].
81 Diagnosis and Management of Hepatocellular Carcinoma 333
Liver mass identified on routine

imaging or detected on workup for
abdominal pain
Obtain vital signs, blood work, including AFP and physical exam
A Review imaging, obtain dedicated imaging i.e., triple-phase
contrast CT and/or MRI of abdomen
HCC identified
B
Staging of disease and
liver
functionality/cirrhosis
Select Child-Pugh class B

Resection if adequate functional
C Extrahepatic liver remnant
metastasis? +/–ablation, TACE, Y–90
Yes No
Palliative care Child-Pugh class

+/–sorafenib A/B/C
Child-Pugh class A Child-Pugh class B/C E
Resection if adequate functional

Liver transplant
D liver remnant
evaluation
+/–ablation, TACE, Y–90
Within milan F
Milan criteria* criteria?*
1 lesion ≤ 5 cm Yes No
3 lesions ≤ 3 cm
No extrahepatic metastatic invasion
Liver transplant listing Palliative Care
No evidence of vascular invasion +/–Sorafenib
+/–Neoadjuvant RFA/TACE/Y-90
Algorithm 81.1
References 4. Bruix J, et al. Prognostic prediction and treatment

strategy in hepatocellular carcinoma. Hepatology.
2002;35:519.
1. Townsend CR, Beauchamp MB, Evers M, Mattox
5. Bruix J, et al. Regorafenib for patients with hepa-
K. Sabiston textbook of surgery. 20th ed. Elsevier:
tocellular carcinoma who progressed on sorafenib
Philadelphia, PA. 2016. p. 1458–63.
treatment (RESORCE): a randomized, double-
2. Xavier A, et al. Usefulness of staging systems and
blind, placebo-controlled, phase 3 trial. Lancet.
prognostic scores for HCC treatments. World J
2017;389(10064):56–66.
Hepatology. 2016;8(17):703–15.
3. National Comprehensive Cancer Network, HCC;
v.1.2016.
of Primary Sclerosing Cholangitis 82
and Joyce Wong
Algorithmic Approach rubinemia is warranted, as ultrasound is not

the most sensitive diagnostic modality for
A. The first step in the evaluation of a patient intrahepatic biliary pathology and is very
with primary sclerosing cholangitis (PSC) is operator dependent. MRCP or ERCP should
a thorough history and physical. Patients may be performed at this point; however, ERCP
experience varying degrees of symptoms, will yield better results from both diagnostic
often long-standing, from 12 to 24 months and therapeutic perspectives [2].
that can include fatigue, jaundice with or D. Liver biopsy is not routinely performed in the
without pruritus, choluria, steatorrhea, and diagnosis of PSC. It is indicated when visual-
acute cholangitis. ization of the biliary ducts by MRCP has not
B. Evaluation of these symptoms includes rou- yielded any abnormal findings and other
tine laboratory studies including a liver func- causes of cholestatic liver disease need to be
tion panel. Marked elevation in bilirubin, investigated. Liver biopsy tends to show an
gamma-glutamyl transferase (GGT), and/or “onion-skin” appearance of concentric peri-
aspartate aminotransferase (AST)/liver func- ductal fibrosis in PSC. As the disease pro-
tion tests (LFTs) will generally prompt an gresses, the periductal fibrosis advances to
ultrasound of the right upper quadrant that necrosis, periportal fibrosis, and eventually
may or may not show dilation of intrahepatic biliary cirrhosis [3].
or extrahepatic bile ducts. If PSC is sus- E. ERCP is the preferred route for cholangiogra-
pected, autoantibodies such as antinuclear, phy and can demonstrate the characteristic
antismooth muscle, anticardiolipin, and IgG4 appearance of PSC: multifocal, diffusely dis-
may be obtained, although they are of uncer- tributed dilations, and strictures of the intra-
tain significance. Intrahepatic saccular dila- hepatic and extrahepatic biliary system. The
tions and signs of portal hypertension may be classic pattern is described as “beading,”
visualized in long-standing disease or end- resembling the arrangement of beads on a
stage PSC [1]. string. Cholangioscopy is currently being
C. If no abnormalities are observed on ultra- investigated and may play a role in directed
sound, further investigation of the hyperbili- tissue biopsy; however, it is not considered to
be a standard of care in PSC [4].
F. Medical treatment of PSC has shown little to
N. M. Mineyev · K. M. Chaffee · J. Wong (*) no lasting effects. Dominant intrahepatic
Department of General Surgery, Lenox Hill Hospital, strictures are best managed endoscopically
New York, NY, USA

with balloon dilation and/or stenting. Biliary formed, the patient must continue to be sur-
reconstruction is an option for a select group veilled. Both the American Association for
of patients with dominant extrahepatic stric- the Study of Liver Disease (AASLD) and the
tures only and minimal intrahepatic disease. European Association for the Study of the
With the high risk of developing cholangio- Liver (EASL) recommend having quarterly
carcinoma in PSC, as well as increased suc- blood work evaluation and yearly MRCP and
cess of orthotopic liver transplantation CA19-9 tumor markers [5].
(OLTx), the use of biliary reconstruction pro- H. OLTx is the only curative option for patients
cedures has decreased. with progressive liver disease due to PSC. The
. If no dominant strictures are identified during
G 5- and 10-year survival rates for PSC after OLTx
ERCP and no endoscopic interventions per- have been reported between 75% and 87%.
82 Diagnosis and Management of Primary Sclerosing Cholangitis 337
History and presentation:

Vague abdominal/RUQ pain,
fatigue, jaundice, pruritis
Obtain vital signs and blood work,

A and perform physical exam
Elevated alkaline phosphatase,

total bilirubin, GGT, AST/ALT
Right upper
B quadrant ultrasound
E F
Dilated Diffuse biliary Endoscopic

Yes Yes
intrahepatic ERCP strictures and brush Dominant dilation and/or
bile ducts? cytology +/– biopsy stricture? stenting
confirm PSC
No No
Surveillance:
C MRCP Labs Q3 months
CA19–9 Q1 Year
MRCP Q1 Year
H
Dilated Yes
intrahepatic
bile ducts? Yes
Severe liver
G disease?
Liver transplant
No
Liver biopsy No
Continue
surveillance +/–
D ERCP for further
interventionsif
necessary
Characteristic
Yes
onion-skin
periductal
fibrosis?
No
Further
investigation
Algorithm 82.1
References 3. Eaton J, et al. Pathogenesis of primary sclerosing

cholangitis and advances in diagnosis and manage-
ment. Gastroenterology. 2013;145(3):521–36.
1. Townsend CR, Beauchamp MB, Evers M, Mattox K.
4. Srinivasan N, et al. Diagnosis and treatment: ERCP
Sabiston textbook of surgery. 20th ed. Philadelphia:
in PSC; endoscopy in inflammatory bowel disease.
Elsevier; 2016. p. 1508–9.
Switzerland: Springer International Publishing; 2014.
2. Kovac J, et al. Primary biliary cirrhosis and primary
p. 309–22.
sclerosing cholangitis: an update on MR imaging
5. Lutz H, et al. PSC: diagnosis and treatment. Dtsch
findings with recent developments. J Gastrointestin
Arztebl Int. 2013;110(51–52):867–74.
Liver Dis. 2016;25(4):517–24.
Portal Hypertension and Shunting
83
and Joyce Wong
Algorithmic Approach
C. Intrahepatic causes of portal hypertension
include schistosomiasis, a parasitic disease
A. The diagnosis of portal hypertension typi-
caused by trematode flukes, particularly S.
cally occurs with imaging demonstrating cir- japonicum and S. mansoni. Because of the
rhosis or secondary signs such as immune response to parasite egg antigens,
hypersplenism, ascites, and/or bleeding from extensive fibrosis and hepatosplenic disease
varices. It is important to perform a history with periportal fibrosis can occur. Praziquantel
and physical examination, obtain blood work, is the treatment of choice, although oxam-
and perform dedicated liver imaging. Portal niquine is also effective [3]. Other intrahe-
hypertension is broadly categorized as related patic causes include biliary disease such as
to cirrhosis or noncirrhosis etiologies. biliary cirrhosis, neoplastic occlusion of the

B. Noncirrhotic etiologies are classified into intrahepatic portal veins, developmental
three general groups: pre-hepatic, intrahe- abnormalities such as polycystic liver disease
patic, and posthepatic. Prehepatic causes or congenital hepatic fibrosis, and acquired
include splenic vein thrombosis or portal vein diseases such as nonalcoholic fatty liver dis-
thrombosis. Left-sided portal hypertension ease or inflammatory viral hepatitis. There
(sinistral hypertension) may be related to are a multitude of intrahepatic etiologies;
severe pancreatitis with splenic vein thrombo- treatment generally centers on the prevention
sis or postsurgical splenic vein ligation. In of severe complications such as variceal
patients with varices and bleeding, splenec- bleeding.
tomy should be considered; asymptomatic D. Posthepatic etiologies include Budd-Chiari

patients can be monitored [1]. Portal vein syndrome or hepatic vein outflow obstruc-
thrombosis can be classified as acute or tion. Treatment options include anticoagula-
chronic and may be related to malignancy, cir- tion, short segment angioplasty, or
rhosis, or a hypercoagulable state. In noncir- transjugular intrahepatic portosystemic shunt
rhotic patients, early anticoagulation is (TIPS) in patients not in liver failure. If the
important to prevent varices from forming [2]. inferior vena cava is patent and there is not a
significant pressure gradient between the
infrahepatic and suprahepatic portions, sur-
gical shunting can also be offered, such as
N. M. Mineyev · K. M. Chaffee · J. Wong (*) portacaval, splenorenal, or mesocaval shunts.
Department of General Surgery, Lenox Hill Hospital,
New York, NY, USA

In patients with symptoms of liver failure, F. For patients with ascites, paracentesis should
liver transplantation is offered [4]. be performed to evaluate cell count and dif-
E. Care of the patient with portal hypertension ferential, total protein, and serum-ascites
is often directed toward management of albumin gradient. Typical management starts
symptoms. Patients with cirrhosis or plate- with sodium restriction (2gm/day) and diuret-
let count <150,000 should undergo screen- ics (spironolactone, furosemide). For those
ing endoscopy. Prophylaxis of bleeding with refractory ascites, serial paracenteses
with a nonselective beta-blocker or endo- can be offered. For ongoing refractory asci-
scopic variceal ligation should be consid- tes, liver transplantation should be considered
ered. Those with upper gastrointestinal [6].
bleeding from esophageal varices should G. For patients with refractory symptoms or

undergo endoscopy with sclerotherapy, development of end-stage liver disease, hepa-
which controls bleeding in more than 90% torenal syndrome, or hepatopulmonary syn-
of patients. If sclerotherapy fails, balloon drome, liver transplantation is an important
tamponade can be used for temporary con- option, and referral to transplantation should
trol, up to 24 h, followed by repeat endos- be done, unless there is an underlying psychi-
copy or TIPS [5]. atric or medical contraindication.
83 Portal Hypertension and Shunting 341
Diagnosed with portal hypertension

Incidentally found
Symptoms such as bleeding, elevated LFTs
A
Obtain vital signs, and blood work and perform a physical examination
Review imaging, obtain dedicated imaging, i.e., MRI, as needed
Cirrhosis Non cirrhosis
C D
B
Pre hepatic Intra hepatic Post hepatic
Symptoms from
portal hypertension?
Splenic vein Portal vein Budd-Chiari

Schistosomiasis Other causes
thrombosis thrombosis* syndrome*
Praziquantel
Splenectomy Anticoagulation
oxamniquine
Liver failure?
D Budd-Chiari syndrome or chronic portal vein

thrombosis*
No Yes
Symptomatic TIPS/stent
Follow-up Liver transplant

If IVC open, consider
mesocaval or portacaval
shunt
Algorithm 83.1
Symptoms from portal

hypertension?
Yes No Follow-up
E F
Esophageal varices
Ascites Liver failure
Bleeding
Endoscopic banding
Diuretics Yes No
Beta-blocker therapy
Follow-up Follow-up
Supportive care
Follow-up
G Liver failure?
No Yes
Supportive care Not a candidate Liver transplant
4. Valla DC. Budd-Chiari syndrome/hepatic venous out-

References flow tract obstruction. Hepatol Int, 2017; Jul 6 [Epub
ahead of print].
1. Loftus JP, Nagorney DM, Ilstrup D, et al. Sinistral 5. Hwang JH, Shergill AK, Acosta RD, et al. The
portal hypertension. Splenectomy or expectant man- role of endoscopy in the management of vari-
agement. Ann Surg. 1993;217(1):35–40. ceal hemorrhage. J Gastrointestinal Endoscopy.
2. Manzano-Robleda Mdel C, Barranco-Fragoso B, 2014;80(2):221–7.
Uribe M, et al. Portal vein thrombosis: what is new? 6. Runyon BA. Management of adult patients with
Ann Hepatol. 2015;14(1):20–7. ascites due to cirrhosis: update: AASLD Practice
3. Colley DG, Bustinduy AL, Secor WE, Guideline; Alexandria, VA. 2012.
et al. Human schistosomiasis. Lancet.
2014;383(9936):2253–64.
Part XI
Biliary
Acute Cholecystitis and Biliary
Colic 84
Algorithmic Approach tive findings of gallstones, gallbladder wall

thickness, and Murphy’s sign have a 95% pre-
Acute calculous cholecystitis (ACC) represents dictive value for ACC [5]. Signs of acute cho-
about 1/3 of surgical admissions and is the most lecystitis on ultrasound are sonographic
frequent complication of cholelithiasis [1]. Risk Murphy sign (tenderness elicited by pressing
of complications such as gallstone pancreatitis the gallbladder with the ultrasound probe),
and choledocholithiasis is 1–4% per year [1]. thickened gallbladder wall (>4 mm, if the
ACC is commonly caused by an impacted gall- patient does not have chronic liver disease and/
stone in the infundibulum or cystic duct that or ascites or right heart failure), enlarged gall-
causes an inflammatory process within the gallbladder (long axis diameter >8 cm, short axis
bladder wall [2]. This can lead to serosal edema, diameter >4 cm), incarcerated gallstone, debris
mucosal sloughing, and venous and lymphatic echo, pericholecystic fluid collection, sonolu-
congestion. It can further be complicated by bac- cent layer in the gallbladder wall, striated
terial infection from the bile duct entering via the intramural lucencies, and Doppler signals [6].
portal system [3]. Diagnosis of ACC typically C. Computed tomography (CT) can aid in the
requires clinical and imaging studies. Grading diagnosis of complicated ACC and may help
systems to qualify severity are available by the differentiate between other intra-abdominal
American Association for the Surgery of Trauma processes [7, 8]. Abnormal liver function tests
(AAST) [4] and the Tokyo Guidelines. such as presence of abnormal transaminases
(e.g., total serum bilirubin >1.5 and/or aspar-
A & B: Ultrasound is typically the first-line tate aminotransferase >60) and mild hyper-
imaging if ACC is suspected. It can help to bilirubinemia may be indicative of worsening
diagnose lumen distention, gallstones, disease, inflammation of the liver, and wors-
Murphy’s sign, and hyperemia [3]. The posi- ening infection [9, 10].
With regard to common bile duct stones
(CBDS), a Cochrane metaanalysis compared
C. J. Chantachote (*)
Department of Surgery, Stony Brook University magnetic resonance cholangiopancreatogra-
Hospital, Stony Brook, NY, USA phy (MRCP) and endoscopic ultrasound
e-mail: CHANAK.CHANTACHOTE@ (EUS) and concluded that both are highly
STONYBROOKMEDICINE.EDU accurate and can determine the presence of
S. Sbayi CBDS with high sensitivity and specificity
Department of General Surgery, Stony Brook (95%) [11].

346 C. J. Chantachote and S. Sbayi
D. The choice of treatment in concomitant CBDS LC to OC. The results showed that male
and ACC ranges from open common bile duct patients, ages 60–65 years, sclerotic gallblad-
exploration (CBDE), laparoscopic cholecys- der or wall thickness (>4 mm) and acute cho-
tectomy with laparoscopic common bile duct lecystitis, were significant risk factors for
exploration, preoperative endoscopic retro- conversion [16, 17].
grade cholangiopancreatography (ERCP), or E. Biliary colic is the most common form of
postoperative ERCP. A systematic review of symptomatic gallbladder disease [18]. It is
randomized controlled trials has shown that often used to describe gallbladder pain expe-
open cholecystectomy (OC) with CBDE has rienced by patients without any obvious
the lowest incidence of retained stones but is signs of gallbladder infection. It develops in
associated with high morbidity and mortality, at least one third of patients with cholelithia-
especially in elderly patients [12]. sis over a 10-year period of follow-up [18].
Treatment of choice remains a laparoscopic It is usually caused by transient gallstone
cholecystectomy (LC) which carries a smaller obstruction of the cystic duct or edema
influence on the immune response reflected in caused by the passage of a stone [18]. Colic
lowered levels of cytokines yielded and a lesser refers to the type of pain that “comes and
systemic inflammatory response severity. This goes” typically after eating a meal that
has improved outcomes [13]. A systematic causes contraction of the gallbladder.
review concluded that, when a difficult gall- Cholecystectomy is considered to be the
bladder is encountered during LC, laparoscopic gold standard treatment [17]. However, the
partial cholecystectomy (LPC) can be a safe timing of cholecystectomy may be on the
alternative to conversion and closing of the cys- index admission or on an elective basis.
tic duct, gallbladder remnant, or both [14, 15]. Elective cholecystectomy may include pain
Another systematic review assessed the control and in some cases a “low-fat” diet
associated factors linked to the conversion of prior to elective surgery.
84 Acute Cholecystitis and Biliary Colic 347
H&P RUQ pain

A
B LFTs and U/S of the RUQ
Normal LFTs;
C Increasing LFTs; cholelithiasis;
+GB thickening normal GB wall
+ Murphys sign no Murphy's Sign
Biliary colic
Acute cholecystitis
On index
admission
E
D
Laparoscopic Pain control and
cholecystectomy low fat diet
Elective
Algorithm 84.1
NT. Measuring anatomic severity of disease in emer-

References gency general surgery. J Trauma Acute Care Surg.
2014;76:884–7.
1. National Institutes of Health consensus devel- 5. Ralls PW, Colletti PM, Lapin SA, Chandrasoma P,
opment conference statement on gallstones Boswell WD, Ngo C, Radin DR, Halls JM. Real-
and laparoscopic cholecystectomy. Am J Surg. time sonography in suspected acute cholecystitis.
1993;165:390–8. Prospective evaluation of primary and secondary
2. Shaffer EA. Gallstone disease: epidemiology of signs. Radiology. 1985;155:767–71.
gallbladder stone disease. Best Pract Res Clin 6. Hirota M, Takada T, Kawarada Y, Nimura Y, Miura
Gastroenterol. 2006;20:981–96. F, Hirata K, Mayumi T, Yoshida M, Strasberg S,
3. Riall TS, Zhang D, Townsend CM, Kuo YF, Goodwin Pitt H, Gadacz TR, de Santibanes E, Gouma DJ,
JS. Failure to perform cholecystectomy for acute Solomkin JS, Belghiti J, Neuhaus H, Büchler MW,
cholecystitis in elderly patients is associated with Fan S-T, Ker C-G, Padbury RT, Liau K-H, Hilvano
increased morbidity, mortality, and cost. J Am Coll SC, Belli G, Windsor JA, Dervenis C. Diagnostic cri-
Surg. 2010;210:668–79. teria and severity assessment of acute cholecystitis:
4. Shafi S, Aboutanos M, Brown CV, Ciesla D, Cohen Tokyo guidelines. J Hepato-Biliary-Pancreat Surg.
MJ, Crandall ML, Inaba K, Miller PR, Mowery 2007;14:78–82.
7. Buonamico P, Suppressa P, Lenato GM, Pasculli G, tion of the common bile duct for cholecystocholedo-
D’Ovidio F, Memeo M, Scardapane A, Sabbà C. Liver cholithiasis. Surg Endosc. 2006;20:424–7.
involvement in a large cohort of patients with heredi- 13. Di Saverio S. Emergency laparoscopy: a new emerg-
tary hemorrhagic telangiectasia: echo-color-Doppler ing discipline for treating abdominal emergencies
vs multislice computed tomography study. J Hepatol. attempting to minimize costs and invasiveness and
2008;48:811–20. maximize outcomes and patients’ comfort. J Trauma
8. Reginelli A, Mandato Y, Solazzo A, Berritto D, Acute Care Surg. 2014;77:338–50.
Iacobellis F, Grassi R. Errors in the radiological 14. Henneman D, da Costa DW, Vrouenraets BC, van
evaluation of the alimentary tract: part II. Semin Wagensveld BA, Lagarde SM. Laparoscopic partial
Ultrasound CT MR. 2012;33:308–17. cholecystectomy for the difficult gallbladder: a sys-
9. Brooks KR, Scarborough JE, Vaslef SN, Shapiro tematic review. Surg Endosc. 2013;27:351–8.
ML. No need to wait: an analysis of the timing of cho- 15. Gomes CA, Junior CS, Di Saveiro S, et al. Acute cal-
lecystectomy during admission for acute cholecysti- culous cholecystitis: review of current best practices.
tis using the American College of Surgeons National World J Gastrointest Surg. 2017;9(5):118–26.
Surgical Quality Improvement Program database. J 16. Philip Rothman J, Burcharth J, Pommergaard HC,
Trauma Acute Care Surg. 2013;74:167. Viereck S, Rosenberg J. Preoperative risk factors for
10. Cameron JL, Cameron AM, editors. Current surgical conversion of laparoscopic cholecystectomy to open
therapy. 11th ed. Philadelphia: Elsevier Saunders; 2014. surgery - a systematic review and meta-analysis of
11. Giljaca V, Gurusamy KS, Takwoingi Y, Higgie D, observational studies. Dig Surg. 2016;33:414–23.
Poropat G, Štimac D, et al. Endoscopic ultrasound ver- 17. Tiderington E, Lee SP, Ko CW. Gallstones: new

sus magnetic resonance cholangiopancreatography for insights into an old story. F1000Res. 2016;5.:
common bile duct stones. Cochrane Database Syst Rev. F1000 Faculty Rev-1817. https://doi.org/10.12688/
2015;2:CD011549. https://doi.org/10.1002/14651858. f1000research.8874.1.
CD011549. 18. Warren KW, EGC T. Surgical approach to disease of
12. Hong DF, Xin Y, Chen DW. Comparison of laparo- the biliary system. In: Schiff L, Schiff ER, editors.
scopic cholecystectomy combined with intraoperative Diseases of the liver. 7th ed. Philadelphia: Lippincott;
endoscopic sphincterotomy and laparoscopic explora- 1993. p. 448–86.
Acalculous Cholecystitis
85
Algorithmic Approach B, C. Imaging plays a major role in the accuracy

of diagnosis and management. Which study to
A. Acute acalculous cholecystitis (AAC), repre- begin with, an ultrasound, a CT scan, or a
senting only 5–10% of all cases of cholecysti- HIDA scan, is debatable [5]. Sonographic fea-
tis, is inflammation of the gallbladder which is tures of abdominal circumference (AC)
not associated with the presence of gallstones include an abnormally distended GB, wall
[1, 2]. This pathology was described initially thickening, pericholecystic fluid (without
in critically ill patients who underwent major ascites), and sludge (in the absence of choleli-
surgery or in the setting of extensive burns [1]. thiasis) [6].
Ischemia is the most likely underlying cause D. Patients who are deemed clinically stable for a
of acute acalculous cholecystitis [1]. Clinical laparoscopic cholecystectomy should undergo
findings of AAC can be difficult to distinguish surgery early in the disease process. If the
from calculous cholecystitis. Oftentimes, the patient is critical with multiple comorbidities
clinical signs and laboratory workup are non- or a poor surgical candidate, then percutane-
specific [3]. ous cholecystostomy tube placement is a safer
Treatment of acute acalculous cholecystitis choice. In some papers, there is a higher inci-
consists of early radiologic imaging and effec- dence of gangrenous cholecystitis of 31%
tive management. The three most common compared to calculous cholecystitis of 5.6%
treatments are open cholecystectomy, laparo- [7]. Mortality from acute acalculous cholecys-
scopic cholecystectomy, or percutaneous cho- titis is at least 30% because of the association
lecystostomy [4], depending on severity of of gangrene and the rapid disease progression
disease. in critically ill patients [8, 9].
e-mail: CHANAK.CHANTACHOTE@
STONYBROOKMEDICINE.EDU
S. Sbayi

References
H&P RUQ Pain
A
1. Poddighe D, Tresoldi M, Licari A, Marseglia
GL. Acalculous acute cholecystitis in previously
healthy children: general overview and analysis of
pediatric infectious cases. Int J Hepatol. 2015;2015
Article ID 459608, 6 pages.
B LFTs U/S RUQ 2. Prashanth GP, Angadi BH, Joshi SN, Bagalkot PS,
Maralihalli MB. Unusual cause of abdominal pain in
pediatric emergency medicine. Pediatr Emerg Care.
2012;28(6):560–1.
3. Hermiz SJ, Diegidio P, Garimella R, Ortiz-Pujols S,
Yu H, Isaacson A, Mauro MA, Cairns BA, Hultman
– Increasing LFTs
CS. Acalculous cholecystitis in burn patients. Clin
C – US: No cholelithiasis,
GB wallthickening Plast Surg. 2017;44(3):567–71.
4. Treinen C, Lomelin D, Krause C, et al. Acute acal-
culous cholecystitis in the critically ill: risk factors
and surgical strategies. Langenbeck's Arch Surg.
2015;400:421.
Acalculous 5. Taoka H. Experimental study on the pathogenesis
cholecystitis
of acute acalculous cholecystitis, with special refer-
ence to the roles of microcirculatory disturbances,
free radicals and membrane-bound phospholipase A2.
Gastroenterol Jpn. 1991;26:633–44.
6. Smith EA, Dillman JR, Elsayes KM, Menias CO,
Is the patient clinically
D Bude RO. Cross-sectional imaging of acute and
stable for surgery?
chronic gallbladder inflammatory disease. Am J
Roentgenol. 2009;192:188–96.
Yes No 7. Gu M, Kim TN, Song J, Nam YJ, Lee JY, Park
JS. Risk factors and therapeutic outcomes of acute
acalculous cholecystitis. Digestion. 2014;90:75–80.
Laparoscopic Percutaneous 8. Barie PS, Eachempati SR. Acute acalculous cholecys-
cholecystectomy cholecystostomy
titis. Gastroenterol Clin N Am. 2010;39(2):343–57.
tube
9. Huffman JL, Schenker S. Acute acalculous cho-
lecystitis: a review. Clin Gastroenterol Hepatol.
Algorithm 85.1 2010;8(1):15–22.
Postcholecystectomy
86
Algorithmic Approach Initial assessment is done by transabdomi-

nal ultrasound and liver function tests some-
A. B. In the case of a patient presenting to the times followed by endoscopic retrograde
emergency department status postcholecys- cholangiopancreatography (ERCP) [5].
tectomy with abdominal pain, suspicion for a Transabdominal ultrasound allows rapid
bile leak should be considered as it can occur detection and can differentiate between
in 0.3–27% of patients [1]. The bile leak can obstructive and nonobstructive jaundice [6].
arise from an injury to the common bile, Endoscopic retrograde cholangiography
hepatic duct, cystic duct stump, or duct of (ERCP) and magnetic resonance cholangiog-
Luschka [2]. These leaks can present as a bili- raphy (MRC) examinations are likely to dem-
ary fistula, subhepatic/subphrenic collection, onstrate the presence of biliary leak and often
or even peritonitis. The principle in manage- provide the level of duct laceration or transac-
ment of a bile leak is drainage of the leak [1]. tion [6, 7]. ERCP in addition provides a thera-
Postcholecystectomy syndrome includes a peutic option in this scenario when
heterogeneous group of disease-manifested sphincterotomy and endobiliary stenting may
findings, characterized as abdominal symp- be considered; other therapeutic intervention
toms. The most common symptoms in commonly used includes percutaneous tran-
descending order include right upper quadrant shepatic cholangiography, transhepatic biliary
pain or epigastric pain, nausea/vomiting, and drainage, and percutaneous drainage of intra-
fever. Total bilirubin can be elevated above abdominal collection [8].
1.5 mg/dl in almost 30% of patients, elevated C. A common bile duct (CBD) diameter less than
LFTs in over 50%, and elevated white blood 10 mm was normal and greater than or equal
cells in over 60% of patients [3, 4]. to 10 mm was abnormal. Direct evidence of a
stone in the bile duct is considered abnormal
irrespective of the size of the duct.
MRCP is able to demonstrate the level and
Department of Surgery, Stony Brook University presence of biliary obstruction with a sensitiv-
Hospital, Stony Brook, NY, USA ity of 95% and a specificity of 97%, being less
e-mail: CHANAK.CHANTACHOTE@ sensitive for detecting stones, especially less
STONYBROOKMEDICINE.EDU than 6 mm in size [9].
S. Sbayi

D. Sphincter of Oddi dysfunction (SOD) is a syn- [11]. The median time for resolution of the
drome of chronic biliary pain or recurrent pan- leak was 3 days (range 1–39 days) [11].
creatitis due to functional obstruction of Kaffes and colleagues reported that stent
pancreaticobiliary flow at the level of the insertion alone for postcholecystectomy bile
sphincter of Oddi [10]. Symptoms attributable leak is superior to sphincterotomy alone,
to SOD can be seen in three clinical scenarios: because fewer patients required additional
(1) postcholecystectomy syndrome, (2) acal- intervention (particularly surgery) to control
culous biliary pain with an intact gallbladder, the leak [11].
and (3) recurrent idiopathic pancreatitis. The F. For clinically benign presentations with no
current gold standard for diagnosis is manom- abnormal lab value and found to have a biloma
etry to detect elevated sphincter pressure, that was treated with drainage, conservative
which correlates with outcome of treatment may be sufficient. A biloma can be
sphincterotomy. managed by percutaneous catheter drainage
E. Endoscopic treatment at ERCP with stent and placed under imaging guidance. If the leak is
sphincterotomy is usually the first line of small, it will resolve spontaneously in a few
treatment with success rate greater than 90% days [12–15].
86 Postcholecystectomy 353
A H&P RUQ pain (post

cholecystectomy)
LFTs/CBC/ultrasound
B C Inc LFTs
Inc LFTs
US: dilated CBD
(+) US: collection
F
Drain collection MRCP

HIDA/MRCP Eovist
+ Positive – Negative
Retained
stone?
D
No
Bile leak Conservative Rx
ERCP/stent Abx Yes
E
Dysfunctional sphincter
of Oddi syndrome
ERCP/sphincterotomy/
stenting
Algorithm 86.1
5. Machado NO. Biliary complications post laparo-

References scopic cholecystectomy: mechanism, preventive
measures, and approach to management: a review.
1. Ahmad F, Saunders RN, Lloyd GM, Lloyd DM, Diagn Ther Endosc. 2011;2011 Article ID 967017, 9
Robertson GS. An algorithm for the management of pages.
bile leak following laparoscopic cholecystectomy. 6. Rogoveanu I, Gheonea DI, Saftoiu A, Ciurea T. The
Ann R Coll Surg Engl. 2007;89:51–6. role of imaging methods in identifying the causes of
2. De Palma GD, Galloro G, Iuliano G, Puzziello extrahepatic cholestasis. J Gastrointestin Liver Dis.
A, Persico F, Masone S, et al. Leaks from laparo- 2006;15:265–71.
scopic cholecystectomy. Hepato-Gastroenterology. 7. Schmidt SC, Langrehr JM, Hintze RE, Neuhaus
2002;49:924–5. P. Long-term results and risk factors influencing out-
3. Woods MS, Shellito JL, Santoscoy GS, et al. Cystic come of major bile duct injuries following cholecys-
duct leaks in laparoscopic cholecystectomy. Am J tectomy. Br J Surg. 2005;92(1):76–82.
Surg. 1994;168(6):560–3. 8. Sandha GS, Bourke MJ, Haber GB, Kortan
4. Eisenstein S, Greenstein AJ, Kim U, Divino PP. Endoscopic therapy for bile leak based on a new
CM. Cystic duct stump leaks after the learning curve. classification: results in 207 patients. Gastrointest
Arch Surg. 2008;143(12):1178–83. Endosc. 2004;60(4):567–74.
9. Tse F, Barkun JS, Romagnulo J, Friedman G, 13. Nunez D Jr, Becerra JL, Martin LC. Subhepatic

Bornstein JD, Barkun AN. Nonoperative imaging collections complicating laparoscopic cholecystec-
techniques in suspected biliary tract obstruction. HPB tomy: percutaneous management. Abdom Imaging.
(Oxford). 2006;8:409–25. 1994;19:248–50.
10. Bistritz L, Bain VG. Sphincter of Oddi dysfunction: 14. Sammak BM, Yousef BA, Gali MH, al Karawi MA,
managing the patient with chronic biliary pain. World Mohamed AE. Case report: radiological and endo-
J Gastroenterol: WJG. 2006;12(24):3793–802. scopic management of bile leak following laparo-
11. Kaffes AJ, Hourigan L, De Luca N, Byth K, Williams scopic chole-cystectomy. J Gastroenterol Hepatol.
SJ, Bourke MJ. Impact of endoscopic intervention in 1997;12:34–8.
100 patients with suspected postcholecystectomy bile 15. Pavlidis TE, Atmatzidis KS, Papaziogas BT, et al.
leak. Gastrointest Endosc. 2005;61(2):269–75. Biloma after laparoscopic cholecystectomy. Ann
12. Festekjian JH, Hassantash SA, Taylor EW. Abdominal Gastroenterol. 2002;15:178–80.
wall biloma: an unusual complication of laparoscopic
cholecystectomy. JSLS. 1997;1:353–5.
Management
of Postcholecystectomy Cholangitis 87
Algorithmic Approach ment. Distal obstructions are usually amena-

ble to ERCP [4].
A. Fever, jaundice, and right upper quadrant
D. A PTC should be attempted if ERCP is unsuc-
pain are classic signs and symptoms of acute cessful or contraindicated.
cholangitis. The diagnosis is also more likely E. If attempts with ERCP and PTC are unsuc-
given a history of recent cholecystectomy. cessful, the patient should undergo open
Initial evaluation should include vitals, a drainage. Distal obstructions should be man-
physical exam, and laboratory values includ- aged with choledochotomy and placement of
ing white blood cell count, which may indi- a tympanostomy tube (T-tube). Surgical man-
cate active inflammation and/or infection, agement should focus on drainage alone
and liver function tests, which may indicate especially in the setting of instability [1, 2].
cholestasis [1, 2].
B. The patient should be immediately resusci- Following decompression of the biliary tree, the
tated, and broad-spectrum antibiotics should patient should undergo treatment of the underly-
be started. An abdominal ultrasound should ing cause where appropriate. A secondary com-
be obtained. A common bile duct diame- mon bile duct stone is often definitively managed
ter >6 mm may be indicative of biliary with removal of the stone alone, whether by
obstruction [3]. ERCP or open surgical management. Definitive
C. An emergent ERCP should be performed for management should be performed on an elective
biliary drainage and possible definitive treat- basis [1, 2].
J. VanderVelde
Department of Surgery, Maricopa Integrated Health
R. F. Goldberg (*)
Creighton University School of Medicine,
Phoenix, AZ, USA
University of Arizona College of Medicine –
Phoenix, Phoenix, AZ, USA

356 J. VanderVelde and R. F. Goldberg
History and physical exam:

A Right upper quadrant pain,
fever, jaundice s/p
cholecystectomy
Obtain vital signs and labs, perform physical exam
Leukocytosis, elevated
B LFTs and bilirubin
Fluid resuscitation, antibiotic therapy, abdominal ultrasound
ERCP
Yes
C
ERCP successful?
No
PTC
Yes
PTC successful? Definitive management
D after stabilization
No
F
E OR for open drainage
Algorithm 87.1
3. Surviving Sepsis Campaign Guidelines Committee.

References Surviving Sepsis Campaign: international guide-
lines for management of sepsis and septic shock:
1. Cameron J, Cameron A. Current surgical therapy. 2016. Crit Care Med. 2017;45(3):486–552.
11th ed. Philadelphia: Elsevier; 2014. 4. Hou LA, Laine L, Motamedi N, Sahakian A, Lane C,
2. Townsend C, Beauchamp R, Evers B, Mattox Buxbaum J. Optimal timing of endoscopic retrograde
K. Sabiston textbook of surgery. 19th ed. Philadelphia: cholangiopancreatography in acute cholangitis. J Clin
Elsevier; 2012. Gastroenterol. 2017;51(6):534–8.
Management
of Choledocholithiasis 88
Algorithmic Approach C. An abdominal ultrasound is the most com-

mon initial imaging study obtained due to
A. A history and physical exam is the first step in low cost, noninvasive nature, and speed at
evaluating a patient with choledocholithiasis. which it may be obtained. Ultrasound has a
Right upper quadrant pain is nonspecific to relatively high sensitivity but low specificity
choledocholithiasis and can be attributed to [3]. Stones within the common bile duct or a
other etiologies such as biliary colic, chole- dilated common bile duct may be seen.
cystitis, and cholangitis, so a thorough history D. If no stone is seen on ultrasound, then a mag-
including onset and associated symptoms is netic resonance cholangiopancreatography
important [1, 2]. (MRCP) should be obtained. MRCP has high

B. Initially differentiating choledocholithiasis sensitivity and specificity for diagnosis of
from other disease processes usually starts choledocholithiasis [4]. This also allows eval-
with initial laboratory values which may uation of the entire biliary tree to rule out
show a cholestatic pattern. Liver function other possible causes of biliary obstruction.
tests, direct bilirubin, and alkaline phospha- E. A common bile duct stone may be identified by
tase may be elevated. Lab findings of elevated ultrasound or MRCP. A preoperative endo-
lipase and leukocytosis plus fever lend suspi- scopic retrograde cholangiopancreatography
cion to gallstone pancreatitis and acute chol- (ERCP) may be obtained at this point.
angitis, respectively, which may also be Preoperative ERCP can be effective in the hands
present with choledocholithiasis. If no chole- of an experienced endoscopist but does add an
static pattern is shown on initial laboratory additional procedure. Laparoscopic cholecys-
values, then another etiology should be tectomy with common bile duct exploration can
sought [1, 2]. also be chosen and has the benefit of offering
the patient only one procedure. However, it is
J. VanderVelde not a commonly performed procedure among
Department of Surgery, Maricopa Integrated Health most general surgeons. Both options are avail-
System, Phoenix, AZ, USA able to the surgeon and should be decided with
R. F. Goldberg (*) utility and patient’s benefit in mind.
Creighton University School of Medicine, F. If a stone is removed with preoperative ERCP
Phoenix, AZ, USA or no stone is seen on MRCP (indicating pas-
University of Arizona College of Medicine – sage of a stone), then a laparoscopic
Phoenix, Phoenix, AZ, USA cholecystectomy should be performed during
the admission.

Right upper quadrant/epigastric pain
A Nausea and vomiting
+/– jaundice
Obtain vital signs and labs,

perform physical exam
No
Elevated LFTs,
B Pursue other diagnosis
bili, alk phos?
Yes
C Obtain abdominal ultrasound
Equivocal
MRCP CBD stone?
Yes Yes E
CBD stone? Preoperative ERCP
No
ERCP successful? Lap chole with CBDE
No Yes
Cholecystectomy during admission
Algorithm 88.1
3. Qiu Y, et al. An analysis of the factors related to

References missed diagnosis of choledocholithiasis by preopera-
tive ultrasound. BM Gastroenterol. 2015;15:158.
1. Cameron J, Cameron A. Current surgical therapy. 4. Yaghoobi M, et al. Diagnostic accuracy of EUS
11th ed. Philadelphia: Elsevier; 2014. compared with MRCP in detecting choledocho-
2. Townsend C, Beauchamp R, Evers B, Mattox lithiasis: a meta-analysis of diagnostic test accu-
K. Sabiston textbook of surgery. 19th ed. Philadelphia: racy of head-to-head studies. Gastrointest Endosc.
Elsevier; 2012. 2017;86(6):986–93.
Acute Cholangitis
89
Algorithmic Approach drainage. If no end-organ dysfunction is evi-

denced, then definitive management of the
A. Acute cholangitis presents classically with
underlying cause may be sought [4].
fever, jaundice, and right upper quadrant pain D. The location of the obstruction within the
(Charcot’s triad). The presence of altered biliary tree dictates the method of drainage if
mental status and hypotension (Reynold’s drainage is necessary. Distal obstructions are
pentad) indicates a more severe form of the usually amenable to ERCP [5]. Proximal
disease. Initial evaluation should include obstructions are not as amenable to ERCP
vitals, a physical exam, and laboratory values and may require PTC drainage [6].
including liver function tests and white blood E. If ERCP and PTC are unsuccessful at biliary
cell count [1, 2]. drainage, then the patient should be taken for
B. Resuscitation with intravenous fluids should open surgical drainage with choledochotomy.
be started immediately as well as initiation Laparoscopic drainage may be performed as
of antibiotic therapy, usually with a third- well but is contraindicated if there is evidence
generation cephalosporin such as ceftriax- of hemodynamic instability [7].
one [3]. F. If biliary drainage is successful with the
C. Evidence of end-organ dysfunction includes above measures, then definitive management
altered mental status and hypotension of the underlying obstructive etiology should
(included in Reynold’s pentad) as well as be performed. If the obstruction is due to gall-
elevated lactate, decreased urinary output, stones, a cholecystectomy should be per-
and acute kidney injury. The presence of end- formed during the same admission.
organ dysfunction necessitates urgent biliary
J. VanderVelde
Department of Surgery, Maricopa Integrated Health
R. F. Goldberg (*)
Creighton University School of Medicine,
Phoenix, AZ, USA
University of Arizona College of Medicine –
Phoenix, Phoenix, AZ, USA

History:
Fever, jaundice, RUQ pain
+/– AMS, hypotension
Obtain vital signs and labs,

A
perform physical exam
Leukocytosis, elevated
LFTs, bili, alk phos
B
Fluid resuscitation, antibiotic therapy with 3rd
generation cephalosporin
No
Evidence of end
C
organ dysfunction?
Yes
D Urgent biliary drainage
Proximal Distal
obstruction obstruction
PTC No ERCP
Successful? Successful?
Yes
Yes
No
Surgical management Definitive management
E F
Algorithm 89.1
5. Hou LA, Laine L, Motamedi N, Sahakian A, Lane C,

References Buxbaum J. Optimal timing of endoscopic retrograde
cholangiopancreatography in acute cholangitis. J Clin
1. Cameron J, Cameron A. Current surgical therapy. Gastroenterol. 2017;51(6):534–8.
11th ed. Philadelphia: Elsevier; 2014. 6. Qureshi WA. Approach to the patient who has sus-
2. Townsend C, Beauchamp R, Evers B, Mattox pected bacterial cholangitis. Gastroenterol Clin N
K. Sabiston textbook of surgery. 19th ed. Philadelphia: Am. 2006;35(2):409–23.
Elsevier; 2012. 7. SAGES guidelines – guidelines for the clinical appli-
3. Gomi H, et al. Tokyo guidelines 2018: antimicro- cation of laparoscopic biliary tract surgery. https://
bial therapy for acute cholangitis and cholecystitis. J www.sages.org/publications/guidelines/guidelines-
Hepatobiliary Pancreat Sci. 2018;25:3–16. for-the-clinical-application-of-laparoscopic-biliary-
4. Surviving Sepsis Campaign Guidelines Committee. tract-surgery/. Accessed 2017.
Surviving Sepsis Campaign: international guidelines
for management of sepsis and septic shock: 2016. Crit
Care Med. 2017;45(3):486–552.
Cholangiocarcinoma
90
Zachary J. Senders, John B. Ammori,
and Jeffrey M. Hardacre
Algorithmic Approach B. If cholangiocarcinoma is suspected, patients

should undergo either multidetector, multipha-
A. The presenting signs and symptoms of a
sic, contrast-enhanced abdominal/pelvic com-
patient with cholangiocarcinoma will depend puted tomography (CT) or magnetic resonance
on the anatomical location of the tumor. imaging (MRI) with magnetic resonance chol-
Extrahepatic tumors, representing approxi- angiopancreatography (MRCP). Tumor mark-
mately 90% of all cholangiocarcinomas, most ers cancer antigen 19-9 (CA 19-9) and
often present with sequelae of biliary obstruc- carcinoembryonic antigen (CEA) should be
tion, including jaundice, pruritis, clay-colored obtained as a baseline measurement in patients
stool, and dark urine. Serum bilirubin and with cholangiocarcinoma, though they are nei-
alkaline phosphatase are often elevated, ther sensitive nor specific for the diagnosis.
though aspartate aminotransferase (AST) and AFP should be obtained if intrahepatic cholan-
alanine aminotransferase (ALT) may initially giocarcinoma is suspected, as an elevated level
be normal. Benign causes of biliary obstruc- is suggestive of hepatocellular carcinoma.
tion must be considered in a patient present- C. Cholangiocarcinoma is classified by the ana-
ing with obstructive jaundice, especially if tomical location of the tumor. Intrahepatic
clinical suspicion for malignancy is low. cholangiocarcinoma appears as an intrahe-
Intrahepatic tumors (10% of all cholangiocar- patic mass on cross-sectional imaging.
cinomas) are much less likely to present with Intrahepatic biliary ductal dilation in both
jaundice. These patients may have a history hepatic lobes is suggestive of an extrahepatic
of dull right upper quadrant pain, fever, or hilar (Klatskin) tumor, which constitutes
weight loss and may have elevated alkaline 60–70% of all extrahepatic cholangiocarcino-
phosphatase. Intrahepatic cholangiocarci- mas. Intra- and extrahepatic biliary ductal
noma may also be discovered incidentally in dilation in the absence of a pancreatic head
an asymptomatic patient during the workup mass or choledocholithiasis suggests distal
of abnormal LFTs [1]. extrahepatic cholangiocarcinoma [2]. Tissue
diagnosis is not needed to proceed with sur-
gery for resectable disease, although endo-
scopic retrograde cholangiopancreatography
Z. J. Senders · J. B. Ammori · J. M. Hardacre (*) (ERCP)/endoscopic ultrasound (EUS) can
Department of Surgery, University Hospitals aid in diagnosis and staging of distal lesions
Cleveland Medical Center, Cleveland, OH, USA and should be considered [3].

362 Z. J. Senders et al.
D. Chest CT scan is recommended as part of the can be considered. Percutaneous biliary

staging workup to detect disseminated dis- drainage, biopsy, or EUS with fine-needle
ease. Esophagogastroduodenoscopy (EGD) aspiration (FNA) can be contraindications to
and colonoscopy are mandatory in the setting transplant; therefore, consultation should be
of an intrahepatic tumor to rule out metastatic obtained before proceeding with these proce-
disease from a distant primary. Criteria for dures in potential transplant candidates [6].
resectability depend on the anatomical loca- Locoregional therapies such as ablation and
tion of the tumor. Distant liver metastasis or transarterial chemoembolization (TACE)
disseminated disease precludes resection. have been shown to increase progression-free
Invasion of the main portal vein, hepatic survival in unresectable intrahepatic cholan-
artery, or extrahepatic adjacent organs is a giocarcinoma and can be considered.
relative contraindication. An intrahepatic F. Surgical management of intrahepatic cholan-
tumor is considered unresectable if there giocarcinoma involves hepatic resection, with
would not be an adequate future liver remnant the goal of achieving negative margins. There
after resection. [4] is no evidence that lymphadenectomy
E. There are few treatment options for patients improves survival; however, it can be consid-
with metastatic or unresectable disease, ered for its prognostic value. Approach to the
though a regimen of gemcitabine plus cispla- resection of hilar tumors is based on the
tin has been shown in a phase III trial to Bismuth-Corlette classification and Blumgart
improve survival. [5] Biliary drainage should staging system. This includes resection of the
be considered in patients with unresectable involved biliary tract usually with en bloc
extrahepatic cholangiocarcinoma for symp- hepatic resection. Distal cholangiocarcinoma
tomatic relief. [1] Orthotopic liver transplan- is treated with pancreaticoduodenectomy.
tation, most notably combined with Various regimens of adjuvant chemotherapy
neoadjuvant chemoradiation, has shown and chemoradiation have shown modest sur-
promise as a treatment modality for highly vival benefit in phase II trials. Patients should
selected patients with hilar cholangiocarci- undergo postoperative surveillance at regular
noma. As such, referral to a transplant center intervals [1].
90 Cholangiocarcinoma 363
Patients may present with jaundice, pruritis, clay-colored stool, dark urine, abnormal
LFTs, dull RUQ pain, and/or weight loss
Obtain right upper No benign etiology of

No
High suspicion of quadrant ultrasound biliary obstruction found
malignancy?
Yes
B Obtain MRI/MRCP or MDCT, tumor markers (CA 19-9, CEA, AFP)
Intrahepatic No Intrahepatic biliary No No Alternative

Extrahepatic biliary diagnosis likely
mass on ductal dilation and/or
ductal dilation on
imaging? perihilar mass on
imaging?
imaging?
Yes Yes
C
Suggests hilar
cholangiocarcinoma
Pancreatic head Yes
No mass or
Positive AFP? Suggests intrahepatic choledocholithiasis
cholangiocarcinoma seen?
Yes No
Suggests HCC or Suggests distal extrahepatic Consider

mixed HCC/CCA cholangiocarcinoma EUS/ERCP
Options include
Yes D
chemotherapy, clinical Metastatic Complete staging workup and evaluate resectability of tumor
trial, best supportive care, disease?
biliary drainage if
indicated.
Intrahepatic cholangiocarcinoma
Surgical management includes hepatic E
No resection. Consider staging
Options include laparoscopy, portal lymphadenectomy.
chemotherapy, clinical trial,
best supportive care, biliary Supportive care,
drainage if indicated. Hilarcholangiocarcinoma surveillance,
No Yes
Consider locoregional Resectable Surgical management includes consider
therapy for intrahepatic tumor? resection of involved biliary tract adjuvant therapy
cholangiocarcinoma. usually withen bloc liver resection.
Consider referral to Consider staging laparoscopy.
transplant center. Biopsy
after transplant evaluation. Distal extrahepatic cholangiocarcinoma
Surgical management includes F
pancreaticoduodenectomy.
Algorithm 90.1
364 Z. J. Senders et al.
References 4. Jarnagin WR, Fong Y, DeMatteo RP, Gonen M, Burke

EC, Bodniewicz BSJ, et al. Staging, resectability, and
outcome in 225 patients with hilar cholangiocarci-
1. Benson AB III. NCCN guidelines: hepatobiliary can-
noma. Ann Surg. 2001;234(4):507–17. discussion
cers. NCCN Clin Pract Guidel Oncol. 2017;2:39–47.
517-9.
2. DeOliveira ML, Cunningham SC, Cameron JL,
5. Valle J, Wasan H, Palmer DH, Cunningham D,
Kamangar F, Winter JM, Lillemoe KD, et al.
Anthoney A, Maraveyas A, et al. Cisplatin plus gem-
Cholangiocarcinoma. A spectrum of intrahe-
citabine versus gemcitabine for biliary tract Cancer. N
patic, perihilar, and distal tumors. Ann Surg.
Engl J Med. 2010;362(14):1273–81.
2007;245(5):755–62.
6. Rosen CB, Heimbach JK, Gores GJ. Liver trans-
3. Abu-Hamda EM, Baron TH. Endoscopic manage-
plantation for cholangiocarcinoma. Transpl Int.
ment of cholangiocarcinoma. Semin Liver Dis.
2010;23(7):692–7.
2004;24(2):165–75.
of Gallbladder Cancer 91
Joshua L. Lyons, John B. Ammori,
and Jeffrey M. Hardacre
Algorithmic Approach resent a variety of benign lesions, any lesion

>1 cm is an indication for surgery as it carries
While uncommon with less than 5000 diagnoses a higher likelihood of malignancy [6]. Cross-
per year in the United States, gallbladder cancer sectional imaging with CT scan of the chest,
(GC) is the most frequently diagnosed cancer of abdomen, and pelvis or magnetic resonance
the biliary tract. It has a high mortality rate due to imaging (MRI)/magnetic resonance cholangi-
its frequently advanced stage at diagnosis [1]. Its opancreatography (MRCP) with chest com-
incidence increases with age, and it is more computed tomography (CT) should be performed
mon in women and blacks [2]. Risk factors preoperatively to evaluate for metastatic spread
include cholelithiasis, gallbladder polyps, obesity, and local invasion. Positron emission tomogra-
and chronic infections of the gallbladder [3, 4]. phy (PET)/CT is done selectively. Endoscopic
Diagnosis of GC is made in one of three ways: ultrasound can further differentiate between
preoperatively, intraoperatively, or postopera- benign and malignant lesions and also has the
tively. Due to the asymptomatic nature of early ability to stage the tumor, although it is not
GC, approximately 50% of diagnoses are inci- commonly used. While there is no laboratory
dentally discovered postoperatively on pathology value that has sufficient specificity or sensitiv-
[5]. The course of treatment and treatment goals ity in the diagnosis of GC, an elevated CA
are different for the varying presentations and 19-9 can be useful. After the workup is com-
therefore will be discussed separately. pleted, the patient is deemed appropriate for
surgery if the mass is locally resectable with-
A. Gallbladder cancer can be suspected preopera- out metastatic spread. At surgery, diagnostic
tively on various imaging modalities. laparoscopy is performed to rule out distant
Ultrasonography is the most common modal- metastases followed by resection at the same
ity used for evaluation of the gallbladder and setting. If the gallbladder mass or polyp is
findings that suggest GC include an intralumi- between 1 and 2 cm without evidence of inva-
nal or fixed mass, no discernable plane between sion, then a laparoscopic cholecystectomy
the liver and gallbladder, and/or mural calcifi- with intraoperative frozen section is appropri-
cations. While small lesions (<1 cm) can rep- ate. Care must be taken to avoid entry into the
gallbladder or bile spillage. Conversion to
J. L. Lyons · J. B. Ammori · J. M. Hardacre (*) open surgery is warranted if findings suggest
Department of Surgery, University Hospitals malignancy or there is no clear plane between
Cleveland Medical Center, Cleveland, OH, USA the gallbladder and liver bed. If frozen section

366 J. L. Lyons et al.
is positive, then oncologic surgery is under- sion should be performed. This second proce-
taken at the same setting. If the gallbladder dure is required due to the high incidence of
mass or polyp is greater than 2 cm, then an residual disease and therefore does convey a
oncologic surgery should be undertaken from survival benefit [8].
the outset. The operation of choice in GC is D. Surgery is the only potentially curative ther-
an extended cholecystectomy which involves apy for GC. Five-year survival for stage IA
resection of the gallbladder and hepatic bed carcinoma is 50% compared to 2% in stage IV
to a negative margin (usually liver segments carcinoma [9]. If an R0 resection is achieved
IVb and V but may require a more extensive on final pathology with no lymph node
hepatectomy), regional lymphadenectomy, involvement, observation or adjuvant chemo-
and cystic/bile duct excision to a negative mar- radiation versus chemotherapy is reasonable.
gin (if cystic margin is positive, a bile duct There are no historical randomized controlled
resection with subsequent reconstruction is trials (RCT) to guide adjuvant therapy for bili-
necessary). ary tract malignancies. However, a recent
B. If a suspicious mass is encountered intraopera- RCT of 447 patients with biliary tract malig-
tively, it is recommended that the operation be nancies (18% were GC) showed a survival
converted to open in order to have better tactile benefit with single agent capecitabine in the
feedback and minimize the risk of entering the adjuvant setting [10]. While further studies
gallbladder. It is also accepted to stop the oper- are needed to compare capecitabine with cur-
ation at this point and refer the patient to a high- rent adjuvant therapy regimes (gemcitabine
volume center. An open cholecystectomy and cisplatin [11]), it emphasizes the need for
should then be performed without bile spillage multidisciplinary care. In addition, there have
and the mass sent for frozen section exam. If been retrospective studies that have shown a
the mass is adherent to the liver bed, it is impor- survival advantage with radiation alone and
tant to resect a portion of the liver in order to chemoradiation [12, 13]. There are no data
avoid violating the tumor and seeding the peri- with regard to surveillance, but a reasonable
toneum. If the frozen section exam is positive schedule could include repeat imaging every
for malignancy and the surgeon is comfortable 6 months for 2 years and then annually for
with proceeding, an extended cholecystectomy 5 years. If the resection has a positive margin
and lymphadenectomy should be done. If the or there is gross residual disease or regional
surgeon is not comfortable proceeding, closing lymph node involvement, the patient should
the patient with referral to a high-volume cen- be referred for adjuvant chemoradiation ver-
ter is the preferred course. sus chemotherapy. There is no role for pallia-
C. Gallbladder cancer is diagnosed most com- tive, debulking, or repeat resections as these
monly on pathologic exam of a cholecystec- procedures do not convey a survival or pallia-
tomy specimen done for benign biliary tive benefit. If the patient has obstructive jaun-
disease. Management is dependent on the dice, endoscopic or percutaneous stenting is
tumor staging. If the tumor is Tis or T1a with preferred over biliary bypass. Multidisciplinary
negative margins, no further resection is nec- cancer care teams are important to guide the
essary, as this does not convey a survival ben- management of this disease.
efit [7]. If the tumor is T1b or greater,
cross-sectional imaging of the chest, abdo- Gallbladder cancer is an uncommon yet often
men, and pelvis should be performed preop- fatal malignancy. However, in selected patients,
eratively to evaluate for metastatic spread and surgical intervention can be curative. It is
local invasion. If no metastatic spread is therefore prudent for surgeons to develop a sys-
found, then diagnostic laparoscopy followed tematic management plan in the event that GC is
by potentially curative hepatic resection, diagnosed on pathologic review or during routine
lymphadenectomy, and cystic/bile duct exci- cholecystectomy.
91 Diagnosis and Management of Gallbladder Cancer 367
Gallbladder cancer
Gallbladder mass on imaging Gallbladder cancer Gallbladder cancer diagnosed

A (US or EUS)
B C
suspected intraoperatively on pathology
MRI/MRCP with Chest CT

OR
Chest/Abd/Pelvis CT scan
Tis or T1a?
No
<1 cm 1–2 cm >2 cm
Yes
Yes MRI/MRCP with Chest CT
Concerning Frozen section exam OR
features? Chest/Abd/Pelvis CT scan
Observe
No
Mass is resectable
Laparoscopic with no evidence of
cholecystectomy metastatic spread?
Yes
Extended cholecystectomy with

lymphadenectomy and possible No
biliary tract
resection/reconstruction
No
R0 resection and
no lymph node Chemoradiation, chemotherapy,
involvement? or clinical trial
Yes
Consider adjuvant chemotherapy ± radiation vs observation
Algorithm 91.1
368 J. L. Lyons et al.
References 8. Shoup M, Fong Y. Surgical indications and extent of

resection in gallbladder cancer. Surg Oncol Clin N
Am. 2002;11(4):985–94.
1. Carriaga MT, Henson DE. Liver, gallbladder, 9. Fong Y, Wagman L, Gonen M, Crawford J, Reed
extrahepatic bile ducts, and pancreas. Cancer. W, Swanson R, Pan C, Ritchey J, Stewart A,
1995;75(S1):171–90. Choti M. Evidence-based gallbladder cancer stag-
2. Scott TE, Carroll M, Cogliano FD, Smith BF, Lamorte ing: changing cancer staging by analysis of data
WW. A case-control assessment of risk factors for gall- from the National Cancer Database. Ann Surg.
bladder carcinoma. Dig Dis Sci. 1999;44(8):1619–25. 2006;243(6):767.
3. Okamoto M, Okamoto H, Kitahara F, Kobayashi 10. Primrose JN, Fox R, Palmer DH, Prasad R, Mirza
K, Karikome K, Miura K, Matsumoto Y, Fujino D, Anthoney DA, Corrie P, Falk S, Wasan HS, Ross
MA. Ultrasonographic evidence of association of PJ, Wall LR. Adjuvant capecitabine for biliary tract
polyps and stones with gallbladder cancer. Am J cancer: the BILCAP randomized study. J Clin Oncol.
Gastroenterol. 1999;94(2):446–50. 2017;35(suppl; abstr 4006):4006.
4. Dutta U, Garg PK, Kumar R, Tandon RK. Typhoid 11.
Malik IA, Aziz Z, Zaidi MS, Sethuraman
carriers among patients with gallstones are at G. Gemcitabine and cisplatin is a highly effec-
increased risk for carcinoma of the gallbladder. Am J tive combination chemotherapy in patients with
Gastroenterol. 2000;95(3):784–7. advanced cancer of the gallbladder. Am J Clin Oncol.
5. Duffy A, Capanu M, Abou-Alfa GK, Huitzel D, 2003;26(2):174–7.
Williams P, Jarnagin W, Fong Y, Kelsen DP, O’Reilly 12. Nakeeb A, Tran KQ, Black MJ, Erickson BA,

EM. Gallbladder cancer (GBC): 10-year experience at Ritch PS, Quebbeman EJ, Wilson SD, Demeure
memorial Sloan-Kettering Cancer Centre (MSKCC). MJ, Rilling WS, Dua KS, Pitt HA. Improved sur-
J Clin Oncol. 2007;25(18_suppl):4648. vival in resected biliary malignancies. Surgery.
6. Toda K, Souda S, Yoshikawa Y, Momiyama T, 2002;132(4):555–64.
Ohshima M. Significance of laparoscopic excisional 13. Kresl JJ, Schild SE, Henning GT, Gunderson LL,
biopsy for polypoid lesions of the gallbladder. Surg Donohue J, Pitot H, Haddock MG, Nagorney
Laparosc Endosc Percutan Tech. 1995;5(4):267–71. D. Adjuvant external beam radiation therapy with
7. Taner CB, Nagorney DM, Donohue JH. Surgical concurrent chemotherapy in the management of gall-
treatment of gallbladder cancer. J Gastrointest Surg. bladder carcinoma. Int J Radiat Oncol Biol Phys.
2004;8(1):83–9. 2002;52(1):167–75.
Choledochal Cysts
92
Shreya Gupta, Jeffrey M. Hardacre,
and John B. Ammori
Algorithmic Approach ital, they often present in adult years with

vague right upper quadrant symptoms, often
Five types of CC are described in this classifica- leading to cholecystectomy for presumed
tion. Type I CC (80–90% of all CC) is a fusiform gallbladder disease. Neonates with obstruc-
dilation of the common bile duct. Type II CC is a tive jaundice and palpable abdominal mass
true diverticula of the common bile duct (CBD). are usually diagnosed promptly [2].
Type III CC is an intraduodenal dilation of the B. Ultrasound is often obtained to evaluate for
common channel also known as choledochocele. right upper quadrant pain or jaundice. An
Type IVA CC (15–20% of all CC) is multiple dila- ultrasound finding of a common bile duct dila-
tions of the intra- and extrahepatic biliary tree, tion >10 mm should alert the physician to
whereas type IV B CC is multiple extrahepatic investigate for choledochal cyst [2]. Ultrasound
dilations only. Type V CC or Caroli’s disease is findings suggestive of CC should be investi-
intrahepatic biliary tree dilation only. Symptoms gated with magnetic resonance cholangiopan-
of CC include abdominal pain, jaundice, and creatography (MRCP), which is considered to
often a palpable abdominal mass if presenting at be a gold standard of diagnosing all types of
the age of <10 years. About 20% of patients are CC. Endoscopic retrograde cholangiopancrea-
older than 20 years of age with the most common tography (ERCP) is unnecessary for diagnosis
symptom of abdominal pain. Untreated CC com- as it is more invasive and increases the risks of
plications include cholangitis, pancreatitis, and cholangitis or pancreatitis [3, 4].
obstructive jaundice. Cancer has been associated C. The type of CC dictates the surgical manage-
with all subtypes of biliary cysts but is most com- ment. Type II requires diverticulectomy or
monly found in type I and type IV CC. simple excision. Type III CC or choledocho-
cele, on the other hand, requires ERCP with
A. Vague patient presentation is often mislead- sphincterotomy. The risk of malignancy is
ing and leads to delayed diagnosis. The most reported to be very low in both type II and III
common presenting symptom is abdominal CC [2].
pain [1]. Even though these cysts are congen- D. Type I and IVB CC warrant surgery including
complete cyst excision and Roux-en-Y hepat-
icojejunostomy for restoration of biliary con-
S. Gupta · J. M. Hardacre (*) · J. B. Ammori tinuity. Some surgeons suggest the use of
Department of Surgery, University Hospitals intraoperative frozen sections to rule out
Cleveland Medical Center, Cleveland, OH, USA dysplasia or malignancy. However, malignancy

370 S. Gupta et al.
can develop anywhere in the biliary tract is <7%, but surgical intervention or liver
including the gallbladder [5]. The literature transplant is warranted secondary to cholan-
suggests a 0.7–6% post-excisional malig- gitis and liver dysfunction/failure [2]. Given
nancy rate in patients with remnant cyst tissue the risk of malignancy in type I and IV CCs,
or subclinical malignant disease that is not postoperative surveillance is performed with
detected during surgery [2].Type IVA and V ultrasonography or cross-sectional imaging
(Caroli’s disease) CC involve the intrahepatic as well as liver enzymes to detect early can-
biliary and may require partial hepatectomy cer. The risk of malignancy is approximately
[6, 7]. 0.7–6% even in complete excision, primarily
E. Type V may require liver transplantation for due to undetectable cancerous lesions before
pan-liver involvement. The risk of neoplasia or at the time of surgery [1, 3, 4].
A Patient presents with vague right upper quadrant pain, jaundice,

nausea and vomiting
B Ultrasound of the right upper quadrant abdomen/MRCP
Type II Choledochocele-
Type III ERCP and
Simple Type of choledochal cyst
excision Sphincterotomy,
C if symptomatic
Type I/IV B
Type IV A/V
Hepatectomy for excision of the cyst,

D and reconstruction with wide hilar Complete excision with Roux-en- Y Hepaticojejunostomy
Roux-en-Y hepaticojejunostomy.
Follow up with surveillance ultrasonography and liver

E Possible liver transplant
enzymes
Algorithm 92.1
92 Choledochal Cysts 371
References 4. Martin RF. Biliary cysts. Surg Clin N Am.

2014;94(2):219–32. https://doi.org/10.1016/j.
suc.2014.01.011. ISSN 0039-6109.
1. Todani T, Tabuchi K, Watanabe Y, Kobayashi
5. Singham J, Yoshida EM, Scudamore CH. Choledochal
T. Carcinoma arising in the wall of congenital bile
cysts: Part 2 of 3: diagnosis. Can J Surg.
duct cysts. Cancer. 1979;44(3):1134–41.
2009;52(6):506–11.
2. Soares KC, Arnaoutakis DJ, Kamel I, Rastegar N,
6. Singham J, Yoshida EM, Scudamore CH. Choledochal
Anders R, Maithel S, Pawlik TM. Choledochal cysts:
cysts: Part 3 of 3: management. Can J Surg.
presentation, clinical differentiation, and manage-
2010;53(1):51–6.
ment. J Am Coll Surg. 2014;219(6):1167–80. https://
7. Roukounakis N, Manolakopoulos S, Tzourmakliotis
doi.org/10.1016/j.jamcollsurg.2014.04.023. ISSN
D, Bethanis S, Mccarty TM, Cuhn J. Biliary tract
1072-7515.
malignancy and abnormal pancreaticobiliary junc-
3. Ohashi T, Wakai T, Kubota M, et al. Risk of sub-
tion in a Western population. J Gastroenterol Hepatol.
sequent biliary malignancy in patients undergoing
2007;22(11):1949–52.
cyst excision for congenital choledochal cysts. J
Gastroenterol Hepatol. 2013;28(2):243–7.
Cholecystectomy of the Pregnant
Patient 93
Avi Hameroff and Jaimey M. Pauli
Algorithmic Approach Additionally, ultrasound should be consid-

ered first line as part of the workup for RUQ
A. Gallbladder disease (GD) is a wide spectrum pain [5].
of disorders of the gallbladder and biliary C. Although cholecystectomy may be performed
tract. Increased levels of sex steroid hor- via open or laparoscopic approaches depend-
mones in pregnancy and the postpartum ing on surgeon preference and experience,
period contribute to biliary stasis, with gall- laparoscopic cholecystectomy is safe and
stones occurring in 7% of nulliparous women effective at treating GD during pregnancy and
and 19% of multiparous women [1, 2]. may decrease the frequency of antepartum
Complicated GD (CGD; cholecystitis, chol- and postpartum admissions of affected patient
angitis, gallstone pancreatitis) is the third [6–8]. The laparoscopic approach has been
most common cause of rehospitalization found to have shorter operative times,
within 60 days of delivery, and compared decreased hospital length of stay, and reduced
with women who deliver vaginally, Cesarean complications with similar risk of antenatal
section increases the risk of CGD [3]. complications compared to the open tech-
B. Initial evaluation of a pregnant patient sus- nique [9, 10]. It has additionally been found
pected to have GD includes history and phys- to be safe in any trimester, and significant
ical exam. Symptoms of cholecystitis include maternal and/or fetal morbidity, including
fever, nausea, and ill appearance, whereas fetal loss, may result from delay in surgical
cholangitis is described as presenting with management [11].
Charcot’s triad of fever, right upper quadrant D. Practitioners should have a low threshold to
(RUQ) pain, and jaundice but is only seen in recommend surgical intervention, although
about 50% of cases [4]. Labs may be drawn to medical management with ursodeoxycholic
evaluate serum bilirubin, alkaline phospha- acid is an option and is generally considered
tase, aspartate aminotransferase (AST), ala- safe during pregnancy. Consultation with an
nine aminotransferase (ALT), and amylase. obstetrician is recommended to assist with
Bile salts may also help in the assessment. monitoring and delivery planning of the fetus,
as well as coordination of surgery if Cesarean
A. Hameroff · J. M. Pauli (*) section is indicated.
Maternal-Fetal Medicine, Department of Obstetrics E. Strategies to minimize the effect of surgery
and Gynecology, Penn State Health Milton S. (open or laparoscopic) on the pregnancy
Hershey Medical Center, Hershey, PA, USA include the following: early diagnosis,

374 A. Hameroff and J. M. Pauli
inimal uterine manipulation, continuous

m matic compression devices, fetal and uterine
urinary catheterization and gastric emptying, activity monitoring during and after surgery,
open entry for laparoscopy, alternative entry consideration of tocolytic therapy, and ante-
sites to avoid the gravid uterus, left lateral natal steroid administration for gestational
recumbent positioning, maintaining intraab- age >23–24 weeks [12].
dominal pressure <12 mm Hg, use of pneu-
History:
A Right upper quadrant pain (especially in relation to eating
and/or cyclic throughout day), anorexia, chills
B Exam:
Vital signs to assess temperature, heart rate, blood pressure
Labs:
CBC, AST/ALT, alk phos, total bilirubin, bile acids, amylase
Ultrasound:
Presence of gallstones, biliary sludge, dilation of biliary tracts
Complicated Uncomplicated
C gallbladder disease? gallbladder disease?
Consultation with OB for fetal monitoring, Consultation with OB for fetal monitoring,
delivery planning delivery planning
Proceed to cholecystectomy (laparoscopy

preferred)
Conservative or medical management Proceed to cholecystectomy

D
with ursodeoxycholic acid (laparoscopy preferred)
Ensure close follow-up with OB (+/– antenatal

surveillance)
E Develop plan for cholecystectomy if symptoms
worsen
Plan elective cholecystectomy postpartum
Algorithm 93.1
93 Cholecystectomy of the Pregnant Patient 375
References stone disease in pregnancy is associated with recur-

rent postpartum symptoms. J Gastrointest Surg.
2013;17(11):1953–9.
1. Gilat T, Konikoff F. Pregnancy and the biliary tract.
8. Muench J, Albrink M, Serafini F, Rosemurgy A,
Can J Gastroenterol. 2000;14(Suppl D):55D–9D.
Carey L, Murr MM. Delay in treatment of biliary dis-
2. Pataia V, Dixon PH, Williamson C. Pregnancy and
ease during pregnancy increases morbidity and can be
bile acid disorders. Am J Physiol Gastrointest Liver
avoided with safe laparoscopic cholecystectomy. Am
Physiol. 2017;313(1):G1–6.
Surg. 2001;67(6):539–42. discussion 542–543.
3. Lydon-Rochelle M. Association between method
9. Cox TC, Huntington CR, Blair LJ, Prasad T,
of delivery and maternal rehospitalization. JAMA.
Lincourt AE, Augenstein VA, et al. Laparoscopic
2000;283(18):2411.
appendectomy and cholecystectomy versus open:
4. Avegno J, Carlisle M. Evaluating the patient with
a study in 1999 pregnant patients. Surg Endosc.
right upper quadrant abdominal pain. Emerg Med
2016;30(2):593–602.
Clin North Am. 2016;34(2):211–28.
10. Corneille MG, Gallup TM, Bening T, Wolf SE,

5. Revzin MV, Scoutt LM, Garner JG, Moore CL. Right
Brougher C, Myers JG, et al. The use of laparoscopic
upper quadrant pain: ultrasound first!: RUQ Pain:
surgery in pregnancy: evaluation of safety and effi-
Ultrasound first! J Ultrasound Med [Internet]. 2017
cacy. Am J Surg. 2010;200(3):363–7.
Jun 6 [cited 2017 Aug 18]; Available from: http://doi.
11. Jackson H, Granger S, Price R, Rollins M, Earle

wiley.com/10.1002/jum.14274.
D, Richardson W, et al. Diagnosis and laparo-
6. Jorge AM, Keswani RN, Veerappan A, Soper NJ,
scopic treatment of surgical diseases during preg-
Gawron AJ. Non-operative management of symp-
nancy: an evidence-based review. Surg Endosc.
tomatic cholelithiasis in pregnancy is associated
2008;22(9):1917–27.
with frequent hospitalizations. J Gastrointest Surg.
12. Weiner E, Mizrachi Y, Keider R, Kerner R, Golan
2015;19(4):598–603.
A, Sagiv R. Laparoscopic surgery performed in
7. Veerappan A, Gawron AJ, Soper NJ, Keswani
advanced pregnancy compared to early pregnancy.
RN. Delaying cholecystectomy for complicated gall-
Arch Gynecol Obstet. 2015;293:1063–108.
Part XII
Pancreas
Acute Pancreatitis
94
Algorithmic Approach be present. Cullen’s sign (periumbilical

ecchymosis) or Grey Turner sign (flank
A. A diagnosis of acute pancreatitis (AP)
ecchymosis) occurs in the setting of retroperi-
requires two of the following criteria: acute toneal hematoma and pancreatic necrosis.
onset of persistent, severe, upper abdominal D. Lipase is more sensitive than amylase in pan-
pain; elevation in serum lipase to three times creatitis. Sensitivity and specificity for AP
the upper normal limit; and imaging (ultra- range from 82% to 100% [2]. Elevations in
sound (US), computed tomography (CT), lipase occur earlier and last longer compared
magnetic resonance imaging (MRI)) demon- with elevations in amylase.
strating characteristic findings [1]. E. Patients with moderately severe or severe AP,
B. AP is classified based on three levels of sever- sepsis, or clinical deterioration 72 h after ini-
ity. Mild acute pancreatitis is characterized tial presentation should undergo contrast-
by the lack of organ failure and local and sys- enhanced CT scan to assess for necrosis or
temic complications. Moderately severe pan- local complications.
creatitis includes transient organ failure (less F. Severity of AP should be evaluated via exam
than 48 h) and/or local or systemic complica- to assess for fluid losses, organ failure, acute
tions without persistent organ failure. Severe physiology and chronic health evaluation
acute pancreatitis is characterized by persis- (APACHE) II score, and systemic inflamma-
tent organ failure in one or more organs. tory response syndrome (SIRS) score.
C. On physical exam, there may be a range of G. Indications for admission to a monitored set-
tenderness to palpation of the epigastrium, as ting or ICU care include hemodynamic or
well as abdominal distention and hypoactive respiratory instability, electrolyte dysregula-
bowel sounds. In severe cases, patients may tion, hyperglycemia, anuria, or changes in
be febrile, hypotensive, tachypnic, and mental status and, additionally, those with
hypoxemic. Jaundice or scleral icterus may SIRS >48 h, age >60 years, APACHE II >8 at
24 h, hematocrit >44%, creatinine >1.8 mg/
K. M. Hartz (*) dL, BUN >20 mg/dL, or coexisting cardio-
Edward Via College of Osteopathic Medicine, pulmonary disease [3].
Blacksburg, VA, USA H. Main goals of initial management consist of
J. Maranki fluid resuscitation, pain control, and nutri-
Department of Gastroenterology and Hepatology, tional support.
Penn State Milton S. Hershey Medical Center,
Hershey, PA, USA

380 K. M. Hartz and J. Maranki
(a). Intravenous hydration with lactated collections, and walled-off necrosis.

Ringer’s (LR) solution or normal saline Pseudocysts and walled-off necrosis occurs
at a rate of 5–10 mL/kg/h. Avoid LR in typically greater than 4 weeks after the initial
hypercalcemic patients. Some data sug- presentation. Infected necrosis should be sus-
gest LR may decrease the SIRS response. pected in patients who clinically deteriorate
Frequently assess adequacy of fluid (elevated white count, fevers, sepsis-like clin-
resuscitation. Goals are MAPs ical picture) or fail to improve after 7–10 days.
65–85 mmHg, urine output >0.5 to 1 cc/ Management of fluid collections and necrosis
kg/h, hematocrit reduced to 35–44%, is discussed elsewhere.
heart rate <120 bpm, and reductions in J. In patients with gallstone pancreatitis who
blood urea nitrogen (BUN) [4]. Limit develop cholangitis, ERCP with sphincterot-
fluid resuscitation to the first 24–48 h. omy should be performed within 24 h. If clin-
(b). Intravenous opioids are effective at pro- ical suspicion for biliary obstruction is
viding pain control. Frequently patient- present without overt cholangitis, an endo-
controlled analgesia (PCA) pumps are scopic ultrasound (EUS) or a magnetic reso-
used. Patients should be closely moni- nance cholangiopancreatography (MRCP)
tored for side effects. should be performed for the evaluation of

(c). Nutritional support is required for common bile duct stones.
patients who are unable to tolerate on K. Cholecystectomy should be performed after
oral diet within 7 days. Nasojejunal tube recovery in all patients with gallstone pancre-
feeding is preferred to total parenteral atitis. For those with mild AP, it should be
nutrition and is recommended for those performed during the index hospitalization
with moderately severe and severe AP [5]. Failure to perform a cholecystectomy is
who cannot tolerate an oral diet. associated with a 25–30% risk of recurrent
I. Local and systemic complications may AP, cholangitis, or cholecystitis within
develop and include acute peripancreatic 6–18 weeks [6].
fluid collections, pseudocysts, acute necrotic
94 Acute Pancreatitis 381
Symptoms of persistent upper abdominal pain

plus serum lipase >3 x ULN and/or cross-sectional
A imaging demonstrating findings characteristic of
pancreatic inflammation
Diagnosis of acute pancreatitis (AP)
B
Organ failure or No
local/systemic Mild AP
complications?
Yes, transient Yes, persistent
Moderately severe
Severe AP
AP
Physical exam/labs: TTP epigastrium, distention,

C, D, G hypoactive bowel sounds, febrile, hemodynamic
instability, jaundice, Cullen’s/Grey Turner signs;
elevated lipase/amylase; elevated glucose,WBC;
elevated BUN/Cr, Hct; electrolyte abnormalities
Assess for ICU monitoring
Treat with LR or NS, 5–10 ml/kg/h

Goal MAP 65-85 mmHg, urine output >0.5 to 1
H cc/kg/h, hematocrit reduced to 35-44%,
heart rate < 120 bpm, reductions in BUN
Pain control
Nutritional support if >7 days with no POs
Algorithm 94.1
E,F
Clinical deterioration
or failure to improve
after 72 h?
CT scan with contrast to assess for necrosis or

local complications
I
Fluid collection or
necrosis and fever,
leukocytosis, or sepsis
physiology?
Treat with IV abx for infected collection (typically

occurs 7–10 days after initial presentation; if no
improvement, debridement via necrosectomy
(wait 4 weeks if possible for wall to form)
If gallstone pancreatitis and cholangitis, ERCP with

J, K sphincterotomy within 24 h;
cholecystectomy within index hospitalization if
mild AP; wait for recovery in other cases
4. Working Group IAP/APA Acute Pancreatitis

References Guidelines. IAP/APA evidence-based guidelines for
the management of acute pancreatitis. Pancreatology.
1. Banks PA, Bollen TL, Dervenis C, et al. Classification 2013;13:e1.
of acute pancreatitis – 2012: revision of the Atlanta 5. Aboulian A, Chan T, Yaghoubian A, et al. Early cho-
classification and definitions by international consen- lecystectomy safely decreases hospital stay in patients
sus. Gut. 2013;62:102. with mild gallstone pancreatitis: a randomized pro-
2. Yadav D, Agarwal N, Pitchumoni CS. A critical eval- spective study. Ann Surg. 2010;251:615.
uation of laboratory tests in acute pancreatitis. Am J 6. Hernandez V, Pascual I, Almela P, et al. Recurrence
Gastroenterol. 2002;97:1309. of acute gallstone pancreatitis and relationship with
3. Wu BU, Banks PA. Clinical management of cholecystectomy or endoscopic sphincterotomy. Am J
patients with acute pancreatitis. Gastroenterology. Gastroenterol. 2004;99:2417.
2013;144:1272.
Chronic Pancreatitis
95
Algorithmic Approach D. A diagnosis of chronic pancreatitis may be

difficult. Pancreatic calcifications identified
A. Chronic pancreatitis is characterized by epi- on imaging is diagnostic; other findings
gastric abdominal pain, often radiating to the include beading and irregularity of the pan-
back, and frequently postprandial. Early in creatic duct with enlarged side branches and
the disease process, pain episodes may be an abnormal secretin study.
discrete, whereas the pain may become con- E. Serum amylase and lipase may be normal in
tinuous later in the course. In over 20% of chronic pancreatitis. Complete blood count
patients with chronic pancreatitis, pain is not (CBC) and basic metabolic panel (BMP) may
a feature of their disease. be normal. Elevations in LFTs may signify
B. Pancreatic insufficiency results from exocrine biliary compression through the intrapancre-
function that is severely compromised; clini- atic portion. Elevations in immunoglobulin
cally significant deficiencies occur when 90% G4 (IgG4), antinuclear antibody (ANA),
of exocrine function is lost. Fat malabsorp- rheumatoid factor (RF), erythrocyte sedimen-
tion presents with greasy, foul-smelling stools tation rate (ESR), anti-Smith (anti-Sm) anti-
and results in losses of vitamins A, D, E, K, body may indicate an autoimmune process.
and B12. Glucose intolerance is common, Increased fecal fat excretion or fecal elastase
and diabetes occurs late in the disease, par- may also aid in the diagnosis.
ticularly in patients with calcific pancreatitis. F. MRCP is the preferred diagnostic imaging
C. Patients may develop benign biliary strictures test since it can show irregularities of the pan-
as a result of chronic pancreatitis, as well as creatic duct (beading and ectatic side
duodenal stenosis, splenic vein thrombosis, branches) as well as calcifications. EUS is
pseudocysts, gastric outlet obstruction, pleu- also helpful in making the diagnosis, particu-
ral effusions and pancreatic ascites, and pan- larly in early disease. In addition to stones,
creatic cancer. suggestive findings include lobularity, cysts,
hyperechoic foci and strands, ductal dilation,
K. M. Hartz (*) and hyperechoic duct walls [1].
Edward Via College of Osteopathic Medicine, G. General treatment includes alcohol and

Blacksburg, VA, USA smoking cessation, hydration, small, low-fat
J. Maranki meals. Pancreatic enzyme supplementation
Department of Gastroenterology and Hepatology, may be helpful in pain control. Pain control
Penn State Milton S. Hershey Medical Center, should follow a step-up pattern, starting with
Hershey, PA, USA

TCAs (amitriptyline or nortriptyline). poreal shock wave lithotripsy. Surgical

NSAIDs may be used in the short-term in approaches may achieve decompression or
conjunction with TCAs. Pregabalin may also drainage, denervation, or resections.
provide pain control. Chronic opioids may be Decompression procedures, such as Frey or
needed for those with persistent significant Puestow, are suited for those with pancre-
pain. Long-acting agents such as MS Contin atic duct dilation and may achieve pain con-
or fentanyl patches may be beneficial. An opi- trol in 60–80% of patients. Resection
ate contract should be maintained by the pre- procedures are reserved for those with a
scribing physician, and a pain management nondilated duct and localized disease.
consultation may be beneficial. [2] Autologous islet cell transplantation may be
H. Additional therapies such as celiac plexus beneficial in post-resection patients with
block, pancreatic duct stenting, and extracor- pancreatic insufficiency.
95 Chronic Pancreatitis 385
History: persistent epigastric abdominal pain, often radiating to the back.

A Pain episodes may initially be episodic but become continuous as the
disease progresses. Pain is not a feature in around 20%
Check 72-h fecal fat or fecal elastase; vitamins A,D,E,K, and B12 may become
B, C, E deficient; assess for glucose intolerance/diabetes; amylase and lipase may be
normal; LFTs may be elevated if biliary obstruction
D Obtain imaging such as RUQ U/S, CT, or MRI to assess for biliary
dilation/stricture, panc calcifications, duodenal stricture/GOO,
effusions/ascites, pancreatic cancer, and pseudocysts
Findings
concerning
chronic
pancreatitis?
Check MRCP to assess for ductal irregularities; perform EUS to exclude

F pancreatic cancer and to assess for parenchymal and ductal characteristics of
chronic pancreatitis
Tx: smoking/EtOH cessation, hydration, low-fat diet, pancreatic enzyme

G supplementation (use PPI if non-enteric coated), TCAs, NSAIDs for short term. If
opiates are needed, rec narcotic contract
If sxs persist: ERCP with PD stenting, CBD stenting if benign biliary

H stricture; celiac plexus block, ESWL for PD stones; surgical drainage
or resection, autologous islet cell transplantation
Algorithm 95.1
atitis: interobserver agreement among experienced

References ultrasonographers. Gastrointest Endosc. 2001;53:294.
2. Braganza JM, Lee SH, McCloy RF, McMahon
1. Wallace MB, Hawes RH, Durkalski V, et al. The reli- MJ. Chronic pancreatitis. Lancet. 2011;377:1184.
ability of EUS for the diagnosis of chronic pancre-
Pancreas Divisum
96
Algorithmic Approach C. From the embryologic foregut, the dorsal

and ventral anlages fuse to form the pan-
A. The first step in the evaluation of a patient creas. Pancreas divisum occurs when the
with suspected pancreas divisum is obtaining dorsal and ventral duct systems fail to fuse.
a thorough history and physical examination. Classic or complete pancreas divisum is
Key aspects of the history include recurrent when the dorsal duct (Santorini) becomes
pancreatobiliary pain, mild acute pancreatitis, enlarged and drains the body and tail through
recurrent idiopathic pancreatitis, or the pres- the minor papilla. The ventral duct (Wirsung)
ence of chronic pancreatitis. While pancreas is disconnected from the main duct and often
divisum may be present in up to 10% of the drains the uncinate. Incomplete pancreas
population, less than 5% of these individuals divisum is when a small branch of the ventral
will become symptomatic. However, in duct communicates with the dorsal duct.
patients with idiopathic pancreatitis, pancreas Reverse divisum may occur if the dorsal duct
divisum may be present in up to 50% [1]. is isolated from the main duct. This is clini-
B. Diagnosis is usually made via cross-sectional cally significant in the setting of gallstone
imaging, including CT scan or MRCP. If the pancreatitis, which may cause a more severe
pancreatic duct is visualized on CT scan, pancreatitis than would have occurred in a
axial images demonstrate the dorsal duct pancreas with typical anatomy.
coursing superiorly and anteriorly to the dis- D. Additional evaluation and/or treatment is
tal common bile duct and draining at the dependent on whether the patient is symp-
minor papilla. The presence of a fat plane tomatic or not. For asymptomatic individu-
between the dorsal and ventral segments is als, no further additional evaluation should
suggestive of pancreas divisum. be pursued. In symptomatic patients, a
secretin-enhanced MRCP and/or endoscopic
ultrasound provides additional information
about the pancreas and improves ductal
K. M. Hartz (*) visualization.
Edward Via College of Osteopathic Medicine, E. ERCP is reserved for endoscopic therapy of
Blacksburg, VA, USA pancreas divisum but may also provide diag-
J. Maranki nostic information. Features of pancreas divi-
Department of Gastroenterology and Hepatology, sum on ERCP are a thin, short ventral duct
Penn State Milton S. Hershey Medical Center, and, when cannulating the dorsal duct, the
Hershey, PA, USA

filling of the entire dorsal duct to the tail. consists of a low-fat diet, analgesics, and
Other suggestive findings are delayed drain- pancreatic enzyme supplementation. For
age of the dorsal duct, a santorinicele, and the patients with severe or recurrent symp-
presence of pain during dorsal ductography. toms, minor papillotomy may be per-
formed, particularly in patients with two or
F. For patients with minimal symptoms, con- more bouts of acute pancreatitis with no
servative treatment is recommended. This other etiology [2].
History and physical exam: recurrent

A pancreatobiliary pain, mild acute
pancreatitis, recurrent idiopathic
pancreatitis, chronic pancreatitis
Obtain cross-sectional imaging (CT or MRCP). Dorsal duct will

B course superiorly and anteriorly to the distal common bile duct
and drain at minor papilla
Assess type of pancreas divisum based on features of the ventral duct. Classic pancreas
divisum occurs when Wirsung is disconnected from Santorini, whereas incomplete pancreas
C divisum describes the presence of a small branch of Wirsung communicating with the
dorsal duct
No
D Is the patient
symptomatic? No further workup is needed
Yes
E Secretin-enhanced MRCP and/or EUS
Minimal Sxs: conservative tx with low-fat diet, analgesics, pancreatic enzymes

F Severe/recurrent Sxs: ERCP with minor papillotomy
Algorithm 96.1
96 Pancreas Divisum 389
References 2. Lehman GA, Sherman S, Nisi R, Hawes RH. Pancreas

divisum: results of minor papilla sphincterotomy.
1. Bernard JP, Sahel J, Giovannini M, Sarles H. Pancreas
divisum is a probable cause of acute pancreatitis: a
report of 137 cases. Pancreas. 1990;5:248.
Walled-Off Pancreatic Fluid
Collections 97
Algorithmic Approach (a) History of pancreatitis or pancreatic

trauma
A. Symptoms of PC and WOPN may include (b) PC: round or oval, well-defined fluid col-
abdominal pain, fever/infection, gastric outlet lection with a clear “rind” or wall around
obstruction, biliary obstruction, venous the collection; homogenous fluid density;
thrombosis or pseudoaneurysm, or fistulas, usually extrapancreatic
hemosuccus pancreaticus, pancreatic ascites, (c) WOPN: well-defined wall around a het-
or pleural effusions. erogenous collection with solid and liq-
B. Walled-off pancreatic fluid collections con- uid components; may be multiloculated
sist of pseudocysts (PC) and walled-off pan- (d) If diagnosis is uncertain, EUS- or CT-
creatic necrosis (WOPN) [1]. guided sampling of fluid is helpful. Fluid
(a) Both typically become mature at least analysis will show an elevated amylase
4 weeks after the onset of acute and low CEA levels; gram stain and cul-
pancreatitis. ture may be helpful in directing antibiot-
(b) PC have a well-defined wall and consist ics for infected collections.
of only liquid (no solid component or D. Treatment depends on the presence of symp-
necrosis). toms or complications. Options for drainage
(c) WOPN is an encapsulated collection of include endoscopic, percutaneous, or surgical
necrosis that contains liquid and solid approaches.
components. (a) Endoscopic cystgastrostomy or cystduo-
C. For diagnosis, a history suggestive of pancre- denostomy is suitable for collections
atitis or pancreatic trauma, plus imaging (CT abutting the gastrointestinal (GI) lumen
or MRI), and sometimes fluid sampling is and accessible with EUS. Placement of a
required. lumen-apposing metal stent or two
double-pigtail stents will provide drain-
age of the liquid component. If necrosis
K. M. Hartz (*) or solid debris is found, necrosectomy
Edward Via College of Osteopathic Medicine, may be needed. Nasocystic drainage tube
Blacksburg, VA, USA placement is also an option. Endoscopic
J. Maranki retrograde cholangiopancreatography
Department of Gastroenterology and Hepatology, (ERCP) with pancreatic duct stenting
Penn State Milton S. Hershey Medical Center, may help heal leak and resolve fluid
Hershey, PA, USA

collection [2]. Multiple procedures may tomy may be needed in the case of dis-
be required. It is not appropriate in the connected pancreatic tail.
case of pseudoaneurysm. (d) If pseudoaneurysm is suspected, emboli-
(b) Percutaneous (via interventional radiol- zation is recommended prior to
ogy) approach is done with CT-guided drainage.
placement of drains. It requires multiple E. Follow-up imaging in patients with expectant
procedures and may cause a cutaneous management every 3 months until resolution.
fistula. Sooner imaging based on symptoms and
(c) Surgical creation of a cystgastrostomy or interventions.
cystenterostomy: left-sided pancreatec-
In a patient with acute or chronic pancreatitis or pancreatic

A trauma, assess for symptoms of walled-off collections, including
fever/infection, nausea/vomiting/inability to tolerate PO,
jaundice, worsening abdominal pain, GI bleeding, ascites, or
symptoms of pleural effusion
Obtain CT or MRI: walled-off collections at least 4 weeks after

onset of pancreatitis
B Pseudocysts: round or oval, thin walls and liquid contents only
WOPN: encapsulated collection with liquid and solid
debris/necrosis, may be multiloculated
Yes
Is diagnosis Obtain EUS- or CT-guided fluid
C in analysis
question? Expect low CEA, very high amylase,
Gram stain and cx may be helpful
No
No Expectant management;
Is patient
symptomatic?
consider CT in 3 months E
and follow until resolution
Yes
Endoscopic treatment: EUS-guided cystgastrostomy, cystduodenostomy; necrosectomy;

D nasocystic drain, ERP with PD stent
Percutaneous: CT-guided drain placement; gradual upsizing to allow for debris removal
Surgical: cystgastrostomy, cystenterosotomy, pancreatic resection
If pseudoaneurysm, then IR for treatment first
Algorithm 97.1
97 Walled-Off Pancreatic Fluid Collections 393
References 2. Baron TH, Harewood GC, Morgan DE, Yates

MR. Outcome differences after endoscopic drainage
of pancreatic necrosis, acute pancreatic pseudocysts,
1. Banks PA, Bollen TL, Dervenis C, et al. Classification
and chronic pancreatic pseudocysts. Gastrointest
of acute pancreatitis – 2012: revision of the Atlanta
Endosc. 2002;56:7.
classification and definitions by international consen-
sus. Gut. 2013;62:102.
Periampullary Carcinoma
98
Heidi N. Overton and Matthew J. Weiss
Algorithmic Approach Diagnosis and Preoperative

Evaluation
Periampullary adenocarcinoma (PAC) is a clin-
icopathologic entity comprised of four distinct A. Patients most frequently present with obstruc-
subtypes in one high-density anatomic region. tive jaundice possibly accompanied by vague
Formally defined as tumors arising ≤2 cm from abdominal pain, nausea, and weight loss [6].
the ampulla of Vater, PAC can originate from PAC should always be considered when eval-
the pancreatic head, duodenum, distal bile uating a patient with obstructive jaundice,
duct, or ampulla of Vater [1]. Pancreatic ductal and basic workup of laboratory studies
adenocarcinoma (PDAC) is the most common including liver function tests (LFTs), cancer
PAC with a recent longitudinal study demon- antigen 19-9 (CA 19-9), carcinoembryonic
strating its occurrence at 66% compared to antigen (CEA), and cross-sectional imaging
16%, 12%, and 6% for ampullary, biliary, and should be obtained [6–8]. A multidetector
duodenal adenocarcinomas, respectively [2]. spiral computed tomography scan with intra-
Though not a common tumor type, PAC has venous contrast performed in both the arterial
low overall survival rates that differ signifi- and portal venous phase (pancreas protocol
cantly by location of origination [3]. To illus- CT) is ideal to evaluate for a periampullary
trate, one series reported primary site-specific mass [7]. Findings on pancreas protocol CT
5-year PAC survival rates of only 17% for pan- are critically important to determining the
creas, 23% for bile duct, 37% for ampulla, and next steps in the patient’s management.
51% for duodenum [4]. The high and variable B. Recent advances in radiographic technology
mortality of PAC is likely attributable to stage now facilitate assessment of many preopera-
at presentation and inherent biologic differ- tive staging factors necessary to determine
ences in pancreatobiliary versus intestinal his- resectability of a periampullary mass, includ-
tology [1, 5]. ing involvement of mesenteric vessels [9].
H. N. Overton · M. J. Weiss (*)

Department of Surgery, Johns Hopkins Hospital,
Baltimore, MD, USA

396 H. N. Overton and M. J. Weiss
Resection and Clinicopathologic D. The standard procedure for PAC is pancreati-

Staging coduodenectomy (PD) with the goal of com-
plete tumor resection with negative oncologic
As surgical resection remains the best chance of margins [2]. Full descriptions of the opera-
cure for PACs, the time from identification of the tive technique and complications of PD are
mass to operation should be optimized [2]. out of the scope of this chapter.
Proceeding to surgery based on pancreas proto- Clinicopathologic staging is based on the
col CT findings alone is acceptable, but the clini- American Joint Committee on Cancer
cal scenario may require further workup [7]. (AJCC) Cancer Staging Manual for each
location of PC origination [14]. Key factors
C. Biliary drainage procedures are typically rec- are resection margins, nodal involvement,
ommended only in cases of cholangitis, sub- microvascular invasion, and perineural inva-
stantially elevated bilirubin, or prolonged sion [14].
period of elevated bilirubin [10, 11]. E. As chemotherapy and immunotherapy con-
Preoperative tissue diagnosis is not necessary tinue to improve for PAC, further important
if there is a high index of suspicion for cancer features are histomolecular markers such as
on cross-sectional imaging and immediate KRAS and DPAC that are more prevalent in
operative intervention is planned. However, PDAC and offer opportunity for targeted
tissue diagnosis is necessary if neoadjuvant therapy [15]. Multidisciplinary oncologic
therapy is considered, as is frequently the care is essential along the continuum of treat-
case for PDAC [12, 13]. ment [16].
98 Periampullary Carcinoma 397
Patient presents with

A obstructive jaundice
Labs: CBC, CMP, LFTs, Ca 19-9, CEA

Imaging:
o pancreas protocol CT scan
No EUS/ERCP for tissue diagnosis

Periampullary
B mass Refer to medical oncology for possible
resectable? neoadjuvant therapy
Reevaluate for resectability after completion of
neoadjuvant therapy
Yes
Consider preoperative biliary stenting:

Biliary Yes o ERCP with biopsy and stent
C obstruction o PTC/PBD if unable to decompress biliary
present? tree with ERCP
No
Legend:
CBC: complete blood count
CMP: complete metabolic panel
LFTs: liver function tests
CEA: carcinoembryonic antigen
Pancreas protocol CT scan: a multidetector
Neoadjuvant therapy (chemo +/– radiation) should be spiral computed tomography scan with
considered by multidisciplinary oncologic team intravenous contrast performed in both the
D arterial and portal venous phase
Proceed to OR for pancreaticoduodenectomy
EUS: endoscopic ultrasound
o Complete resection with negative margins
ERCP: endoscopic retrograde
Adjuvant therapy dependent on pathologic staging cholangiopancreatography
E PTC: percutaneous transhepatic
cholangiography
PBD: percutaneous biliary drainage
OR: operating room
Algorithm 98.1
398 H. N. Overton and M. J. Weiss
Conclusion rates depending on site and specific histology.

Pancreas protocol CT scan is the most useful
PAC consists of pancreatic, biliary, ampullary, imaging modality, and surgical resection with
and duodenal carcinoma which all present with pancreaticoduodenectomy offers the best chance
similar symptoms but have variable survival for cure.
9. House MG, Yeo CJ, Cameron JL, Campbell KA,

References Schulick RD, Leach SD, et al. Predicting resectabil-
ity of periampullary cancer with three-dimensional
1. Sarmiento JM, Nagorney DM, Sarr MG, Farnell computed tomography. J Gastrointest Surg.
MB. Periampullary cancers: are there differences? 2004;8(3):280–8.
Surg Clin North Am. 2001;81(3):543–55. 10. Scheufele F, Schorn S, Demir IE, Sargut M, Tieftrunk
2. He J, Ahuja N, Makary MA, Cameron JL, Eckhauser E, Calavrezos L, et al. Preoperative biliary stenting
FE, Choti MA, et al. 2564 resected periampullary versus operation first in jaundiced patients due to
adenocarcinomas at a single institution: trends over malignant lesions in the pancreatic head: A meta-
three decades. HPB [Internet]. 2014;16(1):83–90. analysis of current literature. Surg (United States).
Available from: 10.1111/hpb.12078. 2017;161(4):939–50.
3. Siegel R, Miller K, Jemal A. Cancer statistics, 2015. 11. Moole H, Bechtold M, Puli SR. Efficacy of preopera-
CA Cancer J Clin [Internet]. 2015;65(1):29. Available tive biliary drainage in malignant obstructive jaundice:
from: http://onlinelibrary.wiley.com/doi/10.3322/ a meta-analysis and systematic review. World J Surg
caac.21254/pdf. Oncol [Internet]. 2016;14(1):182. Available from:
4. Riall TS, Cameron JL, Lillemoe KD, Winter JM, http://wjso.biomedcentral.com/articles/10.1186/
Campbell KA, Hruban RH, et al. Resected periampul- s12957-016-0933-2.
lary adenocarcinoma: 5-year survivors and their 6- to 12. Hartwig W, Schneider L, Diener MK, Bergmann

10-year follow-up. Surgery. 2006;140(5):764–72. F, Büchler MW, Werner J. Preoperative tissue
5. WestgaardA, Tafjord S, Farstad IN, Cvancarova M, Eide diagnosis for tumours of the pancreas. Br J Surg.
TJ, Mathisen O, et al. Pancreatobiliary versus intesti- 2009;96(1):5–20.
nal histologic type of differentiation is an independent 13. Clarke DL, Clarke BA, Thomson SR, Garden OJ,
prognostic factor in resected periampullary adeno- Lazarus NG. The role of preoperative biopsy in pan-
carcinoma. BMC Cancer [Internet]. 2008;8(1):170. creatic cancer. HPB. 2004;6(3):144–53.
Available from: http://bmccancer.biomedcentral.com/ 14. Egner JR. AJCC cancer staging manual. JAMA.

articles/10.1186/1471-2407-8-170. 2010;304:1726.
6. Godellas CV. In: Saclarides TJ, Millikan KW, 15. Chandrasegaram MD, Chiam SC, Chen JW, Khalid
Godellas CV, editors. Surgical oncology: periam- A, Mittinty ML, Neo EL, et al. Distribution and
pullary malignancies. New York: Springer; 2003. pathological features of pancreatic, ampullary, bili-
p. 282–99. ary and duodenal cancers resected with pancreati-
7. Cooper M, Newman NA, Ibrahim AM, Lam E, Herman coduodenectomy. World J Surg Oncol [Internet].
JM, Singh VK, et al. Unnecessary tests and proce- 2015;13(1):85. Available from: http://www.wjso.
dures in patients presenting with solid tumors of the com/content/13/1/85.
pancreas. J Gastrointest Surg. 2013;17(7):1218–23. 16. Kumar R, Herman JM, Wolfgang CL, Zheng

8. Gloor B, Todd KE, Reber HA. Diagnostic workup of L. Multidisciplinary management of pancreatic can-
patients with suspected pancreatic carcinoma. Cancer. cer. Surg Oncol Clin N Am. 2013;22:21231.
1997;79(9):1780–6.
Management of Intraductal
Papillary Mucinous Neoplasms 99
Jonathan G. Sham and Matthew J. Weiss
Algorithmic Approach should undergo resection without additional

testing given the high rate of malignancy in
A. The diagnosis of intraductal papillary muci- these cysts [2, 4].
nous neoplasm (IPMN) of the pancreas most D. Features on imaging concerning for malig-
often stems from the workup of nonspecific nant transformation include cyst size of
clinical symptoms or as an incidental finding ≥3 cm, presence of an enhancing mural nod-
on medical imaging. The most common clini- ule <5 mm, thickened or enhanced cyst walls,
cal symptoms leading to evaluation for IPMN MPD measuring 5–9 mm, abrupt change in
are epigastric discomfort (70–80%), nausea/ MPD caliber with distal pancreatic atrophy,
vomiting (11–21%), backache (10%), weight lymphadenopathy, elevated serum CA19-9,
loss (20–40%), diabetes, and jaundice [1]. and cyst growth rate of >5 mm/2 years [2,
B. Any cystic structure of the pancreas greater 5–7].
than 5 mm in size should be further character- E. Should any of the aforementioned features be
ized with either computed tomography (CT) present, endoscopic ultrasound (EUS) can be
or magnetic resonance imaging (MRI) with a useful adjunct in delineating cyst anatomy,
magnetic resonance cholangiopancreatogra- invasion, and other malignant characteristics
phy (MRCP) in order to evaluate the cyst for [8]. It is important to note that biochemical
“high-risk stigmata” or “worrisome features” analysis of amylase and carcinoembryonic
for malignancy [2]. In general, symptomatic antigen (CEA) can differentiate between
cysts have a higher overall risk of invasive mucinous and non-mucinous, but not benign
carcinoma and high-grade dysplasia than do from malignant cysts [9]. A cyst fluid CEA of
asymptomatic cysts [3]. ≥192–200 ng/ml has been shown to be ~80%
C. “High-risk stigmata” of malignancy include accurate in diagnosing a cyst as mucinous [9,
obstructive jaundice in patients with a cystic 10]. Cytologic evaluation of cyst fluid can be
lesion in the pancreatic head, enhancing useful for confirming the diagnosis of malig-
mural nodules of greater than 5 mm, or a nancy; however, sensitivity is limited [11],
main pancreatic duct (MPD) size of greater and its use should be circumscribed to high-
than 10 mm. If surgically fit, these patients volume centers with expertise in this
technique.
J. G. Sham · M. J. Weiss (*) F. If no concerning features are present or EUS
Department of Surgery, Johns Hopkins Hospital, evaluation of the cyst is reassuring, surveil-
Baltimore, MD, USA lance of IPMN is recommended as long as the

400 J. G. Sham and M. J. Weiss
patient remains fit for surgery. Several sur- patients who will require long-term surveil-
veillance schemes have been suggested, with lance, as the risk of malignant progression
the most recent Fukuoka consensus guide- does not decrease over time [2]. When evalu-
lines summarized at the terminus of the ating a patient’s appropriateness for surgical
attached algorithm. MRI and EUS are recom- resection, any family history of pancreas can-
mended for use when frequent surveillance is cer (PDAC) should be thoroughly explored.
required, given the carcinogenic effects of The risk of developing PDAC in the setting of
ionizing radiation associated with IPMN rises dramatically—2.3-, 6.4-, and
CT. Particularly with larger cysts, resection 32-fold—with one, two, and three affected
should be strongly considered in younger first-degree relatives, respectively [12, 13].
Clinical symptoms: Epigastric/back pain, nausea, weight loss, diabetes, jaundice,

A or Incidental imaging finding of pancreatic cyst
B Pancreas protocol CT or MRI/MRCP
C Any “high-risk stigmata” present?

Enhancing mural nodule ≥5 mm, main pancreatic duct ≥10 mm, obstructive jaundice
No
D Yes Any “worrisome features” present?

Cyst ≥3cm, enhancing mural nodules <5 mm, thickened/enhancing cyst walls, abrupt caliber change of pancreatic
duct, main duct size 5–9mm, lymphadenopathy, elevated serum CA19-9, cyst growth rate ≥5mm/2 yrs, pancreatitis
Yes
Perform EUS: Are any of these present?

Consider surgical Yes Definite mural nodules ≥5 mm, main duct features suspicious E No
resection for involvement, cytology suspicious or positive for malignancy
No
F
What is the size of the largest cyst?

<2 cm: CT/MRI every 6 months x 1 year, yearly thereafter
2–3 cm: EUS in 3–6 months, consider q6 month CT/MRI for 2 years and then yearly thereafter if no change, consider surgery in young pts with need
for prolonged surveillance
Algorithm 99.1
pancreatic cysts: clinicopathologic characteristics and

References comparison with symptomatic patients. Arch Surg.
2003;138(4):427–3. discussion 33–4.
1. Machado NO, Al Qadhi H, Al Wahibi K. Intraductal 4. Kim TH, Song TJ, Hwang JH, Yoo KS, Lee WJ, Lee
papillary mucinous neoplasm of pancreas. N Am J KH, et al. Predictors of malignancy in pure branch
Med Sci. 2015;7(5):160–75. duct type intraductal papillary mucinous neoplasm
2. Tanaka M, Fernández-Del Castillo C, Kamisawa T, of the pancreas: a nationwide multicenter study.
Jang JY, Levy P, Ohtsuka T, et al. Revisions of inter- Pancreatology. 2015;15(4):405–10.
national consensus Fukuoka guidelines for the man- 5. Bassi C, Crippa S, Salvia R. Intraductal papillary
agement of IPMN of the pancreas. Pancreatology. mucinous neoplasms (IPMNs): is it time to (some-
2017;17(5):738–53. times) spare the knife? Gut. 2008;57(3):287–9.
3. Fernández-del Castillo C, Targarona J, Thayer SP, 6. Kwong WT, Lawson RD, Hunt G, Fehmi SM,
Rattner DW, Brugge WR, Warshaw AL. Incidental Proudfoot JA, Xu R, et al. Rapid growth rates of
99 Management of Intraductal Papillary Mucinous Neoplasms 401
s uspected pancreatic cyst branch duct intraductal pap- 10. Cizginer S, Turner BG, Turner B, Bilge AR, Karaca
illary mucinous neoplasms predict malignancy. Dig C, Pitman MB, et al. Cyst fluid carcinoembryonic
Dis Sci. 2015;60(9):2800–6. antigen is an accurate diagnostic marker of pancreatic
7. Brounts LR, Lehmann RK, Causey MW, Sebesta mucinous cysts. Pancreas. 2011;40(7):1024–8.
JA, Brown TA. Natural course and outcome of cystic 11. Pitman MB, Deshpande V. Endoscopic ultrasound-
lesions in the pancreas. Am J Surg. 2009;197(5):619– guided fine needle aspiration cytology of the pan-
22. discussion 22–3. creas: a morphological and multimodal approach
8. Ohno E, Hirooka Y, Itoh A, Ishigami M, Katano to the diagnosis of solid and cystic mass lesions.
Y, Ohmiya N, et al. Intraductal papillary muci- Cytopathology. 2007;18(6):331–47.
nous neoplasms of the pancreas: differentiation 12. Klein AP, Brune KA, Petersen GM, Goggins M,

of malignant and benign tumors by endoscopic Tersmette AC, Offerhaus GJ, et al. Prospective risk
ultrasound findings of mural nodules. Ann Surg. of pancreatic cancer in familial pancreatic cancer kin-
2009;249(4):628–34. dreds. Cancer Res. 2004;64(7):2634–8.
9. Park WG, Mascarenhas R, Palaez-Luna M, Smyrk 13. He J, Cameron JL, Ahuja N, Makary MA, Hirose
TC, O'Kane D, Clain JE, et al. Diagnostic perfor- K, Choti MA, et al. Is it necessary to follow patients
mance of cyst fluid carcinoembryonic antigen and after resection of a benign pancreatic intraductal
amylase in histologically confirmed pancreatic cysts. papillary mucinous neoplasm? J Am Coll Surg.
Pancreas. 2011;40(1):42–5. 2013;216(4):657–65. discussion 65–7.
Pancreatic Necrosis
100
Ammar Asrar Javed and Matthew J. Weiss
Algorithmic Approach mortality [5]. In recent times, a step-up approach

has become more common [2, 5]. This entails ini-
Acute pancreatitis is one of the most common tial stabilization of the patient and management
gastrointestinal disorders requiring hospitaliza- using less invasive approaches, such as percuta-
tion with an incidence of 4.5 to 35 per 100,000 neous or endoscopic drainage followed by video-
people [1, 2]. It is often a sequela of gallstones, assisted retroperitoneal debridement (VARD) or
alcoholism, or endoscopic retrograde cholangio- laparoscopic drainage. In the event of clinical
pancreatography (ECRP). Approximately 20% of deterioration, surgical interventions such as mini-
pancreatitis patients will develop pancreatic mally invasive retroperitoneal necrosectomy or
necrosis, which can lead to a systemic inflamma- open necrosectomy are recommended. Using this
tory response, multi-organ system failure, and approach has been shown to improve patient out-
death [1]. Early intervention is essential to avoid comes [2]. This chapter discusses the step-up
poor outcomes. Pancreatic necrosis can be either approach, which is depicted in the subsequent
sterile or infected, and the outcomes between the algorithm.
two are significantly different. The mortality
associated with sterile necrosis is 10%, while that
of infected necrosis is 30%, which increases to Management of Pancreatic Necrosis
over 40% when presenting with multi-organ sys-
tem failure [3, 4]. In the absence of clinical sep- A. Evaluation should begin with a detailed his-
sis, the distinction between sterile and infected tory and physical examination. A history
necrosis can be made based on computed tomog- should include details regarding symptoms
raphy (CT) findings and/or ultrasound-guided including abdominal pain (onset, duration,
biopsy with gram stain and culture. Distinguishing intensity, and radiation), persistent fever, nau-
between the two is perhaps the most vital step in sea, and vomiting. Past history of chronic
the management of pancreatic necrosis. pancreatitis, gallstones, and alcohol use
Open necrosectomy has historically been the should be elucidated [1]. On physical exami-
treatment of choice for pancreatic necrosis; how- nation, these patients might have abdominal
ever, it is associated with high morbidity and guarding and rebound tenderness. Positive
Grey-Turner or Cullen signs are suggestive of
A. A. Javed · M. J. Weiss (*) pancreatitis, but these are infrequently posi-
Department of Surgery, Johns Hopkins Hospital, tive in clinical practice.
Baltimore, MD, USA

404 A. A. Javed and M. J. Weiss
B. Vital signs, blood work, and imaging can pro- Therefore, appropriate initial management
vide important information to determine the can help delay the need for surgical interven-
type of necrosis. Elevated serum amylase and tion and help stabilize patients to avoid high-
lipase are suggestive of pancreatitis, the latter mortality procedures. However, in patients
being more sensitive and specific. Currently, with worsening clinical condition and multi-
abdominal CT is the standard imaging modal- organ system failure, early surgical interven-
ity used to identify and assess pancreatic tion may be necessary [6].
necrosis. Lack of contrast uptake is represen- D. If the patient has sterile necrosis, then medi-
tative of tissue necrosis, and gas in the pan- cal management via administration of IV flu-
creas is pathognomonic for infected ids and nutritional support should be
pancreatic necrosis. Magnetic resonance continued.
imaging (MRI) and magnetic resonance chol- E. Patients demonstrating evidence of infected
angiopancreatography (MRCP) can also pancreatic necrosis, i.e., persistent fever, wors-
assess the extent of necrosis and identifica- ening condition, or multi-organ system failure,
tion of gallstones. Elevated white cell count, will require intervention. Interventions are per-
persistent fever, gas in the pancreas on imag- formed using a step-up approach [2]. The ini-
ing, adynamic ileus, and progressive multi- tial intervention should include evacuation of
organ system failure are suggestive of infected necrotic tissue and infected fluid collection by
pancreatic necrosis. It is important to distin- percutaneous or endoscopic (transgastric)
guish between sterile and infected necrosis drain placement. This can result in a resolution
because the management differs significantly in approximately 35% of the patients [2]. If the
and placement of drain in a sterile necrosis patient does not show improvement within
can induce infection. 72 h of the procedure, they should be reevalu-
C. The aim of initial management is to stabilize ated using imaging, and a second drainage pro-
the patient, which consists of aggressive cedure should be performed. When there is an
intravenous (IV) fluid replacement and nutri- increase in the extent of necrosis based on
tional support in the setting of an intensive imaging or if the patient continues to deterio-
care unit. The prophylactic use of antibiotics rate, surgical resection/debridement should be
is not recommended; however, if there are adopted [6]. This can be performed via VARD,
signs suggestive of infected necrosis, then minimally invasive retroperitoneal necrosec-
antibiotics should be administered [1]. tomy, or an open necrosectomy based on the
Surgical intervention within 48–72 h of pre- experience of the surgeon [7]. The aim of sur-
sentation has been associated with a high gical resection should be the removal of all
mortality [2]. This risk decreases significantly necrotic tissue, unroofing of all cavities in the
if the surgical intervention can be pushed retroperitoneal spaces, and placement of a
beyond 28–30 days from initial presentation. large-bore catheter for drainage and irrigation.
100 Pancreatic Necrosis 405
Detailed history and physical exam

Sharp abdominal pain, persistent fever, nausea, and vomiting
A History of chronic pancreatitis, gallstones, alcoholism, or ERCP

Abdominal guarding and rebound tenderness on physical examination
Vital signs, blood work, and imaging

Tachycardia and persistent pyrexia
B Elevated serum amylase, lipase, and WBC on labs
CT scan showing pancreatic necrosis. Lack of contrast uptake is representative of
tissue necrosis, and gas in the pancreas is pathognomonic for infected pancreatic necrosis
Initial management and stabilization

Aggressive intravenous fluid replacement and nutritional support
Antibiotics to be used only in case of infected pancreatic necrosis
C
Aimed at pushing surgical intervention beyond 30 days from initial presentation. Surgical
intervention only recommended in patients with worsening clinical conditions or multi-organ
system failure
Development of E
Sterile necrosis
infection
Continue medical management via
D intravenous fluids and nutritional
support Infected necrosis
Intervention using a step-up approach
Surgical resection
Disease escalation
Minimally invasive retroperitoneal
necrosectomy or open necrosectomy
Drainage of necrosis
No improvement
Evacuation of necrotic tissue and

infected fluid collection by
in 72 h
percutaneous or endoscopic
(transgastric) drainage
Improvement
Re-evaluation of disease
No improvement in
Re-evaluation of disease using imaging
72 h
If a fluid collection is identified, a
second drainage procedure should be
performed
Medical management
Improvement Continue medical management via
intravenous fluids and nutritional
support
Algorithm 100.1
406 A. A. Javed and M. J. Weiss
References pancreatic necrosis as determinants of mortality in

patients with acute pancreatitis. Gastroenterology.
2010;139(3):813–20.
1. Donald G, Donahue T, Reber HA, Hines OJ. The
5. Working Group IAPAPAAPG. IAP/APA evidence-
evolving management of infected pancreatic necro-
based guidelines for the management of acute pancre-
sis. Am Surg. 2012;78(10):1151–5. Pubmed Central
atitis. Pancreatology. 2013;13(4 Suppl 2):e1–15.
PMCID: 3678520.
6. Uhl W, Warshaw A, Imrie C, Bassi C, McKay CJ,
2. van Santvoort HC, Besselink MG, Bakker OJ, Hofker
Lankisch PG, et al. IAP guidelines for the surgical
HS, Boermeester MA, Dejong CH, et al. A step-up
management of acute pancreatitis. Pancreatology.
approach or open necrosectomy for necrotizing pan-
2002;2(6):565–73.
creatitis. N Engl J Med. 2010;362(16):1491–502.
7. Alverdy J, Vargish T, Desai T, Frawley B, Rosen
3. Dugernier T, Dewaele J, Laterre PF. Current surgical
B. Laparoscopic intracavitary debridement of peri-
management of acute pancreatitis. Acta Chir Belg.
pancreatic necrosis: preliminary report and descrip-
2006;106(2):165–71.
tion of the technique. Surgery. 2000;127(1):112–4.
4. Petrov MS, Shanbhag S, Chakraborty M, Phillips
AR, Windsor JA. Organ failure and infection of
Part XIII
Spleen
Management of Splenic Abscess
101
Andrew T. Bates and Michael G. Svestka
Algorithmic Approach neous drainage, or deep or hilar abscess or

when more than two abscesses are present.
Splenic abscess is a relatively rare entity with Alternatively, percutaneous drainage is pre-
autopsy reports ranging in incidence from ferred in the pediatric population, critically ill
0.145% to 0.7% [1]. Sources of infection are typ- patients who are unable to tolerate surgery,
ically either hematogenous spread from endocar- and unilocular or bilocular liquid collections
ditis, superinfection of splenic infarction, tumors without phlegmon that are anatomically ame-
or cysts, or trauma, especially in the setting of nable to percutaneous access, such as those in
immunocompromised states [2, 3]. Though rare, the middle and lower poles [3, 6, 7].
splenic abscess carries a mortality rate of 15–20% C. Once percutaneous access is obtained, abscesses
in healthy patients and 70–80% in immunocom- less than 3 cm may be successfully aspirated;
promised patients [4]. however, a drain should be left in place other-
wise. An 8 French catheter is recommended;
A. Ultrasound imaging is a reasonable first-line however, if drainage if unsuccessful, Seldinger
diagnostic tool given its speed and cost- replacement with a 10 French catheter is rea-
effectiveness; however, computed tomogra- sonable. Historically, splenectomy has been the
phy (CT) scan remains the diagnostic method treatment of choice for abscesses; however,
of choice as it can better delineate abscess with the advent of image-guided access, percu-
position, attenuation, and loculation [1, 5]. taneous aspiration and catheter drainage have
B. After diagnostic imaging, it is reasonable to grown increasingly popular with effective
begin broad-spectrum, empiric antibiotics. The results in selected populations [3, 8, 9].
most common causative organisms include D. A post-procedure ultrasound should be per-
Streptococcus, Klebsiella, Staphylococcus, and formed at 24 h post-drainage to rule out
E. coli [1, 5–7]. Given the risk of post-splenec- hematoma and verify successful collection
tomy sepsis, the decision to perform splenec- drainage. Hemorrhage following drainage is
tomy should be reserved for patients over the rare and is reported to be between 0% and 2%
age of 18, hemodynamically stable patients, [10–12]. The drain may be left in position
abscesses where phlegmon prohibits percuta- until output decreases to 3 ml daily.
E. A total antibiotic course of 8 weeks is suffi-
A. T. Bates · M. G. Svestka (*) cient to clear most infections. If percutaneous
Department of Surgery, Stony Brook University drainage fails, salvage splenectomy is accept-
Hospital, Stony Brook, NY, USA able and does not increase mortality [2].

410 A. T. Bates and M. G. Svestka
A CT-diagnosed splenic abscess
Begin empiric antibiotics
>18 years old <18 years old

No contraindications to surgery Critically ill/unable to tolerate
surgery
Hemodynamically stable
B Unilocular/bilocular liquid
Phlegmonous changes collection
>2 loculations or septations
Anatomically amenable to
Deep or hilar abscess percutaneous access
Percutaneous drainage
C Splenectomy
>3cm <3cm
Leave drain Aspirate

D Post-splenectomy vaccines
Post-procedure ultrasound
Remove drain once <3 ml daily
E If recurrent, salvage splenectomy 8 weeks of targeted antibiotics
Algorithm 101.1
5. Lee W, Choi ST, Kim KK. Splenic abscess: a single

References institution study and review of the literature. Yonsei
Med J. 2011;52(2):288–92.
1. Chun CH, Raff MJ, Contreras L. Splenic abscess. 6. Smyrniotis V, Kehagias D, Voros D, et al. Splenic
Medicine. 1980;59:50–65. abscess. Dig Surg. 2000;17:354–7.
2. Ooi L, Leong S. Splenic abscesses from 1987 to 1995. 7. Llenas-Garcia J, Fernandes-Ruiz M, Caurcel
Am J Surg. 1997;174:87–93. L. Splenic abscess: a review of 22 cases in a single
3. Kang M, Kalra N, Gulati M, et al. Image guided institution. Euro J Intern Med. 2009;20:537–9.
percutaneous splenic interventions. Euro J Radiol. 8. Chou Y, Tiu C, Chiou H, et al. Ultrasound-guided
2007;64:140–6. interventional procedures in splenic abscesses. Euro
4. Wiesel O, Fisichella PM. The management of cysts, J Radiol. 1998;28:167–70.
tumors and abscesses of the spleen. In: Cameron JL, 9. Thanos L, dailiana T, Papaioannou G, et al.
Cameron AM, editors. Current surgical therapy. 12th Percutaneous CT-guided drainage of splenic abscess.
ed. Philadelphia, PA: Elsevier, Inc; 2016. Am J Radiol. 2002;179:629–32.
101 Management of Splenic Abscess 411
10. Quinn SF, vanSonnenberg E, Cassola G, et al.

12.
Lucey BC, Boland GW, Maher MM, et al.
Interventional radiology in the spleen. Radiology. Percutaneous nonvascular splenic interven-
1986;161:289–91. tion: a 10-year review. AJR Am J Roentgenol.
11. Caraway NP, Fanning CV. Use of fine needle aspira- 2002;179:1591–6.
tion biopsy in the evaluation of splenic lesions in a
cancer center. Diagn Cytopathol. 1997;16:312–6.
Atraumatic Indications
for Splenectomy 102
Maria S. Altieri and Andrew T. Bates
Algorithmic Approach glucose-6-phosphate dehydrogenase

deficiencies.
Indications for splenectomy for atraumatic rea-
sons can be divided into several categories: Algorithm 102.2. Hemoglobinopathies include
benign, malignant, and others. sickle cell anemia (SCA) and thalassemia, and
Algorithm 102.1. Benign conditions include indications for splenectomy are shown in
red blood cell (RBC) disorders (A), hemoglobin- Algorithm 102.2.
opathies (B), and platelet disorders (C). Malignant
conditions include white blood cell (WBC) disor- A. In case of SCA, restoration of RBC volume is
ders and bone marrow disorders, while other con- important. If patient is refractory, then sple-
ditions include infectious and other lesions. nectomy is needed.
B. Transfusions and iron chelation are the treat-
A. RBC disorders can be divided into congenital ment therapy for thalassemia. Splenectomy is
(hereditary spherocytosis [HS], enzyme defi- indicated in case of refractory symptoms.
ciencies, such as pyruvate kinase and glucose-
6-phosphate dehydrogenase deficiencies) and Algorithm 102.3. Platelet disorders include
acquired such as warm-antibody autoimmune idiopathic thrombocytopenic purpura (ITP),
hemolytic anemia. Among these, indications which is the most common indication for elective
for splenectomy include recurrent transfu- splenectomy, and thrombotic thrombocytopenic
sions and intractable leg ulcers in HS and purpura (TTP).
recurrent transfusions in pyruvate kinase defi-
ciencies. Failure of medical therapy is an A. When platelets are <30,000/mm3, medical

indication for splenectomy in condition such therapy is the first step.
as warm-antibody autoimmune hemolytic B. Indications for splenectomy in case of ITP
anemia. Splenectomy is not indicated in include failure of medical therapy and recur-
rent disease.
M. S. Altieri (*) Algorithm 102.4.


A .
First-line treatment for TTP is
A. T. Bates plasmapheresis.

414 M. S. Altieri and A. T. Bates
B. Excessive plasma exchange requirements can chronic myeloid leukemia, chronic myelomono-
be an indication for splenectomy. cytic leukemia, polycythemia vera, myelofibro-
sis, and essential thrombocythemia. Indications
Malignant conditions include white blood cell for splenectomy in these conditions are usually
(WBC) disorders and bone marrow disorders. due to symptomatic splenomegaly.
WBC disorders include non-Hodgkin’s lym- Other conditions include splenic abscesses,
phoma (NHL), Hodgkin’s disease, hairy cell leu- cysts, and metastasis. Splenectomy is the therapy
kemia, and chronic lymphocytic leukemia. of choice for abscesses and symptomatic para-
Indications for surgery for NHL include symp- sitic cysts, while symptomatic nonparasitic cysts
tomatic splenomegaly and cytopenia. Bone mar- can be treated with partial splenectomy or
row disorders include acute myeloid leukemia, unroofing.
Benign
A B C
Red blood
Platelet
cell
disorders
disorders
Hemoglobino-
pathies
PK ITP
HS TTP AIHA
deficiency
Sickle cell
anemia Thalassemia
Algorithm 102.1
102 Atraumatic Indications for Splenectomy 415
Sickle cell anemia Thalassemia
B
A
Autoinfarction
splenic abscess
splenic sequestration
Restoration of RBC Transfusions

volume iron chelation therapy
No Observation
Refractory?
Yes
Splenectomy
Algorithm 102.2
416 M. S. Altieri and A. T. Bates
ITP
A Platelets <30,000/mm3
Prednisone for 2– 4
weeks, followed by
a taper
B No
Refractory? Observation
Yes
Splenectomy
Algorithm 102.3
TTP
Suggested Reading
ed. St. Louis: B.C. Decker; 2017.
A Plasmapharesis
Refractory? Observation
Splenectomy
Algorithm 102.4
Part XIV
Thyroid/Parathyroid
Hypothyroidism
103
Lukasz Czerwonka
Algorithmic Approach excess (Wolff–Chaikoff effect) [1]. Regardless

of cause, all patients with overt primary hypo-
A. Primary hypothyroidism is the most common thyroidism require treatment with thyroid hor-
form of hypothyroidism and is characterized mone, and synthetic thyroxine (T4,
by a combination of low thyroid-stimulating levothyroxine) is the treatment of choice [2].
hormone (TSH) and low T4. The most com- B. Subclinical hypothyroidism is defined by ele-
mon cause is chronic autoimmune vated TSH and normal free T4. Most patients
(Hashimoto’s) thyroiditis, which is mediated are asymptomatic. The prevalence in the US
by both cellular (cytotoxic T-cells) and population is 4% when primary hypothyroid-
humoral (thyroglobulin and thyroid peroxi- ism is excluded [3]. It is more prevalent
dase antibodies) mechanisms. The gland can worldwide in areas of iodine deficiency. The
be enlarged or atrophic. Antibody testing to etiologies are the same as overt primary hypo-
confirm Hashimoto’s thyroiditis is not rou- thyroidism. Annual progression to overt pri-
tinely required, but measuring thyroperoxi- mary hypothyroidism is 2–4% per year [4].
dase (TPO) antibodies may be useful in C. Subclinical hypothyroidism can increase risk
postpartum/painless thyroiditis or subclinical of cardiovascular disease. A meta-analysis
hypothyroidism to predict likelihood of pro- from 7 prospective studies including >25,000
gression to permanent hypothyroidism. Other participants showed an increased risk of coro-
etiologies of hypothyroidism to consider nary heart disease events from 20.3 to 38.4
include iatrogenic (surgery, radio active events/100 person-years in patients with TSH
iodoine (RAI), external beam radiation ≥10 [5]. Therefore, it is recommended to start
(XRT)), medication-induced (methimazole, levothyroxine if TSH ≥10. Treatment of
propylthiouracil (PTU), lithium, amiodarone, patients aged <65–70 with lower TSH levels
interferon alfa, immune checkpoint inhibitors, is controversial and not recommended in
tyrosine kinase inhibitors, cholestyramine, older individuals [2].
phenytoin, carbamazepine), and iodine defi- D. Several viral illnesses can cause transient

ciency (most common cause worldwide) or subacute thyroiditis, and these patients should
be treated symptomatically and monitored for
resolution [1].
L. Czerwonka (*) E. Less than 1% of patients with hypothyroidism
Department of Surgery, Division of have central hypothyroidism from secondary
Otolaryngology – Head and Neck Surgery, Stony
Brook University Hospital, Stony Brook, NY, USA (TSH deficiency) or tertiary (thyrotropin-
e-mail: [email protected] releasing hormone [TRH] d eficiency) causes.
420 L. Czerwonka
Secondary hypothyroidism is most commonly imaging (MRI) to rule out a sellar mass fol-
the result of a pituitary micro- or macroade- lowed by biochemical testing if a mass is pres-
noma. Other causes of secondary and tertiary ent. All patients should undergo testing for
hypothyroidism include pituitary necrosis secondary adrenal insufficiency prior to initiat-
(Sheehan syndrome), trauma, radiation, ing levothyroxine, as failure to administer glu-
hypophysitis, other cranial tumors or infiltra- cocorticoids along with levothyroxine in
tive diseases, and TSH or TRH gene mutations. patients with adrenal insufficiency can precipi-
TSH may be low, normal, or slightly elevated. tate acute adrenal crisis. Patients with sellar
Evaluation begins with magnetic resonance masses require neurosurgical evaluation [6].
Presentation: fatigue, weight gain, slow movement and speech, constipation, cold
intolerance, bradycardia, HTN, coarse hair, nonpitting edema, periorbital edema
and/or
abnormal thyroid function tests
B
No High Yes No
Low fT4 Low fT4 Subclinical hypothyroidism
TSH
Yes Yes
E Central hypothyroidism A Primary hypothyroidism
MRI brain ACTH stimulation test Identify cause:

· Thyroidectomy
· RAI/XRT
· Drugs that affect thyroid
hormone synthesis or
No clearance
Adrenal
· History of iodine
deficiency or excess
Yes
Levothyroxine Measure TPO

+ antibodies if goiter,
glucocorticoid painless thyroiditis or
subclinical thyroiditis
C
Sellar mass Measure: Yes

gonadotropins, Levothyroxine TSH 10
testosterone, estradiol,
prolactin, IGF1
and
consult neurosurgery D No
Acute nonthyroidal illness
Repeat TSH and fT4 in 4–6 weeks
Follow-up: TSH q6–12 months
Algorithm 103.1
103 Hypothyroidism 421
References Nutrition Examination Survey (NHANES III). J Clin

Endocrinol Metabol. 2002;87(2):489–99.
4. Vanderpump MP, Tunbridge WM, French JM,
1. Sweeney LB, Stewart C, Gaitonde DY. Thyroiditis:
Appleton D, Bates D, Clark F, Grimley Evans J, Hasan
an integrated approach. Am Fam Physician. 2014;
DM, Rodgers H, Tunbridge F, et al. The incidence of
90(6):389–96.
thyroid disorders in the community: a twenty-year
2. Jonklaas J, Bianco AC, Bauer AJ, Burman KD,
follow-up of the Whickham Survey. Clin Endocrinol.
Cappola AR, Celi FS, et al. Guidelines for the treat-
1995;43(1):55–68.
ment of hypothyroidism: prepared by the American
5. Rodondi N, Den Elzen WP, Bauer DC, Cappola AR,
Thyroid Association task force on thyroid hormone
Razvi S, Walsh JP, et al. Subclinical hypothyroidism
replacement. Thyroid. 2014;24(12):1670–751.
and the risk of coronary heart disease and mortality.
3. Hollowell JG, Staehling NW, Flanders WD, Hannon
JAMA. 2010;304(12):1365–74.
WH, Gunter EW, Spencer CA, et al. Serum TSH,
6. Persani L. Central hypothyroidism: pathogenic, diag-
T4, and thyroid antibodies in the United States
nostic, and therapeutic challenges. J Clin Endocrinol
population (1988 to 1994): National Health and
Metabol. 2012 Sep;97(9):3068–78.
Hyperthyroidism
104
Ewen Chao and Lukasz Czerwonka
Algorithmic Approach fibrillation, and a decrease in bone mineral

density. The American Thyroid Association
A. On physical exam, there may be findings recommends repeating TSH and free T4 lev-
associated with Graves’ disease, such as els if subclinical hyperthyroidism is persis-
exophthalmos or pretibial myxedema. tent (TSH <0.1 mU/L). It should be treated in
Children and the elderly may present with patients ≥65 years old, particularly those
alternate symptoms, such as apathetic hyper- with cardiac risk factors, heart disease, or
thyroidism in the elderly, which primarily osteoporosis, in postmenopausal women not
consists of apathy and depression, along on estrogens or bisphosphonates, and in
with weight loss, congestive heart failure, patients who have symptoms of hyperthy-
tachycardia, muscle weakness, dry skin, or roidism [2].
ptosis [1]. D. Thyroid storm is a clinical diagnosis and may
B. If the thyroid-stimulating hormone (TSH) be due to infection, intravenous (IV) contrast
level is normal or elevated, then one should exposure, diabetic ketoacidosis (DKA), or
consider non-thyroidal causes. Elevated T4 surgery. It may be scored with the Burch-
levels in the setting of normal TSH may be Wartofsky scale [3].
due to abnormally elevated levels of thyroid E. Both radioactive iodine uptake and thyroid
hormone-binding proteins, drugs that inhibit hormone receptor antibody (TRAb) are
T4 to T3 conversion such as amiodarone or acceptable tests to differentiate causes of
high-dose propranolol, acute psychosis, high hyperthyroidism. One model showed a 46%
altitude, or amphetamine abuse [2]. faster time to diagnosis and 47% cost savings
C. The incidence of subclinical hyperthyroidism when using TRAb testing [4].
(TSH <0.1 mU/L) is 0.7% in the United F. Radioactive iodine (RAI) ablation, antithy-
States and leads to an increased risk of overall roidal medications, and surgery are all
mortality, cardiovascular mortality, atrial acceptable treatments for Graves’ disease,
but surgery is the most effective [5]. RAI
and surgery are most effective for toxic mul-
E. Chao · L. Czerwonka (*) tinodular goiter or toxic solitary adenoma,
Department of Surgery, Division of Otolaryngology – but antithyroidals may be used to achieve
Head and Neck Surgery, Stony Brook University
Hospital, Stony Brook, NY, USA euthyroidism prior to surgery. If surgery is
e-mail: [email protected] chosen as treatment for Graves’ disease,

424 E. Chao and L. Czerwonka
total thyroidectomy is the most effective of permanent hypocalcemia/hypoparathy-

procedure for reducing risk of recurrent roidism with total vs subtotal thyroidectomy,
hyperthyroidism. In a recent Cochrane but no difference in permanent recurrent
review, some studies showed increased risk laryngeal nerve palsy [6].
Presentation:
A Heat intolerance, increased sweating and thirst,
weight loss, diarrhea, palpitations, tremors, fatigue
Obtain thyroid function tests: TSH, free T4, total T3
C
Yes High Low/Normal
Subclinical Normal Normal Yes
TSH Euthyroidism
hyperthyroidism T4/T3 T4/T3
No
No B
D Fever, agitation, nausea/vomiting, • TSH-secreting pituitary
diarrhea, tachycardia, signs of CHF: adenoma
“Thyroid storm”→beta blockers, • Pituitary resistance to
Lugol’s iodine, PTU, corticosteroids thyroid hormone
• Euthyroid
hyperthyroxinemia
E
Diffuse high uptake Positive

RAIU Graves’ disease TRAb
Negative
• Radioactive iodine
F • Antithyroidals
• Surgery
Focal high uptake • Toxic multinodular goiter Nodules

US
• Toxic solitary adenoma
Low uptake No nodules

Thyroiditis
Follow-up TSH q4–12 months or change in symptoms
Algorithm 104.1
104 Hyperthyroidism 425
References 4. McKee A, Peyerl F. TSI assay utilization: impact on

costs of Graves’ hyperthyroidism diagnosis. Am J
Manag Care. 2012;18(1):e1–14.
1. Arnold BM, Casal G, Higgins HP. Apathetic thyro-
5. Genovese BM, Noureldine SI, Gleeson EM, Tufano
toxicosis. Can Med Assoc J. 1974;111(9):957–8.
RP, Kandil E. What is the best definitive treatment for
2. Ross DS, Burch HB, Cooper DS, Greenlee MC,
Graves’ disease? A systematic review of the existing
Laurberg P, Maia AL, et al. 2016 American Thyroid
literature. Ann Surg Oncol. 2013;20(2):660–7.
Association Guidelines for diagnosis and manage-
6. Liu ZW, Masterson L, Fish B, Jani P, Chatterjee
ment of hyperthyroidism and other causes of thyro-
K. Thyroid surgery for Graves’ disease and Graves’
toxicosis. Thyroid. 2016;26(10):1343–421.
ophthalmopathy. Cochrane Database Syst Rev.
3. Burch HB, Wartofsky L. Life-threatening thyrotoxi-
2015;11:CD010576.
cosis. Thyroid storm. Endocrinol Metab Clin N Am.
1993;22(2):263–77.
Thyroiditis
105
Lukasz Czerwonka
Algorithmic Approach through four stages: first, a hyperthyroid

phase which may require β-blockers if symp-
A. Infections of the thyroid are exceedingly rare tomatic; second, a euthyroid phase; third, a
due to its iodine content, encapsulation, lym- hypothyroid phase in 20–30% of patients
phatic drainage, and blood supply. They which may require thyroid hormone replace-
occur via distant seeding, direct spread from ment; and fourth, a resolution phase to a
persistent pyriform sinus fistulas or thyro- euthyroid state in over 90% of patients.
glossal duct cysts, or penetrating trauma and Treatment is symptomatic with high-dose
are more common in children, patients with aspirin for pain relief and steroids in severe
preexisting thyroid disease, and those who cases. Thyroidectomy is reserved for persis-
are immunocompromised. Streptococcus, tent refractory disease or to rule out malig-
Staphylococcus, or anaerobes account for the nancy [2–5].
majority of cases. Patients present with fever, C. Several drugs can cause painless thyroiditis
neck pain, odynophagia, and dysphonia. including interferon alfa, interleukin 2, amio-
Some patients develop thyrotoxicosis. Fine- darone, lithium, kinase inhibitors, and
needle aspiration (FNA) should be performed immune checkpoint inhibitors. Some of these
for culture, and computed tomography (CT) agents result in hyperthyroidism and some
can be obtained to delineate the extent of hypothyroidism [4, 5].
infection including sinus tracts and drainable D. Painless thyroiditis is a sporadic variant of
collections. Treatment consists of parenteral chronic autoimmune thyroiditis, which is
antibiotics, percutaneous or open drainage of characterized by transient hyperthyroidism,
abscesses, and thyroidectomy for patients sometimes followed by hypothyroidism and
who fail more conservative management [1]. then recovery [4, 5].
B. De Quervain’s thyroiditis or subacute non- E. Postpartum thyroiditis is painless thyroiditis
suppurative thyroiditis is thought to result occurring within 1 year after pregnancy. It
from a viral infection and is characterized by occurs in 8–10% of pregnancies when tested
a tender enlarged gland that progresses for with thyroid antibodies; however, much
fewer women develop symptoms of hyper- or
L. Czerwonka (*) hypothyroidism [4, 5].
Department of Surgery, Division of Otolaryngology – F. Lymphocytic or Hashimoto’s thyroiditis is the
Head and Neck Surgery, Stony Brook University most common cause of hypothyroidism with
Hospital, Stony Brook, NY, USA an annual incidence of 0.3–1.5 cases per 1000
persons. Most cases present with a nontender
428 L. Czerwonka
goiter, but it can also present in atrophic forms. ment of thyroid parenchyma and surrounding
Rarely patients can present with hyperthyroid- tissue by essentially scar. This leads to a firm
ism. Diagnosis can be confirmed with thyroid “woody” thyroid gland which can cause com-
autoantibodies (90% of patients have TPO pression including dysphagia, dysphonia, and
antibodies), although not usually necessary. dyspnea. Diagnosis usually requires open
Treatment consists of thyroid replacement in biopsy. The disease can respond to steroids,
clinically hypothyroid patients. Surgery is but surgery is usually required to decompress
reserved for compressive symptoms [4–6]. the trachea by performing a wedge excision
. Riedel’s thyroiditis is a rare variant of chronic
G of the isthmus. More extensive resection is
thyroiditis that is characterized by replace- not advised due to the fibrosis [4].
Presentation: Thyroid enlarged, +/– firm, +/– tender, TFTs
Clinically hyperthyroid TFTs, Clinically hypothyroid

bB, antithyroidals Levothyroxine
symptoms TSH >10
Yes No
Painful
B C
Subacute Subacute
Timing De Quervain’s thyroiditis Drug-induced thyroiditis Timing
No
Acute Chronic
A
High-dose ASA, No Yes
<1 yr
Suppurative thyroiditis Steroids if severe
parturition
D Painless thyroiditis E Postpartum thyroiditis

FNA culture,
IV antibiotics,
CT neck
No
Firm
F
Fistula Surgery once
Abscess acute inflammation Hashimoto’s thyroiditis Yes
resolves
Stable G Reidel’s thyroiditis

Airway Percutaneous drainage
Compromised
Compressive symptoms Open biopsy,
Urgent I&D or thyroidectomy decompression steroids
Follow-up: TSH q4–12 months or change in symptoms
Algorithm 105.1
105 Thyroiditis 429
References 4. Pearce EN, Farwell AP, Braverman LE. Thyroiditis. N

Engl J Med. 2003;348(26):2646–55.
5. Sweeney LB, Stewart C, Gaitonde DY. Thyroiditis:
1. Paes JE, Burman KD, Cohen J, Franklyn J, McHenry
an integrated approach. Am Fam Physician.
CR, Shoham S, et al. Acute bacterial suppurative thy-
2014;90(6):389–96.
roiditis: a clinical review and expert opinion. Thyroid.
6. Jonklaas J, Bianco AC, Bauer AJ, Burman KD,
2010;20(3):247–55.
Cappola AR, Celi FS, et al. Guidelines for the treat-
2. Fatourechi V, Aniszewski JP, Fatourechi GZE,
ment of hypothyroidism: prepared by the American
Atkinson EJ, Jacobsen SJ. Clinical features and out-
Thyroid Association Task Force on thyroid hormone
come of subacute thyroiditis in an incidence cohort:
replacement. Thyroid. 2014;24(12):1670–751.
Olmsted County, Minnesota, study. J Clin Endocrinol
Metabol. 2003;88(5):2100–5.
3. Ranganath R, Shaha MA, Xu B, Migliacci J,
Ghossein R, Shaha AR. de Quervain’s thyroiditis: a
review of experience with surgery. Am J Otolaryngol.
2016;37(6):534–7.
Goiter
106
Lukasz Czerwonka
Algorithmic Approach patients to decrease or stabilize the size of the

goiter but is effective in less than half of
A. Toxic multinodular goiter is characterized by patients with modest decrease in size of about
hyperthyroidism in the setting of an enlarged 25% and likely rebound after discontinuation
multinodular thyroid gland with hot nodules of treatment [3, 4].
on thyroid radioactive iodine uptake scan C. Surgery for nontoxic goiter is indicated in
(RAIU). It usually presents in older individu- patients with concern for malignancy, com-
als with history of nontoxic multinodular goi- pressive symptoms, persistent enlargement,
ter where one or several nodules have or significant cosmetic deformity [4]. Patients
developed autonomous hormone production. should be at reasonable surgical risk. Elderly
Treatment begins with antithyroidal medica- patients with stable asymptomatic goiters do
tions to control signs and symptoms followed not require surgical treatment.
by definitive treatment ideally total or near- D. Prior to undertaking surgery for goiter with
total thyroidectomy. In patients at high opera- possible substernal extension, a computed
tive risk, radioactive iodine can be used but tomography (CT) of the neck down to the
can cause swelling which may worsen any carina or a CT of the chest should be obtained
airway compromise [1, 2]. to rule out significant substernal extension, so
B. Nontoxic goiter has multiple etiologies rang- appropriate arrangements can be made for
ing from iodine deficiency in areas with possible sternotomy [4].
endemic goiters to enzyme defects or medica- E. Patients who do not undergo surgery should
tion side effects. Goiters can be diffuse or be monitored with thyroid function tests
multinodular. Dominant, enlarging, or tender (TFTs), ultrasound, and/or CT for enlarge-
nodules should undergo fine-needle aspira- ment and thyroid hypo- or hyperfunction.
tion (FNA) biopsy to rule out malignancy. Patients who undergo surgery will require
Patients with clinical hypothyroidism should thyroid hormone replacement and monitoring
be treated with thyroid hormone [2]. Thyroid with first thyroid-stimulating hormone (TSH)
hormone can also be used in euthyroid checked 6–8 weeks postoperatively. Patients
who undergo radioactive iodine (RAI) for
L. Czerwonka (*) toxic multinodular goiter will require contin-
Department of Surgery, Division of ued monitoring for possible recurrence [1, 2].
Otolaryngology – Head and Neck Surgery, Stony
Brook University Hospital, Stony Brook, NY, USA

432 L. Czerwonka
Presentation: enlarged thyroid, +/– compressive symptoms
TSH TSH /nml

T3/T4 T3/T4 /nml
A Toxic TFTs Nontoxic B
Clinically hyperthyroid Clinically hypothyroid

βB, Antithyroidals Levothyroxine
RAIU Ultrasound
Yes Yes
Low Dominant
Thyroiditis FNA
uptake nodule
No C
No
Graves’ or toxic Concern for No
multinodular malignancy
goiter
Yes Yes Compressive

symptoms
Airway
compromise No
Yes
Yes Persistent
No
growth
Antithyroidals RAI
No
D Substernal Obtain Yes Cosmetic

chest CT preop Thyroidectomy deformity
No
E Follow-up: TSH, US/CT q6–12 months
Algorithm 106.1
106 Goiter 433
References Association Guidelines task force on thyroid nod-

ules and differentiated thyroid cancer. Thyroid.
2016;26(1):1–133.
1. Ross DS, Burch HB, Cooper DS, Greenlee MC,
3. Wesche MF, Tiel-v Buul MM, Lips P, Smits NJ,
Laurberg P, Maia AL, et al. 2016 American Thyroid
Wiersinga WM. A randomized trial comparing levo-
Association Guidelines for diagnosis and manage-
thyroxine with radioactive iodine in the treatment of
ment of hyperthyroidism and other causes of thyro-
sporadic nontoxic goiter. J Clin Endocrinol Metabol.
toxicosis. Thyroid. 2016;26(10):1343–421.
2001;86(3):998–1005.
2. Haugen BR, Alexander EK, Bible KC, Doherty GM,
4. Chen AY, Bernet VJ, Carty SE, Davies TF, Ganly I,
Mandel SJ, Nikiforov YE, et al. 2015 American
Inabnet WB, et al. American Thyroid Association
Thyroid Association Management Guidelines for
Statement on optimal surgical management of Goiter.
adult patients with thyroid nodules and differ-
Thyroid. 2014;24(2):181–9.
entiated thyroid cancer: The American Thyroid
Thyroid Nodule
107
Melissa Boltz
Algorithmic Approach calcifications). Additionally, ultrasound may

yield information on the overall vascularity of
A. Evaluation of a patient with a thyroid nodule the gland suggestive of Graves’ disease versus
begins with a thorough history assessing risk a dominant toxic nodule if the patient is hyper-
factors for malignancy. Questions regarding thyroid [2].
compressive symptoms such as voice C. Hyperthyroid patients more commonly have
changes, dysphagia, choking, shortness of either Graves’ disease or a toxic hyperfunc-
breath, coughing, a general feeling of pres- tioning nodule. A thyroid-stimulating immu-
sure and tightness, as well as sudden increase noglobulin (TSI) level can be checked and if
in size are important. Additionally, the patient high indicates Graves’ disease. If US reveals
should be asked about family history of thy- a dominant nodule, a radionuclide scan can
roid disorders, other endocrinopathies, and be done to determine if it is hyperfunctional
personal exposure to ionizing radiation to the (hot nodule). If the US and radionuclide scan
head or neck region [1]. are concordant, FNA is not necessary as the
B. Biochemical assessment of thyroid function incidence of malignancy is low (5%). Both
(serum TSH, free T4, free T3) is required as causes of hyperthyroidism can be treated with
clinical assessment will not reliably indicate either medication, radioactive iodine abla-
thyroid status. If the patient is euthyroid or tion, or total thyroidectomy with life-long
hypothyroid, a fine-needle aspiration (FNA) thyroid supplementation afterward [1].
biopsy should be done to assess the cytology D. Patients with normal thyroid function but

of the nodule. A suppressed TSH indicates with compressive symptoms should have
hyperthyroidism. In general, FNA is not done either a lobectomy or total thyroidectomy.
in this situation as it may cause thyrotoxicosis. E. Patients with normal thyroid function who do
Neck ultrasonography (US) can be performed not have compressive symptoms with nod-
to identify additional nodules not palpable on ules >1 cm meet biopsy criteria with
exam. Some US characteristics may indicate a ultrasound-guided FNA [2].
higher risk of malignancy (irregular nodule F. The Bethesda system for reporting thyroid
borders, hypervascular, hypoechoic, internal FNA cytopathology is used to recommend
clinical management based on implied risk of
M. Boltz (*) malignancy. Patients with nondiagnostic
Department of Surgery, Penn State Hershey Medical cytology should have a repeat FNA 3 months
Center, Hershey, PA, USA after the initial biopsy. Waiting 3 months

436 M. Boltz
reduces the likelihood of having the second has a near 100% risk. Both of those cytology
FNA return as inadequate. If it is, then lobec- results warrant total thyroidectomy [2].
tomy can be done for definitive pathology.
Benign cytology should be followed yearly Of note, a thyroid mass in the setting of ele-
with US versus repeating the FNA if the nod- vated calcitonin levels is pathognomonic for
ule grows or surgery if compressive symp- medullary thyroid cancer. Medullary thyroid can-
toms develop. Atypia of undetermined cer involves the calcitonin-producing parafollicu-
significance has a 5–15% risk of malignancy; lar C cells and is the most common initial
thus a repeat FNA is done, and if the cytology presentation of multiple endocrine neoplasia
remains the same, definitive operation is (MEN) IIa and MEN IIb syndromes. It is neces-
offered. A report of suspicious follicular neo- sary to rule out a pheochromocytoma (and treat if
plasm harbors a 15–30% risk of malignancy, present) and hyperparathyroidism prior to per-
and diagnostic surgery of either lobectomy or forming a total thyroidectomy and central lymph
total thyroidectomy is indicated. A diagnosis node dissection. A modified radical neck dissec-
of follicular cancer is supported by pathology tion is required if lymph nodes are positive for
demonstrating capsular and vascular inva- disease. RET proto-oncogene mutations should
sion. Suspicion for malignancy has a 60–80% be investigated as it predicts the prognosis and
risk of malignancy, and malignant cytology aggressiveness of the disease.
Thyroid nodule
A
B Diagnostic evaluation/imaging Hyperthyroid C

• Thyroid function tests
• Neck ultrasonography
D
Graves’ disease Toxic nodule
Compressive symptoms Normal E
• Medication Radionuclide scan

Surgery • Radioactive iodine
ablation
• Definitive surgery
FNA F
Follicular neoplasm/
Nondiagnostic/ Atypia/follicular lesion of Suspicious for
Benign suspicious for Malignant
unsatisfactory undetermined significance malignancy
follicular neoplasm
Repeat FNA • Repeat US annually Diagnostic surgery

• Repeat FNA vs. • Lobectomy
surgery in case of growth, • Total thyroidectomy Total thyroidectomy
Inadequate compressive symptoms
Repeat FNA
Lobectomy vs. FNA Abbreviations: AUS, Atypia of undetermined
Benign AUS/FLUS significance; FNA, Fine needle aspiration; FLUS,
Follicular lesion of undetermined significance
Algorithm 107.1
107 Thyroid Nodule 437
References SI, Sosa JA, Steward DL, Tuttle RM, Wartofsky L.

2015 American Thyroid Association Management
Guidelines for adult patients with thyroid nodules
1. Suliburk J, Delbridge L. Thyroid nodule. In Morita
and differentiated thyroid cancer: The American
SY, Dackiw APB, Zeiger MA, editors. McGraw-
Thyroid Association Guidelines task force on thyroid
Hill manual endocrine surgery. 1st ed. New York.
nodules and differentiated thyroid cancer. Thyroid.
McGraw-Hill; 2009.
2016;26(1):1–133.
2. Haugen BR, Alexander EK, Bible KC, Doherty GM,
Mandel SJ, Nikiforov YE, Pacini F, Randolph GW,
Sawka AM, Schlumberger M, Schuff KG, Sherman
Thyroid Cancer
108
Melissa Boltz
Algorithmic Approach of the primary tumor with total thyroidec-

tomy and appropriate lymph node dissection
A. Papillary, follicular, medullary, and anaplas- to allow accurate staging and guide postop-
tic make up the majority of thyroid cancers erative treatment. If during thyroidectomy a
with papillary thyroid cancer (PTC) being the suspicious-appearing central neck node is
most common. Patients referred with a diag- encountered, it is sent for frozen section, and
nosis of biopsy-proven PTC should have a if positive for PTC, an ipsilateral central neck
history obtained paying attention to past his- dissection (CND) is done. Patients with
tory of radiation exposure, family history of biopsy-proven lateral neck nodes identified
thyroid malignancy, and symptoms of inva- preoperatively should have an ipsilateral
sive disease such as voice changes or hemop- CND and selective lateral neck dissection
tysis. Physical exam is done to assess for (LND) of level II, III, and IV lymph nodes
local metastasis with palpable cervical lymph during the initial thryoidectomy [2].
nodes [1]. D. Vocal cord paralysis on preoperative laryn-
B. At the very least, patients with PTC undergo goscopy indicates tumor involvement of the
total thyroidectomy, but preoperative staging RLN. An R0 resection should still be
is necessary to determine additional concom- attempted with potential sacrifice of the RLN
itant surgical interventions. Neck ultrasonog- if necessary [2].
raphy is essential to assess for possible E. Invasion of the aerodigestive tract on preop-
metastases to lateral cervical lymph nodes erative staging warrants further assessment
(LN). Pathologic-appearing nodes should be for distant metastases and functional status.
biopsied to aid in operative planning. If his- Patients with poor performance status should
tory is suggestive of invasion of the recurrent not undergo extensive surgical intervention.
laryngeal nerve (RLN) trachea or esophagus, Those with good performance status and
then laryngoscopy, bronchoscopy, and/or regional disease may have a shave resection
endoscopy should be performed [2]. of the tumor for tracheal or esophageal wall
C. In general, the treatment for thyroid malig- invasion. Luminal invasion involves laryngo-
nancies is multifaceted and involves removal tracheal or full-thickness esophageal resec-
tion. While these procedures can be
M. Boltz (*) excessively morbid, it may be appropriate in
Department of Surgery, Penn State Hershey Medical highly selective patients when combined with

440 M. Boltz
multimodal therapy along with radiation and G. Postoperative follow-up includes thyroid

chemotherapy [1]. stimulating hormone (TSH) suppression,
F. For situations where local invasion is identi- ultrasound surveillance of the neck, and mon-
fied intraoperatively, the RLN should be pre- itoring thyroglobulin levels. Depending on
served or reconstructed while tumors invading the stage, pathology, and extent of disease,
the tracheal or esophageal walls can be radioactive iodine ablation, external beam
shaved or segmentally resected [2]. radiation therapy, or chemotherapy may be
used as adjuvant therapies [1, 2].
Biopsy-proven PTC
A
• Fixed nodule, rapid growth
• Symptoms of invasion (hoarseness, hemoptysis)
• Palpable cervical LN
B Preoperative staging
• Neck US: assess cervical LN
• FNA-suspicious LN
• Laryngoscopy/bronchoscopy/endoscopy if suspicious
for involvement of RLN/trachea/esophagus
Operation: total thyroidectomy

C D E F
Intraop-suspicious Biopsy-proven PTC Vocal cord paralysis on Invasion of aerodigestive Introp finding of
central LN in lateral cervical preop laryngoscopy tract on preop staging local invasion
neck node(s)
Send frozen section Attempt R0 resection, Evaluate for distant RLN Tracheal/esophageal
Ipsilateral CND and with involved segment of metastases, assess invasion wall invasion
selective LND RLN if necessary performance status
Ipsilateral CND
Preserve nerve if Shave/segmental
Resectable local Palliative care for possible or resection
disease, good multiple metastases, resect/reconstruct
performance status poor performance status
Tracheal/esophageal Tracheal/esophageal
wall invasion lumen invasion
Abbreviations: CND, central neck dissection; Shave/segmental Laryngotracheal resection

FNA, fine needle aspiration; LN, lymph node; resection or full-thickness
LND, lymph node dissection; PTC, papillary esophageal resection
thyroid cancer; RLN, recurrent laryngeal
nerve; US, ultrasound G Follow-up
Algorithm 108.1
108 Thyroid Cancer 441
References oncology handbook. 4th ed. Philadelphia: Lippincott

Williams & Wilkins; 2012.
2. Elaraj DM, Sturgeon C. Papillary thyroid Cancer.
1.
Thoas RM, Habra MA, Perrier ND, Grubbs
In: Morita SY, Dackiw APB, Zeiger MA, editors.
EG. Well-
differentiated carcinoma of the thyroid
McGraw-hill manual endocrine surgery. 1st ed.
and neoplasms of the parathyroid gland. In: Feig
New York: McGraw-Hill; 2009.
BW, Ching CD, editors. The MD Anderson surgical
Hyperparathyroidism
109
Melissa Boltz
Algorithmic Approach mia (FHH), which is an autosomal dominant

disorder of the renal calcium-sensing recep-
A. The recognition of primary hyperparathy-
tor that can mimic pHPT. The urine calcium
roidism (pHPT) increased in the 1970s with levels in FHH patients are usually less than
the introduction of routine automated cal- 50 mg/24 h [1].
cium testing, and the incidence continues to B. When pHPT is biochemically confirmed, the
rise today [1]. Although pHPT secondary to a patient can be classified as either symptom-
parathyroid adenoma is the most common atic or asymptomatic. Parathyroidectomy is
cause of hypercalcemia in the outpatient set- recommended for all patients with objective
ting, the evaluation of a patient referred for symptoms of nephrolithiasis or fragility frac-
hypercalcemia begins with consideration of tures. Although neurocognitive symptoms are
other non-parathyroid causes. The diagnosis subjective, there is strong evidence to support
of pHPT is biochemical. Initial laboratory parathyroidectomy, as observational studies
evaluation should include concomitant serum suggest improved symptoms of muscle
calcium and intact parathyroid hormone strength and functional capacity after surgery.
(PTH). In classic pHPT, patients present with Surgical treatments in asymptomatic individ-
an elevation of both calcium and PTH and a uals are guided by the National Institutes of
negative history of multiple endocrine neo- Health criteria and include age younger than
plasia (MEN) syndromes. A chloride-to- 50 years, significant hypercalcemia >1 mg/dL
phosphate (Cl/PO4) ratio >33 also suggests over the reference range, 24-h UCa > 400 mg/
pHPT. An X-ray of the hands or skull may dL, renal insufficiency with creatinine clear-
demonstrate cystic formations due to resorp- ance reduced by 30%, osteoporosis with
tion of Ca. A 25-hydroxyvitamin D level is t-score of the spine < −2.5, and patient desire
necessary to assess for a correctable second- for surgical intervention [2].
ary HPT due to vitamin D deficiency. C. Imaging is performed for operative planning
Additionally, a 24-h urine calcium (24-h after deciding whether or not a patient is a
UCa) measurement should be obtained to rule surgical candidate, as it has no utility in con-
out benign familial hypocalciuric hypercalce- firming or excluding the diagnosis of
pHPT. Neck ultrasonography is recom-
M. Boltz (*) mended to localize parathyroid disease as it is
Department of Surgery, Penn State Hershey Medical noninvasive and the least costly. It can also
Center, Hershey, PA, USA assess for concomitant thyroid disease, which

444 M. Boltz
should be addressed during the parathyroid- eucalcemia. If the PTH fails to decrease,
ectomy if appropriate. If ultrasound is incon- hypersecreting parathyroid tissue remains
clusive, then technetium Tc 99 m sestamibi present and requires further bilateral neck
scan or 4D-CT of the neck can be performed exploration [2].
depending on surgeon and institution prefer- F. Oral calcium supplementation is typically
ence [1]. provided for 2 weeks postoperatively to
D. In cases where imaging does not localize a prevent symptomatic transient hypocalce-
parathyroid adenoma or is discordant, a bilat- mia. Patients who are vitamin D deficient
eral neck exploration should be performed. should receive vitamin D supplementation
All four parathyroid glands are identified as well. Serum calcium, PTH, and vitamin
with removal of the abnormal-appearing or D levels should be checked 6 months post-
enlarged gland(s). Intraoperative parathyroid operatively to assess for cure or persistent
hormone monitoring (IOPM) may or may not disease.
be used as an adjunct to assess cure [2]. All
specimens removed should be sent to pathol- Of note, secondary hyperparathyroidism
ogy for frozen section to confirm the presence occurs in the setting of chronic renal failure. Low
of parathyroid tissue. levels of calcium lead to elevated PTH and
E. Focused or unilateral parathyroidectomy with increased calcium resorption from the bone and
IOPM may be done with a localized parathy- hyperplastic parathyroid glands. Biochemical
roid adenoma. A baseline PTH is drawn prior evaluation demonstrates an elevated PTH and
to excision of the abnormal parathyroid decreased calcium levels. Three-and-a-half-gland
gland, and the gland is excised and sent for or four-gland resection with autotransplantation
frozen section to confirm parathyroid tissue. is a treatment of choice. Tertiary hyperparathy-
Another PTH is drawn 10 min post-excision. roidism occurs in renal transplant patients. Both
The post-excision PTH should drop by at PTH and calcium are elevated. Treatment is
least 50% from the baseline PTH and into the either three-and-a-half-gland or four-gland resec-
normal range to predict cure or postoperative tion with autotransplantation.
109 Hyperparathyroidism 445
Referral for hypercalcemia

Suspicious for primary hyperparathyroidism
A
Consider other causes: medications (Thiazides, lithium, bisphosphonates, vitamin D), FHH,
malignancy, sarcoidosis
Confirm biochemical dx: serum calcium, intact PTH, vitamin D, 24-h urine calcium
B
Asymptomatic Symptomatic
Age < 50 years Nephrolithiasis

Ca > 1mg/dL above reference range Fragility fractures
24-hr UCa > 400 mg/dL Neurocognitive symptoms (short-
term memory loss, difficulty
Renal insufficiency
concentrating), irritability, anxiety
Osteoporosis (t-score < –2.5)
Fatigue, myalgias, arthralgias
Patient prefers surgery
Constipation, polyuria, polydipsia
No Yes Surgical candidate
Non-operative management Pre-operative localization C

Ultrasound
Sestamibi (parathyroid scan)
4D-CT (neck/upper chest)
D Non-localized Localized E
Bilateral neck exploration Focused parathyroidectomy vs. bilateral

IOPM neck exploration
IOPM
PTH ¯50% and into normal range
Abbreviations: 4D-CT, four dimensional

computed tomography; Ca, calcium; FHH,
F End surgery/Follow-up familial hypocalciuric hypocalcemia; IOPM,
intraoperative parathyroid hormone monitoring;
PTH, parathyroid hormone; UCa, urine calcium
Algorithm 109.1
446 M. Boltz
References primary h yperparathyroidism. JAMA Surg.

2016;151(10):959–68.
2. Sneider MS, Solorzano CC, Lew JI. Primary hyper-
1. Wilhelm SM, Wang TS, Ruan DT, et al. The
parathyroidism. In: Morita SY, APB D, Zeiger MA,
American Association of Endocrine Surgeons
editors. McGraw-Hill manual endocrine surgery. 1st
guidelines for definitive management of
ed. New York: McGraw-Hill; 2009.
Part XV
Endocrine
Cushing’s Syndrome and Disease
110
Edwina Moore and Vikram D. Krishnamurthy
Algorithmic Approach between pituitary and ectopic etiology. Serum

cortisol levels will not suppress after high-
A. The signs and symptoms associated with
dose dexamethasone intake when the excess
Cushing’s syndrome and disease include cen- ACTH secretion is ectopic in origin [3].
tral obesity, impaired glucose tolerance, D. Once the biochemical diagnosis has been made,
hypertension, fluid retention, dermatologic imaging is obtained to identify the source.
changes (abdominal striae, acne, hirsutism, Cushing’s syndrome may be caused by a solitary
easy bruising), musculoskeletal decline adrenal adenoma, bilateral adrenal hyperplasia,
(proximal muscle weakness, osteopenia), and or adrenocortical carcinoma. All three can be
poor wound healing/immunosuppression [1]. identified on computed tomography of the abdo-

B. The first step in diagnosis is establishing men, especially “adrenal protocol” when avail-
hypercortisolism, which can accurately be able. Cushing’s disease from a pituitary adenoma
assessed with a 24-h urine collection for cor- is identified by magnetic resonance imaging of
tisol and creatinine. Late-night salivary corti- the pituitary and/or inferior petrosal sinus sam-
sol levels on two to three separate evenings, if pling. Neuroendocrine tumors secreting ectopic
elevated, demonstrate the loss of normal diur- ACTH can be found throughout the body and
nal variation [2]. identification should be directed at the lung,

C. The second step involves distinguishing mediastinum, gastrointestinal tract, retroperito-
between Cushing’s syndrome and Cushing’s neum, pancreas, and neck.
disease or ectopic ACTH secretion by obtain- E. Surgery is the first-line treatment option for
ing a serum ACTH measurement. If the ACTH most causes of Cushing’s syndrome, such as
level is normal or suppressed, then autono- unilateral adrenalectomy for adrenal adeno-
mous secretion of cortisol from the adrenal mas and carcinomas, transsphenoidal selec-
should be suspected. If the ACTH level is ele- tive adenectomy for pituitary adenomas, and
vated, then either a pituitary adenoma or ecto- resection of tumor secreting ectopic ACTH
pic secretion from a neuroendocrine tumor [4]. Bilateral adrenalectomy is reserved as a
should be suspected. A high-dose dexametha- palliative measure in severe, debilitating
sone suppression test will differentiate hypercortisolism from occult, refractory, or
metastatic ectopic ACTH secretion [5].
E. Moore · V. D. Krishnamurthy (*) F. After surgery, glucocorticoid replacement
Department of Endocrine Surgery, The Cleveland may be necessary until the patient’s
Clinic, Cleveland, OH, USA hypothalamic-pituitary-adrenal axis recov-

450 E. Moore and V. D. Krishnamurthy
ers. Lifelong follow-up and treatment of necessary. Depending on the etiology,

comorbidities such as hypertension, glu- patients also may need to be followed up
cose intolerance, and bone loss may be for recurrence [4, 6].
Suspicion for hypercortisolism based on phenotype, hypertension, glucose

A intolerance, weight gain, fluid retention, easy bruising, abdominal striae
Obtain a 24-h urine collection for cortisol and creatinine and repeated late-
B night salivary cortisol measurements
Obtain a serum ACTH level, and if elevated, perform a high-dose dexamethasone

C suppression test
D
If Cushing’s syndrome, obtain CT adrenal protocol
If Cushing’s disease, obtain MRI pituitary and/or

inferior petrosal sinus sampling
If ectopic, image chest, mediastinum, abdomen,

neck and/or endoscopy
Treatment is unilateral adrenalectomy for adrenal adenomas and carcinomas,

E trans-sphenoidal selective adenectomy for pituitary adenomas, and resection of
tumor secreting ectopic ACTH
Postoperatively, screen for adrenal insufficiency, treat comorbidities, and

F monitor for recurrence
Algorithm 110.1
110 Cushing’s Syndrome and Disease 451
References a consensus statement. J Clin Endocrinol Metab.

2008;93(7):2454–62.
4. Nieman LK, Biller BM, Findling JW, Murad MH,
1. Dekkers OM, Horvath-Puho E, Jorgensen JO,
Newell-Price J, Savage MO, et al. Treatment of
Cannegieter SC, Ehrenstein V, Vandenbroucke
Cushing’s syndrome: an endocrine society clini-
JP, et al. Multisystem morbidity and mortality in
cal practice guideline. J Clin Endocrinol Metab.
Cushing’s syndrome: a cohort study. J Clin Endocrinol
2015;100(8):2807–31.
Metab. 2013;98(6):2277–84.
5. Findling JW, Raff H. Cushing’s syndrome: impor-
2. Nieman LK, Biller BM, Findling JW, Newell-Price
tant issues in diagnosis and management. J Clin
J, Savage MO, Stewart PM, et al. The diagnosis of
Endocrinol Metab. 2006;91(10):3746–53.
Cushing’s syndrome: an endocrine society clini-
6. Mitchell J, Barbosa G, Tsinberg M, Milas M,
Siperstein A, Berber E. Unrecognized adrenal insuf-
2008;93(5):1526–40.
ficiency in patients undergoing laparoscopic adrenal-
3. Biller BM, Grossman AB, Stewart PM, Melmed
ectomy. Surg Endosc. 2009;23(2):248–54.
S, Bertagna X, Buchfelder M, et al. Treatment of
adrenocorticotropin-dependent Cushing’s syndrome:
Primary Hyperaldosteronism
(Conn’s Syndrome) 111
Iuliana Bobanga, Cassandre Bénay,
and Vikram D. Krishnamurthy
Algorithmic Approach ing may be performed (e.g., oral sodium

loading, saline infusion, or captopril chal-
A. Primary hyperaldosteronism is the most com- lenge testing) [1, 2].
mon cause of secondary hypertension and C. After establishing the diagnosis of hyperaldo-
should be suspected in patients with hyper- steronism by biochemical studies, the next step
tension that is diagnosed earlier than age 35, is localizing the production to either a unilateral
severe (BP < 160/100), persistent despite >3 adenoma or bilateral hyperplasia. The imaging
medications, and present along with hypoka- modality of choice is an “adrenal protocol” thin-
lemia and/or an adrenal incidentaloma [1]. cut computed tomography (CT) scan, which
B. The first diagnostic assessment in patients can demonstrate normal adrenal glands, thick-
with Conn’s syndrome requires demonstra- ened glands, or a unilateral adrenal mass [3]. An
tion of aldosterone excess along with sup- aldosterone-producing adenoma should be sus-
pressed plasma renin activity. Before pected in younger patients with hypertension
measuring serum aldosterone levels and whose CT scan reveals a unilateral adrenal mass
plasma renin activity, medications that can and a contralateral normal adrenal gland.
affect these measurements (e.g., angiotensin- D. The laterality of aldosterone hypersecretion is
converting enzyme inhibitors, angiotensin then confirmed by bilateral adrenal venous
receptor blockers, aldosterone receptor antag- sampling (AVS), which is the gold standard for
onists, and beta-blockers) are replaced with distinguishing between unilateral and bilateral
others (e.g., calcium channel blockers or disease. The acquisition of AVS in every
alpha-adrenergic blockers) for several weeks. patient with primary hyperaldosteronism is
The aldosterone-to-renin ratio (ARR) is then controversial; however, many centers perform
calculated, with a ratio >20–40:1 along with this routinely. Reliance on CT findings alone is
an aldosterone level >15 ng/dL being sugges- questionable for two reasons: (1) aldosterono-
tive of primary hyperaldosteronism (further- mas are typically small and may be missed
more, an ARR >35 is 100% sensitive and (false negatives) and (2) non- aldosterone-
92% specific). If the ARR is equivocal but the secreting/nonfunctional adrenal adenomas
diagnosis is still suspected, confirmatory test- increase with age (false positives). During
AVS, aldosterone levels are obtained from the
I. Bobanga · C. Bénay · V. D. Krishnamurthy (*) SVC, IVC, and adrenal veins. Cortisol levels
Department of Endocrine Surgery, The Cleveland are also obtained, with an adrenal vein to IVC
Clinic, Cleveland, OH, USA cortisol ratio >2:1 confirming successful

454 I. Bobanga et al.
c annulation of the adrenal veins. The aldoste- the vascular damage caused by the prior long-
rone-to-cortisol ratio between both adrenal standing, severe hypertension. Factors pre-
veins is then compared. A ratio of more than dictive of the resolution of hypertension
4:1 lateralizes unilateral hypersecretion and include female sex, body mass index
identifies the gland to be removed [4, 5]. (BMI) ≤25, hypertension lasting ≤6 years,

E. Hyperaldosteronism is typically cured by prescription of ≤2 antihypertensive medica-
laparoscopic adrenalectomy; however, blood tions, and decline in plasma aldosterone by
pressure may remain elevated secondary to 10 ng/dL on postoperative day 1 [6, 7].
Suspect primary hyperaldosteronism in patients when:

Age <35
Severe hypertension (BP>160/100)
>3 antihypertensive medications required
A
Hypokalemia
Adrenal incidentaloma
Obtain Plasma Aldosterone Concentration (PAC)

B and Plasma Renin Activity (PRA)
Unlikely primary
PAC/PRA >20? hyperaldosteronism
No
Y es
C Obtain adrenal CT scan
D Obtain adrenal venous sampling
Lateralization?
No Yes
E
Medical management Unilateral adrenalectomy
Algorithm 111.1
111 Primary Hyperaldosteronism (Conn’s Syndrome) 455
References of Endocrine Surgeons medical guidelines for the

management of adrenal incidentalomas. Endocr
Pract. 2009;15(Suppl 1):1–20.
1. Harvey AM. Hyperaldosteronism: diagnosis, lat-
5. Chao CT, Wu VC, Kuo CC, Lin YH, Chang CC,
eralization, and treatment. Surg Clinic N Am.
Chueh SJ, et al. Diagnosis and management of pri-
2014;94(3):643–56.
mary aldosteronism: an updated review. Ann Med.
2. Yin G, Zhang S, Yan L, et al. One-hour upright
2013;45(4):375–83.
posture is an ideal position for serum aldosterone
6. Aranova A, Gordon BL, Finnerty BM, Zarnegar
concentration and plasma renin activity measuring
R, Fahey TJ. Aldosterone resolution score pre-
on primary aldosteronism screening. Exp Clin
dicts long-term resolution of hypertension. Surgery.
Endocrinol Diabetes. 2012;120(7):388–94.
2014;156(6):1387–92.
3. Bobanga ID, McHenry CR. Chapter 6. Imaging
7. Swearingen AJ, Kahramangil B, Monteiro R,
modalities for adrenal cortical tumors. In: Kebebew
Krishnamurthy VD, Jin J, Shin JJ, et al. Analysis
E, editor. Management of adrenal masses in children
of postoperative biochemical values and clinical
and adults. 1st ed. Switzerland: Springer; 2017.
outcomes after adrenalectomy for primary aldoste-
4. Zeiger MA, Thompson GB, Duh Q-Y, Hamrahian AH,
ronism. Surgery. 2017. https://doi.org/10.1016/j.
Angelos P, Elaraj D, et al. American Association of
surg.2017.10.045.
Clinical Endocrinologists and American Association
Glucagonoma
112
Talia Burneikis and Vikram D. Krishnamurthy
Algorithmic Approach C. Initial treatment is supportive and involves

glycemic control and improving nutritional
A. When suspected, the diagnosis is established status. When disease burden is amenable,
by an elevated fasting glucagon level >500 pg/ resection of the primary tumor and debulking
mL (normal reference range <50 pg/mL). of metastases are indicated. Even for local-
Additional findings include normocytic ane- ized disease, laparotomy is generally the rec-
mia and secondary elevations of neuroendo- ommended surgical approach due to the
crine markers (gastrin, somatostatin, generally large tumor size and need for
vasoactive intestinal peptide, serotonin, and lymphadenectomy [5]. Liver metastasis can
pancreatic polypeptide) [1]. be addressed by anatomic hepatectomy, mul-
B. Once the diagnosis is confirmed, imaging is tiple “wedge” resections, and/or radiofre-
obtained to localize primary tumors and quency and microwave ablation [6].
stage for distant disease, as glucagonomas D. For patients with unresectable disease and/or
are almost always malignant. Computed overwhelming tumor burden, debulking, hepatic
tomography (CT) and magnetic resonance artery embolization (with or without doxorubi-
imaging (MRI) are sensitive initial imaging cin, cisplatin, streptozocin, drug-eluting beads),
modalities to detect primary tumors, as most and somatostatin analogs can be palliative.
glucagonomas are >3 cm [2]. If CT does not Systemic therapies include streptozocin, temo-
demonstrate an obvious pancreatic lesion, zolomide, everolimus, and sunitinib [7].
endoscopic ultrasound (EUS) can detect E. Subsequent follow-up is dependent on stage at
tumors as small as 2–3 mm [3]. Extra- presentation, as well as multiple endocrine neo-
pancreatic disease can be detected with plasia syndrome type 1 (MEN1) status. Generally,
somatostatin-receptor scintigraphy (octreo- guidelines recommend evaluation every
tide scan) or gallium Ga-68 DOTATATE- 3–12 months with history and physical exam,
PET scan [4]. serum glucagon measurement, and imaging [7].
T. Burneikis · V. D. Krishnamurthy (*)

Department of Endocrine Surgery, The Cleveland

458 T. Burneikis and V. D. Krishnamurthy
Suspect glucagonoma with weight loss, rash,

A
glucose intolerance, diabetes, diarrhea,
venous thrombosis and serum glucagon
measurement >500 pg/mL.
Imaging and staging with triple-phase contrast CT

B (or MRI). EUS, octreotide scan, and dotatate-PET
may further characterize tumor burden.
C Provide nutritional
support, optimize
glycemic control, and
determine resectability.
Localized or metastatic
disease with >90% Unresectable disease or
resectable and unacceptable operative risk.
acceptable operative risk.
Pancreatic resection with Palliative procedures:

or without cytoreductive surgery,
resection/ablation of radiofrequency ablation,
D
liver metastases cryoablation, hepatic artery
embolization (+/–
chemotherapy) and/or liver
transplantation and/or
systemic therapies:
somatostatin analogues,
molecular-targeted
(everolimus, sunitinib), and/or
chemotherapy (temozolomide,
stretozocin).
E
Surveillance and follow-up:
H&P, glucagon levels, axial imaging (CT or MRI)
Algorithm 112.1
112 Glucagonoma 459
References 4. Nauck C, Ivancevic V, Emrich D, Creutzfeldt W.

111In-pentetreotide (somatostatin analogue) scin-
tigraphy as an imaging procedure for endocrine
1. Wermers RA, Fatourechi V, Wynne AG, Kvols LK,
gastro-entero-pancreatic tumors. Z Gastroenterol.
Lloyd RV. The glucagonoma syndrome. Clinical
1994;32(6):323–7.
and pathologic features in 21 patients. Medicine
5. Fraker DL, Norton JA. The role of surgery in the
(Baltimore). 1996;75(2):53.
management of islet cell tumors. Gastroenterol Clin
2. Dromain C, de Baere T, Baudin E, Galline J, Ducreux M,
N Am. 1989;18(4):805.
Boige V, Duvillard P, Laplanche A, Caillet H, Lasser P,
6. Karabulut K, Akyildiz HY, Lance C, Aucejo F,
Schlumberger M, Sigal R. MR imaging of hepatic metas-
McLennan G, Agcaoglu O, Siperstein A, Berber
tases caused by neuroendocrine tumors: comparing four
E. Multimodality treatment of neuroendocrine liver
techniques. AJR Am J Roentgenol. 2003;180(1):121.
metastasis. Surgery. 2011;150(2):316–25.
3. Khashab MA, Yong E, Lennon AM, Shin EJ, Amateau
7. National Comprehensive Cancer Network (NCCN).
S, Hruban RH, Olino K, Giday S, Fishman EK,
NCCN Clinical practice guidelines in oncology.
Wolfgang CL, Edil BH, Makary M, Canto MI. EUS
http://www.nccn.org/professionals/physician_gls/f_
is still superior to multidetector computerized tomog-
guidelines.asp. Accessed on 27 Feb 2016.
raphy for detection of pancreatic neuroendocrine
tumors. Gastrointest Endosc. 2011;73(4):691.
Management
of Pheochromocytoma
113
Algorithmic Approach bers or co-exist with other endocrine syn-

dromes [2]. Presenting symptoms are related
A. Pheochromocytomas are rare catecholamine- to the catecholamine excess and tend to be
secreting tumors of the medullary adrenal paroxysmal, although hypertension can be
chromaffin cells. They represent roughly 0.1– sustained or exhibit severe lability with ortho-
0.6% of patients with hypertension (HTN) static hypotension. Symptoms of tremors, pal-
and occur in 3–7% of patients with an inci- pitations, diaphoresis, dyspnea, headache,
dentally found adrenal mass. panic episodes, chest pain, and a sense of
Pheochromocytomas generally occur in the impending death are typical of e pisodic spells
third to fifth decade, and about 10% are malig- and may be spontaneous or exacerbated by
nant. Genetic syndromes associated with postural change, increased abdominal pres-
pheochromocytomas include von Hippel- sure, exercise, and medications. Chronic over-
Lindau disease, neurofibromatosis type 1, and production of catecholamines can lead to
multiple endocrine neoplasia type 2. In addi- weight loss, cardiomyopathy, fatigue, consti-
tion, mutations in any of the succinate dehy- pation, chronic headaches, and sequelae of
drogenase complex subunit genes, including chronic uncontrolled hypertension. Screening
SDHA, SDHB, SDHC, and SDHD, may lead for a pheochromocytoma should be done in
to pheochromocytomas with variable pene- patients with recalcitrant hypertension, hyper-
trance [1]. Genetic testing may be considered tension diagnosed <20 years of age, hyperad-
in all patients diagnosed with a pheochromo- renergic spells, pressor response during
cytoma but should always be performed when procedures, and family history of paragangli-
bilateral, present in children or adults <45, oma, pheochromocytoma, or related syn-
and when they exist in multiple family mem- dromes or in patients with an adrenal mass.
B. Biochemical evaluation is most sensitive and
H. E. Ritter specific with 24-h urine collection of fraction-
Department of Surgery, Indiana University, ated metanephrines and catecholamines and
Indianapolis, IN, USA should include dopamine in addition to creati-
B. C. James (*) nine for adequacy of sample. Plasma testing
Department of Surgery, Harvard Medical School, has a higher false-positive rate but is a useful
Boston, MA, USA screening test in children and patients where
Department of Surgery, Beth Israel Deaconess reliable urine collection is difficult. Clinical
Medical Center, Boston, MA, USA circumstances must be considered regarding

462 H. E. Ritter and B. C. James
the timing of testing as illness, psychiatric Adrenal vein sampling and biopsy of a
medications, and physical stress can confound suspected pheochromocytoma are not recom-
the interpretation of elevated levels. mended and can be detrimental by precipitat-
C. Computed tomography (CT) imaging with ing a hyperadrenergic crisis.
and without intravenous (IV) contrast is rec- D. Medical management is not recommended

ommended for localization of an adrenal but may be needed for treating unresectable
tumor once a biochemical diagnosis has been or diffusely metastatic disease.
made. Pheochromocytomas are usually inho- E. Surgical management with minimally invasive
mogeneous tumors with smooth edges and adrenalectomy is recommended for most
have marked contrast enhancement, which pheochromocytomas. Consideration should
differentiates them from adenomas. Although be given to open adrenalectomy for tumors
they may be of any size, they are usually >6 cm and for any tumor with concerning fea-
greater than 3 cm. Imaging should also be tures of malignancy to ensure complete resec-
reviewed for concerning features of malig- tion and avoid tumor seeding [4].
nancy including local invasion, enhancing Preoperative preparation targeting sympa-
lymphadenopathy, and extra-adrenal pheo- thetic blockade is essential prior to proceeding
chromocytomas, referred to as paraganglio- with surgical intervention to avoid intraopera-
mas. Magnetic resonance imaging (MRI) is tive labile hypertension. Patients should be
useful in patients who are unable to receive started on alpha-blockade until rendered
radiation exposure and may better detail local orthostatic. After adequate alpha-blockade,
invasion. Iodine 123 metaiodobenzylguanidine some patients may develop rebound tachycar-
(123I-MIBG) localization is useful when abdom- dia and should be started on beta-blockade.
inal imaging is negative, when metastatic dis- High-sodium diet and adequate fluid intake
ease is suspected, or when a paraganglioma are recommended for volume expansion.
is identified. F luorodeoxyglucose-positron Intraoperative and postoperative management
emission tomography (FDG-PET) is useful with an experienced team is necessary and
in localizing both primary and metastatic should include arterial monitoring, adequate
tumors with succinate dehydrogenase (SDH) IV access, and pharmacologic preparation for
mutations [1, 3]. rapid cardiovascular shifts.
113 Management of Pheochromocytoma 463

-Hypertension: paroxysmal, sustained or with severe lability
A
-Tremors, palpitations, diaphoresis, dyspnea, headache, panic episodes,
chest pain and a sense of impending death
Laboratory evaluation:
B -Screening with plasma metanephrines
-Confirmatory testing with 24-h urine catecholamines
Tumor localization:
-Non-contrast CT or MRI
Preoperative management:
C
-Alpha-blockade until adequate hypertension control. Addition of beta-blockade
if rebound tachycardia occurs
-High sodium diet and adequate fluid intake for volume expansion
Tumor >6 cm, evidence of

Adenoma concerning features of
malignancy
Consideration for open

D, E Laparoscopic adrenalectomy D, E
adrenalectomy
Algorithm 113.1
References in pheochromocytoma and paraganglioma. J Nucl

Med. 2014;55:1253–9.
4. Lenders JWM, Duh Q-Y, Eisenhofer G, Giminez-
1. Assadiour Y, Sadowski SM, Alimchandani M,
Roqueplo A-P, Grebe SKG, Murad MH, Naruse
Quezado M, Steinberg SM, Nilubol N, Patel D,
M, Pacak K, Young WF Jr. Pheochromocytoma
Prodanov T, Pacak K, Kebebew E. SDHB muta-
and paraganglioma: an endocrine society clini-
tion status and tumor size but not grade are
important predictors of clinical outcome in pheo-
2014;99(6):1915–42.
chromocytoma and abdominal paraganglioma.
Surgery. 2017;161:230–9.
2. Melmed S, Polonsky KS, Larsen PR, Kronenberg
HM. Chapter 16 endocrine hypertension. In: Williams Suggested Reading
textbook of endocrinology. 13th ed. Philadelphia:
Elsevier; 2016. p. 566–88.
Clark OH, Duh Q-Y, Gosnell JE, Shen W. Textbook of
3. Van Berkel A, Rao JU, Kusters B, et al. Correlation
endocrine surgery. 3rd ed. New Delhi: Jaypee Brothers
between in vivo 18F-FDG PET and immunohisto-
Medical Publishers; 2014.
chemical markers of glucose uptake and metabolism
Management of Aldosteronoma
114
Algorithmic Approach B. Biochemical evaluation should include a

plasma aldosterone concentration (PAC),
A. Primary hyperaldosteronism, also known as plasma renin activity (PRA), and a metabolic
Conn’s syndrome, is defined as the excess panel. Patients may occasionally have hypo-
production of aldosterone from an adrenal kalemia and hypernatremia but are more
source. It may be caused by a single adenoma commonly within the normal range. A PAC
or from idiopathic hyperplasia of the adrenal >15 ng/dl, PRA <1.0 ng/ml, and PAC/PRA
glands. Although once considered a rare >20 are suggestive of a primary hyperaldoste-
cause of hypertension, it is now thought to be ronism [1]. A PAC/PRA greater than 35 is
the cause of hypertension in 5–10% of highly sensitive and specific for primary
patients. The majority of patients present hyperaldosteronism. Confirmatory testing
without symptoms. However, patients may with aldosterone suppression tests can be
endorse symptoms of muscle weakness, considered if results are equivocal and in
cramping, headaches, polydipsia, and poly- patients over 40 years of age, as the incidence
uria as a result of hypokalemia. Screening for of both non-functioning adenomas and idio-
hyperaldosteronism should be considered in pathic adrenal hyperplasia (IAH) is higher in
patients diagnosed with hypertension at a this population [3]. Biochemical evaluation
young age, in those with hypokalemia, and in should be performed after cessation of miner-
patients with medically recalcitrant disease alocorticoid receptor antagonists for at least
on three or more antihypertensive agents. 2 weeks. If possible, angiotensin-converting
Additionally, biochemical evaluation for enzyme inhibitors and angiotensin receptor
hyperaldosteronism should be considered in blockers should be discontinued prior to eval-
any patient with an adrenal mass [1, 2]. uation as these medications may falsely ele-
vate the PRA [4].
H. E. Ritter C. Adrenal masses are often identified inciden-
Department of Surgery, Indiana University, tally. If no prior imaging has been performed,
Indianapolis, IN, USA evaluation should include a non-contrasted
B. C. James (*) CT scan. Aldosteronomas are generally small,
Department of Surgery, Harvard Medical School, 1–3 cm, and should have low Hounsfield
Boston, MA, USA units (HU) (>10) indicative of a benign ade-
Department of Surgery, Beth Israel Deaconess noma. If multiple or bilateral small nodules
Medical Center, Boston, MA, USA are identified or if the adrenal glands have a

thickened or micronodular appearance, a oversecretion and may include mineralocorti-

diagnosis of IAH is more likely. Biopsy coid receptor antagonists such as spironolac-
should not be performed [2]. If imaging is tone and eplerenone. Treatment with medical
unclear, adrenal vein sampling (AVS) should therapy may also be considered in patients
be done to lateralize aldosterone hypersecre- with unilateral disease who are not surgical
tion. AVS should also be considered in candidates.
patients >40 to exclude IAH. The presence of E. For patients with clear unilateral disease, sur-
adrenocortical carcinoma in the setting of pri- gical resection with laparoscopic adrenalec-
mary hyperaldosteronism is rare [4]. tomy is recommended and may result in a
D. Medical management is the preferred treat- reduction in the number of required antihy-
ment in patients with bilateral aldosterone pertensives [1, 2, 4].

-Hypertension: early onset, severe or medically recalcitrant
A
-Occasional muscle weakness, cramping, headaches,
polydipsia, polyuria
B -May have hypokalemia and slight hypernatremia
-Elevated PAC >15ng/ml and decreased PRA <1.0 ng/ml per hour
-PAC/PRA >20
If needed confirmatory aldosterone suppression test
Tumor localization, staging, and tissue diagnosis

-Non-contrast CT
If needed: adrenal vein sampling
Idiopathic adrenal
Adenoma
hyperplasia
-Unilateral adrenalectomy
-Mineralocorticoid
D -Mineralocorticoid suppression D, E
suppression
if poor operative candidate
Algorithm 114.1
114 Management of Aldosteronoma 467
References treatment of patients with primary aldosteronism. Eur

J Endocrinol. 2010;162:435–8.
4. Montori VM, WF Y. Use of plasma aldosterone
concentration-to-plasma renin activity ratio as a
HM. Chapter 16: Endocrine hypertension. In:
screening test for primary aldosteronism. A system-
Williams textbook of endocrinology. 13th ed.
atic review of the literature. Endocrinol Metab Clin N
Philadelphia: Elsevier; 2016. p. 566–88.
Am. 2002;31:619–32.
2. Funder JW, Carey RM, Fardella C, Gomez-Sanchez
CE, Mantero F, Stowasser M, Young WF Jr, Montori
V. Case detection, diagnosis, and treatment of patients
with primary aldosteronism: an Endocrine Society Suggested Reading
clinical practice guideline. J Clin Endocrinol Metab.
2008;93(9):3266–81. Clark OH, Duh Q-Y, Gosnell JE, Shen W. Textbook of
3. Arlt W. A detour guide to the Endocrine Society clini- endocrine surgery. 3rd ed. New Delhi: Jaypee Brothers
cal practice guideline on case detection, diagnosis and Medical Publishers; 2014.
Management of Gastrinoma
115
Algorithmic Approach C. Once hypergastrinemia has been established,

other causes of hypergastrinemic state (gas-
A. Gastrinoma is the second most common func- tric outlet obstruction, prior vagotomy, renal
tional pancreatic neuroendocrine tumor [1]. failure, and atrophic gastritis, among others)
The first step in diagnosis is a full history and should be ruled out [1]. A fasting gastric
physical exam. The most common clinical pH <2 is seen in 99% of patients with ZES
presentation includes symptoms of abdomi- [4]. After discontinuation of proton-pump
nal pain, diarrhea, and reflux [2]. These inhibitor therapy for 2 weeks, gastric acid
symptoms are vague with a broad differential. secretion should measure <15 mEq/h. values
Zollinger-Ellison syndrome (ZES) is charac- greater than this suggest hypersecretion of
terized by gastrin-producing tumors stimulat- gastric acid [2].
ing marked gastric acid production, with D. When results are equivocal, a secretin stimu-
resultant refractory peptic ulcer disease [3]. lation test can be performed. On a fasting
Around 75% of gastrinomas are sporadic, patient, 2 IU/kg secretin is administered, and
while 25% are associated with multiple endo- gastrin levels are measured at 0, 2, 5, 10, 15,
crine neoplasia type 1 (MEN 1) [1]. 20, and 30 min. An increase in serum gastrin
B. Multiple tests are utilized to prove that a level of 200 ng/L or greater within 15 min is
patient not only has elevated serum gastrin considered a positive result for the diagnosis
levels but also hypersecretion of gastric acid. of gastrinoma [5].
The first test is a fasting serum gastrin level, E. Once the biochemical diagnosis is made,
which is usually markedly elevated in patients cross-sectional imaging with pancreas proto-
with a gastrinoma (10 times normal col computed tomography (CT) scan or mag-
or >1000 pg/mL) [4]. netic resonance imaging (MRI) is used to
localize the tumor and determine extent of
R. E. Simpson disease and presence of metastases. Ninety
Department of Surgery, Indiana University, percent of tumors will be within the gastri-
Indianapolis, IN, USA noma triangle, bordered by the cystic duct,
B. C. James (*) the junction of the second and third portion of
Department of Surgery, Harvard Medical School, the duodenum, and the junction of the neck
Boston, MA, USA and body of the pancreas. Most gastrinomas
Department of Surgery, Beth Israel Deaconess have somatostatin receptors, so somatostatin

470 R. E. Simpson and B. C. James
receptor scintigraphy may be useful for fur- Tumor debulking surgery may be considered
ther localization [1]. for locoregional and symptomatic control along
F. If imaging fails to localize the tumor, more with other methods including hepatic artery
invasive testing may be employed, including embolization, radiofrequency ablation, or selec-
endoscopic ultrasound or operative explora- tive internal radiation. Systemic therapies such
tion. A reported 24–30% of masses are located as octreotide, interferon, or chemotherapy may
by performing open duodenotomy [1]. also be employed [3].
G. All patients regardless of tumor burden
J. The approach for patients with MEN 1 is less
should be treated with a proton-pump inhibi- clear. Many of these patients have multiple, small
tor [1], with or without the addition of a hista- duodenal tumors [2]. Pancreaticoduodenectomy
mine-2 receptor blocker [3]. (PD) has been shown in some studies to
H. For sporadic, isolated gastrinoma, surgical
improve the length of disease-free period com-
resection is recommended [4]. pared to non-PD resections [6] and may have
I. Fifty percent of patients will have metastatic a higher rate of cure [7]. However, because of
disease at the time of diagnosis [1]. The most the morbidity associated with PD, some recom-
significant predictor of survival is the presence mend this treatment modality only for patients
of liver metastases. Twenty-year survival is near with larger tumors as patients with tumors less
95% with isolated disease versus 15% 10-year than 2 cm have excellent survival rates when
survival in the presence of metastases [3]. managed nonoperatively [4].
115 Management of Gastrinoma 471

A
Abdominal pain, diarrhea, reflux, peptic ulcer disease. Consider ZES and
association with MEN1.
B No Not ZES
Hypergastrinemia?
Yes
Gastric acid No
C Not ZES
hypersecretion?
Yes
Secretin Stimulation Test

No
D for confirmatory testing Not ZES
or equivocal results
Yes
E,F Localization:
Cross-sectional imaging, endoscopic ultrasound, exploration
MEN 1?
No Yes
Metastases -PPI
G,J
Yes -Possible resection
No
-PPI
-PPI; systemic therapies;
-Resection locoregional therapies; G,I
tumor Debulking
G,H
Algorithm 115.1
References Fordtran’s gastrointestinal and liver disease. 10th ed.

5. McPherson RA, Pincus MR. Chapter 22: Laboratory
1. Townsend CM Jr, Beauchamp RD, Evers BM, Mattox
diagnosis of gastrointestinal and pancreatic disor-
KL. Chapter 38: Endocrine pancreas. In: Sabiston
ders. In: Henry’s clinical diagnosis and management
textbook of surgery. 20th ed. Philadelphia: Elsevier;
by laboratory methods. St. Louis: Elsevier; 2017.
2017. p. 941–62.
p. 306–23.
6. Lopez CL, Falconi M, Waldmann J, Boninsegna L,
HM. Chapter 39: Multiple endocrine neoplasia.
Fendrich V, Goretzki PK, Langer P, Kann PH, Partelli
In: Williams textbook of endocrinology. 13th ed.
S, Bartsch DK. Partial pancreaticoduodenectomy
can provide cure for duodenal gastrinoma associated
3. Jameson JL, De Groot LJ, de Kretser DM, Giudice
with multiple endocrine neoplasia Type 1. Ann Surg.
LC, Grossman AB, Melmed S, Potts JT, Weir
2013;257(2):308–14.
GC. Chapter 148: Multiple endocrine neoplasia Type
7. Tonelli F, Fratini G, Falchetti A, Nesi G, Brandi
1. In: Endocrinology: adult and pediatric. 7th ed.
ML. Surgery for gastroenteropancreatic tumours in
Elsevier Saunders: Philadelphia; 2016. p. 2566–93.
multiple endocrine neoplasia type 1: review and per-
4. Feldman M, Friedman LS, Brandt LJ. Chapter
sonal experience. J Intern Med. 2005;257(1):38–49.
33: Neuroendocrine tumors. In: Sleisenger and
Management of Insulinoma
116
Algorithmic Approach B. Patients acutely hypoglycemic should be

treated with supportive care and glucose
A. The history and physical exam are key to administration [3].
diagnosis of insulinoma. The classic C. The gold standard for the diagnosis of an
“Whipple’s triad” of insulinoma includes insulinoma is a 72-h supervised fasting
symptoms of hypoglycemia (neuroglycope- period, during which blood glucose and
nic symptoms including confusion, seizures, symptoms are monitored every 6 h until blood
coma, tachycardia, palpitations, diaphoresis), glucose drops below 60, after which glucose,
concomitant blood glucose <60, and resolu- insulin, proinsulin, and c-peptide levels are
tion with glucose administration [1]. The checked at 1–2-h intervals [1]. Between 60%
patient may note a history of weight gain due and 75% of patients will become symptom-
to frequent meals to maintain blood glucose. atic within the first 24 h, and by 72 h, 85–95%
Timing of symptoms can help differentiate of patients will be symptomatic [1, 4].
insulinoma from other causes of hypoglyce- D. After obtaining a biochemical diagnosis of insu-
mia. Symptoms most commonly occur after linoma, cross-sectional imaging with dual-phase
periods of fasting, in the early morning, or CT or MRI should be used for localization,
during exercise. However, 25% of patients which has a sensitivity of 40–80% for tumors
will also have postprandial symptoms, so this over 1 cm [4]. Other less common modalities
history should be used in concert with addi- include functional imaging with radionuclide-
tional workup [2]. Though only 5% of insuli- labeled glucagon-like peptide (GLP-1), which
nomas are associated with multiple endocrine utilizes the high expression of GLP-1 receptors
neoplasia 1 (MEN1) syndrome, this diagnosis present within insulinomas [5].
should be considered [1]. E. Invasive localization may be necessary when
cross-sectional imaging fails to localize the
tumor. Endoscopic ultrasound (EUS) is highly
R. E. Simpson sensitive (93%) and specific (95%) and can
Department of Surgery, Indiana University, detect tumors as small as 2–3 mm [4, 5]. Calcium
arteriography involves cannulation of the arterial
B. C. James (*) supply via the celiac and superior mesenteric
Department of Surgery, Harvard Medical School,
Boston, MA, USA
branches to the head and neck of the pancreas
and splenic artery branches to the body and tail
Department of Surgery, Beth Israel Deaconess
of the pancreas. Calcium gluconate is then
e-mail: [email protected] infused while monitoring serum insulin levels in
the right hepatic vein. A two-fold increase in calcium channel blockers, and small frequent
insulin in response to calcium gluconate sug- meals to avoid hypoglycemia. Tumor debulking
gests the presence of insulinoma in this location, should be considered if 90% of tumor mass can
which can guide surgical resection [4]. be excised and the patient is a proper surgical
F. The mainstay of treatment is surgical resec- candidate [2]. A number of modalities exist for
tion. Enucleation should be attempted if pos- loco-regional control of metastases that are not
sible, as 90% of insulinomas are solitary, amenable to surgical resection and include, but
small, and benign. Intraoperative ultrasound are not limited to, selective chemoembolization
can help localize the lesion, as well as direct and radiofrequency or microwave ablation to
inspection and palpation [1]. Insulinomas control symptoms [5]. Prognosis is overall good
associated with MEN1 tend to be more for insulinomas after resection. Cure rate after
aggressive and multifocal, so full assessment complete excision of a sporadic insulinoma is
of the pancreas is imperative [1, 5]. 95% [4]. Median survival for metastatic insuli-
G. When metastatic disease is present, patients can noma is 5 years [1].
be managed non-operatively with diazoxide,
A History and physical exam:

“Whipple’s Triad,” neuroglycopenic symptoms, consider MEN 1
B Immediate treatment of hypoglycemia when discovered
72-h supervised fast:

C Assessment of symptoms, serum glucose, insulin/
proinsulin/c-peptide levels
Localized w/ cross-
D sectional imaging or
functional imaging? No
Invasive
Localization: EUS,
Yes E
calcium
arteriography
Metastatic
disease?
No Yes
-Medical therapies
Surgical resection
F -Small, frequent meals G
-Locoregional therapies
-Tumor Debulking
Algorithm 116.1
116 Management of Insulinoma 475
References Fordtran’s gastrointestinal and liver disease.

10th ed. Philadelphia: Elsevier Saunders; 2016.
p. 501–41.
4. Lennard TWJ. Chapter 5: Endocrine tumours of the
pancreas. In: Endocrine surgery. 5th ed. Edinburgh:
Elsevier; 2014. p. 125–46.
2017. p. 941–62.
HM. Chapter 38: Gastrointestinal hormones and gut
GC. Chapter 150: Neuroendocrine tumor syn-
endocrine tumors. In: Williams textbook of endo-
dromes. In: Endocrinology: adult and pediatric.
crinology. 13th ed. Philadelphia: Elsevier; 2016.
p. 101–1722.
p. 2606–14.
Management of Somatostatinoma
117
Algorithmic Approach the head of the pancreas, with the remainder

generally located in the periampullary duode-
A. Somatostatinoma is an extremely rare neuro- num or proximal small intestine [1, 3].
endocrine tumor [1]. The constellation of Pancreatic somatostatinomas tend to be more
signs and symptoms on presentation varies aggressive. Over 70% of patients will have
but may include hyperglycemia, cholelithia- metastatic disease at the time of diagnosis,
sis, steatorrhea, and hypochlorhydria [2]. and the 5-year survival is poor at less than
Many are discovered while evaluating for 50% [5]. Somatostatinomas localized to the
more nonspecific complaints, such as abdom- small intestine carry a much better prognosis,
inal pain, weight loss, or obstructive jaundice with 5-year survival over 80% [5]. These
related to mass effect [3]. individuals should undergo genetic testing for
B. On laboratory evaluation, patients may have neurofibromatosis 1, as 48% of duodenal
hyperglycemia secondary to the inhibited somatostatinomas have been found to be
release of insulin, hypochlorhydria, hyper- associated with this genetic disorder [6].
bilirubinemia, or evidence of malnutrition [2, D. Somatostatin analogs have been shown to

3]. Fasting somatostatin levels >160 pg/mL decrease fasting serum somatostatin levels
suggest the diagnosis of somatostatinoma [1]. and improve the severity of symptoms [3].
C. Cross-sectional imaging and endoscopic E. For patients with localized disease, surgical
ultrasound with fine-needle aspiration biopsy resection is recommended, as this is the only
are commonly used modalities to localize chance for cure [7].
tumors [3, 4]. Somatostatinomas tend to be F. Patients with metastatic disease may be
solitary, large tumors, most often measuring treated with systemic chemotherapy [4].
>2 cm in diameter. Sixty percent are found in Some have also supported surgical debulking
or hepatic artery embolization in the setting
R. E. Simpson of metastatic disease to help with symptom-
Department of Surgery, Indiana University, atic control [6, 8].
B. C. James (*)
Department of Surgery, Harvard Medical School,
Boston, MA, USA
Department of Surgery, Beth Israel Deaconess


A -Obstructive symptoms, jaundice, weight loss, abdominal pain
-Diabetes, cholelithiasis, steatorrhea
B -Hyperglycemia, hyperbilirubinemia, hypoalbuminemia, hypochlorhydria
-Fasting somatostatin level >160 pg/mL
Tumor localization, staging, and tissue diagnosis

C -Cross sectional imaging, endoscopic ultrasound with FNA biopsy
Metastatic
disease?
-Somatostatin analogues
-Surgical resection -Systemic chemotherapy
D, E D, F
-Somatostatin analogues -Surgical debulking/hepatic artery
embolization
Algorithm 117.1
117 Management of Somatostatinoma 479
References 4. Melmed S, Polonsky KS, Larsen PR, Kronenberg

endocrine tumors. In: Williams textbook of endo-
crinology. 13th ed. Philadelphia: Elsevier; 2016.
p. 1701–22.
5. Goldman L, Schafer AI. Chapter 195: Pancreatic neu-
2017. p. 941–62.
roendocrine tumors. In: Goldman-cecil medicine.
25th ed. Philadelphia: Elsevier; 2016. p. 1334–9.
6. Anderson CW, Bennett JJ. Clinical presentation and
GC. Chapter 42: Hyperglycemia secondary to non-
diagnosis of pancreatic neuroendocrine tumors. Surg
diabetic conditions and therapies. In: Endocrinology:
Oncol Clin N Am. 2016;25(2):363–74.
adult and pediatric. 7th ed. Philadelphia: Elsevier;
7. Yeo CJ. Chapter 94: Neuroendocrine tumors of
2016. p. 737–51.
the pancreas. In: Shackelford’s surgery of the ali-
mentary tract. 7th ed. Philadelphia: Elsevier; 2013.
p. 1206–16.
Fordtran’s gastrointestinal and liver disease. 10th ed.
8. Azimuddin K, Chamberlain RS. The surgical man-
agement of pancreatic neuroendocrine tumors. Surg
Clin N Am. 2001;81(3):511–25.
Management of VIPoma
118
Algorithmic Approach levels >200 pg/mL are considered diagnostic,

though individuals with VIPoma tend to have
A. Tumors that secrete vasoactive intestinal pep- levels >1000 pg/mL [5]. In the acute setting, flu-
tide (VIP), or “VIPomas,” are exceedingly ids and electrolytes are replaced [2].
rare with an incidence of 0.01/1,000,000 C. Tumor localization begins with cross-sectional
person-years and tend to be located in the imaging with CT or MRI, proceeding to
body and tail of the pancreas [1, 2]. Diagnosis octreotide scan if these modalities fail to iden-
starts with a careful history and physical tify the tumor. More invasive means including
examination. The most common symptom is endoscopic ultrasound are also commonly
high volume (>1 L/day), watery diarrhea that employed [5]. The use of gallium- labeled
persists despite fasting [3]. Patients may also radioligands to the somatostatin receptors
experience a flushing rash, hypotension, and commonly found on VIPomas has led to the
dehydration [2]. Other common causes of development of other functional studies
secretory diarrhea should be considered and such as the Gadolinium-labeled 1,4,7,10-
ruled out. Most VIPomas are sporadic, but tetraazacyclododecane-1,4,7, 10-tetraacetic
around 5% are associated with multiple endo- acid octreotide Ga-DOTANOC scan that has a
crine neoplasia 1 (MEN1) [4]. sensitivity of 78.3% and specificity of 92.5%
B. The classic biochemical finding is hypochlore- for localizing primary gastrointestinal and
mic, hypokalemic metabolic acidosis [2]. pancreatic neuroendocrine tumors, and even
Clinically patients present with watery diarrhea, better sensitivity and specificity for detecting
hypokalemia and achlorhydria. This is referred metastatic disease approaching 100% [6].
to as Watery diarrhea with hypokalemia and VIPomas tend to be large (2 cm or greater),
achlorhydria (WDHA) syndrome. Fasting VIP solitary, and located in the body or tail of the
pancreas [5]. In rare instances they are found
R. E. Simpson in the adrenal gland or sympathetic chain [2].
Department of Surgery, Indiana University, D. Medical treatment to relieve the symptoms of
Indianapolis, IN, USA VIPoma includes somatostatin inhibitors,
B. C. James (*) which inhibit the release and circulating lev-
Department of Surgery, Harvard Medical School, els of VIP and also directly decrease diarrhea
Boston, MA, USA [5]. Somatostatin inhibits motilin and slows
Department of Surgery, Beth Israel Deaconess intestinal motility [7]. Through these various
Medical Center, Boston, MA, USA mechanisms, long-acting somatostatin ana-

logs have been found to control symptoms in sis [2]. In these cases, somatostatin analogs or
78–100% of patients [3]. systemic chemotherapy is the mainstay of
E. For patients with isolated disease, surgical treatment. Surgical resection is rarely cura-
resection is recommended. Of all patients that tive but may help relieve symptoms [8]. Some
undergo surgical resection, surgical cure is consider cytoreductive surgery if at least 90%
achieved in approximately 30% [3]. of disease burden can be resected [3].
F. Around 75% of patients have regional lymph However, others do not support tumor deb-
node or liver metastases at the time of diagno- ulking surgery [4].

A
-Watery diarrhea, flushing, dehydration
B -Hypokalemia, hypochloremia, metabolic acidosis.
-VIP level >200 pg/mL
Tumor localization:
C
-Cross-sectional imaging (CT/MRI), octreotide scan, endoscopic ultrasound
Metastatic
disease?
No Yes
-Somatostatin analogues
-Somatostatin analogues -Systemic chemotherapy
D,E -Surgical resection -Consideration for cytoreductive D,F
therapy
Algorithm 118.1
118 Management of VIPoma 483
References 5. Jameson JL, De Groot LJ, de Kretser DM, Guidice

GC. Chapter 150: Neuroendocrine tumor syn-
1. James BC, Aschebrook-Kilfoy B, Cipriani N,
dromes. In: Endocrinology: adult and pediatric.
Kaplan EL, Angelos P, Grogan RH. The incidence
and survival of rare cancers of the thyroid, para-
p. 2606–14.
thyroid, adrenal, and pancreas. Ann Surg Oncol.
6. Maxwell JE, O’Dorisio TM, Howe JR. Biochemical
2016;23(2):424–33.
diagnosis and preoperative imaging of gastroentero-
pancreatic neuroendocrine tumors. Surg Oncol Clin
N Am. 2016;25(1):171–94.
endocrine tumors. In: Williams textbook of endocrinol-
7. O’Dorisio TM, Gaginella TS, Mekhjian HS, Rao
ogy. 13th ed. Philadelphia: Elsevier; 2016. p. 1701–22.
B, O’Dorisio MS. Somatostatin and analogues
3. Feldman M, Friedman LS, Brandt LJ. Chapter 33:
in the treatment of VIPoma. Ann N Y Acad Sci.
Neuroendocrine tumors. In: Sleisenger and Fordtran’s
1988;527:528–35.
gastrointestinal and liver disease. Philadelphia:
8. Niederhuber JE, Armitage JO, Doroshow JH, Kastan
Elsevier; 2016. p. 501–41.
MB, Tepper JE. Chapter 71: Cancer of the endo-
crine system. In: Abeloff’s clinical oncology. 5th ed.
2017. p. 941–62.
Part XVI
Pediatric
Congenital Diaphragmatic Hernia
119
Christopher J. McLaughlin, Rachel E. Hanke,
Algorithmic Approach The diaphragm defect disrupts the mechanics

of respiration.
A. Congenital diaphragmatic hernia (CDH) is a C. For infants born with confirmed or suspected
common congenital abnormality (1 in 2500 CDH, initial stabilization includes the
live births) [1]. With advances in prenatal following:
ultrasound, the majority of CDH is diagnosed (i) Intubation and gentle, non-injurious
prenatally, with mean detection time of ventilation (note: bag-valve mask venti-
24 weeks. Delivery should be planned at an lation is avoided due to risk of gastric
experienced tertiary care center to optimize distension)
outcome [2]. Prenatal care of the mother (ii) Nasogastric (NG) tube insertion for gas-
should be optimized to prolong gestation to tric decompression
decrease mortality and the need for extracor- (iii) Arterial and venous cannulation, typi-
poreal membrane oxygenation (ECMO) [3]. cally via the umbilicus
CDH is typically a posterolateral diaphragm (iv) Monitoring of pre-ductal (right radial)
defect (Bochdalek hernia), 80% occurring on and post-ductal (lower extremity) oxy-
the left, 15% on the right, and 5% bilateral. gen saturations, temperature, glucose
B. CDH is usually diagnosed before or at birth. level, volume status/urine output
Two to three percent of CDH is diagnosed (v) Pharmacologic support of cardiac func-
after birth, occasionally long after (see Chap. tion and treatment of pulmonary
121, Other Diaphragmatic Hernias) [4]. hypertension
Symptoms include respiratory distress (espe- D. After stabilization, diagnosis can be con-

cially around feedings) and feeding difficul- firmed through identification of abdominal
ties including intestinal obstruction. The contents (liver, bowel, stomach) above the
abdomen may appear scaphoid. Respiratory hemidiaphragm on a plain chest radiograph,
distress, cyanosis, sternal retractions, dis- with a gastric bubble and/or distal nasogastric
placed heart sounds, and absent breath sounds tube tip found in the chest being pathogno-
on the affected side provide additional clues. monic. Subsequently, echocardiography, cra-
nial ultrasounds, and renal ultrasounds should
be performed to assess for concurrent devel-
C. J. McLaughlin (*) · R. E. Hanke · R. E. Cilley opmental abnormalities and to characterize
Department of Surgery, Penn State Milton S. Hershey the extent of disease. Echocardiography

488 C. J. McLaughlin et al.
should further characterize right ventricular [6]. During ECMO, treatment is focused on
function and pulmonary artery pressures. improving pulmonary hypertension and
E. Following these diagnostic steps, ongoing avoiding further lung injury.
physiologic optimization should occur. Goals G. A recent meta-analysis shows all infants with
of ventilatory support should be to avoid lung CDH benefit from definitive surgical repair
injury while maintaining a pre-ductal [7]. For infants not requiring ECMO, the dia-
PO2 >60 mm Hg (with corresponding pre- phragm defect should be repaired after stabi-
ductal SaO2 90–95%) and tolerating relative lization and reversal of pulmonary
hypercapnia. High-frequency oscillatory ven- hypertension. For infants requiring ECMO,
tilation and nitric oxide may be useful. surgical repair after decannulation decreases
Pulmonary hypoplasia and pulmonary hyper- the risk of hemorrhagic complications.
tension ultimately determine the severity of Laparoscopic, thoracoscopic, or open
respiratory failure and outcome [5]. approach can be used with equal effective-
F. Respiratory failure may progress and become ness of repair, though open repair is recog-
life-threatening as a result of uncorrectable nized as most effective for prevention of
pulmonary hypertension. ECMO is an appro- recurrence [8]. Biologic and synthetic patches
priate treatment unless pulmonary hypoplasia may be used if native tissues are inadequate.
incompatible with life is present. Predictive Recurrence is more likely with prosthetic
indices assist with these difficult decisions patches [9].
119 Congenital Diaphragmatic Hernia 489
A B
Prenatal detection of CDH: Symptoms of CDH at birth:

-Delivery near term, experienced CDH center Respiratory distress, scaphoid abdomen
Delivery and initial management:

C Intubation, NG tube, pre and post-ductal sat
monitoring, umbilical cannulation
Confirm CDH: CXR, ECHO, U/S Alternative

D Determine extent of disease or concurrent abnormalities diagnoses
Monitoring and goals

E Pre-ductal PaO2 >60, non-injurious ventilation
ECMO
required?
Yes No
F
Monitor pulmonary hypertension,

optimize cardiac function, Ongoing resuscitation and
decannulate prior to repair pre-operative optimization
Operative repair: native tissue

G superior to prosthetic patch
Algorithm 119.1
490 C. J. McLaughlin et al.
References 6. Le LD, Keswani SG, Biesiada J, Lim FY, Kingma PS,

Haberman BE, Frischer J, Habli M, Crombleholme
TM. The congenital diaphragmatic hernia compos-
1. Zani A, Zani-Ruttenstock E, Pierro A. Advances
ite prognostic index correlates with survival in left-
in the surgical approach to congenital dia-
sided congenital diaphragmatic hernia. J Pediatr Surg.
phragmatic hernia. Semin Fetal Neonatal Med.
2012;47(1):57–62.
2014;19(6):364–9.
7. Harting MT, Hollinger L, Tsao K, Putnam LR, Wilson
2. Nasr A, Langer JC. Influence of location of delivery
JM, Hirschl RB, Skarsgard ED, Tibboel D, Brindle
on outcome in neonates with congenital diaphrag-
ME, Lally PA, Miller CC. Aggressive surgical man-
matic hernia. J Pediatr Surg. 2011;46(5):814–6.
agement of congenital diaphragmatic hernia: worth
3. Odibo AO, Najaf T, Vachharajani A, Warner B,
the effort? A multicenter, prospective, cohort study.
Mathur A, Warner BW. Predictors of the need for
Ann Surg. 2018;267(5):977–82.
extracorporeal membrane oxygenation and survival
8. Putnam LR, Tsao K, Lally KP, Blakely ML,
in congenital diaphragmatic hernia: a center’s 10-year
Jancelewicz T, Lally PA, Harting MT, Group
experience. Prenat Diagn. 2010;30(6):518–21.
CD. Minimally invasive vs open congenital diaphrag-
4. Kitano Y, Lally KP, Lally PA. Late-presenting
matic hernia repair: is there a superior approach? J
congenital diaphragmatic hernia. J Pediatr Surg.
Am Coll Surg. 2017;224(4):416–22.
2005;40(12):1839–43.
9. Moss RL, Chen CM, Harrison MR. Prosthetic
5. Dillon PW, Cilley RE, Mauger D, Zachary C, Meier
patch durability in congenital diaphragmatic her-
A. The relationship of pulmonary artery pressure and
nia: a long-term follow-up study. J Pediatr Surg.
survival in congenital diaphragmatic hernia. J Pediatr
2001;36(1):152–4.
Surg. 2004;39(3):307–12.
Tracheoesophageal Fistula
120
Rachel E. Hanke, Morgan K. Moroi,
Algorithmic Approach the abdomen may become distended as air

fills the stomach. Only with high clinical
A. Tracheoesophageal fistula (TEF) and esopha- suspicion will the more rare, isolated TEF
geal atresia (EA) can occur separately or in (type E) be diagnosed after patients pres-
several combinations with an incidence of ent with choking when eating or unex-
1 in 3000 live births [1]. There are five EA/ plained cyanotic episodes [3].
TEF anomalies: B. If there is suspicion for EA/TEF in a new-
• Type A: EA without TEF (pure esophageal born, diagnosis often starts by placing an
atresia). esophageal tube. In EA, a chest radiograph
• Type B: EA with proximal TEF. will show the tube coiled in the blind-ending
• Type C: EA with distal TEF (most esophageal pouch within the chest. If there is
common). stomach/intestinal gas on radiograph, this
• Type D: EA with proximal and distal TEF. confirms distal TEF. Preoperative esophageal
• Type E: TEF without esophageal atresia pouch studies may also be used to determine
(commonly called “H” or “N” fistula). EA/TEF type and length of esophageal
The most common is type C, found in pouch, an important consideration for even-
about 85% of patients. Patients with EA tual surgical repair [1].
may be detected prenatally, with ultra- C. Initially, management should be focused on
sound evidence of polyhydramnios and a decreasing risk of aspiration and respiratory
small or absent stomach bubble [2]. All issues by maintaining the esophageal drain-
except type E present shortly after birth age catheter on suction. Perioperative antibi-
with some combination of excessive drool- otics are indicated. Long antibiotic courses
ing, choking, regurgitation, and/or respira- are reserved for the treatment of specific
tory distress [3]. If distal TEF is present, infection or infection risk [4]. Positive-
pressure ventilation is avoided, if possible,
and used with THE lowest possible pressures
if needed. Vascular access is obtained using
umbilical or peripheral sites [4].
D. A thorough physical exam may reveal associ-
R. E. Hanke · M. K. Moroi · R. E. Cilley (*) ated anomalies. Echocardiography evaluates
Department of Surgery, Penn State Milton S. Hershey structural heart disease, which occurs in
Medical Center, Hershey, PA, USA about 35% of patients. The position of the

492 R. E. Hanke et al.
aortic arch may influence the surgical rarely, if ever, needed [3]. Repair within the
approach [2]. Studies have shown that for first few days of life allows for full evaluation
patients with EA/TEF, two-thirds had at least of the patient. Thoracoscopic repair and open
one other anomaly [1]. A renal ultrasound thoracotomy (using either trans-pleural or
and genetic testing identify other anomalies. extra-pleural approaches) are used for TEF
EA/TEFs are seen in or associated with many division/repair and EA repair, with the choice
syndromes, including VACTERL and based on surgeon preference [2, 3].
CHARGE [3]. E. Post-operatively, antibiotics are discontin-
Surgical division and repair of TEF with ued [4]. Parenteral nutrition, gastrostomy
repair of the esophagus is the mainstay of feeds, or trans-anastomotic feeding tubes
treatment. In patients with severe respiratory are used to support nutrition [3]. An esopha-
distress, urgent thoracotomy with clip liga- gram is performed post-operatively to eval-
tion of the TEF may be life-saving. Patients uate the integrity of the esophageal repair.
with progressive gastric distention may Recent data indicate that the study may be
require emergent trans-abdominal needle performed safely as early as 5 days after
decompression followed by urgent gastros- surgery [4]. Most leaks, found in up to 23%
tomy. Primary one-stage repair is preferred. of patients, are contained and will heal
Gastrostomy is rarely required. However, if without further surgery [1]. Narrowing and
definitive correction is delayed due to serious strictures occur in up to 40% of patients and
concurrent disease such as congenital heart usually respond to dilation [1].
disease or extreme prematurity, a gastros- Gastroesophageal reflux is common. Acid
tomy may be performed. Although not per- suppression previously was standard practice
formed by all surgeons, direct visualization in all patients; however, recent data indicate
with diagnostic bronchoscopy can evaluate that acid suppression does not reduce stric-
for multiple fistulas [1, 3]. In pure atresia ture rate [4]. Antireflux surgery is avoided if
(type A) and when the esophageal gap is too possible. All patients have some degree of
long for primary repair, esophageal lengthen- esophageal dysmotility. Recurrent TEF is
ing and staged repairs are performed [3, 5]. rare and requires intervention (“re-do sur-
Many pediatric surgeons have abandoned gery” or fibrin glue plugging). Airway insta-
esophageal replacement. Colon interposition, bility/tracheomalacia usually responds to
gastric “pull-up,” or reversed gastric tubes are supportive care [3].
120 Tracheoesophageal Fistula 493
A Newborn noted to have excessive salivation

and regurgitation/choking after feeding
Perform thorough physical exam, place esophageal tube, obtain

PA/lateral CXR
B CXR shows coiling of tube in chest
C Air in
Yes No
stomach?
Esophageal atresia with TEF Pure esophageal atresia
D Obtain echocardiogram Work up per EA/TEF. Plan staged/delayed

repair using lengthening techniques *
E Severe respiratory No Further evaluation:

distress with gastric renal ultrasound and genetic testing
distension (consider
gastric decompression/
gastrostomy
first)
Thoracoscopy vs thoracotomy to
divide/repair of TEF
Yes
Urgent thoracotomy and

ligation/clipping of TEF
Yes Long gap EA?
Repair after clinical improvement No
Primary repair
* Esophageal lengthening and/or
of EA
staged repair of EA
F Post-operative esophagram and surveillance
Algorithm 120.1
References 4. Lal DR, Gadepalli SK, Downard CD, Ostlie DJ,

Minneci PC, Swedler RM, Chelius TH, Cassidy L,
Rapp CT, Billmire D, Bruch S, Burns RC, Deans
1. Lal DR, Gadepalli SK, Downard CD, Ostlie DJ,
KJ, Fallat ME, Fraser JD, Grabowski J, Hebel F,
Minneci PC, Swedler RM, Chelius T, Cassidy L,
Helmrath MA, Hirschl RB, Kabre R, Kohler J,
Rapp CT, Deans KJ, Fallat ME, Finnell ME, Helmrath
Landman MP, Leys CM, Mak GZ, Raque J, Rymeski
MA, Hirschl RB, Kabre RS, Leys CM, Mak G, Raque
B, Saito JM, St. Peter SD, von Allmen D, Warner
J, Rescorla FJ, Saito JM, St. Peter SD, von Allmen D,
BW, Sato TT. Challenging surgical dogma in the
Warner BW, Sato TT. Perioperative management and
management of proximal esophageal atresia with
outcomes of esophageal atresia and tracheoesopha-
distal Tracheoesophageal fistula: outcomes from the
geal fistula. J Pediatr Surg. 2017;52(8):1245–51.
Midwest Pediatric Surgery Consortium. J Pediatr
2. Slater BJ, Rothenberg SS. Tracheoesophageal fistula.
Surg. 2018;53(7):1267–72.
Semin Pediatr Surg. 2016;25(3):176–8.
5. Foker JE, Krosch TCK, Catton K, Munro F, Khan
3. Harmon C, Coran A. Pediatric surgery. In: Coran AG,
KM. Long-gap esophageal atresia treated by growth
Adzick NS, Krummel TM, Laberge JM, Shamberger
induction: the biological potential and early follow-up
RC, Caldamone AA, editors. Congenital anomalies of
results. Semin Pediatr Surg. 2009;18:23–9.
the esophagus. 7th ed. Philadelphia: Saunders; 2012.
Other Diaphragmatic Hernias:
Late-Presenting Bochdalek Hernia, 121
Morgagni Hernia, and Giant Hiatal
Hernia of Infancy
Morgan K. Moroi, Christopher J. McLaughlin,
Algorithmic Approach B. These unusual diaphragmatic hernias are

associated with a wide spectrum of symptoms
A. Congenital diaphragmatic hernias (CDH) are ranging from mild respiratory problems to
classically posterolateral diaphragm defects intestinal obstruction. The rarity of late-
(Bochdalek hernia) that present in early presenting diaphragmatic hernias and their
infancy [1]. CDH clinical algorithms are diverse clinical presentation can delay diag-
found in Chap. 119, Congenital Diaphragmatic nosis. Diaphragm hernia should be consid-
Hernia. A smaller number of CDH (10–20%) ered in the differential diagnosis of a child
patients may present later in life, even as with dysphagia, decreased appetite, vomiting,
adults [1, 2]. These late-presenting patients constipation, abdominal pain, recurrent respi-
most often have a posterolateral defect with a ratory infections, dyspnea, cough, cyanosis,
well-formed hernia sac containing viscera. or tachypnea [1–3]. Patients may also be
Morgagni hernias, on the other hand, are cen- asymptomatic. There are rarely clues on the
trally located anterior diaphragm defects with physical exam, such as abnormal breath
similar contents [3]. Rarely, an infant with sounds or a scaphoid abdomen. Diagnosis is
feeding or respiratory symptoms will be most often suggested by an abnormal chest
found to have an “intrathoracic stomach,” radiograph.
consistent with giant hiatal hernia, where a C. The initial step in the evaluation is to obtain
significant portion of the stomach has herni- an anterior-posterior and lateral chest radio-
ated through a large opening in the esopha- graph. Gastrointestinal contrast studies are
geal hiatus. Regardless of location, also diagnostic. Occasionally, body imaging
late-presenting patients do not have pulmo- may be necessary to understand the anatomy.
nary hypoplasia or pulmonary hypertension Presence of abdominal contents above the
[1, 2]. diaphragm confirms diagnosis. Other con-
genital abnormalities may be present and
should be evaluated [1–3]. Echocardiography
should be considered to evaluate for struc-
tural heart disease.
D. Intestinal obstruction, caused by incarcerated
M. K. Moroi · C. J. McLaughlin · R. E. Cilley (*) intestine or gastric volvulus, may require
Department of Surgery, Penn State Milton S. Hershey emergency operation. For all of these dia-
Medical Center, Hershey, PA, USA phragmatic defects, correction is performed

496 M. K. Moroi et al.
promptly. Late-presenting CDH may be practice is not well supported by outcome

repaired traditionally via laparotomy or tho- studies [3, 4].
racotomy. Thoracoscopic repair is gaining E. Post-operative imaging requirements are lim-
popularity [1, 2]. For Morgagni hernias, lapa- ited to chest radiographs for most procedures.
roscopic repair is the method of choice [3]. Gastrointestinal contrast studies may help
For giant hiatal hernias of infancy with an assess for recurrence. Hernia recurrence rates
intrathoracic stomach, open or laparoscopic are low, and prognosis for patients with late-
methods may be used. An antireflux proce- presenting diaphragmatic hernia is excellent
dure and a gastrostomy are also typically when compared to those who present as neo-
included after the reduction of the stomach, nates [1, 2]. Postoperative complications are
resection of the hernia sac, and closure of the extremely rare after Morgagni hernia repair
hiatal defect [4]. In all of these diaphragmatic [3]. Giant hiatal hernia patients do well,
hernia defects, a hernia sac will be encoun- although they may experience typical prob-
tered. Resection of the hernia sac likely lems associated with antireflux surgery and
reduces recurrence, although this customary gastrostomies.
121 Other Diaphragmatic Hernias: Late-Presenting Bochdalek Hernia, Morgagni Hernia, and Giant Hiatal… 497
A 15 month-old, recurrent respiratory infections, respiratory distress, vomiting
Obtain vital signs and blood work.

Perform a thorough physical examination.
B 37.1°C, HR 120, RR 30, BP 90/60, WBC 11,000

Possible decreased breath sounds at left lung base
Presentation concerning for late-presenting diaphragmatic hernia.

C Obtain a chest radiograph.
Yes Diaphragmatic
No
hernia identified?
Intestinal Continue workup for

incarceration or alternative diagnoses
gastric volvulus?
Yes No
Imaging for preoperative planning (GI contrast, possibly CT) and

evaluation of other congenital abnormalities (consider
echocardiography)
Emergent operative D Prompt surgical repair

repair
E
Repair via thoracic or abdominal

approach, open or video-endoscopic Post-operative imaging
procedure and surveillance
Algorithm 121.1
498 M. K. Moroi et al.
References 3. Golden J, Barry WE, Jang G, Nguyen N, Bliss

D. Pediatric Morgagni diaphragmatic hernia: a
descriptive study. Pediatr Surg Int. 2017;33(7):771–5.
1. Stolar C, Dillon P. Pediatric surgery. In: Coran AG,
4. Kohn GP, Price RR, Demeester SR, Zehetner J,
Adzick NS, Krummel TM, Laberge JM, Shamberger
Muensterer OJ, Awad Z, Mittal SK, Richardson
RC, Caldamone AA, editors. Congenital diaphrag-
WS, Stefanidis D, Fanelli RD. Guidelines for
matic hernia and eventration. 7th ed. Philadelphia:
the management of hiatal hernia. Surg Endosc.
Saunders; 2012.
2013;27(12):4409–28.
2. Kitano Y, Lally KP, Lally PA. Late-presenting
congenital diaphragmatic hernia. J Pediatr Surg.
2005;40(12):1839–43.
Duodenal Obstruction
in Newborns 122
and Todd A. Ponsky
Algorithmic Approach diagnose a duodenal atresia and mandates sur-

gical intervention [3]. Presence of air distal to
A. Prenatal diagnosis of duodenal atresia is usu- the ligament of Treitz could mean malrotation
ally achieved in more than 70% of the cases with or without volvulus, so urgent surgery is
[1] where antenatal ultrasound can show required. Duodenal web can also present with
polyhydramnios and a distended stomach [2]. duodenal obstruction but with presence of distal
B. Signs and symptoms: Bile-stained emesis air in the stable newborn [3]. Pneumoperitoneum
(occurs in 90% of the infants) without abdom- on the abdominal X-ray also demands urgent
inal distension and failure to pass meconium surgery. Echocardiogram is the most important
are the main presenting symptoms [3]. Pre- test that should be conducted preoperatively.
ampullary duodenal atresia, which occurs in Other work-up, such as renal and spinal ultra-
10% of patients, can be differentiated from sounds, should also be performed during the
the other causes of non-bilious vomiting hospitalization to diagnose potential associated
using the patient history (i.e., prenatal, age, renal or spinal abnormalities [3].
and gender of the patient) and using abdomi- E. Treatment: Laparoscopic or open duodeno-
nal X-ray, by placing a nasogastric (NG) tube duodenostomy is the most common surgical
and injecting about 50 ml of air into the stom- approach to repair this anomaly, and this
ach which can work as a contrast medium and should be carefully performed to avoid injury
makes the double-bubble sign clearer [3]. to the ampulla of Vater in the second portion
C. Initial resuscitation includes starting intrave- of the duodenum. The same procedure or web
nous (IV) fluids, keeping the patient NPO, excision can be used to treat a duodenal web.
and inserting an NG tube to correct the elec- This same surgery may also be used to bypass
trolyte abnormalities and fluid balance and to an annular pancreas [3].
stop vomiting. Workup for associated anoma- F. Postoperatively: Consider starting feeds when
lies, such as cardiac defects, is of utmost the gastric drainage is decreasing in amount,
importance during the preoperative time [2]. becoming lighter in color, when the patient has
D. Abdominal plain X-ray should first be per- return of bowel function or after a leak negative
formed, and a double-bubble sign with no air upper GI contrast study postoperative day 5 [3].
distal to the ligament of Treitz is sufficient to Potential long-term complications to evaluate
for are intestinal obstruction, gastroesophageal
A. Y. Lamoshi · S. Abdulhai · T. A. Ponsky (*) reflux, peptic ulcer, megaduodenum, duodeno-
Division of Pediatric Surgery, Akron Children’s gastric reflux, gastritis, delayed gastric empty-
ing, and blind-loop syndrome [3].
500 A. Y. Lamoshi et al.
Antenatal
Diagnosis
made?
A
Yes
No
B Will present with early bilious vomiting

Open or Lap
Duodeno-duodenostomy
Obtain V/S and physical exam
C NPO, NG tube, & IVF
D KUB (AXR)
Free air
Double-bubble with No free air
Urgent surgery
With air distal to the
ligament of Treitz
No air distal to the
ligament of Treitz
Duodenal atresia
Urgent surgery for
possible malrotation
+/- volvulus
Echo
Surgery (Duodeno-duodenostomy)
E Duodeno-duodenostomy
or web-excision) if no then likely
duodenal web
F Follow-up care
Algorithm 122.1
ECR 2016 / C-0486 / Final Year Radiography Students’

References Perception of Stressors in Clinical Placement. -
EPOS™, European Congress of Radiology 2016. 2013.
1. Burjonrappa S, Crete E, Bouchard S. Comparative posterng.netkey.at/esr/viewing/index.php?module=
outcomes in intestinal atresia: a clinical outcome viewing_poster&doi=10.1594/ecr2013/C-1059.
and pathophysiology analysis. Pediatr Surg Int. 3. Ashcraft KW, et al. Ashcraft’s pediatric surgery.
2011;27(4):437–42. London/New York: Saunders; 2014.
2. Elsayed M, et al. “Abnormal Neonatal Bowel Gas
Patterns on Plain Radiography - Back to the Basics”.
Small Intestinal Atresia
123
Abdulraouf Y. Lamoshi, Sophia Abdulhai, and
Todd A. Ponsky
Algorithmic Approach meconium ileus, meconium plug syndrome,

or colonic atresia [3]. A meconium pseudo-
A. Antenatal diagnosis can be established in
cyst may occur in the case of intrauterine
41% of the patients [1], where polyhydram- intestinal perforation, and intraluminal calci-
nios and dilated bowels together are highly fications of meconium have also been docu-
suggestive of the diagnosis [2]. Postnatally, mented with intestinal atresia [3].
bilious vomiting, upper abdominal disten- D. Treatment: The operative technique is based
sion, and failure to pass meconium are the on the site, type, the intraoperative findings,
main presenting signs and symptoms. Rarely, and the length of the intestine. End-to-end
the patient will pass normal meconium, and anastomosis with tapering of the proximal
about 10% of patients present with meconium bowel, when needed, and resection of the
peritonitis, which requires urgent surgical dilated and hypertrophied proximal bowel is
intervention [3]. the most common approach. Checking for
B. Initial resuscitative measures include starting another atresia by passing a small catheter
IV fluids, inserting a nasogastric (NG) tube, and instilling saline is of utmost importance
and keeping the patient NPO to correct hypo- before performing the anastomosis [2].
volemia, electrolyte abnormalities, and mini- E. Follow-up care should include watching the
mize vomiting. Antibiotics are needed when amount and color of NG tube output and
there is suspicion for intestinal perforation. bowel function, which should be used to
C. Diagnosis: Abdominal plain X-ray is the ini- decide when to initiate feeds [3]. Clear fluids
tial imaging study. Multiple dilated loops of or breast milk is usually introduced before
bowel and the absence of distal air are highly any formula. After discharge, follow-up care
suggestive of small bowel atresia, and most is a crucial measure, especially when there is
pediatric surgeons would take patients to the loss of a considerable amount of small bowel
operating room (OR) based on these findings length. The nutritional status and signs of
alone. Presence of distal air requires a con- intestinal obstructions should always be eval-
trast enema, which can diagnose other poten- uated [3]. Temporary gastrointestinal dys-
tial causes such as Hirschsprung disease, function is a common observation in these
newborns [3].
A. Y. Lamoshi · S. Abdulhai · T. A. Ponsky (*)

Division of Pediatric Surgery, Akron Children’s

502 A. Y. Lamoshi et al.
A Antenatal
Diagnosis
made? Yes
No
Will present with early bilious vomiting Resection and

anastomosis
Obtain V/S and physical exam
Peritonitis: Yes Peritonitis: No
Urgent Surgery
B NPO, NGT, and IVF
C KUB (AXR)
Dilated bowels
Double-bubble (consider
other pathologies)
Distal loops of bowel distal

to the ligament of Treitz
Dilated loops of bowel proximal
to the ligament of Treitz
Contrast Enema
Proximal small bowel atresia

Yes Micro-colon
Distal small bowel with no
atresia/other surgical meconium
pathologies
Surgery
D
No
Follow-up care Consider other pathologies

E
Algorithm 123.1
123 Small Intestinal Atresia 503
References 2. Adams SD, Stanton MP. Malrotation and intestinal

atresias. Early Human Dev. 2014;90(12):921–5.
3. Holcomb GW, Murphy JD, Ostlie DJ. Ashcraft’s pedi-
1. Burjonrappa S, Crete E, Bouchard S. Comparative
atric surgery e-book. Philadelphia: Elsevier Health
outcomes in intestinal atresia: a clinical outcome
Sciences; 2014.
and pathophysiology analysis. Pediatr Surg Int.
2011;27(4):437–42.
Management of Malrotation
124
Sophia Abdulhai, Abdulraouf Y. Lamoshi,
and Todd A. Ponsky
Algorithmic Approach to evaluate for proximal or distal bowel

obstructions, free air, or pneumatosis [3]. If
A. Malrotation, which has an incidence of about there are no significant abnormalities seen on
1 in 500 patients, classically presents as a the AXR that require emergent exploration
full-term infant with bilious emesis [1]. Up to and the patient is clinically stable, an upper
89% of patients present with symptoms by gastrointestinal (GI) contrast study should be
the age of 1 year, and full-term infants are performed next.
more likely to have malrotation compared to (a) Free air/pneumatosis: Ischemic bowel
preterm children. Bilious emesis presents in may occur from a malrotation with mid-
48% of patients, followed by abdominal dis- gut volvulus. Pneumatosis, portal venous
tention (21%) [2]. Any pediatric patient that gas, and/or free air may be seen in these
presents with bilious emesis should be patients, and they should be taken to the
assumed to have midgut volvulus until proven operating room (OR) for immediate
otherwise. Initial evaluation of these patients exploration. If malrotation is identified,
should include vital signs and abdominal surgery would include counterclockwise
examination. detorsion of the volvulus and performing
B. If there is evidence of peritonitis or hemody- a Ladd’s procedure. Necrotizing entero-
namic instability, then intestinal ischemia colitis should also be considered with
from malrotation with midgut volvulus these X-ray findings.
should be considered and NG tube place- (b) Proximal obstruction: Although duode-
ment, fluid resuscitation, broad-spectrum nal atresia and duodenal web commonly
antibiotics, and immediate operative explora- present as a double-bubble sign on AXR,
tion should be performed. If the patient has a this sign cannot be used to definitively
benign abdominal examination, then further rule out malrotation, since Ladd’s bands
work-up with an abdominal X-ray (AXR) or midgut volvulus can also present this
should be performed. way. So any patient with proximal bowel
C. An AXR has limited utility in diagnosing obstruction and evidence of air past the
malrotation, but it should be performed first duodenum should go to the OR emer-
gently. Additionally, duodenal atresia and
web are the most common congenital
S. Abdulhai · A. Y. Lamoshi · T. A. Ponsky (*) anomaly associated with malrotation,
Division of Pediatric Surgery, Akron Children’s occurring in up to 11% of patients [2].

506 S. Abdulhai et al.
(c) Distal obstruction: Distal obstruction (b) Equivocal: If the results are equivocal,
may occur with Hirschsprung disease, then consider a repeat UGI after 1 month
colonic obstruction, etc. or may occur if the patient is asymptomatic now. If the
from malrotation with a distal volvulus. patient continues to have bilious emesis,
A contrast enema should be performed then consider laparoscopy.
next, and if it is positive for distal pathol- (c) If the DJJ is in the abnormal position,
ogy, then consider other diagnoses. If the then malrotation should be considered
contrast enema is negative and the patient and treated with an NGT and an open or
is clinically stable, then obtain an upper laparoscopic Ladd’s procedure.
GI contrast study. F. Follow-up care: Outcomes depend on pres-
D. An upper GI contrast study is the current gold ence of volvulus with intestinal ischemia and
standard for diagnosing malrotation. The key extent of bowel resection. Most common
feature to look for is the location of the complication after a Ladd’s procedure is
duodenal-jejunal junction (DJJ). adhesive bowel obstruction, occurring in
E. Location of the DJJ: 5.6% of patients. Although rare and occurring
(a) Normal position: If the DJJ is in the nor- in less than 1% of patients, recurrent volvulus
mal anatomical position, which is left of should also be considered in patients who
midline and at the level of the gastric present with obstructive symptoms [1].
antrum, then the diagnosis is unlikely
malrotation and other etiologies should
be considered.
124 Management of Malrotation 507
A History and Physical

Infant with bilious emesis.
Obtain vital signs and perform physical examinations
B Peritonitis,
hemodynamic
YES
instability?
OR
NO
Abdominal
C X-ray
Free Air/Pneumatosis Distal Obstruction

or Proximal Obstruction
Unremarkable
OR
Contrast
Enema with
NO Distal
Pathology?
Upper GI
D Contrast
Study
Equivocal Normal DJJ YES

Abnormal DJJ position
position
E
Repeat UGI in 1 Malrotation

month or
Laparoscopy if still Consider other
symptomatic pathology
Ladd’s Procedure
F Follow-up Care
Algorithm 124.1
508 S. Abdulhai et al.
References 2. Ford EG, Senac MO, Srikanth MS, Weitzman

JJ. Malrotation of the intestine in children. Ann Surg.
1992;215(2):172–8.
1. El-Gohary Y, Alagtal M, Gillick J. Long-term com-
3. Langer JC. Intestinal rotation abnormalities and mid-
plications following operative intervention for intes-
gut volvulus. Surg Clin North Am. 2017;97(1):147–
tinal malrotation: a 10-year review. Pediatr Surg
59. https://doi.org/10.1016/j.suc.2016.08.011.
Int. 2010;26(2):203–6. https://doi.org/10.1007/
s00383-009-2483-y.
Management of Imperforate Anus
125
Sophia Abdulhai and Aaron Garrison
Algorithmic Approach (d) Musculoskeletal: Tethered cord, which

occurs in about 25% of anorectal malfor-
A. Anorectal malformations (ARMs), or imper- mations and is associated with worse
forate anus, should be diagnosed during the future bowel function, should be evaluated
newborn examination. A fistula may be more using US or magnetic resonance imaging
difficult to diagnose immediately, as it can (MRI) of the spine prior to 3 months of
take 16–24 h after birth for meconium to pass age [3]. Anterior-Posterior (AP) and lat-
through the fistula. eral films of the sacrum should also be
B. ARMs are commonly associated with other performed to evaluate for other sacral
congenital anomalies, and appropriate work- anomalies, such as hemisacrum and hemi-
up should be performed within the first 24 h. vertebrae. This information can also be
(a) Cardiovascular defects: Up to 27% of used to calculate the sacral ratio.
patients with imperforate anus also have (e) In females with cloaca, pelvic US should
cardiovascular defects [1], so an echocar- be performed to rule out hydrocolpos.
diogram should be performed prior to C. Males:
any surgical intervention. (a) Perineal fistula: This presents as an ori-
(b) Urologic defects: About 50% of all ARM fice anterior to the sphincter complex.
patients also have some type of urologi- (b) Flat perineum: If there is meconium in
cal defect, and incidence of urologic the urine, then this indicates a recto-
abnormalities is highest with cloaca. urethral fistula. This is the most common
Renal ultrasound (US) should be per- type of fistula in males. Eventually, a
formed on every patient. contrast study through the mucous fistula

(c) Gastrointestinal defects: Esophageal should be performed to evaluate the anat-
atresia occurs in 5–10% of all ARMs [1, omy and/or level of the fistula.
2] and duodenal atresia in about 4% [1]. (c) Undefined: A cross-table lateral film
This should be ruled out using a nasogas- should be performed 24 h after birth to
tric tube and abdominal X-ray. evaluate the rectal gas pattern.
(d) There will be times where the definitive
anatomy is not known until a contrast
S. Abdulhai · A. Garrison (*) study through the mucous fistula is per-
Division of Pediatric Surgery, Akron Children’s formed after the colostomy.

510 S. Abdulhai and A. Garrison
D. Females: (b) Recto-urethral fistulas in males and

(a) Perineal fistula: This is an orifice anterior cloacas in females should be managed
to the sphincter but posterior to the with a colostomy initially. Definitive
introitus. repair with a posterior sagittal anorecto-
(b) Vestibular fistula: This is diagnosed by plasty or posterior sagittal anorectovag-
finding an orifice within the introitus, inourethroplasty should be performed
typically distal to the hymen in the poste- about 1–2 months later after work-up
rior wall of the vagina. for additional anomalies has been com-
(c) Single perineal orifice/cloaca: Imperforate pleted and the patient is exhibiting nor-
anus in a female with a single perineal mal growth patterns. Management of
opening is a cloaca, which varies in com- obstructive urologic issues can be an
plexity based on common channel length. emergency in females with cloaca, and
This evaluation requires cystoscopy, vagi- hydrocolpos should be drained if
noscopy, and in some centers an MRI. present.
(d) No fistula: This occurs in less than 10% (c) If the fistula is undefined in males, or if
of patients. there is no fistula in a female, then a
E. Treatment: cross-table lateral film should be per-
(a) Perineal and sub-epithelial fistulas in males formed to evaluate the distance between
and perineal or vestibular fistulas in females the dilated gas and the skin.
may be managed with anoplasty without (i) If the rectal gas is below the coccyx,
diversion. Some may choose to perform anoplasty should be performed with
colostomy prior to posterior sagittal ano- or without a colostomy (depending
rectoplasty (PSARP), and some surgeons on surgeon preference).
may choose to dilate the fistula and perform (ii) If the rectal gas is above the coccyx,
anoplasty later if needed. Fistula dilation then a colostomy should be per-
and referral to a colorectal center should be formed, and definitive repair deferred
considered by inexperienced surgeons. to a later date.
125 Management of Imperforate Anus 511

A Newborn child with lack of anal opening, evaluate for potential fistulas. May need to wait 16-24 hours for meconium to pass
through a separate orifice.
In first 24 hours, work up for other congenital anomalies.

B Echocardiogram, Renal US, r/oesophageal and duodenal atresia using NGT and abdominal X-ray film,
AP and lateral films of the sacrum In females, if cloaca, also obtain pelvic US.
C Male D Female
Perineal fistula Flat Perineum

No Fistula Perineal or Single Perineal
vestibular Opening (Cloaca)
fistula
Meconium
in Urine
Anoplasty (or No Anoplasty (or Colostomy and
fistula dilation) fistula dilation) drainage of
Yes Hydrocolpos (if
Undefined/
No fistula present)
E
Colostomy
Cross Table
Lateral Film Fistulogram
Rectal Gas Rectal Gas

Fistulogram ABOVE coccyx BELOW coccyx < 3 cm common > 3 cm common
channel channel
Colostomy Anoplasty (with

PSARP or without
PSARVUP Transfer to
Colostomy)
colorectal center
PSARP for PSARVUP
Algorithm 125.1
2. Peña A, Hong AR. In: Mattei P, editor. Anorectal

References malformation. Philadelphia: Lippincott Williams &
Wilkins; 2003.
1. Cho S, Moore SP, Fangman T. One hundred three 3. Tsuda T, Iwai N, Kimura O, Kubota Y, Ono S, Sasaki
consecutive patients with anorectal malformations Y. Bowel function after surgery for anorectal mal-
and their associated anomalies. Arch Pediatr Adolesc formations in patients with tethered spinal cord.
Med. 2001;155(5):587. https://doi.org/10.1001/ Pediatr Surg Int. 2007;23(12):1171–4. https://doi.
archpedi.155.5.587. org/10.1007/s00383-007-2025-4.
Hirschsprung Disease
126
Rachel E. Hanke, Morgan K. Moroi,
and Kathryn Lynn Martin
Algorithmic Approach associated enterocolitis (HAEC). HAEC is

characterized by fever, abdominal distension,
A. Hirschsprung disease (HD) is characterized and diarrhea. It can be life-threatening and is
by incomplete migration of ganglion cells the most common cause of death in children
within the myenteric and submucosal with HD [1].
plexuses to the distal bowel leading to C. Initial evaluation includes abdominal radio-

aganglionosis and functional obstruction.
graphs which demonstrate obstruction.
Approximately 75% of cases involve a transi- Contrast enema reveals proximal distended
tion point within the rectosigmoid region, bowel that tapers through the transition zone
while 15% have long-segment colonic dis- to a decompressed distal rectum. Anorectal
ease, 5–10% have total colonic disease, manometry may be helpful in documenting
and <5% have total intestinal disease [1]. the recto-anal inhibitory reflex, which is
B. HD is typically diagnosed in early infancy, absent in HD [1].
with a small proportion of cases presenting D. Definitive diagnosis is made by tissue biopsy.
later in childhood. Neonates present with In infants, suction rectal biopsy is performed
abdominal distension, bilious vomiting, feed- at the bedside without the need for anesthe-
ing intolerance, and failure to pass meconium sia. The yield of suction rectal biopsy
within the first 48 h of life [1]. Rectal exami- decreases with age; thus full-thickness rectal
nation causes explosive decompression of gas biopsy is required in older children. The
and stool. HD diagnosed beyond the neonatal pathognomonic findings are the absence of
period presents with chronic abdominal dis- ganglion cells with hypertrophied nerve fibers
tension, constipation, overflow incontinence, [1]. Additional staining may assist with diag-
encopresis, and failure to thrive. Ten percent nosis including stains demonstrating
of patients will present with Hirschsprung- increased acetylcholinesterase uptake and
absent calretinin [2].
E. HD is associated with several other congeni-
R. E. Hanke · M. K. Moroi
Department of Surgery, Penn State Milton S. Hershey tal anomalies including malrotation, congeni-
Medical Center, Hershey, PA, USA tal heart disease, genitourinary abnormalities,
K. L. Martin (*) and several syndromes, the most common of
Department of Surgery, Division of Pediatric Surgery, which is Trisomy 21 [1]. Preoperatively,
Penn State Milton S. Hershey Medical Center, patients should be stabilized and screened for
Hershey, PA, USA associated anomalies.

F. Initial management is dictated by the stability age [3]. Surgical techniques differ in the
of the patient. With signs of peritonitis, intes- method used to bring the proximal ganglion-
tinal perforation, or HAEC, the first priority ated bowel through the pelvis to the dentate
is resuscitation with intravenous fluids, line and include the Swenson extra-rectal
broad-spectrum antibiotics, gastric decom- pull-through, Soave endo-rectal pull-through,
pression, and colonic irrigations, followed by and Duhamel rectal sparing pull-through.
emergent surgical intervention. The goal of Laparoscopic and transanal approaches are
surgery in this setting involves control of per- gaining popularity with a trend toward one-
foration, if present, and leveling stoma. A lev- stage repair in newborns [4]. In total colon
eling stoma involves determination of the HD, a staged approach is preferred, with ini-
level of aganglionosis with stoma creation tial leveling stoma and subsequent pull-
proximal to this point [1]. through [4]. In total intestinal aganglionosis,
G. Definitive treatment of HD is surgical.
patients require a stoma to decompress the
Surgery involves removal of the aganglionic gastrointestinal (GI) tract with total paren-
bowel segment followed by anastomosis of teral nutrition (TPN) for nutritional support
the normally innervated proximal bowel to and possible intestinal transplantation.
the distal rectum just above the dentate line. H. Post-operatively, patients are assessed for

This may be done as a single stage or in mul- stricture and followed closely as the risk of
tiple stages. Historically, all patients received HAEC persists. Long-term complications
leveling stomas prior to definitive repair. include stricture and stooling dysfunction,
Presently, surgical correction depends on the which may present as constipation, soiling, or
patient’s level of disease, comorbidities, and fecal incontinence.
126 Hirschsprung Disease 515
A
3 day-old with abdominal distension and 2-year-old with chronic severe constipation
failure to pass meconium and abdominal distension
B
Plain Radiograph
C Contrast Enema
Concerning
for HD
No
Yes
Alternative
diagnoses
Positive rectal No
D biopsy
Yes
E Evaluate for other

congenital abnormalities
F
Plan for surgical
intervention
Unstable patient +/-peritonitis Long segment, total colon or Long segment or recto-sigmoid
Stabilization and urgent OR delayed diagnosis. OR for leveling disease in stable patient. OR for
for leveling stoma stoma with delayed pull-through pull-through without colostomy
Post-operative surveillance for HAEC, stooling

H dysfunction (fecal incontinence,constipation)
Algorithm 126.1
References staining in Hirschsprung disease. J Pediatr Surg.

2016;51(12):2005–9.
3. Bradnock TJ, Knight M, Kenny S, Nair M, Walker
1. Langer JC. Pediatric surgery. In: Coran AG, Adzick
GM. The use of stomas in the early management of
NS, Krummel TM, Laberge JM, Shamberger RC,
Hirschsprung disease: findings of a national, prospec-
Caldamone AA, editors. Hirschsprung disease. 7th
tive cohort study. J Pediatr Surg. 2017;52(9):1451–7.
ed. Philadelphia: Saunders; 2012.
4. Langer JC. Laparoscopic and transanal pull-through
2. Tran VQ, Lam KT, Truong DQ, Dang MH, Doan
for Hirschsprung disease. Semin Pediatr Surg.
TT, Segers V, Butler MW, Robert A, Goyens P,
2012;21(4):283–90.
Steyaert H. Diagnostic value of rectal suction
biopsies using calretinin immunohistochemical
Pediatric Inguinal Hernia
127
Algorithmic Approach can be performed electively. In young infants

a wait time for surgery of more than 14 days
A. Primary goals in the evaluation of patients is associated with a doubling of the risk of
with inguinal hernias are to confirm the pres- incarceration, and thus repair should be pur-
ence of the inguinal hernia, excluding other sued in a time-sensitive fashion [2]. Premature
etiologies of groin swelling, and to determine infants with asymptomatic inguinal hernias
whether the hernia is incarcerated with the may benefit from delayed repair to allow co-
potential for visceral injury. An inguinal her- morbid lung disease to improve prior to
nia should be suspected in any infant or child surgery.
with a “bulge” in the groin, labia, or scrotum. C. If the inguinal hernia is incarcerated, all
This bulge may be accentuated during times attempts should be made to reduce the hernia
of increased intra-abdominal pressure such as before it progresses to strangulation. This
crying or straining. Primary differential diag- may require sedation to permit adequate
noses include hydrocele, undescended testi- manipulation and reduction of contents. If
cles, inguinal adenopathy, and testicular bowel is incarcerated in the hernia, the patient
torsion. may present with signs of bowel obstruction
B. In the pediatric population, inguinal hernias such as pain, distension, vomiting, obstipa-
are typically indirect secondary to a patent tion, and even peritonitis. If there is concern
processus vaginalis. Inguinal hernias com- for strangulation or if peritoneal findings are
monly present during the first year of life, present, urgent exploration is mandated.
with nearly one-third of cases presenting While the intestine is the most commonly
before 6 months of age [1]. The incidence is incarcerated visceral component, the omen-
higher in premature infants. There is a 3:1 tum, ovary, mesosalpinx, or bladder may also
male-to-female preponderance. Incarceration become entrapped within a hernia sac.
is most common in the first 6 months of life. D. If the diagnosis is unclear on physical exam,
In older children and adolescents, in whom ultrasound can be a useful adjunct [3]. If the
the risk of incarceration is low, hernia repair hernia cannot be reproduced at the time of
examination, follow-up examination may be
A. N. Kulaylat · K. L. Martin (*) helpful. Many surgeons will still proceed to
Department of Surgery, Division of Pediatric Surgery, elective hernia repair if the appropriate his-
Penn State Milton S. Hershey Medical Center, tory is given or appropriate images are pro-
Hershey, PA, USA vided by the family.

518 A. N. Kulaylat and K. L. Martin
E. If the inguinal hernia is not incarcerated, empty, the hernia sac is ligated at the level of
elective repair can be scheduled with the fam- the internal ring. In females, the sac is simi-
ily appropriately educated regarding the risk larly mobilized and ligated at the level of the
of incarceration and strangulation. internal ring which may then be sutured
F. If the hernia is initially incarcerated but closed. While many surgeons still prefer open
maneuvers to reduce it are successful, a brief inguinal hernia repair [4], laparoscopic
period of observation allows for resuscitation, approaches are becoming increasingly com-
resolution of tissue edema, and confirmation mon with some evidence suggesting lower
of a normal abdominal exam prior to pro- recurrence rates [5–8].
ceeding with operative repair. I. Investigation of the contralateral side remains
G. For those with truly incarcerated hernias with a controversial topic. Traditionally, the high
concerns for strangulation or peritonitis, likelihood of contralateral patent processus
expeditious arrangements are made for the vaginalis (PPV) was cited as an indication for
operating room (OR) following appropriate routine exploration. However, the progres-
and often parallel resuscitative efforts includ- sion of PPV to symptomatic inguinal hernia
ing intravenous fluids and antibiotics. is lower than previously believed. Recent
Nasogastric tube should be considered on the studies have suggested PPV occurs in ~10–
basis of obstructive symptoms. 45% of patients, [5, 6, 9] but of those with
H. In males, open operative repair involves an PPV, only 10% develop an inguinal hernia [9,
inguinal incision with identification of the 10]. One study estimated that 21 contralateral
cord structures followed by isolation of the PPV would need to be closed to prevent one
hernia sac away from the vas deferens and metachronous inguinal hernia [8]. As such,
testicular vasculature. After reduction of its there has been a trend away from routine con-
contents and confirmation that the sac is tralateral exploration [8–10].
127 Pediatric Inguinal Hernia 519
History:
A 2 year old male, intermittent right groin bulge with crying and straining
Obtain vital signs and perform physical examination.

Make note of the location of the swelling/bulge, ability to
transilluminate, presence and location of testes, presence
of inguinal adenopathy
Reducible Unclear exam

B hernia
No Yes
Consider
ultrasound D
Incarcerated Schedule for elective
hernia E hernia repair
Attempt Obstructive symptoms? Open or Laparoscopic

C reduction +/- IV resuscitation, + Antibiotics, + Hernia Repair +/-
sedation Nasogastric tube as clinically contralateral
appropriate exploration
Reducible
hernia
No Yes
F
G Expedite OR Observe 24-48 hours to allow
edema to decrease
Open or Laparoscopic
Hernia Repair
+/-resection via
inguinal/midline/laparoscopic
approach per surgeon
H I
preference
Algorithm 127.1
References 6. Miyake H, Fukumoto K, Yamoto M, et al. Risk fac-

tors for recurrence and contralateral inguinal her-
nia after laparoscopic percutaneous extraperitoneal
1. Lao OB, Fitzgibbons RJ Jr, Cusick RA. Pediatric
closure for pediatric inguinal hernia. J Pediatr Surg.
inguinal hernias, hydroceles, and undescended tes-
2017;52(2):317–21.
ticles. Surg Clin North Am. 2012;92(3):487–504, vii.
7. Gause CD, Casamassima MG, Yang J, et al.
2. Zamakhshary M, To T, Guan J, Langer JC. Risk of
Laparoscopic versus open inguinal hernia repair in
incarceration of inguinal hernia among infants and
children </=3: a randomized controlled trial. Pediatr
young children awaiting elective surgery. CMAJ.
Surg Int. 2017;33(3):367–76.
2008;179(10):1001–5.
8. Zhao J, Chen Y, Lin J, et al. Potential value of rou-
3. Sameshima YT, Yamanari MG, Silva MA, Neto MJ,
tine contralateral patent processus vaginalis repair in
Funari MB. The challenging sonographic ingui-
children with unilateral inguinal hernia. Br J Surg.
nal canal evaluation in neonates and children: an
2017;104(1):148–51.
update of differential diagnoses. Pediatr Radiol.
9. Centeno-Wolf N, Mircea L, Sanchez O, et al. Long-
2017;47(4):461–72.
term outcome of children with patent processus vagi-
4. Zani A, Eaton S, Hoellwarth M, et al. Management of
nalis incidentally diagnosed by laparoscopy. J Pediatr
pediatric inguinal hernias in the era of laparoscopy:
Surg. 2015;50(11):1898–902.
results of an international survey. Eur J Pediatr Surg.
10. Weaver KL, Poola AS, Gould JL, Sharp SW, St

2014;24(1):9–13.
Peter SD, Holcomb GW 3rd. The risk of developing
5. Esposito C, Escolino M, Cortese G, et al. Twenty-year
a symptomatic inguinal hernia in children with an
experience with laparoscopic inguinal hernia repair in
asymptomatic patent processus vaginalis. J Pediatr
infants and children: considerations and results on
Surg. 2017;52(1):60–4.
1833 hernia repairs. Surg Endosc. 2017;31(3):1461–8.
Meconium Ileus
128
Kathryn Lynn Martin and Afif N. Kulaylat
Algorithmic Approach exam reveals a distended but soft abdomen

with palpable, doughy bowel loops.
A. Meconium ileus is characterized by newborn Abdominal x-rays in uncomplicated meco-
bowel obstruction secondary to inspissated nium ileus demonstrate loops of dilated bowel
meconium impacted within the distal small typically without air-fluid levels with a clas-
bowel. Over 75% of children with meconium sic “ground glass” or “soap bubble” appear-
ileus will have cystic fibrosis [1]. Meconium ance [3]. Infants with complicated meconium
ileus is the initial presentation of cystic fibro- ileus due to atresia, volvulus, meconium peri-
sis in 15–27% of affected individuals [1]. tonitis, or meconium pseudocyst often pres-
There may be a positive family history lead- ent with abdominal distension at birth [1].
ing to increased suspicion of the diagnosis. This may be associated with signs of fetal
Prenatal diagnosis may be suggested in the distress and peritonitis. Abdominal x-rays in
setting of sonographic evidence of polyhy- complicated meconium ileus classically dem-
dramnios, dilated bowel, a calcified mass, or onstrate calcifications suggestive of in utero
echogenic intra-abdominal cyst. These find- perforation with a possibility of meconium
ings are variable and nonspecific [1, 2]. peritonitis with or without a well-formed
Postnatally, the history of early-onset abdom- pseudocyst [3].
inal distension, bilious emesis, and delayed C. Contrast enema in meconium ileus will dem-
passage of meconium, with palpable loops of onstrate a microcolon secondary to disuse
bowel and/or an abdominal mass, is highly with outlining of meconium pellets in the dis-
suggestive of meconium ileus [2, 3]. tal small bowel. Use of a water-soluble hyper-
Malrotation with volvulus should be ruled out osmolar contrast agent such as Gastrografin®
in any neonate presenting with bilious can be diagnostic and therapeutic [1–3].
emesis. D. Initial treatment of uncomplicated meconium
B. Infants with uncomplicated meconium ileus ileus is non-surgical with solubilizing ene-
present with progressive abdominal disten- mas. Hyperosmolar enemas are effective but
sion in the first 24–48 h of life. Abdominal can cause significant fluid shifting and dehy-
dration; thus concurrent IV fluid administra-
K. L. Martin (*) · A. N. Kulaylat tion is required [1–3]. Gastrografin® is the
Department of Surgery, Division of Pediatric Surgery, most commonly used agent [3]. The addition
Penn State Milton S. Hershey Medical Center, of N-acetylcysteine can be helpful as it has
Hershey, PA, USA been shown to aid in the dissolution of inspis-

522 K. L. Martin and A. N. Kulaylat
sated meconium in animal studies [2]. stoma formation versus anastomosis. If an

Enemas can be repeated until the meconium atresia is present, the proximal dilated seg-
obstruction clears as long as the infant ment is resected, the distal bowel cleaned of
remains clinically well without worsening meconium, and a decision made regarding
obstruction or signs of intestinal compromise stoma formation versus anastomosis [1–3]. In
[1, 2]. cases of perforation with meconium peritoni-
E. Operation is required for infants with uncom- tis or a meconium pseudocyst, abdominal
plicated meconium ileus that fails to improve washout with debridement of the cyst fol-
with solubilizing enemas to evacuate the lowed by resection and stoma formation is
inspissated meconium and relieve their preferred [3].
obstruction before complications develop. A G. A diet may started once the infant is stabi-
wide variety of surgical options exist includ- lized and stooling. Work-up for cystic fibrosis
ing enterotomy with irrigation or stoma cre- should be initiated. Diagnostic modalities
ation. Classically described stomas for include genetic analysis and sweat chloride
meconium ileus include the Mikulicz double- testing [1, 2]. Genetic testing assesses for an
barreled stoma, Bishop-Koop (distal “chim- array of causative mutations, with the most
ney”) stoma, or Santulli (proximal “chimney”) common being ΔF508 [1]. Sweat chloride
stoma [1–3]. Resection is rarely required in testing can be done after 48 h of life; how-
uncomplicated disease. ever, it may be difficult to obtain the volume
F. Complicated meconium ileus is managed for sweat needed in children less than 2 weeks
operatively. If volvulus is present, the bowel of age [1, 2]. Long-term complications of
should be detorted and assessed for viability. meconium ileus associated with cystic fibro-
If viable, the distal bowel is cleaned of meco- sis include failure to thrive, pancreatic insuf-
nium as described earlier. If the bowel is non- ficiency, constipation, rectal prolapse, and
viable, then resection should be completed distal intestinal obstruction syndrome (DIOS)
after which the distal bowel is cleaned of also known as meconium ileus equivalent
meconium and a decision is made regarding syndrome [1, 3].
128 Meconium Ileus 523
Newborn with progressive abdominal distension, emesis and failure to

A pass meconium +/- Family history of cystic fibrosis
Place NG tube
Initiate fluid resuscitation
38 C, HR 200, BP 70/40, RR 50,

Sats 99% Perform physical examination
Obtain abdominal radiographs
Peritonitis or meconium
pseudocyst on AXR?
No Yes
Exam - distension, palpable, doughy, bowel loops. Exam - distension, rigidity, tenderness +/- palpable
AXR - Distension with air fluid levels, ground mass AXR – Signs of bowel obstruction with
glass/soap-bubbles in terminal ileum suggestion of cystic mass and scattered calcifications
Obtain contrast enema

OR F
C
Enema - micro-colon with meconium pellets
in the distal small bowel and proximal colon
Initiate diet when appropriate
Complete work-up for cystic fibrosis
G
D Non-surgical management
with solubilizing enemas
Enemas
successful?
Yes No
Initiate diet OR E
G Complete work-up for
cystic fibrosis
Initiate diet when appropriate

Complete work-up for cystic fibrosis G
Algorithm 128.1
524 K. L. Martin and A. N. Kulaylat
References 2. Carlyle BE, Borowitz DS, Glick PL. A review of

pathophysiology and management of fetuses and neo-
nates with meconium ileus for the pediatric surgeon. J
1. Boczar M, Sawicka E, Zybert K. Meconium ileus in
Pediatr Surg. 2012;47:772–81.
newborns with cystic fibrosis – results of treatment in
3. Rescirka FJ, Grosfeld JL. Contemporary management
the group of patients operated on in the years 2000–
of meconium ileus. World J Surg. 1993;17:318–25.
2014. Dev Period Med. 2015;XIX(1):32–40.
Pediatric Intussusception
129
Algorithmic Approach a few minutes, with intervening periods of

remission and normal behavior typically
A. Intussusception in children is typically ileo- about 30 min apart. Fifteen percent of patients
colic in nature. It is a common source of present with atypical symptoms, such as pale-
bowel obstruction which results from the ness or lethargy, suggestive of advanced dis-
telescoping of bowel onto itself. ease [2]. Fever, tachycardia, hypotension,
Intussusception should be suspected in any and/or peritonitis are also suggestive of
patient with episodic abdominal pain, which advanced disease with risk of bowel ischemia
may be accompanied by emesis, an abdomi- or perforation.
nal mass, and rectal bleeding. Ninety percent C. The workup of patients with suspected intus-
of patients with intussusception are between susception begins with plain radiographs
the ages of 3 months and 3 years (peak inci- which will show signs of bowel obstruction.
dence, 5–9 months) [1]. Infants are more Ultrasound should follow [2]. Ultrasound
likely to present with emesis than older chil- classically demonstrates a target sign and,
dren. Rectal bleeding is classically a mucous- when present, may also identify a lead point.
like texture described as “red-currant jelly” If intussusception is not present, ultrasound
and is often a late finding. Ileal lymphade- may reveal other etiologies of the patient’s
nopathy, including hypertrophic Peyer’s symptoms such as appendicitis, ovarian tor-
patches, is thought to be the lead point for sion, or urinary tract abnormalities. In suspi-
ileocolic intussusception. Non-ileocolic cious clinical presentations, a negative
intussusception is rare in children and typi- ultrasound study should not exclude repeat
cally presents with a pathologic lead point, of ultrasound or progression to air/contrast
which Meckel’s diverticulum and small enema if deemed clinically indicated. Of
bowel tumors are the most common. note, small bowel-small bowel intussuscep-
B. The presentation of intussusception includes tion without signs of obstruction or a patho-
severe, acute, and intermittent abdominal logic lead point is a common finding in
pain which is episodic in nature, often lasting patients with abdominal pain and is typically
self-limited.
A. N. Kulaylat · K. L. Martin (*) D. On the basis of clinical examination and labo-
Department of Surgery, Division of Pediatric Surgery, ratory studies, the child or infant should be
Penn State Milton S. Hershey Medical Center, treated with intravenous fluids and correction
Hershey, PA, USA of electrolyte abnormalities. Placement of a

nasogastric tube and antibiotic coverage tion proceeds easily and the child does not
should also be considered. Children who are demonstrate signs of peritonitis or clinical
unstable or have peritonitis should be taken deterioration. This strategy decreases the rate
directly to the operating room. If ileocolic of bowel resection and reduces mean length
intussusception is present and the child is of stay and costs [6]. If enema reduction is
stable without peritonitis, then air/contrast unsuccessful, the child is taken to the operat-
enema should be pursued. In the hands of an ing room.
experienced radiologist, enema reduction is G. Once the decision has been made to operate,
successful in over 80% of cases [3]. the approach, laparoscopic or open, should be
E. If enema reduction is successful, patients dictated by the clinical stability of the patient
should be observed for signs of recurrence. and surgeon preference [7]. Initial open
Historically, recurrence rates were cited as maneuvers include attempted reduction by
5–15% after enema reduction with the major- gently pulling on the bowel just proximal to
ity occurring within the first few days of ini- the intussusception while applying pressure
tial presentation and up to one-third in the distally (traction-pulsion technique).
first 24 h [2]. Recent meta-analysis suggests Laparoscopically a traction-traction technique
24- and 48-h recurrence rates of less than 3% is used with gentle pulling on the involved
following fluoroscopic-guided air enema bowel to allow reduction. Once reduced the
reduction [4]. Accordingly, select patients bowel should then be assessed for viability. If
may be safely discharged from the emergency intestinal ischemia or perforation is present,
department after reduction with proper edu- or if reduction is unsuccessful, then bowel
cation and follow-up [5]. resection is completed. If a pathologic lead
F. If abdominal pain recurs ultrasound should be point is identified, the patient should undergo
repeated to assess for recurrence. If recur- resection with primary anastomosis.
rence is confirmed then air/contrast enema is Pathologic small bowel-small bowel intussus-
repeated. Air/contrast enemas can be repeated ceptions cannot be reduced with air enemas
as often as necessary, as long as each reduc- and thus require operative intervention.
129 Pediatric Intussusception 527
History:
2 year old male, episodic abdominal pain, emesis, red-currant jelly stools
Obtain vital signs, blood work and

perform physical examination
37.1 C, HR 100, RR 14, BP 90/50, WBC 11,000

Na 140, Cl 110, HCO3 24
RUQ/epigastric tenderness + palpable mass
Presentation concerning for intussusception in otherwise stable

C patient AXR suggest obstruction. Obtain an ultrasound
Intussusception
identified
No
Alternative
diagnosis on U/S? Yes
Continue workup
for other etiologies
D Air/contrast enema
E Failure to reduce intussusception Intussusception reduced
Repeat
Operating room Observe Ultrasound
Operative
G reduction
F
+/-resection Discharge Recurrent pain
Algorithm 129.1
References 4. Gray MP, Li SH, Hoffmann RG, Gorelick

MH. Recurrence rates after intussusception
enema reduction: a meta-analysis. Pediatrics.
1. Savoie KB, Thomas F, Nouer SS, Langham MR
2014;134(1):110–9.
Jr, Huang EY. Age at presentation and manage-
5. Raval MV, Minneci PC, Deans KJ, et al. Improving
ment of pediatric intussusception: a Pediatric
quality and efficiency for intussusception manage-
Health Information System database study. Surgery.
ment after successful enema reduction. Pediatrics.
2017;161(4):995–1003.
2015;136(5):e1345–52.
2. Columbani PM, Scholz S. Intussusception. In:
6. Lautz TB, Thurm CW, Rothstein DH. Delayed repeat
Coran AG, Adzick NS, Krummel TM, Laberge J,
enemas are safe and cost-effective in the manage-
Shamberger RC, Calamone AA, editors. Pediatric sur-
ment of pediatric intussusception. J Pediatr Surg.
gery, vol. 2. 7th ed. Philadelphia: Elsevier Saunders;
2015;50(3):423–7.
2012.
7. Apelt N, Featherstone N, Giuliani S. Laparoscopic
3. Sadigh G, Zou KH, Razavi SA, Khan R, Applegate
treatment of intussusception in children: a systematic
KE. Meta-analysis of air versus liquid enema for intus-
review. J Pediatr Surg. 2013;48(8):1789–93.
susception reduction in children. Am J Roentgenol.
2015;205(5):W542–9.
Pyloric Stenosis
130
Algorithmic Approach tips underneath the liver in the midline; the

fingertips are pulled back and down, trying to
A. The typical patient presenting with pyloric trap the “olive.” [1]
stenosis is between 2 and 8 weeks of age with B. These patients can present with mild-to-
a male/female (M/F) ratio of 4:1. Patients ini- severe dehydration depending on the duration
tially tolerate feeds well with gradual and severity of symptoms. The levels of
increases in episodes of emesis which eventu- severity have been largely based on bicarbon-
ally occur with every feed. The emesis is non- ate levels and are defined as slight
bilious in color and is forceful or “projectile.” (<25 mEq/L), moderate (26–35 mEq/L), and
In a relaxed and calm infant, the enlarged severe (>35 mEq/L) [2]. Other abnormal lab
pylorus will feel like an olive in the upper values seen in these patients are the tradi-
abdomen in 72–89% of patients. The physical tional hypokalemic, hypochloremic meta-
exam is performed by palpating the infant’s bolic alkalosis with possible decreased urine
liver edge and sliding the examiner’s finger- output and a paradoxical aciduria. If the phys-
ical exam and lab values are unrevealing, an
D. W. Parrish ultrasound or upper gastrointestinal series
Department of Pediatric Surgery, Batson Children’s may be performed. Positive ultrasound find-
Hospital, University of Mississippi Medical Center,
ings include a pyloric muscle thickness of
Jackson, MS, USA
≥4 mm and a pyloric channel length ≥16 mm.
J. H. DeAntonio
Stopping feeds is necessary. As these infants
Division of Pediatric Surgery, Department of General
Surgery, Virginia Commonwealth University Health, are typically able to tolerate their gastric
Richmond, VA, USA secretions, a nasogastric tube is not a require-
Department of Surgery, Virginia Commonwealth ment, but it may be necessary in severe cases
University School of Medicine, Richmond, VA, USA [1, 3].
D. A. Lanning (*) C. This condition is not a surgical emergency.
Division of Pediatric Surgery, Department of General The primary emphasis needs to be placed on
Surgery, Virginia Commonwealth University Health, resuscitation and correcting electrolyte
Richmond, VA, USA
abnormalities. If the patient were taken to the
Department of Surgery and Pediatrics, Children’s operating room prior to resuscitation, it could
Hospital of Richmond, Richmond, VA, USA
precipitate difficulty weaning from the venti-
Department of Surgery, Virginia Commonwealth lator due to postoperative apnea secondary to
University School of Medicine, Richmond, VA, USA
metabolic alkalosis. Correction of dehydra-

530 D. W. Parrish et al.
tion is traditionally started with an intravenous roscopic. The open approach utilizes a right
(IV) fluid bolus with normal saline at 20 cc/ upper quadrant transverse or umbilical inci-
kg followed by continuous IV fluids at 1.25– sion, while the laparoscopic approach uses an
2x maintenance rate of D5 ½ normal saline umbilical port for the camera and two stab
with 20–30 mEq KCl/L. [1] Historical teach- incisions lateral to the umbilicus to follow the
ing was to delay potassium replacement until same principles as the open incision [1, 3].
urine output returns or increases, but this
E. Two schools of thought exist for post-
unnecessarily delays needed replacement. We pyloromyotomy feeding schedule with no
recommend monitoring of urine output and significant differences in length of stay
rechecking labs every 6–8 h until electrolytes being observed. An ad lib feeding schedule
normalize [1, 3]. has been associated with more episodes of
D.
Once the lab values are normalized emesis but a shorter time to full feeds. A
(CO2 < 30 mEq/L, Cl > 100, K > 4), the typical regimented feeding schedule con-
patient is deemed resuscitated and is taken to sists of pedialyte followed by increasing
the operating room for a pyloromyotomy. The amounts of formula or breast milk every few
two traditional approaches are open and lapa- hours [1].
130 Pyloric Stenosis 531

· Previously healthy baby now intolerant of feeds
A · History of nonbilious, forceful emesis
· Possible “olive-shaped” enlarged pylorus is palpable in the upper abdomen
in 75% of patients
Obtain vital signs, including urine output (uop), blood work

(including BMP); if physical exam is unrevealing, obtain
ultrasound; place nasogastric tube
B
· Decreased urine output, nonbilious emesis, positive US
findings of thickened pylorus
· Typical lab values: CO 2 > 30, Cl < 100, K < 3
· Typical lab values: CO2 > 25
· Bolus with NS @ 20cc/kg

C · Begin fluid resuscitation with D5 ½ NS 20 KCl @ 1.25-2x
maintenance rate until uop 1 cc/kg/hr
· Monitor urine output, recheck abnormal labs every
6 hours
· Lab recheck: CO 2 > 30, Cl < 100, K < 3 · Lab recheck: CO 2 < 30, Cl > 100, K > 4
D
· Continue resuscitation, recheck labs
in 8 hours Proceed with
Pyloromyotomy
· Once labs normalize
Post-pyloromyotomy Feeding Schedule:

E
· Regimented feeding schedule
· Ad lib feeding
Algorithm 130.1
References 2. Schwartz M. Hypertrophic pyloric stenosis. In: Coran

A, et al., editors. Pediatric surgery. 7th ed. London:
Elsevier Inc; 2012.
1. Koontz C, Wulkan M. Lesions of the stomach. In:
3. Benson C, Alpern E. Preoperative and postoperative
Holcomb III G, Murphy J, Ostlie D, editors. Ashcraft’s
care of congenital pyloric stenosis. AMA Arch Surg.
pediatric surgery. 6th ed. London: Elsevier Inc; 2014.
1957;75(6):877–9.
Necrotizing Enterocolitis
131
Algorithmic Approach remains largely unknown but is believed to be

due to the immature epithelial gastrointesti-
A. Necrotizing enterocolitis (NEC) typically
nal lining of neonates, leading to transloca-
occurs in preterm, low-birth-weight infants tion of bacteria resulting in an inflammatory
and has an increasing incidence due to response that may eventually cause perfora-
advances in neonatal intensive care. It is asso- tion [2]. NEC often presents with lethargy,
ciated with hypoxia, infections, cyanotic feeding intolerance (vomiting, high gastric
heart defects, and initiation of enteral nutri- residuals), bloody stools, respiratory distress,
tion [1]. Less than 10% of cases occur in term or hypoperfusion. Physical exam findings
infants; these infants will commonly have an consist of a tender, erythematous, and dis-
insult, such as cyanotic heart defects, leading tended abdomen with more advanced disease
to hypoxia [1]. NEC’s pathophysiology demonstrating systemic symptoms such as
hemodynamic instability (hypotension,
shock), respiratory distress (apnea, respira-
tory acidosis), or decreased peripheral perfu-
J. H. DeAntonio sion (metabolic acidosis) [1, 3].
Division of Pediatric Surgery, Department of General B. Evaluation continues with laboratory assess-
Surgery, Virginia Commonwealth University Health, ments including complete blood count
Richmond, VA, USA
(CBC), chemistry panel (basic metabolic
Department of Surgery, Virginia Commonwealth panel—BMP), arterial blood gas (ABG), and
cultures. These labs, depending on severity of
D. W. Parrish disease, may demonstrate a leukocytosis or
Department of Pediatric Surgery, Batson Children’s
Hospital, University of Mississippi Medical Center, leukopenia, thrombocytopenia, metabolic or
Jackson, MS, USA respiratory acidosis, hypoxia, and bactere-
D. A. Lanning (*) mia. Abdominal radiographs can be used to
Division of Pediatric Surgery, Department of General look for radiographic signs such as pneuma-
Surgery, Virginia Commonwealth University Health, tosis intestinalis, portal venous gas, free air,
Richmond, VA, USA
or a “fixed” portion of small bowel (multiple
Department of Surgery, Virginia Commonwealth x-rays with a portion of small bowel in same
position) [1]. Ultrasound is highly user
Department of Surgery and Pediatrics, Children’s dependent but may be used as well.
Differential diagnosis includes sepsis, ileus,

534 J. H. DeAntonio et al.
small bowel obstruction, and bacterial or viral bowel loop, abdominal wall erythema, and
enteritis. bacteria positive paracentesis [1]. A patient
C. The Modified Bell’s Staging Criteria is often can become an operative candidate due to
used to clinically and radiographically diag- failure of medical management or develop-
nose and stage NEC [1, 4]. Management of ment of the above indications.
suspected or confirmed NEC begins with gas- E. If a patient meets criteria for surgical inter-
tric decompression with nasogastric or oro- vention and is >1500 g, then may proceed
gastric tube (NGT, OGT) placement, stopping with laparotomy. If <1500 g and hemody-
enteral nutrition, broad IV antibiotics, and namically unstable, a percutaneous drain may
correction of respiratory and metabolic be placed in the right lower quadrant in the
derangements as needed (IV fluid resuscita- NICU to help stabilize the patient; however,
tion, ventilation, or vasopressors). Fifty per- if hemodynamically stable, then the patient
cent of infants with NEC require surgical may proceed with exploratory laparotomy.
intervention [1]. The goal at laparotomy is removal of all
D. The only absolute surgical indication for
necrotic bowel (source control) while pre-
NEC is pneumoperitoneum. However, rela- serving as much intestinal length as possible.
tive indications include hemodynamic insta- Mortality rate ranges from 10% to 50% but
bility or failure of medical management, increases toward 100% with panintestinal
portal venous gas, abdominal mass, “fixed” disease [1].
131 Necrotizing Enterocolitis 535

· Preterm/Low Birth Weight Infant OR Term with insult (10%- listed below)
· Association: Hypoxia, Bacterial infection, Cyanotic heart defects, and Initiation of
enteral nutrition
A
· Presentation: Lethargy, Feeding intolerance, Bloody stools, Respiratory distress, or
Hypoperfusion
· VS: Bradycardia, Apnea, or Temp. instability. Distended, tense, and erythematous
abdomen. Advanced: Hemodynamic (HD) instability, Respiratory collapse, Sepsis, Shock
· Advanced Disease: Systemic Signs of Seps
Testing/Evaluation:
B · Labs: CBC, BMP, ABG, and Cultures
· Imaging: Abdominal XR (supine and left lateral decubitus) Pneumatosis intestinalis,
Portal venous gas, Free air, and a “Fixed” bowel loop
Suspect or Confirm
NEC? (Modified Bell’s
Staging) No
Evaluate Diff. Diagnoses

Yes
C
Medical Management:
· OGT/NGT placement, Stop enteral feeding, Broad
spectrum IV antibiotics
· Correct respiratory & metabolic derangements
· HD stability · Pneumoperitoneum
· · HD instability
D No pneumoperitoneum OR Relative
surgical indications · Relative surgical indications
Yes
Failure
Continue Medical Management Weight < 1500g Weight > 1500g
E
HD Instability HD Stable
No Improvement
RLQ Percutaneous Laparotomy
Drainage
Algorithm 131.1
References rotizing enterocolitis: modeling the innate immune

response. Am J Pathol. 2015;185(1):4–16.
3. Neu J. Necrotizing enterocolitis. World Rev Nutr
1. Castle SL, Speer AL, Grikscheit TC, Ford
Diet. 2014;110:253–63.
H. Necrotizing enterocolitis. In: Ziegler MM, Azizkhan
4. Walsh MC, Kliegman RM. Necrotizing enterocolitis:
RG, Dv A, Weber TR, editors. Operative pediatric sur-
treatment based on staging criteria. Pediatr Clin N
gery. New York: McGraw-Hill Education; 2014.
Am. 1986;33(1):179–201.
2. Tanner SM, Berryhill TF, Ellenburg JL, Jilling T,
Cleveland DS, Lorenz RG, et al. Pathogenesis of nec-
Omphalocele and Gastroschisis
132
Algorithmic Approach factors including environmental exposures

(tobacco), lower maternal age or socioeco-
A. Gastroschisis involves an abdominal wall
nomic status [1]. Classically, not associated
defect (usually 2–5 cm in size), typically to with other congenital defects; however,
the right of the umbilical cord, with eviscer- recently a few cases are suggestive of rare
ated abdominal contents that are not sur- genetic causes. Intestinal atresia is also noted
rounded by a membranous sac. The bowel is in 10–12% of patients [1]. Gastroschisis inci-
usually thickened and edematous. Believed to dence was noted to be 5.2 out of 1000 live
be caused by embryological failure, the lat- births in a recent US database study [2].
eral body wall folds to migrate to the midline Omphaloceles are considered midline
[1]. Occurs earlier in embryonic development abdominal wall defects (usually 4–10 cm in
than omphaloceles with possible causative size) with a peritoneal membrane covering
the abdominal contents and the umbilical
cord coming directly off the defect. It is
believed to result from the intestinal loops not
J. H. DeAntonio returning to the abdomen after the tenth week
Division of Pediatric Surgery, Department of General of gestation. Omphalocele occurs embryo-
Surgery, Virginia Commonwealth University Health, logically after gastroschisis and is not sus-
Richmond, VA, USA
pected to be associated with teratogen
Department of Surgery, Virginia Commonwealth exposure [1]. Omphaloceles are classically
associated with numerous congenital anoma-
D. W. Parrish lies including cardiac, renal, chromosomal,
Department of Pediatric Surgery, Batson Children’s
Hospital, University of Mississippi Medical Center, and genetic syndromes (i.e., Beckwith–
Jackson, MS, USA Wiedemann and Pentalogy of Cantrell). The
D. A. Lanning (*) incidence of omphaloceles has decreased in
Division of Pediatric Surgery, Department of General developed countries due to prenatal diagnosis
Surgery, Virginia Commonwealth University Health, and termination of some pregnancies, cur-
Richmond, VA, USA
rently 1–2 out of 1000 live births in the United
Department of Surgery, Virginia Commonwealth States [1, 2].
Both are typically diagnosed on prenatal
Department of Surgery and Pediatrics, Children’s ultrasound (U/S) in developed countries.
Elevated maternal serum α-fetoprotein should

be evaluated with diagnostic pre-natal ultra- transparent film dressing, after reducing the
sound due to its correlation with gastroschisis bowel into the abdomen, can avoid the need
[1]. If an omphalocele is diagnosed prena- for surgical closure in the perinatal period but
tally, then chromosome evaluation by amnio- may lead to the development of an umbilical
centesis is typically offered to parents, as well hernia.
as more extensive evaluation for associated Omphalocele repair is more complex and
anomalies by U/S or MRI. varies by size and associated congenital mal-
B. Gastroschisis and omphalocele diagnosed formations. The repairs can be described as
prenatally may be delivered vaginally if there immediate (small to medium), staged
are no fetal or obstetric concerns. For gastros- (medium to large), or delayed (giant) [1]. If
chisis, the bowel is assessed looking for sus- the defect is small or medium sized and there
pected areas of dilatation, atresia, or necrosis, are no other congenital concerns, attempts at
then contents are covered with saline- an immediate closure may proceed. If
moistened gauze, and infant is placed inside a medium to large, a staged closure with a
plastic bag-like device, which covers the modified silo and gradual reduction with
lower limbs to above the defect. This cover- eventual primary closure are attempted. Some
ing prevents evaporative and heat losses. omphaloceles are too large (giant) to be
Intravenous fluid resuscitation, antibiotics, closed, or the neonate has other congenital
and gastric decompression should begin. malformations that prevent repair during this
Omphaloceles with an intact sac are covered period; these can be closed in a delayed fash-
with petroleum or saline-moistened gauze. ion (6 months to 1 year). Ruptured omphalo-
U/S evaluation for associated congenital celes will require resuscitation, plastic
defects is performed and karyotype analysis covering, and antibiotics, as in gastroschisis,
is sent if not done previously. than usually staged or delayed closure.
C. Surgical management of gastroschisis con- D. Post-op management for gastroschisis consists
sists of placing abdominal contents within a of antibiotics, bowel rest with parenteral nutri-
silo and slowly reducing them back into the tion (proper intestinal motility delayed by sev-
abdomen over usually several days or, if able, eral weeks), and close monitoring (HD status
closing the defect primarily after safely and abdominal compartment syndrome).
reducing the contents and no resultant
changes in hemodynamic status (evaluating For omphalocele, postoperative management
peak/mean airway pressures and vitals). If depends on time frame of repair but also is con-
there is ischemic bowel, perforation, or cerned with evaluation and management of other
hemodynamic (HD) instability, immediate congenital defects. Abdominal compartment syn-
exploration in the NICU or operating room drome (ACS) should be a concern, like gastros-
for further evaluation may be necessary. A chisis, for immediate repairs, but these patients
newer technique for closure of applying a typically have improved intestinal motility.
132 Omphalocele and Gastroschisis 539

· Abdominal wall defect on prenatal ultrasound (most likely) or at birth
· Evaluate for maternal risk factors: tobacco, socioeconomic, low maternal age
· Elevated maternal serum -fetoprotein evaluate for Gastroschisis
· Vaginal delivery appropriate unless fetal of obstetric concerns
Gastroschisis Omphalocele
A · Abdominal wall defect-Right of · Midline abdominal wall defect
umbilical cord · Sac covering eviscerated contents
· No sac covering eviscerated contents · Associated with cardiac, renal, genetic
defects
B · Evaluate bowel for dilatation, atresia, or

necrosis
· If intact sac over contents, cover with
· Cover contents with saline soaked gauze
petroleum or saline soaked gauze
and place plastic covering to above defect.
· U/S evaluation for associated anomalies and
karyotype sent if not done prenatally
Evidence of Size and Other

perforation, mal- Congenital
YES Abnormalities
rotation, necrosis
and HD instability?
Exploration,
Source Control,
Immediate-
NO & Staged Closure Staged
Primary
Closure
Closure
Immediate- Silo with daily Delayed

Primary Fails/Unable reduction & Closure
closure delayed fascial
closure
Postoperative Management
D · Depends significantly on Immediate (ACS) vs Staged (Silo management) vs Delayed
(Wound care) closure
· Gastroschisis => TPN for intestinal Dismotiliy
· Omphalocele => Evaluation/Management of Congenital Malformations
Algorithm 132.1
References New York: McGraw-Hill Education; 2014.

2. Allman R, Sousa J, Walker MW, Laughon MM,
Spitzer AR, Clark RH. The epidemiology, prevalence
1. Islam S. Abdominal wall defects: omphalocele and
and hospital outcomes of infants with gastroschisis. J
gastroschisis. In: Ziegler MM, Azizkhan RG, Dv
Perinatol. 2016;36(10):901–5.
A, Weber TR, editors. Operative pediatric surgery.
Biliary Atresia
133
Algorithmic Approach caused primarily by elevated conjugated

bilirubin, an investigation into the cause

A. Neonatal jaundice may be present in 50–60% needs to begin [2].
of newborns in the first week of life [1]. More B. There is no definitive test to diagnose biliary
than 50% of patients with biliary atresia may atresia, so the workup consists of ruling out
present with passage of meconium and col- other diseases. Lab tests to be obtained are
ored stools for the first few days of life. liver function tests, including GGT. One will
Typical symptoms of biliary atresia are per- typically see elevated transaminases and
sistently elevated conjugated bilirubin, jaun- GGT, in addition to the hyperbilirubinemia.
dice, acholic stools, and possibly Cultures and biochemical studies to evaluate
hepatomegaly. Once jaundice has persisted for TORCH (Toxoplasmosis, Other (syphilis,
for >2 weeks or the hyperbilirubinemia is varicella-zoster, parvovirus B19), Rubella,
Cytomegalovirus (CMV), and Herpes infec-
tion) infections, hepatitis, alpha 1-antitrypsin
deficiency, should be obtained. An ultrasound
D. W. Parrish allows for the evaluation of the liver contour
Department of Pediatric Surgery, Batson Children’s and biliary anatomy. A HIDA scan or intraop-
Hospital, University of Mississippi Medical Center, erative cholangiogram can demonstrate the
Jackson, MS, USA
biliary anatomy and can rule out biliary atre-
J. H. DeAntonio sia if contrast is seen in the intestines. A liver
Division of Pediatric Surgery, Department of General
Surgery, Virginia Commonwealth University Health, biopsy should be performed to evaluate the
Richmond, VA, USA presence of bile ducts and ductular prolifera-
Department of Surgery, Virginia Commonwealth tion [2, 3].
University School of Medicine, Richmond, VA, USA C. Once the diagnosis of biliary atresia is made,
D. A. Lanning (*) operative timing is key. More favorable out-
Division of Pediatric Surgery, Department of General comes are seen when the definitive procedure
Surgery, Virginia Commonwealth University Health, is performed within 60 days of age, as cirrho-
Richmond, VA, USA
sis begins to develop by 3–4 months of age.
Department of Surgery, Virginia Commonwealth Preoperative steps to take are to maintain
good hydration with intravenous fluid resus-
Department of Surgery and Pediatrics, Children’s citation, broad-spectrum antibiotic coverage,

and fat-soluble vitamin supplementation with ostomy are traditionally divided into thirds.
IM vitamin K, if needed [2, 3]. One-third of patients will have successful
D. The operation of choice for biliary atresia is long-term biliary drainage, one-third will
the Roux-en-Y hepatic portoenterostomy — have temporary drainage but will be older
the Kasai procedure. It has been modified and more able to withstand a liver transplant,
many times, including open and minimally and one-third will receive no relief and will
invasive techniques. The principal steps of progress to liver failure requiring transplan-
the procedure continue to be excision of the tation [3]. Cholangitis is the most common
extrahepatic biliary tree, transection of the postoperative complication following a
portal plate near the hilum of the liver, and Kasai procedure.
biliary drainage with bilioenteric limb [4]. If F. Controversy exists regarding the use of corti-
a choledochal cyst is noted, the cysts are costeroids postoperatively to decrease scarring
excised and a Roux-en-Y hepaticojejunos- and subsequent obstruction at the anastomosis.
tomy is performed. Antibiotics, choleretic agents, and fat-soluble
E. There is a large discrepancy in the morbidity vitamin supplementation are commonly used.
and mortality rates that are presented in the The most common complications after porto-
literature, but successful postoperative out- enterostomy are cholangitis (33–60%) and
comes after Roux-en-Y hepatic portoenter- portal hypertension (34–76%) [2, 3].
133 Biliary Atresia 543

· Infant typically >2 weeks old
A · Persistent conjugated hyperbilirubinemia, jaundice, acholic stools,
hepatomegaly
· Begin workup to rule out other conditions
· Lab tests: CMP (including GGT), cultures to rule out

perinatal infections
· Ultrasound: evaluate liver contour and presence of
gallbladder, bile ducts, or choledochal cysts
B
· HIDA: evaluate passage of contrast into intestines
· Liver Biopsy: evidence of ductular proliferation
· Intraoperative cholangiogram and liver biopsy
C · Intravenous fluid resuscitation as needed

· Preoperative antibiotics
· Oral fat-soluble vitamin supplementation or IM vitamin K
D Proceed with
Roux-en-Y hepatic
portoenterostomy
1/3 of patients will 1/3 of patients will 1/3 of patients will

E have successful long have temporary have no relief from
term biliary drainage biliary drainage but the operation and
will ultimately will progress to
require a transplant liver failure
Complications: Cholangitis, Decreased bile flow, Portal

F Hypertension, Essential Fatty Acid deficiency, Intrahepatic cysts
References 2. Koontz C, Wulkan M. Lesions of the stomach. In:

Holcomb III G, Murphy J, Ostlie D, editors. Ashcraft’s
pediatric surgery. 6th ed. London: Elsevier Inc; 2014.
1.
Practice parameter: management of hyperbiliru-
3. Schwartz M. Hypertrophic pyloric stenosis. In: Coran A,
binemia in the healthy term newborn. American
et al. Pediatric surgery. 7th. London. Elsevier Inc; 2012.
Academy of Pediatrics. Provisional Committee
4. Nio M, Ohi R. Biliary atresia. Semin Pediatr Surg.
for Quality Improvement and Subcommittee on
2000;9:177–86.
Hyperbilirubinemia. Pediatrics. 1994;94(4 Pt
1):558–65.
Part XVII
Vascular
Carotid Artery Stenosis
134
Ian Bailey and Faisal Aziz
Algorithmic Approach carotid endarterectomy (CEA) reduced the

incidence of stroke from 26% to 9% [2].
A. Introduction: Stroke is among the most com- B. Evaluation/ Physical Examination: Key ele-
mon causes of disability in the world. In the ments of the physical examination include a
United States, over 85% of the strokes are neurologic exam, cardiac exam, neck auscul-
considered to be ischemic in origin [1]. The tation, as well as an ocular exam. The neuro-
most common cause of ischemic stroke is logic exam may reveal signs of stroke such as
extracranial carotid artery disease. cranial nerve deficits, pronator drift, and
Management of extracranial carotid disease motor or sensory deficits. An ocular exam
is the cornerstone of ischemic stroke preven- may demonstrate Hollenhorst plaques in the
tion. In majority of the cases, ischemic stroke setting of transient blindness as a result of
is preceded by episodes of transient ischemic retinal artery emboli. Neck auscultation may
attacks (TIA). Typical symptoms of TIA reveal carotid bruits. Finally, a cardiac exam
include dysarthria, amaurosis fugax, and may point against a carotid source of stroke if
hemiparesis. North American Symptomatic an arrhythmia is detected or if a cardiac mur-
Carotid Endarterectomy Trial (NASCET) mur is transmitted through the carotid
showed that in patients presenting with TIAs circulation.
and ipsilateral carotid stenosis of >70%, C. Imaging: Carotid artery duplex ultrasound is
often the first-choice modality which displays
increased peak systolic velocities, diastolic
velocities, and spectral widening with hemo-
I. Bailey dynamically significant lesions.
Division of Vascular Surgery, Penn State Heart and
Vascular Institute, Pennsylvania State University D. Indications for Surgery: Surgical intervention
College of Medicine, Hershey, PA, USA is recommended for patients presenting with
Penn State Health Milton S. Hershey Medical Center, TIAs and >50% ipsilateral carotid stenosis
Hershey, PA, USA and for asymptomatic patients with >80%
F. Aziz (*) carotid stenosis.
Division of Vascular Surgery, Penn State Heart and E. Management: Management options for suit-
Vascular Institute, Pennsylvania State University able patients include carotid endarterectomy
College of Medicine, Hershey, PA, USA (CEA) and carotid artery stenting (CAS).
Department of Surgery, Penn State Milton S. Hershey CREST trial [3] showed that CEA was asso-
Medical Center, Hershey, PA, USA ciated with 4.7% risk of stroke and death

548 I. Bailey and F. Aziz
while CAS was associated with 6.4% risk of F. Postoperative Complications: Postoperative
these complications. Carotid shunt is placed complications for CEA include stroke and
if there is evidence of cerebral ischemia on cranial nerve injuries. CAS is associated with
electroencephalogram (EEG) or if stump stroke and access site complications.
pressure is below 50 mm Hg. These differ- G. Long-term Follow-up: Generally, a carotid

ences were statistically significant. Generally, duplex is performed at 6 weeks and subse-
CEA is the preferred treatment of choice for quently 6 months to determine patency of the
suitable candidates. Patients who are deemed carotid and to monitor the contralateral
high risk for CEA (previous neck radiation, carotid stenosis. Most surgeons recommend
previous carotid surgery, and unfit for general lifelong follow-up with carotid duplex scans.
anesthesia) are best treated with CAS.
A History of TIAs and Carotid Bruit on

neck examination
Thorough physical examination: assessment

B of systemic vascular disease, neurologic
examination, ocular examination
Duplex ultrasound: Degree of carotid

C stenosis
D Symptomatic Carotid Stenosis Asymptomatic Carotid Stenosis

>50% >80%
CEA
(Carotid Shunting, if
indicated)
E Contra indications to CEA (previous

neck radiation, previous carotid
surgery, unfit for general anesthesia)
CAS
Postoperative Care
F
Algorithm 134.1
134 Carotid Artery Stenosis 549
References 3. Brott TG, Hobson RW 2nd, Howard G, Roubin

GS, Clark WM, Brooks W, Mackey A, Hill MD,
Leimgruber PP, Sheffet AJ, Howard VJ, Moore WS,
1. Grysiewicz RA, Thomas K, Pandey DK. Epidemiology
Voeks JH, Hopkins LN, Cutlip DE, Cohen DJ, Popma
of ischemic and hemorrhagic stroke: incidence,
JJ, Ferguson RD, Cohen SN, Blackshear JL, Silver
prevalence, mortality, and risk factors. Neurol Clin.
FL, Mohr JP, Lal BK, Meschia JF, Investigators
2008;26(4):871–95.
CREST. Stenting versus endarterectomy for treat-
2. North American Symptomatic Carotid
ment of carotid-artery stenosis. N Engl J Med.
Endarterectomy Trial Collaborators, HJM B, Taylor
2010;363(1):11–23.
DW, Haynes RB, Sackett DL, Peerless SJ, Ferguson
GG, Fox AJ, Rankin RN, Hachinski VC, Wiebers DO,
Eliasziw M. Beneficial effect of carotid endarterec-
tomy in symptomatic patients with high-grade carotid
stenosis. N Engl J Med. 1991;325(7):445–53.
Abdominal Aortic Aneurysm
135
Erin K. Greenleaf and Faisal Aziz
Algorithmic Approach nal mass, which is widely believed to repre-

sent an impending aneurysmal rupture [2].
A. Definitions: An aneurysm is defined as a focal C. Evaluation/physical examination: Risk fac-
dilatation of an artery to a diameter of at least tors for development of AAA include
1.5 times larger than the expected normal advanced age, male gender, and a history of
diameter. As this pertains to the infrarenal smoking. The positive predictive value of
abdominal aorta, a diameter of 3 cm or greater physical exam for detection and diagnosis of
typically qualifies as aneurysmal. AAA is only 15% [3]. Physical examination
B. Evaluation/history: With the widespread use should also focus on identifying factors that
of cross-sectional imaging, many abdominal may impact intervention, such as surgical
aortic aneurysms are discovered incidentally. scars on the abdominal wall, hernias, and
Others are found on screening exams, as the body habitus.
US Preventative Services Task Force recom- D. Imaging: Screening and monitoring of

mends that men aged 65–75 years who have AAA typically use duplex ultrasonography
ever smoked undergo screening ultrasound as it is low risk and the least invasive. In
[1]. A small proportion of patients present the setting of preoperative planning, com-
with symptomatic AAA, with abdominal and/ puted tomographic angiography (CTA)
or back pain and a palpable pulsatile abdomi- provides greater accuracy and more reli-
able measurements.
E. Indications and management: Any patient
with a reasonable life expectancy and an
E. K. Greenleaf AAA >5 cm, or aortic expansion of 1 cm or
Division of Vascular Surgery, Penn State Heart and
Vascular Institute, Pennsylvania State University more in 1 year, is a candidate to be considered
College of Medicine, Hershey, PA, USA for surgical repair. Surgical operations for the
Penn State Milton S. Hershey Medical Center, treatment of AAA include open surgical
Hershey, PA, USA repair (OSR) and endovascular aortic repair
F. Aziz (*) (EVAR). The character of the aortic neck
Division of Vascular Surgery, Penn State Heart and bears great influence over the suitability for
Vascular Institute, Pennsylvania State University endovascular repair, as length, angulation,
College of Medicine, Hershey, PA, USA diameter, and shape will impact the ability to
Department of Surgery, Penn State Milton S. Hershey obtain an adequate proximal seal. Both OSR
Medical Center, Hershey, PA, USA and EVAR must remain in the armamentarium

552 E. K. Greenleaf and F. Aziz
of the vascular surgeon as conversion to open duplex ultrasound combined with non-con-
repair may be necessary when safe execution trast CT serves as a viable option in patients
of EVAR is not possible; moreover, long-term with renal insufficiency. An endoleak, a com-
outcomes demonstrate that no differences in plication unique to EVAR, occurs due to per-
long-term mortality exist between the two sistent blood flow into the aneurysmal sac
modalities [4]. and if seen at 1 month would necessitate a
F. Postoperative complications: Depending on CTA at 6 months to determine need for re-
the circumstances of the presentation and of intervention. There are five types of endole-
the repair, common complications following aks: Type 1 (incomplete seal between the
AAA repair include cardiac ischemia, renal stent and native vessel – Immediate repair
failure, and ischemia of the sigmoid colon, required), type 2 (blood flow into aneurysm
lower extremities, or spinal cord. Graft- sac via branch vessels of the intrarenal
related complications following EVAR abdominal aorta), type 3 (blood flow between
include endoleaks. Ischemic colitis can pres- the separate components of the graft), type 4
ent with bloody diarrhea, abdominal pain, (blood flow through the graft fabric), and type
distension, fever, leukocytosis, or metabolic 5 (persistently elevated pressure within the
acidosis. aneurysm sac). Thereafter, surveillance fol-
G. Long-term follow-up: Following EVAR, con- lowing EVAR should be continued at least on
trast-enhanced CT scanning should be under- an annual basis. Following open repair, imag-
taken at 1 and 12 months; alternatively, ing should be obtained at least every 5 years.
135 Abdominal Aortic Aneurysm 553
A Presence of risk factors for development of aortic

aneurysm (AAA)
Physical examination reveals pulsatile aortic

B mass
Duplex ultrasound: AAA diagnosis. Infrarenal

C AAA >5.5cm.
Weigh the risks and benefits of surgery. For
patients deemed to be appropriate for surgery,
obtain CTA to delineate anatomy.
Acceptable landing zones and

D access vessels: Feasible for EVAR
Yes No
EVAR Open AAA Repair
E Post-operative Evaluation: Post-operative Evaluation: ischemica

Endoleaks of the colon, lower extremities, or
spinal cord, cardiac ischemia, and
renal failure
Algorithm 135.1
3. Beede SD, Ballard DJ, James EM, Ilstrup DM, Hallet

References JW Jr. Positive predictive value of clinical suspicion
of abdominal aortic aneurysm. Implications for effec-
1. US Preventive Services Task Force: Abdominal tive use of abdominal ultrasonography. Arch Intern
Aortic Aneurysm. Web address: https://www. Med. 1990;150(3):549–51.
uspreventiveservicestaskforce.org/Page/Document/ 4. Schermerhorn M, Dominique B, O’Malley J, Curran
UpdateSummaryFinal/abdominal-aortic-aneurysm- T, McCallum J, Darling J, Landon BE. Long term
screening. Accessed 25 Aug 2017. outcomes of abdominal aortic aneurysm in the medi-
2. Sullivan CA, Rohrer MJ, Cutler BS. Clinical manage- care population. N Engl J Med. 2015;373:328–38.
ment of symptomatic but unruptured abdominal aortic
aneurysm. J Vasc Surg. 1990;11(6):799–803.
Ruptured Abdominal Aortic
Aneurysm 136
Faisal Aziz
Algorithmic Approach D. Imaging: Computed tomography angiography

(CTA) is the standard imaging technology to
A. Definitions: Rupture is the most feared com- diagnose rAAA and to determine the feasibil-
plication of an abdominal aortic aneurysm ity of endovascular repair (EVAR). However,
(AAA). It is a catastrophe associated with it should only be utilized for patients with
mortality rates in excess of 80% [1]. It is the stable hemodynamics. Patients presenting
15th leading cause of death. Elective repair of with hemodynamic instability should be
AAA to prevent rupture is the best strategy rushed to the operating room, where on-table
for dealing with aneurysms. angiography can be used to determine land-
B. Evaluation/history: The blood loss from rup- ing zones required for EVAR placement.
tured AAA (rAAA) is massive and majority E. Management: Ruptured AAA can be repaired
of patients do not make it to the hospital. The with either open surgical operation or endo-
classic description of patients presenting with vascular repair (EVAR). For patients who
rAAA is severe abdominal pain, abdominal present with relatively stable hemodynamics,
distension, and hypotension. Previous history CTA is used to determine the choice of opera-
of a known AAA or a family history of AAAs tion. For patients who are unstable, a percuta-
are useful clues to make a prompt diagnosis. neous sheath can be placed in the common
C. Evaluation/physical examination: a clinician femoral artery on the operating room table
should look for hypotension with tachycar- and a diagnostic aortogram is performed to
dia. Majority of patients in older age groups determine suitability for EVAR. The basic
are on beta-blockers, and this may mask principles for open surgical repair and EVAR
tachycardia. Abdominal examination reveals for ruptured AAA patients are essentially the
firm distension. Timely diagnosis and prompt same as those for elective repair. The key dif-
operation is the only strategy to save lives of ference in patients presenting with ruptured
persons presenting with this fatal diagnosis. AAA is the importance of obtaining quick
inflow control to stop active hemorrhage. In
F. Aziz patients undergoing EVAR, an occlusion bal-
Division of Vascular Surgery, Penn State Heart and loon inserted via femoral access can be used
Vascular Institute, Pennsylvania State University for inflation in aorta and in patients undergo-
College of Medicine, Hershey, PA, USA ing open surgical repair, a supraceliac clamp
Department of Surgery, Penn State Milton S. Hershey can be used to stop active hemorrhage.

556 F. Aziz
F. Postoperative complications: Overall mortal- with EVAR is significantly lower than those
ity for patients with rAAA remains high, with treated with open surgical repair [3].
majority of patients dying even before reach- G. Long-term Follow-up: Long-term follow-up
ing a hospital. Thirty-day mortality for for patients who survive an operation for
patients treated with EVAR is significantly rAAA is not different from those who require
better than those treated with open surgical elective repair. Patients who have undergone
repair (21% vs. 44%) [2]. New-onset postop- EVAR require a close follow-up with CTA at
erative acute renal failure after rAAA surgery 6 months to determine endoleaks, and patients
is the biggest predictor of mortality, and inci- who have stable sac size require less frequent
dence of acute renal failure in patients treated follow-ups.
Triad of Abdominal Pain, Abdominal Distension

A and Hypotension
High Suspicion for rAAA. Determine

B feasibility of getting CTA
Unstable hemodynamics: Directly

C to OR, on table angiography to Stable hemodynamics: Obtain CTA and
determine if candidacy for EVAR determine if candidacy for EVAR
(Landing zone length of at least
D 15mm, neck angulation of <60
degrees)
Suitable for EVAR: EVAR Unsuitable for EVAR: Open

Surgical Repair
E
Key Step: Hemorrhage Control
by supra celiac aortic clamp
Key Step: Hemorrhage Control Ligate IMA if no back bleeding or
by using supra celiac aortic pulsatile back bleeding
occlusion balloon
F G Postoperative Care
Algorithm 136.1
136 Ruptured Abdominal Aortic Aneurysm 557
References 2. Mehta, et al. Endovascular repair of ruptured infra-

renal AAA is associated with lower 30 day mortal-
ity and better 5 year survival rates than open surgical
1. Kantonen I, Lepantalo M, Brommels M, et al.
repair. J Vasc Surg. 2013;57(2):368–75.
Mortality in ruptured abdominal aortic aneurysms.
3. Aziz F, Azab A, Schaefer E, Reed AB. Endovascular
TheFinnvasc study group. Eur J Vasc Endovasc Surg.
repair of ruptured abdominal aortic aneurysm is asso-
1999;17:208–12.
ciated with lower incidence of post-operative acute
renal failure. Ann Vasc Surg. 2016;35:147–55.
Aortic Dissection
137
Katelynn Ferranti and Faisal Aziz
Algorithmic Approach accurate diagnosis of aortic dissection and to

determine its extent. Patients presenting with
A. Definitions: Acute aortic dissection or a
extremely high blood pressure should receive
“tear” in the aortic wall is among the most immediate treatment of HTN. Cardiac exami-
lethal catastrophes affecting the aortic wall. If nation should include auscultation for mur-
left untreated, almost 50% of patients will die murs. The abdominal examination should
within 24 h [1]. Of those who survive the focus on identifying clinical signs of bowel
acute phase of this pathology, few survive ischemia. Pulses should be palpated in all
beyond 5 years due to aneurysmal degenera- four extremities to determine involvement of
tion and rupture of the weakened wall of false one or more peripheral blood vessels as well
lumen [2]. as a motor and sensory exam in each
B. Evaluation/history: Most patients will present extremity.
with acute onset of severe chest or abdominal D. Laboratory testing: Blood work should be

pain, radiating to the back. It is pertinent to obtained and include a CBC, CMP, troponin,
ask about history of previous, family history and lactic acid. This will help to identify signs
of aortopathies and history of hypertension. of end-organ malperfusion such as cardiac,
C. Evaluation/physical examination: A thorough liver, kidney, or intestinal ischemia.
physical examination is key to making an E. Imaging: Emergent computed tomography
angiography (CTA) should be obtained
including the chest, abdomen, and pelvis.
K. Ferranti Emergent transthoracic echocardiogram
Division of Vascular Surgery, Penn State Heart and should be obtained to determine the involve-
Vascular Institute, Pennsylvania State University
College of Medicine, Hershey, PA, USA ment of aortic valve, pericardial effusion, and
proximal extent of dissection.
Department of Vascular Surgery, Penn State Health,
Milton S. Hershey Medical Center, F. Indications: Indications for emergent surgical
Hershey, PA, USA treatment include type A aortic dissection
F. Aziz (*) (involving the ascending aorta) and involve-
Division of Vascular Surgery, Penn State Heart and ment of visceral arteries (celiac artery, supe-
Vascular Institute, Pennsylvania State University rior mesenteric artery, and renal arteries) and
College of Medicine, Hershey, PA, USA iliac arteries.
Department of Surgery, Penn State Milton S. Hershey G. Management: Surgical management of aortic
dissection is complicated and requires sound

560 K. Ferranti and F. Aziz
clinical judgment. Type A dissections need significantly high risk of mortality and
emergent surgical repair by aortic arch postoperative complications including
replacement. Type B dissections involving damage to the bowels and kidneys and
mesenteric blood vessels are treated with limb loss.
endovascular repair (TEVAR), fenestrations, I. Long-term follow-up: Patients surviving
or mesenteric bypasses. Type B dissections acute aortic dissections need to be followed
compromising lower extremity arterial flow for the rest of their lives with serial CTAs to
are managed by TEVAR, fenestrations, extra- monitor for development of aneurysmal
anatomic bypasses, or iliac stenting. degeneration, which may require further
H. Postoperative complications: Operations
intervention.
for aortic dissections are associated with
137 Aortic Dissection 561
Clinical scenario:
60 y.o. Man with PMHx of HTN presents with severe stabbing
chest pain that radiates to back.
Vital signs, Physical exam

Laboratories: CBC, BMP, Lactic Acid, Troponin
EKG
VS: Tachycadia? HTN?
PE: New Murmur? Abdominal pain or

tenderness? Lower extremity pain,
tenderness, pulses, palor?
Labs:Leukocytosis? Elevated Creatinine?

Lactic acidosis? Elevated Troponin?
CTA Chest/Abdomen/Pelvis
Stanford type A dissection Stanford type B dissection
Surgical emergency!
Consult cardiac surgery
OR
562 K. Ferranti and F. Aziz
Stanford type B dissection
Anti-impulse therapy:
Blood pressure control: Goal SBP 100-120
Heart rate control: Goal HR <60 bpm
Absence of Lower extremity

Bowel ischemia ischemia
malperfusion
Malperfusion?
1st line: Labetalol drip

OR for revascularization OR for revascularization
or esmolol drip
2nd line: Nicardipine drip
3rd line: Nitroprusside
drip (after beta blocker)
Unilateral ischemia:
Arterial Line Surgical options: · Fem-Fem
· TEVAR · Iliac stent
Admit to ICU · Endovascular aortic
fenestration Bilateral ischemia:
Serial abdominal and · Open aortic · Endovascular aortic
lower extremity exams fenestration fenestration
· Mesenteric bypass · Open aortic
fenestration
Algorithm 137.1
137 Aortic Dissection 563
References 2.
Crawford ES, Crawford JL, Safi HJ, Coselli
JS, Hess KR, Brooks B, Norton HJ, Glaeser
DH. Thoracoabdominal aortic aneurysms: preopera-
1. Khan IA, Nair CK. Clinical, diagnostic, and man-
tive and intraoperative factors determining immediate
agement perspectives of aortic dissection. Chest.
and long-term results of operations in 605 patients. J
2002;122(1):311–28.
Vasc Surg. 1986;3(3):389–404.
Acute Lower Extremity Ischemia
138
Algorithmic Approach D. In the setting of profound ischemia >6–8 h,

there is an unlikely potential for limb salvage
A. Acute limb ischemia (ALI) must be recog- (nonviable limb). The risk of multi-organ fail-
nized rapidly in order to limit the skeletal ure and cardiovascular collapse is also high
muscle damage. A comprehensive history is with reperfusion and circulation of ischemic
necessary in order to determine the cause of metabolites. However, if pain can be con-
thrombosis/embolization. Possible causes trolled and there is no evidence of infection,
include atrial fibrillation, left ventricular amputation may be deferred to meet patient
thrombus, aortic dissection, trauma, hyperco- needs.
agulable state, thrombosis, history of MI, E. Follow-up schedule: Patient should follow
CHF or possible endocarditis, history of DVT up at 4–6 weeks, 6 months, and 12 months in
with patent foramen ovale, or a family history the first year and yearly thereafter. Clinical
of thrombosis. evaluation of cardiovascular risk factors,
B. Medical therapy for ALI: In all patients, sys- functional status, adherence to medical ther-
temic anticoagulation with IV unfractionated apy and smoking cessation should be reas-
heparin is recommended unless contraindi- sessed at each subsequent visit. For patients
cated, as it limits thrombus propagation. If with revascularization, reassess limb symp-
history of heparin-induced thrombocytopenia toms and interval change in functional sta-
is suspected, a direct thrombin inhibitor is tus, pulse examination, and ABI. Change in
recommended. ABI >0.15 is clinically significant. Duplex
C. Revascularization using catheter-directed ultrasound for routine surveillance of
thrombolysis (CDT) or surgical thromboem- infrainguinal, autogenous vein bypass grafts
bolectomy has demonstrated similar limb sal- or after endovascular revascularization is
vage rates but better survival with CDT [1–5]. recommended.
A. Aurshina
Department of Vascular Surgery, Vascular Institute
of New York, Brooklyn, NY, USA
A. Hingorani (*)

566 A. Aurshina and A. Hingorani
A Definition: Acute (<2 week), severe hypoperfusion of the limb characterized by

features: pain, pallor, pulselessness, poikilothermia, paresthesia and paralysis. (6P’s)
Patient History to assess symptom duration, pain intensity, sensory and motor nerve deficit. Physical exam:
Vascular exam of pulses/ bruit. Assess for signs and symptoms of myonecrosis. Clinical bedside assessment:
arterial and venous exam with hand held Doppler. (Class I)
Audible arterial Inaudible arterial Inaudible arterial

Audible venous Audible venous Inaudible venous
Assess motor Category III: Irreversibly

Category I: Viable limbs3 function non-viable3
Normal sensory/motor function Absent capillary Refill Complete
Intact Capillary Refill Sensory Loss Complete motor
function loss
Category IIa: Category IIb:

Marginally threatened3 Immediately threatened3
Slow to normal capillary Refill Slow to absent capillary Refill
Sensory Loss limited to toes Sensory loss more than toes with D
No motor weakness ischemic rest pain
B Mild/Moderate motor weakness
Anticoagulation first, then Primary amputation (Class I)

revascularization (urgent: Salvageable if treated Salvageable if treated
within 6-24 h), unless promptly emergently
contraindicated (Class I)
Anticoagulation followed by revascularization

(Emergency, within 6 h), unless contraindicated
(Class I)1
C
Revascularization options: Catheter-Directed Thrombolysis (Class I), Percutaneous mechanical

thrombectomy with/without TPA (Class IIa) & Open Surgical Revascularization (Class IIa)4, 5
Monitor and Treat for compartment syndrome after revascularization with fasciotomy for elevated
pressures (Symptoms: Increased pain, muscle tenderness, tense muscle, pain on passive flexion,
nerve Injury)
Follow-up care E
Algorithm 138.1
138 Acute Lower Extremity Ischemia 567
References ACC guideline on the management of patients with

lower extremity peripheral artery disease: a report of
the American College of Cardiology/American Heart
1. Berridge DC, Kessel D, Robertson I. Surgery ver-
Association Task Force on clinical practice guide-
sus thrombolysis for acute limb ischaemia: ini-
lines. Circulation. 2017;135(12):e726–e79.
tial management. Cochrane Database Syst Rev.
4. Norgren L, Hiatt WR, Dormandy JA, Nehler MR,
2000;4:CD002784.
Harris KA, Fowkes FG. Inter-society consensus
2. Ouriel K, Veith FJ, Sasahara AA. Thrombolysis or
for the Management of Peripheral Arterial Disease
peripheral arterial surgery: phase I results. TOPAS
(TASC II). J Vasc Surg. 2007;45(Suppl S):S5–67.
investigators. J Vasc Surg. 1996;23(1):64–73. discus-
5. Rutherford RB, Baker JD, Ernst C, Johnston KW,
sion 4-5.
Porter JM, Ahn S, et al. Recommended standards
3. Gerhard-Herman MD, Gornik HL, Barrett C, Barshes
for reports dealing with lower extremity ischemia:
NR, Corriere MA, Drachman DE, et al. 2016 AHA/
revised version. J Vasc Surg. 1997;26(3):517–38.
Chronic Lower Extremity Ischemia
139
Algorithmic Approach C. The goal of surgical or endovascular revascu-

larization should be to provide blood flow
A. After proper history and physical examina- through at least one patent artery, help
tion, the ankle brachial index is the best initial decrease ischemic pain, and allow healing of
test to diagnose chronic lower extremity isch- ulcers while preserving a functional limb and
emia and evaluate the next steps in minimizing tissue loss and need for major
management. amputation. The decision of open or endovas-
B. In patients with chronic limb ischemia, timely cular procedure is based on a patient-focused
diagnosis and treatment are essential to pre- approach after proper cardiovascular risk
serve tissue viability; therefore, invasive assessment [3].
angiography and expeditious revasculariza-
tion are most effective to avoid delay due to
additional noninvasive imaging (Class I rec-
ommendation) [1, 2].
A. Aurshina
Department of Vascular Surgery, Vascular Institute of
New York, Brooklyn, NY, USA
A. Hingorani (*)

Definition: Chronic (>2 week) ischemic rest pain, non-healing wound/ulcers, or

gangrene in 1 or both legs attributable to objectively proven arterial occlusive disease.
History and Physical exam of lower extremity including: Inspection of legs/feet

A for signs of PAD, non-healing wound/ulcer or gangrene.
Initial Test: Ankle Brachial Index

(ABI) (Class I)3
Abnormal ABI: <0.90 Borderline ABI: 0.91–0.99 Non-compressible arteries

Normal ABI: 1.00–1.40 ABI >1.40
Assess for local perfusion

especially if ABI <0.70 Non-healing ulcer or gangrene + Toe Brachial Index
(TBI)
YES NO
Pulse volume Abnormal Normal

recordings (PVR) (TBI <0.7) (TBI >0.7)
B
Abnormal
Normal Search for
alternate diagnosis
Further anatomical
assessment: Endovascular
Cardiovascular revascularization
· Duplex ultrasound risk assessment
· CTA or MRA C
· Invasive angiography
(Class I)
Open procedure
Algorithm 139.1
139 Chronic Lower Extremity Ischemia 571
References 3. Gerhard-Herman MD, Gornik HL, Barrett C, Barshes

ACC guideline on the management of patients with
1. Norgren L, Hiatt WR, Dormandy JA, Nehler MR,
Harris KA, Fowkes FG. Inter-society consensus for
the management of peripheral arterial disease (TASC
Association task force on clinical practice guidelines.
II). J Vasc Surg. 2007;45(Suppl S):S5–67.
Circulation. 2017;135(12):e726–e79.
2. Rutherford RB, Baker JD, Ernst C, Johnston KW,
Porter JM, Ahn S, et al. Recommended standards
for reports dealing with lower extremity ischemia:
revised version. J Vasc Surg. 1997;26(3):517–38.
Intermittent Claudication
140
Algorithmic Approach dorsalis pedis (DP) or posterior tibial (PT) pres-

sure by the higher of the right- or left-arm blood
A. To diagnose patients at increased risk of
pressure for each leg. Segmental blood pressures
peripheral arterial disease (PAD), a proper and Doppler waveforms (pulse volume record-
history and physical exam is necessary. ings) can be used to further localize the anatomi-
Table 1 summarizes the common risk factors cal segments of disease (e.g., aortoiliac,
for PAD (Table 140.1). femoropopliteal, or infrapopliteal) [2, 3].
B. It is essential rule out other causes of lower D. Supervised exercise program for 6 weeks
extremity pain which may mimic claudica- and conservative medical management are
tion. Table 2 summarizes the differential the first steps in management. However, if
diagnosis for leg pain/claudication [1] claudication persists, consider revascular-
(Table 140.2). ization [4].
C. Ankle-brachial index (ABI) is the best initial test
and is calculated by dividing the higher of the
Table 140.2 Differential diagnosis for leg pain/claudica-
tion (Non-PAD related with normal ABI)
Symptomatic Increases with Present at rest
Table 140.1 Patient at increased risk for PAD Bakers cyst exercise
Age >65 years Venous Increases after Subsides
Claudication walking slowly,
Age 50–64 years with Risk Factors for atherosclerosis
improves with
(DM, HTN, smoking, hyperlipidemia, family Hx of
elevation
PAD)
Chronic After much Subsides very
Age <50 years with DM and one additional RF for
Compartment exercise (Jogging) slowly
atherosclerosis
Syndrome
Individual with known atherosclerotic disease
Spinal Stenosis Mimics Variable, relief
(Coronary, Carotid, renal, Mesenteric, AAA)
claudication; by spinal
onset with flexion
variable distance
Nerve Root Induced by Present at rest,
A. Aurshina Compression sitting, standing improved by
Department of Vascular Surgery, Vascular Institute of or walking change in
New York, Brooklyn, NY, USA position
A. Hingorani (*) Hip/ Foot/Ankle After variable Improves
Division of Vascular Services, NYU Langone Arthritis degree of exercise when
Hospital-Brooklyn, Brooklyn, NY, USA non-weight
bearing

Definition: Pain or cramping of vascular origin in the muscles of lower extremity that is
induced by walking consistently at fixed distance & relieved by rest (within 10 minutes)
A
A thorough lower extremity vascular examination including:

· Inspection of legs/feet for signs of peripheral artery disease
· Assessment of LE pulse: DP, PT, Fem, Pop – Abnormal/Absent
B
Initial Test: Resting Ankle Brachial Index (ABI) with or without segmental
pressures and waveforms (Grade IA recommendation)2, 4
· <0.90: Diagnostic for PAD
· 0.91–0.99: Borderline C
· 1–1.4: Normal. No further testing needed.
· >1.4: Non-compressible likely due to arterial calcification
IF PAD+: ABI <0.90 If borderline/normal If ABI>1.4: non-

in suspected PAD compressible artery
Noninvasive BP in both arms patients:
to rule out subclavian artery Exercise Treadmill
stenosis/ UE PAD. ABI
Toe Brachial Index TBI >0.7, Search

Exercise Treadmill ABI or (TBI) <0.7 is for alternative
Symptoms not diagnostic of PAD diagnosis
6 minute walk test very suggestive
of PAD
Supervised Exercise Search for

Program (SEP) for 6 alternative
weeks. diagnosis
D
Medical Management:
Cilostazol
Antiplatelet drugs Anatomic Assessment (Class I):
Statin therapy If claudication persistent
Duplex Ultrasound with SEP, consider
CTA/MRA based on availability revascularization
(Grade 1B)2
No response to conservative
management Invasive Angiography (Class II)
Algorithm 140.1
140 Intermittent Claudication 575
References asymptomatic disease and claudication. J Vasc Surg.

61(3):2S–41S.e1.
3. Shishehbor MH, Hammad TA, Zeller T, Baumgartner
I, Scheinert D, Rocha-Singh KJ. An analysis of
IN.PACT DEEP randomized trial on the limitations of
the societal guidelines-recommended hemodynamic
parameters to diagnose critical limb ischemia. J Vasc
Surg. 2016;63(5):1311–7.
Association Task Force on clinical practice guide-
lines. Circulation. 2017;135(12):e726–e79.
NR, Corriere MA, Drachman DE, et al. 2016. AHA/
2. Conte MS, Pomposelli FB, Clair DG, Geraghty PJ,
McKinsey JF, Mills JL, et al. Society for vascular
lower extremity peripheral artery disease: executive
surgery practice guidelines for atherosclerotic occlu-
summary. Vasc Med. 2017;22(3):Np1–np43.
sive disease of the lower extremities: management of
Acute Deep Venous Thrombosis
141
Algorithmic Approach early/late complications including extension,

pulmonary embolism, major bleeding, and
A. The first step in the evaluation of acute deep death. Late complications include recurrent
vein thrombosis (DVT) is a proper history and clot and post-thrombotic syndrome [3].
physical examination. The Modified Wells Assess for bleeding risk and hepatic/renal
score (Table 141.1) should be used to evaluate insufficiency especially with use of direct
the risk and next step in management. oral anticoagulants (DOACs).
B. Unprovoked DVT is defined as no idenfiable E. Patients with phlegmasia cerulea dolens (ilio-
provoking event for DVT. In contrast, provoked femoral DVT) should be considered for more
DVT is due to a known risk factor. The tran- aggressive management, usually catheter-
sient major risk factors include major surgery, directed thrombolysis and/or surgical or
hospitalization, or immobility >3 days. Minor mechanical thrombectomy. Intravenous (IV)
risk factors include minor surgical procedures, unfractionated heparin should be initiated
pregnancy, or estrogen therapy. Persistent risk early with no delay prior to procedure [4].
factors include reversible risk factors (malig-
nancy or inflammatory bowel disease) and irre-
versible risk factors (inherited thrombophilia, Table 141.1 Modified wells criteria [1]
CHF, and metastatic cancer) [2]. Active cancer 1
C. Proximal DVT has higher risk of complica- Bedridden recently >3 days or major surgery 1
tion and includes thrombus in popliteal, within 4 weeks
femoral, or iliac veins. Isolated distal DVT is Calf swelling >3 cm compared to the other leg 1
confined to the calf veins. Measured 10 cm below tibial tuberosity 1
Collateral (nonvaricose) superficial veins present 1
D. Anticoagulation is the mainstay treatment of
Entire leg swollen 1
choice for acute DVT with the primary objec-
Localized tenderness along the deep venous 1
tive of prevention of further thrombosis and system
Pitting edema, confined to symptomatic leg 1
Paralysis, paresis, or immobilization of the lower 1
A. Aurshina extremity
Department of Vascular Surgery, Vascular Institute of Previously documented DVT 1
New York, Brooklyn, NY, USA Alternative diagnosis to DVT as likely or more −2
likely
A. Hingorani (*)
Division of Vascular Services, NYU Langone Score of 0 or less, DVT is unlikely
Hospital-Brooklyn, Brooklyn, NY, USA Score or 1–2, risk of DVT is moderate
Score of >2, risk of DVT is likely
Patient presents with signs and symptoms of acute DVT: pain, swelling, tenderness,
inflammatory signs of warmth and redness
Excluding other causes

A based on H&P
Clinical suspicion of DVT
B Determine Pretest probability using Modified Wells Score1

Assess for known risk factors of DVT
Low Intermediate or High

Perform D-Dimer assay Perform compression ultrasonography
Negative Positive Negative Positive

DVT excluded Perform compression DVT excluded DVT confirmed
ultrasonography
C
Contraindication to anticoagulation? Proximal DVT Distal DVT
Yes No contraindication High risk Low risk /

D contraindicated
anticoagulation
Phlegmasia No phlegmasia No contraindication

IVC filter cerulea dolens to anticoagulation
cerulean dolens
Retrievable or
Permanent filter; follow OBSERVE, repeat
for 3-6 months with proximal compressive
anticoagulation when Consider ultrasound in 1–2
Initial Anticoagulation weeks
contraindication resolves thrombolytics or Heparin/Warfarin or
thrombectomy LMWH for 3–6 months
Alternative: DOAC3
E
DVT extension Stable, no
Consider long-term Provoked DVT into or towards extension,
Unprovoked proximal DVT/ with identified continue
indefinite anticoagulation the proximal
Recurrent DVT/ Persistent DVT risk factors: STOP surveillance
Follow-up with risk- veins, patient
benefit assessment anticoagulation at risk of
after 3–6 months extension
Algorithm 141.1
141 Acute Deep Venous Thrombosis 579
References 3. Kearon C, Akl EA, Ornelas J, Blaivas A, Jimenez

D, Bounameaux H, et al. Antithrombotic therapy for
VTE Disease: CHEST guideline and expert panel
1. Wells PS, Anderson DR, Rodger M, Forgie M, Kearon
report. Chest. 2016;149(2):315–52.
C, Dreyer J, et al. Evaluation of D-dimer in the diag-
4. Meissner MH, Gloviczki P, Comerota AJ, Dalsing
nosis of suspected deep-vein thrombosis. N Engl J
MC, Eklof BG, Gillespie DL, et al. Early thrombus
Med. 2003;349(13):1227–35.
removal strategies for acute deep venous thrombosis:
2. Kearon C, Ageno W, Cannegieter SC, Cosmi B,
clinical practice guidelines of the Society for Vascular
Geersing GJ, Kyrle PA. Categorization of patients as
Surgery and the American Venous Forum. J Vasc
having provoked or unprovoked venous thromboem-
Surg. 2012;55(5):1449–62.
bolism: guidance from the SSC of ISTH. J Thromb
Haemost. 2016;14(7):1480–3.
Management of Acute Mesenteric
Ischemia 142
Josh Radtka
Algorithmic Approach occlusion. NOMI occurs in 20% of cases and

is typically due to a low flow start due to
A. The first step in the management of a patient shock or vascocontstrictors [3].
with acute mesenteric ischemia is the history
D. An emergent operative intervention will be
and physical exam. The diagnosis of acute required if the CTA demonstrates an arterial
mesenteric ischemia requires a high index of occlusion. The patient should receive intrave-
suspicion. These patients usually develop nous fluids, antibiotics, and a heparin drip.
sudden onset of acute abdominal pain. The Restoration of blood flow should be attempted
physical exam is classically characterized by by an embolectomy/thrombectomy of the SMA.
abdominal pain that is more severe than E. If an embolectomy is unsuccessful, the patient
would be expected; however, this classic may require an open bypass. This can be per-
description of abdominal pain may be absent formed in a retrograde fashion originating
in 20–25% of individuals [1]. The patients from either the aorta or iliac artery. Another
can have leukocytosis or an elevated lactic option would be retrograde stenting of the
acid level. The prompt diagnosis of this con- superior mesenteric artery (SMA) occlusion
dition is important as there is only a 30% sur- through the previous embolectomy incision.
vival rate when the diagnosis is delayed more F. Once arterial flow has been restored, any non-
than 24 h [2]. viable bowel should be resected. The patient’s
B. When acute mesenteric ischemia is suspected, abdomen should be left open and transported
the first test that should be performed is a to the ICU for further resuscitation. In
computed tomography angiogram (CTA) of 24–48 h after the initial procedure, the patient
the abdomen and pelvis. This is a rapid study should return to the operating room for a re-
that can provide information regarding the exploration. At this time, any non-viable
diagnosis and cause of acute mesenteric isch- bowel should be removed. Once all non-
emia, as well as the viability of the bowel [2]. viable bowels have been resected, the abdo-
C. The etiology of the patient’s symptoms could men can be closed. An echocardiogram with
be secondary to NOMI, or another source, if agitated saline and a CTA of the chest should
the CTA fails to show evidence of an arterial be performed to determine a potential source
of the vascular occlusion if an embolism is
J. Radtka () believed to be the source of the acute mesen-
Division of Vascular Surgery, Penn State Milton teric ischemia.

582 J. Radtka
History:
Sudden onset of severe diffuse abdominal pain
A
Obtain vital signs, blood work, perform a physical exam,
Vital Signs: May demonstrate fever, tachycardia, hypotension

Labs: May demonstrate leukocytosis and elevated lactic acid
PE: Abdominal pain that is more severe then would be expected
No arterial
CTA of occlusion, possible C
B Abdomen and NOMI, or another
Pelvis diagnosis
Arterial occlusion
D Start heparin drip, ABX, IVF, Exploratory Laparotomy
SMA embolectomy/thrombectomy
Arterial Flow
Restored?
No
E Bypass or Retrograde Yes

stenting
F Bowel Resection and Second look

Laparotomy
Follow-up Care: Echo with agitated saline;

and CTA of the chest
Algorithm 142.1
142 Management of Acute Mesenteric Ischemia 583
References 3. Cheng CC, Choi L, Cheema Z, Silva MB Jr. Acute

mesenteric ischemia. Current surgical therapy. In:
Cameron JL, Cameron AM, editors. Current surgi-
1. Jimenez, Quinones-Baldrich. Mesenteric vascular dis-
cal therapy. 11th ed. Philadelphia: Elsevier; 2014.
ease: general considerations. In: Cronenwett J, editor.
p. 939–46.
Rutherford’s vascular surgery. Philadelphia: Elsevier;
2010. p. 2260–1.
2. Wyers. Mesenteric vascular disease: acute ischemia.
In: Cronenwett J, editor. Rutherford’s vascular sur-
gery. Philadelphia: Elsevier; 2010. p. 2292–4.
Management of Chronic
Mesenteric Ischemia 143
Josh Radtka
Algorithmic Approach scans, and endoscopies. If another diagnosis

is suspected, it should be investigated and
A. The first step in the management of a patient treated prior to the treatment of CMI.
with chronic mesenteric ischemia is the his- C. After ruling out other possible etiologies, the
tory and physical exam. The classic descrip- patient should be evaluated for chronic mes-
tion of a patient with chronic mesenteric enteric ischemia. The diagnosis of CMI
ischemia is mid-epigastric abdominal pain requires that two of the three mesenteric ves-
15–45 min after a meal due to the inability of sels have severe stenosis or occlusion and one
the vascular supply to meet metabolic of these vessels needs to be the superior mes-
demands of the visceral organs [1]. The enteric artery. The work-up begins with a
underlying etiology of this inadequate blood duplex of the mesenteric arteries. This modal-
supply is arterial stenosis of the mesenteric ity has minimal patient risk and provides
vessels. These patients tend to experience a dynamic information about the degree of ste-
significant weight loss and present with a nosis within the mesenteric vessels. Bowel
cachectic appearance. Weight loss is typically gas and patient body habitus may obscure the
due to “food fear” or an avoidance of eating visualization of the vessels. A computed
in order to limit their postprandial abdominal tomography angiogram (CTA) of the abdo-
pain. Similar to arterial occlusive disease men and pelvis provides anatomic informa-
patients, a history of smoking is common. tion about the extent and degree of stenosis in
Although these patients tend to have abdomi- the mesenteric vessels. The gold standard
nal pain, they do not usually have peritoneal method of diagnosis is an aortogram. Though
signs. A mid-epigastric bruit may be an aortogram is invasive, it allows for imme-
appreciated. diate therapeutic intervention.

B. The majority of these chronic mesenteric D. After the diagnosis of chronic mesenteric

ischemia (CMI) patients are initially referred ischemia has been made, the treatment
to a gastroenterologist. Patients commonly options are either an endovascular interven-
undergo an extensive work-up which may tion or an open bypass. Medical management
include x-rays, computed tomography (CT) is not successful as it does not treat the under-
lying etiology. The stenosis of the mesenteric
J. Radtka (*) arteries occurs most commonly at the orifice
Division of Vascular Surgery, Penn State Milton of the artery. This allows for the endovascular
S. Hershey Medical Center, Hershey, PA, USA stenting of the origins of the celiac and

586 J. Radtka
superior mesenteric arteries as a possible in many institutions. Open bypass options

treatment option. A balloon expandable stent include an antegrade aorto-mesenteric, a ret-
is often utilized during endovascular treat- rograde aorto-mesenteric, or an ileo-mesen-
ments. Currently, endovascular techniques teric artery bypass [2].
for revascularization are the first-line therapy
History:
A Patients typically present with complaints of abdominal pain
30 minutes after meals, food fear, and weight loss
Obtain Vital Signs, blood work, and perform a

physical exam
Due to chronicity, vital signs are often normal

PE: Patient with diffuse mild abdominal pain and mid-epigastric
bruit Patient is Cachectic
Positive
B Standard GI Treat Other
Workup Pathology
Negative
Evaluate for Negative

C Continue to evaluate
Mesenteric
for other etiologies
Stenosis
Positive
D Revascularization Procedure:
Endovascular or Open
Algorithm 143.1
143 Management of Chronic Mesenteric Ischemia 587
References 2. Schwartz, et al. Diagnosis and surgical management

of the visceral ischemic syndromes. In: Moore W, edi-
tor. Vascular and endovascular surgery. Philadelphia:
1. Huber, Lee. Mesenteric vascular disease: chronic isch-
Elsevier; 2013. p. 423–35.
emia. In: Cronenwett J, editor. Rutherford’s vascular
surgery. Philadelphia: Elsevier; 2010. p. 2274–5.
Thoracic Outlet Syndrome
144
Tarik Z. Ali and Josh Radtka
Algorithmic Approach B. In order to determine if the extremity swell-

ing is secondary to venous thrombosis, a
A. Thoracic outlet syndrome (TOS) is a constel- venous duplex should be performed. If there
lation of upper extremity symptoms resulting is no thrombus present, then an alternative
from compression of the brachial plexus, sub- diagnosis for the upper extremity swelling
clavian artery, and subclavian vein as they should be sought. A chest X-ray should be
pass between the clavicle and the first rib. The obtained to determine if cervical ribs or
majority of patients are between 20 and anomalous first ribs are present. If the patient
50 years of age and about 70% are females is suspected to have neurogenic TOS, the
[1]. There are three types of TOS: neurogenic, Adson test, elevated arm stress test, or the
arterial, and venous. The history and physical Elvey test are maneuvers that may be helpful
exam are important in differentiating the dif- in establishing a diagnosis.
ferent types of thoracic outlet syndromes. C. If a subclavian-axillary vein thrombosis is
Neurogenic is the most common type and is discovered, patient should be considered for
usually a diagnosis of exclusion. Common pharmaco-mechanical thrombolysis. Long-
symptoms are pain, paresthesia, and weak- term anticoagulation alone has been associ-
ness in the affected extremity without local- ated with significant disability [3]. Prior to
ization to a particular nerve distribution [2]. thrombolytic therapy, the patient’s history
Arterial TOS occurs when compression of the should be reviewed to ensure that there are no
subclavian artery creates an aneurysm distal any absolute contraindications to treatment.
to the site of compression. This could cause After successful thrombolysis, definitive
embolization distally, leading to ischemic treatment includes venous stent placement in
symptoms. Venous TOS or Paget-von the offending vessel.
Schrotter syndrome occurs when repetitive D. Once the thrombus is removed and the venous
movement in the compressed vein results in narrowing is identified, extrinsic compression
endothelial damage and can lead to subcla- needs to be corrected with a first rib resection.
vian and axillary vein thrombosis. This leads The timing of this intervention is controver-
to swelling, pain, and possible cyanosis. sial and has been recommended to occur from
immediate to 3 months post thrombolysis [3].
T. Z. Ali · J. Radtka (*) After the first rib is removed, venous stenosis
Division of Vascular Surgery, Penn State Milton requires treatment with either open or endo-
S. Hershey Medical Center, Hershey, PA, USA vascular methods.

590 T. Z. Ali and J. Radtka
History:
The presentation can be variable based on the type of TOS. Neurogenic symptoms may be
chronic while the vascular symptoms may be acute.
Obtain vital signs, perform a physical exam

A
Physical Exam
Findings
Pain without vascular

Embolic/Ischemic Event Arm Swelling
compromise
CXR
B Arterial and Venous Duplex
Arterial Duplex,
Venous Duplex of RUE
CT Angiogram
MRI
Thrombolysis of
C Physical Therapy Revascularization Axillary and
Subclavian Veins
D First Rib Resection First Rib Resection

First Rib Resection Possible Arterial Possible Venous
Reconstruction Reconstruction
Follow-up Care
Algorithm 144.1
144 Thoracic Outlet Syndrome 591
References 2. Thompson, Driskill. Thoracic outlet syndrome: neuro-

genic. In: Cronenwett J, editor. Rutherford’s vascular

1. Sanders. Thoracic outlet syndrome: general con-
3. Schanzer, Messina. Thoracic outlet syndrome: general
siderations. In: Cronenwett J, editor. Rutherford’s
considerations. In: Cronenwett J, editor. Rutherford’s
vascular surgery. Philadelphia: Elsevier; 2010.
vascular surgery. Philadelphia: Elsevier; 2010.
p. 1865–77.
p. 1907–17.
AV Shunt Complications
145
Josh Radtka
Algorithmic Approach alysis catheter should be placed preferentially

in the right internal jugular vein [1]. Following
A. It is important to take a good history and hemodialysis the occluded dialysis access
physical exam in a patient with an arterial should be de-clotted.
venous (AV) shunt problem. Vital informa- D. The occluded dialysis access should be de-
tion includes the following: is the access a clotted with either open or endovascular sur-
fistula or graft, when it was created, when gical techniques [2].
was the last dialysis session, and if any prob-
E. Following a successful procedure to re-
lems were occurring with the access (i.e., par- establish blood flow through the access, it is
esthesia in the digits could represent steal, important to evaluate the access for the cause
and a prolonged time prior to hemostasis after of failure. This can be performed best with a
dialysis could represent venous stenosis). fistulagram. This procedure will be both diag-
During the physical exam, it is important to nostic and therapeutic. It will allow for treat-
assess the access for a thrill and a bruit. A ment of common causes of dialysis access
vascular exam of the affected extremity occlusion such as arterial anastomotic steno-
should also be performed. If you are unable to sis, venous anastomotic stenosis, and central
determine if the access is patent, a duplex vein stenosis. After the cause of failure is dis-
should be obtained. covered and corrected, the access can again
B. Blood work should be obtained and include a be used for dialysis.
basic metabolic panel and a complete blood F. If the dialysis access cannot be de-clotted,
count. It is important to determine if the then a long-term dialysis catheter should be
patient requires emergent dialysis prior to any placed. This catheter should be placed prefer-
intervention on the dialysis access. Common entially in the right internal jugular vein. The
reasons to perform emergent dialysis are ure- patient will then require a work-up for place-
mia, fluid overload, acidosis, and ment of a new hemodialysis access. This may
hyperkalemia. require ultrasound-guided vein mapping or
C. If the patient requires hemodialysis and the moving the site of access creation to another
access is occluded, then a temporary hemodi- extremity.
J. Radtka ()
Division of Vascular Surgery, Penn State Milton

594 J. Radtka
History:
A Patient had dialysis 1 day ago through a left AV Graft, which
now no longer has a thrill
Obtain Vital Signs, blood work, perform

a physical exam,
PE: No Thrill in Graft on exam.
Is emergent
B hemodialysis
required?
Yes
C Place Temporary Dialysis

No
Catheter
Surgical intervention to
D restore blood flow to
access successful?
Yes No
Evaluate for cause of failure Place a permanent catheter

E Evaluate for other permanent F
Use for Hemodialysis
dialysis options
Algorithm 145.1

2. Meier G. Hemodialysis access: failing and throm-
References bosed. In: Cronenwett J, editor. Rutherford’s vascular
1. Macasta, Sidawy. Hemodialysis access: general con-
siderations. In: Cronenwett J, editor. Rutherford’s vas-
cular surgery. Philadelphia: Elsevier; 2010. p. 1105–6.
Part XVIII
Genitourinary
Management of the Renal Mass
146
J. Chris Riney, Neil J. Kocher, and Matthew Kaag
Algorithmic Approach tain internal septa, solid components, or cal-

cifications. Bosniak I and II cysts are benign
A. A history and physical examination is the ini- and do not require follow-up. Increasingly
tial step in renal mass evaluation. Patients complex features are useful in predicting risk
may exhibit nonspecific symptoms including of malignancy. Management strategies for
gross or microscopic hematuria, flank pain, more complex cysts are based on AUA guide-
weight loss, fever, or diaphoresis. Physical lines which recommend routine surveillance
signs are uncommon but may include hyper- (Bosniak IIF) versus surgical resection
tension, palpable flank mass, and/or ipsilat- (Bosniak III and IV) [3].
eral varicocele. The majority of renal masses D. The differential diagnosis for a solid renal
(up to 70%) are incidental findings and other- mass includes malignant and non-malignant
wise asymptomatic [1]. entities. Macroscopic fat (HU –20 to −80) is
B. Dedicated imaging is necessary to further virtually diagnostic for angiomyolipoma
characterize the renal mass as cystic or solid. (AML). Asymptomatic patients with small
A three-phase abdominal computed tomogra- (<4 cm) AML are monitored with serial
phy (CT) scan with/without intravenous (IV) imaging. The risk of spontaneous rupture
contrast (“renal mass protocol”) is considered increases with AML size >4 cm. Significant
the gold standard cross-sectional study. active bleeding necessitates embolization or
Abdominal magnetic resonance imaging open surgical control for life-threatening
(MRI) with gadolinium and ultrasonography hemorrhage [4]. Renal mass biopsy should be
are additional imaging modalities for renal considered when a mass is suspected to be
mass characterization [2]. metastatic [5]. Enhancing solid renal masses
C. The Bosniak classification system defines are usually malignant and most commonly
cystic renal masses based on their contrast- renal cell carcinoma (RCC).
enhanced CT imaging characteristics. Simple E. If RCC is suspected after imaging, obtain
renal cysts (Bosniak I) do not enhance or con- CBC, CMP, and UA. Chest imaging (CXR or
CT) is performed to rule out pulmonary
J. Chris Riney and Neil J. Kocher contributed equally to metastases. Additional studies include a
this work. nuclear medicine bone scan and/or brain MRI
J. C. Riney · N. J. Kocher · M. Kaag (*) if patients present with bone pain or neuro-
Division of Urology, Penn State Milton S. Hershey logic symptoms, respectively [6].

598 J. C. Riney et al.
F. Treatment of suspected RCC is based on nephrectomy. Select medium-sized masses

tumor size, location, and characteristics. (T1b 4–7 cm) amenable to nephron-sparing
Other considerations include patient’s age, surgery are managed with partial nephrec-
comorbidities, presence of lymphadenopathy, tomy. Large tumors are managed with radical
venous tumor thrombus, and invasiveness of nephrectomy [5]. Locally advanced tumors
tumor. Small renal masses are managed with may invade the vena cava and require cavot-
active surveillance, ablation, or partial omy and reconstruction.
Perform focused history and physical exam

A · Nonspecific symptoms
· Physical signs uncommon
Obtain 3-phase abdominal CT scan with/without IV

B contrast (“renal mass protocol”)
C Cystic D Solid
Bosniak I or II Bosniak IIF Bosniak III or IV Enhancing Concern for Fat present
>15HU metastasis or
lymphoma
No follow-up Surveillance Surgical

AML
necessary imaging removal
Consider
biopsy
Obtain CBC, CMP, and UA.

Imaging: CXR or CT chest, bone scan if pathologic fracture or bone pain, elevated calcium
or alkaline phosphatase; MRI of brain if neurologic signs/symptoms; biopsy in select patients
Small renal mass

F 4 cm(T1a) Select T1b (4-7cm) Large mass or not amendable
to nephron sparing
Active Partial Radical

Ablation Nephrectomy
Surveillance Nephrectomy
Algorithm 146.1
146 Management of the Renal Mass 599
References 4. Nelson CP, Sanda M. Contemporary diagnosis and

management of renal angiomyolipoma. J Urol.
2002;168:1315.
1. Chen DY, Uzzo RG. Evaluation and management of the
5. National Comprehensive Cancer Network (NCCN)
renal mass. Med Clin North Am. 2011;95(1):179–89.
clinical practice guidelines in oncology: kidney can-
2. Kang SK, Chandarana H. Contemporary imaging of
cer. V.1.2018, 2018. www.nccn.org.
the renal mass. Urol Clin North Am. 2012;39:161–70.
6. Campbell S, Uzzo RG, Allaf ME, et al. Renal mass
3. Israel GM, Bosniak MA. An update of the
and localized renal cancer: AUA guideline. J Urol.
Bosniak renal cyst classification system. Urology.
2017 Sep;198(3):520–9.
2005;66(3):484.
Prostate Cancer
147
Rosa Park and Matthew Kaag
Algorithmic Approach patient is at higher risk due to positive family

history or African American descent. Patients
A. Prostate cancer is the most common malig- over the age of 70 or with less than 10-year
nancy in US males after skin cancer, but the life expectancy should not be screened.
mortality rate in this usually unaggressive Recommendations also include an increased
cancer is low [1]. Most patients present with- interval between screening visits (2 years)
out symptoms, and the question of appropri- and involving the patient in shared decision-
ate screening is addressed below. Patients making when deciding whether to embark on
with locally advanced disease complain of screening [4].
obstructive or irritative voiding symptoms D. In most cases an abnormal DRE will prompt
and hematuria. Metastatic prostate cancer a prostate needle biopsy. PSA is assessed
may manifest as hydronephrosis or bone pain. based on temporal trends and normal ranges
Pathologic fractures are possible [2]. that are dependent on the patient’s age.
B. Screening of asymptomatic men was preva- Normal fluctuation as well as inflammation
lent in the United States until 2012 and con- and local trauma may increase the PSA in the
sists of a prostate-specific antigen (PSA) absence of malignancy, and an isolated ele-
blood test and digital rectal exam (DRE). In vated level should prompt a repeat test [5].
2012 the US Preventative Services Task Force E. Definitive diagnosis of prostate cancer is via
(USPSTF) identified a lack of data support- transrectal ultrasound-guided prostate needle
ing prostate cancer screening, driven by the biopsy. Typically, a single dose of fluoroqui-
historic over-treatment of men with low-risk nolone antibiotic prophylaxis is recom-
disease who were unlikely to die of their can- mended at least 1 h prior to biopsy. Rectal
cer regardless of therapy [3]. Prostate cancer cultures can be obtained prior to biopsy in
screening was given a D grade, with the patients with increased risk of resistant bacte-
USPSTF recommending against its use. ria. Expected sequelae include gross hematu-
C. The American Urological Association revised ria, hematochezia, and hematospermia. Risk
recommendations include narrowing the age of sepsis is 0.3–3.1%, and the risk of post-
range for screening to men 55–69, unless the biopsy urinary retention is 0.2–2.6% [6].
F. If there is cancer present on biopsy, staging
R. Park · M. Kaag (*) work-up may be indicated. Obtain a bone
Division of Urology, Penn State Milton S. Hershey scan for Gleason score ≥8, PSA >20 ng/mL,
Medical Center, Hershey, PA, USA T2 and PSA >10 ng/mL, and clinical T3 and

602 R. Park and M. Kaag
T4 or if symptomatic. Pelvic CT or MRI is risk of progression may be offered radical

considered for T3 and T4 disease, or T1–T2 if prostatectomy or radiotherapy. Androgen
nomogram indicates >10% lymph node deprivation therapy may be combined with
involvement [7]. radiation to increase survival rates in men
G. Risk stratification of clinically localized
with intermediate- or high-risk disease.
disease guides therapy. Patients deemed to Patients with disseminated disease are man-
be at low risk (low volume of cancer on aged via a therapeutic algorithm that is
biopsy, low-grade disease, low PSA) may structured around depriving tumor cells of
be offered active surveillance using one of a their androgen source but also may include
number of accepted protocols. Patients immunotherapy and conventional chemo-
desiring active treatment and those at high therapy [7].
147 Prostate Cancer 603

· Most are asymptomatic
A · Family History
· Perform DRE
Normal DRE Abnormal DRE
B
Does the patient meet PSA
based screening criteria?
D No Yes Check PSA
Normal PSA Elevated PSA

No further work-up
C Follow up as indicated
May repeat PSA
E Prostate needle biopsy
No further work-up
Repeat DRE and Normal biopsy Prostate cancer
PSA as appropriate
F Imaging by risk category: MRI pelvis, CT, or bone scan
Metastatic
G Clinically localized disease Regional disease
disease
Active Radical prostatectomy ± Androgen

Radiation Refer to oncology
surveillance pelvic lymph node deprivation
dissection
Algorithm 147.1
604 R. Park and M. Kaag
References 5. Eastham JA, Riedel E, Scardino PT, Shike M, Fleisher

M, Schatzkin A, Lanza E, Latkany L, Begg CB, Polyp
Prevention Trial Study Group. Variation of serum
1. Siegel RL, Miller KD, Jemal A. Cancer statistics,
prostate-specific antigen levels: an evaluation of year-
2015. CA Cancer J Clin. 2015;65(1):5–29.
to-year fluctuations. JAMA. 2003;289(20):2695–700.
2. Loeb S, Eastham JA. Chapter 111, Diagnosis and stag-
6. Liss MA, Ehdaie B, Loeb S, Meng MV, Raman JD,
ing of prostate cancer. In: Campbell-Walsh urology.
Spears V, Stroup SP. An update of the American
11th ed. Philadelphia: Elsevier; 2016. p. 2601–8.
Urological Association White Paper on the pre-
3. Moyer VA, U.S. Preventive Services Task Force.
vention and treatment of the more common com-
Screening for prostate cancer: U.S. preventive services
plications related to prostate biopsy. J Urol. 2017
task force recommendation statement. Ann Intern
Aug;198(2):329–34.
Med. 2012;157(2):120–34.
7. National Comprehensive Cancer Network, Inc.
4. Carter HB, Albertsen PC, Barry MJ, Etzioni R,
NCCN clinic practice guidelines in oncology: pros-
Freedland SJ, Greene KL, Holmberg L, Kantoff
tate cancer. Version 1.2018. [Internet]. 2018. [cited
P, Konety BR, Murad MH, Penson DF, Zietman
2018 Feb 14]. Available from: https://www.nccn.org/
AL. Early detection of prostate cancer: AUA guide-
professionals/physician_gls/pdf/prostate.pdf.
line. J Urol. 2013;190(2):419–26.
Management of Scrotal/Testicular
Mass 148
Brian M. Blair and Matthew Kaag
Algorithmic Approach monly in the neonatal period and at puberty.

Cryptorchidism is a predisposing factor.
A. Initial evaluation of a scrotal or testicular
Testicular torsion is a urologic emergency as
mass requires a thorough history and focused testicular loss can occur if blood flow is not
physical examination, including genital, restored within 6 h. Immediate surgical
abdominal, lymph node, breast, and neuro- exploration with detorsion of the testicle and
logical exams. The examiner should attempt fixation via orchiopexy is required [3].
to distinguish intratesticular from paratesticu- D. Trauma to the scrotum/testicle is a common
lar lesions. The causes of scrotal masses may cause of testicular enlargement or irregular-
be classified as follows: benign/malignant, ity. A scrotal mass may be noted, secondary
infectious/noninfectious, and emergent/non- to hematoma. Penetrating injury of the scro-
emergent. Consultation of a urologist is often tum requires surgical exploration and repair.
necessary [1]. Blunt scrotal trauma may result in rupture of
B. Scrotal ultrasound should be obtained early to the tunica albuginea, requiring prompt surgi-
confirm diagnosis. The urgency in which to cal repair. Eighty to ninety percent of testicles
obtain the ultrasound will be determined are salvaged when surgery is performed
upon the patient presentation and suspected within 72 h of injury, whereas only 32–45%
diagnosis [2]. are salvageable after 72 h [1].
C. Testicular torsion results when the testicle E. Epididymo-orchitis is an infectious/inflam-
twists on its blood supply causing loss of matory cause for testicular mass that may
blood flow to the testicle. The testicle may be involve outpatient management with NSAIDs
enlarged or present in an abnormal position. ± oral antibiotics. However if significant
Exam findings include abnormal testicular infection is present, admission to the hospital
lie, loss of cremasteric reflex, swelling/edema may be required for intravenous (IV) antibi-
with exquisite tenderness, and Prehn sign. It otics ± surgical drainage/orchiectomy if
is accompanied by acute, severe pain with abscess develops.
nausea/vomiting. Incidence is distributed in a F. Benign cystic masses of the epididymis or
bimodal fashion with torsion seen most com- spermatic cord include spermatocele, epidid-
ymal head cyst, and varicocele. These entities
B. M. Blair · M. Kaag (*) are classified as paratesticular and are gener-
Division of Urology, Penn State Milton S. Hershey ally benign. Observation is usually appropri-
Medical Center, Hershey, PA, USA ate unless symptomatic. Varicoceles may be

606 B. M. Blair and M. Kaag
associated with infertility, prompting repair. H . All suspected testicular tumors should be
While left-sided varicoceles are fairly com- resected through an inguinal incision. The
mon, right-sided varicoceles are rare and may scrotum should not be violated. High liga-
indicate the presence of a mass compressing tion of the spermatic cord is required and
the gonadal vein. CT of the abdomen/pelvis is assists in future surgery if a retroperito-
warranted in this setting [4]. Paratesticular neal lymph node dissection becomes
sarcomas may be mistaken for one of these necessary.
benign cystic entities on palpation but are I. Further management of testicular cancers is
solid on ultrasound and very rare. predicated on tumor subtype (seminoma vs.
G. A solid testicular mass on exam is concerning nonseminoma). Patients without clinical
for testicular neoplasm. These are often painless evidence of retroperitoneal metastasis and
but may be painful if infarcted. Testicular ultra- normal post-orchiectomy tumor markers
sound demonstrates a hypoechoic vascular may be observed, though some patients may
intratesticular lesion. Obtain testicular tumor opt for prophylactic radiation, chemother-
markers including α-fetoprotein, quantitative apy, or RPLND. Patients with seminoma
β-human chorionic gonadotropin, and lactate who have low-volume metastasis are eligi-
dehydrogenase. Additional labs to include are a ble for low-dose chemotherapy or radiation
CBC, BMP, LFTs, and a PT/INR. Sperm bank- to the retroperitoneum, whereas patients
ing should be discussed. Metastatic work-up with low-volume seminoma may opt for
must be obtained and should include a CT of the RPLND or low-dose chemotherapy. The
abdomen/pelvis with IV and oral contrast and a mainstay of treatment for disseminated dis-
chest X-ray. In patients at higher risk of pulmo- ease is platinum-based multi-drug chemo-
nary metastasis, a chest CT is warranted [5]. therapy [1, 5].
148 Management of Scrotal/Testicular Mass 607
History of Present Illness and Vitals/Physical Exam

A (genitals, abdomen, lymph nodes, breasts, neuro)
Painful and
Scrotal or
suspect
Painless/painful testicular
testicular
mass and torsion trauma?
torsion?
not suspected?
Scrotal Scrotal
B Ultrasound Scrotal Ultrasound
STAT Ultrasound STAT
E
No blood
C flow?
Epididymo-
Hydrocele
Evidence of D
orchitis testicular
rupture?
Immediate
No Observe or
exploration Abscess
Abscess elective
and
orchiopexy repair Scrotal
exploration
Antibiotics and repair
IV antibiotics,
surgical and/or Epididymal/Spermatic
drainage or NSAIDS Cord Mass
orchiectomy
Spermatocele,
Varicocele
F Solid Testicular Mass Epididymal cyst
· Obtain labs (CBC, BMP, PT/INR, LFTs)and tumor markers (AFP, quantitative b-HCG, LDH)
· Discuss sperm banking
· Staging with CT abdomen/pelvis with PO and IV contrast and either chest X-ray or CT chest
Radical inguinal orchiectomy with high

G spermatic cord ligation
Algorithm 148.1
608 B. M. Blair and M. Kaag
References 4. Rybenstein R, Dogra V, Seftel A, Resnick M. Benign

intrascrotal lesions. J Urol. 2004;171(5):1765–72.
5. NCCN Clinical Practice Guidelines in Oncology –
1. Wein A, Kavoussi L, Partin A, Peters C. Campbell-
Testicular Cancer – Version 2.2017 [Internet]. www.
Walsh urology. 11th ed. Philadelphia: Elsevier; 2016.
nccn.org. 2017 [cited 8 Oct 2017]. Available from:
2. Montgomery J, Bloom D. The diagnosis and man-
https://www.nccn.org/professionals/physician_gls/
agement of scrotal masses. Med Clin North Am.
pdf/testicular.pdf.
2011;95(1):235–44.
3. Rabinowitz R, Hulbert W. Acute scrotal swelling.
Urol Clin North Am. 1995;22(1):101–5.
of Fournier’s Gangrene 149
Augustyna Gogoj and Matthew Kaag
Algorithmic Approach Radiographs may show swelling or subcuta-

neous emphysema. Ultrasound can demon-
A. Fournier’s gangrene is a necrotizing infection strate a thickened, edematous scrotal wall
involving the genitals. Patients often present containing hyperechoic foci or paratesticular
with, or rapidly develop signs of, sepsis, and fluid. Computed tomography can be used to
this condition may be progressive and life- guide surgical management and visualize
threatening. Treatment hinges on the rapid asymmetric fascial thickening, fluid collec-
initiation of broad-spectrum antibiotics and tions, abscess formation, fat stranding, and
aggressive local debridement with/without subcutaneous emphysema. Computed tomog-
urinary and fecal diversion. raphy has greatest specificity [4].
B. Diagnosis of Fournier’s gangrene starts with D. Broad-spectrum parenteral antibiotic therapy
clinical findings on physical exam including is to be started immediately upon diagnosis.
fluctuance, localized tenderness, and wounds Antibiotics must cover staphylococcal, strep-
of the genitalia and perineum [1]. Crepitus or tococcal, and gram-negative bacteria, coli-
evidence of tissue necrosis differentiates forms, Pseudomonas, Bacteroides, and
Fournier’s gangrene from genital cellulitis Clostridium. Adequate coverage typically
and scrotal abscess. Vital signs and blood includes a broad-spectrum penicillin with
work may be used as predictors for mortality. third-generation cephalosporin or aminogly-
Patients older than 50 with diabetes mellitus coside and metronidazole or clindamycin.
(in 20–70% of patients) or an alcohol abuse Expansion to include vancomycin or line-
history (in 20–50% of patients) are at greatest zolid may be necessary based on the clinical
risk and should be thoroughly examined. situation. Culture results can guide eventual
Other predisposing factors include poor narrowing of antibiotic coverage [3].
hygiene, malnutrition, malignancy, steroid E. Aggressive and timely surgical debridement
use, or an immunocompromised state [2, 3]. is imperative for decreased mortality. All
C. Imaging is not necessary unless disease devitalized tissues must be resected. Serial
extent or diagnosis is not clear and should debridement is often necessary [5].
never prolong time to surgical management. F. Fecal diversion is indicated with anal sphinc-
ter involvement, fecal incontinence, or con-
A. Gogoj · M. Kaag (*) tinued fecal contamination of the wound’s
Division of Urology, Penn State Milton S. Hershey margins [6]. In the event of extensive penile

610 A. Gogoj and M. Kaag
or urethral involvement, suprapubic urinary H. Reconstruction may be necessary for skin

diversion may be necessary; however, ure- coverage of wounds, cosmesis, and preserva-
thral catheterization is often sufficient [1]. tion of genital function. Primary closure of
G. Post-operatively wounds are managed with wounds shows the best functional and cos-
dressing changes or negative-pressure wound metic results. Closure of wounds through sec-
therapy and continued antibiotics [7]. Adjunct ondary intention increases healing time and
hyperbaric oxygen therapy may enhance scrotum deformity [9]. Large defects may
patient survival, but reported results have require myocutaneous or fasciocutaneous
been inconsistent [8]. flaps [10, 11]. Follow-up may include further
reconstruction.
149 Diagnosis and Management of Fournier’s Gangrene 611
Physical exam:
fluctuance, crepitus, localized tenderness,
wounds of the genitalia and perineum
A Obtain vital signs, blood work, assess risk factors
Yes
B Disease extent Obtain imaging
or diagnosis CT is most specific: fascial thickening,
unclear? fluid collections, paratesticular fluid
No
C Start broad-spectrum IV antibiotics
D Aggressive surgical debridement
Fecal diversion through colostomy or Flexi-seal

E Need for
fecal management system
fecal/urinary
urinary diversion through cystostomy,
diversion? Yes catheterization, or suprapubic diversion
No
F Open wound care with sterile dressings or negative-pressure wound therapy

Hyperbaric oxygen therapy used as adjunct
G Reconstruction: closure of wounds through primary or secondary intention, full

thickness or split thickness skin grafts, myocutaneous or fasciocuteous flaps
Follow-up care
Algorithm 149.1
612 A. Gogoj and M. Kaag
References 6. Ozturk E, Sonmez Y, Yilmazlar T. What are the indi-

cations for a stoma in Fournier’s gangrene? Color Dis.
2011;13:1044–7.
1. Chennamsetty A, Khourdaji I, Burks F, Killinger
7. Ozkan O, Koksal N, Altinli E, Celik A, Uzun M,
K. Contemporary diagnosis and manage-
Cikman O, Akbas A, Ergun E, Kiraz HA, Karaayvaz
ment of Fournier’s gangrene. Ther Adv Urol.
M. Fournier’s gangrene current approaches. Int
2015;7(4):203–15.
Wound J. 2011;15(5):713–6.
2. Clayton M, Fowler J, Sharifi R, Pearl R. Causes, pre-
8. Jallali N, Withey S, Butler P. Hyperbaric oxygen as
sentation and survival of fifty-seven patients with nec-
adjuvant therapy in the management of necrotizing
rotizing fasciitis of the male genitalia. Surg Gynecol
fasciitis. Am J Surg. 2005;189(4):462–6.
Obstet. 1990;170:49–55.
9. Maguina P, Palmieri T, Greenhalgh D. Split thickness
3. Mallikarjuna M, Vijayakuma A, Patil V, Shivswamy
skin grafting for recreation of the scrotum following
B. Fournier’s gangrene: current practices. ISRN Surg.
Fournier’s gangrene. Burns. 2003;29:857–62.
2012;942437.
10. Lee S, Rah D, Lee W. Penoscrotal reconstruction with
4. Levenson R, Singh A, Novelline R. Fournier gangrene:
gracilis muscle flap and internal pudendal artery per-
role of imaging. Radiographics. 2008;28:519–28.
forator flap transposition. Urology. 2012;79:1390–6.
5. Sorenson M, Krieger J, Rivara F, Klein M, Wessells
11. Chen S, Fu J, Wang C, Lee T, Chen S. Fournier gan-
H. Fournier’s gangrene: management and mortal-
grene: a review of 41 patients and strategies for recon-
ity predictors in a population based study. J Urol.
struction. Ann Plast Surg. 2010;64:765–9.
2009;182:2742–7.
Part XIX
Trauma
Hypotension and Blunt Abdominal
Trauma 150
Cheyenne C. Sonntag and Steven R. Allen
Algorithmic Approach crystalloid solution given. Patients will often

have IV access and fluids initiated in the field
A. On patient arrival, a brief history provided by by EMS, and this volume should be consid-
transporting EMS may identify blunt mecha- ered. If hypotension does not correct with
nism. The first steps in evaluation of patients crystalloid bolus, early transition to blood
with blunt traumatic injury is the primary sur- products is warranted.
vey (ABCs) to identify and intervene for life- C. In the setting of persistent hypotension, it is
threatening conditions [1]. The airway is inappropriate to transport the patient for com-
assessed for patency, with maintenance of puted tomography (CT) scan, and other diag-
spinal precautions. Bilateral chest is auscul- nostic modalities must be considered to
tated for presence of breath sounds, oxygen evaluate for internal hemorrhage. For abdom-
saturation obtained, chest wall inspected, and inal evaluation, these include diagnostic peri-
oxygen therapy initiated. Interventions such toneal aspirate (DPA), diagnostic peritoneal
as obtaining definitive airway and needle lavage (DPL), and focused assessment sonog-
decompression are performed as indicated if raphy in trauma (FAST) and are equally
life-threatening condition is found during air- appropriate and dependent on provider/insti-
way and breathing assessment. tution capabilities. Thoracic and pelvic
B. In continuation of the primary survey, circu- trauma are elsewhere covered.
latory assessment consists of blood pressure, D. DPA is considered positive if gross blood is
heart rate evaluation, and assessment of found on aspiration (10 mL); DPL is positive
peripheral pulses. Sources of large external if microscopic analysis reveals >100 K/mm3
blood loss are controlled. Two large-bore IVs red blood cells and >500 K/mm3 white blood
should be obtained preferentially and, in cells or lavage shows bile or particulate mat-
patients found to be hemodynamically unsta- ter [2, 3]. It should be remembered that false-
ble, a bolus of 1–2 liters of warm isotonic positive DPL may occur in the setting of
pelvic fractures, when retroperitoneal hema-
C. C. Sonntag toma may unintentionally be sampled [3].
Department of Surgery, Penn State Milton S. Hershey Positive DPA/DPL in a hemodynamically
Medical Center, Hershey, PA, USA unstable patient warrants emergent operative
S. R. Allen (*) exploration.
Department of Surgery, Penn State Health Milton E. Exploratory laparotomy is indicated in
S. Hershey Medical Center, Hershey, PA, USA patients who are hemodynamically unstable

616 C. C. Sonntag and S. R. Allen
with positive intraabdominal fluid found on as repeat FAST. Negative FAST should not
FAST exam. FAST is sensitive in detecting a delay exploratory laparotomy in unstable
minimum of 200 mL of fluid in experienced patients with clear physical findings on
hands; however, sensitivity may vary with examination.
operator ultrasound experience and patient G. As with negative FAST, persistent hypoten-
body habitus [4, 5]. sion in a patient with negative DPA and DPL
F. Hypotensive patients with negative FAST should trigger further investigation for other
exam should have continued resuscitation, sources of shock. Repeat procedure may be
and alternate sources of shock should be eval- considered.
uated. DPA/DPL may be considered as well
150 Hypotension and Blunt Abdominal Trauma 617
Patient arrival & EMS report
A
Primary survey
E
Airway
Breathing
Circulation
B · Assess BP, HR, peripheral
pulses
· IV access and fluid bolus
Persistent Continue
No
hemodynamic primary
instability survey
C YES
· Transfuse blood products

· DPA/DPL or FAST
DPA positive? FAST positive?

· aspirate 10mL gross blood
or
DPL positive?
E No F
· RBC>100 K/mm3 YES
· WBO >500 K/mm3
· Bile or particulates D
YES · Continue resuscitation

G No
· Investigate other sources
of shock
· Consider DPA/DPL
· Continue resuscitation
Exploratory
· Investigate other sources laparotomy
of shock
Algorithm 150.1
618 C. C. Sonntag and S. R. Allen
References for the evaluation of blunt abdominal trauma: the

East practice management guidelines work group. J
Trauma. 2002;53(3):602–15.
1. Initial Assessment and Management. Advanced
4. Branney SW, Wolfe RE, Moore EE, Albert NP, Heinig
trauma life support ATLS: student course manual. 9th
M, Mestek M, et al. Quantitative sensitivity of ultra-
ed. Chicago: American College of Surgeons; 2012.
sound in detecting free intraperitoneal fluid. J Trauma.
p. 6–13.
1995;39(2):375–80.5.
2. Root HD, Hauser CW, McKinley CR, Lafave JW,
5. Carter JW, Falco MH, Chopko MS, Flynn WJ Jr,
Mendiola RP Jr. Diagnostic peritoneal lavage.
Wiles Iii CE, Guo WA. Do we really rely on fast for
Surgery. 1965;57:633–7.
decision-making in the management of blunt abdomi-
3. Hoff WS, Holevar M, Nagy KK, Patterson L, Young
nal trauma? Injury. 2015;46(5):817–21.
JS, Arrillaga A, et al. Practice management guidelines
Traumatic Brain Injury
151
Shannon R. Kotch and Steven R. Allen
Algorithmic Approach D. If the CT scan is negative for further injury,

the patient with a normal neuro exam who has
A. In a patient who has sustained significant
been observed for 4–6 h and without further
trauma, especially if there is high suspicion concerns for neurologic injury can be dis-
for a head injury based on the mechanism of charged with instructions on symptoms which
injury, advanced trauma life support (ATLS) would prompt a return visit. If there are con-
protocol should be followed [1]. cerns for the patient despite a normal exam,
B. After calculating the Glasgow Coma Score 12–24 h of continued observation prior to dis-
(GCS), traumatic brain injury (TBI) can be charge is appropriate [1].
classified into mild, moderate, and severe. E. A patient with a GCS of 9–12 on presentation
Patients with mild TBI have a GCS of 13–15 to the emergency department has a moderate
upon arrival to the emergency department, TBI. These patients should also undergo a
those with moderate TBI have a GCS of 9–12, complete neurological and mental status
and those with severe TBI have a GCS of 8 or exam along with a CT scan. These patients
less [2]. warrant admission to an intensive care unit
C. In a patient with a GCS of 13–15, a complete for frequent neurological and mental status
neurological and mental status exam should exams [1].
be performed. If there is any report of loss of F. If there is improvement in the patient’s condi-
consciousness or amnesia reported, a com- tion, the patient may be downgraded and
puted tomography (CT) scan should be neuro exams performed with less frequency.
obtained to rule out a more significant brain If the patient does not show any signs of
injury. It is common for patients with moder- improvement within 6–12 h, a CT scan should
ate or severe TBI to present with a lucid inter- be repeated to rule out worsening of known
val, as seen with epidural hematoma, and lesions or the formation of new lesions [1].
subsequently decompensate rapidly [1]. G. Nearly all patients with a moderate TBI will
need some sort of rehab. Patients who have a
S. R. Kotch severe TBI will need extensive rehab, assum-
Department of General Surgery, Penn State Health ing they recover from the inciting insult [1].
Milton S. Hershey Medical Center, Hershey, PA, USA H. Patients with a GCS of 8 or less have a
S. R. Allen (*) severe TBI. Based on GCS alone, these
Department of Surgery, Penn State Health Milton patients are unable to properly protect their
S. Hershey Medical Center, Hershey, PA, USA airway and should be intubated immediately.

620 S. R. Kotch and S. R. Allen
yperventilation can be considered in the

H tract from the ABCDEs. A CT scan should be
acute setting, maintaining PaCO2 between 30 performed as soon as the patient is stable
and 35 mm Hg. Patients also need to be ade- enough to undergo the test. These patients
quately resuscitated, and the patient should also prompt an intensive care unit (ICU)
also be properly assessed for any sources of admission with frequent neurological exami-
ongoing bleeding which may lead to a hypo- nations [1].
volemic state. It is critical to avoid hypoten- J. Intracranial pressure (ICP) monitoring should
sion (systolic blood pressure <90 mm Hg) be performed in patients with severe TBI. If
and hypoxia (oxygen saturation <90%) to ICP is found to be elevated (≥25 mm Hg), the
prevent further ischemic insult to the brain. patient can be managed medically with man-
Early neurosurgical consultation is advisable nitol (0.5–1 g/kg) or hypertonic saline. If an
[1, 2]. epidural hematoma or subdural hematoma is
I. If possible, prior to intubation, a complete responsible for the elevated ICP, surgical
neurological and mental status exam should management with a decompressive craniec-
be performed. This should not, however, dis- tomy is necessary [1].
151 Traumatic Brain Injury 621
History:
Teenager who fell from trampoline, hit head on concrete wall
Brief loss of consciousness, remembers event
A ABCDEs –ATLS protocol
Mild TBI: Moderate TBI: Severe TBI:

B GCS 13-15 GCS 9-12 GCS 8
C Complete neurological Complete neurological Intubate, resuscitate H

and mental status exam and mental status exam
Complete neurological
Positive loss of CT scan and mental status exam
consciousness or amnesia
Admission to ICU, I
CT scan
E frequent neuro exams
CT scan
D F
Admission to ICU,
frequent neuro exams
Observation 4–6 h, Observation 4–6 h, Improvement No
normal exam, no normal exam, improvement
concerns concerns
ICP monitoring J
Downgrade
level of care Repeat CT
Discharge Observe in 6–12 h Elevated ICP
12–24 h
Rehab likely
Medical Surgical
management management
G
G Rehab
Algorithm 151.1
2. Chesnut RM. Care of central nervous system injuries.

References Surg Clin North Am. 2007;87(1):119–56., vii. https://
doi.org/10.1016/j.suc.2006.09.018.
1. Decuypere M, Klimo P Jr. Spectrum of traumatic
brain injury from mild to severe. Surg Clin North Am.
2012;92(4):939–57., ix. https://doi.org/10.1016/j.
suc.2012.04.005.
Penetrating Neck Trauma
152
Alexis Lauria and Steven R. Allen
Algorithmic Approach trates the platysma, requiring further work-up

according to the algorithm. If no platysmal
A. Initial resuscitation and wound assessment: violation is present, the trauma team should
Initial resuscitation of a patient with PNT continue with secondary and tertiary surveys.
should follow the Advanced Trauma Life In follow-up care, the wound should be fre-
Support (ATLS) guidelines [1]. Dependent quently monitored for signs of occult dam-
upon the location of injury, airway manage- age. While injuries to the neck can be
ment may be a challenge. Orotracheal intuba- significant and require prioritization, it is also
tion is the preferred method of securing the important not to overlook potential concomi-
airway and should be performed immediately tant injuries in a patient with PNT.
if any hard signs are present (table in B. Hard signs of vascular or tracheoesophageal
Alogrithm 152.1). If orotracheal intubation is injury: Listed are commonly accepted hard
contraindicated (extensive midface trauma, signs of vascular (hemorrhage, hematoma,
significant upper airway distortion, or inabil- thrills or bruits, unresponsive shock, dimin-
ity to visualize the glottis), an emergent crico- ished pulses, and neurological deficits) and
thyroidotomy should be performed. aerodigestive (air bubbling from the wound,
Once ATLS resuscitation has been com- hemoptysis or hematemesis, and respiratory
pleted, the wound should be assessed more distress) injuries. If any of these signs are
carefully. Spine immobilization, which may present, or the patient is clinically deteriorat-
hinder a full examination or rapid manage- ing, the patient should be taken immediately
ment of the neck injury, is not recommended to the operating room (OR). The airway
if there are no neurological deficits in a con- should be secured, if this had not been indi-
scious patient with isolated penetrating neck cated during the initial resuscitation. Pressure
trauma (PNT) [2]. Without probing, the sur- should be applied to the wound to tamponade
geon should assess whether the injury pene- any active bleeding. For significant bleeding
not amendable to tamponade, Foley balloon
A. Lauria catheterization may be considered [3, 4]. Soft
Department of Surgery, Walter Reed National signs of injury (wounds within 1–2 cm of a
Military Medical Center, Bethesda, MD, USA major vessel, minor hemorrhage, non-
S. R. Allen (*) expanding hematoma, fluid responsive mild
Department of Surgery, Penn State Health Milton hypotension, minor hemoptysis or
S. Hershey Medical Center, Hershey, PA, USA hematemesis, and dysphonia or dysphagia)

624 A. Lauria and S. R. Allen
should prompt more frequent and close moni- nal branches, vertebral arteries, jugular veins,
toring of the patient. trachea, esophagus, larynx, pharynx, spinal
Operative management for a rapidly decom- cord, and the vagus and recurrent laryngeal
pensating patient should focus on source con- nerves. The standard incision described in B
trol of bleeding and repair of vital structures. should allow access to most structures in
The standard operative approach is a vertical Zone II. However, additional horizontal inci-
incision along the anterior border of the sterno- sions may be used to provide maximum
cleidomastoid, extending from the angle of the access as necessary [5].
mandible to the sternoclavicular junction, with F. Zone I injury: Zone I, the most caudal zone,
variations by zone, as described below [5]. includes the thoracic inlet structures (subcla-
C. Zone III injury: Important structures within vian arteries and veins and internal jugular
Zone III include the vertebral arteries, distal veins), proximal carotid arteries, vertebral
internal carotid arteries, jugular veins, phar- arteries, trachea, esophagus, spinal cord, tho-
ynx, spinal cord, sympathetic chain, and cra- racic duct, thyroid gland, and apices of the
nial nerves IX, X, XI, and XII. Structures in lungs. A lower neck injury should increase
Zone III can be challenging to reach due to the suspicion for mediastinal injury or pneumo-
bony structures of the jaw. As such, endovas- thorax. For Zone I injuries, the standard inci-
cular management, when possible, may be sion [B] may be modified with horizontal
more appropriate for more distal Zone III inju- limbs along the superior aspect of the clavicle
ries. For an open approach, the vertical inci- for full exposure [5]. Furthermore, if medias-
sion [B] is extended with a horizontal limb tinal vessels are injured, a median sternot-
extending to the mastoid on the appropriate omy, disarticulation of the sternoclavicular
side [5]. More invasive measures such as sub- joint, or anterolateral thoracotomy may be
luxation, dislocation, or resection of the man- necessary [5].
dible may be necessary to reach more distal G. Suspicion for T/E injury: If there is clinical
structures [6]. (hemoptysis, hematemesis, dysphonia, dys-
D. CTA—indications and findings: For patients phagia, or subcutaneous or mediastinal air) or
who are hemodynamically stable, but with an radiographic concern for tracheoesophageal
injury violating the platysma, or any soft signs injury, more invasive studies should be per-
of injury, further imaging should be obtained. formed. These may include swallow studies
Computed tomography angiography (CTA) or esophagoscopy for concern for esophageal
can allow quick, noninvasive visualization of injury and/or bronchoscopy for concern for
major vascular and aerodigestive structures in airway disruption. Operative or interventional
the neck. Most trauma centers today have CT endoscopy/bronchoscopy should be per-
capabilities within feet of the trauma bay. formed based on findings here.
Positive findings on CTA include notable dis- H. Follow-up care: Patients who underwent pro-
ruption or abnormality in the lumen of any cedures involving the neck require close mon-
vascular or aerodigestive structures or a itoring for the formation of a hematoma,
“blush” indicating active bleeding. If CTA is which may compromise the airway. If an
positive, surgical, endoscopic, or endovascu- expanding hematoma is seen, the wound
lar management should be considered based should be immediately opened to avoid air-
on the location and type of identified injury. way compromise. Clinicians should continue
E. Zone II injury: Zone II is the largest and thus to monitor all patients for signs of decompen-
most commonly injured area of the neck. sation, development of hard or soft signs,
Important structures within Zone II include wound infection, or any other indications for
the common carotid and its internal and exter- further management.
152 Penetrating Neck Trauma 625
Resuscitate patient according to ATLS

A Assess wound (without probing)
Proceed with secondary and tertiary survey

Platysma No Continue to monitor for hard signs or
violated? worsening exam
Yes
Hard Yes Straight to OR

signs? Secure airway
B Apply direct pressure to tamponade wound
No
(+) OR
D Endo/embo
Zone III Injury
C (above angle of mandible)
CTA
(-)
H
OR
Symptomatic Endo/embo Follow-up care
Bronchoscopy
Zone II Injury
E (cricoid cartilage to angle of
mandible No
Asymptomatic (-)
Suspicion
for T/E
CTA
Injury?
Yes
Zone I Injury G
(between clavicles and cricoid (+)
F cartilage)
Swallow study
EGD
Bronchoscopy
OR
Endo/embo
Bronchoscopy
EGD
Table Hard signs of vascular (1-6) or aerodigestive

(7-9) Injury
1. Significant uncontrolled hemorrhage
2. Large, expanding or pulsatile hematoma
3. Thrill or bruits
4. Shock unresponsive to fluid resuscitation
5. Absent or diminished radial pulse
6. Neurologic deficits consistent with cerebral ischemia
7. Air bubbling from wound
8. Massive hemoptysis or hematemesis
9. Respiratory distress
Algorithm 152.1
626 A. Lauria and S. R. Allen
References 4. Navsaria P, Thoma M, Nicol A. Foley catheter

balloon tamponade for life-threatening hemor-
rhage in penetrating neck trauma. World J Surg.
1. American College of Surgeons Committee on Trauma.
2006;30(7):1265–8.
Advanced trauma life support for doctors. 8th ed.
5. Bagheri SC, Khan HA, Bell RB. Penetrating neck
Chicago: American College of Surgeons; 2012.
injuries. Oral Maxillofac Surg Clin North Am.
2. Stuke LE, Pons PT, Guy JS, Chapleau WP, Butler FK,
2008;20(3):393–414.
McSwain NE. Prehospital spine immobilization for pen-
6. Sperry JL, Moore EE, Coimbra R, Croce M, Davis
etrating trauma—review and recommendations from the
JW, Karmy-Jones R, McIntyre RC Jr, Moore FA,
Prehospital Trauma Life Support Executive Committee.
Malhotra A, Shatz DV, Biffl WL. Western Trauma
J Trauma Acute Care Surg. 2011;71(3):763–70.
Association critical decisions in trauma: pen-
3. Van Waes OJ, Cheriex KC, Navsaria PH, Van Riet PA,
etrating neck trauma. J Trauma Acute Care Surg.
Nicol AJ, Vermeulen J. Management of penetrating
2013;75(6):936–40.
neck injuries. Br J Surg. 2012;99(S1):149–54.
Penetrating Chest Trauma
153
Melissa Linskey and Steven R. Allen
Algorithmic Approach gently with incision planned based on the

location of injury [3]. The anatomy of the
A. The first step in evaluating a patient that has thoracic cavity and the trajectory of the pen-
suffered a penetrating injury to the chest is to etrating injury should be considered when
assess for pulses. Cardiopulmonary arrest or determining the structures possibly injured
severe hemodynamic instability may neces- and how they are best approached
sitate a prompt resuscitative thoracotomy operatively.
through a left anterolateral thoracotomy to D. Emergent anterolateral thoracotomy should
control life-threatening hemorrhage [1]. be performed in unstable patients with lateral
B. Patients with pulses should be evaluated for or posterior wounds and with massive hemo-
life-threatening injuries quickly through thorax [2].
advanced trauma life support protocols and E. Anterior or central injuries (those between
should receive appropriate resuscitative flu- the midclavicular lines), or patients with pos-
ids, blood products, and tube thoracostomy itive FAST consistent with cardiac tampon-
for treatment of hemothorax and pneumotho- ade, should undergo median sternotomy [2].
rax. Chest x-ray (CXR) and focused assess- F. In stable patients, further diagnostic testing
ment sonography in trauma (FAST) can aid in can be performed to identify specific struc-
identifying transmediastinal injuries or those tures that have been injured. Diagnostic test-
with laterality [2]. Possible findings seen on ing can include computed tomography (CT)
physical exam in penetrating chest trauma scans, CT angiography, esophagogastroduo-
can include distended neck veins, tracheal denoscopy (EGD), and bronchoscopy [2, 3].
deviation, subcutaneous emphysema, bub- G. Median sternotomy is the approach of choice
bling from wound, absent breath sounds, for cardiac, ascending aorta, innominate
muffled heart sounds, and absence of upper artery, right and left carotid arteries, and right
extremity pulse. subclavian artery injuries. Extension of this
C. Unstable patients not responding to fluid, incision to the neck or supraclavicular inci-
blood products, and chest tube placement sion can be performed based on the injury [4].
should be taken to the operating room emer- H. In penetrating chest trauma, 14% of stab

wounds and 15–20% of gunshot wounds will
M. Linskey · S. R. Allen (*) require thoracotomy [3]. Posterolateral thora-
Department of Surgery, Penn State Health Milton cotomy is appropriate for the intrathoracic
S. Hershey Medical Center, Hershey, PA, USA trachea, intrathoracic esophagus, and retained

628 M. Linskey and S. R. Allen
hemothorax. Left thoracotomy is specifically phragm injuries. Because of the variability in

helpful for descending aorta, intrathoracic diaphragmatic excursion, low thoracic inju-
left subclavian artery, and left mainstem ries should raise suspicion for intraabdominal
bronchus injuries [4]. injury [2].
I. Video-assisted thoracoscopy or laparoscopy
can be utilized on an urgent basis for dia-
A History and physical exam

Penetrating injury to chest (i.e. stabbing or gunshot wound)
No Pulses?
Resuscitative
thoracotomy
Yes
B Initial ATLS assessment and resuscitation
CXR, FAST
C No
Stable?
Yes F
Proceed to OR
Diagnostic tests/procedures: CT,
CTA, EGD, Bronchoscopy
Posterior/Lateral wound Anterior/Central wound

massive hemothorax tamponade
Cardiac/Great Trachea/ Diaphragm

vessel bronchus
Esophagus
G
D E I
H
Anterolateral Median Posterolateral VATS/

thoracotomy sternotomy thoracotomy laparoscopy
Algorithm 153.1
153 Penetrating Chest Trauma 629
References Association critical decisions in trauma: pen-

etrating chest trauma. J Trauma Acute Care Surg.
2014;77(6):994–1002.
1. Seamon MJ, Haut ER, Van Arendonk K, Barbosa RR, 3. DuBose JA, O’Connor JV, Scalea TM. Lung, trachea,
Chiu WC, Dente CJ, et al. An evidence-based approach and esophagus. In: Mattox KL, Moore EE, Feliciano
to patient selection for emergency department thora- DV, editors. Trauma. 7th ed. New York: McGraw-Hill
cotomy: a practice management guideline from the Medical; 2013. p. 468–84.
Eastern Association for the Surgery of Trauma. J 4. Mattox KL, Wall MJ, Tsai P. Trauma thoracotomy:
Trauma Acute Care Surg. 2015;79(1):159–73. principles and techniques. In: Mattox KL, Moore EE,
2. Karmy-Jones R, Namias N, Coimbra R, Moore EE, Feliciano DV, editors. Trauma. 7th ed. New York:
Schreiber M, McIntyre R Jr, et al. Western Trauma McGraw-Hill Medical; 2013. p. 461–7.
ED Thoracotomy
154
Algorithmic Approach B2. If the patient has cardiac activity, then

consider placement of resuscitative
Any patient presenting to the emergency depart- endovascular balloon occlusion of the
ment (ED) in extremis should be quickly evalu- aorta (REBOA).
ated to ascertain whether they have a blunt or B3. If the patient has cardiac activity and
penetrating mechanism of injury. there is evidence of tamponade, proceed
directly to ED thoracotomy to evacuate
A. In the patient with blunt injury, the first step is blood from the pericardial space.
to evaluate for presence of vital signs (defined C. In the patient with penetrating trauma, there
as pupillary response, spontaneous ventila- should be a much lower threshold for perform-
tion, presence of carotid pulse, measurable or ing ED thoracotomy due to higher salvage rates
palpable blood pressure, extremity movement in comparison to blunt traumatic cardiac arrest.
or cardiac electrical activity). C1. If the patient never had vital signs in the
A1. If the patient had no vital signs en route prehospital setting or if CPR has been
and has no vital signs on arrival, the ongoing for more than 20 min, the
patient should be pronounced with no patient should be pronounced and fur-
further resuscitation. ther resuscitative efforts stopped after
A2. If the patient lost vital signs en route, pulse check on arrival to the ED.
then cardiopulmonary resuscitation D. If the patient had loss of vital signs en route to
(CPR) should be temporarily stopped the hospital or in the ED, aggressive interven-
and vital signs reassessed while a car- tion should be performed depending upon
diac ultrasound is performed. whether there is obvious penetrating trauma
B. Cardiac ultrasound is used to assess the need to the chest or abdomen.
for further resuscitative efforts. D1. If there is obvious penetrating trauma to
B1. If there is no cardiac activity on ultra- the chest, then ED thoracotomy should
sound, then the patient should be probe performed without hesitation to cor-
nounced, and no further efforts at rect any obvious injury to the heart,
resuscitation should be performed. lungs, or great vessels.
D2. If there is obvious penetrating trauma to
N. R. Manley · G. O. MaishIII (*) the abdomen, consider placing a REBOA
Department of Surgery, University of Tennessee or proceeding with ED thoracotomy and
Health Science Center, Memphis, TN, USA cross-clamping the aorta to prevent fur-
e-mail: [email protected] ther truncal hemorrhage.
632 N. R. Manley and G. O. Maish
If REBOA or ED thoracotomy is performed, the as soon as possible. ATLS and ACLS should
patient should go immediately to the operating guide resuscitative interventions if REBOA or
room to definitively address destructive injuries ED thoracotomy is not pursued immediately.
Mechanism of injury
A C
Blunt Penetrating
D
A1. No vital C1. No vital Loss of vital
A2. Loss of
signs en route signs or CPR signs en route or
vital signs en
or in ED > 20 min in ED
route
B
Pronounce Pronounce D2.
D1. Chest
Abdominal
Cardiac Injury
injury
ultrasound
B1. No B3. + Fluid

cardiac B2. + (Tamponade)
activity Cardiac Consider
Emergent
activity REBOA
thoracotomy
Emergent
thoracotomy
Consider
REBOA
Abbreviations: CPR: cardiopulmonary resuscitation; ED: emergency department;

REBOA: resuscitative endovascular balloon occlusion of the aorta.
Algorithm 154.1
endovascular occlusion of the aorta as an alterna-

Suggested Readings tive to resuscitative thoracotomy for noncompress-
ible truncal hemorrhage. J Trauma Acute Care Surg.
American College of Surgeons. Advanced trauma life sup- 2015;79(4):523–30.
port. 9th ed. Chicago: American College of Surgeons; Seamon MJ, Haut ER, Van Arendonk K, Barbosa RR, Chiu
2012. 336 p. WC, Dente CJ, et al. An evidence-based approach to
American Heart Association. Advanced cardiac life sup- patient selection for emergency department thora-
port. Dallas: American Heart Association; 2015. 183 p. cotomy: a practice management guideline from the
Burlew CC, Moore EE, Moore FA, Coimbra R, McIntyre Eastern Association for the Surgery of Trauma. J
RC, Davis JW, et al. Western Trauma Association criti- Trauma Acute Care Surg. 2015;79(1):159–73.
cal decisions in trauma: resuscitative thoracotomy. J Western Trauma Association [Internet]. Resuscitative
Trauma Acute Care Surg. 2012;73(6):1359–63. Thoracotomy. 2012 [cited 2017 Aug 13]. Available from:
Moore LJ, Brenner M, Kozar RA, Pasley J, Wade CE, http://westerntrauma.org/algorithms/WTAAlgorithms_
Baraniuk MS, et al. Implementation of resuscitative files/gif_8.htm
Blunt Chest Wall Trauma
155
Algorithmic Approach is found. Further management will be dictated by

whether the patient is stable or unstable.
All patients presenting with blunt chest wall
trauma and history of significant mechanism A. In the stable patient with significant mecha-
should initially be assessed following ATLS pro- nism and evidence of blunt chest wall trauma
tocols, including airway, breathing, circulation, on history and physical exam, a CT scan with
disability, and exposure (ABCDE). Any threat to contrast should be performed to rule out
ABCDE should be addressed immediately. Next, injury to the heart, lungs, and great vessels. If
screening imaging should be performed, includ- there is any concern for blunt cardiac injury,
ing focused assessment with sonography for perform a 12-lead electrocardiography (EKG)
trauma (FAST) exam and chest x-ray. Immediate and send troponins.
life-threatening injuries include pneumothorax, • Severe injuries include blunt aortic injury,
hemothorax, and pericardial effusion and should flail chest, sternal fracture, and severe pul-
be treated as they are found. A chest tube should monary contusions. Blunt aortic injury
be placed for pneumothorax or hemothorax (with should be managed with a vascular surgery
hemothorax, note how much came out of chest consult, angiography, and possible open
tube on placement, and if it is >1500 mL, then the versus endovascular management of aortic
patient should go to the OR for immediate thora- injury. With flail chest, sternal fracture, and
cotomy). A pericardial effusion can be managed severe pulmonary contusions, there should
in the emergency department (ED) with pericar- be a low threshold for intubating the patient
diocentesis, although we recommend going to the if they are in respiratory distress.
OR for subxiphoid pericardial window if these Management for these conditions is mostly
resources are available. If the patient loses vital conservative (i.e., mechanical ventilation,
signs, consider an ED thoracotomy (see separate pain control, and aggressive pulmonary
Algorithm). A high degree of suspicion for intra- toilet). Rib plating for flail chest or multi-
thoracic or intra-abdominal injury should be ple rib fractures will be dictated by existing
maintained if scapula fracture, flail chest, sternal institutional protocols, as there are no con-
fracture, or posterior sternoclavicular dislocation clusive guidelines at this time.
• Mild to moderate injuries include rib frac-
N. R. Manley · G. O. Maish III (*) tures, mild pulmonary contusions, soft tis-
Department of Surgery, University of Tennessee sue injury, thoracic spine fractures, and
Health Science Center, Memphis, TN, USA clavicle fractures. Rib fractures and mild

634 N. R. Manley and G. O. Maish III
pulmonary contusions can be managed amount of blood from the chest tube placed
with pain control, incentive spirometry, for hemothorax: if greater than 1500 mL ini-
and early ambulation. Repeat chest x-rays tially or more than 250 mL/h for 4 h, then OR
can be performed as needed to assess thoracotomy should be performed. If the
respiratory status and resolution of injury. patient responds to resuscitation, then CT
Local wound care will generally suffice angiography should be performed and spe-
for soft tissue injury. Any thoracic spine cific injuries managed as in the stable patient.
fractures should be evaluated by the spine If the patient does not respond to further
service for specific management. Clavicle resuscitation and remains unstable, re-
fractures should be managed by the ortho- evaluate for life-threatening injury—includ-
pedic service, and general management is ing blunt cardiac injury—before sending the
a sling for comfort. patient to the CT scanner. Any arrhythmia
B. In the unstable patient, continue resuscitation should be managed by ACLS protocols. If the
with fluid and close monitoring of any life- patient remains unstable or loses vital signs,
threatening injuries found on the primary sur- an emergent thoracotomy should be strongly
vey. Special attention should be paid to considered.
155 Blunt Chest Wall Trauma 635
Patient with blunt chest wall trauma
ATLS
Screening
Imaging:
FAST, CXR
Management of life-threatening injuries:

· HTX/PTX: Chest tube
· Pericardial effusion: OR for
pericardial window
· Loss of VS: Consider ED
thoracotomy
A B
Stable Unstable
Chest CT Continued
Angiography Resuscitation
Severe Mild or No
Injury Moderate Responds
response
Injury
Manage per
etiology Manage per · Reassess for
etiology life-threatening
injury
· Consider Blunt
Abbreviations: ACLS: Advanced cardiac life support; ATLS: Advanced
Cardiac Injury
trauma life support; CXR: Chest x-ray; ED: Emergency department; FAST:
· ACLS
Focused assessment with sonography in trauma; HTX: Hemothorax; OR:
Operating room; PTX: Pneumothorax; VS: Vital signs
Algorithm 155.1
rib fractures after blunt trauma: a practice manage-

Suggested Reading ment guideline from the Eastern Association for
the Surgery of Trauma. J Trauma Acute Care Surg.
American College of Surgeons. Advanced trauma life sup- 2017;82(3):618–26.
port. 9th ed. Chicago: American College of Surgeons; Legome E, Hammel JM. Initial evaluation and man-
2012. 336 p. agement of chest wall trauma in adults. Up to Date
American Heart Association. Advanced cardiac life support. [Internet]. 2016 Sep [cited 2017 Aug 14]. Available
Dallas: American Heart Association; 2015. 183 p. from: https://www.uptodate.com/contents/initial-
Galvagno SM, Smith CE, Varon AJ, Hasonboehler EA, evaluation-and-management-of-chest-wall-trauma-
Sultan S, Shaefer G, et al. Pain management for blunt in-adults.
thoracic trauma: A joint practice management guide- Simon B, Ebert J, Bokhari F, Capella J, Emhoff T,
line from the Eastern Association for the Surgery Hayward T, et al. Management of pulmonary con-
of Trauma and Trauma Anesthesiology Society. J tusion and flail chest: an Eastern Association for
Trauma Acute Care Surg. 2016;81(5):936–51. the Surgery of Trauma practice management guide-
Kasotakis G, Hasenboehler EA, Streib EW, Patel N, line. J Trauma Acute Care Surg. 2012;73(5 Suppl
Patel MB, Alarcon L, et al. Operative fixation of 4):S351–61.
Blunt Cardiac Injury
156
Algorithmic Approach B. In the patient with abnormal EKG and normal

troponin, consult cardiology for further work-
Any patient presenting to the emergency depart- up. Consider cardiac ECHO. If injury is

ment (ED) with blunt trauma to the anterior chest found, manage per specific etiology and con-
should be worked up for blunt cardiac injury sider ICU admission for cardiac monitoring.
(BCI). In addition to routine trauma labs (e.g., Continue full trauma work-up as indicated. In
CBC, BMP, lactate, PT/INR, PTT, ABG) and the patient with abnormal troponin and nor-
imaging (e.g., portable chest), a 12-lead EKG mal EKG, consult cardiology for further
should be done, and cardiac troponins should be work-up. Again, consider cardiac ECHO. If
sent to assess baseline level. It is also important injury is found, manage per specific etiology
to obtain a thorough past medical history (PMH) and consider ICU admission for cardiac mon-
if possible to put the patient’s current injuries into itoring. Continue with full trauma work-up as
context. Pertinent PMH to BCI includes any pre- appropriate.
existing cardiac abnormality (e.g., murmur or C. In the hemodynamically unstable patient, fol-
pacemaker), current medications (especially car- low ATLS protocols in the trauma bay and
diac meds and anticoagulants), previous myocar- ensure ABCs are secure. Even if BCI is high
dial infarction, previous cardiac catheterizations on the differential diagnosis for instability,
and any stents placed, baseline functional status, assume tachycardia is secondary to hemor-
and any past cardiac surgeries (e.g., coronary rhage until proven otherwise. Any arrhyth-
artery bypass surgery [CABG]). Always consider mias should be managed with ACLS
myocardial infarction as the inciting event that protocols. Echocardiogram should be done to
led to the trauma, especially in the elderly. assess for structural defects; if any valve, sep-
tum, or ventricular wall injuries are found,
A. In the patient with normal EKG and normal cardiothoracic surgery should be consulted
troponin, blunt cardiac injury (BCI) is effec- immediately for possible operative interven-
tively ruled out. Continue with trauma work- tion. Consider inserting a pulmonary artery
up per chief complaint and mechanism of catheter (PAC) to guide fluid resuscitation
injury. when cardiac abnormalities are present, as it
is important to avoid fluid overload in these
N. R. Manley · G. O. Maish III (*) patients. Once trauma work-up is complete,
Department of Surgery, University of Tennessee these patients require ICU admission, con-
Health Science Center, Memphis, TN, USA tinuous telemetry, and close observation.

Patient with blunt trauma to

anterior chest and concern for BCI
· EKG: New arrhythmia, ST

changes, heart block,
ischemia, etc.
· Troponin: Baseline and trend
· PMH: Pre-existing cardiac
abnormality, meds, previous
stents, functional capacity, etc.
Abnormal EKG or abnormal Hemodynamically

A Normal EKG B troponin; hemodynamically
C unstable
and Troponin
stable
· No BCI · ATLS & ACLS

· Continue · Cardiology · ICU admit
trauma consult · Cardiology
work up · Consider consult
ECHO · ECHO
· Specific · Consider PAC
management · Specific
per etiology[1] management per
etiology[1]
1. Specific Management:
· Valve, septum or ventricular wall injury: Cardiothoracic surgery consult
· Acute coronary syndrome: ACLS protocols, cardiology consult, possible
catheterization with stent, avoid thrombolytics
· Cardiac dysfunction: Cardiology consult and ECHO
· Arrhythmias: ACLS protocols. If patient tachycardic, assume hemorrhage until
proven otherwise in the setting of trauma
Abbreviations: ACLS: Advanced cardiac life support; ATLS: Advanced trauma life
support; BCI: Blunt cardiac injury; EKG: Electrocardiogram; ECHO: Echocardiogram;
ICU: Intensive care unit; PAC: Pulmonary artery catheter; PMH: Past medical history
Algorithm 156.1
156 Blunt Cardiac Injury 639
Suggested Reading Clancy K, Velopulos C, Bilaniuk JW, Collier B, Crowley

W, Kurek S, et al. Screening for blunt c ardiac injury:
An Eastern Association for the Surgery of Trauma
American College of Surgeons. Advanced trauma life sup-
practice management guide. J Trauma Acute Care
port. 9th ed. Chicago: American College of Surgeons;
Surg. 2012;73(5 Suppl 4):S301–6.
2012. 336 p.
Legome E, Kadish H. Cardiac injury from blunt trauma.
American Heart Association. Advanced cardiac life
Up to Date [Internet]. 2017 Jul [cited 2017 Aug 13].
Support. Dallas: American Heart Association; 2015.
Available from: http://www.uptodate.com/contents/
183 p.
cardiac-injury-from-blunt-trauma.
Deceleration Injury: Blunt Aortic
Injury 157
Algorithmic Approach enough, a CT of the chest with contrast

should be obtained.
A. Blunt aortic injury should be suspected in any D. If an aortic injury is found on CT, urgent vascu-
patient involved in high-speed motor vehicle lar and/or thoracic surgery consultation is rec-
crash or fall from a great height. The follow- ommended, depending on location of injury.
ing algorithm should be followed in the stable Heart rate should be maintained below 100
patient. beats per minute and blood pressure less than
B. Initial management of any trauma patient pre- 100 mmHg by using medication. Esmolol, a
senting to the trauma bay should follow beta-blocker, is recommended, given its short
advanced trauma life support (ATLS) proto- half-life. If a beta-blocker is contraindicated,
cols. It is especially important in patients diltiazem, a calcium channel blocker, can be
with a high degree of suspicion for blunt aor- used. If esmolol is insufficient in controlling
tic injury to have two large-bore IVs placed blood pressure, Nicardipine is another option
for administration of fluid and medications to and can be easily titrated. Nitroglycerin or nitro-
control heart rate and blood pressure. Baseline prusside can be added to achieve target blood
labs (CBC, BMP, PT/INR, PTT, lactate and pressure as second-line agents. Please be aware
ABG) should be sent. that nitroprusside can cause reflex tachycardia
C. Initial work-up for suspected blunt aortic and, therefore, increase ventricular contractility
injury is done with an anterior-posterior por- (dP/dt). Vascular and/or thoracic surgery may
table chest x-ray. Common features indicat- elect to pursue an arch angiogram prior to repair.
ing aortic injury include widened Both endovascular and open approaches to aor-
mediastinum, abnormal-appearing aortic tic injury are possible, although current recom-
arch, left “apical cap” (i.E., blood above apex mendations favor the endovascular approach
of left lung), left hemothorax, displacement unless the patient is hemodynamically unstable
of the left main stem bronchus, deviation of and the patient has other obvious injuries requir-
trachea to the right, and wide left paraverte- ing operative exploration. Aortic injuries based
bral stripe. Next, if the patient is stable on CT findings and general management guide-
lines are as follows:
• Type I: Intimal tear. Typically managed
N. R. Manley · G. O. Maish III (*) conservatively with heart rate (<100 BPM)
Department of Surgery, University of Tennessee and blood pressure (<100 mmHg) control,
Health Science Center, Memphis, TN, USA as well as serial imaging (although there is

not currently any standard timing for fol- • Type IV: Rupture. Endovascular or opera-
low-up imaging—use clinical judgment). tive intervention is recommended.
• Type II: Intramural hematoma. Endovascular E. If the chest CT is negative for blunt aortic
or operative intervention is recommended. injury, the trauma work-up should proceed
• Type III: Pseudoaneurysm. Endovascular based on history, physical exam, and mecha-
or operative intervention is recommended. nism of injury.
A Patient with suspected blunt aortic injury
B ATLS
C Chest x-ray
CT chest
D Positive Negative E
· Control BP < 100 mm Continue work

Hg & HR < 100 BPM up for other
· Vascular and/or thoracic injuries
surgery consult
· Consider arch angiogram
· Possible open vs
endovascular surgery
Grade of aortic injury on CT: Recommended medications

· Type I: Intimal tear Non- for HR and BP control:
operative management · Esmolol: Rapid acting beta
· Type II: Intramural hematoma blocker with short half-life
Endovascular or operative · Diltiazem: Can be used if beta
management blocker contraindicated
· Type III: Pseudoaneurysm · Nicardipine: Easily titratable
Endovascular or operative · Nitroglycerin: Can be added if
management esmolol not controlling BP alone
· Type IV: Rupture · Nitroprusside: Can be added if
Endovascular or operative esmolol not controlling BP alone
management
Abbreviations: ATLS: Advanced trauma life support; BP: Blood pressure; BPM:
Beats per minute; CT: Computed tomography.
Algorithm 157.1
157 Deceleration Injury: Blunt Aortic Injury 643
Suggested Reading blunt traumatic aortic injury: a practice manage-

ment guideline from the Eastern Association for
the Surgery of Trauma. J Trauma Acute Care Surg.
American College of Surgeons. Advanced trauma life sup-
2015;78(1):136–46.
port. 9th ed. Chicago: American College of Surgeons;
Neschis DG. Blunt thoracic aortic injury. Up to
2012. 336 p.
Date [Internet]. 2017 May [cited 2017 Aug 14].
Fox N, Schwartz D, Salazar JH, Haut ER, Dahm P,
Available from: https://www.uptodate.com/contents/
Black JH, et al. Evaluation and management of
blunt-thoracic-aortic-injury.
Penetrating Abdominal Trauma
158
Algorithmic Approach have careful but rapid evaluation of the abdo-

men; patients with peritoneal signs or signs of
A. Under most circumstances, patients with
fascial penetration should be taken to the
penetrating abdominal trauma are brought operating room. In patients without these
to the emergency room as a leveled trauma. clear signs, a focused assessment sonography
Immediately, the patient should have his or for trauma (FAST) exam should follow; those
her vital signs measured. Initial evaluation patients with free fluid should be taken to the
should begin by obtaining a history from the operating room for exploration. In isolated
patient, if able, and additional information penetrating abdominal injury, the area of pen-
from emergency medical transport. The etration should be marked with a radio-
advanced trauma life support (ATLS) algo- opaque marker such as a paper clip. Upright
rithm should be followed and chest tubes chest x-ray (CXR) will demonstrate the pres-
placed as needed. The most critical infor- ence of free air. Abdominal and pelvic roent-
mation that needs to be obtained from the genograms should be taken to identify foreign
history is the type of penetration (high or bodies, noting their location to the radio-
low energy), the location of the wounds, the opaque markers.
approximate time that has passed since the D. Patients who sustained high-energy penetrat-
injury, and the hemodynamic stability of the ing abdominal trauma (gunshot wounds) and
patient. are hemodynamically stable, without free
B. If hemodynamic instability is encountered in fluid on FAST, warrant computerized tomog-
penetrating abdominal injury, the patient raphy to further elucidate the path of the bul-
should be immediately transported to the let and any associated injuries. It is not
operating room for emergent laparotomy. uncommon, especially in the obese patient,
C. During ATLS work-up of the patient, con- that a bullet track is seen in the soft tissues
comitant thoracic trauma should be evaluated without fascial violation. Wound irrigation
for. Hemodynamically stable patients should and light packing of the bullet wound sites
should occur. It should also be noted that bul-
M. Smith lets may cause thermal injury to the surround-
Department of Surgery, Westchester Medical Center, ing structures and blast injury from the
Valhalla, NY, USA temporary cavitation that occurs; therefore, it
F. Vinces (*) is not unwise to admit these patients for over-
Department of Surgery, Vassar Brothers Medical night observation on NPO status while
Center, Poughkeepsie, NY, USA

646 M. Smith and F. Vinces
performing serial abdominal exams. Any wounds, it may be beneficial to irrigate and
intra-abdominal path of the bullet warrants debride these in the operating room as well as
immediate exploration. place a drain and provide additional IV anti-
E. In patients that have sustained low-energy biotics during an overnight observation
penetrating abdominal trauma (knife wounds, period. If fascial violation is identified, at this
glass lacerations, etc.), local wound explora- point, diagnostic peritoneal aspiration (DPA)
tion is indicated. This may be performed with and diagnostic peritoneal lavage (DPL) may
local anesthesia and by lengthening the lac- be beneficial to determine if the patient
eration by one to two centimeters at each end. requires further exploration. Positive DPA/
If no fascial penetration is identified, hemo- DPL should then undergo either diagnostic
stasis, irrigation, and wound closure may be laparoscopy or exploratory laparotomy based
performed, based upon the level of contami- upon the comfort level of the performing
nation of the wound. In grossly contaminated surgeon.
158 Penetrating Abdominal Trauma 647
A Penetrating abdominal injury
Vital signs
Hemodynamically No
B stable?
Yes
Evisceration or Yes
peritoneal? Exploratory
laparotomy
Yes
C CXR –free air?
Positive
FAST exam
High energy Low energy
Local wound
exploration E
D Computerized
tomography
Fascial
Intraperitoneal penetration?
Extraperitoneal
injury injury
Yes No
Observation and Laparotomy / Wound management

discharge laparoscopy and discharge
Algorithm 158.1
Suggested Reading Gonzalez RP, Turk B, Famlimirski M, Holevar

M. Abdominal stab wounds: diagnostic peritoneal
lavage criteria for emergency room discharge. J
American College of Surgeons. ATLS student course
Trauma Inj Infect Crit Care. 2001;51:939–43.
manual: advanced trauma life support. 9th ed.
Zajtchuk R, Jenkins DP, Bellamy RF, Quick CM, editors.
Chicago: American College of Surgeons; 2012.
Warfare, weaponry, and the casualty. In: Textbook of
Biffl WL, Leppaniemi A. Management guideline for
military medicine. Office of the Surgeon General,
penetrating abdominal trauma. World J Surg.
Department of the Army, United States of America;
2015;39:1373–80.
1991.
Blunt Abdominal Trauma
159
Algorithmic Approach should be noted and recorded. Careful per-

cussion and palpation of the abdomen noting
A. Management of suspected blunt abdominal any areas of fullness or tenderness should be
trauma begins immediately upon viewing the undertaken.
patient. The patient that is lying still and C. A focused abdominal sonography for trauma
extremely tenuous to move has either a sig- (FAST) should be performed. Fluid is identi-
nificant intra-abdominal injury or an injury of fiable on FAST exam when 250 mL has accu-
the lower extremity. However, even in those mulated. A FAST exam should not take more
patients who appear comfortable, careful than 60 s. A caveat is in the patient with
evaluation is required. The patient’s vital underlying cirrhosis and ascites. If the history
signs should be immediately measured and taken suggests this and the patient has a large
with routine repeated measurements. A thor- amount of free fluid, while hemodynamically
ough history should be taken. Stabilization of stable, the patient should be taken for a com-
all patients should be performed in accor- puterized tomography (CT) with intravenous
dance with advanced trauma life support contrast.
algorithm (airway, breathing, circulation, D.
The hemodynamically unstable patient
etc.). Large-bore intravenous access should should have packed red blood cell and fresh
be established, and blood should be drawn for frozen plasma transfused and the operating
analysis, including a type and cross, if the room should be notified. The hemodynami-
need arises for blood transfusion. Upright cally stable patient should be transported with
chest x-ray (CXR) should be performed to physician escort to computerized tomography
rule out associated thoracic trauma and free (CT) scan. CT scan with arterial phase and
intra-abdominal air. delayed phase contrast should be performed.
B. Physical exam should follow. A careful and If an arterial blush is seen on the computer-
systematic examination of the abdomen must ized tomographic injury, the hemodynami-
be undertaken. All ecchymosis or markings cally stable patient should undergo
angiography and embolization. The patient
M. Smith should then be observed, serial hemoglobin
Department of Surgery, Westchester Medical Center, and hematocrit should be monitored, and
Valhalla, NY, USA serial abdominal exams should be performed.
F. Vinces (*) If the patient continues to hemorrhage or if
Department of Surgery, Vassar Brothers Medical the serial abdominal exams demonstrate

p eritoneal signs, the patient should be taken namically stable patient, observation, serial
for laparotomy or laparoscopy (with low hematocrits, and serial abdominal exam may
threshold to open conversion) based upon the be performed with a low threshold for emer-
comfort of the surgeon. gent laparoscopy converted to laparotomy.
E. If the CT scan demonstrates no injury, the Diagnostic peritoneal aspiration (DPA) or
patient may be observed and discharged if diagnostic peritoneal lavage (DPL) can be
they remain hemodynamically stable. If CT performed; if positive, the patient should be
scan demonstrates free fluid without obvious prepared for laparotomy. If the DPA/DPL is
solid organ injury (FFWOSOI), the patient negative, the patient may be discharged.
must be observed carefully. In the hemody-
Vital signs History

Suspected abdominal trauma 25 year-old as possible Initial
female ejected from a motor vehicle complaining A stabilization Initial B
of back and low abdominal pain Abdominal Exam
Focused
C abdominal
sonography
Free fluid No free fluid
No
Exploratory Hemodynamically
D stable?
D
laparotomy
Yes
CT scan E
Active Free fluid

extravasation No injury without
extravasation
or
Hemodynamically
stable? DPA /
No DPL
Yes (+)
(-)
Angiography Laparoscopy /
and laparotomy
embolization
Observation,
Failure serial Discharge
abdominal
exams
Algorithm 159.1
159 Blunt Abdominal Trauma 651
Suggested Reading injury following blunt trauma becomes clinically

apparent within 9 hours. J Trauma Acute Care Surg.
2014;76:1020–3.
Carter JW, Falco MH, Chopko MS, Flynn WJ, Wiles CE, Kelley SR, Tsuei BJ, Bernard AC, Boulanger BR,
Guo WA. Do we really rely on FAST for decision- Kearney PA, Chang PK. The effectiveness of focused
making in the management of blunt abdominal assessment with sonography for trauma in evaluating
trauma? Injury. 2014; https://doi.org/10.1016/j. blunt abdominal trauma with a seatbelt mark sign. J
injury.2014.11.023. Curr Surg. 2014;4:17–22.
2. Chereau N, Wagner M, Tresallet C, Lucidarme O, Moore FA, Davis JW, Moore EE, Cocanour CS, West MA,
Raux M, Menegaux F. CT scan and diagnostic peri- McIntyre RC. Western Trauma Association (WTA)
toneal lavage: towards a better diagnosis in the area critical decisions in trauma: management of adult
of operative management of blunt abdominal trauma. blunt splenic trauma. J Trauma. 2008;65:1007–11.
Injury. 2016;47:2006–11. Ng AKT, Simons RK, Torreggiani WC, Ho SG,
Dammers D, El Moumni M, Hoogland II, Veeger N, Kirkpatrick AW, Brown DRG. Intra-abdominal free
ter Avest E. Should we perform a FAST exam in fluid without solid organ injury in blunt abdominal
hemodynamically stable patient presenting after trauma: an indication for laparotomy. J Trauma Inj
blunt abdominal injury: a retrospective cohort study. Infec Crit Care. 2002;52:1134–40.
Scand J Trauma Resus Em Med. 2017; https://doi. Robinson JD, Sandstrom CK, Lehnert BE, Gross
org/10.1186/s13049-016-0342-0. JA. Imaging of blunt abdominal solid organ trauma.
Harris HW, Morabito DJ, Mackersie RC, Halvorsen RA, Sem Roentgen. 2016;51:215–29.
Schecter WP. Leukocytosis and free fluid are impor- Rodriguez C, Barone JE, Wilbanks TO, Rha C-K, Miller
tant indicators of isolated intestinal injury after blunt K. Isolated free fluid on computed tomographic scan
trauma. J Trauma Inj Infec Crit Care. 1999;46:656–9. in blunt abdominal trauma: a systematic review of
Jones EL, Stovall RT, Jones TS, Bensard DD, Burlew incidence and management. J Trauma Inj Infect Crit
CC, Johnson JL, Jurkovich GJ, et al. Intra-abdominal Care. 2002;53:79–85.
Management Algorithm for Acute
and Chronic Diaphragmatic 160
Injuries
Elif Onursal and Fausto Vinces
Algorithmic Approach [3]. It is important to note that these imaging

studies are intended more to rule in a dia-
A. Diagnosis of diaphragmatic injury in the
phragmatic injury rather than to rule one out.
acute setting presents a unique challenge to C. If clinical suspicion of a diaphragmatic injury
many clinicians as these injuries are often persists after imaging studies, operative inter-
asymptomatic or masked by other concomi- vention to establish a diagnosis is mandated
tant injuries. As such, the most important tool [4]. Operative approach for management of
in the initial assessment of the patient with diaphragmatic injury is stratified based upon
diaphragmatic injury is a high index of suspi- chronicity of injury, the presence of concomi-
cion [1]. Primary and secondary surveys tant injuries, and the hemodynamic stability
should be completed as outlined by advanced of the patient [3].
trauma life support (ATLS) protocol [2]. D. For acute injuries in which there is no other
After establishing that the patient is hemody- indication for laparotomy, a minimally inva-
namically stable, additional history should sive technique may be employed with prefer-
include questions regarding the mechanism ence given to thoracoscopy over laparoscopy
of injury, severity of impact, and trajectory of due to an increased risk of precipitating ipsi-
any missile-related injuries [3]. lateral tension pneumothorax during estab-
B. Once hemodynamic stability has been establishment of pneumoperitoneum [3]. For acute
lished, adjuncts to history and physical may diaphragmatic injuries associated with con-
include the focused abdominal sonography comitant injury, operative approach is via
for trauma (FAST) exam (may detect large laparotomy with trans-diaphragmatic explo-
ruptures associated with blunt force), plain ration of the ipsilateral thoracic cavity to rule
chest radiographs (intra-abdominal contents out further hemorrhage or injury [1].
may be seen in the left hemithorax), or helical E. In the event that a damage control laparotomy
computed tomography (CT) scan of the chest, in a hemodynamically unstable patient
abdomen, and pelvis with 3D reconstructions reveals a diaphragmatic injury, it is consid-
ered acceptable to temporarily pack any
E. Onursal defect found with laparotomy pads and return
Department of General Surgery, St. Barnabas for definitive repair once the patient is stabi-
Hospital Health System, Bronx, NY, USA lized [3].
F. Vinces (*) F. For chronic injuries, many favor thoracot-
Department of Surgery, Vassar Brothers Medical omy due to tendency of dense pleural

654 E. Onursal and F. Vinces
adhesions to form in this setting requiring tion and re-implantation of the diaphragm
extensive lysis. cephalad by 1–2 interspaces [1].
G. Intraoperative technique in all settings is
J. In the event of gross spillage of intestinal
dependent upon size of defect and degree of contents, a washout of the thoracic cavity
contamination from associated injury or per- should be done using saline irrigation con-
foration of a strangulated viscus [5]. taining antibiotics [3]. The use of synthetic
H. Because of the natural progression of dia- mesh should be avoided in these cases in
phragmatic defects toward herniation, stran- favor of autologous tissue (latissimus dorsi,
gulation, and obstruction or perforation of rectus abdominis, or external oblique muscle
intra-abdominal viscera, all defects should be flaps) or biologic mesh (that is replaced with
repaired regardless of size [3]. All non-viable synthetic mesh at a later date) [1].
tissue should be debrided. Primary repairs K. Postoperatively, the clinician should be mind-
should be conducted using non-absorbable ful of complications such as breakdown of
suture due to an increased risk of recurrence repair, iatrogenic injuries to the phrenic nerve
when absorbable sutures are utilized [1]. leading to hemiparesis of the diaphragm, atel-
I. Defects less than 8 cm in size can be repaired ectasis, empyema, pneumonia, and morbidity
primarily. Defects larger than this size favor related to concomitant injuries [3].
prosthetic synthetic mesh repair or transposi-
160 Management Algorithm for Acute and Chronic Diaphragmatic Injuries 655
Traumatic injury to thoraco-

abdominal region A
Damage control No
laparotomy with Perform
temporary packing of Patient HD stable? primary
any diaphragmatic survey
injury found
C
Yes
Proceed to chronic Secondary survey

including imaging No
diaphragmatic injury Findings concerning for or Manage other injuries
algorithm (CXR, CT scan or
diagnostic of diaphragm present
FAST exam)
injury?
F B E
Yes
Minimally invasive
techniques: No Indications for
thoracoscopy > laparotomy present?
laparoscopy
D Yes
Perform exploratory laparotomy
Explore ipsilateral thorax

through defect to rule out G
Primary repair with non- hemorrhage and further injury
absorbable suture
Yes H
Washout thorax with saline Yes

I Defect < 8 cm in antibiotic irrigation + avoid use
Gross contamination
present?
size? of synthetic mesh for repair
No J No
Prosthetic biologic mesh repair OR

autologous tissue repair OR transposition Yes
and re-implantation of diaphragm cephalad I Defect < 8 cm in
Primary repair with non-
absorbable suture
size?
No
Prosthetic
synthetic mesh
repair
Algorithm 160.1
656 E. Onursal and F. Vinces
Perform history &

physical exam
A
Obtain adjunctive imaging

studies: CXR, US, CT scan B
Perform thoracotomy and lysis

of adhesions and reduction of F
abdominal contents
Washout thorax with saline Yes

Gross contamination
H antibiotic irrigation + avoid use
present?
G
of synthetic mesh for repair
No
Yes Defect <8

Primary repair with non-
absorbable suture
cm in
size?
I Defect <8 Yes Primary repair with non-
cm in
absorbable suture
size?
No
No
Prosthetic biologic mesh repair or Prosthetic synthetic

autologous tissue repair OR transposition mesh repair
and re-implantation of diaphragm cephalad
Algorithm 160.2
3. Cameron J, Cameron A. Cameron current surgical ther-

References apy. 11th ed. Philadelphia: Saunders; 2014. p. 2692–9.
4. Brunicardi F, Anderson D, Billiar T, Dunn D, Hunter

1. Mattox K, Moore E, Feliciano D. Trauma. 7th J, Matthews J, Pollock R. Schwartz’s principles of sur-
ed. New York: McGraw-Hill Medical; 2013. gery. 9th ed. New York: McGraw-Hill; 2010. p. 324.
p. 901–17. 5. Townsend C, Beauchamp R, Evers B, Mattox

2. American College of Surgeons. Advanced trauma K. Sabiston textbook of surgery: the biological basis
life support. 9th ed. Chicago: American College of of modern surgical practice. 19th ed. Philadelphia:
Surgeons; 2013. Saunders; 2012. p. 454–5.
Management of Traumatic Liver
Injuries 161
Melissa Amberger and Fausto Vinces
Algorithmic Approach B. As part of the primary survey, circulation or

hemodynamic (HD) stability should be
A. As with all trauma patients, initial evaluation assessed. Tachycardia and hypotension are
of the patient with a traumatic injury concern- hallmarks of intravascular depletion and
ing for hepatic injury should be taken accord- should prompt investigation for source of
ing to the advanced trauma life support hemorrhage in the traumatically injured
(ATLS) algorithm [1]. This should include an patient. This includes a focused assessment
immediate primary survey assessing airway with sonography in trauma (FAST) exam or a
(A), breathing (B), circulation (C), disability diagnostic peritoneal lavage (DPL) to evaluate
(D), and exposure (E). Once a patient is ini- for intra-abdominal source of hemorrhage.
tially stabilized during the primary survey, a Any study positive for intra-abdominal source
further history is elicited and can be taken of hemorrhage in a HD-unstable patient
from emergency medical personnel or should prompt emergent laparotomy [2].
bystanders if the patient is obtunded or intu- C. In patients who are HD stable with a mecha-
bated and unable to provide history. A focused nism concerning for solid organ injury,
history should include allergies, medications, including hepatic injury, a computed tomog-
past medical history, last meal, and events or raphy (CT) scan [2] should be completed
mechanism of injury (AMPLE). A complete with intravenous contrast administration to
head to toe physical should then occur inves- evaluate for injuries with active hemorrhage.
tigating for any signs of traumatic injury. Any Hepatic injury is graded using a radiographi-
decompensation in clinical status should cally or clinically based system [3] developed
prompt a re-evaluation of the primary by the American Association for the Surgery
survey. of Trauma (AAST). Increasing grade of
injury is associated with higher likelihood of
need for angioembolization or operative man-
agement [4].
D. Angioembolization of the proper hepatic

M. Amberger artery or segmental branches can be per-
Department of Surgery, St. Barnabas Health System, formed to control hepatic hemorrhage. This is
Bronx, NY, USA particularly useful in the traumatically injured
F. Vinces (*) patient who is HD stable, but with active
Department of Surgery, Vassar Brothers Medical extravasation of contrast (a blush) on CT

658 M. Amberger and F. Vinces
scans [5]. Angioembolization can be used as placed in all four quadrants of the abdomen.
an adjunct to operative intervention for The decision to pursue definitive repair ver-
further control of hemorrhage. Additionally it sus damage control measures should be made
can be used in the patient admitted and man- early so as to minimize risk of progression to
aged non-operatively with ongoing transfu- the lethal triad (hypotension, acidosis, and
sion requirements as a primary intervention coagulopathy) [6]. If packing does not suc-
for control of hemorrhage [6]. cessfully obtain hemostasis of liver injury
E. Patients with hepatic injury who are HD sta- then a Pringle maneuver (intermittent clamp-
ble without a blush on CT scan or who are ing of the hepatoduodenal ligament and its
HD stable with a blush on CT scan who have contained structures) can be employed [7].
undergone angioembolization should be This is successful for many liver injuries with
admitted for observation. Serial monitoring the exception of a retrohepatic caval injury.
of hemoglobin or hematocrit should be under- This particular injury has been treated with
taken. The patient should receive serial cavo-atrial shunting but is often fatal [8].
abdominal examinations, initially be kept nil G. After successful management of hepatic

per os (NPO), be placed initially on bed rest, injury, the practitioner should remain wary
and be closely HD monitored. If clinical for complications of hepatic injury such as a
decompensation occurs or there are ongoing liver abscess, biloma (which may require
transfusion requirements, the patient should ERCP for management) [8], biliary ascites, or
undergo angioembolization. If this has hemoperitoneum. These entities can be man-
already been performed and is deemed unsuc- aged with a combination of interventional
cessful, operative exploration is mandated. radiology or laparoscopically placed drainage
F. Operative exploration should proceed via catheters. In the case of hemobilia, repeated
exploratory laparotomy. Packing should be angioembolization may be required [9].
161 Management of Traumatic Liver Injuries 659
A Initial assessment of trauma

patient w/ suspected Hepatic Injury
HD unstable or Yes FAST exam or

B peritonitis? DPL
No
C Positive? No
CT scan demonstrating liver injury
Continue resuscitation
Consider other sources of
No instability
Blush Yes Repeat FAST/DPL
Yes
No Emergent
Continued laparotomy/damage F
HD stability? control surgery
Yes
No Previous IR
D Angioembolization w/ embolization
interventional radiology ??
No
Admission for serial abdominal exams, Continued HD

E serial hemoglobin measurements, HD Stability?
monitoring
Yes
G Follow up Care
Algorithm 161.1
660 M. Amberger and F. Vinces
References 5. East.org. Blunt hepatic injury, selective nonopera-

tive management of – practice management guide-
line. 2012. [online] Available at: http://www.east.org/
1. American College of Surgeons. Advanced trauma
education/practice-management-guidelines/blunt-
life support. 9th ed. Chicago: American College of
hepatic-injury%2c-selective-nonoperative-manage-
Surgeons; 2013.
ment-of [Accessed 30 Oct 2017].
2. East.org. Blunt abdominal trauma, evaluation of –
6. Kozar R. WTAAlgorithms. n.d. [online]
practice management guideline. [online] East.
Westerntrauma.org. Available at: http://www.western-
org. Available at: http://www.east.org/education/
trauma.org/algorithms/WTAAlgorithms_files/gif_3.
practice-management-guidelines/blunt-abdominal-
htm [Accessed 30 Oct 2017].
trauma%2c-evaluation-of [Accessed 30 Oct 2017].
7. Kozar R. WTAAlgorithms. [online] Westerntrauma.
3. Moore E, Cogbill T, Malangoni M, Jurkovich G,
org. n.d. Available at: http://www.westerntrauma.
Champion H. Injury scoring scales – the American
org/algorithms/WTAAlgorithms_files/gif_5.htm.
Association for the Surgery of Trauma. [online]
[Accessed 30 Oct 2017].
Aast.org. 1994. Available at: http://www.aast.org/
8. Hirshberg A, Mattox K. Top knife. Nr Shrewsbury:
Library/TraumaTools/InjuryScoringScales.aspx#liver
TFM; 2005. p. 83–98.
[Accessed 30 Oct 2017].
9. Baillie J. Hemobilia. Gastroenterol Hepatol.
4. Mattox K, Moore E, Feliciano D. Trauma. 7th ed.
2012;8(4):270–2.
New York: McGraw-Hill Medical; 2013.
Management of Pancreatic Trauma
162
Shreya Jammula and Eric H. Bradburn
Algorithmic Approach (b) It is important to serially follow levels of

enzymes. Repeat abdominal CT scan
A. Mechanism of injury (MOI) is important to may be warranted.
discern as it can provide valuable information (c) In the event of positive findings on abdom-
on patient presentation. Blunt pancreatic inal CT and/or rising enzyme levels,
injury can be notoriously difficult to diagnose exploratory laparotomy may be indicated.
but presence of concurrent abdominal injuries C. In the absence of CT findings and normal levels
can result in distinct physical exam findings, of enzymes, additional diagnostic imaging may
including shock and peritonitis. Initial workup be warranted. Magnetic resonance cholangio-
with positive findings on focused assessment pancreatography (MRCP) is a noninvasive
with sonography in trauma (FAST) and/or diagnostic imaging modality that can help diag-
diagnostic peritoneal lavage (DPL) can also nose injury to the pancreatic duct. Visualization
help aid diagnosis and provide clear indica- of a normal duct indicates preserved pancreatic
tions for exploratory laparotomy. duct and likely does not require surgery.
B. For blunt MOI, it is important to maintain a high (a) MRCP should only be attempted on

index of suspicion in order to accurately diag- hemodynamically stable patients and
nose pancreatic injury as physical exam and ini- does not offer any therapeutic value.
tial imaging can frequently be negative [1]. D. The presence of extravasation on MRCP indi-
(a) It is important to obtain additional imag- cates ductal injury and likely warrants surgi-
ing such as an abdominal computed cal exploration. The degree of extravasation
tomography (CT) scan as well as serum and location of ductal injury (proximal vs.
lipase and amylase levels. distal) can affect decision to preserve/resect
(i) It should be noted that elevated serum portions of the pancreas.
lipase/amylase levels are not diagnos- E. Penetrating pancreatic injury typically war-
tic of pancreatic injury as elevations rants surgical exploration. The presence of
can occur secondary to other sources non-specific physical exam findings such as
of injury. shock and peritonitis as well as positive find-
ings on initial diagnostic imaging makes it
S. Jammula · E. H. Bradburn (*) easier to diagnose. In addition, certain mech-
Department of Trauma and Acute Care Surgery, Penn anisms of injury themselves can prompt an
Medicine Lancaster General Health, automatic exploratory laparotomy i rrespective
Lancaster, PA, USA of presence of other findings.

662 S. Jammula and E. H. Bradburn
F. Once the appropriate surgical exposure has G. Negative findings on cholecystocholangiog-

been achieved, a thorough inspection of the raphy warrant conservative therapy with pres-
entire pancreas must be performed. ervation of pancreatic tissue. A surgical drain
(a) Pancreatic injury can be graded on a scale (closed suction) is indicated.
from I to V, with Grades I–II indicating H.
Extravasation indicates ductal injury.
preservation of pancreatic duct and Location of ductal injury is important as
majority of pancreatic tissue. proximal injury can be managed with a surgi-
(b) Once the pancreatic tissue has been exam- cal drain without pancreatic resection. Distal
ined, direct inspection of the pancreatic ductal injury requires pancreatectomy.
duct should also be performed. In addi- (a) It should be noted that extensive pancre-
tion, a fluoroscopic cholecystocholan- atic injury with or without ductal involve-
giography can be performed for definitive ment may warrant pancreatectomy and/or
knowledge regarding status of the duct. splenectomy [2, 3].
Blunt
mechanism
of injury
A B
Findings: shock, peritonitis, + FAST, + Minimal/no physical exam findings,

DPL normal FAST
Exploratory Enzyme assays,

laparotomy abdominal CT
scab
F, G, H + –
Exploratory MRCP (stable

C
laparotomy patient)
Extravasation –
D Ductal injury Conservative management
Exploratory
laparotomy
H
Algorithm 162.1
162 Management of Pancreatic Trauma 663
Penetrating
mechanism of
injury
Non-specific indications: shock, peritonitis, Mechanism of injury (transperitoneal

+ FAST, + DPL, + abdominal CT scan gunshot wound)
Exploratory laparotomy
Fluoroscopic
F ERCP optional
cholecystocholangiography
Extravasation –
Conservative therapy (closed

H Ductal injury
suction drainage) G
Proximal Distal
Closed suction Distal

drainage pancreatectomy
Algorithm 162.2
664 S. Jammula and E. H. Bradburn
References 2. Jacobs LM, Gross RI, Luk SS. Advanced trauma oper-

ative management. Woodbury: Cine-Med, Inc; 2004.
3. Maggio PM, Clark D. Management of duodenal and pan-
1. Debi U, Kaur R, Prasad KK, Sinha SK, Sinha A,
creatic trauma in adults. In: Post TW, editor. UpToDate.
Singh K. Pancreatic trauma: A concise review. World
Waltham: UpToDate. Accessed on 29 Nov 2017.
J Gastroenterol. 2013;19(47):9003–11.
Management of Traumatic Splenic
Injuries 163
Eric H. Bradburn, Kameron Durante,
and Shreya Jammula
Algorithmic Approach positive FAST in the setting of hemodynamic

instability is an absolute indication for emer-
A. Splenic injury is commonly encountered in gent exploratory laparotomy. Diffuse perito-
the setting of blunt abdominal trauma, of nitis and decreasing hemoglobin suspected to
which motor vehicle collisions (MVCs) be secondary to splenic bleeding also warrant
account for roughly 75% [1]. Initial trauma prompt operative care [2].
evaluation follows the principles established C. In patients who did not initially require emer-
by advanced trauma life support (ATLS) in gent surgical intervention, computed tomog-
order to rapidly identify life-threatening inju- raphy (CT) scan with intravenous (IV)
ries and provide appropriate resuscitation [1, contrast is indicated as it can provide infor-
2]. Following primary assessment, a thorough mation regarding the grade of injury [2].
history and physical examination are essen- Grades I–III are classified as injuries to the
tial. Acute trauma to left thoracic/abdomen/ spleen up to lacerations greater than 3-cm
flank areas should prompt special consider- parenchymal depth or involving trabecular
ation of splenic injury. Physical findings can vessels as well as subscapular hematoma
include left upper quadrant pain, referred greater than 50% surface area/intraparenchy-
pain to the left shoulder (Kehr’s sign), abdom- mal equal to or greater than 5-cm or expand-
inal wall contusion, and peritonitis [2]. It is ing [2, 3]. Grades IV–V include laceration
vital to remember that some patients with sig- involving segmental or hilar vessels resulting
nificant splenic injuries can present without in major devascularization (>25%) up to
any remarkable physical exam findings. complete devascularization or shattered
B. Along with physical examination, vital signs, spleen [2, 3]. Grades I–III can be managed
diagnostic imaging, and laboratory values non-operatively, while grades IV–V are typi-
can be helpful in detecting injury and guiding cally managed surgically [3]. For grades I–
subsequent management. Abdominal trauma III, in the setting of a large hemoperitoneum
patients typically undergo focused assess- or ongoing bleeding, angiography could be
ment with sonography in trauma (FAST). A an alternative to laparotomy.
D. Angiography with or without embolization
E. H. Bradburn (*) · K. Durante · S. Jammula has been useful for patients managed non-
Department of Trauma and Acute Care Surgery, Penn operatively. A negative result can avoid
Medicine Lancaster General Health, unnecessary surgery, and a positive result can
Lancaster, PA, USA be treated with embolization. A retrospective

666 E. H. Bradburn et al.
analysis of blunt splenic injuries at a Level I with open approach regarded as the standard
trauma center determined that embolization of care [6]. Grades IV–V spleen typically
in non-operative patients was associated with necessitate surgery [3].
salvage rates of 92% [4]. Angioembolization G. Patients with splenic injuries are advised to
is not without risks as evidenced by incidence refrain from participating in contact sports
of complications in 20% of patients including and other high-risk activities for 3 months
failure to control bleeding [5]. following trauma [3]. While delayed rupture
E. All patients managed non-operatively should of splenic pseudoaneurysm is a documented
be monitored closely for changes in vitals complication in patients managed non-
(tachypnea, tachycardia, hemodynamic insta- operatively, repeat CT imaging is not per-
bility). Patients should also receive a secondary formed routinely, though there may be an
CT scan with IV contrast 48 h post-trauma [3]. indication in select patients to lift activity
F. Twenty to forty percent of patients with restrictions.
splenic injury warrant surgical intervention,
163 Management of Traumatic Splenic Injuries 667
History and physical examination

Blunt abdominal trauma and/or left upper quadrant abdominal tenderness
A
Obtain vital signs, physical examination
B
Hemodynamically
unstable?
peritonitis?
No Yes
F
CT scan abdomen with IV contrast to identify splenic Immediate

injury grade laparotomy
Grade I-III Grade IV-V
Ongoing splenic Consider

bleeding, moderate to angiography
Small hemoperitoneum large hemoperitoneum
F
E
If unsuccessful or
unable, immediate
laparotomy
Manage non-operatively, monitor closely, repeat CT
with IV contrast 48 h post trauma/symptoms
G Follow up care
Algorithm 163.1
References 4. Haan J, Scott J, Boyd-Kranis RL, Ho S, Kramer M,

Scalea TM. Admission angiography for blunt splenic
injury: advantages and pitfalls. J Trauma Acute Care
1. Diercks DB, Clarke S. Initial evaluation and man-
Surg. 2001;51(6):1161–5.
agement of blunt abdominal trauma in adults. In:
5. Stassen NA, Bhullar I, Cheng JD, Crandall ML,
UpToDate, Post TW, editors. UpToDate, Waltham,
Friese RS, Guillamondegui OD, Jawa RS, Maung
Accessed via https://www.uptodate.com/contents/ini-
AA, Rohs TJ, Sangosanya A, et al. Selective non-
tial-evaluation-and-management-of-blunt-abdominal-
operative management of blunt splenic injury: an
trauma-in-adults on 7 July, 2017.
Eastern Association for the Surgery of trauma practice
2. Maung AA, Kaplan LJ. Management of splenic injury
management guideline. J Trauma Acute Care Surg.
in the adult trauma patient. In: UpToDate, Post TW,
2012;73(5.4):S294–300.
editors. UpToDate, Waltham. Accessed via https://
6. Maung AA, Kaplan LJ. Surgical management of
www.uptodate.com/contents/management-of-splenic-
splenic injury in the adult trauma patient In: UpToDate,
injury-in-the-adult-trauma-patient on 7 July, 2017.
Post TW, editors. UpToDate, Waltham. Accessed via
3. Watson GA, Hoffman MK, Peitzman AB. Nonoperative
https://www.uptodate.com/contents/surgical-manage-
management of blunt splenic injury: what is new? Eur
ment-of-splenic-injury-in-the-adult-trauma-patient on
J Trauma Emerg Surg. 2015;41(3):219–28.
7 July, 2017.
Management of Kidney and Ureter
Injuries 164
Eric H. Bradburn, Madison Morgan,
and Danielle Von Nieda
lgorithmic Approach: Kidney

A of kidney injury, a CT scan of the abdomen
Injuries should be obtained. If an AKI is suspected by
radiology, the injury should be graded in
A. First, when performing an evaluation of a order to determine the next steps in treating
patient with an acute kidney injury (AKI), the the injury [3].
physician should recount the history and per- D. If the patient is determined to be hemody-
form a physical examination. AKI is defined namically unstable upon initial examination,
by a sudden decline in kidney function. The immediate surgical intervention is suggested.
major indicators of AKI are flank or abdomi- This intervention may include a laparotomy
nal pain and tenderness. Abdominal bruising to explore the abdomen to determine the
and skin rashes are also indicators of AKI, as mechanism and severity of injury, allowing
well as reported decreased urine output if the the physician to decide what further actions
evaluation does not immediately follow the are necessary [4].
injury [1]. E. Grade I injuries are likely microscopic contu-
B. Vital signs, blood work, and a physical exam- sions or subcapsular hematomas. Grade II
ination can provide essential information, injuries are nonexpanding perirenal hemato-
which may raise suspicion for an acute kid- mas or less than 1.0 cm parenchymal depth of
ney injury. An increase in blood urea nitro- renal cortex lacerations without urinary extrav-
gen, an electrolyte imbalance, and hematuria agation [4]. Grade I and II AKIs should be
are all concerns for AKI. An increase in managed with clinical observation, necessary
serum creatinine levels, a 50% or greater antibiotics, and a repeat of the blood work [2].
decrease in globular filtration rate, and a urine F. Grade III injuries are lacerations less than
output less than 0.5 ml/kg/h over 12 h are 1.0 cm parenchymal depth of renal cortex
additional concerns for kidney injury [2]. without collecting system rupture or urinary
C. If the patient is determined to be hemody- extravagation [4]. Grade III AKIs may or may
namically stable but presents with indicators not require surgical intervention. It is to the
physician’s discretion as to whether or not
surgery is required, in which case the injury
E. H. Bradburn (*) · M. Morgan · D. Von Nieda will follow grade IV protocol [2].
Department of Trauma and Acute Care Surgery, Penn G. Grade IV injuries are parenchymal lacera-
Medicine Lancaster General Health, tions that extend through the renal cortex,
Lancaster, PA, USA medulla, and collecting system, or vascular

main renal artery or vein injuries with con- [4]. Grade IV or V AKIs require immediate
tained hemorrhage. Grade V injuries include surgical intervention [2].
lacerations that completely shatter the kidney H. Follow up care should be obtained when

and vascular injuries with the avulsion of appropriate.
renal hilum which devascularizes the kidney

A Flank or abdominal pain and bruising
Decreased urine output
B Obtain vital signs, blood work and perform a physical examination
Is the patient
hemodynamically
stable?
Yes No
C Analyze blood work further Immediate surgical intervention D
Increased Creatinine x2 or GFR decrease > 50%,Urine

Output < .5 ml/kg/h × 12 h, electrolyte imbalance, and
hematuria are concerns for kidney injury.Obtain
a CT scan
What grade of
kidney injury
does CT show?
G
Grade 4-5
Grade 1-2
E
Grade 3
Observation, antibiotics, and
repeat labs
F
No
Do the
associated Yes
injuries require
a laparotomy? H
Follow-up
Algorithm 164.1
164 Management of Kidney and Ureter Injuries 671
lgorithmic Approach: Ureter

A nosed by surgical exploration due to the
Injuries traumatic nature of the injuries [8]. A normal
retrograde pyelography also requires no
A. When evaluating a patient with a suspected treatment.
ureter injury, the first step should be obtain- C. If the injury is detected initially and the
ing the history and performing a physical patient is not hemodynamically stable, imme-
exam. A major sign of a ureter injury is flank diate surgical intervention is necessary. The
pain. Penetrating injury near the ureter or a principles of repair include spatulation, lack
recent surgical procedure should also be of tension, stenting, postoperative drainage,
taken into consideration during evaluation. debridement, and a water-tight anastomosis
Ureter injuries are very uncommon. They with fine nonreactive absorbable suture [6].
make up less than 1% of genitourinary inju- D. A delay in diagnosis is the biggest factor con-
ries. Ureter injuries are to be suspected if tributing to morbidity in ureter injuries [6]. If
there is a multisystem injury, specifically if the ureter injury is not detected initially,
there is a penetrating injury near the ureter ultrasound (US), intravenous pyelogram
[5]. Most damage to the ureter occurs during (IVP), and/or CT should be done to diagnose
surgery, with the most commonly occurring the delayed injury. If an abscess or urinoma is
during gynecological surgeries or a surgical present, percutaneous nephrostomy and peri-
pelvic procedure [6]. ureteral drainage should be performed. In the
B. If the injury is detected initially, and the case of an incomplete urethral injury, retro-
patient appears to be hemodynamically sta- grade ureteral stenting should be considered
ble, a CT scan with delayed contrast should [5].
be performed [5]. If the CT is normal, no fur- E. If symptoms still persist following these pro-
ther treatment is necessary. However, if the cedures, surgical exploration should be
CT is nondiagnostic, retrograde pyelography considered.
should be done [7]. If either the CT or the F. No further treatment is necessary if the symp-
retrograde pyelography shows extravasation, toms are resolved.
surgical exploration/stenting should be com- G. Follow-up should be done in ureter injuries
pleted. Many ureter injuries are first diag- requiring surgical intervention.

Flank pain, penetrating injury to the abdomen, or recent surgical procedure
Is the ureter
injury detected
initially?
hemodynamically
D
Yes No
US, IVP, and/or CT

Is the patient
hemodynamically
Urinoma drainage and/or Urinary
stable?
diversion by stent or PCN
B Yes No C
Immediate surgical intervention Are

CT with delayed contrast
symptoms
persisting?
Not diagnostic
E No Yes F
Retrograde
Pyelography
Surgical
Extravasation Normal No treatment required
exploration
Surgical
exploration Follow-up
/stent
G
Algorithm 164.2
164 Management of Kidney and Ureter Injuries 673
References 5. Zinman LN, Vanni AJ. Surgical Management of

Urologic Trauma and Iatrogenic Injuries. Surg Clin
North Am. 2016;96(3):425–39.
1. Moore EE, Cogbill TH, Malangoni M, Jurkovich GJ,
6. Teber D, Egey A, Gozen AS, Rassweiler J. Ureteral
Champion HR. Injury scoring scale: a resource for
injuries. Diagnostic and treatment algorithm. Urologe
trauma care professionals: The American Association
A. 2005;44(8):870–7.
for the Surgery of Trauma; 2017. http://www.aast.org/
7. Asali MG, Romanowsky I, Kaneti J. External ureteral
Library/TraumaTools/InjuryScoringScales.aspx
injuries. Harefuah. 2007;146(9):686–9, 734.
2. Khwaja A. KDIGO clinical practice guideline for acute
8. Siram SM, Gerald SZ, Greene WR, Hughes K,
kidney injury. Nephron Clin Pract. 2012;120(4):179–84.
Oyetunji TA, Chrouser K. Ureteral trauma: patterns
3. Kalantarinia K. Novel imaging techniques in acute kid-
and mechanisms of injury of an uncommon condition.
ney injury. Curr Drug Targets. 2009;10(12):1184–9.
Am J Surg. 2010;199(4):566–70.
4. Rahman M, Shad F, Smith M. Acute kidney injury:
a guide to diagnosis and management. Am Fam
Physician. 2012;86(7):631–9.
Urethral Trauma
165
Cheyenne C. Sonntag and Susan MacDonald
Algorithmic Approach at the meatus to opacify the urethra while

oblique X-rays are obtained. Extravasation of
A. On arrival, the first step in the evaluation of contrast confirms urethral injury. The immedi-
patients with blunt trauma is the primary sur- ate primary goal of treatment is to obtain cath-
vey (ABCs) to identify and treat life- eter drainage of the bladder (suprapubic or
threatening conditions. urethral). A secondary goal of treatment may
B. Adjuncts and secondary survey are performed. be placement of a urethral catheter over a wire
Urethral injury is strongly suspected if there is placed into the bladder using a cystoscope by
blood at the urethral meatus, gross hematuria, the urology team in so-called primary realign-
or a high-riding, ballotable prostate on rectal ment to reduce future scarring or urethral
examination, especially if these findings are in stricture disease.
conjunction with a pelvic fracture. While D. In males, urethral injury is categorized by the
female urethral injury is rare and almost segment of the urethra it occurs in: the poste-
exclusively associated with pelvic fracture, it rior urethra (at or above membranous urethra)
should be suspected in cases of vaginal bleed- vs. the anterior urethra (penile or bulbar ure-
ing, severe pelvic fracture, labial edema, gross thra). Pelvic fracture urethral injury (PFUI) is
hematuria, or external genitalia injury [1–4]. an injury of the posterior urethra which occurs
If urethral injury is suspected, blind insertion in 1.5–10% of pelvic fractures and can occur
of a catheter should not be performed as it with concomitant bladder injury in up to 15%
risks conversion of partial laceration to a com- of cases [5, 6]. If PFUI is confirmed on retro-
plete urethral disruption. grade urethrogram, an evaluation of the blad-
C. If suspicion of urethral injury exists, a retro- der should also be considered with a CT
grade urethrogram should be performed. cystogram or filling through a catheter at the
Contrast is instilled through a catheter placed time of open exploration to identify any
lacerations.
E. Anterior injury may result from blunt (straddle
C. C. Sonntag
Department of Surgery, Penn State Milton S. Hershey injury), penetrating injury or penile fracture.
Medical Center, Hershey, PA, USA F. Penetrating injury to the anterior urethra should
S. MacDonald (*) be immediately surgically repaired, as long as
Division of Urology, Department of Surgery, Penn there is no major tissue loss or other major
State Milton S. Hershey Medical Center, injuries [4]. Devitalized tissue is debrided, and
Hershey, PA, USA the edges of the remaining urethra are

676 C. C. Sonntag and S. MacDonald
reapproximated over a catheter that will months after the initial injury and thus require
remain in place for 3–4 weeks postoperatively. referral to a urologist for follow-up and possi-
A peri-catheter retrograde urethrogram is then ble future intervention. Primary realignment of
performed to assess for appropriate healing the posterior urethral injury, where a catheter is
prior to removing the catheter. placed across the region of disruption, is
G. Straddle injury to the anterior urethra is treated increasingly common with advancements in
with suprapubic or urethral catheter drainage urethroscopic equipment; however, it should
and is associated with a high risk of future ure- only be performed by experienced providers.
thral stricture. Following primary realignment, a pericatheter
H. and I. Posterior urethral/PFUI injury is tradi- retrograde urethrography is performed at
tionally managed with suprapubic catheter 3–4 weeks.
drainage and reconstruction in a delayed fash- J. All patients should be followed for at least
ion 3–6 months later. Straddle injuries typi- 1 year to monitor for stricture, erectile dys-
cally result in anterior urethral stricture disease function or incontinence [4].
165 Urethral Trauma 677
Primary survey, adjuncts, secondary survey

A
No
Signs of
Continue with
B urethral
standard trauma
trauma?
assessment and care
Yes
Retrograde
C urethrogram
D Posterior/ PFUI Injury Anterior E

location?
Evaluation for Straddle Penetrating

concomitant
bladder injury
G F
I H
Primary realignment: Traditional:

1. Catheter placement 1. Immediate reconstruction
1. Experienced/specialist 1. Suprapubic catheter (suprapubic or urethral) 2. Pericatheter Retrograde
2. Pericatheter Retrograde 2. Reconstruction delayed 2. Remove catheter in Urethrogram in
Urethrogram in 3-4 weeks 3-6 months 3-4 weeks 3-4 weeks
J All: Follow-up at least 1 year
Algorithm 165.1
4. Morey AF, Brandes S, Dugi DD 3rd, Armstrong JH,

References Breyer BN, Broghammer JA, et al. Urotrauma: AUA
guideline. J Urol. 2014;192(2):327–35.
1. Venn SN, Greenwell TJ, Mundy AR. Pelvic fracture inju- 5. Bjurlin MA, Fantus RJ, Mellett MM, Goble
ries of the female urethra. BJU Int. 1999;83(6):626–30. SM. Genitourinary injuries in pelvic fracture mor-
2. Goldman HB, Idom CB Jr, Dmochowski RR. Traumatic bidity and mortality using the National Trauma Data
injuries of the female external genitalia and their associa- Bank. J Trauma. 2009;67(5):1033–9.
tion with urological injuries. J Urol. 1998;159(3):956–9. 6. Brandes S, Borrelli J Jr. Pelvic fracture and associated
3. Perry MO, Husmann DA. Urethral injuries in urologic injuries. World J Surg. 2001;25(12):1578–87.
female subjects following pelvic fractures. J Urol.
1992;147(1):139–43.
Pelvic Fractures
166
Algorithmic Approach ture include an abdominal seatbelt sign, ten-

derness of pelvis, ecchymosis to perineum,
A. Pelvic ring injuries in the setting of high- and evidence of other pelvic injuries. Also
energy trauma can be life threatening and are consider the mechanism of injury when eval-
often associated with a significant risk of uating for possible pelvic fractures. A fall
morbidity and mortality [1]. Evaluation from standing may be sufficient to generate a
begins with adherence to advanced trauma clinically severe fracture in an osteoporotic
life support (ATLS) protocols for primary and patient. A younger patient would likely need
secondary survey. Pelvic instability with a larger transfer of energy to sustain severe
compression on physical exam is highly spe- pelvic fractures, such as a motor vehicle col-
cific for unstable pelvic ring fractures (99%). lision at highway speeds.
However, according to retrospective reviews, B. As part of the primary survey, it is imperative
physical examination has 26% sensitivity for to determine if the patient is hemodynamically
detecting unstable fractures and 8% for stable stable. If the patient is unstable and pelvic frac-
fractures [1]. Repeated compression should ture is suspected, immediate interventions are
be avoided due to the risk of iatrogenic dis- indicated. If the patient is stable, then he or she
placement of fractures. Associated injuries can undergo additional imaging with CT scan
may include but are not limited to blunt chest prior to any interventions.
trauma; hollow viscus or other abdominal, C. An unstable patient with suspected pelvic frac-
neurologic, long bone fractures; hemorrhagic tures should immediately undergo resuscita-
shock; and urologic, gynecologic, or rectal tion with balanced blood products, utilizing a
injury [2]. Other physical exam findings that massive transfusion protocol if one is avail-
should raise the suspicion for a pelvic frac- able. Chest X-ray and pelvic X-rays should be
obtained. If an anterior posterior compression
fracture is present, a pelvic binder should be
placed to minimize pelvic expansion and hem-
orrhage. A binder should not be left in place
for >48 h [3]. The patient should also be
R. M. Staszak · L. J. Laufenberg (*) prepped for resuscitative endovascular balloon
Department of Surgery, Division of Trauma, Acute occlusion of the aorta (REBOA), if this is
Care, and Critical Care Surgery, Penn State Milton available at your institution.

680 R. M. Staszak and L. J. Laufenberg
D. Focused assessment with sonography for

tiary survey after initial stabilization, looking
trauma (FAST) should be performed in the for evidence of gynecologic, urologic, or rec-
workup of the unstable patient as well. This tal trauma not previously identified [4].
can help identify any intra-abdominal hemor- H. If there is evidence of pelvic fracture with
rhage contributing to the instability [4]. active extravasation and arterial injury noted
E. If the FAST is positive for intra-abdominal on CT scan, the patient requires intensive
bleeding, one should proceed to the operating care unit (ICU) admission or serial exams.
room for exploratory laparotomy. If pelvic Additionally, the patient requires immediate
fracture is present, consider hemorrhage con- consultation with orthopedic surgery and
trol with preperitoneal packing, external fixa- consideration for pelvic fixation, arteriogra-
tion, REBOA, pelvic arteriography and phy, or a combination of approaches. If there
embolization, or a combination of approaches. is no evidence of active hemorrhage, the
F. If the FAST is negative, for intra-abdominal patient may still need ICU admission and
hemorrhage, but the patient remains unstable serial exams with serial CBCs to follow for
and has evidence of pelvic trauma, procced to new hemorrhage. Typical management of
the operating room (OR) for preperitoneal pelvic fractures is a combination of opera-
packing, external fixation of fractures, pelvic tive and nonoperative management, with
arteriography and embolization, or a combi- weight bearing as tolerated. Orthopedic sur-
nation of these approaches. gery should be involved early in the stabili-
G. If the patient is hemodynamically stable,
zation of pelvic fractures and for
obtain further imaging with pelvic X-rays recommendations for or against operative
(anterior-posterior and inlet-outlet) and with fixation and weight- bearing status of the
CT scan [2]. Remember the associated risk of affected limbs [3].
other injuries, and perform a thorough ter-
166 Pelvic Fractures 681
Patient involved in an accident/traumatic injury suspected
A
Trauma evaluation ->ATLS guidelines & ABCDE
Obtain vital signs, lab work, establish 2 large bore IV’s
B Is the patient
hemodynamically stable?
No Yes
Immediate interventions:
C CT scan of abd/pelvis with
-Activate MTP
signs of bleeding from the F
-CXR & Pelvis, Pelvic binder (if
pelvis or major pelvic
amendable) -Femoral Arterial
injury?
catheter for possible REBOA
Yes No
D FAST
G -Obtain immediate
Admission with serial
surgical/ortho
re-examinations
consultation
orthopedics
- Pelvic arteriography
Positive Negative consultation as
or other intervention
indicated
-Control extra-abdominal
hemorrhage
-To operating room:
-Control pelvic hemorrhage
Control intra-abdominal
E hemorrhage
- Pelvic
stabilization/fixation ICU admission
-Control any pelvic follow
-Preperitoneal packing if
hemorrhage (pelvic CBC & exam
uncontrolled bleeding
fixation, preperitoneal
Consider Pelvic
packing, arteriography,
arteriography, REBOA
REBOA)
* REBOA –Resuscitative
endovascular balloon occlusion
ICU admission of the aorta
follow
CBC & exam
Algorithm 166.1
References [Internet]. Waltham, MA: UpToDate; 2017.

Available from: https://www.uptodate.com/contents/
pelvic-trauma-initial-evaluation-and-management.
1. Coccolini F, et al. Pelvic trauma: WSES classification
4. Cullinane DC, et al. Eastern Association for the sur-
and guidelines. World J Emerg Surg. 2017;12(5):1–18.
gery of trauma practice management guidelines for
2. Mattox KL, Moore EE, Feliciano DV. Trauma: pelvis.
hemorrhage in pelvic fractures – update and systemic
7th ed. New York: McGraw-Hill; 2013. p. 655–68.
review. J Trauma. 2011;71(6):1850–68.
3. Fiechtl J, et al. Pelvic trauma: initial evaluation
and management. In: Post T, editor. UpToDate.
Bladder Injuries
167
Algorithmic Approach workup should proceed without delay. An

unstable patient should first be stabilized
A. The bladder is a hollow organ that is normally before proceeding with a bladder injury
protected by pelvic rami. As the bladder is workup.
distended with urine it rises above the pelvic The cardinal sign for traumatic injury to
rim, exposing itself to injury during compres- the bladder is gross hematuria, found in about
sive or shear forces. An appropriate mecha- 95% of the cases [3]. All pelvic fractures or
nism and sufficient force can still result in patients with concern for bladder injury need
pelvic fracture and bladder injury. Bladder a genitourinary exam and should have urine
injuries occur in 1.6% of blunt abdominal evaluated for gross hematuria. Rule out ure-
trauma cases [1]. Of all bladder injuries, thra injury by exam or retrograde urethro-
60–80% are from blunt trauma and 15–40% gram prior to insertion of a Foley.
are from penetrating [2]. Injuries associated C. Stress cystography should be performed; one
with high risk of bladder injuries include pel- of the most sensitive is CT cystogram. A
vic fractures, blunt trauma to the lower abdo- proper study requires an initial image, fol-
men, abdominal seatbelt sign, or penetrating lowed by filling the bladder with 300–400 ml
injuries to the lower abdomen or pelvis. of contrast. The pelvic CT is then repeated
Bladder injuries can also occur iatrogenically when the bladder is distended with contrast.
during pelvic surgery. After emptying the bladder, delayed images
B. Evaluation for a suspected bladder injury fol- are also obtained. Clamping the Foley just
lowing a traumatic mechanism should begin prior to a CT with IV contrast will not pro-
with an advanced trauma life support (ATLS) vide sufficient distention of the bladder and
workup. If the patient is hemodynamically will result in a high rate of false negatives [4].
stable and concern for a bladder injury exists, Cystoscopy can also be performed to evaluate
for bladder injury.
D. Extraperitoneal injuries with urine leak lim-
ited to the perivesical space are often associ-
ated with pelvic fracture. Eighty-three percent
R. M. Staszak · L. J. Laufenberg (*) of patients with bladder ruptures have pelvic
Department of Surgery, Division of Trauma, Acute fractures, but less than 10% of patients with
Care, and Critical Care Surgery, Penn State Milton pelvic fracture have bladder ruptures [5].

E. For uncomplicated extraperitoneal injuries,

and that there is clear efflux from both ure-
the bladder catheter is left in place for teral orifices. The bladder should always be
10–14 days. Usually >85% of injuries will be repaired with heavy absorbable suture in two
healed by this point. layers. The bladder is then drained for
At times an extraperitoneal injury will need 5–10 days with a Foley catheter.
to be repaired due to concurrent injury. These H. After the designated drainage period, a repeat
are often repaired by making an anterior cys- cystogram is performed, whether the injury
totomy cephalad to the pelvic hematoma. The was repaired operatively or non-operatively.
laceration is then repaired from the inside in a The Foley is removed if there is no evidence
single-layer full-thickness closure followed of leak. If a leak persists, the Foley is left in
by drainage with a catheter for 5–10 days. place for an additional 7–10 days.
F. Intraperitoneal injuries occur when the peri- I. Combined injuries are often associated with
toneal surface has been disrupted with con- major pelvic trauma or penetrating injuries
comitant urinary extravasation. These and could have urethral, bladder, rectal, and
bladder injuries often occur when blunt trau- vaginal involvement. Injuries may also be
matic forces are applied to a distended associated with cystoscopic procedures, usu-
bladder. ally during resection of a bladder tumor or
G. For intraperitoneal injuries open repair of the during biopsies. Injury can also occur during
bladder is undertaken after debridement and other surgical procedures in the pelvis.
evaluation of the interior of the bladder. This Complex injuries, i.e., bladder neck or trigone,
helps to verify that there are no other injuries require expert consultation.
167 Bladder Injuries 685

(Lower abdominal trauma or pelvic fractures)
A
Obtain vital signs, blood work, physical examination, and

B Urine for gross blood (r/o urethral trauma before placing a
Foley)
Consult urology
Complex
No injury I
Gross blood or
Treat other injuries significant
concern for
Bladder injury? Yes
CT
cystogram C
or
cystoscopy
No injury
F Intraperitoneal
bladder injury Extraperitoneal
bladder injury D
OR for repair
G –2 layered closure
–Foley 5–10 days
Foley 10–14 days E
Repeat cystogram prior to

H removing foley
Algorithm 167.1
3. Carroll PR, McAninch JW. Major bladder trauma:

References mechanisms of injury and a unified method of diagno-
sis and repair. J Urol. 1983;132:254.
4. Peng MY, Parisky YR, Cornwell EE, et al. CT cystog-
1. Iverson AJ, Morey AF. Radiographic evaluation of raphy versus conventional cystography in evaluation
suspected bladder rupture following blunt trauma: of bladder injury. Am J Roentgenol. 1999;173:1269.
critical review. World J Surg. 2001;25(12):1588–91. 5. Zacharias C, Robinson JD, Linnau KF, et al. Blunt
2. Cass AS, Luxenberg M. Features of 164 bladder rup- urinary bladder trauma. Curr Probl Diagn Radiol.
tures. J Urol. 1987;138(4:743–5. 2012;41(4):140–1.
Rectal Injuries
168
Amanda E. Lee, Karima Fitzgerald,
and Lacee Jay Laufenberg
Algorithmic Approach tinal bleeding, potential pelvic fractures, and

presence of foreign bodies. Although DRE
A. The first step in the evaluation of any trauma has traditionally been part of the secondary
patient is a systematic, thorough evaluation survey in ATLS protocol, it has been found to
utilizing the advanced trauma life support have poor sensitivity for diagnosis of spinal
(ATLS) protocol, with a primary and second- cord, urethral, small bowel, colon, and rectal
ary survey to exclude life-threatening injuries injuries [5]. It does not cause physical harm to
[1]. There are both blunt and penetrating patient, but is also unlikely to affect immedi-
mechanisms by which rectal injuries can ate management of the patient, so perfor-
occur. Blunt injuries to the rectum are uncom- mance of a DRE should be based on clinical
mon due to protection from bony pelvis but judgment and presentation of the patient [6].
can be seen with severe pelvic fractures [2]. C. If concern for a rectal injury exists, the next
Penetrating injuries can result from transpel- step is to confirm the presence of the injury
vic gunshot wounds, perineal impalement, and then determine if it is intraperitoneal ver-
iatrogenic processes, foreign body insertion, sus extraperitoneal. If there are any concern-
or sex-related injuries [3]. ing findings on the initial evaluation that
B. The patient’s physical exam findings (abdom- require immediate operative intervention, the
inal tenderness, seat belt sign, ecchymosis, or patient should proceed to the operating room
blood at the perineum) can be suggestive of (OR). However, if the patient is stable and
injury to the rectum [4]. If any concern for a appropriate to undergo further workup, a
rectal injury is present, a digital rectal exam computerized tomography (CT) scan should
(DRE) could be performed, which assesses be performed (with intravenous and water-
anal tone, position of the prostate, gastrointes- soluble rectal contrast, if appropriate). The
CT scan can show bowel injury, extraluminal
air, contrast extravasation, or intestinal dis-
continuity [7].
A. E. Lee
Department of Surgery, Penn State Milton S. Hershey D. Whether the patient went immediately to the
Medical Center, Hershey, PA, USA OR for another reason (but suspicion of rectal
K. Fitzgerald · L. J. Laufenberg (*) injury remains) or had a rectal injury found on
Department of Surgery, Division of Trauma, Acute imaging, the patient should then undergo rigid
Care, and Critical Care Surgery, Penn State Milton proctoscopy or sigmoidoscopy which is con-
S. Hershey Medical Center, Hershey, PA, USA sidered the gold standard for detection and

688 A. E. Lee et al.
localization of rectal injuries [8–10]. This will G. If there is significant fecal contamination, the
serve to determine if the injury is intraperito- patient should undergo pre-sacral drainage in
neal or extraperitoneal. If the injury is to the addition to diversion [4]. If the injury is
intraperitoneal portion of the rectum, then fur- destructive, one should consider distal rectal
ther management should be as a distal colonic washout in addition to diversion and pre-
injury with exploratory laparotomy [11]. sacral drainage [15]. Ultimately, management
E. If the rectal injury is extraperitoneal, the type of injury to the rectum should be handled on
of intervention will be determined by the an individualized patient basis.
extent of injury and presence of fecal con- H. Once the acute phase of the injury has passed,
tamination. Historically, the gold standard the patient can be evaluated for stoma rever-
treatment for extraperitoneal rectal injuries is sal. The area of injury needs to be assessed
proximal diversion [12, 13]. However, if the with a rectal contrast study (CT or X-ray) to
injury is easily accessible and only partial ensure that the injury has healed without
thickness, it can safely be managed with pri- stricture formation. Furthermore, sphincter
mary repair only [4, 14]. integrity must be assessed to ensure the
F. Any rectal injury that is either inaccessible or patient will not have fecal incontinence or
more severe should undergo proximal diver- significant pelvic floor dysfunction. This can
sion. If the injury is nondestructive (<25% be done with physical exam, anoscopy, endo-
loss of circumference), diversion alone is sat- anal ultrasound, anal manometry, or pudendal
isfactory [15]. nerve studies [4].
168 Rectal Injuries 689
A Evaluate patient using ATLS protocol. Identify mechanism (blunt vs

penetrating) and determine if the patient is hemodynamically stable.
Is the patient
hemodynamically stable?
Yes No
Treat life
Perform thorough physical exam, making note of findings indicative threatening
of possible rectal injury injuries
Physical exam findings: abdominal

Consider DRE (digital rectal
tenderness, pelvic tenderness/pain,
exam)
seat belt sign, ecchymosis or blood at
the perineum
CT scan findings:
Obtain CT scan with IV and rectal free intraperitoneal
C air, intraperitoneal
contrast or rigid proctoscopy
free fluid,
intraperitoneal
bowel injury
Rigid proctoscopy
findings:
Intraperitoneal
injury
Is there evidence of
either
D
extraperitoneal or
intraperitoneal
rectal injury?
Exploratory laparotomy;
see management of colon
injuries
Algorithm 168.1
690 A. E. Lee et al.
CT scan findings:
extra luminal
extraperitoneal air,
contrast
extravasation,
extraperitoneal bowel
wall discontinuity
Rigid proctoscopy
findings:
Extraperitoneal injury
G
Injury is full
thickness, not easily
accessible,
Rigid proctoscopy if not significant fecal
already done contamination,
>25% circumference
E
Injury is
F
partial Fecal diversion with colostomy,
Injury is full
thickness and pre-sacral drainage, distal rectal
thickness, not easily
easily washout
accessible, <25%
accessible
circumference
Fecal diversion with

Primary repair diverting colostomy
only
Evaluate for reversal with rectal

contrast study and functional H
sphincter/pelvic floor analysis
Is there evidence of
stricture, leak,
incontinence or pelvic
floor dysfunction?
No
Yes
Elective colostomy reversal

Colorectal surgery referral
168 Rectal Injuries 691
References 9. Shlamovitz G, Mower W, Bergman J, Crisp J, DeVore

H, Hardy D, et al. Poor test characteristics for the dig-
ital rectal examination in trauma patients. Ann Emerg
1. Committee on Trauma, American College of Surgeons.
Med. 2007;50:25–33.
ATLS: advanced trauma life support program for doc-
10. Ferraro F, Livingston D, Odom J, Swan K, McCormack
tors. 8th ed. Chicago: American College of Surgeons;
M, Rush B Jr. The role of sigmoidoscopy in the man-
2008.
agement of gunshot wounds to the buttocks. Am Surg.
2. Wei R, Cao X, Tu D. Clinical treatment of open pelvic
1993;59:350–2.
fractures associated with perineal injury. Zhongguo
11. Pasquale M, Fabian T. Practice management guide-
Xiu Fu Chong Jian Wai Ke Za Zhi. 2012;25:550–3.
lines for trauma from the Eastern Association for the
3. Duncan A, Phillips T, Scalea T, Maltz S, Atweh
Surgery of Trauma. J Trauma. 1998;44:941–56.
N, Sclafani S. Management of transpelvic gunshot
12. Grasberger R, Hirsch E. Rectal trauma: a retrospec-
wounds. J Trauma. 1989;29:1335–40.
tive analysis and guidelines for therapy. Am J Surg.
4. Cheong J, Keshava A. Management of colorectal
1983;145:795–9.
trauma: a review. ANZ J Surg. 2017;87:547–53.
13. Gonzalez R, Falimirski M, Holevar M. The role of
5. Docimo S Jr, Diggs L, Crankshaw L, Lee Y. No
pre-sacral drainage in the management of penetrating
evidence supporting the routine use of digital rec-
rectal injuries. J Trauma. 1998;45:656–61.
tal examinations in trauma patients. Indian J Surg.
14. Ahern D, Kelly M, Courtney D, Rausa E, Winter
2015;77:265–9.
D. The management of penetrating rectal and anal
6. Ahl R, Riddez L, Mohseni S. Digital rectal exami-
trauma: a systematic review. Injury. 2017;48:1133–8.
nation for initial assessment of the multi-injuried
15. Bosarge P, Como J, Fox N, Falck-Ytter Y, Haut E,
patient: can we depend on it? Ann Med Surg (Lond).
Dorion H, et al. Management of penetrating extraperi-
2016;9:77–81.
toneal rectal injuries: an Eastern Association for the
7. Greer L, Gillern S, Vertrees A. Evolving colon injury
Surgery of Trauma practice management guideline. J
management: a review. Am Surg. 2013;79:119–27.
Trauma Acute Care Surg. 2016;80:546–51.
8. Mangiante E, Graham A, Fabian T. Rectal gunshot
wounds: management of civilian injuries. Am Surg.
1986;52:37–40.
Extremity Compartment
Syndrome 169
Karima Fitzgerald, Amanda E. Lee,
and Lacee Jay Laufenberg
Algorithmic Approach rial ischemia due to peripheral vascular

disease [2]. The diagnosis of extremity com-
A. In the setting of extremity trauma, acute
partment syndrome is nebulous and requires a
extremity compartment syndrome should be high index of suspicion; the consequences of
considered after the patient has been other- delay to diagnosis or delay to treatment are
wise evaluated and stabilized. Extremity severe and include loss of function, loss of
compartment syndrome can develop after limb, and renal failure if rhabdomyolysis
blunt or penetrating injury, with or without accompanies the compartment syndrome [4].
fractures [1]. Up to 75% of extremity com- It is suggested that functional impairment of
partment syndrome is due to tibia fractures, muscle becomes permanent after 4–12 h, and
with the incidence of extremity compartment nerve damage becomes irreversible after
syndrome in tibia fractures reported from 1% 12–24 h [5].
to 10% [2]. Vascular injuries should be identi- B. In addition to obtaining the details of the
fied promptly, as they can be associated with inciting event, key symptoms of extremity
compartment syndrome due to lack of arterial compartment syndrome to elicit are pain out
inflow, lack of venous outflow, or both [3]. of proportion to exam; a persistent, deep, ach-
Other patients at significant risk for extremity ing pain or burning pain; and paresthesias.
compartment syndrome are those with a sig- These symptoms may indicate ischemic
nificant crush injury or motor vehicle colli- changes of the muscles and nerves. The “6
sion with entrapment. Recreational use of Ps” of arterial ischemia (pain, pallor, pulsel-
injection drugs, infiltrated IV sites, burns, and essness, paresthesia, pressure, poikilother-
prolonged compression due to poor OR posi- mia) are associated with, but not diagnostic
tioning are other risk factors, as is acute arte- of, extremity compartment syndrome. When
present, they have a high specificity, but the
sensitivity is lacking [5]. Pain, particularly
pain out of proportion to exam or to apparent
K. Fitzgerald · L. J. Laufenberg (*)
Department of Surgery, Division of Trauma, Acute injury, is the most common and consistent
Care, and Critical Care Surgery, Penn State Milton finding [2].
S. Hershey Medical Center, Hershey, PA, USA C. The physical exam should consist of gross
e-mail: [email protected] examination of the extremity observing for
A. E. Lee pallor or mottling. Pain with palpation and
Department of Surgery, Penn State Milton S. Hershey with passive stretch should be evaluated. A

694 K. Fitzgerald et al.
full neuromuscular exam should be pressures. A patient with risk factors and pro-
performed, noting strength and sensation in gressive signs and symptoms of pain, pares-
all the affected muscle groups and derma- thesias, and decreased sensation or motor
tomes. Make note of the size of the extremity. function has sufficient indications for fasci-
Circumferential measurements of the affected otomy. Fasciotomy should not be delayed
and contralateral extremity should be while awaiting the Stryker needle to reach the
obtained when able. Tense, tight extremities, designated reading [6]. There is no consensus
with shiny-appearing skin and a woody feel- as to the specific indications for fasciotomy,
ing on palpation, have a high likelihood of and the diagnosis of extremity compartment
extremity compartment syndrome. Distal syndrome is “capricious and elusive” [6].
pulses should be palpated or Doppler signals G. Emergent fasciotomy as soon as the diagnosis
obtained. Pulselessness is uncommon in has been made is the mainstay of treatment
extremity compartment syndrome as it for extremity compartment syndrome.
requires the extremity pressure to exceed the Adjunct maneuvers to improve flow include
arterial blood pressure but can occur given relieving any external forces causing
the right combination of a hypotensive increased compartment pressure such as
patient, severe compartment syndrome, and/ dressings, splints, or casts. Reduction of frac-
or a vascular arterial injury [4]. If the patient tures or dislocations can often restore blood
is awake, alert, and able to be examined, flow to the affected extremity. The limb
serial examinations should be performed should remain in neutral position so as to
every hour as compartment syndrome pro- avoid changes in blood flow that can either
gresses rapidly. Sedated or obtunded patients increase vascular congestion or cause further
who cannot undergo serial exams should reduction in arterial blood flow. If the patient
undergo evaluation of compartment pressures is noted to be hypotensive, resuscitation with
[4] with consideration given for prophylactic either crystalloid or colloid should be imple-
or empiric fasciotomy if suspicion is high for mented to avoid hypoperfusion and further
extremity compartment syndrome. tissue injury [4]. Fasciotomy should be per-
D. There are a few accepted methods of measur- formed promptly to prevent permanent dam-
ing compartment pressures. The simplest and age. Although there is evidence to support
most common is a pressure monitoring nee- that irreversible muscle damage begins as
dle, such as the one made by Stryker. Other early as 4 h after onset of ischemia, the gener-
methods include transducing the pressure ally accepted timeframe is 6 h to fasciotomy
using a needle and arterial line setup and the to preserve muscle [4–6].
slit catheter technique [4]. Compartment The key to fasciotomy is complete release
pressures vary depending on the compart- of all involved compartments. There are two
ment, and even vary within a single compart- procedures that will allow for release of com-
ment, necessitating multiple measurements in partments. There are four compartments in
multiple areas [5]. the leg: anterior, lateral, superficial posterior,
E. There is no agreed-upon value for diagnosis and deep posterior. These are most commonly
of extremity compartment syndrome. released via two incisions (medial and lat-
Commonly used values are compartment eral). The anterior and lateral compartments
pressure of >/=30 mmHg or delta pressure of are released via the lateral incision, and the
</=30 mmHg [6]. (Delta pressure is the dif- superficial and deep posterior compartments
ference between the diastolic pressure and the are released through the medial incision. The
compartment pressure.) anterior compartment of the leg is the most
F. Clinical suspicion must be high, and the diag- common site of extremity compartment syn-
nosis must be made with a combination of drome [2].
physical exam findings and compartment
169 Extremity Compartment Syndrome 695
In the thigh there are three compartments: incision and release of the fascial planes of
anterior, posterior, and medial. Typically the biceps brachii and triceps brachii.
extremity compartment syndrome in the thigh H. Wound closure should not be attempted prior
can be managed with a long lateral incision to to resolution of tissue edema, due to the
release the anterior and posterior compart- inherent risk of repeat tissue ischemia [4].
ments, as compartment syndrome in the Patients should return to the operating room
medial adductors is rare [4]. The forearm has (OR) for attempted closure on postoperative
four compartments: deep volar, superficial days 3–5. Delayed primary closure is pre-
volar, dorsal compartment, and lateral com- ferred, but if necrosis persists, further
partment [2]. Fasciotomy is performed debridement should be undertaken prior to
through volar and dorsal incisions, with closure. Similarly, if tissue edema remains
access to the lateral compartment (aka the too high to allow skin coverage, the wounds
“mobile wad”) via the dorsal incision [1]. should be allowed to heal via secondary
There are only the anterior and posterior intention. Once an adequate bed of granula-
components which must be released in the tion tissue has formed, a split-thickness skin
arm [2]. This is accomplished with a lateral graft should be used for coverage [4].
696 K. Fitzgerald et al.
Patient with risk factors for extremity compartment syndrome:

A extremity trauma, injection drug abuse, reperfusion of ischemic
limb
History: Pain out of proportion to exam/injury, persistent deep

B ache or burning pain, paresthesias
Physical exam: pain with passive stretch, tense compartments,

C pallor, decreased sensation, weakness, paralysis, pulselessness
Findings positive for

compartment syndrome?
Yes No
Evaluation of compartment pressure: Continued observation

Stryker Quick Pressure Monitor with hourly exams.
D
Instrument, manometric IV pump
method, slit catheter technique
No
F
Compartment pressure No
>/=30mmHg or delta High clinical
E P</=30mmHg? suspicion?
Yes Yes
G Immediate fasciotomy
H Delayed primary closure or healing by secondary

intention and skin graft
Algorithm 169.1
169 Extremity Compartment Syndrome 697
References hand surgery. Philadelphia: WB Saunders; 1986.

p. 1–18.
4. Olson SA, Glasgow RR. Acute compartment syn-
1. Morin RJ, Swan KG, Tan V. Acute forearm com-
drome in lower extremity musculosketal trauma. J Am
partment syndrome secondary to local arterial injury
Acad Orthop Surg. 2005;13(7):436–44.
after penetrating trauma. J Trauma. 2009;66(4):
5. Ivatury RR. Pressure, perfusion and compartments:
989–93.
challenges for the acute care surgeon. J Trauma Acute
2. Stracciolini A, Hammerberg EM. Acute compartment
Care Surg. 2014;76(6):1341–8.
syndrome of the extremities. In: Moreira ME, Bachur RG,
6. Kosir R, Moore FA, Selby JH, Cocanour CS, Kozar
editors. UpToDate. [Internet]. 2018 Feb 12. Retrieved
RA, Gonzalez EA, Todd SR. Acute lower extrem-
from UpToDate: https://www.uptodate.com/contents/
ity compartment syndrome (ALECS) screening
acute-compartment-syndrome-of-the-extremities.
protocol in critically ill trauma patients. J Trauma.
3. Urbaniak JR, Roth JH. Arterial injuries of the upper
2007;63(2):268–75.
extremity. In: Boswisk J, editor. Complications in
Part XX
Critical Care
Management of Intracranial
Hemorrhage 170
Ariel P. Santos
Algorithmic Approach D. According to the American Heart Association/

American Stroke Association (AHA/ASA)
Intracranial hemorrhage (ICH) is a devastating Guidelines, rapid neurologic imaging with CT
condition that carries a significant morbidity and scan or magnetic resonance imaging (MRI) is
mortality but can be mitigated by proper medical recommended to distinguish ischemic stroke
care. Early assessment and diagnosis using CT from ICH [1]. CT scan is considered the gold
scan is imperative. standard considering performance of a MRI
can be precluded by time, cost, patient’s clini-
A. A cardinal sign of increased intracranial pres- cal status, and its availability. CT angiography
sure (ICP) is decreased level of consciousness (CTA) and contrast-enhanced imaging may be
and may be associated with headache, nausea, considered to help identify patients at risk for
vomiting, hypertension, focal neurologic defi- hematoma expansion. CTA, CT venography,
cit, and sudden progression of symptoms. magnetic resonance angiogram (MRA), and
B. Whether it is in the pre-hospital or hospital catheter angiography can also be useful to
situation, the primary objective is to secure evaluate for underlying lesions including vas-
airway management if needed, provide ade- cular malformations and tumors when there is
quate cardiovascular support, and transfer to clinical or radiological suspicion [1].
the closest facility where patient can defini- E. Baseline severity score should be performed
tively be managed with the full complement as part of the initial evaluation of patients
of neuro-radiologist, neurologist, neurosur- with ICH [1]. ICH score is the most widely
geon, and intensivist. Primary survey will used and externally validated severity scoring
help identify life-threatening condition. [2] (table in Algorithm 170.1).
Detailed history will help identify risk factors F. The Neurocritical Care Society recommends
and possible etiology of the ICH. an efficient, standardized, and integrated
C. Early referral to neurologist, neurosurgeon, management of patients with acute intracra-
and intensivist facilitates early treatment of nial hemorrhage, and this should be initiated
the patient avoiding risk of early neurological as soon as possible.
deterioration.
(a) Admission to neurocritical care unit:
Intracranial hemorrhage may be
A. P. Santos ()
associated with depressed sensorium;

Department of Surgery, Texas Tech University Health for this reason, oxygen supplementation
Sciences Center, Lubbock, TX, USA and securing the airway is of utmost

702 A. P. Santos
importance. It is highly recommended to probably indicated in ICH patients with

admit a patient with intracranial hemor- symptomatic DVT or PE but should take
rhage to the stroke or neuroscience into account time from hemorrhage onset,
intensive care unit managed by intensiv- hematoma stability, cause of hemorrhage,
ist and nurse experts. Prolonged stay in and overall patient condition [1, 9].
the emergency department may lead to (e) EEG monitoring if needed and seizure
worse outcome [3], but early neurocriti- treatment. Cortical involvement of the
cal care management in the ED may ICH is the most important risk factor for
ameliorate its effect [4]. early seizure which can occur as high as
(b) Blood pressure monitoring and control: 16% [10]. As per AHA/ASA recommen-
Uncontrolled high systolic blood pres- dations, clinical seizure should be treated
sure is associated with intracranial hema- with antiseizure drugs; continuous EEG
toma expansion, neurological monitoring should be considered in ICH
deterioration, higher mortality, and poor with depressed mental status that is dis-
clinical outcome [5]. Intensive Blood proportionate to the degree of brain
Pressure Reduction in Acute Cerebral injury; and there is no data to suggest that
Hemorrhage 2 (INTERACT-2) trial, the prophylactic antiseizure medication is
largest randomized clinical trial evaluat- effective [1].
ing the efficacy of intensive blood pres- (f) Fever management after ICH is reason-
sure lowering treatment to a SBP level able as per AHA/ASA guidelines.
<140 mm Hg, found this management to Therapeutic cooling may reduce perihe-
be safe and effective in improving func- matomal edema but is considered investi-
tional outcome [6]. gational in ICH at this time [11].

(c) Correction of coagulopathy: (g) Glycemic monitoring and control to

Anticoagulation taken by the patient avoid hypoglycemia and hyperglycemia.
must be stopped. Measurement of coagu- (h) Head of bed elevation. Keep the head of
lation profile and elastography can help bed elevated to help reduce ICP as well
guide the correction of the coagulopathy. as preventing aspiration.
Accurate account of the medication taken (i) ICP monitor and control if indicated.
by the patient will guide the appropriate Increasing intracranial pressure and
antidote or reversal agent. decreasing cerebral perfusion pressure

(d) Deep vein thrombosis (DVT) prophy- (CPP) are associated with increased mor-
laxis: Patients with ICH have an increased tality. AHA/ASA recommends that
risk of thromboembolic disease. The patients with Glasgow (GCS) score of <8,
CLOTS 3 trial (Clots in Legs Or sTock- clinical evidence of transtentorial hernia-
ings after Stroke) showed that intermittent tion, or significant IVH or hydrocephalus
pneumatic compression started as early as be considered for ICP monitoring and
the day of hospital admission reduced the treatment; ventricular drainage as treat-
occurrence of proximal DVT [7]. Once ment for hydrocephalus is reasonable
cessation of intracranial bleed is docu- especially in patient with decreased level
mented, early use of low molecular of consciousness; and corticosteroids
weight heparin or unfractionated heparin should not be administered for treatment
(day 1 to 6 after admission) showed of elevated ICP in ICH [1]. Medical treat-
reduction of pulmonary embolism and ment includes head e levation, ventilation,
non-significant reduction in mortality but sedation, analgesia, use of paralytics,
no difference in DVT or intracranial mannitol, loop diuretics, hypertonic
hematoma enlargement [8]. Systemic saline, hypothermia, and barbiturates.
anticoagulation or IVC filter placement is Randomised Evaluation of Surgery with
170 Management of Intracranial Hemorrhage 703
Craniectomy for Uncontrollable Elevation (j) Joint physical, occupational, and speech
of Intracranial Pressure (RESCUEicp) therapy evaluation and treatment as early
trial showed that decompressive craniec- as possible.
tomy in patients with severe and refrac- G. Multidisciplinary rehabilitation is recom-

tory intracranial hypertension after mended including education of the patient,
traumatic brain injury resulted in lower family members, and caretaker regarding sec-
mortality and higher rate of vegetative ondary prevention of stroke and prevention of
state than medical management [12]. sequelae and complications of ICH.
704 A. P. Santos
A patient presents with severe headache, nausea, vomiting, focal neurologic

A
deficit, decreased level of consciousness and elevated blood pressure.
Causes of intracranial bleed

Hypertension
Primary survey: Coagulopathy, use of anticoagulant
A- Airway protection with C-spine immobilization in trauma Trauma
B- Breathing
B C- Circulation, avoid hypotension, control bleeding
Arteriovenous malformation
Intracranial aneurysm
D- Assess GCS and any neurologic deficit Cavernous angioma
E- Exposure Cerebral amyloid angiopathy
Secondary survey: History, PE, PMH, medications, family and Vasculitis
social history; Check for history of dementia, trauma, and illicit drug. Dural venous sinus thrombosis
Ancillary tests: CBC, CMP, UA, Coagulation profile, Troponin, Intracranial neoplasm
Glucose level and toxicology screen Dural arteriovenous fistula
Hemorrhagic conversion of infarct
Alcoholism
Cocaine abuse
C Consult: Neurosurgery, Neurology, Neuroradiology and Recreational drugs
NO
CT Scan findings of ICH? Re-evaluate

D
YES
Determination of the ICH score
Component ICH score points

GCS Score
3–4 2
5 – 12 1
13 – 15 0
ICH volume, cm3
E >30 1
< 30 0
Intraventricular hemorrhage
Yes 1
No 0
Infratentorial origin of ICH
Yes 1
No 0
Age in Years
80 1
< 80 0
Total ICH score 0–6
GCS score indicates GCS score on initial presentation (or after resuscitation); ICH volume, volume on initial
CT calculated using ABCI method; and IVH, presence of any IVH on initial CT.
Hemphill JC III, Bonovich DC, Besmertis L, et al. The ICH score: a simple, reliable grading scale for intracerebral hemorrhage. Stroke
2001; 32:891-897.
Algorithm 170.1
170 Management of Intracranial Hemorrhage 705
A – Admit to Stroke, Neurocritical Care or Specialized Intensive Care Unit

F
B – Blood pressure control
C – Correct coagulopathy if present.

Vitamin K antagonist (VKA) – Withhold VKA, Vitamin K IV, FFP, PCC
Direct Oral Anticoagulants (DOACs) – PCC, FEIBA, FFP, activated charcoal if taken < 2 hours
Dabigatran – Idarucizumab (Praxbind)
Heparin – Protamine 1 mg per 100 unit of heparin
Factor deficiency – Factor transfusion
Thrombocytopenia – platelet transfusion
D – DVT control using intermittent pneumatic compression device and early DVT prophylaxis
E – EEG monitoring if depressed mental status and seizure treatment
F – Fever control
G – Glycemic monitoring and control
H – Head of bed elevation and hematoma evacuation if needed
I – ICP monitoring and control if needed

Ventilation control
Hypertonic saline solution
Mannitol
Diuretics
Sedation
Paralytics
Barbiturates
Decompressive Craniectomy
J – Joint physical, occupational and speech therapy evaluation and treatment and family support.
Constant re-evaluation
G Secondary prevention
Rehabilitation
706 A. P. Santos
References 7. Dennis M, Sandercock P, Reid J, et al. CLOTS Trial

Collaboration. Effectiveness of intermittent pneu-
matic compression in reduction of risk of deep vein
1. Hemphill JC III, Greenberg SM, Anderson CS, et al.
thrombosis in patients who have had a stroke (CLOTS
Guidelines for the management of spontaneous intra-
3): a multicenter randomized controlled trial. Lancet.
cerebral hemorrhage: a guideline for healthcare profes-
2013;382:516–24.
sionals from the American Heart Association/American
8. Paciaroni M, Agnelli G, Venti M, et al. Efficacy and
Stroke Association. Stroke. 2015;46:2032–60.
safety of anticoagulants in the prevention of venous
2. Hemphill JC III, Bonovich DC, Besmertis L, et al.
thromboembolism in patients with acute cerebral
The ICH score: a simple, reliable grading scale for
hemorrhage: a meta-analysis of controlled studies. J
intracerebral hemorrhage. Stroke. 2001;32:891–7.
Thromb Haemost. 2011;9:893–8.
3. Rincon F, Mayer SA, Rivolta J, et al. Impact of delayed
9. Kelly J, Hunt BJ, Lewis RR, Rudd A. Anticoagulation
transfer of critically ill stroke patients from the emer-
or inferior vena cava filter placement for patient with
gency department to the Neuro-ICU. Neurocrit Care.
primary intracerebral hemorrhage developing venous
2010;13:75–81.
thromboembolism? Stroke. 2003;34:2999–3005.
4. Elmer J, Pallin DJ, Liu S, et al. Prolonged emergency
10. Beghi E, D’Alessandro R, Beretta S, et al. Incidence
department length of stay is not associated with worse
and predictors of acute symptomatic seizure after
outcomes in patients with intracerebral hemorrhage.
stroke. Neurology. 2011;77:1785–93.
Neurocrit Care. 2012;17:334–42.
11. Kollmar R, Juettler E, Huttner HB, et al. Cooling in
5. Sakamoto Y, Koga M, Todo K, et al. Relative sys-
intracerebral hemorrhage (CINCH) trial: protocol of
tolic blood pressure reduction and clinical out-
a randomized German-Austrian clinical trial. Int J
comes in hyperacute intracerebral hemorrhage: the
Stroke. 2012;7:168–73.
SAMURAI-ICH observational study. J Hypertens.
12. Hutchinson PJ, Kolias AG, Timofeev EA, et al.

2015;33(5):1069–73.
Trial of decompressive craniectomy for trau-
6. Anderson CS, Heeley E, Huang Y, et al. Rapid blood-
matic intracranial hypertension. N Engl J Med.
pressure lowering in patients with acute intracerebral
2016;375(12):1119–30.
hemorrhage. N Engl J Med. 2013;368:2355–65.
Airway Management
171
Algorithmic Approach occipital extension) if possible; if patient

cannot tolerate the sniffing position or is

A. Prior to any airway manipulation, evaluate obese, elevate the head of bed to 20–30
the patient. This includes assessment of perti- degrees. Pre-oxygenate with 100% FiO2 for
nent medical history for findings which may 2 min if the patient is stable; if more expedi-
influence your plan or ability to perform dif- ent intervention is indicated, attempt four
ferent airway procedures (e.g., heart disease, inspiratory-expiratory cycles at near-vital
chronic kidney disease, head/neck radiation capacity. Administer an appropriate sedative
or surgery, hospital course), performance of a and adequate neuromuscular blockade.
focused physical exam (mouth opening, C. Attempt first laryngoscopy. Consider the use
Mallampati classification, cervical spine of video laryngoscopy or other adjuncts in
range of motion), review of nil per os (NPO) order to maximize the chance for success on
status and recent labs, and review of prior air- first attempt, as the chance of successful intu-
way intervention records if available. During bation declines with each subsequent attempt
the initial evaluation, continually monitor for [1]. If successful, confirm placement by direct
hemodynamic instability and acute worsen- visualization, observation of symmetric chest
ing of hypoxemia. Do not hesitate to call for rise, bilateral auscultation, and capnography.
assistance if any difficulty is anticipated. Continuous quantitative capnography
B. Ensure all appropriate equipment is available remains the gold standard for confirmation of
and functioning; this includes, but is not lim- ETT placement [1]. If unsuccessful, attempt
ited to, a bag-valve-mask (BVM) apparatus, to mask ventilate.
suction with large-bore rigid catheter (e.g., D. If oxygenation is adequate with mask ventila-
Yankauer catheter), appropriately sized endo- tion, oxygenate and attempt up to two addi-
tracheal tubes (ETT) and laryngoscopes, ETT tional laryngoscopies. Consider the addition
introducer (e.g., gum elastic bougie), and of video laryngoscopy or other adjuncts in
supraglottic airway devices. Assume the order to improve likelihood of success with
sniffing position (neck flexion, atlanto- subsequent attempts. If unable to achieve
adequate oxygenation with mask ventilation,
R. S. Schoaps · S. W. Hazard III (*) attempt insertion of a supraglottic airway
Department of Anesthesiology and Perioperative (SGA) device.
Medicine, Penn State Hershey Medical Center, E. If SGA insertion is successful, continue oxy-
Hershey, PA, USA genation and consider options for the next step.

708 R. S. Schoaps and S. W. Hazard III
This may include intubating through the SGA at mask ventilation, and failed SGA inser-
with a fiberoptic bronchoscope, utilization of tion. CICO situations necessitate emergent
video laryngoscopy if not already attempted, surgical airway intervention in order to
use of additional adjunctive devices if avail- achieve adequate oxygenation. In such an
able, or procession to surgical airway interven- emergency setting, cricothyroidotomy is the
tion (e.g., tracheotomy, cricothyroidotomy). procedure of choice as it allows the fastest
Appropriate consultant services should be noti- reliable tracheal access [1].
fied of the situation (e.g., anesthesiology, air- Cricothyroidotomy is not recommended in
way surgical team, etc.). If you are unable to children due to a high incidence of subglot-
oxygenate via the SGA, it is reasonable to tic stenosis. If critical hypoxemia is
make one final attempt at mask ventilation. observed, consider inserting a 14-gauge nee-

F.
“Cannot intubate, cannot oxygenate” dle through the cricothyroid membrane in
(CICO) indicates at least one failed attempt order to initiate passive oxygenation while
at laryngoscopy, at least one failed attempt preparations for the procedure are made.
171 Airway Management 709
Assess patient
A Pertinent medical history, recent labs, NPO status, prior airway interventions, etc.
Prepare equipment, position, and pre-oxygenate with 100% FiO2

B
Induce with appropriate sedation and adequate neuromuscular blockade
Attempt first direct laryngoscopy (DL)
Successful?
No Yes
C Mask ventilate Confirm ETT placement:

Chest rise, auscultation, ETCO2
Oxygenating?
Yes
No
Insert supraglottic Oxygenate and attempt additional DL

D airway device Consider video laryngoscopy or other adjunct
Successful?
Yes
No
Oxygenate and consider options:

E Final attempt to Intubate through SGA, other adjunct, or
mask ventilate proceed to surgical airway
CICO
F Emergent surgical airway

(cricothyroidotomy)
Algorithm 171.1
Reference
1. Frerk C, et al. Difficult Airway Society 2015 guide-
lines for management of unanticipated difficult intu-
bation in adults. Br J Anesth. 2015;115(6):827–48.
Intubation and Extubation
172
Ariel P. Santos
Algorithmic Approach should be placed in sniffing position which

can be achieved by placing the back of the
Intubation bed at 30 degrees angle and placing a shoul-
der roll. However, in the presence of a possi-
A. The need for emergent intubation is one of ble or suspected cervical injury, cervical
the most stressful situations any clinician can stabilization will be required.
potentially encounter. But awareness of the
indications, readiness, preparation, and prac- The most common induction agents used for
tice can render this daunting task seamless. intubation are ketamine, propofol, and etomidate.
B. Early identification of patients at risk for dif- Propofol should be avoided in a hemodynami-
ficult intubation is imperative. Findings on cally unstable patient. In addition, paralytics are
external airway examination like decreased also administered during the rapid sequence intu-
cervical mobility and increased distance bation. The two most common paralytics used are
between the thyroid cartilage and tip of chin succinylcholine and rocuronium. Succinylcholine
or thyromental distance of less than 7 cm are should be avoided in patients with renal failure,
predictive of potentially difficult intubation. hyperkalemia, crush injuries, neuromuscular
De Jong et al. devised the MACOCHA scor- pathologies, and myopathies.
ing system which correlates with intubation It is imperative to have a working laryngo-
difficulty [1] (see table in Algorithm 172.1). scope (either a Miller or a Macintosh blade)
C. According to Jaber et al., use of the ten-step depending on the operator’s preference. Recently
bundle could lower life-threatening compli- direct laryngoscope was supplanted by video
cations, i.e., death, cardiac arrest, severe laryngoscope as the preferred method of intuba-
hypotension, and severe hypoxemia from tion. Griesdale et al. showed that the latter allows
34% to 21%. Other complications, such as for improved glottis visualization, faster intuba-
aspiration, dysrhythmia, and esophageal intu- tion, and higher success rate [3].
bation, could also be lowered from 21% to After intubation, placement should be verified by
9% [2]. Proper airway positioning is impor- physical examination, auscultation, and capnog-
tant to achieve adequate oxygenation. Patient raphy. The American Heart Association (AHA)
International Consensus on Cardiopulmonary
A. P. Santos (*) Resuscitation (CPR) and Emergency
Department of Surgery, Texas Tech University Health Cardiovascular Care (ECC) recommended rou-
Sciences Center, Lubbock, TX, USA tine use of waveform capnography for

712 A. P. Santos
e ndotracheal verification [4]. It is considered the placed to decompress the stomach. Chest X-ray
gold standard for verification of proper endotra- should be requested to evaluate placement of the
cheal tube placement and carries a 100% sensi- endotracheal tube. Complications of inappropri-
tivity and specificity even in low perfusion state ate placement of endotracheal tube are clinically
such as cardiac arrest [5]. The endotracheal tube significant that post-intubation chest X-rays are
should be properly secured and an orogastric tube deemed necessary [6].
172 Intubation and Extubation 713
A Indications of Intubation:
1. Respiratory Failure
2. Relief of airway obstruction
3. Airway protection
4. Shock
5. Low GCS < 8
6. Severe maxilla-facial injury
7. Reduction of work of breathing
8. Facilitation of pulmonary toilet
Any of the above No

indication exists? Continue to Monitor
Yes
Table 172.1 Examine the patient using the MACOCHA Scoring

B
POINTS
Factors related to patient
Mallampati Score III or IV 5
Obstructive sleep apnea Syndrome 2
Reduced mobility of cervical Spine 1
Limited mouth opening < 3 cm 1
Factors related to pathology
Coma 1
Severe Hypoxemia < 80% 1
Factor related to operator
Non-Anesthesiologist 1
Total 12
Definition of Abbreviation: MACOCHA= Mallampati score III or IV, Apnea syndrome
(obstructive), Cervical spine limitation, Opening mouth < 3 cm, Coma, Hypoxia,
Anesthesiologist non-trained. Coded 0 to 12: 0 = easy;12 = very difficult
Reprinted with permission of the American Thoracic Society. Copyright © 2017 American
Thoracic Society.
De Jong A, Molinari N, Terzi N et al. Early identification of patients at risk for difficult
intubation in the intensive care unit: development and validation of the MACOCHA
score in a multicenter cohort study. Am J Respir Crit Care Med. 2013 187(8): 837.
The American Journal of Respiratory and Critical Care Medicine is an official journal of the
American Thoracic Society
714 A. P. Santos
Difficult Intubation Cart (table 2)

Possible Yes
Difficult Call Anesthesiologist on call
Intubation?
Notify Surgery Team for possible
surgical airway
No
Intubation Protocol
Pre-intubation
C 1. Presence of intensivist, respiratory therapist, bedside RN, and intubation cart
2. Preparation for long term sedation
3. Procedure time-out
4. Positioning and Pre-oxygenation for at least 3 minutes
Intubation
5. Rapid Sequence intubation technique:

a. Induction agents: ketamine, propofol or etomidate
b. Paralytics:Succinylcholine 1.5-2.0 mg/kg or Rocuronium 1.0-1.2 mg/kg
6. Application of cricoid pressure
7. Visualization of glottic opening and intubation
Post-intubation
8. Immediate confirmation with capnography/capnometry

9. Confirmation with auscultation and chest x-ray
10. Secure the tube, place oro-gastric tube
Endotracheal intubation
Algorithm 172.1
Extubation minute, vital capacity of >10 ml/kg, negative

inspiratory force (NIF) >−20, tidal volume
A. Extubation or removal of the endotracheal (TV) >5 ml/kg, and rapid shallow breathing
tube is the final step of liberation from index (RSBI) less than 100.
mechanical ventilator. Prior to extubation, it C. If airway edema is suspected, cuff-leak test
is important to review the indication for intu- can be done by deflating cuff and occluding
bation and ensure that the reason for intuba- the endotracheal tube to ascertain whether
tion has been resolved. patient can breathe around the tube.
B. Intubated patients should be assessed daily Administration of steroids can help amelio-
for possible extubation. The patient must be rate the edema. A failed cuff-leak test does
hemodynamically stable and on minimal not always lead to failed extubation particu-
pressor support with appropriate and ade- larly when inappropriately sized tube was
quate gas exchange. Sedation should be dis- used.
continued and the patient must pass a D. Diligence must be observed prior to endo-
spontaneous breathing trial. The patient tracheal extubation especially if intubation
should be awake and be able to support their was noted to be difficult. Caution must be
airway with adequate respiratory drive and taken in extubating patient with obstructive
muscle and cough strength to clear one’s sleep apnea, maxillofacial trauma, general-
secretions. Patients with cough peak flow ized edema, postoperative procedure like
(CPF) of less than 60 ml/min and secretions thyroid and ENT surgeries, and cervical
of more than 2.5 ml/hour and those unable to spine procedures. Informing the anesthesia
complete four simple tasks (open one’s eyes, or surgical team prior to extubating a previ-
tracking with one’s eyes, hand grasping, and ously difficult intubation patient is not
stick out tongue) are at high risk for extuba- unreasonable. Reintubation has been asso-
tion failure [7]. Traditionally, we prefer to ciated with higher mortality (see table in
have a respiratory rate of less than 35 per Algorithm 172.2).
716 A. P. Santos
Criteria for Extubation:
1. Patient is awake and can support airway

A 2. Adequate respiratory drive and muscle strength
3. Adequate oxygenation and ventilation
4. Passed spontaneous breathing trial
5. (+) Cough reflex and able to clear secretions
Indication for the Do not Extubate

intubation
resolved? No Re-assess again
Yes
Spontaneous Breathing Trial Do not Extubate

B for 30 minutes or more
No Re-assess again
Yes
Yes
IV Steroids
C Cuff Leak?
No Re-assess again
Yes
Previous Difficult
Intubation? Prepare to extubate
No
D
Yes
Call anesthesiology
Difficult airway cart (Table 2)
Call Surgery: preparation for surgical airway
Table 172.2 Contents of Difficult Intubation Cart
Bag-and-mask Ventilation System

Nasopharyngeal airway in various sizes
Oral airway in various sizes
Yankauer suction, tubing and machine
Video laryngoscope
Laryngoscope: Macintosh and Miller in various blade sizes
Endotracheal tubes in various sizes
ET stylets
Eschmann stylet
Bougie
Magill forceps
Ventilating tube exchange catheters
Carbon dioxide detectors
Fiberoptic scope
Supraglottic airway devices like laryngeal mask airway
Cricothyroidotomy kit
Emergency Surgical Airway Kit
Algorithm 172.2
4. Neumar RW, Otto CW, Link M, et al. Part 8: adult

References advanced cardiovascular life support: 2010 American
Heart Association guidelines for cardiopulmonary
1. De Jong A, Molinari N, Terzi N, et al. Early iden- resuscitation and emergency cardiovascular care.
tification of patients at risk for difficult intuba- Circulation. 2010;122:S729–67.
tion in the intensive care unit: development and 5. Silvestri S, Laddle J, Brown J, et al. Endotracheal
validation of the MACOCHA score in a multi- tube placement confirmation: 100% sensitivity and
center cohort study. Am J Respir Crit Care Med. specificity with sustained four-phase capnographic
2013;187(8):832–9. waveforms in a cadaveric experimental mode.
2. Jaber S, Jung B, Corne P, et al. An intervention to Resuscitation. 2017;115:192–8.
decrease complications related to endotracheal intuba- 6. Hossein-Nejad H, Payandemehr P, Bashiri S, et al.
tion in the intensive care unit: a prospective, multiple- Chest radiography after endotracheal tube place-
center study. Intensive Care Med. 2010;36(2):248–55. ment: is it necessary or not? Am J Emerg Med.
3. Griesdale DE, Chau A, Isac G, Canadian Critical Care 2013;31(8):1181–2.
Trial Group, et al. Video-laryngoscopy versus direct 7. Salam A, Tilluckdharry L, Amoateng-Adjepong Y,
laryngoscopy in critically ill patients: a pilot random- et al. Neurologic status, cough, secretions and extuba-
ized trial. Can J Anaesth. 2012 Nov;59(11):1032–9. tion outcomes. Intensive Care Med. 2004;30:1334–9.
Acute Respiratory Distress
Syndrome (ARDS) 173
Dan A. Galvan
Algorithmic Approach D. Additional therapeutic measures may be

undertaken for patients with moderate-to-
A. If one should have a patient developing new- severe ARDS including the use of neuro-
onset or worsening respiratory compromise muscular blockade agents [7] and prone
within 1 week of exposure to relevant risk positioning [8].
factors, one should be aware that the patient E. Measurement of driving pressure [9] and
could be developing ARDS [1]. This syn- trans-pulmonary pressure [10] may impede
drome is underrecognized in 40% of all cases injury to the lung in the form of volutrauma,
[2] and has a mortality rate of greater than barotrauma, atelectrauma, and biotrauma.
40% in moderate-to-severe disease [3]. F. Recruitment maneuvers to open collapsed
B. Once you suspect ARDS, apply the criteria alveoli [11] and utilization of a directed fluid
cited in the Berlin definition of ARDS [1]. management strategy for specific subpheno-
Recognition of a patient with ARDS and stag- types of ARDS [12] may benefit certain
ing (mild, moderate, severe) of the syndrome ARDS patients.
clarifies the mortality risk and drives treat- G. To optimize care of the ARDS patient, the
ment efforts maximizing survival. (see table ABCDEF [13] bundle must be employed.
in Algorithm 173.1) H. Presently, there is no compelling evidence to
C. Patients meeting ARDS criteria must have support the routine use of extracorporeal
lung protective strategies initiated. These membrane oxygenation [14], high-frequency
strategies include low tidal volume ventila- oscillatory ventilation [15], inhaled nitric
tion, elevated positive end-expiratory pres- oxide [16], or glucocorticoids [17] in the
sure (PEEP), and diminished plateau treatment of ARDS patients.
pressures [4]. PEEP strategy should be deter-
mined by the ARDS stage [5, 6].
D. A. Galvan (*)
Geisinger Holy Spirit Hospital, Harrisburg, PA, USA

720 D. A. Galvan
A young blunt trauma patient on the ventilator develops increasing

hypoxemia 4 days following a damage control laparotomy. The abdomen
is still open. Chest film reveals bilateral opacities and an echocardiogram
does not demonstrate cardiac contusion or fluid overload.
Risk Factors for ARDS:

Pneumonia
Non-pulmonary sepsis
Aspiration of gastric contents
Major trauma
A Is this ARDS? Pulmonary contusions
Pancreatitis
Inhalational injury
Severe burns
Non-cardiogenic shock
Drug overdose
Multiple transfusions or transfusion-
associated acute lung injury
Pulmonary vasculitis
Drowning
The Berlin Definition of Acute Respiratory Distress Syndrome

Acute Respiratory Distress Syndrome
Timing Within 1 week of a known clinical insult or new or worsening respiratory
symptoms
Chest imaginga Bilateral opacities—not fully explained by effusions, lobar/lung collapse, or
nodules
Origin of edema Respiratory failure not fully explained by cardiac failure or fluid overload
Need objective assessment (eg, echocardiography) to exclude hydrostatic
edema if no risk factor present
Oxygenationb
B Mild 20 mmHg < PaO2/FIO2 ≤300 mmHg with PEEP or CPAP ≥ 5 cmH2OC
Moderate 100 mmHg < PaO2/FIO2 ≤ 200 mmHg with PEEP ≥ 5 cmH2O
Severe PaO2/FIO2 ≤ 100 mmHg with PEEP ≥ 5 cmH2O
Abbreviations: CPAP, continuous positive airway pressure; FIO2, fraction of inspired oxygen; PaO2, partial pressure of arterial oxygen;
PEEP, positive end-expiratory pressure.
aChest radiograph or computed tomography scan.
bIf altitude is higher than 1000 m, the correction factor should be calculated as follows: [PaO /FIO x (barometric pressure/760)].
2 2
cThis may be delivered noninvasively in the mild acute respiratory distress syndrome group.
From Ranieri VM, Rubenfeld GD, Thompson BT, et al. The ARDS Definition Task
Force. Acute respiratory distress syndrome: the Berlin Definition. JAMA
2012;307(23):2526–2533; with permission from the American Medical Association.
Timing: worsening respiratory symptoms within one week of

major trauma
Chest imaging: bilateral opacities not explained by effusions or
lung collapse
Origin of edema: not explained by cardiac failure or fluid
overload as proven on echocardiography
Oxygenation: ABGs - PaO2 of 60; on an FIO2 of 1.0 and

PEEP 10 PaO2 /FIO2 of 60 mm Hg consistent with severe ARDS
Algorithm 173.1
173 Acute Respiratory Distress Syndrome (ARDS) 721
Begin lung protective strategies including low tidal volume ventilation

(4-6 mL/kg predicted body weight), elevated PEEP while keeping plateau
C pressures ≤ 30 cm H2O. Utilize low PEEP for mild ARDS and high PEEP
for moderate-to-severe ARDS.
Utilizing appropriate sedation, begin a continuous infusion of Cisatracurium

D for 48 hours. Then initiate proning of the patient for at least 16 consecutive
hours per day until desired improvement in oxygenation is achieved.
Initiate measurement of driving pressure and transpulmonary pressure as

E described in the referenced articles.
Utilize recruitment maneuvers; obtain plasma levels of IL-8, bicarbonate and

F tumor necrosis factor receptor-1 levels to accurately classify the patient’s
subphenotype and appropriately direct fluid management.
Assess, prevent and manage pain (particularly in the presence of significant

pain) utilizing numerical rating scales.
Both spontaneous awakening trials and spontaneous breathing trials should be

employed daily to liberate the patient from the ventilator in a timely fashion.
Choice of analgesia and sedation strategy should utilize numerical rating

scales and be deliberate and judicious; avoid benzodiazepines.
Delirium should be assessed utilizing numerical rating scales, prevented and

managed. Delirium leads to prolonged mechanical ventilation, increased
G
length of ICU and hospital stay as well as long-term cognitive impairment and
elevated mortality.
Early mobility decreases delirium and improves functional outcomes.
Family engagement is a critical foundational support needed by every

intensive care unit patient.
From Marra A, Ely EW, Pandharipande PP, Patel MB. The ABCDEF bundle in critical care. Crit Care Clin
2017;33(2):225-43; with permission from Elsevier.
722 D. A. Galvan
References acute respiratory distress syndrome. N Engl J Med.

2013;368(23):2159–68.
9. Amato MB, Meade MO, Slutsky AS, et al. Driving
1. Ranieri VM, Rubenfeld GD, Thompson BT, et al.
pressure and survival in acute respiratory distress syn-
The ARDS definition task force. Acute respiratory
drome. N Engl J Med. 2015;372(8):747–55.
distress syndrome: the Berlin definition. JAMA.
10. Talmor D, Sarge T, Malhotra A, et al. Mechanical ven-
2012;307(23):2526–33.
tilation guided by esophageal pressure in acute lung
2. Bellani G, Laffey JG, Pham T, LUNG SAFE
injury. N Engl J Med. 2008;359(20):2095–104.
Investigators; ESICM Trials Group, et al.
11. Constantin JM, Godet T, Jabaudon M, Bazin JE, Futier
Epidemiology, patterns of care, and mortality for
E. Recruitment maneuvers in acute respiratory dis-
patients with acute respiratory distress syndrome
tress syndrome. Ann Transl Med. 2017;5(14):290–5.
in intensive care units in 50 countries. JAMA.
12. Famous KR, Delucchi K, Ware LB, et al. Acute respi-
2016;315(8):788–800.
ratory distress syndrome subphenotypes respond dif-
3. Villar J, Blanco J, Kacmarek RM. Current incidence
ferently to randomized fluid management strategy.
and outcome of the acute respiratory distress syn-
Am J Respir Crit Care Med. 2017;195:331–8.
drome. Curr Opin Crit Care. 2016;22(1):1–6.
13. Marra A, Ely EW, Pandharipande PP, Patel MB. The
4. The Acute Respiratory Distress Syndrome Network.
ABCDEF bundle in critical care. Crit Care Clin.
Ventilation with lower tidal volumes as compared
2017;33(2):225–43.
with traditional tidal volumes for acute lung injury
14. Abrams D, Brodie D. Extracorporeal membrane oxy-
and the acute respiratory distress syndrome. N Engl J
genation for adult respiratory failure 2017 update.
Med. 2000;342(18):1301–8.
Chest. 2017;152(3):639–49.
5. Briel M, Meade M, Mercat A, et al. Higher vs lower
15. Meade MO, Young D, Hanna S, et al. Severity of
positive end-expiratory pressure in patients with
hypoxemia and effect of high-frequency oscillatory
acute lung injury and acute respiratory distress syn-
ventilation in acute respiratory distress syndrome. Am
drome systematic review and meta-analysis. JAMA.
J Respir Crit Care Med. 2017;196(6):727–33.
2010;303(9):865–73.
16.
Gebistorf F, Karam O, Wetterslev J, Afshari
6. Chiumello D, Cressoni M, Carlesso E, et al. Bedside
A. Inhaled nitric oxide for ARDS in children and
selection of positive end-expiratory pressure in mild,
adults. Cochrane Database Syst Rev. 2016;(6). Art.
moderate and severe acute respiratory distress syn-
No.:CD002787.
drome. Crit Care Med. 2013;42(2):1–13.
17. The National Heart, Lung, and Blood Institute Acute
7. Papazian L, Forel JM, Gacouin A, ACURASYS Study
Respiratory Distress Syndrome (ARDS) Clinical
Investigators, et al. Neuromuscular blockers in early
Trials Network. Efficacy and safety of corticosteroids
acute respiratory distress syndrome. N Engl J Med.
for persistent acute respiratory distress syndrome. N
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8. Guérin C, Reignier J, Richard JC, PROSEVA
Study Group, et al. Prone positioning in severe
Management of Sepsis
174
Algorithmic Approach perfusion, elevated lactate, and decreased

capillary refill or mottling [2].
A. Sepsis is a life-threatening organ dysfunction C. Establish IV access and start fluid resuscita-
due to dysregulated response infection. Septic tion. Obtain cultures and then start empiric
shock occurs when underlying circulatory, broad-spectrum intravenous antibiotics as
metabolic, and cellular abnormalities occur soon as possible [1]. Trend labs including
including lactate >2.0 mmol/L and refractory lactate and ScVO2, frequently reassess fluid
hypotension despite sufficient IV fluid resus- responsiveness while providing fluid resus-
citation and vasopressor requirement to main- citation. Patients requiring surgical explora-
tain MAP >65 mmHg [1]. The first step in tion or intervention for emergent source
evaluation of a patient with sepsis is the his- control should be identified with manage-
tory and physical. Questions regarding the ment as soon as medically possible after
onset of infection and concern for organ dys- diagnosis [1].
function are essential. Findings may include D. Initial resuscitation for sepsis-induced hypo-
altered mental status, tachycardia, hypoten- perfusion or lactate >4 mmol/L includes
sion, and tachypnea. Others include fever or 30 mL/kg of IV crystalloid fluid within the
hypothermia, substantial edema or positive first 3 h with initial mean arterial pressure
fluid balance, hyperglycemia in absence of goal of 65 mmHg. Complete reassessments
diabetes mellitus, leukocytosis, leukopenia, of volume status and tissue perfusion with
or normal white count with >10% bands, ele- focused exam and/or with at least two of the
vated CRP, hypotension, elevated mixed following: measure CVP, measure ScvO2,
venous oxygen saturations, and elevated car- bedside echocardiography, or dynamic
diac index [2]. assessment of fluid responsiveness with pas-

B. Signs of organ dysfunction include acute sive leg raise or fluid challenge [3–5].
hypoxemia, acute oliguria, increase in creati- E. Vasopressors may be used for patients in sep-
nine, coagulopathy, ileus, thrombocytopenia, tic shock with hypotension refractory to ade-
hyperbilirubinemia. Signs of decreased tissue quate fluid bolus [1].
F. Antimicrobial therapy should be started
within 1 h of diagnosis. Narrow antibiotics
J. Engelbart · L. J. Garcia (*) once pathogen is identified and sensitivities
Department of Surgery, University of Iowa Hospitals are determined. Typically a treatment dura-
and Clinics, Iowa City, IA, USA tion of 7–10 days, though longer courses may

724 J. Engelbart and L. J. Garcia
be appropriate with slow clinical response, H. Continuous or intermittent sedation should

bacteremia, undrainable source of infection, be minimized in patients with sepsis,
immunologic deficiencies, and certain bacte- though adequate sedation and analgesia
rial, viral, and fungal infections [1, 6]. must be maintained when neuromuscular
G. Supplemental oxygen or mechanical ventila- blockade is in place. Neuromuscular block-
tion may be required for patients with sepsis. ade should be avoided in patients without
A large trial of pressure- and volume-limited ARDS [1].
strategy showed 9% absolute decrease in mor- I. Continue supportive intensive care unit thera-
tality in ARDS patients ventilated with tidal pies including glucose control and deep vein
volume of 6 mL/kg compared with 12 mL/kg thrombosis prophylaxis. Blood product
in adult patients with sepsis-induced ARDS administration is appropriate in symptomatic
and goal of plateau pressure ≤ 30 cm H2O [7]. patients with hemoglobin <7.0 g/dL in the
Spontaneous breathing trials and weaning absence of extenuating circumstances includ-
protocol should be attempted in patients who ing myocardial ischemia or infarction, acute
are ready for wean [1]. hemorrhage, or severe hypoxemia [1, 8].
174 Management of Sepsis 725
H&P:Temp > 38.3°C or < 36.0°C, HR >100, RR > 20, WBC > 12,000
A or < 4,000 or > 10% bands, hypotension altered mental status, edema
or positive fluid balance, hyperglycemia in absence of diabetes,
infection
Findings: signs of organ dysfunction (sepsis), SBP <90 mmHg after

B sufficient IV fluid resuscitation with vasopressor requirement to
maintain MAP > 65 mmHg, lactate > 2 mmol/L (septic shock)
Trend vital signs, frequent physical exams, cardiac and hemodynamic

C monitoring, pulse oximetry, obtain labs including cultures, ScVO2, and
lactate, obtain arterial and venous access and give fluid bolus
Initial resuscitation & fluid therapy

Administer 30 mL/kg crystalloid for hypotension or lactate 4 mmol/L
D within the first 4–6 hours.
Reassess volume status and tissue perfusion
Vasoactive medications
E Use vasopressors for hypotension that does not respond to IV fluid
resuscitation to maintain a mean arterial pressure (MAP) ≥65 mmHg
Antimicrobial therapy & source control

Start broad-spectrum IV antibiotics as soon as possible within 1 hr
F of identifying sepsis. Deescalate when culture data is available.
Pursue source control or surgical intervention as soon as possible
Supplemental O2 or mechanical ventilation

Ventilate with pressure and volume limited ventilation such as 6
G mL/kg tidal volumes in the setting of ARDS/ALI and plateau pressure
≤ 30 cm H2O in patient with sepsis-induced ARDS
Sedation & analgesia

Appropriate sedation and analgesia must be maintained if
H neuromuscular blockade is used in sepsis, avoid NMBA in patients
without ARDS due to risk of prolonged blockade
Supportive management
I Glucose control, DVT prophylaxis, blood product administration,
goals of care, communication of prognosis
Algorithm 174.1
4. Yealy DM, Kellum JA, Huang DT, et al. A random-

References ized trial of protocol-based care for early septic
shock. N Engl J Med. 2014;370(18):1683–93.
1. Rhodes A, et al. Surviving sepsis campaign: interna- 5. Peake SL, Delaney A, Bailey M, et al. Goal-directed
tional guidelines for management of severe sepsis and resuscitation for patients with early septic shock. N
septic shock: 2016. Crit Care Med. 2017;45:486–552. Engl J Med. 2014;371(16):1496–506.
2. Angus DC, Van der Poll T. Severe sepsis and septic 6. Ferrer R, Martin-Loeches I, Phillips G, et al. Empiric
shock. N Engl J Med. 2013;369(9):840–51. antibiotic treatment reduces mortality in severe sepsis
3. Mouncey PR, for the ProMISe Trial Investigators, and septic shock from the first hour: results from a
et al. Trial of early, goal-directed resuscitation for guideline-based performance improvement program.
septic shock. N Engl J Med. 2015;372(14):1301–11. Crit Care Med. 2014;42:1749–55.
7. Brower RG, Matthay MA, Morris A, et al. Ventilation 8. Holst LB, Haase N, Wetterslev J, TRISS Trial
with lower tidal volumes as compared with tradi- Group; Scandinavian Critical Care Trials Group,
tional tidal volumes for acute lung injury and the et al. Lower versus higher hemoglobin threshold
acute respiratory distress syndrome. N Engl J Med. for transfusion in septic shock. N Engl J Med.
2000;342(18):1301–8. 2014;371:1381–91.
Management of Shock
175
Algorithmic Approach with central venous access, pulmonary artery

catheters, and arterial lines. Occasionally
A. Shock can be described as a failure of the cir- echocardiograms are necessary for visualiza-
culatory system to adequately deliver blood, tion. Necessary laboratory studies and imag-
oxygen, and nutrients to vital organs, prefer- ing may include basic metabolic panel
entially the heart and brain. Patients may (BMP), complete blood count (CBC), coagu-
present with tachypnea, altered mental status, lation studies, serum lactate, renal function
and hypotension. tests, liver function tests, electrocardiogram
B. Initial stabilization of the patient’s airway, (ECG), chest X-ray (CXR), arterial blood gas
breathing, and circulation should be estab- (ABG), or blood cultures with more targeted
lished first. This includes securing an airway studies depending on clinical presentation. At
through intubation if necessary, establishing this time, any early interventions should be
IV access, and administering an initial intra- attempted depending on the etiology of
venous fluid bolus while considering the eti- shock.
ology of shock. Total volume of fluid C. Distributive shock occurs in the setting of
resuscitation is determined by the type of anaphylaxis, adrenal crisis, neurogenic shock,
shock. Patients with cardiogenic shock due to and sepsis. Adrenal insufficiency or crisis
infarction of the left ventricle or obstructive presents as an acute cardiovascular collapse
shock due to pulmonary embolism may unresponsive to fluids and vasopressors.
require only small volumes for fluid resusci- Treatment with IV dexamethasone is first line
tation, whereas those with hemorrhagic and can be given empirically with suspicion
shock, sepsis, or infarction of the right ven- of adrenal crisis as it does not interfere with
tricle often require larger volumes for fluid the corticotrophin stimulation test used for
resuscitation. Patients with massive hemor- diagnosis. Neurogenic shock occurs as a
rhage will require blood products. Patient result of a spinal or head injury leading to loss
with shock may require vasopressors and ino- of the sympathetic tone. This results in
tropes in addition to resuscitation, and hemo- decrease in systemic vascular resistance,
dynamic monitoring should be established decrease in blood pressure, decrease in heart
rate, and warm extremities. Initial treatment
J. Engelbart · L. J. Garcia (*) is intravascular fluid resuscitation followed
Department of Surgery, University of Iowa Hospitals by phenylephrine. Anaphylaxis may occur
and Clinics, Iowa City, IA, USA with severe allergic reactions and patients

should be administered epinephrine. Sepsis obvious during the primary or secondary

presents with hypotension, confusion, hyper- survey. Treatment includes volume resuscita-
ventilation, hyperglycemia, and shock in the tion, direct pressor, and emergent surgical
setting of a known or suspected infection. control of bleeding.
Treatment involves a multifaceted approach E. Obstructive shock often presents in the set-
with volume resuscitation, antibiotics, and ting of cardiac tamponade, pulmonary embo-
source control at its core. Fluid resuscitation lism, or tension pneumothorax. All three of
can be guided by mean arterial pressure and these may require emergent intervention
central venous pressure goals as well as uri- including pericardiocentesis, thrombolysis,
nary output, lactate clearance, and venous or needle decompression and a chest tube,
saturations. respectively.
D. Hypovolemic shock occurs as a result of large F. Cardiogenic shock may occur as a result of
fluid losses such as with trauma, burns, hem- massive myocardial infarction, ruptured
orrhage, vomiting, or diarrhea. In hemody- valve, unstable arrhythmia, or severe conges-
namically unstable trauma patients, they are tive heart failure. Treatment includes coro-
considered to be bleeding unless proven oth- nary revascularization, intra-aortic balloon
erwise. A focused assessment with sonogra- pump, surgical valve repair, or following
phy for trauma (FAST) exam or other imaging advanced cardiac life support (ACLS) proto-
may be necessary to identifying bleeding not cols in the case of unstable arrhythmias.
175 Management of Shock 729
A Focused clinical history and examination of patient with shock
Initial stabilization, secure the airway if necessary, establish

B IV access, and administer initial intravenous fluid bolus while
considering etiology of shock, draw laboratory studies
Anaphylaxis – Epinephrine
Adrenal crisis – IV dexamethasone

C Distributive shock
Neurogenic shock – Volume resuscitation,
then phenylephrine
Sepsis – IV antibiotics, volume resuscitation,

vasopressors, source control
D Hemorrhagic shock – Volume resuscitation,

Hypovolemic shock
direct pressure, emergent surgery
Cardiac tamponade – Pericardiocentesis or

pericardial window, volume resuscitation
E Obstructive shock Pulmonary embolism – Thrombolysis
Tension pneumothorax – Chest tube
Myocardial infarction – Inotrope, coronary

revascularization, intra-aortic balloon pump
F Cardiogenic shock Ruptured valve – Surgical valve repair
Arrhythmia – ACLS protocols
Algorithm 175.1
Van Diepen S, Katz JN, Albert NM, et al. Contemporary

Suggested Reading management of cardiogenic shock: a scientific
statement from the American Heart Association.
Rhodes A, et al. Surviving sepsis campaign: international Circulation. 2017;136(16):e232–68.
guidelines for management of severe sepsis and sep- Vincent JL, De Backer D. Circulatory shock. N Engl J
tic shock: 2016. Crit Care Med. 2017;45:486–552. Med. 2013;369(18):1726–34.
Blood Transfusion Indications
176
Algorithmic Approach include orthostatic hypotension or tachycar-

dia not responsive to fluid replacement, myo-
A. Numerous guidelines on blood transfusion
cardial ischemia, angina, or dyspnea, hypoxia,
indications have been published with many and neurologic changes. Chronic anemia
describing specific thresholds for transfusion typically presents with additional symptoms
within specified clinical scenarios. Most also such as fatigue. Chronic anemia may occur
stress that blood products should only be with chronic blood loss (hepatic disorders,
given when clinically necessary as hemoglo- bleeding disorders) or decreased erythropoi-
bin levels do not guarantee adequate delivery esis (malignancies, chemotherapy, other
of oxygen to tissue. Indications for blood drugs suppressing bone marrow, renal disor-
transfusion may include trauma with massive ders, nutritional deficiencies). No definite
blood loss (hemorrhage – surgical, traumatic, triggers have been defined so the decision to
or nonsurgical), anemia, major surgical oper- transfuse is considered on an individual basis
ation, cancer patients requiring therapy, mas- guided by symptoms or functional impair-
sive blood loss or anemia in the setting of ment [1, 2].
pregnancy and childbirth, hereditary disor- D. In hospitalized, hemodynamically stable

ders like hemophilia and thalassemia, critical patients with acute coronary syndrome (i.e.,
illness, and severe burn victims. unstable angina, myocardial infarction), the
B. In the setting of acute hemorrhage with hypo- evidence is unclear in support of liberal or
volemia, hematocrit does not immediately restrictive transfusion thresholds. However,
correlate with blood loss. In this setting, most guidelines recommend transfusion for
transfusions are indicated regardless of the hemoglobin <8 g/dL and considering transfu-
hemoglobin or hematocrit given the acute sions with hemoglobin 8–10 g/dL [3–5].
hemorrhage will result in significant ongoing
E. In hospitalized, hemodynamically stable
blood loss at the time of presentation [1]. patients with preexisting cardiovascular dis-
C. Transfusions should be given in patients with ease, transfusions should be considered with
symptomatic or life-threatening anemia. hemoglobin concentrations of 8 g/dL or less
Signs of ischemia or symptomatic anemia if the patient has congestive heart failure or if
patients are symptomatic with chest pain,
J. Engelbart · L. J. Garcia (*) orthostatic hypotension, or tachycardia unre-
Department of Surgery, University of Iowa Hospitals sponsive to fluid resuscitation. If a patient is
and Clinics, Iowa City, IA, USA undergoing cardiac or orthopedic surgery,

consider transfusion for hemoglobin <8 g/dL dance with a restrictive transfusion strategy
based on clinical evaluation of patient and [1, 2, 11, 12]. In hospitalized, hemodynami-
expected blood loss during surgery [2, cally stable patients, hemoglobin concentra-
5–10]. tion and symptoms should be considered in
F. In intensive care unit patients (i.e., nonsurgi- transfusion decisions. In patients with hemo-
cal/nontraumatic hemorrhage, sepsis), hemoglobin <7 g/dL, transfusion is generally indi-
globin concentrations of 7 g/dL or less should cated; however decision should still be made
prompt consideration of transfusion in accor- based on clinical signs and symptoms [1, 2].
176 Blood Transfusion Indications 733
A Anemia/acute blood loss/massive hemorrhage
Yes
Life-threatening acute blood loss such as massive
B hemorrhage or trauma?
Consider transfusion
No
No
C Hemoglobin < 10 g/dL or hematocrit < 30%? No transfusion
Signs of ischemia or symptomatic?

(myocardiali schemia, orthostatic hypotension or Yes
D Consider transfusion
tachycardia not responsive to fluid replacement,
angina/dyspnea, hypoxia, neurologic changes)
No
Hemoglobin 8-10 g/dL – diagnosis of acute Yes

coronary syndrome or severe thrombocytopenia Consider transfusion
in hematology oncology patient?
No
Hemoglobin 7-8 g/dL – does the patient have Yes

E stable cardiovascular disease or is the patient Consider transfusion
undergoing orthopedic or cardiac surgery?
No
Hemoglobin < 7 g/dL – symptomatic Yes

F hospitalized or ambulatory patients, intensive Consider transfusion
care unit patients
Algorithm 176.1
References American College of Physicians. Ann Intern Med.

2013;159(11):770–9.
6. Ferraris VA, Brown JR, Despotis GJ, et al. 2011
1. Napolitano LM, Kurek S, Luchette FA, et al. Clinical
update to the Society of Thoracic Surgeons and the
practice guideline: red blood cell transfusion in adult
Society of Cardiovascular Anesthesiologists blood
trauma and critical care. J Trauma Inj Infect Crit Care.
conservation clinical practice guidelines. Ann Thorac
2009;67(6):1439–42.
Surg. 2011;91(3):944–82.
2. Carson JL, Stanworth SJ, Roubinian N, et al.
7. Murphy GJ, Pike K, Rogers CA, et al. Liberal or
Transfusion thresholds and other strategies for
restrictive transfusion after cardiac surgery. N Engl J
guiding allogeneic red blood cell transfusion.
Med. 2015;372(11):997–1008.
Cochrane Database Syst Rev. 2016;(10). Article No:
8. Brunskill SJ, Millette SL, Shokoohi A, et al. Red
CD002042.
blood cell transfusion for people undergoing hip
3. Hamm CW, Bassand JP, Agewall S, et al. ESC
fracture surgery. Cochrane Database Syst Rev.
guidelines for the management of acute coronary
2015.
syndromes in patients presenting without persistent
9. Carson JL, Terrin ML, Noveck H, et al. Liberal or
ST-segment elevation: the task force for the manage-
restrictive transfusion in high-risk patients after hip
ment of acute coronary syndromes (ACS) in patients
surgery. N Engl J Med. 2011;365:2453–62.
presenting without persistent ST-segment elevation of
10. Carson JL, Brooks MM, Abbott JD, et al. Liberal ver-
the European Society of Cardiology (ESC). Eur Heart
sus restrictive transfusion thresholds for patients with
J. 2011;32(23):2999–3054.
symptomatic coronary artery disease. Am Heart J.
4. Hanna EB, Alexander KP, Chen AY, Roe MT, Funk
2013;165:964–71.
M, Saucedo JF. Characteristics and in-hospital out-
11. Holst LB, Haase N, Wetterslev J, et al. Lower versus
comes of patients with non-ST-segment elevation
higher hemoglobin threshold for transfusion in septic
myocardial infarction undergoing an invasive strat-
shock. N Engl J Med. 2014;371(15):1381–91.
egy according to hemoglobin levels. Am J Cardiol.
12. Retter A, Wyncoll D, Pearse R, et al. Guidelines on
2013;111(8):1099–103.
the management of anaemia and red cell transfu-
5. Qaseem A, Humphrey LL, Fitterman N, Starkey M,
sion in adult critically ill patients. Br J Haematol.
Shekelle P. Treatment of anemia in patients with
2013;160(4):445–64.
heart disease: a clinical practice guideline from the
Abdominal Compartment
Syndrome 177
Algorithmic Approach elevated central venous pressure though

decreased venous return leading to decreased
A. Abdominal compartment syndrome results
cardiac output [1–3].
from massive interstitial swelling in the abdo-
B. The gold standard for measuring intra-
men or rapid development of mass within the abdominal pressure (IAP) is via the bladder by
abdomen such as ascites or hematoma lead- instilling a specified volume and, measuring
ing to increase in pressure within the abdo- the pressure. Non-specific findings on CT may
men. The first step in evaluation of a patient include collapse of the vena cava, bowel wall
with abdominal compartment syndrome thickening, or bilateral inguinal hernias [2].
(ACS) is the history. Questions regarding the
C. Normal intra-abdominal pressure (IAP) in
risk factors should be targeted toward identi- critically ill adults is 5–7 mmHg and in chil-
fying diminished abdominal wall compli- dren is 4–10 mmHg. When IAP is sustained
ance, increased intra-luminal contents, or repeatedly ≥12 mmHg in adults
increased abdominal contents, capillary leak, or >10 mmHg in children, it is considered
or massive fluid resuscitation. These risk fac- intra-abdominal hypertension (IAH). IAH
tors include but are not limited to acute respi- can be classified as hyperacute (lasts only
ratory failure with prone positioning, elevated seconds—laughing, coughing, sneezing, def-
intrathoracic pressure, major trauma or burns, ecation), acute (develops over hours—trauma
abdominal surgery with fascial closure, obe- or hemorrhage), subacute (develops over
sity, gastroparesis, ileus, pseudo-obstruction, days), and chronic (develops over months—
sepsis, oliguria, ascites, pancreatitis, liver pregnancy, morbid obesity) [4]. IAH can be
dysfunction, coagulopathy, hemoperitoneum, graded by intra-abdominal pressure: Grade
pneumoperitoneum, acidosis, or hypotension. I = IAP 12–15 mmHg, Grade II = IAP
Patients with ACS may present with acute 16–20 mmHg, Grade III = IAP 21–25 mmHg,
kidney injury, bowel wall ischemia and and Grade IV = IAP > 25 mmHg. Abdominal
edema, reduced lung compliance with ele- compartment syndrome (ACS) in adults is
vated airway pressures and decreased tidal defined as sustained IAP > 20 mmHg with or
volumes, increased intracranial pressure, and without an abdominal perfusion pressure
(APP = MAP – IAP) < 60 mmHg (children
J. Engelbart · L. J. Garcia (*) with IAP > 10 mmHg) and is associated with
Department of Surgery, University of Iowa Hospitals new or progressing organ dysfunction and
and Clinics, Iowa City, IA, USA failure that can be attributed to elevated IAP

[2, 5, 6]. ACS is better defined without E. Abdominal decompression with delayed
specified pressure thresholds as no specific c losure may be attempted with IAP >20 mmHg
IAP threshold can consistently be used to and organ dysfunction not responding to medi-
diagnose ACS [5]. cal treatment. There is a risk of hypotension
D. Supportive management and temporizing
leading to pulseless electrical activity (PEA)
measures for ACS include drainage of intra- arrest from reperfusion and sudden decrease in
luminal contents with nasogastric and rectal systemic vascular resistance. Even with tem-
decompression; removal of intra-abdominal porary closure there remains risk of recurrent
ascites or hematomas; reduction of intra- abdominal compartment syndrome [1, 2, 8].
abdominal volume by avoiding positive fluid F. Temporary abdominal closure techniques
balance after initial resuscitation and diure- include negative pressure systems including
sis; improving abdominal wall compliance towel- and sponge-based techniques (vacuum-
with analgesia, sedation, and paralysis; assisted closure), patch closure, silo closure, or
decreasing head elevation; escharotomy in skin-only closure. If primary approximation is
burn victims; and removal of constrictive not able to be achieved upon return to the oper-
binders or dressings. Vasopressors may be ating room, other adjunctive techniques may
used to maintain an abdominal perfusion be used to facilitate primary closure or approx-
pressure > 60 mmHg. IAP should be mea- imate the fascia closer to midline. If no
sured at least every 4 h while patient is criti- improvement, functional closure or a planned
cally ill or with elevated IAP [1, 2, 7]. ventral hernia may need to be attempted.
177 Abdominal Compartment Syndrome 737
Patient has two or more risk factors or in the presence of

A organ failure with clinical suspicion for ACS
B Assess intraabdominal pressure by measuring the

bladder pressure
C IAP < 20 mmHg IAP > 20 mmHg
Supportive management
No
(sedation, analgesia, Organ
D paralysis, paracentesis, dysfunction
nasogastric decompression)
Yes
No response
Surgical management
E (abdominal decompression)
Close monitoring Recurrence
Temporary abdominal
F closure
Improvement Return to OR
No
Advance fascial edges Abdominal
towards midline closure
No improvement
Yes
Functional closure
Planned ventral hernia Primary fascial closure
Algorithm 177.1
References 4. Van Mook WN, et al. Abdominal compartment

syndrome. Lancet. 2002;360:1502.
5. Malbrain ML, et al. Results from the international
1. Rogers WK, Garcia L. Intra-abdominal hyperten-
conference of experts on intra-abdominal hyper-
sion, abdominal compartment syndrome, and the
tension and abdominal compartment syndrome.
open abdomen. Chest. Forthcoming 2017. https://doi.
I. Definitions. Intensive Care Med. 2006;32:1722–32.
org/10.1016/j.chest.2017.07.023.
6. Sugrue M. Abdominal compartment syndrome. Curr
2. Kirkpatrick AW, et al. Intra-abdominal hyperten-
Opin Crit Care. 2005;11:333–8.
sion and the abdominal compartment syndrome:
7. Bailey J, et al. Abdominal compartment syndrome.
updated consensus definitions and clinical practice
Crit Care. 2000;4:23–9.
guidelines from the World Society of the Abdominal
8. Chang MC, et al. Effects of abdominal decompres-
Compartment Syndrome. Intensive Care Med.
sion on cardiopulmonary function and visceral perfu-
2013;39:1190–206.
sion in patients with intra-abdominal hypertension. J
3. Vidal MG, et al. Incidence and clinical effects of
Trauma. 1998;44:440–5.
intra-abdominal hypertension in critically ill patients.
Crit Care Med. 2008;36:1823–31.
Acute Renal Failure
178
Algorithmic Approach times the patient’s baseline, or (3) a urine

volume ≤0.5 ml/kg/h for 6 h [1]. Etiology of
A. Evaluation of acute renal failure requires
AKI is broad and includes hypovolemia due
careful review of clinical history and physical to dehydration or hemorrhage, intrinsic renal
examination. Recent studies have estimated disease, exposure to nephrotoxic agents, as
that 3–21% of all hospitalized patients and up well as obstructive pathology such as renal
to 50% of ICU patients develop acute kidney stones and bladder outlet obstruction due to
injury (AKI) [1]. Clinicians should be aware neoplasm. A common cause of acute renal
that AKI etiology is often multifactorial and failure is acute tubular necrosis secondary to
that epidemiological evidence purports that impaired blood flow and resultant hypoxic
even mild, reversible renal injury can have injury to renal tubular cells [1]. Post-surgical
severe clinical consequences, including acute tubular necrosis (ATN) contributes to
increased mortality [2]. Baseline renal status 20–25% of all cases of hospital-acquired
should be obtained along with vital signs, AKI [3].
chest X-ray, and pertinent laboratory studies, Classification systems such as RIFLE can
including BUN/Cr, hemoglobin/hematocrit, assist in clinical evaluation. To help differen-
and electrolytes. The presence of anuria or tiate etiology, a clinician should first rule out
oliguria can be prognostic. common post-renal causes with renal ultra-
B. AKI is diagnosed when a patient meets any sound. Urinalysis in addition to urine electro-
one of the following criteria: (1) an increase lytes should be obtained if renal ultrasound is
in serum creatinine by ≥0.3 mg/dl within found to be negative [2]. A clinician should
48 h, (2) an increase in serum creatinine ≥1.5 be mindful that certain pathologic states such
as compartment syndrome can lead to vascu-
lar compromise and decreased renal perfu-
K. A. Iles
SUNY Upstate Medical University College of sion with resultant kidney injury.
Medicine, Syracuse, NY, USA C. The fluid challenge is the gold standard used
Department of Surgery, University of North Carolina to assess fluid responsiveness and to guide
Hospitals, Chapel Hill, NC, USA fluid administration [4]. The most common
e-mail: [email protected] approach involves the administration of
R. J. King (*) 500 cc crystalloid within a 30-min time
Department of Surgery, SUNY Upstate Medical frame. Although there exists variability in
University, Syracuse, NY, USA measurement and precision of technology

740 K. A. Iles and R. J. King
used, responsiveness is defined as an increase F. Mortality has been estimated to be up to 50%

of 10–15% in stroke volume following a fluid when patients with AKI require renal replace-
challenge [4]. Fractional excretion of Na ment therapy [3]. There are currently no pub-
(FeNa) can differentiate between pre-renal lished guidelines indicating when renal
and renal causes. A FeNa <1% is diagnostic replacement therapy should be initiated.
of pre-renal causes. FeNa = (urine Na/creati- Nevertheless, consensus agrees that early
nine)/(plasma Na/creatinine). renal replacement therapy results in better
D. Certain insults such as major burns and sepsis clinical outcomes. Absolute indications for
often create a mixed etiology of hypovolemia renal replacement therapy include the fol-
and acute tubular necrosis due to depressed lowing: (1) anuria for 6 h, (2) severe oliguria
renal blood flow. Such a condition may (urine output <200 ml over 12 h), (3) hyper-
require renal replacement therapy (RRT) as a kalemia (>6.5 mmol/L), (4) severe metabolic
temporizing measure until the kidneys acidosis (pH <7.2 despite low-normal pCO2
recover. A nephrology consult should be con- level), (5) volume overload, (6) severe azote-
sidered to help guide renal replacement ther- mia (urea concentration > 30 mmol/L or cre-
apy management. atinine concentration > 300 μmol/L), and (7)
E. If the patient is responsive to fluid resuscita- uremic complications (encephalopathy, peri-
tion, this is diagnostic for AKI secondary to carditis, etc.) [1]. Hemodynamic stability
pre-renal hypovolemia. Urine output guides should be taken into consideration prior to
fluid resuscitation in addition to blood pressure, initiation of RRT. Patients that are stable and
heart rate, and both invasive and noninvasive more apt to tolerate larger fluid shifts may be
monitoring. Central venous pressure (CVP), candidates for intermittent hemodialysis,
PA catheter monitoring, cardiovascular ultra- while unstable patients may benefit from
sound, and hemodynamic monitors such as continuous veno- venous hemofiltration
Vigileo™ are often utilized for this purpose. (CVVH).
178 Acute Renal Failure 741
24 year-old with multiple gunshot wounds s/p exploratory

laparotomy. Post-operative day #1 urine output < 0.5 ml/kg/h over
past 6 h
A Obtain vital signs and blood work
T 37.6 C, HR 103, RR 19, BP 110/82 Life-threatening

Labs: elevated crt, electrolytes fluid or electrolyte
abnormalities?
Yes
AKI
B etiology? Urgent
dialysis
Renal ultrasound Post-renal

Check Foley catheter
Obtain UA & Renal

urine electrolytes
Compartment syndrome Patient reassessment Nephrology consult
C D
Yes Responsive No
Pre-renal to fluid Mixed etiology: pre-renal & renal
challenge?
F Renal replacement therapy
Consider adjunct
Fluid measures to assess
resuscitation volume status Stable?
Yes No
Hemodialysis CVVH
Algorithm 178.1
References 3. Lameire N, Biesen WV, Vanholder R. Acute renal

failure. Lancet. 2005;365(9457):417–30.
4. Toscani L, Aya HD, Antonakaki D, Bastoni D, Watson
1. Koza Y. Acute kidney injury: Current concepts and
X, Arulkumaran N, et al. What is the impact of the
new insights. J Inj Violence Res. 2016;8(1):58–62.
fluid challenge technique on diagnosis of fluid respon-
2. Kellum JA, Lameire N. Diagnosis, evaluation, and
siveness? A systematic review and meta-analysis. Crit
management of acute kidney injury: a KDIGO sum-
Care. 2017;21(1):207.
mary (Part 1). Crit Care. 2013;17(1):204–19.
Postoperative Pulmonary Emboli
179
Algorithmic Approach ologies for this compilation of symptoms

such as acute myocardial infarction should be
A. Surgical injury alters the balance between
considered.
coagulation and fibrinolysis, predisposing B. Immediate empiric treatment is determined
patients to increased risk of deep venous and by patient stability and contraindications to
pulmonary thromboembolism [1]. The first anticoagulation and/or thrombolysis.
step in evaluation of a patient with possible Hemodynamically unstable patients require
pulmonary embolism is history and physical immediate therapeutic anticoagulation and
examination. High index of clinical suspicion consideration of thrombolysis or thrombec-
based on patient history for genetic risk fac- tomy. Stable patients can undergo further
tors in addition to recent behavioral changes diagnostic evaluation based on clinical suspi-
and health risks should guide work-up and cion and availability of resources.
evaluation. Presenting symptoms for pulmo- C. The Wells prediction scoring system is com-
nary emboli are often nonspecific and can monly employed to categorize patient risk for
range from an asymptomatic presentation to thrombus formation. This scoring stratifica-
fever, dyspnea, hypotension, tachypnea, or tion is often used to predict need for chemo-
unexplained tachycardia. Chest pain may prophylaxis but can also guide clinical
develop acutely or worsen over several days. suspicion when pulmonary embolus is sus-
Pleuritic chest pain and hemoptysis may also pected. Patients with moderate to high prob-
be present; however this is likely to be sec- ability for PE can progress to imaging such as
ondary to pulmonary infarction [2]. Other eti- CT angiography or ventilation-perfusion
radionuclide scanning. Patients who have low
probability and not subject to recent surgery,
trauma, or who are pregnant can be evaluated
K. A. Iles
SUNY Upstate Medical University College of with a D-dimer assay. Although a positive
Medicine, Syracuse, NY, USA D-dimer test is often nonspecific, it can be a
Department of Surgery, University of North Carolina useful diagnostic tool in combination with
Hospitals, Chapel Hill, NC, USA clinical probability. ELISA-based D-dimer
e-mail: [email protected] tests have been shown to demonstrate supe-
R. J. King (*) rior sensitivity [2].
Department of Surgery, SUNY Upstate Medical D. Angiography is definitive imaging, although
University, Syracuse, NY, USA now CT angiography has high sensitivity and

specificity for PE, especially in hemodynami- however, retrievable IVC filter insertion
cally significant PE. Common radiologic should be performed if anticoagulation is con-
findings include filling defects in pulmonary traindicated or temporary cessation of antico-
vasculature on CTA +/− evidence of pulmo- agulant is required in 1 month [3]. IVC filters
nary infarction. V/Q scanning will often show should be removed within the recommended
mismatched segment perfusion defects. time scale in order to limit associated compli-
E. Treatment for pulmonary embolism is depen- cations of IVC filter placement and retrieval.
dent on anticoagulation contraindications and The incidence of confirmed hospital-
patient stability. Treatment consists of thera- acquired DVT is approximately 10–40%
peutic anticoagulation to prevent clot propa- among medical or general surgical patients
gation. If the patient is hemodynamically and up to 40–60% among orthopedic surgical
unstable, catheter-directed thrombolysis may patients. The incidence of hospital deaths
be indicated and potentially thoracotomy attributed to pulmonary embolus is estimated
with thrombectomy. Patients unable to be to be 10% [4]. These statistics highlight the
anticoagulated or undergo thrombolysis due irrefutable need and awareness for thrombo-
to recent surgery such as central nervous sys- prophylaxis, intermittent pneumatic compres-
tem (CNS) interventions may need temporary sion devices, and early mobilization in the
IVC filter placement. hospitalized in order to decrease thrombus
Routine placement of IVC filters in sub- formation and prevent PE, especially in the
massive PE and proximal deep vein thrombo- surgical patient [1].
sis (DVT) is not supported by evidence; *signifies a level <500μg/L reliably excludes PE.
179 Postoperative Pulmonary Emboli 745
A Obtain vital signs, blood work, ECG and

73 year-old female with femur fracture s/p open
perform physical examination
reduction and internal fixation. Post-operative day
#6 with new onset dyspnea, tachycardia, and
chest pain
T 38.1 C, HR 108, RR 24, BP 100/78, WBC 16
Exam findings: mild distress, leg swelling, tachypnea
ECG: tachycardia, non-specific ST segment changes and T-wave abnormalities
B
No
C Assess PE probability Yes Hemodynamically
stable?
Resuscitate
Thrombolytics
embolectomy
Low Moderate High
D-dimer level*
D
Yes Anticoagulation
Elevated D-dimer & Obtain Imaging
high clinical suspicion
Stable?
E No
CTA: filling defects in pulmonary vasculature No
V/Q Scan: mismatched segment perfusion defects Thrombolytics
Contraindication
to anticoagulation
& thrombolytics?
Embolectomy
Yes thoracotomy
Yes
Stable?
No IVC filter
Algorithm 179.1
3. Condliffe R, Elliot CA, Hughes RJ, et al. Management

References dilemmas in acute pulmonary embolism. Thorax.
2014;69(2):174–80.
1. Kehlet H, Wilmore DW. Multimodal strate- 4. Hirsh J, Guyatt G, Albers GW, Schünemann HJ. The
gies to improve surgical outcome. Am J Surg. seventh ACCP conference on antithrombotic and
2002;183(6):630–41. thrombolytic therapy. Chest. 2004;126(3):172S–3S.
2. Tapson VF. Acute pulmonary embolism. N Engl J
Med. 2008;358(10):1037–52.
Burns Management
180
Algorithmic Approach resuscitation, and appropriate wound treat-

ments. These may include escharotomy and
A. Initial burns assessment can be performed in early excision and grafting of full-thickness
the field or at the closest medical facility for burns in addition to ancillary support of nutri-
initial stabilization. Inhalation injuries may tion, physical and occupational therapy, and
require immediate securing of the airway. psychological support needs. Smaller burns
Burns associated with structure or usually <20% TBSA can also require admis-
transportation- related fires may require sion for burn wound and pain management as
trauma assessment and stabilization at the well as for social services support. This
nearest facility. Chemical burns may require includes, but is not limited to, Child Protective
decontamination. Services (CPS) consultations for possible
B. With the advent of regional burn centers and Non-accidental Trauma (NAT) and Red Cross
advancements in burn care, a concurrent, assistance for possible housing and/or homec-
multimodal approach of fluid resuscitation, are coordination. Most burns <10% TBSA
wound care, nutritional support, and modula- can be managed as an outpatient.
tion of the hypermetabolic state has led to Regardless of inpatient versus outpatient
significant improvement in the care of the care, a physician or social worker should
critically ill burn patient [1]. Burns greater address the patient’s functional capacity in
than 20–30% TBSA and/or with inhalation addition to their ability to provide adequate
injury require triage preferably to an ABA- wound care and infection monitoring prior to
verified burn center for ICU-level care, fluid discharge. Visiting nurses or transportation
may be needed to ensure patients receive suf-
ficient follow-up care [2].
C. Indeterminate and/or partial-thickness burns
K. A. Iles
SUNY Upstate Medical University College of are treated initially with dressings that
Medicine, Syracuse, NY, USA maintain a moist bacteriostatic or bacteri-
Department of Surgery, University of North Carolina cidal wound environment to facilitate reepi-
Hospitals, Chapel Hill, NC, USA thelialization. Dressings should also be
e-mail: [email protected] tailored to the needs of the patient to mini-
R. J. King (*) mize painful dressing changes, especially in
Department of Surgery, SUNY Upstate Medical children. Dressings impregnated with silver
University, Syracuse, NY, USA can be helpful in this regard as they can

remain in place for upward of 1–2 weeks. blood loss, infection, and length of hospital
Disruption of the innate immune barrier stay and provide increased graft take [3].
provided by the skin predisposes patients to Full-thickness burns are dressed with salves
bacterial infection. Infection in burn patients which penetrate eschar such as silver sulfadi-
is a ssociated with significant morbidity and azine (Silvadene) and mafenide acetate
mortality. Wound infection requires a rapid (Sulfamylon) to reduce risk of infection.
diagnosis followed by possible excision of Alternatively, large areas of partial and inde-
infected tissue and appropriate antibiotic terminate thickness mixed with areas of full-
coverage. Inspection of wounds should be thickness burns may be dressed with
carried out by a qualified surgeon or wound petroleum-based salves such as bacitracin,
care expert [3]. Xeroform, and Vaseline while awaiting end
D. Fluid resuscitation with the modified Brooke points of initial resuscitation prior to excision
formula (2 ml/kg/%TBSA) can be titrated and grafting.
according to urine output, heart rate, and F. Various burn dressings exist and can be
blood pressure in addition to guidance by selected based on patient needs. Dressing of
invasive and noninvasive cardiac monitoring. the wound serves multiple purposes, includ-
Care should be taken to avoid over- ing a reduction in wound pain, protection,
resuscitation, which may result in acute lung and isolation from the environment and
injury or compartment syndrome. Use of absorption of wound drainage [2]. Most
nurse-driven protocols can be helpful in this silver-impregnated dressings such as hydrofi-
regard. Nutritional support in the face of bers (Aquacel Ag) or silicone base (Mepitel
burn-induced catabolism should begin early, Ag) can be left in place for 2 weeks and are
and consideration should be given to the use useful for outpatient treatment, especially in
of the anabolic steroid oxandrolone and pro- children. Petroleum-based dressings, such as
pranolol. Use of high-dose vitamin C should bacitracin and Vaseline, Xeroform, and
be considered early in resuscitation to blunt Adaptic, are inexpensive and readily avail-
the deleterious inflammatory response. able but may require frequent reapplication.
E. Early excision and grafting is performed pref- Silver sulfadiazine (Silvadene), while com-
erably within 72 h of admission. This stan- monly known and widely available, may
dard of care has been shown to decrease impair wound healing and is less favored.
180 Burns Management 749
A
56 year-old female presenting Burns Assessment
with burn after house fire
>20–30% BSA
B
Fluid Resuscitation
Severe Pain Admission No
Outpatient Care
Wound Care Required?
Social Factors
NAT
D Yes
Fluid Resuscitation Social Services Nutrition Pain Control Hospital Admission
Modified Brooke Formula Initiate Tube Feeds Burn Depth
Nurse Driven C
Resuscitation
Protocol
Circumferential Full Deep Partial Indeterminate/Partial
Consider E Consider Reassess in 3 Weeks Infection?

Early Excision
Escharotomy
& Grafting
F Yes
Burn Dressings Consider Healing?

Grafting No
Antibiotics
Short Term Long Term
Algorithm 180.1
2. Herndon DN. Total burn care. Saunders Elsevier:

References Edinburgh; 2012.
3. Rowan MP, Cancio LC, Elster EA, Burmeister DM,
1. Snell JA, Loh N-HW, Mahambrey T, Shokrollahi Rose LF, Natesan S, et al. Burn wound healing and
K. Clinical review: the critical care management of treatment: review and advancements. Crit Care.
the burn patient. Crit Care. 2013;17(5):241–51. 2015;19:243–55.
Acid-Base Disorders
181
Algorithmic Approach gap can be calculated from the following for-

mula: anion gap = Na − (Cl + HCO3).
A. Diagnosis of acid-base disturbances can be Etiologies of non-anion gap metabolic acido-
challenging and requires careful clinical eval- sis are most often secondary to defective
uation. The initial approach to acid-base dis- acidification by renal tubules, gastrointestinal
orders requires arterial blood gas analysis. pH bicarbonate losses, and iatrogenic excess
and determination of pCO2 indicate either chloride administration [2]. If an anion gap is
primary respiratory or metabolic disorder present, consider the classically taught
with either metabolic or respiratory compen- “MUDPILES” differential diagnoses: metha-
sation. Note that respiratory compensation nol, uremia, diabetic ketoacidosis, paralde-
for primary metabolic disorders occurs rap- hyde, iron/isoniazid, lactic acidosis, ethylene
idly. Conversely, metabolic compensation for glycol, and salicylates.
primary respiratory disturbances may require Surgical patients with a metabolic acidosis
3–5 days for renal adjustment [1]. may suggest reduced end-organ perfusion
B. Respiratory acidosis may require respiratory and ischemia. Metabolic acidosis is impera-
support such as noninvasive positive pressure tive to diagnose in this population as it predis-
ventilation or intubation with mechanical poses the patient to arrhythmias, impaired
ventilation. This state can occur from leukocyte function, decreased cardiac output,
hypoventilatory states, airway obstruction, insulin resistance, suppression of lymphocyte
and CNS depression. Metabolic acidosis function, and leads to an overall increase in
requires determination of anion gap and a dif- morbidity and mortality [3]. Lactic acidosis
ferential diagnosis to guide treatment. Anion accounts for 50% of all cases presenting with
a high anion gap metabolic acidosis. However,
anion gap is an insensitive measure of lactate
K. A. Iles
SUNY Upstate Medical University College of level and a lactate level should be obtained
Medicine, Syracuse, NY, USA for further evaluation [2].
Department of Surgery, University of North Carolina
C. Metabolic alkalosis as a primary disorder
Hospitals, Chapel Hill, NC, USA reflects either a loss of acid through vomiting
e-mail: [email protected] and/or nasogastric suctioning or excessive
R. J. King (*) loss of bicarbonate-rich fluids through diar-
Department of Surgery, SUNY Upstate Medical rhea or renal losses [4]. Respiratory alkalosis
University, Syracuse, NY, USA
often presents in mechanically ventilated

patients with increased minute ventilation promise, consider administration of bicar-

either due to improved lung compliance or bonate if pH <7.1. These patients should be
correction of an underlying metabolic carefully monitored with the goal of main-
acidosis. If the patient is intubated, ventilator taining a blood pH at approximately 7.2 [3].
settings should be reevaluated and minute Adequate monitoring of response to resusci-
ventilation reduced. In non-ventilated tative efforts may require frequent laboratory
patients, CNS lesions, anxiety/pain, and analysis as well as invasive and noninvasive
chronic pulmonary disease should be monitoring of volume status, blood pressure,
considered. and cardiac function.
D. Correction of lactate acidosis requires ade-
E. Adequate fluid resuscitation and oxygen
quate volume and oxygen-carrying hemoglo- delivery can often be accomplished with
bin, as well as maintenance of adequate treatment of the underlying pathophysiology.
perfusion pressure. Blood transfusion and/or If sepsis is suspected by either clinical history
vasopressors may be needed depending on or pro-calcitonin levels, then appropriate
the underlying pathophysiology. In patients broad-spectrum antibiotic therapy should be
with lactic acidosis and cardiovascular com- initiated.
58 year-old male trauma victim s/p exploratory Assess Arterial pH, Partial Pressure of CO2,
A laparotomy. Mechanically ventilated, post- and Bicarbonate
operative day #1.
Gastrointestinal
Losses
Excessive
B pH <7.40 pH >7.40 Diuresis
Acidosis Alkalosis C NG Suction
Hypoventilation
181 Acid-Base Disorders
Airway
PCO2 >45
Obstruction Respiratory Acidosis Bicarbonate Level >30 mmol/L Metabolic Alkalosis
mmHg
CNS Depression
Drug Overdose
Metabolic Acidosis Bicarbonate Level <22 mmol/L PCO2 <35 mmHg Respiratory Alkalosis
Anion Gap Yes No

Present? Intubated?
No Yes
Dialysis
Hemofiltration
Non-Anion Gap Hyperventilation
Metabolic Acidosis “MUDPILES” Confirm Ventilator Settings Anxiety/Pain
Yes CNS Disease
Lung Disease
GI Bicarbonate Losses Decrease Minute Pregnancy
Lactate Level
Renal Tubular Acidoses Ventilation High Altitude
Excess Chloride Administration Overload or Renal
Impairment?
D
E
Hypotensive
Adequate Yes Despite Fluid
Resuscitation Assess Volume Status Hypovolemic? Resuscitate
Resuscitation (MAP
and Perfusion?
<65 mmHg)?
Sepsis? Yes
No Reversible Yes
Etiology & pH Bicarbonate
<7.1? Yes
Vasopressor
Antibiotics Support
753
Algorithm 181.1
References 3. Kraut JA, Madias NE. Metabolic acidosis:

pathophysiology, diagnosis and management. Nat

Rev Nephrol. 2010;6(5):274–85.
1. Harber R. A practical approach to acid-base disor-
4. Seifter JL, Chang H-Y. Disorders of acid-base balance:
ders. West J Med. 1991;155(2):146–51.
new perspectives. Kidney Dis. 2016;2(4):170–86.
2. Berend K, de Vries APJ, Gans ROB. Physiological
approach to assessment of acid–base disturbances. N
Engl J Med. 2015;372(2):193–5.
Part XXI
Electrolytes
Hyponatremia
182
Algorithmic Approach transdermal sodium loss from heavy sweat-

ing, diuretics (especially thiazides), primary
A. Hyponatremia is one of the most common adrenal insufficiency, kidney dysfunction, or
electrolyte disorders seen in hospitalized third spacing from a process such as pancre-
patients. It can be categorized as either hyper- atitis or sepsis [2]. Euvolemic hyponatremia
tonic, isotonic, or hypotonic hyponatremia, can be seen in cases of syndrome of inappro-
where the tonicity is determined by the sol- priate antidiuretic hormone secretion
utes that cannot move freely across cell mem- (SIADH), secondary adrenal insufficiency,
branes [1]. Hypertonic or isotonic hypothyroidism, and instances where the
hyponatremia can occur when there are addi- patient has a high water and low solute intake
tional osmoles in the serum, such as glucose, [2, 3]. In hypervolemic hyponatremia, the
mannitol, or glycine (absorbed via irrigation patient may have renal disease, heart failure,
fluids used in urological or gynecological liver failure, or nephrotic syndrome [4].
procedures). These osmoles increase tonicity Assessing volume status and measuring
and reduce the serum sodium concentration urine electrolyte excretion can be used to help
by drawing water from the intracellular com- determine the underlying etiology and decide
partment [2]. The most common is hypotonic on further workup and therapy [4]. Unless the
hyponatremia, where there is an excess of kidney is the reason for the sodium loss, the
water relative to sodium stores and an overall spot urine sodium should be <30 mmol/L in
dilution of body solutes [1]. In the hypovole- hypovolemic hyponatremia [2]. A spot urine
mic patient, possible etiologies include gas- should be > or = 20 to 30 mmol/L in cases of
trointestinal sodium loss such as in vomiting, euvolemic hyponatremia. In hypervolemic
hyponatremia, the spot urine sodium is typi-
cally low (<20–30 mmol/L) because of acti-
K. W. Shaw (*) vation of the renin-angiotensin-aldosterone
Department of Surgery, Division of Transplant system despite total-body volume overload
Surgery, University of Washington Medical Center,
Seattle, WA, USA [4].
e-mail: [email protected] B. Nearly 8% of surgical patients have preopera-
A. A. S. Dick (*) tive hyponatremia, and it has been shown to
Department of Surgery, Section of Pediatric be a prognostic marker for perioperative
Transplantation, Seattle Children’s Hospital and 30-day morbidity and mortality [5].
University of Washington, Seattle, WA, USA Symptoms of hyponatremia can be mild to

758 K. W. Shaw and A. A. S. Dick
severe, including nausea, confusion, restricted. Three percent NaCl can be used in
headache, emesis, cardiorespiratory distress, either repeated 100–150 ml IV boluses up to
seizures, and coma [2]. Acute hyponatremia 3 times or as an infusion, depending on the
is known to have a high mortality rate due to severity of symptoms, for an initial target of a
osmotically induced brain edema. Chronic 5–6 mmol/l increase [2, 3]. Serum sodium
hyponatremia is associated with more subtle levels should be checked 20 min after each
abnormalities, including gait disturbances 3% NaCl IV bolus and every 4 h for as long
and concentration and cognitive deficits, as a 3% NaCl IV infusion is running [2].
though the affected patient may appear D. In chronic hyponatremia patients who are
“asymptomatic” on initial exam [4]. either asymptomatic or have mild symp-
C. In correcting severe, symptomatic hyponatre- toms, management should be cause-specific.
mia, there must be a balance between timely Hypervolemic and euvolemic patients are
therapy for this potentially fatal condition and generally treated with fluid intake restric-
avoiding the severe neurological deficits and tion, and the hypovolemic patient can be
death that can occur from osmotic demyelin- treated with 0.9% NaCl IV infusion to
ation when correction is done too rapidly. An restore extracellular volume [2]. In cases
acceptable limit of correction of chronic where there is concern for brain injury from
hyponatremia is 10–12 mmol/L/d or overcorrection, an electrolyte free water IV
18 mmol/L within 48 h [4, 6]. In acute symp- infusion or IV desmopressin should be
tomatic hyponatremia, where the known administered under expert guidance to lower
duration of hyponatremia is <24–48 h, the the serum sodium level to the acceptable
rate of correction does not need to be correction range [2, 3].
182 Hyponatremia 759
Patient found to have hyponatremia on

inpatient laboratory results

Assess for severity of symptoms, whether it is acute or chronic in nature, volume status,
obtain blood and urine studies to help determine etiology
A
Hypertonic or
isotonic Yes
hyponatremia Treat hyperglycemia
from
hyperglycemia?
No
Consider immediate treatment with 3% hypertonic saline

Moderate to Yes
B severe
with frequent sodium level checks
May use repeated bolus dosing if severe symptoms vs
symptoms?
starting with infusion +/- initial bolus if moderate symptoms
No
If acute (<48hrs) → Do not need to restrict rate of correction

Treatment will be cause-specific
If chronic → Limit increase serum sodium concentration to
10-12 mmol/L/d and 18 mmol/L within first 48hrs
D Fluid restriction
Expanded Limit increase serum sodium concentration to 10-12 mmol/L/d and
18 mmol/L within first 48hrs for chronic hyponatremia
Expanded or reduced
cellular volume?
Reduced
Or euvolemic with
IV infusion of 0.9% saline solution at 0.5-1.0 ml/kg per hour
SIADH?
Limit increase serum sodium concentration to 10-12 mmol/L/d and
18 mmol/L within first 48hrs for chronic hyponatremia
SIADH
First line: restrict fluid intake for moderate or profound hyponatremia
Second line: increase solute intake with urea or use a combination of low-
dose loop diuretics and oral sodium chloride
Algorithm 182.1
References 4. Verbalis JG, Goldsmith SR, Greenberg A,

Korzelius C, Schrier RW, Sterns RH, Thompson
CJ. Diagnosis, evaluation, and treatment of hypona-
1. Adrogue HJ, Madias NE. Hyponatremia. N Engl J
tremia: expert panel recommendations. Am J Med.
Med. 2000;342:1581–9.
2014;126(10):S1–S42.
2. Spasovski G, Vanholder R, Allolio B, Annane D, Ball
5. Leung AA, McAlister FA, Rogers SO Jr, Pazo V,
S, Bichet D, Decaux G, Fenske W, Hoorn EJ, Ichai
Wright A, Bates DW. Preoperative hyponatremia
C, Joannidis M, Soupart A, Zietse R, Haller M, van
and perioperative complications. Arch Intern Med.
der Veer S, Van Biesen W, Nagler E. Clinical prac-
2012;172(19):1474–81.
tice guideline on diagnosis and treatment of hypo-
6. Sterns RH, Cappuccio JD, Silver SM, Cohen
natraemia. Nephrol Dial Transplant. 2014;29(Suppl.
EP. Neurologic sequelae after treatment of severe
2):i1–i39.
hyponatremia: a multicenter perspective. J Am Soc
3. Sterns RH, Nigwekar SU, Hix JK. The treatment of
Nephrol. 1994;4(8):1522–30.
hyponatremia. Semin Nephrol. 2009;29(3):282–99.
Hypernatremia
183
Algorithmic Approach sodium accumulation [4]. Less common is

pure sodium excess from instances of inges-
A. Hypernatremia is essentially a deficit of water tion, such as taking sodium tabs, salt poison-
relative to the sodium stores of a patient. It is ing from near-drowning in sea water, or even
a common electrolyte disorder, affecting up excessive soy sauce consumption in a suicide
to 3% of hospitalized patients and up to 9% of attempt [3].
patients admitted to the intensive care unit Sustained hypernatremia can only occur
(ICU), with associated mortality rates rang- when thirst or access to water is limited, so
ing from 42% to 60% [1]. Hypernatremia the populations most at risk include patients
occurs when there is either a net water loss, with altered mental status or intubation as
which is most often the case, or a hypertonic well as infants and elderly persons [2]. The
sodium gain [2]. Net water loss can occur by signs and symptoms of hypernatremia pri-
either renal mechanisms, such as diabetes marily come from neurologic dysfunction;
insipidus or osmotic diuresis, or by extra hyperosmolality leads to a shift of free water
renal mechanisms, such as gastrointestinal from the intracellular to the extracellular
losses, excessive sweating, or insensible loss space, causing brain cell shrinkage. The brain
through the airway [3]. Sodium gain often cell shrinkage can progress to vascular rup-
occurs in an ICU setting, where administer- ture and subsequent bleeding that results in
ing sodium-rich antibiotics, using hypertonic permanent neurological deficit or death [2].
solutions such as sodium bicarbonate, or The cerebral demyelination that can be seen
resuscitating with 0.9% saline in a patient in the rapid correction of hyponatremia has
with multisystem organ failure can lead to also been reported in hypernatremia as well
[4]. Hypernatremia can also cause impaired
glucose utilization and gluconeogenesis,
K. W. Shaw (*) decreased left ventricular contractility, rest-
Department of Surgery, Division of Transplant lessness or coma, seizures, cramping, and
Seattle, WA, USA even rhabdomyolysis with consequent acute
e-mail: [email protected] renal failure [4]. In chronic hypernatremia,
A. A. S. Dick (*) there has been time for the brain to adapt with
Department of Surgery, Section of Pediatric solute to restore cell water volume, so aggres-
Transplantation, Seattle Children’s Hospital and sive correction of the hypernatremia with
University of Washington, Seattle, WA, USA hypotonic fluid does not address the

h yperosmolarity in the brain and can conse- convulsions, the serum sodium concentration
quently cause cerebral edema leading to should be reduced at a maximal rate of
coma, convulsions, and death [2]. 0.5 mmol per liter per hour, with a target of
B. In treating hypernatremia, both the overall 10 mmol per liter per day [2].
state of hypertonicity and the underlying etiol- E. When the patient is hypovolemic, the patient
ogy causing the disorder must be corrected. should be resuscitated with isotonic solutions
Depending on the cause, this may entail con- prior to attempting to correct the hypernatre-
trolling pyrexia, hyperglycemia, and glucos- mia. In cases where the patient is euvolemic
uria, effectively managing gastrointestinal or hypervolemic, loop diuretics may also be
secretions, withholding lactulose and diuret- used in combination with 5% dextrose in
ics, or correcting the feeding of the patient [2]. water to induce natriuresis [4]. The volume of
C. Acute hypernatremia, which develops over hypotonic saline that is needed to reduce the
the course of hours, in the setting of severe serum sodium concentration to a given target
neurological symptoms or a sodium level level may be calculated, but ongoing fluid
above 160 mmol/L, should be considered a losses may be unpredictable and greatly influ-
medical emergency. The patient should be ence the rate of correction. These losses as
administered 5% dextrose in water intrave- well as the patient’s electrolytes and glucose
nously with the goal of normalizing the serum levels must be monitored and addressed
sodium level within 24 h. appropriately, with the serum sodium levels
D. In cases of chronic hypernatremia, where
being checked every 4-6 h [3].
rapid correction may lead to brain edema and
183 Hypernatremia 763
Patient found to be hypernatremic on

inpatient laboratory results
A Review history and physical for signs and symptoms of

hypernatremia and to help identify underlying etiology
B Make changes to patient management to prevent further hypernatremia
Assess whether hypernatremia is acute or chronic in nature
C D
Patient with acute (<48h) hypernatremia with

severe neurological symptoms or a sodium level Patient with chronic (>48h) hypernatremia
above 160 mmol/L
Administer 5% dextrose in water IV

Administer 5% dextrose in water IV Reduce serum sodium concentration at a maximal
Goal of normalizing serum sodium within 24hrs rate of 0.5 mmol per liter per hour
Target reduction of 10 mmol per liter per day
Is the patient Consider the addition of loop diuretics for natriuresis,

hypovolemic? particularly if hypervolemic
Resuscitate with balanced crystalloid prior

to attempting to correct hypernatremia
Algorithm 183.1
References 3. Rondon-Berrios H, Argyropoulos C, Ing TS,

Raj DS, Malhotra D, Agaba EI, Rohrscheib M,
Khitan ZJ, Murata GH, Shapiro JI, Tzamaloukas
1. Alshayeb HM, Showkat A, Babar F, Mangold T,
AH. Hypertonicity: clinical entities, manifestations
Wall BM. Severe hypernatremia correction rate and
and treatment. World J Nephrol. 2017;6(1):1–11.
mortality in hospitalized patients. Am J Med Sci.
4. Lindner G, Funk GC. Hypernatremia in critically
2011;341(5):356–60.
ill patients. J Crit Care. 2013;28(2):216.e11. Epub
2. Adrogué HJ, Madias NE. Hypernatremia. N Engl J
2012 Jul 2.
Med. 2000;342(20):1493–9.
Hypokalemia
184
Algorithmic Approach rhabdomyolosis [2, 3]. Dysrhythmias can

also be seen, and classic findings on electro-
A. Causes of hypokalemia are multifactorial.
cardiogram demonstrate ST segment depres-
They generally include redistribution of sion, prolongation of QT interval, flattened T
potassium into the intracellular space, gastro- waves, and prominent U waves.
intestinal losses, or excessive renal losses. C. The primary goal of treatment is to replete
Shifting of potassium into the intracellular potassium in order to prevent life-threatening
space can be secondary to acute alkalosis, dysrhythmias and muscle weakness leading to
administration of insulin and glucose (both paralysis or rhabdomyolysis. Treatment is
treatments for hyperkalemia), or response to based on the underlying causes of hypokale-
catecholamines [1]. Hypokalemia can also mia. Redistributive hypokalemia may be asso-
result from increased gastrointestinal losses ciated with hypomagnesemia. In this scenario,
in the setting of diarrhea, vomiting, or mucous hypokalemia treatment with potassium
secreting colonic tumors such as villous ade- replacement alone may be refractory until
nomas. Excessive renal losses of potassium magnesium is appropriately replaced.
leading to hypokalemia are most commonly Redistributive hypokalemia associated with
seen with diuretic therapy [1]. increased catecholamine levels can be treated
B. Generally, patients do not become symptom- with administration of nonselective beta-
atic until potassium levels fall below blocker. Patients undergoing diuretic therapy
3.0 mEq/L. Clinical manifestations of hypo- may need to be supplemented with oral potas-
kalemia include muscle weakness progress- sium or use a potassium-sparing diuretic;
ing to paralysis and in extreme cases however, this treatment regimen should be
used with caution in patients with chronic kid-
ney disease. Patients who are symptomatic
K. W. Shaw (*) with dysrhythmias, muscle weakness, or rhab-
Department of Surgery, Division of Transplant domyolysis need urgent correction of hypoka-
Seattle, WA, USA lemia. This can be achieved orally in doses of
e-mail: [email protected] 40 mEq three to four times per day or intrave-
A. A. S. Dick (*) nously 20 mEq every 2–3 h [4]. These patients
Department of Surgery, Section of Pediatric will require careful monitoring until symp-
Transplantation, Seattle Children’s Hospital and toms have resolved. Care should be taken to
University of Washington, Seattle, WA, USA avoid rebound hyperkalemia.

Patient found to be hypokalemic on

laboratory results
A Consider potential etiologies of hypokalemia, including acute

alkalosis, administration of insulin and glucose, response to
catecholamines, increased GI losses, or excessive renal loss
Evaluate for manifestations of hypokalemia, including muscle

B weakness, check for dysrhythmias with and EKG, and rule out
rhabdomyolysis if the case is severe
C Replete potassium, either PO or IV, depending on the clinical

condition of the patient, addressing the underlying etiology
Algorithm 184.1
3. Shintani S, Shiigai T, Tsukagoshi H. Marked hypo-

References kalemic rhabdomyolysis with myoglobinuria due to
diuretic treatment. Eur Neurol. 1991;31:396.
1. Gennari FJ. Hypokalemia. N Engl J Med. 4. Pullen H, Doig A, Lambie AT. Intensive intravenous
1998;339(7):451–8. potassium replacement therapy. Lancet. 1967;2:809.
2. Comi G, Testa D, Cornelio T, et al. Potassium deple-
tion myopathy: a clinical and morphological study of
six cases. Muscle Nerve. 1985;8:17.
Hyperkalemia
185
Algorithmic Approach progressing to paralysis. Classic electrocar-

diogram changes associated with hyperkale-
Causes The major intracellular cation is potas- mia include peaked T-waves and widened
sium, and it determines intracellular osmolality. QRS that progresses to a sine wave [2]. These
Alterations in the potassium concentration gradi- changes may lead to ventricular fibrillation
ent have profound effects on transmembrane and arrest.
potential and subsequently cellular function. B. Multimodal therapies are needed for definitive
Hyperkalemia is a common clinical problem and management to urgently correct severe hyper-
rarely develops from excessive potassium intake kalemia. The goal is to reduce the effect of
in the absence of renal insufficiency. It most often hyperkalemia on membrane potential, shifting
occurs as a result of impaired urinary excretion potassium intracellularly and increasing
due to acute kidney injury, chronic kidney dis- potassium excretion. These patients should be
ease, or redistribution of potassium [1]. Examples placed under continuous cardiac monitoring
of redistributive hyperkalemia include crush inju- with serial electrolyte evaluations. Therapy
ries, massive transfusion, reperfusion of ischemic should include rapid administration of intra-
limbs, and diabetic ketoacidosis. venous calcium, which antagonizes the mem-
brane potential influence of hyperkalemia.
A. Clinical manifestations of hyperkalemia
Calcium can be given in the form of calcium
which require urgent treatment include car- gluconate or calcium chloride [3]. This effect
diac conduction abnormalities and neuromus- is transient and as a result should not be
cular abnormalities, such as weakness administered as monotherapy. Shifting potas-
sium from the extracellular to the intracellular
space can be achieved with infusion of sodium
bicarbonate or insulin with glucose [4].
K. W. Shaw (*) Sodium bicarbonate antagonizes the effects of
Department of Surgery, Division of Transplant hyperkalemia on the membrane potential,
Seattle, WA, USA while the increase in extracellular pH redis-
e-mail: [email protected] tributes potassium into the intracellular space.
A. A. S. Dick (*) Hydrogen is exchanged for potassium to
Department of Surgery, Section of Pediatric maintain electroneutrality. Insulin achieves
Transplantation, Seattle Children’s Hospital and the same redistribution of potassium from the
University of Washington, Seattle, WA, USA extracellular to the intracellular space.

Glucose has to be given concurrently to pre- In summary the urgency of hyperkalemic treat-
vent hypoglycemia. These effects are also ment is based on the severity of the signs and
transient and may need to be repeated with symptoms of hyperkalemia. In severe cases
serial laboratory evaluations. Definitive man- antagonizing the membrane potential and redis-
agement requires increasing potassium excre- tribution of potassium to the intracellular space
tion, and this can be achieved via loop are temporary first-line management until defini-
diuretics, administration of Na+-K+ resins tive management with exchange resins or dialy-
such as Kayexalate, and dialysis [5]. In sis is available to excrete excess potassium from
patients with normal renal function, loop the extracellular space.
diuretics increases potassium excretion in the
urine. In patients with compromised renal
function, dialysis is indicated.
185 Hyperkalemia 769
A Does the patient have clinical

manifestations of hyperkalemia?
B yes No
yes
Treat with rapid acting therapies: Potassium > 6.5 meq/L
· Intravenous Calcium
· Insulin & Glucose
· Sodium bicarbonate No
· Diuretics
If renal impairment:
· All of the above±diuretics Potassium > 5.5 meq/L and
· Dialysis renal impairment (ESRD or
oliguria)
· Cation exchange binders
Potassium should be lowered

with cation exchange binders
and dialysis
Patient should be monitored throughout

this process
Algorithm 185.1
3. Weisberg LS. Management of severe hyperkalemia.

References Crit Care Med. 2008;36:3246–51.
4. Harel Z, Kamel KS. Optimal dose and method of
1. Palaka E, Leonard S, Buchanan-Hughes A, Langford administration of intravenous insulin in the manage-
B, Grandy S. Evidence in support of hyperkalaemia ment of emergency hyperkalemia: a systemic review.
management strategies: a systematic literature review. PLoS One. 2016;11:e0154963.
Int J Clin Pract. 2018;72:e13052. 5. Sterns RH, Rojas M, Bernstein P, Chennupati S. Ion-
2. Montague BT, Ouellette JR, Buller GK. Retrospective exchange resins for the treatment of hyperkalemia:
review of the frequency of ECG changes in hyperka- are they safe and effective? J Am Soc Nephrol.
lemia. Clin J Am Soc Nephrol. 2008;3:324–30. 2010;21:733–75.
Management of Hypocalcemia
186
Algorithmic Approach levels directly measure unbound calcium and

do not require correction.
A. The first step in evaluating a patient with
C. Physical exam findings encountered in hypo-
hypocalcemia is a thorough history and phys- calcemia include paresthesias, carpopedal
ical examination. Certain aspects of the his- spasm, neuromuscular irritability, Chvostek’s
tory may indicate specific etiologies, such as (contraction of facial muscles elicited by tap-
a history of chronic kidney disease, acute ping on the facial nerve) and Trousseau’s
pancreatitis, or hyper-catabolic states such as (carpopedal spasm induced by BP cuff
rhabdomyolysis or tumor lysis syndrome. A inflated for 3 min) signs, and, if chronic or
history of neck surgery or radiation may indi- severe, disorientation, seizures, cardiac dys-
cate iatrogenic hypoparathyroidism, while rhythmia, impaired cardiac contractility, and
history of autoimmune disorders may indi- QT prolongation.
cate autoimmune hypoparathyroidism. Many D. Serum intact parathyroid hormone (PTH)

medications can lower serum calcium levels should be measured in all patients with hypo-
(e.g., calcium chelators [citrate, phosphate, calcemia [1]. Low or normal PTH indicates
EDTA], bone resorption inhibitors [bisphos- hypoparathyroidism or hypomagnesemia.
phonates, calcitonin], cinacalcet, foscarnet, Elevated PTH indicates renal losses, vitamin
and phenytoin). D deficiency, or pseudohypoparathyroidism.
B. Serum calcium is largely bound to proteins, Hypomagnesemia can induce PTH resistance
primarily albumin; thus, free calcium concen- or deficiency, while hypermagnesemia is con-
trations will not be accurately reflected by sistent with renal losses. Hypophosphatemia
serum calcium measurements in patients with indicates secondary hyperparathyroidism or
derangements in serum albumin. This can be low dietary intake (rare in developed coun-
corrected using the following formula: tries), while hyperphosphatemia can occur
Ca(corrected) = Ca(serum) + [0.8*(normal with chronic kidney disease, hypercatabolic
albumin – Serum albumin)]. Ionized calcium states, hypoparathyroidism, or pseudohypo-
parathyroidism. Low 25-hydroxyvitamin D
levels indicate deficient absorption or dietary
R. S. Schoaps · S. W. Hazard III (*) intake of vitamin D, while low
Department of Anesthesiology and Perioperative 1,25-dihydroxyvitamin D levels indicate
Medicine, Penn State Hershey Medical Center, hypoparathyroidism or renal insufficiency
Hershey, PA, USA (when coupled with hyperphosphatemia).

E. Hypomagnesemia should be corrected with temia prior to calcium repletion. Patients with
2.0 g magnesium sulfate prior to replacing vitamin D deficiency should have concurrent
calcium to prevent ongoing PTH resistance. vitamin D supplementation.
Severe hyperphosphatemia, such as is seen in F. Intravenous calcium replacement is indicated
tumor lysis syndrome, should be corrected in the setting of symptomatic hypocalcemia,
with fluids and phosphate restriction prior to prolonged QTc interval, or inability to take
administration of IV calcium to prevent in situ oral supplements, administered as 1–2 g over
formation of calcium-phosphate precipitates 10–20 min. Asymptomatic hypocalcemia
[2]. In the setting of renal failure, hemodialy- may be replaced with 1500–2000 mg elemen-
sis may be indicated to correct hyperphospha- tal calcium divided daily.
186 Management of Hypocalcemia 773
A History & physical exam

Medications? CKD? Pancreatitis? Hypercatabolic state?
Confirm with repeated testing

B Serum calcium corrected for albumin vs. ionized calcium
Obtain blood work and perform a physical examination

C Paresthesias, Carpopedal spasm, Chvostek’s and
Trousseau’s signs
Serum intact Other

Serum Serum Vitamin D
D parathyroid
magnesium phosphorous metabolites
(creatinine, alkaline
hormone (PTH) phosphatase,
amylase/lipase,
urinary calcium)
E
Correct prior to replacing calcium
Symptomatic?
Yes No
F IV Calcium PO Calcium
Algorithm 186.1
2. Coiffier B, et al. Guidelines for the management of

References pediatric and adult tumor lysis syndrome: an evidence-
based review. J Clin Oncol. 2008;26(16):2767–78.

1. Goltzman D, Cole DEC. “Hypoparathyroidism.”
Primer on the metabolic bone diseases and disorders of
bone metabolism. 6th ed. Washington, DC: American
Society of Bone and Mineral Research; 2006. p. 216.
Management of Hypercalcemia
187
Algorithmic Approach nausea, anorexia), or neuropsychiatric

disturbances (cognitive dysfunction, lethargy,
coma).
hypercalcemia is a thorough history and C. Serum calcium is largely bound to proteins,
physical examination. Hyperparathyroidism primarily albumin; thus, free calcium concen-
and malignancy account for >90% of cases of trations will not be accurately reflected by
hypercalcemia [1]. Certain aspects of the his- serum calcium measurements in patients with
tory, however, may indicate other specific eti- serum albumin derangements. This can be
ologies, such as prolonged immobilization, a corrected using the following formula:
long-standing history of chronic kidney dis- Ca(corrected) = Ca(serum) + [0.8*(normal
ease, granulomatous disease such as sarcoid- albumin – serum albumin)]. Ionized calcium
osis or tuberculosis, and review of medications levels directly measure unbound calcium and
and supplements (e.g., calcium supplements, do not require correction.
vitamin A and D supplements, lithium, thia- D. The first step in work-up after confirming
zide diuretics, theophylline). hypercalcemia is measuring serum intact
B. Signs and symptoms attributable to hypercal- parathyroid hormone (PTH) [2]. Elevated
cemia vary based on acuity and severity. Mild PTH indicates either primary hyperparathy-
or chronic hypercalcemia may be asymptom- roidism (the most common cause of hypercal-
atic or associated with relatively mild symp- cemia in the outpatient setting, typically
toms such as constipation, fatigue, or manifested by corrected serum calcium
depression. Acute or severe elevations in <11 mg/dL and mild symptoms) or familial
serum calcium (>14 mg/dL) may be associ- hypocalciuric hypercalcemia (FHH, a rare
ated with more severe symptoms, such as genetic condition). If PTH is low, the next
renal dysfunction (nephrolithiasis, renal tubu- step in diagnosis is measuring parathyroid
lar acidosis), nephrogenic diabetes insipidus hormone-related peptide (PTHrp, elevation
(polydipsia, polyuria, dehydration), gastroin- associated with various malignancies) and
testinal disturbances (severe constipation, vitamin D metabolites. Elevation of
25-hydroxyvitamin D is associated with
R. S. Schoaps · S. W. Hazard III (*) hypervitaminosis D, while elevation of
Department of Anesthesiology and Perioperative 1,25-dihydroxyvitamin D is associated with
Medicine, Penn State Hershey Medical Center, granulomatous disease and lymphoma.
Hershey, PA, USA

E. If PTH, PTHrp, and vitamin D metabolites H. The first step in treatment is ensuring
are all low or normal, proceed with further euvolemia with isotonic crystalloid intrave-
testing: thyroid stimulating hormone (TSH, nous fluids to increase urinary calcium excre-
15–20% of patients with hyperthyroidism tion. Loop diuretics are generally not
exhibit thyroid-mediated increase in bone recommended as additional metabolic
resorption), serum vitamin A (hypervitamin- derangements should be avoided but may be
osis A leads to increased bone resorption), necessary in the setting of renal insufficiency
urinary calcium excretion (reduced in milk- or heart failure.
alkali syndrome, thiazide diuretic use, and I. Initial medical therapy is aimed at inhibition
FHH; elevated in hyperparathyroidism), and of bone resorption with the use of calcitonin
serum/urinary protein electrophoresis (multi- (4 IU/kg q6h) and bisphosphonates.
ple myeloma results in increased bone resorp- Zoledronic acid and pamidronate are the
tion). Because malignancy is the most agents of choice in hypercalcemia of malig-
common cause of hypercalcemia in the inpa- nancy. Denosumab is an emerging treatment
tient setting, additional work-up for occult option for patients with severe renal failure
neoplasms or granulomas may be indicated or other contraindications for bisphospho-
(e.g., chest radiograph, bone survey, CT nates, although clinical data to support its
scans, etc.). use is limited.
F. Patients who are asymptomatic or exhibit only J. Etiology-specific therapy is aimed at pre-
mild symptoms (e.g., constipation) may often venting recurrence after normalization of
be treated with avoidance of inciting factors serum calcium levels. Dietary calcium
(e.g., dehydration, supplements, medications) restriction is often useful, especially in the
or by treatment of the causative condition. setting of lymphoma, sarcoidosis, or other
Immediate treatment is indicated for patients granulomatous diseases. Glucocorticoids
with severe symptoms, an acute increase in (prednisone 20–40 mg daily) decrease
corrected serum calcium levels to >12 mg/dL, intestinal calcium absorption and may be
or corrected serum calcium >14 mg/dL. used in the setting of hypervitaminosis D or
G. Patients with severe elevations (>18 mg/dL), chronic granulomatous disease [4]. In
neurologic symptoms (lethargy, coma), or patients with parathyroid carcinoma and
renal failure should undergo hemodialysis. secondary hyperparathyroidism in the set-
Hemodialysis may also be indicated in ting of renal failure, cinacalcet is used to
patients with severe heart failure who cannot agonize calcium-sensing receptors and
tolerate IV hydration [3]. inhibit PTH secretion.
187 Management of Hypercalcemia 777
A History and physical exam

Medications/supplements? Malignancy? CKD? Granulomatous disease?
Signs/symptoms: constipation, fatigue, renal dysfunction, cognitive dysfunction
B
Confirm with repeated testing

C Serum calcium corrected for albumin vs. ionized calcium
Additional lab work, start with: intact parathyroid

D hormone (PTH), parathyroid hormone related peptide
(PTHrp), and vitamin D metabolites
Based on results in conjunction with clinical presentation,

E consider: TSH, serum vitamin A, serum & urinary protein
electrophoresis, urinary calcium excretion, imaging
F Symptomatic?
Yes
No
Neurologic
symptoms or
renal failure?
Avoid inciting No Yes G
factors, dietary
restriction,
etiology-specific
intervention H Crystalloid IVF Hemodialysis
Calcitonin +
I Bisphosphonate
J Etiology-specific
therapy
Algorithm 187.1
References 3. Koo WS, et al. Calcium-free hemodialysis for

the management of hypercalcemia. Nephron.
1996;72(3):424.
1. Lafferty FW. Differential diagnosis of hypercalcemia.
4. Sandler LM, et al. Studies of the hypercalcemia of sar-
J Bone Miner Res. 1991;6(S2):S51–9.
coidosis: effect of steroids and exogenous vitamin D3
2. Ratcliffe WA, et al. Role of assays for parathyroid-
on the circulating concentrations of 1,25-dihydroxy
hormone-related protein in investigation of hypercal-
vitamin D3. Q J Med. 1984;53(210):165–80.
cemia. Lancet. 1992;339(8786):164–7.
Paradoxical Aciduria
188
Algorithmic Approach ference (SID = [Na+] + [K+] + [Ca+2] + [Mg+

] – [Cl−] – [HCO3−]) [1]. In hypochloremic
hypokalemic metabolic alkalosis, due to
paradoxical aciduria is a thorough history and excessive gastric strong ion losses, the strong
physical examination. Paradoxical aciduria ion difference will be >40.
occurs most commonly as a result of exces- C. Urinary electrolytes vary as the kidney transi-
sive gastric losses via vomiting, suction of tions from acutely alkalotic urine production
gastric contents, or a proximal fistula. As to paradoxical aciduria. In the acute alkalotic
large volumes of gastric contents are lost, phase (urine pH > 6.5), urine Na, K, and
there is depletion of hydrochloric acid (HCl) HCO3 are elevated, with depressed Cl. As the
and extracellular fluid, producing a hypochlo- kidneys attempt to correct the strong iron dif-
remic hypokalemic metabolic alkalosis with ference, urine becomes paradoxically acidic
an increased strong ion difference. Persistent (pH < 5.5) with low concentrations of Na, K,
hypovolemia induces the renin-angiotensin- Cl, and HCO3.
aldosterone axis, stimulating retention of D. Initial therapy should be aimed at volume
sodium (Na) and water via Na-H exchangers expansion and replacing depleted strong ions,
in the proximal tubule and collecting duct initially with normal sterile saline (NSS)
sodium channels. Hypokalemic alkalosis infusion and potassium chloride (KCl). Aim
stimulates potassium (K) retention via H-K- to replace the volume deficit with NSS, fol-
ATPase and maximal bicarbonate (HCO3) lowed by an infusion rate adequate to replace
reabsorption to maintain electroneutrality, ongoing losses plus an additional 100 mL/h.
resulting in paradoxical acidification of urine. If profoundly hypovolemic, start with 20 mL/
The alkalemia produced in this setting can kg bolus NSS and then infusion rate of 4 mL/
only be corrected by replacement of the kg/h, and titrate to replace losses plus
depleted strong ions: Na, Cl, and K [1]. 100 mL/h. Add KCl replacement to goal

B. Confirm metabolic alkalosis with arterial K > 3.5 mmol/L.
blood gas and calculate plasma strong ion dif- E. If indicated, reduce gastric HCl secretion
with H2 blockers or proton pump inhibitors.
R. S. Schoaps · S. W. Hazard III (*) Limit alkalotic solutions as much as possible
Department of Anesthesiology and Perioperative (e.g., solutions containing HCO3, citrate, and
Medicine, Penn State Hershey Medical Center, lactate).
Hershey, PA, USA

F. Trend labs to normal values for all plasma alkalinize (pH > 7) [2]. The plasma pH will
strong ions and aim for normal volume status. follow as the strong ion difference is
As renal fluid and chloride delivery increase, corrected.
Cl-HCO3 exchangers will promote HCO3 G. Treat the underlying etiology for definitive
secretion in the collecting duct, and urine will resolution.
History & physical exam

A Vomiting? Gastric outlet obstruction? Fistula?
Arterial blood gas, serum electrolytes

B Calculate strong ion difference
Urine pH & electrolytes

C pH < 5.5, UNa < 10, UK < 20, UCl < 10, UHCO3 < 15
Volume Expansion with NSS (losses + 100mL/h)

D KCl replacement to K > 3.5
Reduce gastric HCl secretion (H2-blocker,

E PPI) Limit administration of alkalotic solutions
Goal of intravascular euvolemia Trend labs

F to normal plasma values and follow urine pH
G Treat underlying etiology
Algorithm 188.1
2. Luke RG, Galla JH. It is chloride depletion a lkalosis,

References not contraction alkalosis. J Am Soc Nephrol.
2012;23(2):204–7.
1. Seifter JL. Integration of acid-base and electrolyte
disorders. N Engl J Med. 2014;371(19):1821–31.
Part XXII
Hernia
Inguinal Hernia
189
Algorithmic Approach C. Ultrasound may be helpful when no palpable

defect or bulge is detected on exam in a
A. The first step in the evaluation of a patient symptomatic patient. While the reported
with an inguinal hernia is a thorough history. diagnostic sensitivity and specificity of ultra-
The most common symptom is a groin bulge sound reach upwards of 97.6% and 99.8%,
that is worse with straining (urination, defe- respectively, it is highly operator dependent
cation, or lifting), exercise, or prolonged [2]. When there is clinical uncertainty, com-
periods of standing. The patient may or may puted tomography (CT) or magnetic reso-
not complain of symptoms of pain or dis- nance imaging (MRI) may provide a more
comfort. Associated pain, gastrointestinal or consistent and detailed view of the groin
urinary dysfunction should raise concern for anatomy. If an inguinal hernia is not detected
intermittent incarceration. on cross-sectional imaging, other differential
B. Clinical examination of the groin is the gold diagnoses for groin bulge or discomfort
standard of hernia diagnostics, with sensitiv- should be pursued.
ity and specificity of 74.5% and 96.3%, D. Once the diagnosis of inguinal hernia is
respectively [1]. The groin area should be established, either by physical exam, imag-
observed for evidence of bulging or asymme- ing, or both, the next step is to assess how
try with coughing. Then, the tip of the exam- symptomatic the patient is.
iner’s index finger is invaginated into the E. If the hernia is asymptomatic or minimally
external ring to palpate for a bulge or mass symptomatic, watchful waiting is a safe and
while the patient coughs or performs a reasonable alternative in male patients.
Valsalva maneuver. However, the natural history is progressive
enlargement and an intervention will likely
be needed eventually [3]. In women, repair is
advisable due to higher incidence of femoral
Q. L. Hu hernias and hernia adverse events [4].
Department of Surgery, David Geffen School of
Medicine at University of California, Los Angeles, F. For symptomatic inguinal hernias, the next
Los Angeles, CA, USA step is to establish the reducibility of the her-
D. C. Chen (*) nia. If the mass is incarcerated, it is important
Department of Surgery, Lichtenstein Amid Hernia to assess for signs of strangulation (fever,
Clinic at University of California at Los Angeles, tenderness, erythema, or overlying skin
Los Angeles, CA, USA changes). If strangulation is suspected, blood

784 Q. L. Hu and D. C. Chen
work such as white blood cell count and lac- ally and inguinal ligament laterally. The
tate can be informative. mesh is split and wrapped around the sper-
G. If the hernia is reducible, elective repair in an matic cord and then the tails sutured together
outpatient setting is recommended. to recreate the inguinal ring. The advantages
H. If the hernia is incarcerated, but not strangu- of this technique are that it can be performed
lated, manual reduction may be attempted. with local anesthesia in the outpatient setting
Sedation, Trendelenburg positioning, and ice and is associated with low cost and low
over the groin may be helpful. If the hernia is recurrence rates.
reduced, the patient maybe scheduled for L. Although primary tissue repairs have been
elective or urgent repair given the risk for re- largely abandoned owing to the success of
incarceration and strangulation. If the hernia mesh-based repairs, they may be useful in
cannot be reduced manually, surgery is small hernias, young patients, or circum-
indicated. stances where there is a contraindication for
I. A strangulated inguinal hernia is a surgical mesh placement (strangulated hernia with
emergency. Preoperatively, the patient should bowel ischemia and perforation or contami-
be optimized with IV hydration, electrolyte nated field or patient refusal). Options for tis-
correction, nasogastric tube decompression, sue repairs include Shouldice, Bassini, and
and IV antibiotics when possible. McVay repairs. The best available tissue-
J. There are many options for surgical inguinal based technique is the Shouldice repair,
hernia repair and should be individualized to which is a four-layer imbricated repair of the
the patient’s and the surgeon’s preference posterior wall of the inguinal canal with run-
and level of experience. The open anterior ning sutures. In specialized centers, its effi-
tension-free mesh approach remains the gold cacy is similar to mesh-based repairs.
standard, but minimally invasive (laparo- M. The open posterior approach uses a trans-
scopic and robotic) techniques have become verse skin incision above the traditional ante-
highly effective, standardized, and safe in rior incision. The preperitoneal space may be
recent decades and have been found to be entered using either a transinguinal preperi-
associated with less postoperative pain and toneal (TIPP) or trans-rectus sheath extra-
numbness, reduced recovery time, and the peritoneal (TREPP) technique. The
ability to treat bilateral hernias through the preperitoneal space is then dissected and the
same incision [5]. However, operative times myopectineal orifice exposed. A prosthetic
are longer, and there is a higher risk of rare mesh is used to cover the entire myopectineal
serious complications such as visceral and orifice. In bilateral hernias, a lower midline
vascular injuries [5]. Recurrence rates or Pfannenstiel incision may be used to
between open mesh-based repairs and lapa- address both sides (Stoppa repair).
roscopic repairs are similar [5]. Given the N. In the totally extraperitoneal (TEP) tech-
learning curve associated with laparoscopic nique, a specialized dissecting balloon is
repairs, use of these techniques depends on passed along the posterior rectus sheath and
the availability of surgical expertise. In is used to dissect the preperitoneal space. The
women, the operation of choice is laparo- hernia sac is dissected from the cord and
scopic or open pre-peritoneal repair given the reduced. A prosthetic mesh is then used to
higher risk of concurrent femoral hernia. cover the entire myopectineal orifice. The
K. The open anterior tension-free mesh tech- advantage of this technique is that it avoids
nique (modified Lichtenstein repair) is the violation of the peritoneum.
most common technique for inguinal hernias. O. In the transabdominal preperitoneal (TAPP)
In this technique, the apex of the prosthetic approach, the peritoneal cavity is entered
mesh is fixed to the pubic tubercle and the first. Then, peritoneum is incised and a flap is
mesh is sutured to the conjoint tendon medi- created to enter to preperitoneal space. Once
189 Inguinal Hernia 785
in the preperitoneal space, the technique is sible to perform bowel resection and anasto-
similar to the TEP approach. At the end of the mosis through the inguinal incisions but
procedure, the mesh is covered with the peri- there should be a low threshold for convert-
toneum. The advantage of this technique is ing to a midline incision. An open preperito-
that is allows for easy visual inspection of the neal (posterior) approach may also be used.
contralateral side and intra-abdominal The advantage of this incision is ability to
organs. However, the disadvantage of enter- convert to laparotomy without creating a sep-
ing the peritoneal cavity is that it exposes the arate incision. Finally, if surgical expertise is
patient to potential intra-abdominal injury available, a laparoscopic or hybrid laparo-
and adhesion formation. scopic approach may be used to address the
P. Emergent repair for strangulated hernia may bowel. Due to concern for infection, mesh is
be performed through the standard anterior not recommended in the repair of strangu-
inguinal incision. The hernia sac is dissected lated inguinal hernia. From an anterior
and controlled at the base to prevent drop- approach, a tissue-based repair, such as the
ping the contents into the abdominal cavity. Shouldice repair, may be performed. In an
The sac should be opened under direct vision open posterior or laparoscopic approach, a
and all contents inspected carefully. It is pos- delayed mesh-based repair is recommended.
Groin bulge
A Pain/vague discomfort
B Perform a physical examination
Palpable bulge No
or defect?
Imaging C
Yes
D
Asymptomatic inguinal No Yes Inguinal hernia

E hernia
Symptomatic?
on imaging?
Yes No
Watchful waiting Symptomatic inguinal hernia No inguinal hernia. Consider

other differential diagnoses.
F
Reducible?
G Reducible H Incarcerated Strangulated I
Yes Manually No
Elective/urgent repair
reducible?
Emergent repair P
Operative approach depends on surgeon expertise and

J surgeon/patient preference
Laparoscopic/robotic approach Open approach
N TEP O TAPP Anterior approach Posterior approach M
K Mesh-based repair Tissue-based repair L

Algorithm 189.1
189 Inguinal Hernia 787
References 3. Ramanan B, Maloley BJ, Fitzgibbons RJ Jr. Inguinal

hernia: follow or repair? Adv Surg. 2014;48:1–11.
4. Koch A, Edwards A, Haapaniemi S, Nordin P, Kald
1. van den Berg JC, de Valois JC, Go PM, Rosenbusch
A. Prospective evaluation of 6895 groin hernia repairs
G. Detection of groin hernia with physical examina-
in women. Br J Surg. 2005;92(12):1553–8.
tion, ultrasound, and MRI compared with laparo-
5. McCormack K, Scott NW, Go PM, Ross S, Grant
scopic findings. Investig Radiol. 1999;34(12):739–43.
AM. Laparoscopic techniques versus open techniques
2. Niebuhr H, Konig A, Pawlak M, Sailer M, Kockerling
for inguinal hernia repair. Cochrane Database Syst
F, Reinpold W. Groin hernia diagnostics: dynamic
Rev. 2003;(1):CD001785.
inguinal ultrasound (DIUS). Langenbeck’s Arch
Surg. 2017;402(7):1039–45.
Recurrent Inguinal Hernia
190
Algorithmic Approach However, in the recurrent inguinal hernia,

physical exam may not always be
A. Inguinal hernia recurrence rates are report- diagnostic.
edly between 1% and 40% depending on the C. When the physical exam findings are equivo-
type of initial repair technique [1]. Similar to cal, imaging modalities may be helpful.
a primary inguinal hernia, the first step in the Ultrasound should still be used as the initial
evaluation of a patient with a recurrent ingui- modality as it is an inexpensive and effective
nal hernia is a thorough history. The patient tool, but its sensitivity is lower in recurrent
may present with a groin bulge at the site of a compared to primary inguinal hernias. When
prior hernia repair. More commonly, the pre- the ultrasound is non-diagnostic, cross-
senting symptom of a recurrent inguinal her- sectional imaging such as computed tomog-
nia is pain or discomfort. It is important to raphy (CT) or magnetic resonance imaging
assess for risks of recurrence, including prior (MRI) may provide a more consistent and
repair technique, factors that contribute to detailed view of the groin anatomy. If an
poor healing (immunosuppression, diabetes, inguinal hernia is not detected on cross-
infection, smoking, and obesity), and genet- sectional imaging, other differential diagno-
ics (collagen synthesis disorders). ses should be pursued. It is especially
B. Physical exam remains the initial method in important to differentiate chronic groin pain
the diagnosis of a recurrent inguinal hernia. from recurrence.
A bulge or mass can be palpated for by D. Once the diagnosis of recurrent inguinal her-
invaginating the tip of the examiner’s index nia is established, either by physical exam,
finger into the external ring while the patient imaging, or both, the next step is to assess
coughs or performs a Valsalva maneuver. how symptomatic the patient is.
E. Because redo hernia repairs are associated
with higher risk of complications and recur-
Q. L. Hu rence rates and the risk of strangulation is
Medicine at University of California, Los Angeles, low, watchful waiting is a reasonable treat-
Los Angeles, CA, USA ment approach in the asymptomatic or mini-
D. C. Chen (*) mally symptomatic patient [2].
Department of Surgery, Lichtenstein Amid Hernia F. For symptomatic inguinal hernias, the next
Clinic at University of California at Los Angeles, step is to establish the reducibility of the her-
Los Angeles, CA, USA nia. If the mass is incarcerated, it is important

to assess for signs of strangulation (fever, approach is appropriate as well [3]. Either
tenderness, erythema, or overlying skin the totally extraperitoneal (TEP) or transab-
changes). If strangulation is suspected, blood dominal preperitoneal (TAPP) technique
work such as white blood cell count or lactate may be used [4]. In TEP, a specialized bal-
can be informative. loon is passed along the posterior rectus
G. If the hernia is incarcerated but not strangu- sheath and is used to dissect the preperitoneal
lated, manual reduction may be attempted. If space. The hernia sac is reduced and a pros-
the hernia cannot be reduced manually or thetic mesh is used to cover the entire myo-
there is evidence of strangulation, then emer- pectineal orifice. The TAPP technique is
gent repair is indicated. The approach to performed in the same manner except that
emergent repair is similar to that of the emer- the peritoneal cavity is first entered and then
gent primary hernia repair and depends on the peritoneum is incised to enter the preperi-
surgeon preference, experience, and toneal space.
expertise. J. If the prior repair was a mesh-based repair,
H. If the hernia is reducible, elective repair in an the redo operation technique depends on the
outpatient setting is recommended. The oper- original approach.
ative repair technique for a recurrent inguinal K. If the original repair was performed using an
hernia depends on the prior technique of open anterior approach, a posterior approach
repair and surgeon expertise. is advised for the redo operation given lower
I. If the prior repair was a primary tissue repair, complication rates and the ability to operate
the redo operation should be a mesh-based in the non-scarred field. Depending on sur-
repair if not otherwise contraindicated and geon expertise, either an open posterior
may be approached from either the open (TIPP, TREPP, or Stoppa repair) or laparo-
anterior or posterior approach. For open scopic approach (TEP or TAPP) may be
anterior repairs, the Lichtenstein tension-free used.
mesh repair, in which a prosthetic mesh is L. If the original repair was performed using a
used to reinforce the inguinal floor, is recom- posterior approach (either open posterior or
mended. For open posterior repairs, either laparoscopic), an open anterior approach,
transinguinal preperitoneal (TIPP) or trans- such as the Lichtenstein technique, is advis-
rectus sheath extra-peritoneal (TREPP) tech- able for the redo operation.
nique may be used to enter the preperitoneal M. If surgical expertise is available, it is rea-
space and a prosthetic mesh used to cover the sonable to attempt the redo operation lapa-
entire myopectineal orifice. If the recurrence roscopically through a transabdominal
is bilateral or if the patient has a primary her- preperitoneal (TAPP) approach. The poten-
nia on the contralateral side, a Stoppa repair tial advantage of this technique is the abil-
through a lower midline or Pfannenstiel inci- ity to assess and fix the problem from the
sion is advisable to address both sides simul- prior repair and this may be performed in
taneously. If surgical expertise is available, a conjunction with an open anterior
minimally invasive (laparoscopic or robotic) technique.
190 Recurrent Inguinal Hernia 791
Groin bulge/pain/discomfort at site of

A prior inguinal hernia repair
Palpable bulge No
or defect?
Imaging C
Yes
Yes
D
Asymptomatic No Inguinal hernia
recurrent inguinal Symptomatic?
on imaging?
hernia
Yes No
Symptomatic recurrent inguinal hernia No inguinal hernia. Consider

other differential diagnoses.
F
Reducible? Signs
E Watchful waiting
of strangulation? Incarcerated/strangulated
Reducible Manual reduction

Emergent repair
G
Elective repair H
Prior repair
technique?
Prior tissue repair Prior mesh-based repair J
Open anterior or Prior anterior approach Prior posterior/laparoscopic approach

I posterior approach
Laparoscopic approach
Open posterior or Open anterior
K laparoscopic approach approach L TAPP M
Algorithm 190.1
3. Pisanu A, Podda M, Saba A, Porceddu G, Uccheddu

References A. Meta-analysis and review of prospective random-
ized trials comparing laparoscopic and Lichtenstein
1. Barrat C, Surlin V, Bordea A, Champault techniques in recurrent inguinal hernia repair. Hernia
G. Management of recurrent inguinal hernias: a J Hernias Abdominal Wall Surg. 2015;19(3):355–66.
prospective study of 163 cases. Hernia J Hernias 4. Gass M, Scheiwiller A, Sykora M, Metzger J. TAPP
Abdominal Wall Surg. 2003;7(3):125–9. or TEP for recurrent inguinal hernia? Population-
2. Haapaniemi S, Gunnarsson U, Nordin P, Nilsson based analysis of prospective data on 1309 patients
E. Reoperation after recurrent groin hernia repair. Ann undergoing endoscopic repair for recurrent inguinal
Surg. 2001;234(1):122–6. hernia. World J Surg. 2016;40(10):2348–52.
Femoral Hernia
191
Algorithmic Approach C. Imaging can be especially helpful in identify-

ing femoral hernias when there is diagnostic
A. The presentation of a femoral hernia is very uncertainty. Ultrasound is the initial modality
similar to that of an inguinal hernia. Patients of choice and can help differentiate femoral
will complain of a groin bulge, pain, or dis- hernias from inguinal hernias or detect an
comfort. Femoral hernias are relatively occult hernia. When the ultrasound finding is
uncommon and account for 3–4% of all her- equivocal, cross-sectional imaging such as
nias [1]. They occur more frequently in computed tomography (CT) or magnetic res-
women, with incidence traditionally reported onance imaging (MRI) may provide a more
to vary between 2% in males and 7% in consistent and detailed view of the groin
females [2]. anatomy. If a hernia is not detected on cross-
B. Femoral hernias are more difficult to detect sectional imaging, other differential diagno-
on physical exam compared to inguinal her- ses should be pursued.
nias. The femoral canal is bounded by the D. Femoral hernias are often diagnosed intraop-
iliopubic tract superiorly, Cooper’s ligament eratively when patients are taken to the oper-
inferiorly, lacunar ligament medially, and ating room for a small bowel obstruction or
femoral vein laterally. On exam, the femoral concurrent inguinal hernia. If found intraop-
hernia may be found medial to the femoral eratively, it is advisable to repair the hernia
artery pulsation below the inguinal ligament. concurrently.
However, sometimes the hernia sac may pro- E. All femoral hernias should be repaired due to
trude through the femoral canal and then slide the high risk of incarceration and strangula-
up over the inguinal ligament, making it dif- tion [3]. There are many options for surgical
ficult to distinguish from an inguinal hernia. repair and should be individualized to the
patient and the surgeon’s preference and level
of experience.
Q. L. Hu (*) F. Minimally invasive (laparoscopic and
Medicine at University of California, Los Angeles, robotic) techniques are preferable when
Los Angeles, CA, USA expertise is available as it provides direct
D. C. Chen (*) access to the femoral canal and allows for
Department of Surgery, Lichtenstein Amid Hernia easy assessment of the contralateral side for
Clinic at University of California at Los Angeles, an occult hernia.
Los Angeles, CA, USA

G. Either a totally extraperitoneal (TEP) or

triangular extension is included to ensure
transabdominal preperitoneal (TAPP) tech- proper coverage of the femoral canal and
nique may be used for the repair of a femoral secured to the inguinal ligament, ilioinguinal
hernia. In TEP, a specialized balloon is passed tract, lacunar ligament, and Cooper’s
along the posterior rectus sheath and is used ligament.
to dissect the preperitoneal space. The hernia K . In an open posterior preperitoneal approach,
sac is reduced and a prosthetic mesh is used a transinguinal preperitoneal (TIPP) or tran-
to cover the entire myopectineal orifice. The srectus preperitoneal repair (TREPP) may be
TAPP technique is performed in the same used to place a mesh prosthetic into the pre-
manner except that the peritoneal cavity is peritoneal space to cover the entire myopec-
first entered and then the peritoneum is tineal orifice including the femoral canal. For
incised to enter the preperitoneal space. This large, complicated, or bilateral defects, a
approach is advantageous as it allows for Stoppa repair or giant prosthetic reinforce-
visual inspection of the bowel to assess for ment of visceral sac (GPRVS) may be per-
viability. As in the open approach, if there is formed though a lower midline or Pfannenstiel
suspicion for bowel compromise, the perito- incision. A large synthetic mesh is placed in
neal cavity should be entered (if using TEP the preperitoneal space to the cover the entire
technique) to assess bowel viability and per- myopectineal orifices bilaterally, including
form bowel resection if necessary. both the femoral and inguinal areas. If there is
H. If the bowel is necrotic and surgical expertise concern for bowel compromise on visual
is available, the bowel resection may be per- inspection, the peritoneal cavity may be
formed laparoscopically. Due to concern for entered for bowel resection.
infection, a synthetic mesh is not advisable in L. If bowel compromise is suspected, then an
the setting of strangulation, perforation, gross open anterior or abdominal approach is
contamination, or bowel resection. In this advised. In the setting of strangulation, per-
scenario, a tissue repair or delayed mesh- foration, gross contamination, or bowel
based repair at a later date is advised. resection, a McVay (Cooper’s ligament)
I. There are many options for open femoral her- repair is preferred as it obliterates the femo-
nia repairs. If the diagnosis is made preopera- ral space without the need for mesh. In this
tively and strangulation is not suspected, the technique, a relaxing incision is made to
repair may be performed from either an ante- relieve tension in the suture line. Then, the
rior or posterior approach. transversus abdominis aponeurosis is
J. From the anterior approach, a femoral modi- approximated to Cooper’s ligament medial
fied Lichtenstein technique may be used. This to the femoral canal and iliopubic tract lat-
technique follows the traditional Lichtenstein eral to the canal with interrupted nonabsorb-
technique, except that an additional lateral able sutures.
191 Femoral Hernia 795
Groin bulge
A Pain/vague discomfort
C
Palpable bulge No
below inguinal Imaging
ligament?
Yes
Yes Femoral hernia
D Intraoperative diagnosis Femoral hernia
on imaging?
E Operative repair No
No femoral hernia.
Operative approach depends on surgeon expertise and Consider other
surgeon/patient preference differential diagnoses.
F Laparoscopic approach Open approach I
Bowel viable? Bowel viable?
Viable bowel Necrotic bowel Viable bowel Necrotic bowel
G TEP TAPP Laparoscopic

J Anterior
K Posterior Open anterior or
L
bowel approach approach abdominal approach
H resection and
delayed repair
Femoral Modified TIPP, TREPP, McVay
Lichtenstein Stoppa repair repair
Algorithm 191.1
Hernias Abdominal Wall Surg. 2015;19(3):513–6.

References 2. Bendavid R. Femoral pseudo-hernias. Hernia J
Hernias Abdominal Wall Surg. 2002;6(3):141–3.
1. Powell BS, Lytle N, Stoikes N, Webb D, Voeller 3. Dahlstrand U, Wollert S, Nordin P, Sandblom G,
G. Primary prevascular and retropsoas hernias: Gunnarsson U. Emergency femoral hernia repair:
incidence of rare abdominal wall hernias. Hernia J a study based on a national register. Ann Surg.
2009;249(4):672–6.
Obturator Hernia
192
Algorithmic Approach Howship-Romberg sign (medial thigh pain

on extension, adduction, or medial rotation of
A. Obturator hernias occur when the abdominal the hip) and Hannington-Kiff sign (loss of
contents protrude through the obturator canal. adductor reflex) and are suggestive of an
They are quite rare, accounting for less than obturator hernia when positive [2].
1% of abdominal wall hernias, and are more C. Due to the diagnostic difficulty, if physical
common in thin elderly women, likely due to exam findings are suggestive of obturator her-
loss of supporting connective tissue and nia or if clinical suspicion is high, computed
wider female pelvis [1]. In more than 90% of tomography (CT) is considered the gold stan-
cases, the presenting symptom is a small dard for diagnosis. CT scan may also provide
bowel obstruction and the diagnosis is made information on bowel compromise or
intraoperatively [2]. Other symptoms include perforation.
groin pain radiating medially to the knee D. As previously described, the majority of

(obturator neuralgia), palpable proximal obturator hernias are diagnosed intra-
thigh mass, or ecchymosis of the thigh in the operatively when patients are taken to the
setting of bowel necrosis. operating room for a small bowel obstruction
B. Obturator hernias are often not detectable on or concurrent inguinal or femoral hernia.
physical exam as the hernia is concealed E. Obturator hernias are associated with high
beneath the adductor muscles. However, mortality of 13–40%, likely due to late diag-
sometimes a palpable mass in the groin can nosis, and, thus, whenever an obturator hernia
be identified when the patient is supine with is discovered, operative repair is strongly rec-
the hip flexed and laterally rotated. Two exam ommended [3].
maneuvers that have been described are F. If the diagnosis is made preoperatively and
strangulation is not suspected, a posterior
approach is advised.
Q. L. Hu G. For an open approach, a Stoppa repair or

Medicine at University of California, Los Angeles, giant prosthetic reinforcement of visceral sac
Los Angeles, CA, USA (GPRVS) may be performed though a lower
D. C. Chen (*) midline or Pfannenstiel incision. A large syn-
Department of Surgery, Lichtenstein Amid Hernia thetic mesh is placed in the preperitoneal
Clinic at University of California at Los Angeles, space to cover the obturator orifice as well as
Los Angeles, CA, USA the rest of the myopectineal orifice, including

both the femoral and inguinal areas. If there is may also be used if the diagnosis is made
concern for bowel compromise on visual intraoperatively in an abdominal operation.
inspection, the peritoneal cavity may be J. Depending on surgical expertise availability,
entered for bowel resection. an open lower midline laparotomy incision or
H. If surgical expertise is available, a minimally laparoscopic approach may be used. The her-
invasive (laparoscopic or robotic) approach is nia sac should be reduced and the sac content
preferred. Either the totally extraperitoneal inspected for viability.
(TEP) or transabdominal preperitoneal K. If the bowel is viable and an open approach
(TAPP) technique may be used. In TEP, a was used, then the preperitoneal space may
specialized balloon is passed along the poste- be entered by opening the parietal perito-
rior rectus sheath and is used to dissect the neum. Once in the preperitoneal cavity, a
preperitoneal space. The hernia contents are Stoppa repair maybe performed by placing a
reduced, and a prosthetic mesh is used to synthetic mesh over the obturator orifice as
cover the obturator orifice as well as the rest well as the rest of the myopectineal orifice. If
of the myopectineal orifice. The TAPP tech- a laparoscopic approach was used, then the
nique is performed in the same manner except TAPP technique may be used to complete the
that the peritoneal cavity is first entered and repair by incising the peritoneum and enter-
then the peritoneum is incised to enter the ing the preperitoneal space.
preperitoneal space. This approach is advan- L. If the bowel is necrotic and an open approach
tageous as it allows for visual inspection of was used, then an open bowel resection
the bowel to assess for viability. It is impor- should be performed. Due to concern for
tant to note that in both techniques, the mesh infection, a synthetic mesh is not advisable in
prosthesis should be larger than that used in the setting of strangulation, perforation, gross
traditional inguinal hernia repairs as it must contamination, or bowel resection. The her-
cover both the inguinal and obturator spaces. nia defect may be suture repaired in two lay-
If the appropriate-size mesh is not available, ers [4]. Alternatively, a biologic mesh may be
the mesh may be seated more inferiorly than used or the defect may be reinforced with
usual to ensure proper coverage of the obtura- adjacent tissues such as periosteal flaps, blad-
tor orifice. As in the open approach, if there is der wall, uterine fundus, or ligaments [2]. If a
suspicion for bowel compromise, the perito- laparoscopic approach was used, then a lapa-
neal cavity should be entered (if using the roscopic bowel resection may be performed if
TEP technique) to assess bowel viability and surgical expertise is available. The hernia
perform bowel resection if necessary. defect then may be repaired primarily.
I. If bowel compromise is suspected, a transab- Alternatively, a delayed mesh repair may be
dominal approach is advised. This algorithm performed at a later date.
192 Obturator Hernia 799
Proximal thigh mass

A Groin/medial thigh pain
Small bowel obstruction
C Imaging
No No obturator hernia.
Obturator hernia
Consider other differential
on imaging?
diagnoses.
Yes
D Intraoperative diagnosis Obturator hernia
E Operative repair
Preoperative
diagnosis?
Strangulation?
Preoperative diagnosis Intraoperative diagnosis

F AND OR I
Strangulation not suspected Strangulation suspected
G
Stoppa Laparoscopic
H Abdominal approach J
repair repair
K Viable bowel Necrotic bowel L
Open posterior Laparoscopic Open tissue Laparoscopic

repair repair repair repair
Algorithm 192.1
References 2. Salameh JR. Primary and unusual abdominal wall

hernias. Surg Clin North Am. 2008;88(1):45–60, viii.
3. Hodgins N, Cieplucha K, Conneally P, Ghareeb
1. Stamatiou D, Skandalakis LJ, Zoras O, Mirilas
E. Obturator hernia: a case report and review of the
P. Obturator hernia revisited: surgical anatomy,
literature. Int J Surg Case Rep. 2013;4(10):889–92.
embryology, diagnosis, and technique of repair. Am
4. Shipkov CD, Uchikov AP, Grigoriadis E. The obtura-
Surg. 2011;77(9):1147–57.
tor hernia: difficult to diagnose, easy to repair. Hernia
J Hernias Abdominal Wall Surgery. 2004;8(2):155–7.
Ventral Hernia Repair
193
Algorithmic Approach D. Operative approach to defect closure is based

upon surgeon expertise/preference but modi-
A. Initial evaluation for any ventral hernia
fied based on patient risk factors that will per-
requires complete medical history, physical mit (or preclude) a minimally invasive
examination, and radiologic assessment approach, the presence (or absence) of old
(ultrasound [US] or computed tomography mesh, and the potential need for a component
[CT] scan). Modification of patient-specific separation [2].
risk factors (including smoking cessation, E. For patients with risk factors and 10–20 cm
reduction in immunosuppression, improved defects, a minimally invasive operation
glycemic control, and weight loss/obesity reduces wound complications at the expense
surgery) has been shown to reduce complica- of fascial closure difficulties (e.g., component
tions and recurrence. separation may be needed). An open opera-
B. Multiple hernia staging systems exist, includ- tion facilitates defect closure but at a greater
ing the Ventral Hernia Working Group clas- risk of surgical site infections. We prefer a
sification, European Hernia Society robotic repair with transversus abdominus
classification, and Hernia, Patient, Wound release or an open retrorectus repair depend-
(HPW) staging system. Here, we utilize the ing on patient candidacy for a minimally
HPW staging system as it provides a TNM- invasive herniorrhaphy [3, 4].
like (Tumor Node Metastasis) approach help- F. The presence of a contaminated wound
ful for patient management and operative (ostomy, fistula, concomitant organ resection,
decision-making [1]. or inadvertent enterotomy) is the predomi-
C. Fascial defect closure is preferred for most nant consideration for stage III hernia. Wound
patients as it may provide a more durable contamination decreases the likelihood of a
repair and reduce the risks of both short- and safely performed definitive repair in a single
long-term complications. However, closure procedure. Thus, attempts to downstage her-
of the hernia defect in a geriatric population nia with a staged operative approach can be
or patients with comorbid diseases may considered.
exceed the risk-to-benefit ratio. G. In a contaminated field, options for tempo-
rary repair include suture repair (if possible
J. A. Doble · E. M. Pauli (*) passed on defect size) or bridge repair using a
Department of Surgery, Penn State Milton S. Hershey biologic or a bioabsorbable mesh. Although
Medical Center, Hershey, PA, USA bridged repairs have a high recurrence rate,

802 J. A. Doble and E. M. Pauli
controlling wound contamination first per- logic mesh in select contaminated circum-
mits definitive repair during a second opera- stances; however, the long-term results are
tion. This controlled/planned failure approach not known [5].
permits downstaging of wound class. I. While attempting definitive repair for large
H. Mesh choice in the setting of potential bacte- defects (>20 cm), complete fascial closure may
rial contamination remains a controversial be impossible, resulting in a partial bridged
topic. Options include no mesh, biologic repair (usually in the mid-abdomen). The use
mesh, bioabsorbable mesh, or permanent of lighter-weight mesh may reduce risk for
synthetic mesh. The risks-to-benefit ratio infection but carries increased risk for central
should be determined by the surgeon on a mesh fracture and recurrence. Conversely,
case-by-case basis. Emerging data suggests heavyweight mesh is less likely to fracture but
that the retromuscular placement of carries increased risk for mesh infection. Mesh
intermediate-weight polypropylene synthetic determination for each individual patient
mesh may have short-term benefits over bio- should be based off intraoperative findings.
A H&P; abdominal imaging (CT/US)
1. Smoking cessation
Yes 2. Reduce immunosuppression
Risk reduction possible?
3. Lower HbA1c
No 4. Weight loss/obesity surgery
B Stage hernia
Stage I Stage II Stage III Stage IV
<10cm, low risk, clean <20cm, ± risk factors >20cm, ± risk factors
<10cm + risk factors

C
F
Close No Contamination
LVHR (Bridged) present?
defect?
No
Yes Definitive repair

D E
Yes
Minimally invasive vs <20cm, ± risk factors I
open VHR
See abdominal wall
reconstruction
Robotic vs open vs
hybrid VHR ± G
component separation Downstage
possible?
H Remove contamination,
temporary repair
Algorithm 193.1
193 Ventral Hernia Repair 803
References 3. Carbonell AM, Cobb WS, Chen SM. Posterior com-

ponents separation during retromuscular hernia repair.
Hernia. 2008;12(4):359–62.
1. Kanters AE, Krpata DM, Blatnik JA, Novitsky YM,
4. Martin-Del-Campo LA, Weltz AS, Belyansky I,
Rosen MJ. Modified hernia grading scale to stratify
Novitsky YW. Comparative analysis of perioperative
surgical site occurrence after open ventral hernia
outcomes of robotic versus open transversus abdomi-
repairs. J Am Coll Surg. 2012;215(6):787–93.
nis release. Surg Endosc. 2018;32(2):840–5.
2. Orenstein SB, Dumeer JL, Monteagudo J, Poi MJ,
5. Majumder A, Winder JS, Wen Y, Pauli EM, Belyansky
Novitsky YW. Outcomes of laparoscopic ventral her-
I, Novitsky YW. Comparative analysis of biologic
nia repair with routine defect closure using “shoelac-
versus synthetic mesh outcomes in contaminated her-
ing” technique. Surg Endosc. 2011;25(5):1452–7.
nia repairs. Surgery. 2016;160(4):828–38.
Incarcerated and Strangulated
Hernia 194
Algorithmic Approach C. In a hemodynamically stable patient, abdomi-

nal imaging (CT) has some utility for pre-
A. It is essential to determine the chronicity of operative surgical planning (especially
incarceration during initial patient history ventral hernia). Abdominal imaging may also
and examination as this will establish the identify the bowel that is at risk for necrosis
need for an emergent surgery. Directed ques- and/or perforation (wall thickening, abdomi-
tioning should elicit any previous history of nal free fluid, pneumatosis, and free air), but
incarceration (chronic vs. acute), history of obtaining imaging should not delay surgery
obstruction symptoms, and timing/duration in a patient with surgical indications.
of an acute event. Examination must identify D. Operative approach to defect closure is based
any “hard” surgical signs such as peritonitis, upon surgeon expertise/preference but modi-
subcutaneous crepitus, discoloration of her- fied based on patient risk factors that will
nia sac, and necrotic skin changes. Chronically permit (or preclude) a minimally invasive
incarcerated hernias without symptoms of approach (inability to close defect).
obstruction or strangulation can be managed E. Laparoscopy may be necessary to inspect the
electively. bowel even in an open inguinal repair. This
B. Indication for manual reduction (taxis) of an can be easily performed in an open procedure
incarcerated hernia is an area of debate in via insertion of the laparoscope into the her-
surgical literature. Published literature sug- nia sac [4].
gests bedside reduction not be attempted if F. The choice of a primary suture repair vs.
acute incarceration occurred 4–12 h prior to mesh-based repair of the hernia defect
attempt. Several laboratory findings (WBC depends on the following: Defect size, defect
>20,000, lactic acid >2 mmol/L) can help location, surgeon expertise, patient risk fac-
with determination of safety, but attempting tors (Diabetes Mellitus, tobacco use, immu-
bedside reduction is ultimately a clinical nosuppressed), and presence of bowel injury/
decision [1–3]. contamination (bacterial translocation with
incarcerated bowel). The surgeon must deter-
mine if the risk of hernia recurrence with a
suture repair exceeds the potential risk of
J. A. Doble · E. M. Pauli (*) mesh complication. We advocate for a tempo-
Department of Surgery, Penn State Milton S. Hershey rary repair (suture repair or a simple bridged
Medical Center, Hershey, PA, USA mesh repair) rather than a component separa-

tion for the majority of acute presentations. In tion will identify any consequence from
these patients, definitive repair proceeds elec- failed identification/manual reduction of
tively with pre-operative risk reduction and necrotic bowel. Diagnostic laparoscopy may
patient optimization [5]. be used selectively to assist in ruling out this
G. Although rarely reported in the literature, the possibility. Patients with successfully reduced
successful manual reduction of necrotic hernias should be closely followed and sched-
bowel can occur. A brief period of observa- uled for definitive repair electively [1].
Algorithm 194.1
examination
A
B
Yes
Safe to reduce Reducible?
No No Yes
Acute repair indicated C Observe
Ventral hernia Inguinal hernia
D E
Open repair MIS repair Open repair MIS repair
Perform necessary concomitant Plan definitive

F procedures (e.g. organ resection) repair
Repair hernia defect
194 Incarcerated and Strangulated Hernia 807
References 4. Sajid MS, Ladwa N, Colucci G, Miles WF, Baig

MK, Sains P. Diagnostic laparoscopy through
deep inguinal ring: a literature-based review on
1. Harissis HV, Douitsis E, Fatouros M. Incarcerated
the forgotten approach to visualize the abdominal
hernia: to reduce or not to reduce? Hernia.
cavity during emergency and elective groin her-
2009;13(3):263–6.
nia repair. Surg Laparosc Endosc Percutan Tech.
2. Tanaka K, Hanyu N, Iida T, et al. Lactate levels in
2013;23(3):251–4.
the detection of preoperative bowel strangulation. Am
5. Slater NJ, van Goor H, Bleichrodt RP. Large and
Surg. 2012;78(1):86–8.
complex ventral hernia repair using “components
3. Kauffman HM Jr, O’Brien DP. Selective reduc-
separation technique” without mesh results in a high
tion of incarcerated inguinal hernia. Am J Surg.
recurrence rate. Am J Surg. 2015;209(1):170–9.
1970;119(6):660–73.
Management of Open Abdomen
195
Algorithmic Approach ally makes the reoperation much more com-

plex. The division of anterior and posterior
A. All abdominal closure therapies should pri- components should not be performed during
marily focus on prevention of gastrointestinal a single operation, and operations that inten-
fistula formation with a secondary endpoint tionally divide the linea semilunaris should
of definitive abdominal closure. be avoided altogether.
B. Options for a bridged repair include Vicryl D. The success rate for primary fascial closure
and biologic mesh. Vicryl mesh will be reab- decreases by approximately 1.1% for each
sorbed quickly (6 weeks) leading to an early, 24-h delay after initial laparotomy [2]. Early
large hernia formation, and in the event of a anticipation of delayed closure and the use of
deep surgical site infection, the exposed mesh a Wittman patch and transabdominal wall
will permit large hydraulic shift of fluid from traction (TAWT) systems have been shown to
the bowel leading to desiccation and possible increase abdominal closure rates. These
fistula formation. Biologic mesh will fail over devices facilitate closure by facilitating
a longer period of time (80% at 2 years [1]) sequential isometric contraction of the
but carries an increase in cost and risk of bio- abdominal wall via Velcro sheets sewn to the
logic mesh complication (seroma/wound fascia (Wittman patch) or with transfascial
infection). However, in the event of a deep sutures secured over a plastic skin bolster
surgical site infection, the mesh will not per- (TAWT). Additionally, chemical component
mit underlying bowel desiccation and permits separation with botulinum toxin has been
negative-pressure wound therapy (NPWT) described to increase the probability of pri-
directly on the mesh. mary fascial closure [3].
C. Performing a component separation in an
E. Negative-pressure wound therapy systems
acute or emergent procedure should be done are available as a preassembled commercial
with extreme caution as it carries a higher set product or can be created out of standard
of risks than in the elective setting. Moreover, operative supplies. NPWT devices can be
it eliminates viable options for definitive safely used for temporary abdominal closures
repair of the hernia at a later date and gener- and may improve success rates of primary
fascial closure [4].
J. A. Doble · E. M. Pauli (*) F. There are several options for temporary
Department of Surgery, Penn State Milton S. Hershey abdominal closure when a large intraabdomi-
Medical Center, Hershey, PA, USA nal fluid (blood or ascites) volume is not

expected. The Bogota bag technique is per- attaches both fascial edges to a slide fastening
formed by suturing a sterile, clear plastic device, thus allowing for quick temporary
sheet, usually from an X-ray cassette sheet or exposure/closure of the abdomen. The barker
IV bag to the fascia. A silo closure wraps the bag technique is an intraoperatively con-
externalized bowel in plastic secured to the structed negative-pressure wound therapy
fascial edges. The zipper closure method system.
Open abdomen
A
Re-exploration
indicated
No Yes
Fascial closure Delayed closure D

possible anticipated
Yes Consider: Wittman
patch, TAWT or
No
Yes No abdominal wall botox
Suture
closure NPWT Yes
available? E
NPWT
No
Anticipate
Mesh available fluid egress
No Yes
Yes No
B F
Bridged Bogota bag Barker bag
repair Zipper closure or
Silo closure NPWT
Delayed
incisional
hernia repair
Component
separation
possible?
C
Yes No
Component Skin closure w/

separation retention sutures
Algorithm 195.1
195 Management of Open Abdomen 811
References 3. Zielinski MD, Goussous N, Schiller HJ, Jenkins

D. Chemical components separation with botuli-
num toxin a: a novel technique to improve primary
1. Blatnik J, Jin J, Rosen M. Abdominal hernia repair
fascial closure rates of the open abdomen. Hernia.
with bridging acellular dermal matrix – an expensive
2013;17(1):101–7.
hernia sac. Am J Surg. 2008;196(1):47–50.
4. Cirocchi R, Birindelli A, Biffl WL, et al. What is the
2. Pommerening MJ, DuBose JJ, Zielinski MD, et al.
effectiveness of the negative pressure wound ther-
Time to first take-back operation predicts successful
apy (NPWT) in patients treated with open abdomen
primary fascial closure in patients undergoing damage
technique? A systematic review and meta-analysis. J
control laparotomy. Surgery. 2014;156(2):431–8.
Trauma Acute Care Surg. 2016;81(3):575–84.
Abdominal Wall Reconstruction
196
Algorithmic Approach C. Operative planning prior to abdominal wall

reconstruction requires consideration of several
A. The initial evaluation for any hernia requires patient-specific risk factors. For the purpose of
a complete medical history, physical exami- this chapter, we will use hernia defect size to
nation, and radiologic assessment (ultrasound simplify the treatment algorithm. Other factors
[US] or computed tomography [CT] scan). (patient risk factors, hernia stage, and wound sta-
Modification of patient-specific risk factors tus) have been discussed in previous chapters.
(including smoking cessation, reduction in All listed measurements are approximations and
immunosuppression, improved glycemic should not be used in isolation to determine the
control, and weight loss/obesity surgery) has operative technique or type of component sepa-
been shown to reduce complications and ration. Patient body habitus, abdominal anat-
recurrence [1]. omy, and laxity of the abdominal wall must also
B. The phrase “Abdominal Wall Reconstruction” be considered pre-operatively.
describes a variety of surgical procedures D. A minimally invasive operative (robotic, lapa-
aimed at restoring abdominal wall anatomy roscopic, or enhanced-view totally extraperito-
and function. In general, this is achieved neal [eTEP]) approach is ultimately determined
through closure of fascial defects and wide by surgeon experience and preference; how-
reinforcement with mesh prosthesis. There ever, the presence of adhesive disease, previous
are a variety of indications that determine the mesh, or wound contamination may require an
need for an abdominal wall reconstruction open procedure [2].
procedure, including abdominal pain limiting E. Rectus muscle size is an important consider-
activities of daily life, risk for bowel strangu- ation during an open retrorectus repair as
lation or incarceration, young age, and the midline fascial closure with prosthetic mesh
need for a functional abdominal wall. A large overlap is required. Technical limitations of
hernia with loss of domain is not, by itself, an the minimally invasive approach may require
indication for abdominal wall reconstruction additional component separation (transversus
as in some patients the risk of surgery may abdominis release [TAR]), whereas an open
outweigh the benefit [1]. approach may not require any additional
component release. The surgeon must deter-
J. A. Doble · E. M. Pauli (*) mine if the decreased wound morbidity and
Department of Surgery, Penn State Milton S. Hershey enhanced recovery benefits outweigh the risk
Medical Center, Hershey, PA, USA of an extended tissue dissection.

F. The vast majority of medium-sized defects section will enable sublay mesh placement or
are managed by either a transversus abdomi- “sandwich” repair with two pieces of mesh
nis release (TAR) or external oblique release (sublay and onlay mesh placement) [3, 4].
(EO). The TAR allows for greater medializa- H. As mentioned earlier, TAR is preferably given
tion of the tissues and wider mesh overlap for a lower rate of wound complication and hernia
midline and non-midline defects. It is rapidly recurrence. When approaching large defects,
becoming the more commonly used proce- you must acknowledge the possibility that
dure as data indicates that TAR has a lower midline fascial closure may not be possible. In
recurrence and complication rate than an EO such a case, accept the necessity of a bridged
release. However, the EO remains viable as repair. It is generally felt that a simultaneous
the dissection is entirely extraperitoneal. It external oblique (EO) and transversus abdom-
can be used when the abdominal cavity is inis release (TAR) should not be performed. If
deemed unapproachable/hostile (adhesive you suspect you may be unable to re-approxi-
disease, previous radiation, and previous pos- mate fascia, you may consider preoperative
terior component resection). pneumoperitoneum or botulism toxin injec-
G. External oblique release can be completed by tions to the lateral musculature to provide
creating subcutaneous flaps that preserve additional fascial mobility [3].
periumbilical perforator vessels from a mid- I. All patients, regardless of technique, should
line incision or with laparoscopic tools from a be closely monitored for signs of abdominal
lateral incision. EO repairs allow for the mesh hypertension and abdominal compartment
to be placed directly on the muscle with fas- syndrome (high peak airway pressure,
cia closed underneath (onlay). If additional decreased urine output, and elevated bladder
mesh coverage is required, a retrorectus dis- pressure).
196 Abdominal Wall Reconstruction 815
H&P; abdominal CT imaging
Surgical risk reduction:

1. Smoking cessation
A 2. Reduce immunosuppression
3. Lower HbA1c
4. Weight loss/obesity surgery
See previous Yes

Contaminated
chapter
No
B Meets criteria for abdominal
wall reconstruction?
C
Small defect Large defect
8–10 cm >20 cm
Contamination Fascia
or adhesions? closable?
Yes No
D No Yes
E H
Open Medium weight Bridged repair
MIS repair mesh w/ heavy mesh
retrorectus
I
ICU evaluation and
monitoring if
Medium defect needed
10–20 cm
TAR F EO
G
MIS w/ Open repair w/ MIS or open EO
sublay mesh sublay mesh ± retrorectus
mesh: onlay, sublay,
or ‘sandwich’
Algorithm 196.1
retromuscular hernia repair. Surg Endosc.

References 2018;32(3):1525–32.
3. Novitsky YW, Fayezizadeh M, Majumder A, Neupane
1. Majumder A, Novitsky Y. Retrorectus hernia and R, Elliott HL, Orenstein SB. Outcomes of posterior
transversus abdominis release. In: Hope WW, Cobb component separation with transversus abdominis
WS, Adrales GL, editors. Textbook of hernia. New muscle release and synthetic mesh sublay reinforce-
York: Springer; 2017. p. 225–32. ment. Ann Surg. 2016;264(2):226–32.
2. Belyansky I, Daes J, Radu VG, et al. A novel 4. Pauli EM, Rosen MJ. Open ventral hernia repair
approach using the enhanced-view totally extra- with component separation. Surg Clin North Am.
peritoneal (eTEP) technique for laparoscopic 2013;93(5):1111–33.
Part XXIII
Bariatric Surgery
Indications for Bariatric Surgery
197
Jin Sun Kim and Ann M. Rogers
Algorithmic Approach impaired quality of life, and disqualification

from other surgeries resulting from obesity
A. Bariatric surgery is the only effective and
(e.g., joint replacement, organ transplanta-
durable treatment for most patients with tion, and ventral hernia repair) [4].
severe obesity and its associated medical D. Patients generally excluded from bariatric

comorbidities [1]. Guidelines and recommen- surgery include those with reversible disor-
dations for application of bariatric surgery ders that cause obesity, active drug or alcohol
differ by country [2]. abuse, poorly controlled psychiatric condi-
B. In the United States, patients with Class 3 tions, current pregnancy, history of active sui-
obesity, defined as having a body mass index cide attempt, cancer not in remission, and
(BMI) >40 kg/m2, or Class 2 obesity with a severe heart or lung disease making the
BMI between 35 and 39.9 kg/m2 along with patient a poor surgical candidate [3]. Patients
at least one severe associated weight-related under the age of 18 are best treated at a mul-
condition are potential candidates for weight tidisciplinary adolescent weight loss surgery
loss surgery [3]. program with appropriate pediatric special-
C. Obesity-related comorbidities include type 2 ties available [5].
diabetes, obstructive sleep apnea, hyperten-
E. Currently approved surgical weight loss
sion, hyperlipidemia, obesity-hypoventilation options in the United States include vertical
syndrome, nonalcoholic fatty liver disease, sleeve gastrectomy, roux-en-Y gastric bypass,
pseudotumor cerebri, gastroesophageal reflux adjustable gastric band, and biliopancreatic
disease, asthma, venous stasis disease, stress diversion with duodenal switch [6].
urinary incontinence, debilitating arthritis,
J. S. Kim
Pennsylvania State University College of Medicine,
Hershey, PA, USA
A. M. Rogers (*)

820 J. S. Kim and A. M. Rogers
Algorithm 197.1
Patient has obesity
Does the
A patient live
in the US?
No
Yes
See local guidelines B
Does the
patient have
a BMI > 35?
No
Yes
Seek medical weight
management
BMI 35–39.9 BMI > 40
Yes
Any contraindications? D
Yes No E
Does the
patient have
No Recommend
significant
weight-related bariatric surgery
problems?
4. Mechanick JI, Youdim A, Jones DB, et al. Clinical

References practice guidelines for the perioperative nutritional,
metabolic, and nonsurgical support of the bariat-
1. Burguera B, Agusti A, Arner P, et al. Critical assess- ric surgery patient—2013 update: cosponsored by
ment of the current guidelines for the management and American Association of Clinical Endocrinologists,
treatment of morbidly obese patients. J Endocrinol The Obesity Society, and American Society for
Investig. 2007;30:844. Metabolic & Bariatric Surgery. Obesity (Silver Spring,
2. Borisenko O, Colpan Z, Dillemans B, et al. Clinical Md). 2013;21(1):S1–27. https://doi.org/10.1002/
indications, utilization, and funding of bariatric sur- oby.20461.
gery in Europe. Obes Surg. 2015;25:1408–16. 5. Michalsky M, Reichard K, Inge T. ASMBS pediatric
3. NIH conference. Gastrointestinal surgery for severe committee best practice guidelines. Surg Obes Relat
obesity. Consensus Development Conference Panel. Dis. 2012;8:1–7.
Ann Int Med. 1991;115:956–61. 6. Lee WJ, Almalki O. Recent advancements in bar-
iatric/metabolic surgery. Ann Gastroenterol Surg.
2017;1:171–9.
Work-Up of Abdominal Pain
in the Bariatric Patient 198
Sarayna S. McGuire and Ann M. Rogers
Algorithmic Approach contents. An upper gastrointestinal (UGI)

swallow study is the test of choice for band-
A. Bariatric patients presenting with abdominal related symptoms, but its performance is not
pain should undergo a complete history and always feasible outside of normal work hours.
physical examination to determine the qual- D. An abdominal and pelvic computed tomogra-
ity, location, and timing of the pain and asso- phy (CT) scan should be performed to exclude
ciated symptoms. The operative date, the potentially life-threatening complications
procedure type, and postoperative course will of a closed-loop bowel obstruction, bowel
help guide the work-up and diagnosis. Vital perforation, or gastric prolapse when UGI is
signs and laboratory work can provide crucial not available [2]. Use of oral contrast is gen-
information on acuity and potential causes of erally helpful in the examination of post-
the pain. bariatric anatomy; use of intravenous contrast
B. In the early postoperative setting, complica- is optional, depending on suspected diagno-
tions may arise specific to the patient’s proce- ses related to inflammation or blood supply.
dure type—the anastomoses in the roux-en-Y
E. Exploratory laparotomy or laparoscopy
gastric bypass (RYGB) (e.g., strictures, leaks, (depending on surgeon preference or patient
ulcers), band malfunction or malposition factors) is indicated when internal hernia is
after adjustable gastric band (AGB), and suspected as this can be missed with plain
leaks after biliopancreatic diversion with films, upper gastrointestinal contrast studies,
duodenal switch (BPD/DS). Missed bowel and CT scans [1]. It is also appropriate for
perforation due to mobilization difficulty perforated marginal ulcer, intussusception,
(e.g., strictures, adhesions, and internal her- adhesive bowel obstruction, or symptomatic
nia) may also occur after any of these [1]. gastric prolapse through a band.
C. For any AGB patient with abdominal pain, F. In the later postoperative setting (>30 days),
nausea, or vomiting, the band should be com- patients may present with intestinal obstruc-
pletely deflated prior to pursuing a diagnostic tion after any bariatric procedure, marginal
work-up because of the risk of gastric isch- ulcers with or without associated strictures
emia or the possibility of aspiration of gastric after RYGB, dumping syndrome after RYGB,
gallstone disease, nutritional deficiencies, and
S. S. McGuire · A. M. Rogers (*) gastrointestinal problems unrelated to bariat-
Department of Surgery, Penn State Milton S. Hershey ric surgery (e.g., gastroesophageal motility

822 S. S. McGuire and A. M. Rogers
disorders, celiac disease, or irritable bowel ultrasound and/or nuclear scan (gallstone dis-
syndrome). ease), abdominal CT scan (intestinal obstruc-
G. Depending on the history, symptoms, and
tion), stool specimen (C. difficile colitis and
physical examination, work-up of the patient hematochezia), upper or lower endoscopy, or
in this later setting can include abdominal contrast studies (reflux/regurgitation).
Abdominal pain in bariatric patient

· History and physical exam
A · Establish operative date, procedure type, and postoperative course
· Obtain vital signs and blood work. If febrile, work up for infection.
B Early postoperative setting:
Consider complications specific to procedure: Later postoperative setting

· RYGB: strictures, leaks, and ulcers (>30 days)
· AGB: band malfunction/malposition
F
· BPD/DS: leaks
Rule-out missed bowel perforation.
Patients may present with intestinal
obstruction after any procedure,
marginal ulcers with or without
associated strictures after RYGB,
C dumping syndrome after RYGB,
gallstone disease, nutritional
For AGB patients, Obtain abdominal and deficiencies, and gastrointestinal
deflate the band pelvic CTscan to problems unrelated to bariatric
prior to work-up. D exclude closed loop surgery.
UGI swallow study bowel obstruction,
is the test of choice. bowel perforation, or
gastric prolapse when
UGI is not available.
Use of oral contrast is
preferred.
Depending on history, symptoms,
G and physical exam, work-up can
Exploratory laparotomy or include abdominal ultrasound
E and/or nuclear scan, abdominal
laparoscopy is indicated when
internal hernia, perforated CT scan, stool specimen, upper
marginal ulcer, intussusception, or lower endoscopy, or contrast
adhesive bowel obstruction, or studies.
symptomatic gastric prolapse
through a band are suspected.
Follow-up care
Algorithm 198.1
198 Work-Up of Abdominal Pain in the Bariatric Patient 823
References metabolic, and nonsurgical support of the bariat-

ric surgery patient—2013 update: cosponsored by
American Association of Clinical Endocrinologists,
1. Lee CW, Kelly JJ, Wassef WY. Complications of
The Obesity Society, and American Society for
bariatric surgery. Curr Opin Gastroenterol. 2007;
Metabolic & Bariatric Surgery. Surg Obes Relat Dis.
23(6):636–43.
2013;9:159–91.
2. Mechanick JI, Youdim A, Jones DB, et al. Clinical
practice guidelines for the perioperative nutritional,
Internal Hernia: Diagnosis
and Treatment 199
Brandon LaBarge and Ann M. Rogers
Algorithmic Approach B. Because of altered anatomy, internal hernia

bowel obstructions in post-bariatric patients
A. Internal hernias have an incidence of less than cannot be definitively diagnosed with plain
1% after abdominal operations but constitute abdominal radiographs [2]. Contrast com-
up to 5.8% of all small bowel obstructions puted tomography (CT) is most commonly
and if untreated have a reported mortality rate used to make a diagnosis, with characteristics
exceeding 50% in some series when associ- including bowel configuration consisting of a
ated with strangulation [1]. Patients who have saclike mass of dilated bowel loops in the
undergone gastric bypass or the duodenal presence of small bowel obstruction, or a
switch procedure are at risk for internal her- mesenteric “swirl,” related to a mesenteric
nia bowel obstructions because of new mes- vascular pedicle that is engorged, stretched,
enteric defects created during such operations. displaced, or twisted, along with converging
Patients with acute intestinal obstruction vessels at the hernia orifice [3].
related to an internal hernia may present with C. Definitive diagnosis and treatment is via
vomiting, abdominal distension, and colicky diagnostic laparoscopy or laparotomy in
abdominal pain, as well as physical signs of cases where bowel dilation precludes safe
rebound tenderness and involuntary guard- port placement, when laparoscopic correc-
ing. If the hernia is spontaneously reducible, tion is unsuccessful or when such skills are
symptoms may be vague and can include unavailable [4].
recurrent central abdominal pain and nausea.
B. LaBarge
Department of Surgery, Penn State College of
Medicine, Penn State Milton S. Hershey Medical
A. M. Rogers (*)

826 B. LaBarge and A. M. Rogers
Post-bariatric patient with acute

A or recurrent central abdominal
pain, +/– nausea and vomiting
CT scan with oral, +/–

B i.v. contrast
Normal/Non-
Internal hernia Other findings
diagnostic
Symptoms persist Symptoms improve
C Exploration Observe Treat as appropriate
Algorithm 199.1 Reprinted by permission from Springer Nature: Obes Surg. Diagnosis and management of internal
hernias after laparoscopic gastric bypass. Parakh S, Soto E, Merola S. Copyright 2007
2. Martin L, Merkle E, Thompson W. Review of inter-

References nal hernias: radiographic and clinical findings. Am J
Roentgenol. 2006;186(3):703–17.
1. Salar O, El-Sharkawy AM, Singh R, et al. Internal 3. Takeyama N, Gokan T, Ohgiya Y, et al. CT of internal
hernias: a brief review. Hernia. 2013;17(3):373–7. hernias. Radiographics. 2005;25(4):997–1015.
4. Parakh S, Soto E, Merola S. Diagnosis and manage-
ment of internal hernias after laparoscopic gastric
bypass. Obes Surg. 2007;17(11):1498–502.
Marginal Ulcer: Diagnosis
and Treatment 200
Ye Tian and Ann M. Rogers
Algorithmic Approach ment of these conditions is beyond the scope

of this algorithm.
A. Marginal ulcer (MU), which occurs at or near B. If upper GI symptoms are present in post-
the intestinal side of a gastrojejunal anasto- RYGB patients, an upper endoscopy should
mosis, is a common complication of Roux- be the first diagnostic step [3]. Endoscopic
en-Y gastric bypass (RYGB) surgery, seen in findings may include normal anatomy, MU
up to 16% of patients. Most MUs are seen alone, MU with associated anastomotic stric-
within 1 year after RYGB [1]. Early MU is ture, MU with exposed staples or sutures, or
likely due to technical issues such as anasto- MU with gastro-gastric fistula [4]. If MU is
motic tension, ischemia, or type of staples or present with or without associated findings,
sutures used but may be related to patient fac- biopsy for Helicobacter pylori (H. pylori)
tors such as diabetes or hypertension. Later testing should be performed [5].
presentations of MUs may be associated with C. Management of uncomplicated MUs includes
smoking, alcohol use, steroid or nonsteroidal 3 months of proton pump inhibitor (PPI) +/−
anti-inflammatory drug (NSAID) use, or sucralfate. Cessation of smoking and steroid
larger pouch size [2]. Patients with MUs or chronic NSAID use should be imple-
commonly present with upper gastrointesti- mented if applicable [2]. If H. pylori testing is
nal (GI) symptoms including abdominal pain, positive, the patient should be treated with
nausea, vomiting, dysphagia, and hemateme- appropriate antibiotic therapy in addition [5].
sis. Fever may also be present [3]. History If anastomotic stricture is identified, then
and physical exam are the first steps in the endoscopic balloon or Bougie dilation of the
diagnosis of MU. It is important to note that stricture should be performed [5]. If exposed
28–61% of patients report no prior symptoms staples or sutures are identified, they should
and may present with massive bleeding or be removed and associated ulceration should
perforation of the MU [2]. Emergent treat- be treated as above [3]. If gastro-gastric fis-
tula is identified in addition to MU, then elec-
Y. Tian tive interventions to close the fistula are
Penn State Health Milton S. Hershey Medical Center, appropriate.
Hershey, PA, USA D. If MU symptoms persist or worsen after

A. M. Rogers (*) 3 months of treatment, or in the setting of
Department of Surgery, Penn State Milton S. Hershey bleeding or perforation, then surgical inter-
Medical Center, Hershey, PA, USA vention may be necessary.

828 Y. Tian and A. M. Rogers

Epigastric abdominal pain, nausea, vomiting, dysphagia, hematemesis +/-fever
A after RYGB surgery
Obtain upper endoscopy

Further work-up
Normal post-
RYGB anatomy What does the MU with
upper endoscopy
B show?
Stricture
Gastro-gastric
fistula with
marginal ulcer
Marginal ulcer
C Cessation of smoking and

Treat MU and NSAID use if applicable Endoscopic balloon
consider closure dilation; initiate 3
of fistula mo therapy with
PPI, +/- sucralfate
Check H. pylori
biopsy result
Initiate 3 mo medical
H. pylori Yes therapy with PPI, +/-
positive? Sucralfate, and eradicate
H pylori
No
Initiate 3 months of medical

therapy with PPI +/-
Sucralfate
Are symptoms
persisting or
worsening?
Algorithm 200.1
200 Marginal Ulcer: Diagnosis and Treatment 829
Yes No
D
Repeat endoscopy Continue medical

therapy for
another 3 months
and discontinue if
asymptomatic
No
MU
present?
Yes
Consider surgical intervention to resect

and reconstruct GJ anastomosis
Follow-up Care
3. Racu C, Mehran A. Marginal ulcers after roux-en-Y

References gastric bypass: pain for the patient…pain for the sur-
geon. Bariatric Times. 2010;7(1):23–5.
1. Steinemann DC, Bueter M, Schiesser M, et al. 4. Nguyen NT, Hinojosa MW, Gray J, Fayad
Management of anastomotic ulcers after roux-en-Y C. Reoperation for marginal ulceration. Surg Endosc.
gastric bypass: results of an international surgery. 2007;21:1919–21.
Obes Surg. 2014;24:741–6. 5. Chaves LCL, Borges IKLC, de Souza MDG, et al.
2. Coblijn UK, Lagarde SM, de Castro Inflammatory disorders associated with helicobacter
SMM. Symptomatic marginal ulcer disease after pylori in the roux-en-Y gastric pouch. Arq Bras Cir
roux-en-Y gastric bypass: incidence, risk factors and Dig. 2016;29(Suppl 1):31–4.
management. Obes Surg. 2015;25:805–11.
Ventral Hernia Repair in Bariatric
Patients 201
Anish Shah and Salvatore Docimo Jr.
Algorithmic Approach B. Once the need for acute surgical intervention

for hernia repair in bariatric patients is ruled
A. The risk of ventral hernia development
out, careful consideration must be given to the
increases from 13% to 39% for patients timing and feasibility of hernia surgery. Hernia
with body mass index (BMI) > 25 kg/m2. repair in the obese patient has been associated
An increased incidence of ventral hernias with complications such as surgical site infec-
in these patients is secondary to elevated tion and recurrence [1, 2]. As a result, it is
intra-abdominal pressure as well as systemic important to determine whether hernia repair
hypertension often seen with morbid obesity. can be delayed, what the safest and most effec-
In patients undergoing open gastric bypass tive surgical approach would be, and whether
surgery, the incidence of incisional hernias the patient qualifies for weight loss surgery.
has been reported to be as high as 20%. With C. If a ventral hernia is known prior to bariat-
laparoscopic bariatric surgery, this rate has ric surgery, the surgeon has three options:
decreased to less than 1%. The first step in (1) perform planned bariatric surgery and
managing ventral hernias in bariatric patients defer hernia repair to a later time; (2) per-
is determining the presence and severity of an form the bariatric surgery and hernia repair
obstruction based on a complete history and simultaneously; (3) repair the hernia first
physical exam. Because physical exam can be and then perform bariatric surgery at a later
limited in bariatric patients, further imaging time. However, the third option is only viable
can help in the evaluation of hernias in this if the hernia becomes clinically significant
patient population. Particularly, computed (obstructed, incarcerated, and strangulated)
tomography (CT) can be useful in monitoring prior to bariatric surgery.
hernias over time and determining the extent D. Significant weight loss is the benefit of per-
of any underlying obstruction. forming bariatric surgery prior to ventral her-
nia repair. In setting of open ventral hernia in
the obese patient, the incidence of compli-
A. Shah
Department of Surgery, Stony Brook University cations (surgical site infection (SSI), recur-
Hospital, Stony Brook, NY, USA rence, cardiovascular, pulmonary) has been
S. Docimo Jr. (*) directly correlated to the rise in BMI [1, 3].
Division of Bariatric, Foregut, and Advanced An evaluation of BMI and ventral hernia dem-
Gastrointestinal Surgery, Stony Brook Medicine, onstrated that patients with a BMI > 40 kg/
Stony Brook, NY, USA m2 had a 2.89 times greater chance of h aving
© Springer Nature Switzerland AG 2019

A. Shah and S. Docimo Jr.
a
complication compared to a group with form the hernia repair first and defer bariat-
BMI < 25 kg/m2 [4]. ric surgery until recovery. However, it is key
E. In bariatric patients with a hernia causing that this time is short so that recurrence is
worsening symptoms, it is reasonable to per- unlikely.
Patient undergoing bariatric surgery with a

concomitant hernia
A
History, physical exam, radiography to

evaluate hernia characteristics
Symptomatic hernia: obstruction,

incarceration, strangulation Asymptomatic hernia
Obstructed or BMI > 40 kg/m2 BMI < 40 kg/m2 C

Strangulated or
incarcerated
contaminated case
hernia
Weight loss prior to Concomitant

ventral hernia repair: hernia and D
surgical or medicinal bariatric surgery
Open or LVHR: Open or LVHR:
fascial closure Fascial closure
with mesh with no mesh
Ventral hernia Concomitant
repair after adequate hernia and
weight loss bariatric surgery: E
Bariatric surgery Bariatric surgery
fascial closure
with mesh
Algorithm 201.1
201 Ventral Hernia Repair in Bariatric Patients
References elective abdominal surgery based on 12,373 cases.

The case for targeted prophylactic intervention. Ann
Surg. 2016;263:1010–7.
1. Veljkovic R, Protic M, Gluhovic A, et al. Prospective
3. Flancbaum L, Choban PS. Surgical implications of
clinical trial of factors predicting the early develop-
obesity. Annu Rev Med. 1998;49:215–34.
ment of incisional hernia after midline laparotomy. J
4. Pernar LIM, Pernar CH, Dieffenbach BV, Brooks
Am Coll Surg. 2010;210:210–9.
DC, Smink DS, Tavakkoli A. What is the BMI thresh-
2. Fischer JP, Basta MN, Mirzabeigi MN, et al. A risk
old for open ventral hernia repair? Surg Endosc.
model and cost analysis of incisional hernia after
2017;31(3):1311–7.
Acute Leak Following Bariatric
Surgery: Endoscopic Stent 202
Management
Algorithmic Approach sis within the gastric sleeve causing an increase

of proximal intraluminal pressure [7]. The
A. The occurrence of anastomotic and staple line optimal diagnostic modality is a contrast com-
leak is 1.6–4.8% after laparoscopic roux-en-y puted tomography (CT) as it offers improved
gastric bypass (LRYGB) and 1.7–2.4% after sensitivity and additional information such as
laparoscopic sleeve gastrectomy (LSG), possible fluid collections in the left upper
respectively [1–3]. Patients often present with quadrant or free intraperitoneal air [8].
complaints of abdominal pain, fever, chills, C. Once a leak is confirmed, peritonitis or septic
and possible nausea and vomiting. Vitals shock will play a large role in deciding which
typically demonstrate tachycardia, fever,
treatment paradigm to follow. If a patient
tachypnea, and possibly hypotension. A leuko- demonstrates septic shock or peritonitis, intra-
cytosis is often noted. Typical surgical options operative management with surgical explora-
for leak management include anastomotic tion should be the first line of treatment. If the
revision, primary repair, and bowel, gastric, or patient is stable, leaks in the proximal or mid-
omental patching [4]. Flexible endoscopy has portion of the sleeve are most amenable to
recently emerged as both diagnostic and thera- treatment with a stent [9, 10]. Any abscess that
peutic options in the setting of acute leaks. A is associated with a sleeve gastrectomy leak
covered (full or partial) self-expanding metal will require radiographic drainage if a stent is
stent is the ideal type to be utilized. being utilized as treatment. In a study of 21
B. Sleeve Gastrectomy Leaks: Most sleeve gas- SG leak cases, 71% of patients treated with a
trectomy leaks occur in the proximal third of stent noted leak closure in 55 days. Of the
the stomach (75–89%) [5], carry a mortality remaining six patients, five noted leak resolu-
rate of 0.11–9.0%, and occur at a mean of tion at 128 days [11].
7 days [6]. Risk factors for a leak include isch- D. Roux-en-Y Leaks: Leaks most commonly

emia at the proximal portion of the staple line, occur at the gastrojejunal anastomosis (70–
previous gastric surgery, and an area of steno- 80%), at the gastric pouch (10–15%), at the
jejuno-jejunal anastomosis (5%), and at the
gastric remnant (3–5%) [12]. Factors such as
S. Docimo Jr. (*) excess tension, staple-line malformation, and
Division of Bariatric, Foregut, and Advanced ischemia all play a role in leak occurrence.
Gastrointestinal Surgery, Stony Brook Medicine,
Stony Brook, NY, USA Evaluation of a suspected leak following a
e-mail: [email protected] Roux-en-Y gastric bypass includes an

S. Docimo, E. M. Pauli (eds.), Clinical Algorithms in General Surgery,
836 S. Docimo Jr.
upper gastrointestinal (GI) contrast study E. Postoperative Care: An upper GI is obtained

or a contrast computed tomography (CT) following placement of an endoscopic stent.
scan. An upper GI study has demonstrated a If no leak is noted, the patient is started on a
sensitivity and specificity of 79.4% and 95%, clear liquid diet. Stents are typically left in
whereas a contrast CT scan has demonstrated position for at least 2 weeks. Stent migration
a sensitivity and specificity of 95% and 100% is noted in 5–62% of cases [15–18] requiring
[13]. Leaks noted at the gastrojejunal anasto- endoscopic repositioning. A well-embedded
mosis are amenable to treatment with stent stent due to hyperplastic mucosal growth may
deployment. Jejuno-jejunal anastomosis be treated with argon plasma coagulation of
leaks are often not amenable to treatment the hyperplastic tissue. A second option
with a stent due to the difficulty to access the includes the “stent-in-stent” method whereby
site and also stent migration. A retrospective a new stent of the same diameter and length is
review of 35 Roux-en-Y patients who under- placed within the first stent. Radial tension on
went stent placement for gastrojejunal leaks the hyperplastic mucosa causes ischemia and
notes complete closure in 30 cases on con- necrosis. Both stents are then removed
trast studies [14]. 1–2 weeks later [19].
202 Acute Leak Following Bariatric Surgery: Endoscopic Stent Management 837
Recent history of a Roux-en-Y gastric bypass or sleeve

gastrectomy. Patient presents with complaints of abdominal pain.
A
· Vital signs: possible tachycardia, hypotension, fever, and tachypnea
· Laboratory analysis: leukocytosis
· Physical exam: likely will demonstrate abdominal pain
No
Patient Stable? · Resuscitation and operative intervention
B Yes
· Demonstration of a Leak within

· CT Scan with PO/IV Contrast
· Localize Leak if possible
the sleeve or gastro-jejunal D
C anastomosis
· Evaluate for an abscess
Yes
· CT guided drainage · Abscess?
No
· Endoscopic placement of a self-

· UGI to evaluate for leak occlusion
expanding covered metal stent
· Continued Leak
· Leak is occluded
E
· Endoscopic re-positioning or
placement of a second stent
· Any change in clinical status
warrants evaluation of stent
position with an UGI or X-ray
· Difficult removal due to mucosal

· Remove in 2 weeks.
hyperplasia during initial removal
· Argon plasma coagulation of hyperplastic mucosa and

immediate removal of stent
· Stent-in-stent with removalin 2 weeks of both stents
Algorithm 202.1
838 S. Docimo Jr.
References 10. Martin-Malagon A, Rodriguez-Ballester L, Arteaga-

Gonzalez I. Total gastrectomy for failed treatment
with endotherapy of chronic gastrocutaneous fis-
1. Whitlock KA, Gill RS, Ali T, et al. Early outcomes of
tula after sleeve gastrectomy. Surg Obes Relat Dis.
roux-en-Y gastric bypass in a publically funded obe-
2011;7:240–2.
sity program. ISRN Obes. 2013;2013:296597.
11. El Mourad H, Himpens J, Verhofstadt J. Stent treat-
2. van Rutte PW, Smulders JF, de Zoete JP, et al.
ment for fistula after obesity surgery: results in 47
Outcome of sleeve gastrectomy as a primary bariatric
consecutive patients. Surg Endosc. 2013;27:808–16.
procedure. Br J Surg. 2014;101(6):661–8.
12. Lo Menzo E, Szomstein S, Rosenthal RJ. Laparoscopic
3. Weiner RA, El-Sayes IA, Theodoridou S, et al.
gastric bypass: management of complications. In:
Early post operative complications: incidence, man-
Brethauer SA, Schauer PR, Schirmer BD, edi-
agement, and impact on length of hospital stay.
tors. Minimally invasive bariatric surgery. 2nd ed.
A retrospective comparison between laparoscopic
New York: Springer; 2015. p. 261–9.
gastric bypass and sleeve gastrectomy. Obes Surg.
13. Bingham J, Shawhan R, Parker R, Wigboldy J, Sohn
2013;23(12):2004–12.
V. Computed tomography scan versus upper gastroin-
4. Andrade JE, Martinez JM. Management of postsurgi-
testinal fluoroscopy for diagnosis of staple line leak fol-
cal leaks and fistulae. In: Thompson C, Ryan MB, edi-
lowing bariatric surgery. Am J Surg. 2015;209(5):810–4.
tors. Bariatric endoscopy. New York: Springer; 2013.
14. Salinas A, Baptista A, Santiago E, Antor M, Salinas
p. 91–101.
H. Self-expandable metal stents to treat gastric leaks.
5. Aurora AR, Khaitan L, Saber AA. Sleeve gastrectomy
Surg Obes Relat Dis. 2006;2:570–2.
and the risk of leak: a systematic analysis of 4,888
15. Puli SR, Spofford IS, Thompson CC. Use of self-
patients. Surg Endosc. 2012;26(6):1509–15.
expandable stents in the treatment of bariatric sur-
6. Sakran N, Goitein D, Raziel A, Keidar A, Beglaibter
gery leaks: a systematic review and meta-analysis.
N, Grinbaum R, et al. Gastric leaks after sleeve
gastrectomy: a multicenter experience with 2,834
16. Ross AS, Kozarek RA. Esophageal stents: indications
patients. Surg Endosc. 2013;27(1):240–5.
and placement techniques. Self-expandable stents in
7. Kim J, Azagury D, Eisenberg D, DeMaria E, Campos
GI tract. In: Kozarek R, Baron T, Song H, editors.
GM. ASMBS position statement on prevention, detec-
Self-expandable stents in the gastrointestinal tract.
tion, and treatment of gastrointestinal leak after gastric
bypass and sleeve gastrectomy, including the roles of
17. Eisendrath P, Cremer M, Himpens J, et al. Endotherapy
imaging, surgical exploration, and nonoperative man-
including temporary stenting of fistulas of the upper
agement. Surg Obes Relat Dis. 2015;11(4):739–48.
gastrointestinal tract after laparoscopic bariatric sur-
8. Prathanvanich P, Chand B. Laparoscopic sleeve
gery. Endoscopy. 2007;39:625–30.
gastrectomy: management of complications. In:
18. Efthimiou E, Stein L, Szego P, et al. Stent migration
Brethauer SA, Schauer PR, Schirmer BD, edi-
causing alimentary limb obstruction necessitating
tors. Minimally invasive bariatric surgery. 2nd ed.
laparotomy and surgical stent extraction. Surg Obes
Relat Dis. 2009;5:375–7.
9. de Aretxabala X, Leon J, Wiedmaier G, Turu I,
19. Aiolfi A, Bona D, Ceriani C, Porro M, Bonavina

Ovalle C, Maluenda F, Gonzalez C, Humphrey J,
L. Stent-in-stent, a safe and effective technique to
Hurtado M, Benavides C. Gastric leak after sleeve
remove fully embedded esophageal metal stents:
gastrectomy: analysis of its management. Obes Surg.
case series and literature review. Endosc Int Open.
2011;21:1232–7.
2015;3(4):E296–9.
Vitamin and Micronutrient
Deficiencies After Bariatric 203
Surgery
Algorithmic Approach should be completed in order to narrow one’s

differential diagnoses.
A preoperative assessment of micronutrients
B. Thiamin: Absorbed in the proximal small
prior to bariatric surgery demonstrated deficien- intestine, it plays a role in the metabolism of
cies of zinc (24.6%), vitamin B12 (18.1%), mag- carbohydrates and can quickly be depleted in
nesium (6.9%), phosphorous (4.7%), folic acid the setting of intractable vomiting. Prolonged
(3.4%), and vitamin D (25.4%) [1]. These miner- deficiency can lead to Wernicke’s encepha-
als are cofactors vital to functions of appetite, lopathy with concern for visual disturbances,
metabolism, nutrient absorption, glucose homeo- ataxia, peripheral neuropathy, memory loss,
stasis, and neural activities [2]. Procedures such and confusion. Thiamin supplementation,
as Roux-en-Y gastric bypass and biliopancreatic including IV infusion, is required [2].
diversion with or without duodenal switch are
C. Vitamin B12: Roux-en-Y gastric bypass
inherently at risk for deficiencies such as vitamin patients are at a high risk for vitamin B12
B12, calcium, iron, as well as vitamins A, D, E, deficiency due to incomplete digestion and
and K [2, 3]. However, sleeve gastrectomy release of B12 from proteinaceous food [2,
patients are not without nutrient deficiency [4]. 3]. Vitamin B12 deficiency measured at 1 and
3 years postoperatively in sleeve gastrectomy
A. The initial work-up of any patient under con- patients demonstrated a 10–26% prevalence
sideration for bariatric surgery should include [5]. Patients may experience parenthesis of
a full laboratory assessment of micronutrients. the limbs and macrocytic anemia. Treatment
Postoperatively, evaluation of micronutrients involves 700–2000 mcg of vitamin B12
should take place at 3 months. A patient with weekly [6].
a history of bariatric surgery who is newly D. Folic Acid: Folic acid is absorbed in the

enrolled into our program will undergo labo- proximal small bowel. Poor oral intake, lack
ratory evaluation for micronutrient derange- of adherence to vitamin regimen, malabsorp-
ments. A history and physical examination tion, and medications such as anticonvulsants
and oral contraceptives can lead to deficiency
[7]. Symptoms of deficiency lead to fatigue,
S. Docimo Jr. (*) headaches, diarrhea, and palpitations [2].
Division of Bariatric, Foregut, and Advanced Treatment involves 1 mg/day of folic acid [8].
Gastrointestinal Surgery, Stony Brook Medicine, E. Iron: Obesity itself increases the risk of iron
Stony Brook, NY, USA deficiency due to low-grade inflammation

840 S. Docimo Jr.
and induction of hepcidin, which blocks iron oxidase (electron transport chain), superox-
absorption proteins [9], making iron defi- ide dismutase (antioxidant), amine oxidases
ciency prevalent prior to surgery. Iron defi- (synthesis of neurotransmitter norepineph-
ciency has been noted in 10% of sleeve rine), and lysyl oxidase (involved in collagen
gastrectomy patients [5]. Symptoms of defi- crosslinking) [cousin RJ, allied health].
ciency include cravings for ice, pallor, leth- Severe deficiency of copper may present as
argy, koilonychias, and anemia [8]. Total unsteady gait, extremity numbness, parenthe-
iron-binding capacity or serum transferrin sis, or paralysis [cousins RJ]. In some cases,
receptors are better measures of iron defi- copper deficiency can be misdiagnosed as an
ciency compared with serum iron or ferritin iron or vitamin B12 deficiency, delaying
[10]. Supplementation of two daily vitamins proper copper replacement.
(for a total of 36 mg of iron) is sufficient. H. Zinc: Zinc is a cofactor for enzymes utilized
Anemia may require additional supplemen- in protein synthesis, digestion, immunity,
tation [2]. and gene transcription [14]. Zinc absorption
F. Calcium and Vitamin D: Calcium functions in requires an acidic environment and is absorbed
cell signaling and the mineralization of bone in the proximal intestines. Deficiency presents
and teeth and vitamin D functions in the as hair loss, poor wound healing, and changes
homeostasis of calcium via absorption of cal- of taste perception [10]. Excess zinc can cause
cium in the small intestines [11, 12]. Calcium sequestration of copper in the gut enterocytes,
deficiency can lead to low bone density, osteo- preventing the uptake of copper [14]. Zinc
porosis, muscle contractions, spasms, and supplementation requires 1 mg of copper for
parenthesis. Recommendations are for daily every 8–15 mg of zinc [13].
supplementation of 1200–1500 mg calcium I. All micronutrient derangements should be
and 3000 international units of vitamin D [13]. managed to improve any acute symptoms.
G. Copper: Copper is an essential cofactor in Long-term micronutrient replacement with
many enzymes that function in electron trans- daily vitamins is of critical importance and
fers. These enzymes include cytochrome c should be stressed for the patient.
203 Vitamin and Micronutrient Deficiencies After Bariatric Surgery 841
A patient undergoing bariatric surgery or presenting with acute

symptomatology not explained by an anatomical abnormality
· Any preoperative micronutrient and vitamin deficiencies should be replaced

· A history and physical examination is utilized to narrow the possible micronutrient deficiency
· Visual disturbance,
ataxia, peripheral Thiamin
B deficiency
neuropathy, memory
loss, confusion
· Parathesias of limbs,
C macrocytic anemia Vitamin B12
deficiency
D · Fatigue, headache,
diarrhea, palpatations Folic Acid
deficiency
· Pallor, lethargy,
Draw blood Iron
E koilonychias, anemia,
samples deficiency
craving for ice
· Low bone density, Calcium &

F osteoporosis, muscle vitamin D
contractions, spasms deficiency
· Unsteady gait, extremity

G numbness, parathesiasis, Copper
paralysis deficiency
· Hair loss, poor wound

H healing, change in taste Zinc
perception deficiency
· Treat all acute deficiencies

with replacement
I · Maintenancewith daily
vitamin use
Algorithm 203.1
842 S. Docimo Jr.
References 9. Cepeda-Lopez AC, Aeberli I, Zimmermann MB. Does

obesity increase the risk for iron deficiency? A review
of the literature and the potential mechanisms. Int J
1. Ernst B, Thurnheer M, Schmid SM, Schultes
Vitam Nutr Res. 2010;80:263–70.
B. Evidence for the necessity to systematically assess
10. Zimmermann MB, Hurrell RF. Nutritional iron defi-
micronutrient status prior to bariatric surgery. Obes
ciency. Lancet. 2007;370:511–24. Hoffman HNI,
Surg. 2009;19(1):66–73.
Phyliky RL, Fleming CR. Zinc-induced copper defi-
2. Allied Health Sciences Section Ad Hoc Nutrition
ciency. Gastroenterology. 1988;94:508–12.
Committee, Aills L, Blankenship J, Buffington C,
11. Wood RJ. Calcium and phosphorus. In: Stipanuk

Furtado M, Parrott J. ASMBS allied health nutritional
MH, editor. Bio- chemical and physiological aspects
guidelines for the surgical weight loss patient. Surg
of human nutrition. Philadelphia: Elsevier; 2000.
Obes Relat Dis. 2008;4(5 Suppl):S73–108.
p. 643–70.
3. Bloomberg RD, Fleishman A, Nalle JE, Herron
12. Weaver CM, Fleet JC. Vitamin D requirements: cur-
DM, Kini S. Nutritional deficiencies following bar-
rent and future. Am J Clin Nutr. 2004;80:S1735–9.
iatric surgery: what have we learned? Obes Surg.
13. Mechanick JI, Youdim A, Jones DB, Garvey WT,

2005;15(2):145–54. Review.
Hurley DL, McMahon MM, Heinberg LJ, Kushner
4. Schweiger C, Weiss R, Keidar A. Effect of different
R, Adams TD, Shikora S, et al. Clinical practice
bariatric operations on food intolerance and quality of
guidelines for the perioperative nutritional, meta-
eating. Obes Surg. 2010;20:1393–9.
bolic, and nonsurgical support of the bariatric sur-
5. Himpens J, Dapri G, Cadière GB. A prospective, ran-
gery patient-2013 update: cosponsored by American
domized study between laparoscopic gastric banding
Association of Clinical Endocrinologists, The
and laparoscopic isolated sleeve gastrectomy: results
Obesity Society, and American Society for Metabolic
after 1 and 3 years. Obes Surg. 2006;16(11):1450–6.
and Bariatric Surgery. Obesity (Silver Spring).
6. Kaplan LM. Pharmacological therapies for obesity.
2013;21(Suppl 1):S1–27.
Gastroenterol Clin N Am. 2005;34:91–104.
14. Cousins RJ, Blanchard RK, Moore JB, Cui L,

7. Charney P, Malone A, editors. ADA pocket guide to
Green CL, Liuzzi JP, Cao J, Bobo JA. Regulation
nutrition assessment. Chicago: American Dietetic
of zinc metabolism and genomic outcomes. J Nutr.
Association; 2004.
2003;133:S1521–6.
8. Malinowski SS. Nutritional and metabolic com-
plications of bariatric surgery. Am J Med Sci.
2006;331:219–25.
Part XXIV
Pregnancy and General Surgery
Pregnancy and Cholelithiasis
204
Jaimey M. Pauli
Algorithmic Approach epigastric pain, anorexia, intolerance of fatty

foods, fever, elevated white blood cell count,
A. Gallstones are present in 1–3% of pregnant elevated amylase and lipase, elevated AST
women, although approximately 50% of and ALT, jaundice, and positive Murphy’s
them are asymptomatic [1]. Pregnant and sign (pain with deep inspiration on palpation
postpartum women are predisposed to gall- or ultrasound examination of the right upper
stone formation due to the hormonal effects quadrant in the area of the gallbladder fossa).
of estrogen and progesterone that increase It is imperative that other potentially life-
cholesterol secretion and decrease soluble threatening pregnancy-related diagnoses are
bile acid secretion. Additionally, progester- considered on the differential of right upper
one also decreases smooth muscle contractil- quadrant pain, including preeclampsia;
ity and slows gallbladder emptying. Up to hemolysis, elevated liver enzymes, and low
30% of pregnant women have biliary sludge, platelet count (HELLP) syndrome; placental
which develops into gallstones, on ultra- abruption; uterine rupture; acute fatty liver
sound. Risk factors for cholelithiasis include of pregnancy; and myocardial infarction [4].
obesity, multiparity, and increasing gesta- Fetal monitoring should occur during evalu-
tional age [2]. Complications of gallbladder ation as appropriate for gestational age per
disease are the second most common reason obstetrics.
for non-obstetric surgery in the pregnant C. Ultrasound has 95–98% accuracy in diagnos-
patient and the most common non-obstetric ing cholelithiasis [2, 5]. Findings of cholecys-
cause of hospitalization in the first postpar- titis are thickened gallbladder wall (>3–5 mm)
tum year [2, 3]. or edema, presence of gallstones, perichole-
B. Gallstones cause biliary colic, acute chole- cystic fluid, and a sonographic Murphy’s sign
cystitis, gallstone pancreatitis, choledocho- [1, 5]. Other imaging that may be useful for
lithiasis, gallstone ileus, and cholangitis. the diagnosis and treatment of small or extra-
Signs and symptoms include intermittent hepatic stones includes endoscopic retro-
right upper quadrant pain, nausea, vomiting, grade cholangiopancreatography (ERCP),
which has minimal radiation exposure (300
J. M. Pauli (*) mrad) in an acceptable range for pregnancy
Maternal-Fetal Medicine, Department of Obstetrics after the first trimester [1]. Non-contrast
and Gynecology, Penn State Health Milton S.
Hershey Medical Center, Hershey, PA, USA

846 J. M. Pauli
magnetic resonance cholangiopancreatogra- spectrum antibiotics for patients with sys-

phy (MRCP) may also be considered in com- temic signs [1, 4–6].
plex cases. E. Lu et al. demonstrated that if conservative
D. Surgical intervention via laparoscopic or
management is chosen for gallstone disease
open cholecystectomy (see algorithm for in pregnancy, there is a 38% chance of sub-
“Cholecystectomy of the Pregnant Patient”) optimal outcome, with a 34% risk of relapse
is clearly indicated for obstructive jaundice, and increased severity of disease at relapse
acute cholecystitis failing medical manage- [6]. Although there is a higher risk for pre-
ment (approximately one-fourth of patients term contractions with operative manage-
with cholecystitis), gallstone pancreatitis, ment, there is a higher rate of preterm
or suspected peritonitis [3, 6]. The appropri- delivery, induction of labor for symptoms,
ate management of symptomatic cholelithi- and cesarean delivery with non-operative
asis and biliary colic is less clear, particularly management. Consideration for definitive
since there is a hesitancy to operate on preg- surgical management of gallstone disease
nant patients [2, 6]. Conservative manage- during pregnancy, especially in the first and
ment includes intravenous hydration, bowel second trimesters, has further been supported
rest, and pain management, with broad- by other authors [1].
204 Pregnancy and Cholelithiasis 847
History:
A Pregnant
Right upper quadrant pain +/– nausea/vomiting/fever
Obtain vital signs, blood work, and perform a physical examination

B Consider fetal monitoring depending on gestational age
+ Fever, leukocytosis, abnormal labs

+ Murphy’s sign
No evidence of pregnancy related diseases(HELLP,
preeclampsia, etc.)
Ultrasound
C
Thickened gallbladder wall or edema No gross abnormalities:

Gallstones consider ERCP or MRCP to
Pericholecystic fluid evaluate/treat small stones or
Sonographic Murphy’s sign extrahepatic stones
D
Obstructive jaundice Biliary colic

Gallstone pancreatitis Acute cholecystitis
Peritonitis
Immediate Conservative management:

cholecystectomy IVF, bowel rest, narcotics
(open or laparoscopic) +/– antibiotics
E Symptoms worsen or recur

Symptoms improve:
plan postpartum followup
Algorithm 204.1
848 J. M. Pauli
References 4. Sharp HT. The acute abdomen during pregnancy. Clin

Obstet Gynecol. 2002;45(2):405–13.
5. Diegelmann L. Nonobstetric abdominal pain and sur-
1. Gilo NB, Amini D, Landy HJ. Appendicitis and
gical emergencies in pregnancy. Emerg Med Clin N
cholecystitis in pregnancy. Clin Obstet Gynecol.
Am. 2012;30:885–901.
2009;52(4):586–96.
6. Lu EK, Curet MJ, El-Sayed YY, Kirkwood KS. Medical
2. Williamson C, Mackillop L, Heneghan MA. Diseases
versus surgical management of biliary tract disease in
of the liver, biliary system, and pancreas. In: Creasy
pregnancy. Am J Surg. 2004;188:755–9.
and Resnik’s maternal fetal medicine: principles and
practice. 7th ed. Philadelphia: Elsiever; 2014.
3. Date RS, Kaushal M, Ramesh A. A review of the
management of gallstone disease and its complica-
tions in pregnancy. Am J Surg. 2008;196(4):599–608.
Pregnancy and Appendicitis
205
Emily Smith and Jaimey M. Pauli
Algorithmic Approach right lower lobe pneumonia [4]. Consider an

obstetrical consult for fetal evaluation and
A. Evaluation of possible appendicitis in preg- evaluation for obstetrical or gynecologic
nancy begins with a thorough history and etiologies.
physical examination. Right lower quadrant C. Further imaging in pregnancy to demonstrate
pain, even in pregnancy, is still the most com- an inflamed appendix is complicated by the
mon presenting symptom (regardless of ges- need to minimize radiation exposure to the
tational age) [1, 2]. However, the differential fetus [5]. Ultrasound is the first-choice imag-
diagnosis should also include cholecystitis, ing modality. Unfortunately, ultrasound accu-
pancreatitis, gastroenteritis, right lower lobe racy may be operator dependent, and as
pneumonia, ovarian torsion, uterine myoma gestational age increases, the appendix is fre-
degeneration, pyelonephritis, urinary tract quently not visualized [5]. MRI without gad-
infection (UTI), and round ligament pain [3]. olinium has no known adverse fetal effects
B. Vital signs, blood work, and physical exami- [6]. It also has a high negative predictive
nation are the foundation of diagnosis; how- value [7].
ever, in pregnancy, some abnormalities, such D. Treatment for appendicitis in pregnancy is
as mild tachycardia and leukocytosis, can be appendectomy [8]. Early intervention (opera-
normal physiologic changes [1]. Additionally, tion within 24 h of presentation) is associated
the Rovsing and Psoas signs are not clinically with better outcomes [9]. Unlike non-
significant [4]. Urine analysis should be con- pregnant appendicitis, conservative manage-
sidered in order to eliminate urinary tract ment with antibiotics is not currently
infection as the source of symptoms. Chest considered appropriate [8]. More extensive
X-ray should also be considered to rule out studies in support of conservative manage-
ment are currently ongoing [8].
E. The surgical approach may be either open or
E. Smith laparoscopic depending on both surgeon
Maternal-Fetal Medicine, Department of Obstetrics preference and the size of the gravid uterus,
and Gynecology, Medical College of Wisconsin,
Milwaukee, WI, USA
which limits the placement of laparoscopic
ports [10]. If there is a concern for uterine
J. M. Pauli (*)
Maternal-Fetal Medicine, Department of Obstetrics
injury when placing Veres needle for insuffla-
and Gynecology, Penn State Health Milton S. tion, a Hassan/open entry should be consid-
Hershey Medical Center, Hershey, PA, USA ered [11]. Other considerations are to use a

850 E. Smith and J. M. Pauli
midline incision if peritonitis is suspected increased risk of preterm delivery or fetal

and to keep intraabdominal pressures loss [8]. Appendiceal perforation, which is
<12 mmHg during laparoscopy. All patients most likely to occur in the third trimester,
for appendectomy should receive preoperative determines the risk of fetal loss (1.5%
antibiotics (second-generation cephalospo- without perforation versus up to 36% with
rin, extended-spectrum penicillin, or triple- perforation) [9]. Although the rate of pre-
agent therapy) [8]. Continuous fetal term contractions after appendectomy is as
monitoring during surgery and corticosteroid high as 83%, the rate of preterm labor and
administration for fetal lung maturity after delivery is much lower at 5–14% [9].
fetal viability (generally defined as Tocolytic therapy, although not proven to
23–24 weeks) should be considered if feasi- be effective, may be considered by the
ble. Preparations for emergent cesarean deliv- obstetrical consultants. Monitoring for
ery if indicated should be made by the fetal well-being and labor symptoms
obstetrical service. should occur in the postoperative period,
F. Women diagnosed with appendicitis in with frequency and intensity determined
pregnancy should be counseled about the by gestational age.
205 Pregnancy and Appendicitis 851

A Right lower quadrant pain in pregnancy
Obtain vital signs, blood work and perform a physical

examination
B Consider chest x-ray and urinalysis
Obstetrical consult
Fetal monitoring
Unable to suggest etiology other than appendicitis. Obtain imaging.
C Ultrasound
Normal ultrasound Unable to visualize appendix

(appendix visualized) and no other pelvic pathology
Continue to monitor
Consider other MRI
etiologies
Abnormal appendix Normal appendix
Continue to monitor
D Pre-operative antibiotics
Consider other
Corticosteroids
Appendectomy (open or laparoscopic) etiologies
Fetal monitoring
E
Postoperative care:
Counseling about risk of fetal loss or preterm labor
F Fetal monitoring
Tocolytic therapy if indicated
Algorithm 205.1
852 E. Smith and J. M. Pauli
References 2004 update and revisions. AJR Am J Roentgenol.

2004;182:1111–4.
7. Long SS, Long C, Lai H, et al. Imaging strategies for
1. Mourad J, Elliott JP, Erickson L, et al. Appendicitis
right lower quadrant pain in pregnancy. AJR Am J
in pregnancy: new information that contradicts
Roentgenol. 2011;196:4–12.
long-held clinical beliefs. Am J Obstet Gynecol.
8. De Franca NA, Ramos do Amorim M, Nobrega
2000;182:1027–9.
SV. Acute appendicitis in pregnancy: literature
2. Tracey M, Fletcher HS. Appendicitis in pregnancy.
review. Rev Assoc Med Bras. 2015;61(2):170–7.
Am Surg. 2000;66:555–9.
9. Sharp HT. The acute abdomen during pregnancy. Clin
3. Weingold AB. Appendicitis in pregnancy. Clin Obstet
Obstet Gynecol. 2002;45(2):405–13.
Gynecol. 1983;26:801–9.
10. Affleck DG, Handrahan DL, Egger MJ, et al. The lap-
4. Al-Mulhim AA. Acute appendicitis in pregnancy: a
aroscopic management of appendicitis and cholelithi-
review of 52 cases. Int Surg. 1996;81:295–7.
asis during pregnancy. Am J Surg. 1999;178:523–9.
5. Williams R, Shaw J. Ultrasound scanning in the diag-
11. Friedman JD, Ramsey PS, Ramin KD, et al.

nosis of acute appendicitis in pregnancy. Emerg Med
Pneumoamnion and pregnancy loss after second-
J. 2007;24:359–60.
trimester laparoscopic surgery. Obstet Gynecol. 2002;
6. Kanal E, Borgstede JP, Barkovich AJ, et al. American
99:512–3.
College of Radiology White Paper on MR safety:
Pregnancy and Breast Cancer
206
James M. O’Brien and Jaimey M. Pauli
Algorithmic Approach Mammography is not contraindicated in

pregnancy as long as there is abdominal
A. The definition of pregnancy-associated breast shielding, although breast ultrasound is often
cancer is cancer diagnosed during pregnancy the first image modality ordered in pregnancy
itself, within the first postpartum year, or any [2]. MRI itself is not contraindicated,
time during lactation, and has an incidence of although gadolinium should be avoided in
15–35/100,000 deliveries [1, 2]. Breast can- pregnancy due to its association with adverse
cer remains one of the most common cancers fetal and neonatal events. Other diagnostic
in the pregnant population, with up to 20% of methods such as chest X-ray with abdominal
breast cancer in women less than 30 years shielding and breast biopsy remain appropri-
being associated with pregnancy [3]. The ate, while computed tomography (CT) scans
majority of breast cancer associated with are generally avoided due to their high level
pregnancy is infiltrating ductal adenocarci- of radiation exposure. Breast and axillary
noma and is predominately poorly differenti- lymph node dissection during any trimester
ated and advanced at the time of diagnosis. of pregnancy is associated with minimal fetal
There is additionally a lower incidence of risk [7]. However, sentinel lymph node biop-
hormone receptor positivity seen in sies during pregnancy remain controversial
pregnancy-associated breast cancer [4, 5]. and are not currently recommended [8].
B. Diagnosis is often challenging due to normal C. Treatment should not be delayed secondary to
physiological changes which occur in breast pregnancy; therefore, a treatment plan should
tissue during pregnancy such as engorgement be made by a multidisciplinary team of mater-
and hypertrophy. Rarely an early diagnosis is nal fetal medicine, breast surgery, and oncol-
suspected and aided by a phenomenon called ogy. Although a difficult conversation,
the milk rejection sign, in which a nursing termination should be discussed and offered as
infant refuses breast milk with occult carci- an option prior to initiating treatment, although
noma [6]. elective termination of pregnancy has not been
shown to improve maternal outcomes [9].
Treatment should be approached similarly
J. M. O’Brien · J. M. Pauli (*) to non-pregnant women, with certain modifi-
Maternal-Fetal Medicine, Department of Obstetrics cations and considerations. Mastectomy is an
and Gynecology, Penn State Health Milton S. appropriate treatment option, while radiation
Hershey Medical Center, Hershey, PA, USA therapy is contraindicated due to associated

854 J. M. O’Brien and J. M. Pauli
fetal risks of miscarriage, congenital malfor- cancer is diagnosed early and appropriately
mations, development and growth alterations, treated. The risk of vertical transmission to
and carcinogenic effects [10]. Chemotherapy the fetus remains unknown, but metastasis to
in the second and third trimester of pregnancy the placenta is rarely encountered [13].
has been associated with intrauterine growth Studies have demonstrated that survival is
restriction, prematurity, and low birth weight not negatively affected with breast cancer
in approximately 50% of exposed fetuses being diagnosed during the pregnant state,
[11]. However, there appears to be a low level that women who become pregnant after
of neonatal complications with in utero expo- undergoing treatment for breast cancer do
sure to chemotherapy [12]. not worsen their prognosis, and that preg-
D. Long-term prognosis for both the mother
nancy after breast cancer may in fact have a
and fetus appears to be reassuring if breast protective effect [14–16].
206 Pregnancy and Breast Cancer 855
Non physiologic breast changes during pregnancy, first year post-

A partum, or during lactation
B Imaging
Chest X ray Mammography Breast MRI (without

with abdominal with abdominal ultrasound gadolinium if
shielding shielding pregnant)
Continued suspicion of pregnancy associated breast cancer
Breast and axillary lymph node biopsy
Diagnosis of pregnancy associated breast cancer
Is treatment Is termination
No during of pregnancy
Yes
pregnancy desired?
desired?
Treatment after
delivery
Treatment No Yes
Chemotherapy Treatment after

D after appropriate Mastectomy termination
counseling
Algorithm 206.1
856 J. M. O’Brien and J. M. Pauli
References sus conference of the role of sentinel lymph node

biopsy in carcinoma of the breast, April 19-22, 2001,
Philadelphia, Pennsylvania. Cancer. 2002;94:2542.
1. Wallack MK, Wolf JA Jr, Bedwinek J, et al. Gestational
9. Nugent P, O’Connell TX. Breast cancer and preg-
carcinoma of the female breast. Curr Probl Cancer.
nancy. Arch Surg. 1985;120:1221.
1983;7:1.
10. Kal HB, Struikmans H. Radiotherapy during preg-
2. Liberman L, Giess CS, Dershaw DD, et al. Imaging
nancy: fact and fiction. Lancet Oncol. 2005;6:328.
of pregnancy associated breast cancer. Radiology.
11. Cardonick E, Iacobucci A. Use of chemotherapy dur-
1994;191:245.
ing human pregnancy. Lancet Oncol. 2004;5:283.
3. Antonelli NM, Dotters DJ, Katz VL, Kuller JA. Cancer
12. Giacalone PL, Laffargue F, Benos P. Chemotherapy
in pregnancy: a review of the literature. Part I. Obstet
for breast carcinoma during pregnancy: a French
Gynecol Surv. 1996;51:125.
national survey. Cancer. 1999;86:2266.
4. Anderson BO, Petrek JA, Byrd DR, et al. Pregnancy
13. Dessolle L, Dalmon C, Roche B, Darai E. Placental
influences breast cancer stage at diagnosis in women
metastases from maternal malignancies: review of
30 years of age and younger. Ann Surg Oncol.
the literature. J Gynecol Obstet Bio Reprod (Paris).
1996;3:204.
2007;36:344.
5. Stensheim H, Moller B, van Dijk T, Fossa SD. Cause
14. Amant F, von Minckwitz G, Han SN, et al. Prognosis
specific survival for women diagnosed with can-
of women with primary breast cancer diagnosed dur-
cer during pregnancy and lactation: a registry based
ing pregnancy: results from an international collab-
cohort study. J Clin Oncol. 2009;27:45.
orative study. J Clin Oncol. 2013;31:2532.
6. Saber A, Dardik H, Ibrahim IM, Wolodiger F. The
15. Mueller BA, Simon MS, Deapen D, et al. Childbearing
milk rejection sign: a natural tumor marker. Am Surg.
and survival after breast carcinoma in young women.
1996;62:998.
Cancer. 2003;98:1131.
7. Woo JC, Yu T, Hurd TC. Breast cancer in pregnancy:
16. Azim HA Jr, Santoro L, Pavlidis N, et al. Safety of
a literature review. Arch Surg. 2003;138:91.
pregnancy following breast cancer diagnosis. A meta-
8. Schwartz GF, Giuliano AE, Veronesi U, Consensus
analysis of 14 studies. Eur J Cancer. 2011;47:74.
Conference Committee. Proceedings of the consen-
Pregnancy and Hernia
207
Jaimey M. Pauli
Algorithmic Approach fetus during surgery, the risk of recurrence as

the abdominal wall expands during preg-
A. Abdominal wall hernias (umbilical, inguinal, nancy, and concern for limited abdominal
femoral, incisional, ventral, parastomal, etc.) wall stretching in a subsequent pregnancy
are defects in the fascia that can develop as a have all been cited as reasons for the delay.
result of increased intraabdominal pressure C. Evaluation of the patient with a suspected
and tension on the abdominal wall, both of hernia should include a history and physical
which are increased during pregnancy [1]. exam, with attention to history of prior sur-
This may lead to discomfort during a preg- geries (for incisional hernias) and bowel
nancy in a previously undiagnosed or asymp- symptoms such as vomiting, distention, or
tomatic hernia [2]. Symptoms of pain and absence of flatus or stool passage. Generally,
obstruction occur when intraabdominal con- there is a lump or palpable defect with con-
tents, such as peritoneum, omentum, bowel, tents that may or may not be reducible. The
or other visceral organs, protrude through the presence of skin erythema or tenderness indi-
hernia; however, the growing uterus may cates possible bowel perforation [4].
block these from protruding. A very rare Ultrasound is not often required but may be
complication occurs when the gravid uterus used to confirm the diagnosis and rule out
itself prolapses through the hernia (usually other conditions such as round ligament vari-
incisional or umbilical), which can cause cosities, which mimic a groin hernia on exam
uterine strangulation, fetal demise, uterine but have a characteristic appearance of a “bag
rupture, preterm labor, hemorrhage, and skin of worms” on color Doppler that become
necrosis [3]. more prominent with Valsalva [4, 5].
B. Hernia repair is one of the most common sur- D. Pregnant patients should undergo emergent

geries in the nonpregnant population but tra- hernia repair for the same indications as non-
ditionally has been postponed until the pregnant patients—bowel incarceration,
postpartum period and preferably until child- strangulation, or suspected perforation [2, 4].
bearing is complete. The potential risks to the Due to the risk of worsening symptoms or
incarceration that may increase the risk of
J. M. Pauli (*) perinatal complications, symptomatic irreduc-
Maternal-Fetal Medicine, Department of Obstetrics ible umbilical hernias should be urgently
and Gynecology, Penn State Health Milton S. repaired and asymptomatic irreducible umbil-
Hershey Medical Center, Hershey, PA, USA ical hernias should be semi-urgently repaired

858 J. M. Pauli
during pregnancy [4]. Monitoring for fetal the ultimately preferred method. A review of
well-being and signs and symptoms of labor the literature suggests that laparoscopic
should occur as appropriate for gestational age repair may be used safely in pregnancy (as it
in the perioperative period per obstetrical is with appendectomy and cholecystectomy)
recommendations. and has the advantages of smaller incisions,
E. Although the data are limited, more recent shorter hospital stay, earlier mobilization,
reviews indicate that elective hernia repair etc. [2, 4, 7]. The use of mesh is associated
during pregnancy should be considered to with a lower hernia recurrence rate [2, 4, 6,
avoid worsening symptoms or incarceration 8]. The risk of obstetrical complications
during the pregnancy that could lead to emer- does not appear higher in pregnancies after
gent surgery with higher perinatal complica- hernia repair, but increased abdominal wall
tions [2]. The decision to proceed with a pain has been noted in the third trimester,
non-urgent hernia repair during pregnancy purportedly due to decreased elasticity of
should take into account several consider- the repair abdominal wall. Combined repair
ations: gestational age (with the second tri- of small inguinal and umbilical hernias at
mester being the ideal time to operate), the the time of cesarean has also been reviewed
likelihood of hernia recurrence causing in small case series with the advantage of
symptoms during pregnancy, the risk of her- convenience and saved time and cost, with
nia recurrence requiring reoperation, the risk no difference in outcomes beyond longer
of pregnancy complications related to the sur- operating times [9]. Until larger, random-
gery, and the risk of complications in a future ized prospective trials are performed to
pregnancy as a result of the hernia repair [6]. determine the best way to approach these
F. Both open and laparoscopic hernia repairs, patients, the repair will be determined by the
with and without mesh, have been performed clinical judgment and experience of the sur-
during pregnancy, with little consensus on geon performing it.
207 Pregnancy and Hernia 859
A
History:
Abdominal “lump”or discomfort Known hernia
Nausea/vomiting/distention/absence of flatus or stool
B Prior surgery
C Obtain vital signsandperform a physical examination
Palpable Yes No Perforation

Irreducible
lump or or
(umbilical)?
defect? obstruction?
Yes
D OR Yes
No
Skin Yes
erythema or Urgent
tenderness? Hernia repair
No
Consider ultrasound for further

evaluation
Hernia Yes Evaluate for elective hernia

present? repair
E
No
Evaluate for other conditions
Start antibiotics, NPO, IVF, CT-guided drainage
Pain for over 24 hours, a wbc greater than 15, and temperature exceeding 39.4 C are
concerns for a perforated appendix. Obtain a CT Scan.
· Discharge home on antibiotics.

· Colonoscopy followed by interval
appendectomy is recommended
Algorithm 207.1
860 J. M. Pauli
References 5. Uzun M, Akkan K, Coskun B. Round ligament vari-

cosities mimicking inguinal hernias in pregnancy:
importance of color Doppler sonography. Diagn
1. Wai PY, Ruby JA, Davis KA, Roberts AC, Roberts
Interv Radiol. 2010;16:150–2.
KE. Laparoscopic ventral hernia repair during preg-
6. Schoenmaeckers E, Stirler V, Raymakers J, Rakic
nancy. Hernia. 2009;13:559–63.
S. Pregnancy following laparoscopic mesh repair of
2. Jensen KK, Henriksen NA, Jorgensen LN. Abdominal
ventral abdominal wall hernia. JSLS. 2012;16:85–8.
wall hernia and pregnancy: a systematic review.
7. Bisharah M, Tulandi T. Laparoscopic surgery in preg-
Hernia. 2015;19:689–96.
nancy. Clin Obstet Gynecol. 2003;46(1):92–7.
3. Banerjee N, Deepika D, Sinha A, Prasad R. Gravid
8. Buch KE, Tabrizian P, Divino CM. Management
uterus in an incisional hernia. J Obstet Gynaecol Res.
of hernias in pregnancy. J Am Coll Surg.
2001;27(2):77–9.
2008;207(4):539–42.
4. Augustin G, Matosevic P, Kekez T, Majerovic M,
9. Ochsenbein-Kölble N, Demartines N, Ochsenbein-
Delmis J. Abdominal hernias in pregnancy. J Obstet
Imhof N, Zimmermann R. Cesarean section and simul-
Gynaecol Res. 2009;35(2):203–11.
taneous hernia repair. Arch Surg. 2004;139:893–5.
Index
A Adrenal vein sampling (AVS), 466

Abdominal aortic aneurysms (AAA), 551, 552 Adrenalectomy, 449, 454
Abdominal bruising, 669 Adrenocorticotropic hormone (ACTH), 449
Abdominal compartment syndrome (ACS), 735, 736 Adson test, 589
Abdominal evaluation, 615 Advanced cardiac life support (ACLS), 632, 634, 637
Abdominal injuries, concurrent, 661 Advanced trauma life support (ATLS)
Abdominal pain, 195, 225, 237, 391, 821 bladder injury, 683
Abdominal perfusion pressure (APP), 735, 736 blunt aortic injury, 641
Abdominal plain X-ray, 501 blunt cardiac injury, 637
Abdominal roentgenograms, 645 blunt chest wall trauma, 633
Abdominal trauma, 665 diaphragmatic injury, 653
Abdominal wall hernia, 857, 858 ED thoracotomy, 632
Abdominal wall reconstruction, 191, 192, 813, 814 pelvic fracture, 679
ABI, see Ankle brachial index (ABI) penetrating abdominal trauma, 645
Abscess drainage, 325 penetrating chest trauma, 627
Absent esophageal contractility, 120 penetrating neck trauma, 623
Acarbose, 157 splenic injury, 665
Accelerated partial breast irradiation (APBI), 91 traumatic brain injury, 619
Achalasia, 123 Aganglionosis, 513, 514
Acholic stools, 541 Airway compromise, 17, 18, 431
Acid-base disorder, 751, 752 Airway management, 43, 707, 708
ACS, see Abdominal compartment syndrome (ACS) Airway, breathing, circulation, disability and exposure
Acute abdomen, 225 (ABCDE), 633
Acute acalculous cholecystitis (AAC), 349 AKI, see Acute kidney injury (AKI)
Acute appendicitis, 225, 226 Alcohol, 17
Acute calculous cholecystitis (ACC), 345, 346 abuse, 609
Acute cholangitis, 355, 359 Aldosterone, 453
Acute deep vein thrombosis (DVT), 577 Aldosterone to renin ratio (ARR), 453
Acute kidney injury (AKI), 669, 670 Aldosteronoma, 465, 466
diagnosis, 739 ALI, see Acute limb ischemia (ALI)
etiology, 739 Allergies, Medications, Past Medical History,
Acute leak, 835, 836 Last Meal, and Events or Mechanism of
Acute limb ischemia (ALI), 565 injury (AMPLE), 657
Acute mesenteric ischemia, 581 Alpha-blockade, 462
Acute pancreatitis (AP), 379, 380, 403 American Association for the Study of Liver Diseases
Acute renal failure, 739, 740 (AASLD), 308
Acute respiratory distress syndrome (ARDS), 719 American Association for the Surgery of Trauma
sepsis, 724 (AAST), 657
Adenocarcinoma, 181 American Urological Association, 601
Adenomatous colon polyp, 251 Amoebic liver abscess, 324–325
Adjuvant therapy, male breast cancer, 114 Anal cancer, 301
Adrenal crisis, 727 Anal fissure, 293, 294
Adrenal insufficiency, 727 Anal margin, 301
Adrenal masses, 465 Anaphylaxis, 727

https://doi.org/10.1007/978-3-319-98497-1
862 Index
Androgen deprivation therapy, 602 Biliary atresia, 541, 542

Angioembolization, 657, 658, 666 Biliary colic, 346
Angiography Biliary cysts, see Choledochal cyst (CC)
pulmonary emboli, 743 Biliary disease, 199
with/without embolization, 665 Biliary drainage procedure, 396
Angiomyolipoma (AML), 597 Biliary ducts, 335
Anion gap, 751 Biliary leak, 351
Ankle brachial index (ABI), 569, 573 Biliary reflux, 159
Anoplasty, 510 Biliary tract, 362, 365, 366, 373
Anorectal malformations (ARMs), 509, 510 Bilious emesis, 505
Anorectal physiology, 303 Biloma, 352
Anterior injuries, 627, 675 Biofeedback therapy, 303
Anterolateral thoracotomy, 627 Biologic mesh, 798, 809
Antibiotics, 325, 609 Biomarker analysis, male breast cancer, 114
Anticoagulation, 702, 743, 744 Bladder injury, 683, 684
acute DVT, 577 Bleb, 59
thoracic outlet syndrome, 589 Blood glucose, 473
Anti-diarrheals, 303 Blood pressure, intracranial hematoma, 702
Anti-HER-2 treatment, 106 Blood transfusions, 731, 732
Anti-HER-2/neu therapy, 101, 102 Bloody diarrhea, 229, 237
Antimicrobial therapy, 723 Blunt abdominal trauma, 649, 650
Antireflux surgery, 127 Blunt aortic injury, 633, 641, 642
Anti-TNF agents, 230 Blunt cardiac injury (BCI), 634, 637
Antral G-cell hyperplasia, 162 Blunt chest wall trauma, 633, 634
Aortic dissection, 559, 560 Blunt injury, 631, 687
Aortogram, 585 Blunt scrotal trauma, 605
Aperistalsis, 123 Blunt traumatic cardiac arrest, 631
Appendicitis, 225, 226, 849, 850 Blunt traumatic injury, 615, 616
ARDS, see Acute respiratory distress syndrome (ARDS) Bochdalek hernia, 495
Arrhythmias, 637 Bogota bag technique, 810
Arterial blood gas analysis, 751 Bosniak classification system, 597
Arterial embolization, 316 Botulinum toxin injection, 119, 120, 293–294
Arterial occlusive disease, 585 Bowel obstruction, 525, 825
Arterial TOS, 589 Brachytherapy, 44
Arterial venous (AV) shunt, 593 Brain cell shrinkage, 761
Atraumatic indications, for splenectomy, 413, 414 BRCA2 mutation, 113, 114
Atypical fissure, 293 Breast cancer
Autologous islet cell transplantation, 384 inflammatory, 105, 106
Autologous tissue reconstruction, 110 locoregional recurrence of, 97, 98
Autosomal dominant polycystic kidney disease metastatic, 87, 101, 102
(ADPKD), 313 pregnancy, 853, 854
Axillary lymph node, enlarged, 85 recurrent, 91, 92
Breast conservation surgery (BCT), 95, 97
Breast Imaging Reporting and Data System (BIRADS),
B 73, 74
Balloon expandable stent, 586 Breast mass evaluation, 73, 74
Bariatric surgery, 819, 821 Breast reconstruction, 109–111
acute leak, 835, 836 Broad spectrum antibiotics, 11
vitamin/micronutrient deficiencies, 839, 840 Bronchoscopy for bleeding, 35
work-up of abdominal pain, 821, 822 Brooke formula, 748
Barium esophagram, 119, 123 Bullectomy, 59
Barker bag technique, 810 Burns, 747, 748
Barrett’s esophagus, 127
Basal cell carcinoma, 5
Baseline severity score, 701 C
Bell’s Staging Criteria, 534 CA19-9, 399
Benign cystic masses, 605 Calcium, 771, 772
Benign liver masses, 319, 320 arteriography, 473
Beta blocker, 641 channel blocker, 641
Bethesda system, 435 deficiency, 840
Bilateral chest, 615 testing, 443, 444
Index 863
Cancer staging, 256 Cholelithiasis, 477

Cancer treatment, 255–257 pregnancy and, 845, 846
Cannot intubate, cannot oxygenate (CICO), 708 Chromogranin A level (CgA), 185
Capnography, 707 Chronic anemia, 731
Carcinoembryonic antigen (CEA), 399 Chronic hyponatremia, 758
Carcinoid syndrome, 182, 185, 186 Chronic limb ischemia, 569
Carcinoid tumors, 181 Chronic mesenteric ischemia (CMI), 585, 586
Cardiac arrest, 711, 712 Chronic pancreatitis, 383, 384
blunt traumatic, 631 Cirrhosis, 339
Cardiac tamponade, 627 Claudication, 573
Cardiac ultrasound, 631 Clavicle fractures, 634
Cardinal sign, for traumatic injury, 683 Cloaca, 509, 510
Cardiogenic shock, 727, 728 Clostridium difficile, 229
Cardiopulmonary arrest, 627 colitis, 241, 242
Cardiopulmonary resuscitation (CPR), 631 toxin, 241
Caroli disease Closure of wounds, see Wound closure
etiology, 313 Clots in Legs Or sTockings after Stroke 3 (CLOTS 3)
imaging, 314 trial, 702
management, 316 CMI, see Chronic mesenteric ischemia (CMI)
Carotid artery stenosis, 547, 548 Coagulation necrosis, 143
Carotid artery stenting (CAS), 547 Coagulopathy, 702
Carotid duplex, 548 Colon cancer, 251, 255–257
Carotid endarterectomy (CEA), 547 Colon polyps, 251
Catecholamines, 461 Colonic ischemia, 237, 238
Catheter Directed Thrombolysis (CDT), 565 Colonic pseudo-obstruction (CPO), 217, 218
Caustic injuries, 143, 144 Colonic volvulus, 221, 222
Cavo-atrial shunting, 658 Colonoscopy, 205, 230, 245, 256, 257
Cecal volvulus, 221, 222 Combined resection, 327
Central injuries, 627 Common bile duct, 357
Central lumpectomy, 95 Common bile duct exploration, 357
Central neck dissection (CND), 439 Common bile duct stones (CBDS), 345
Cerebral demyelination, 761 Compartment syndrome, 739
Cerebral perfusion pressure (CPP), 702 Complete skin exam, 5
Cervical lymph node, enlarged, 25, 26 Complex injury, 684
Cervical lymphadenopathy, 26 Compressive symptom, 435, 436
Charcot’s triad, 359 Computed tomography (CT), 17
Chemoradiation, 136 bladder injury, 683
Chemotherapy, pregnancy, 854 blunt abdominal trauma, 649, 650
Chest imaging, renal cell carcinoma, 597 blunt aortic injury, 641
Chest pain, 743 cystadenoma, 315
Chest trauma, penetrating, 627, 628 diaphragmatic injury, 653
Chest tube, 59, 60 Fournier’s gangrene, 609
Chest X-ray (CXR), 679 hepatic abscess, 324
appendicitis, 849 hydatid cysts, 314
blunt aortic injury, 641 hypotension, 615
blunt chest wall trauma, 634 inguinal hernia, 783
intubation, 712 intracranial hemorrhage, 701
penetrating chest trauma, 627 neck mass, 21
thoracic outlet syndrome, 589 obturator hernia, 797
Child Protective Services (CPS), 747 pancreatic injury, 661
Child-Pugh Classification, 331, 332 PCLD, 315
Cholangiocarcinoma, 361, 362 pelvic fracture, 680
Cholangioscopy, 335 rectal injury, 687
Cholangitis, 373 recurrent inguinal hernia, 789
Cholecystectomy, 346, 357, 373, 374, 380 renal mass, 597
Cholecystectomy, post, 351, 352 simple cysts, 314
Cholecystitis, 373 traumatic brain injury, 619, 620
Cholecystocholangiography, 662 traumatic liver injury, 657, 658
Choledochal cyst (CC), 369–370 ureter injury, 671
Choledocholithiasis, 357 ventral hernia repair, 831
Choledochotomy, 359 von Meyenburg complexes, 315
864 Index
Computed tomography angiography (CTA) Deep vein thrombosis (DVT), 577, 744
abdomen and pelvis, 581, 585 Definitive repair, 658
arterial occlusion, 581 Delayed mesh-based repair, 785
penetrating neck trauma, 624 Delayed primary closure, 695
pulmonary emboli, 743, 744 Denosumab, 776
Concurrent abdominal injuries, 661 Dermal lymphatics, 105
Congenital anomaly, 21, 537 Dermoid cyst, 26
Congenital diaphragmatic hernia (CDH), 487, 488, Devitalized tissue, 675
495, 496 Diabetes mellitus, 609
Conn’s syndrome, 453, 454 Diagnostic peritoneal aspiration (DPA), 615, 616,
Contrast enema, 501, 506 646, 650
Cooper’s ligament, 793, 794 Diagnostic peritoneal lavage (DPL), 615, 616, 646, 650
Copper deficiency, 840 Dialysis access, 593
Core needle biopsy (CNB) Diaphragmatic injury, 628, 653, 654
neck mass, 21 Diarrhea, 159, 241, 481
salivary gland tumors, 29 Dietary calcium restriction, 776
Corkscrew appearance, 123 Diffuse esophageal spasm, 119
Cortisol, 449 Digital rectal exam (DRE), 276, 687
Cough peak flow (CPF), 715 prostate cancer, 601
C-peptide, 473 Diligence, 715
Crawford classification, 63 Diltiazem, 641
Creatinine, 669 Diphenoxylate-atropine, 303
Cricothyroidotomy, 623, 708 Direct oral anticoagulants (DOACs), acute DVT, 577
Crohn’s disease, 195, 233, 234 Disseminated disease, 602
Cryptorchidism, 605 treatment, 606
CT angiography (CTA), 205 Distal esophageal spasm, 119
Cuff-leak test, 715 Distal intestinal obstruction syndrome (DIOS), 522
Cullen’s sign, 379 Distributive shock, 727
Cushing’s syndrome, 449 Diverticular abscess, 209
Cutaneous anesthesia, 11 Diverticular fistula, 209
Cutaneous lesion, 7 Diverticulitis, 209, 210
Cutaneous melanoma, 3 Doppler waveforms, 573
Cyst aspiration, 314 Dorsalis pedis (DP), 573
Cystadenocarcinoma Dressings, burns, 747, 748
etiology, 313 Driving pressure, 719
imaging, 315 Ductal carcinoma in situ (DCIS), 77, 78
management, 316–318 Ductal injury, 661, 662
Cystadenoma Ductography, 70
etiology, 313 Ductoscopy, 70
imaging, 315 Dumping syndrome, 157
management, 316 Duodenal adenocarcinoma, 395
Cystduodenostomy, 391 Duodenal atresia, 499
Cystectomy, hydatid cyst, 315 Duodenal obstruction, 499
Cystgastrostomy, 391, 392 Duodenal-jejunal junction (DJJ), 506
Cystic fibrosis, 522 Dysphagia, 17
Cystogram, 683, 684 Dysrhythmia, 765
Cystoscopy, bladder injury, 683, 684
Cysts of the liver, 313–316
Cytology, 435, 436 E
Cytomegalovirus (CMV), 229 Early satiety, 169
Echocardiogram, 637
Electrocardiogram (EKG), 637
D Elevated Arm Stress Test, 589
Damage control surgery, 658 Elvey test, 589
DCIS, see Ductal carcinoma in situ (DCIS) Embolization, 665, 666
D-dimer test, 743 Emergency department (ED), thoracotomy, 631, 632
De Quervian’s thyroiditis, 427 Empyema, management of, 55, 56
Debridement, 11 Endoanal ultrasound testing, 303
De-clot, 593 Endobronchial ultrasound (EBUS), 52
Decompressive craniectomy, 620, 703 Endocrine therapy, 102
Index 865
Endorectal advancement flap, 285 Fasciotomy, extremity compartment syndrome, 694, 695
Endo-rectal pull-through, 514 Fecal contamination, 688
Endoscopic eradication therapy, 127 Fecal diversion, 609
Endoscopic mucosal resection (EMR), 127 Fecal incontinence, 285, 303
Endoscopic retrograde cholangiopancreatography Fecal microbiota transplant, 242
(ERCP), 335, 336, 351, 352, 355, 357, 359, Feeding intolerance, 533
380, 387, 403, 845 Femoral hernia, 793, 794
Endoscopic ultrasound (EUS), 383, 387, 399 Fenestration
Endotracheal tube, 712 PCLD, 316
Endovascular abdominal aortic aneurysm simple cyst, 315
(EVAR), 555, 556 Fever management, intracranial hemorrhage, 702
Endovascular aortic repair (EVAR), 551, 552 Fiber supplementation, 293
Endovascular blunt aortic injury, 641 Fiberoptic bronchoscope, 708
Endovascular techniques, revascularization, 586 Fiberoptic endoscopy, 17, 143
Enema reduction, 526 Fibrinolytic, 55, 56
Enlarged axillary lymph node, 85 Fine needle aspiration (FNA), 427, 435
Enlarged cervical lymph node, 25, 26 neck mass, 21
Enterocutaneous (EC) fistula, 191, 192 Fine Needle Aspiration Biopsy (FNAB), 309
Enterolithotomy, 199 Fine Needle Aspiration cytology (FNAC), 29
Epididymis, 605 First rib resection, 589
Epididymo-orchitis, 605 Fistula, 199
Epidural hematoma, 619, 620 Fistulagram, 593
Eplerenone, 466 Fistulogram, 192
Escharotomy, 747 Fistulotomy, 298
Esmolol, 641 5-Hydroxyindole acetic acid (5-HIAA), 185
Esophageal adenocarcinoma, 135 Flat perineum, 509
Esophageal atresia (EA), 491 Flexible endoscopy, acute leaks, 835
Esophageal cancer, 135, 136 Fluid resuscitation, 747, 748
Esophageal injury, 624 and oxygen delivery, 752
Esophageal motility disorders, management of, 119, 120 Fluoroquinolone antibiotic prophylaxis, 601
Esophageal mucosa, 144 Fluoroscopic cholecystocholangiography, 662
Esophageal perforation, 139, 140, 144 Flushing rash, 481
Esophageal stenting, 139 Focal nodular hyperplasia (FNH), 308, 320
Esophagectomy, 135 Focused assessment with sonography in trauma
Esophagogastroduodenoscopy (EGD), 123, 161 (FAST), 616
Etiology-specific therapy, 776 blunt abdominal trauma, 649
Euvolemic hyponatremia, 757, 758 diaphragmatic injury, 653
Ewing’s sarcoma, 9 pelvic fracture, 680
Excisional biopsy penetrating chest trauma, 627
breast mass, 74 splenic injury, 665
lobular carcinoma in situ, 81 Foley, 683, 684
Exercise program, supervised, 573 Foley balloon catheterization, 623
Extended cholecystectomy, 366 Folic acid, 839
External hemorrhoid, 289, 290 Fournier’s gangrene, 609, 610
External oblique release (EO), 814 Fractionated metanephrines, 461
Extracorporeal membrane oxygenation Free fluid without obvious solid organ injury
(ECMO), 487, 488 (FFWOSOI), 650
Extrahepatic cholangiocarcinoma, 362 Fulminant Colitis, 234
Extrahepatic hilar tumor, 361 Functional liver remnant, 327
Extrahepatic metastasis, 331 Fundoplication, 134
Extraperitoneal injury, 683, 684
Extravasation, 671
Extremity compartment syndrome, 693–695 G
Extubation, 715 Ga-DOTANOC scan, 481
Galactorrhea, 69
Gallbladder cancer (GC), 365, 366
F Gallbladder disease (GD), 373
Familial hypocalciuric hypercalcemia (FHH), 443 Gallium-labeled radioligands, 481
Fascial defect closure, 801 Gallstone disease, 845, 846
Fascial violation, 646 Gallstone ileus, 199
866 Index
Gallstone pancreatitis, 373, 380 epidemiology, 323

Gallstones, 345 etiology, 323–324
Gastrectomy, 162 malignant, 324
Gastric cancer, 165–166 management, 324–326
Gastric outlet obstruction, 391 primary source control, 325
Gastric stasis, 159 pyogenic, 323
Gastrinoma, 469, 470 serologic diagnosis, 324
Gastrinoma triangle, 469 stool, 324
Gastroenterologist, 585 Hepatic adenoma, 308, 319–320
Gastroesophageal reflux disease (GERD), 119, 129, 130 Hepatic hemangioma, 309, 319
Gastrografin ®, 521 Hepatic injury, 657, 658
Gastrointestinal stromal tumors (GISTs), 169, 181 Hepatic resection, 362
Gastro-jejunal anastomosis, 827 Hepatocellular carcinoma (HCC), 307, 331, 332
Gastro-jejunal anastomosis leaks, 836 Hereditary colon cancer syndromes, 243–245
Gastroschisis, 537, 538 Hereditary nonpolyposis colorectal cancer (HNPCC),
Genital cellulitis, 609 243–245
Genitourinary injury, 671 Hernia
Giant hiatal hernia, 495, 496 bedside reduction, 805
Giant omphalocele, 538 pregnancy and, 857, 858
Giant prosthetic reinforcement of visceral sac small bowel obstruction, 177
(GPRVS), 797 See also specific hernia
Glasgow Coma Score (GCS), 619 Hernia, Patient, Wound (HPW) staging system, 801
Gleason score, 601 Hiatal hernia, 133, 134
Globular filtration rate, 669 HIDA, 349
Glucagon-like peptide (GLP-1), 473 High-grade dysplasia, 399
Glucagonoma, 457 Highly selective vagotomy (HSV), 157
Glucocorticoids, 776 Hirschsprung disease (HD), 513, 514
Goiter, 431 Histamine-2 (H2) Blockers, 161
Graves’ disease, 423 Histology, sarcoma, 9
Grey Turner sign, 379 Howship-Romberg sign, 797
Gross hematuria, 683 Human-papilloma virus (HPV), 17
Hydatid cysts
etiology, 313
H imaging, 314
Hannington-Kiff sign, 797 management, 315
Hard signs, vascular/tracheoesophageal injury, 623–625 Hydrocolpos, 509, 510
Hashimoto’s thyroiditis, 419, 427 Hyperbaric oxygen therapy, Fournier’s gangrene, 610
Heartburn, 129, 130, 133 Hypercalcemia, 443, 775, 776
Hedgehog pathway inhibitor, 5 Hypercortisolism, 449
Hematemesis, 171 Hypergastrinemia, 469
Hematochezia, 171 Hyperglycemia, 477
Hematoma, 624 Hyperkalemia, 767, 768
Hematuria, 669 Hypernatremia, 761, 762
Hemodialysis, 593 Hyperosmolality, 761
Hemodynamic instability, 627, 645 Hyperparathyroidism, 443, 444, 775, 776
Hemodynamic stability, 657 Hyperphosphatemia, 772
Hemodynamically unstable, 615, 637 Hyperthyroidism, 423, 424, 435
Hemoptysis, massive, 35, 36 Hypertonic hyponatremia, 757
Hemorrhagic shock, 727 Hypertonicity, 762
Hemorrhoid thrombosis, 290 Hyperventilation, 620
Hemorrhoidectomy, 290 Hypervolemic hyponatremia, 757, 758
Hemorrhoids, 289, 290 Hypocalcemia, 771, 772
Hemothorax, 627, 628, 633 Hypoglycemia, 473, 474
Heparin-Induced Thrombocytopenia, 565 Hypokalemia, 765
Hepatic abscess Hypokalemic alkalosis, 779
amoebic, 323 Hypokalemic hypochloremic metabolic alkalosis, 529
clinical presentation and diagnosis, 323 Hypomagnesemia, 765, 771, 772
culture, 324 Hyponatremia, 757, 758
diagnosis, 324 Hypoperfusion, 237
drainage, 325 Hypophosphatemia, 771
Index 867
Hypotension, 615, 616, 657 Intraperitoneal injury, 684

Hypothyroidism, 419, 420 Intravenous (IV) fluid replacement, 404
Hypotonic hyponatremia, 757 Intubation, 707, 711, 712
Hypotonic saline, 762 Intussusception, 525, 526
Hypovolemic shock, 728 Iron deficiency, 840
Ischemia, 731
Ischemic bowel, 538
I Ischemic colitis, 213, 237, 238
Iatrogenic injuries, 654 Ischiorectal abscesses, 297
ICH, see Intracranial hemorrhage (ICH) Ischiorectal fossa, 297
Idiopathic adrenal hyperplasia (IAH), 465 Isotonic hyponatremia, 757
Idiopathic pancreatitis, 387 IVC filter, 744
Ileal pouch anal anastomosis (IPAA), 229
Imatinib, 169
Immediate empiric treatment, 743 J
Immediate life-threatening injuries, 633 Jackhammer esophagus, 119
Imperforate anus, see Anorectal malformations (ARMs) Jejuno-jejunal anastomosis leaks, 836
Implant reconstruction, 110
Incarcerated hernia, 805, 806
Incarceration, 517 K
Incentive spirometry, 634 Kaopectate, 303
Incidental lung nodule, 47 Kasai procedure, 542
Incomplete pancreas divisum, 387 Ki-67 index, 186
Indeterminate/partial thickness burns, 747 Kidney grades, 669
Inflammatory bowel disease (IBD), 195, 233 KIT, 169
Inflammatory breast cancer, 105, 106 Klatskin tumor, 361
Inguinal hernia, 517, 518, 783–785
recurrence, 789, 790
Inspissated meconium, 521–522 L
Insulin, 473 Laboratory Risk Indicatory for Necrotizing
Insulinoma, 473, 474 Fasciitis (LRINEC), 11
Intensive Blood Pressure Reduction in Acute Cerebral Laceration, 684
Hemorrhage 2 (INTERACT-2) trial, 702 Lactate acidosis, 752
Intensive care unit (ICU) Lactic acidosis, 752
blood transfusion, 732 Ladd’s procedure, 505, 506
sepsis, 724 Laparoscopic adrenalectomy, 466
Intermittent hemodialysis, 740 Laparoscopic appendectomy, 225, 226
Intermittent sedation, sepsis, 724 Laparoscopic cholecystectomy (LC), 346, 373
Internal hemorrhoid, 289 Laparoscopic hernia repair, 858
Internal hernia, 825 Laparoscopic partial cholecystectomy (LPC), 346
Internal rectal intussusception, 270 Laparoscopy
Intersphincteric abscesses, 297 obturator hernia, 798
Intersphincteric fistula tract (LIFT), 298 open inguinal repair, 805, 806
Interventional bronchoscopy, 35 penetrating chest trauma, 628
Intestinal transplantation, 201 Laparotomy, 615
Intimal tear, 641 acute kidney injury, 669
Intraabdominal hypertension (IAH), 735 blunt abdominal trauma, 650
Intra-abdominal injury, 649 diaphragmatic injury, 653
Intraabdominal pressure (IAP), 735, 736 splenic injury, 665
Intracranial hemorrhage (ICH), 701–703 Large bowel obstruction (LBO), 213, 214
Intracranial pressure (ICP), 701, 702 Laryngoscopy, 707, 708
monitoring, 702 Lateral neck dissection (LND), 439
traumatic brain injury, 620 LCIS, see Lobular carcinoma in situ (LCIS)
Intraductal papillary mucinous neoplasms Left-sided portal hypertension, 339
(IPMN), 399 Lethal triad, 658
Intrahepatic cholangiocarcinoma, 361, 362 Levothyroxine, 419, 420
Intramural hematoma, 642 Life-threatening complications, intubation, 711
Intraoperative parathyroid hormone monitoring Life-threatening injury, 634, 665
(IOPM), 444 Limb preservation, 9
Intraoperative technique, diaphragmatic injury, 654 Linezolid, Fournier’s gangrene, 609
868 Index
Lipase, 379 Mammalian target of rapamycin inhibitors, 316

Liquefactive necrosis, 143 Mammography, 853
Liver cyst, 313 axillary lymph node, 85
Liver directed therapies, 328 breast mass, 73
Liver nodule, 307–309 Marginal ulcer (MU), 827
Liver resection, 331 Marsupialization, simple cyst, 316
Liver transplantation, 331 Martius flap, 285, 286
PCLD, 316 Massive hemoptysis, 35, 36
Lobectomy, 51 Massive hemorrhage, 727
Lobular carcinoma in situ (LCIS), 81, 82 Mastectomy, 91, 853
Local recurrence, 92 breast reconstruction after, 109, 110
Locoregional recurrence, of breast cancer, 97, 98 male breast cancer, 114
Longitudinal intestinal lengthening and tailoring Paget’s disease, 95
(LILT), 201 McVay repair, 794
Loperamide, 303 Mechanical ventilation, 724
Low birth weight infants, 533 Mechanism of injury (MOI), 661
Lower gastrointestinal bleeding (LGIB), 205, 206 Meconium ileus, 521, 522
Lumen-apposing metal stent, 391 Meconium peritonitis, 521, 522
Lung cancer, 47 Meconium pseudocyst, 521, 522
management of, 51, 52 Median sternotomy, 627
Lung lesion, 47 Mediastinal cyst, 39
Lung nodule, 47, 51 Mediastinal mass, 39, 40
Lung resection, 51 Mediastinoscopy, 52
Lymph node Mediastinum, 39, 40
axillary, 85 Medical management, hydatid cyst, 315
cervical, 25, 26 Medical therapy, acute limb ischemia, 565
Lymphadenectomy, 3 Melanoma, 3
Lymphadenopathy, 29 Melena, 171
Lymphocytic thyroiditis, 427 Mesenteric angiography, 206
Lymphoma, 182 Mesenteric arteries, stenosis of, 585
Lynch syndrome, 243, 244 Mesenteric ischemia, 581
acute, 581
chronic, 585, 586
M Mesocaval shunting, 339
MACOCHA scoring system, 711 Metabolic acidosis, 751
Mafenide acetate, 748 Metabolic alkalosis, 751, 779
Magnetic resonance cholangiopancreatography (MRCP), Metastatic breast cancer, 85, 87, 101, 102
335, 336, 357, 383, 846 Metastatic disease, 308
Magnetic resonance imaging (MRI), 17 Metastatic liver disease, 327, 328
axillary lymph node, 85 Metronidazole, 284, 324
cystadenocarcinoma, 315 Micronutrient deficiency, 839, 840
cystadenoma, 315 Midgut, 185
hepatic abscess, 324 Midgut volvulus, 505
hydatid cysts, 314 Milk rejection sign, 853
inguinal hernia, 783 Minimally invasive technique
neck mass, 21 diaphragmatic injury, 653
Paget’s disease, 95 femoral hernia, 793
PCLD, 314 ventral hernia repair, 801
recurrent inguinal hernia, 789 Modified Wells score, acute DVT, 577
renal cell carcinoma, 597 Mohs micrographic surgery, 5, 7
salivary gland tumors, 29 Morgagni hernia, 495, 496
simple cysts, 314 Motor vehicle collisions (MVCs), 665
von Meyenburg complexes, 315 MUDPILES differential diagnoses, 751
Magnetic sphincter augmentation, 130 Multidisciplinary rehabilitation, 703
Major salivary gland, 29 Multiple Endocrine Neoplasia 1 (MEN1)
Malabsorption, 201 syndrome, 457, 469, 470, 473, 481
Male breast cancer, 113, 114 Multisystem organ failure, 761
Mallory-Weiss tear, 139, 171 Murphy’s sign, 845
Malperfusion, 559 Mycobacterial infection, 26
Malrotation, 505, 506 Myocardial infarction, 637
Index 869
N Organ dysfunction, 723

N-acetylcysteine, 521 Oropharyngeal squamous cell carcinoma
Nasogastric tubes, 18 (OPSCC), 17, 18
Neck mass, 21, 22, 25 Orotracheal intubation, 623
Neck trauma, penetrating, 623, 624 Orthopedic surgery, 680
Neck ultrasonography (US), 435, 439 Orthotopic liver transplantation (OLTx), 332, 336
Necrosectomy, 403, 404 Osteoporosis, 443
Necrotizing Enterocolitis (NEC), 533, 534 Ovarian stromal (OS), 314
Necrotizing soft tissue infection (NSTI), 11 Oxandralone, 748
Negative pressure wound therapy (NPWT), 809
Neoadjuvant chemotherapy, 91, 106
Neoadjuvant therapy, 276, 278, 279, 396 P
Neonatal jaundice, 541 PAC, see Periampullary adenocarcinoma (PAC)
Neostigmine, 217 Paget’s disease, 95
Nephrectomy, 598 Paget-von Schrotter syndrome, 589
Neurocritical Care Society, 701 Pain out of proportion to exam, 11
Neurocritical care unit, 701, 702 Painless mass, 9
Neurogenic shock, 727 Painless thyroiditis, 427
Neurogenic TOS, 589 Palliative care, 101, 102
Neurologic injury, 619 Palliative mastectomy, 87
Neuromuscular blockade, 724 Palpable lymph nodes, 7
Neuromuscular blockade agent, 719 Pamidronate, 776
Nicardipine, 641 Pancreas, 399
Nipple discharge, 69, 70 Pancreas divisum, 387, 388
Nipple-areolar complex (NAC), 95 Pancreatectomy, 662
Nissen fundoplication, 130, 134 Pancreatic ascites, 391
Nitroglycerin, 641 Pancreatic calcification, 383
Nitroprusside, 641 Pancreatic cancer, 383
Non-anion gap metabolic acidosis, 751 Pancreatic duct stent, 384
Non-cirrhotic etiologies, 339 Pancreatic ductal adenocarcinoma (PDAC), 395
Non-epithelial GI tumor, 169 Pancreatic injury, 661, 662
Non-occlusive mesenteric ischemia, 581 Pancreatic insufficiency, 383, 384
Non-steroidal anti- inflammatory drugs (NSAIDs), 161 Pancreatic necrosis, 403, 404
Non-toxic goiter, 431 Pancreatic trauma, 391
Normal sterile saline (NSS) infusion, 779 Pancreaticoduodenectomy (PD), 362, 396, 398, 470
North American Symptomatic Carotid Endarterectomy Pancreatitis, 391
Trial (NASCET), 547 Papillary thyroid cancer (PTC), 355, 359, 439
Nuclear scintigraphy, 205 Papillotomy, 388
Paradoxical aciduria, 779, 780
Paraesophageal hernia, 133, 134
O Parapneumonic effusion, 55
Obstetrical trauma, 283, 285 Paratesticular sarcomas, 606
Obstructive shock, 727, 728 Parathyroid hormone (PTH), 443, 444, 771, 775
Obturator hernia, 797, 798 Partial hepatectomy, 316
Occluded dialysis access, 593 Past medical history (PMH), 637
Occult breast cancer, 85 Patent processus vaginalis (PPV), 517, 518
Octreotide, 157, 470 Patient-controlled analgesia (PCA) pumps, 380
Olive, 529 PCT, see Penetrating chest trauma (PCT)
Omphaloceles, 537, 538 Pediatric inguinal hernia, 517, 518
Oncoplastic techniques, 109 Pediatric intussusception, 525, 526
Oopiods, 303 Pelvic fracture, 679, 680, 687
Open anterior tension-free mesh technique, 784 Pelvic fracture urethral injury (PFUI), 675, 676
Open cholecystectomy (OC), 346 Pelvic ring injuries, 679
Open surgical repair (OSR), 551 Pelvic roentgenograms, 645
Operating room (OR), 687 Penetrating abdominal trauma, 645, 646
Operative approach, diaphragmatic injury, 653 Penetrating chest trauma (PCT), 627, 628
Operative management, penetrating neck trauma, 624 Penetrating neck trauma (PNT), 623, 624
Oral calcium supplementation, 444 Penetrating trauma, 631, 675, 687
Oral contraceptives (OCPs), 308 abdomen, 631
Orchiectomy, 605, 606 chest, 631
870 Index
Peptic ulcer disease (PUD), 157–159, 161 cholecystectomy, 373–374

Peptic ulcers, 171 and cholelithiasis, 845, 846
Percutaneous catheter drainage(PCD), 325 hernia, 857, 858
Percutaneous cholecystostomy, 349 termination of, 853
Percutaneous drain, 534 Pre-renal, 740
Percutaneous needle aspiration (PNA), 325 Pre-sacral drainage, 688
Perforation, 144, 533, 538 Primary hyperaldosteronism, see Conn’s syndrome
Periampullary adenocarcinoma (PAC), 395, 396, 398 Primary hyperparathyroidism (pHPT), 443, 444
Pericardial effusion, 633 Primary realignment, urethral injury, 675, 676
Pericatheter retrograde urethrography, 676 Primary sclerosing cholangitis (PSC), 335, 336
Perineal fistula, 509, 510 Proctoscopy, 289
Perineal proctosigmoidectomy, 265 Proinsulin, 473
Perineural invasion, 7 Propranolol, 748
Peripheral arterial disease (PAD), 573 Prostate cancer, 601, 602
Peripheral lymphadenopathy, 25 Prostate specific antigen (PSA), 601
Perirectal abscess and fistulae, 297, 298 Prostatectomy, 602
Peritoneal seeding, 165 Prosthetic mesh, 784
Persistent hypovolemia, 779 Proton pump inhibitors (PPI), 127, 161
Pertinent medical history, 707 Provoked DVT, 577
PET-CT, 17 Proximal DVT, 577
Petroleum-based dressings, 748 PSC, see Primary sclerosing cholangitis (PSC)
Peyer’s patches, 525 Pseudoaneurysm, 642
Pharmaco-mechanical thrombolysis, 589 Pseudocysts (PC), 391
Phenylephrine, 727 Pseudo-Zollinger Ellison syndrome, 162
Pheochromocytomas, 461, 462 Psoas signs, 849
Phlegmasia cerulea dolens, 577 Pulmonary artery catheter (PAC), 637
Plasma aldosterone concentration (PAC), 465 Pulmonary emboli
Plasma renin activity (PRA), 465 post-operative, 743, 744
Platelet-derived growth factor receptor alpha symptoms, 743
(PDGFRA), 169 Pulselessness, 694
Platinum-based multi-drug chemotherapy, 606 Puncture, Aspiration of cyst, Injection of protoscolicidal
Pleomorphic LCIS (PLCIS), 82 solution, reaspiration of fluid (PAIR), 315
Pleurodesis, 59, 60 Pyloric stenosis, 529, 530
Pneumatic dilation, 123, 124 Pyloromyotomy, 530
Pneumatosis intestinalis, 533 Pyogenic liver abscess, 325–327
Pneumonectomy, 51
Pneumothorax, 59, 60, 627, 633
Polycystic liver disease (PCLD), 314 Q
etiology, 313 Quality of life, 102
imaging, 314
management, 316
Polypectomy, 251, 252 R
Portal hypertension, 339, 340 Radiation therapy
Portal venous gas, 533, 534 breast, 97, 98
Positive end expiratory pressure (PEEP), 719 pregnancy, 853
Post cholecystectomy, 351, 352 sarcoma, 9
Post-cholecystectomy cholangitis, 355 Radiation treatment (RT), 98
Posterior mesh rectopexy, 266 Radical surgery, hydatid cyst, 315
Posterior Sagittal Anorectoplasty, 510 Radioactive iodine, 423, 431, 440
Posterior Sagittal Anorectovaginourethroplasty, 510 Radiofrequency Ablation (RFA), 331
Posterior tibial (PT), 573 Radiographs, Fournier’s gangrene, 609
Posterolateral thoracotomy, 627 Radiotherapy, squamous cell carcinoma, 17, 18
Post-mastectomy radiation therapy (PMRT), 110 Randomised Evaluation of Surgery with Craniectomy for
Postoperative ileus, 197 Uncontrollable Elevation of Intracranial
Postpartum thyroiditis, 427 Pressure (RESCUEicp) trial, 702–703
Post-renal, 739 Rapid shallow breathing index (RSBI), 715
Pregnancy Reconstruction, Fournier’s gangrene, 610
appendicitis in, 849, 850 Recruitment maneuver, 719
breast cancer, 853, 854 Rectal adenocarcinoma, 278
btermination of, 853 Rectal bleeding, 270
Index 871
Rectal cancer, 275–279 simple cyst, 315

Rectal gas, 510 Scrotal abscess, 609
Rectal injury, 687, 688 Scrotal mass, 605, 606
Rectal prolapse, 263–266, 270, 271 Scrotal ultrasound, 605
Recto-urethral fistula, 509, 510 Seatbelt sign, 683
Rectovaginal fistula, 283–286 Secretin stimulation test, 469
Recurrent breast cancer, 91, 92 Secretin test, 161
Recurrent inguinal hernia, 789, 790 Secretin-enhanced MRCP, 387
Recurrent laryngeal nerve (RLN), 439 Secretory diarrhea, 481
Recurrent peptic ulcer disease, 161–162 Segmental blood pressures, 573
Redistributive hypokalemia, 765 Self-examination, 7
Regional recurrence, 92 Sellar mass, 420
Regorafenib, 332 Seminoma, 606
Regurgitation, 133 Sentinel lymph node biopsy, 78
Reintubation, 715 Sentinel node biopsy, 3
Renal cell carcinoma (RCC), 597, 598 Sepsis, 723, 724
Renal cysts, 597 Sepsis-induced ARDS, 724
Renal insufficiency, 767 Sepsis-induced hypoperfusion, 723
Renal replacement therapy (RRT), 740 Septic shock, 723
Renal ultrasound, 739 Serial endoscopy, 144
Renin-angiotensin-aldosterone system, 757 Serial transverse enteroplasty procedure (STEP), 201
Repair options, thoracoabdominal aortic aneurysm, 64 Serotonin, 185, 186
Resection rectopexy, 265, 266 Serum cortisol, 449
Respiratory acidosis, 751 Serum gastrin levels, 469
Respiratory alkalosis, 751 Sestamibi scan, 444
Resuscitation, 631 Seton, 298
Resuscitative endovascular balloon occlusion of the aorta Shock, 727, 728
(REBOA), 631, 632, 679 Short bowel syndrome, 201
Resuscitative thoracotomy, 627 Sigmoid colectomy, 210
Retained antrum, 161 Sigmoid volvulus, 221
Retrograde pyelography, 671 Silo, 538
Retrograde urethrogram, 676 Silver impregnated dressings, 747, 748
Retrohepatic caval injury, 658 Silver sulfadiazine, 748
Revascularization, 565 Simple cysts
CDT, 565 etiology, 313
chronic limb ischemia, 569 imaging, 314
Rhabdomyosarcoma, 9 management, 315–316
Rib fractures, 633 Single perineal orifice, 510
Rib plating, 633 “6 Ps” of arterial ischemia, 693
Riedel’s thyroiditis, 428 Skin rashes, 669
Right lower quadrant (RLQ), 225 Sleeve gastrectomy (SG), leaks, 835
Right Upper Quadrant US, hepatic abscess, 324 Small bowel mass, 181, 182
Rigler’s triad, 199 Small bowel neuroendocrine tumors, 185, 186
Roux-en-Y gastric bypass (RYGB), 130, 827 Small bowel obstruction
Roux-en-Y hepatic portoenterostomy, 542 concerning findings, 176
Roux-en-Y leaks, 835, 836 hernia, 177
Roux-en-Y reconstruction, 158, 176 imaging, 176
Rovsing signs, 849 impaired patient, 177
RRT, see Renal replacement therapy (RRT) laboratory, 176
Rupture, 642 Roux-en-Y reconstruction, 176
Ruptured abdominal aortic aneurysm (AAA), 555, 556 symptoms, 175
treatment, 177
virgin abdomen, 176
S work-up, 175
Sacral neuromodulation, 303 Small bowel tumors, 181, 182
Salivary gland tumors, 29 Small intestine atresia, 501
Sarcoma, 9, 181 Soft tissue sarcoma, 9
SBRT, 51, 52 Solitary rectal ulcer syndrome (SRUS), 269–272
Sclerotherapy, 289 Somatostatin, 185, 186, 477
PCLD, 316 Somatostatin analogs, PCLD, 316
872 Index
Somatostatin inhibitors, 481 Testicular ultrasound, 606

Somatostatinoma, 477 TEVAR, 560
Somatostatin-receptor-scintigraphy, 470 Thiamin, 839
Spermatic cord, 605 Thoracentesis, 55
Sphincterotomy, 293, 294, 380 Thoracic outlet syndrome (TOS), 589
Spine immobilization, 623 Thoracic spine fractures, 634
Spironolactone, 466 Thoracic surgery, 641
Spleen, 665, 666 Thoracoabdominal aortic aneurysm (TAAA), 63, 64
Splenectomy, 409 Thoracotomy, 56, 744
atraumatic indications for, 413, 414 emergency department, 631, 632
Splenic abscess, 409 3-phase abdominal CT scan, renal mass, 597
Splenic injury, 665, 666 Thrombolysis, thoracic outlet syndrome, 589
Spontaneous pneumothorax, management of, 59, 60 Thymoma, 39, 40
Squamous cell carcinoma, 7, 135 Thyroglossal duct cyst, 26
of oropharynx, 17, 18 Thyroid cancer, 439, 440
Staged resection, 327 Thyroid function, 435
Standard meal test, 162 Thyroid hormone, 431
Steatorrhea, 477 Thyroid masses, 21
Step-up approach, 403 Thyroid nodule, 435, 436
Stoppa repair, 790, 797, 798 Thyroid peroxidase, 419
Straddle injury, 676 Thyroid stimulating hormone (TSH) level, 423
Strangulated hernia, 805, 806 Thyroid storm, 423
Strangulation, 517, 518 Thyroidectomy, 427
Stricturoplasty, 195 Thyroiditis, 427, 428
Stroke, 547, 548 Tissue expander, 110
Stryker needle, 694 TNM-staging, 135, 136, 277
Subclavian-axillary vein thrombosis, 589 Tobacco, 17
Subdural hematoma, 620 Tocolytic therapy, 850
Sub-epithelial fistula, 510 TOS, see Thoracic outlet syndrome (TOS)
Subphenotypes of ARDS, 719 Total abdominal colectomy, 229, 234
Substernal extension, 431 Total proctocolectomy, 234
Succinylcholine, 711 Total thyroidectomy, 435, 436, 439
Sun exposure, 7 Totally extraperitoneal (TEP) technique, 784
Superior mesenteric artery (SMA), 581 femoral hernia, 794
Supervised exercise program, 573 obturator hernia, 798
Supraglottic airway (SGA), 707, 708 recurrent inguinal hernia, 790
Supralevator abscess, 297 Toupet fundoplication, 130
Surgical airway, intervention, 708 Toxic megacolon, 195, 234
Sustained hypernatremia, 761 Toxic multinodular goiter, 423, 431
Suture rectopexy, 265, 266 Tracheal dilation, 44
Symptomatic anemia, 731 Tracheal laser ablation, 44
Symptomatic gallbladder disease, 346 Tracheal resection, 43
Symptomatic hyponatremia, 758 Tracheal stenosis, 43
Syndrome of inappropriate antidiuretic hormone Tracheal stenting, 43
secretion (SIADH), 757 Tracheoesophageal fistula (TEF), 491, 492
Synthetic mesh, 654 Tracheoesophageal injury, hard signs of, 623
Systemic therapy, 98, 101, 102 Tracheotomy, 18
Trans abdominal wall traction (TAWT) system, 809
Transabdominal preperitoneal (TAPP) technique, 784
T femoral hernia, 794
Tachycardia, 657 obturator hernia, 798
Target sign, 525 recurrent inguinal hernia, 790
TBI, see Traumatic brain injury (TBI) Transient ischemic attacks (TIA), 547
Telemetry, 637 Transinguinal preperitoneal (TIPP) technique, 784, 790
Temporary abdominal closure, 736, 809 Transmediastinal injury, 627
Tension pneumothorax, 728 Trans-pulmonary pressure, 719
Teratoma, 39 Trans-rectus sheath extra-peritoneal (TREPP)
Termination of pregnancy, 853 technique, 784
Testicular mass, 605, 606 recurrent inguinal hernia, 790
Testicular torsion, 605 Transversus abdominis release (TAR), 814
Index 873
Traumatic brain injury (TBI), 619, 620 Vascular malformation, 26

Traumatic cardiac arrest, 631 Vascular surgery, 641
Traumatic injury, cardinal sign for, 683 Vasoactive intestinal peptide (VIP), 481, 482
Traumatic liver injury, 657, 658 Vasopressors, sepsis, 723
Troponin, 637 Vedolizumab, 230
T-tube, 355 Venous TOS, 589
Tube thoracostomy, 59 Ventilation-perfusion radionuclide scanning, 743
Tumors of the mediastinum, 39, 40 Ventral hernia repair, 801, 802, 831, 832
Typical fissure, 293 Ventral mesh rectopexy, 266
Vestibular fistula, 510
Vicryl mesh, 809
U Video-assisted retroperitoneal debridement (VARD),
U.S. Preventative Services Task Force (USPSTF), 601 403, 404
Ulceration, 3 Video-assisted thoracoscopic surgery
Ulcerative colitis (UC), 229, 230 (VATS), 55, 56
Ultrasound (US) Video-assisted thoracoscopy, penetrating chest
axillary lymph node, 85 trauma, 628
breast mass, 73 Virgin abdomen, 176
cholelithiasis, 845 Vitamin B12 deficiency, 839
cystadenocarcinoma, 315 Vitamin C, 748
cystadenoma, 315 Vitamin D, 443, 444, 775, 776
femoral hernia, 793 Vitamin D deficiency, 772
Fournier’s gangrene, 609 Vitamin deficiency, 839, 840
hydatid cysts, 314 Vocal cord paralysis, 439
inguinal hernia, 783 von Meyenburg complexes
male breast cancer, 114 etiology, 313
PCLD, 314 imaging, 315
scrotal, 605 management, 316
simple cysts, 314
splenic abscess, 409
testicular, 606 W
von Meyenburg complexes, 315 Walled-off pancreatic fluid collection, 391, 392
Umbilical hernia, 857, 858 Walled-off pancreatic necrosis (WOPN), 391, 392
Unprovoked DVT, 577 Waveform capnography, 711–712
Upper extremity swelling, 589 WDHA syndrome, 481
Upper gastrointestinal symptoms, 827 Weight loss, 585
Upper gastrointestinal swallow, 821 Wells prediction scoring system, 743
Upper gastrointestinal endoscopy, 165 Whipple’s triad, 473
Upper gastrointestinal hemorrhage, 171 Whole breast radiation (WBI), 91
Ureter injury, 671 Wittman patch, 809
Urethral catheterization, 610 Wound closure, 610, 695
Urethral injury, 675, 676
Urinalysis, 739
Urinary electrolytes, 779 Y
Urinoma, 671 Yttrium-90, 328, 332
V Z
Valsalva maneuver, 789 Zinc deficiency, 840
Van Nuys Prediction Index, 78 Zipper closure method, 810
Vancomycin, 241 Zoledronic acid, 776
Fournier’s gangrene, 609 Zollinger-Ellison syndrome (ZES), 161, 469
Varicoceles, 605, 606 Zone I injury, 624
Vascular injury, 693 Zone II injury, 624
hard signs of, 623–625 Zone III injury, 624

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Clinical Algorithms

ISBN 978-3-319-98496-4 ISBN 978-3-319-98497-1 (eBook)

Library of Congress Control Number: 2019930648

© Springer Nature Switzerland AG 2019

My surgical career has been the epitome of dumb luck. I have

Stony Brook, NY, USA Salvatore Docimo Jr.

Part I Skin and Soft Tissue

1 Management of Cutaneous Melanoma������������������������������������������ 3

Part II Head and Neck

6 Management of Squamous Cell Carcinoma

10 Massive Hemoptysis������������������������������������������������������������������������ 35

13 Incidental Lung Nodule������������������������������������������������������������������ 47

18 Nipple Discharge������������������������������������������������������������������������������ 69

31 Management of Esophageal Motility Disorders���������������������������� 119

Part VI Stomach and Duodenum

39 Gastric Ulcer Management ������������������������������������������������������������ 149

Part VII Small Bowel

46 Small Bowel Obstruction���������������������������������������������������������������� 175

48 Management of Small Bowel Neuroendocrine Tumors���������������� 185

Part VIII Large Bowel

54 Management of Lower Gastrointestinal Bleeding������������������������ 205

Part IX Rectum and Anus

67 Rectal Prolapse�������������������������������������������������������������������������������� 263

76 Evaluation of Liver Nodule ������������������������������������������������������������ 307

84 Acute Cholecystitis and Biliary Colic�������������������������������������������� 345

94 Acute Pancreatitis���������������������������������������������������������������������������� 379

101 Management of Splenic Abscess ���������������������������������������������������� 409

103 Hypothyroidism�������������������������������������������������������������������������������� 419

110 Cushing’s Syndrome and Disease �������������������������������������������������� 449

117 Management of Somatostatinoma�������������������������������������������������� 477

119 Congenital Diaphragmatic Hernia ������������������������������������������������ 487

132 Omphalocele and Gastroschisis������������������������������������������������������ 537

134 Carotid Artery Stenosis ������������������������������������������������������������������ 547

146 Management of the Renal Mass������������������������������������������������������ 597

149 Diagnosis and Management of Fournier’s Gangrene ������������������ 609

150 Hypotension and Blunt Abdominal Trauma �������������������������������� 615

167 Bladder Injuries ������������������������������������������������������������������������������ 683

Part XX Critical Care

170 Management of Intracranial Hemorrhage������������������������������������ 701

182 Hyponatremia���������������������������������������������������������������������������������� 757

184 Hypokalemia������������������������������������������������������������������������������������ 765

189 Inguinal Hernia�������������������������������������������������������������������������������� 783

Part XXIII Bariatric Surgery

197 Indications for Bariatric Surgery �������������������������������������������������� 819

202 Acute Leak Following Bariatric Surgery: Endoscopic Stent

Part XXIV Pregnancy and General Surgery

204 Pregnancy and Cholelithiasis���������������������������������������������������������� 845

Sophia Abdulhai, MD Division of Pediatric Surgery, Akron Children’s

Cassandre Bénay, MD, MSc Department of Endocrine Surgery, The

Department of Surgery, Virginia Commonwealth University School of

Augustyna Gogoj, BS Division of Urology, Penn State Milton S. Hershey

Q. Lina Hu, MD Department of Surgery, David Geffen School of Medicine

Afif N. Kulaylat, MD, MSc Department of Surgery, Division of Pediatric

Jeffrey M. Marks, MD, FACS, FASGE Department of Surgery, University

Elif Onursal, DO, MS Department of General Surgery, St. Barnabas

Zachary J. Senders, MD Department of Surgery, University Hospitals

Anthony R. Tascone, MD Department of General Surgery, Saint Luke’s

Algorithmic Approach and pelvis with IV contrast or full body PET/

© Springer Nature Switzerland AG 2019 3

Stony Brook, NY, USA Salvatore Docimo Jr.

Part I Skin and Soft Tissue

1 Management of Cutaneous Melanoma�� 3

Part II Head and Neck

6 Management of Squamous Cell Carcinoma

10 Massive Hemoptysis�� 35

13 Incidental Lung Nodule�� 47

18 Nipple Discharge�� 69

31 Management of Esophageal Motility Disorders�� 119

Part VI Stomach and Duodenum

39 Gastric Ulcer Management �� 149

Part VII Small Bowel

46 Small Bowel Obstruction�� 175

48 Management of Small Bowel Neuroendocrine Tumors�� 185

Part VIII Large Bowel

54 Management of Lower Gastrointestinal Bleeding�� 205

Part IX Rectum and Anus

67 Rectal Prolapse�� 263

76 Evaluation of Liver Nodule �� 307

84 Acute Cholecystitis and Biliary Colic�� 345

94 Acute Pancreatitis�� 379

101 Management of Splenic Abscess �� 409

103 Hypothyroidism�� 419

110 Cushing’s Syndrome and Disease �� 449

117 Management of Somatostatinoma�� 477

119 Congenital Diaphragmatic Hernia �� 487

132 Omphalocele and Gastroschisis�� 537

134 Carotid Artery Stenosis �� 547

146 Management of the Renal Mass�� 597

149 Diagnosis and Management of Fournier’s Gangrene �� 609

150 Hypotension and Blunt Abdominal Trauma �� 615

167 Bladder Injuries �� 683

Part XX Critical Care

170 Management of Intracranial Hemorrhage�� 701

182 Hyponatremia�� 757

184 Hypokalemia�� 765

189 Inguinal Hernia�� 783

Part XXIII Bariatric Surgery

197 Indications for Bariatric Surgery �� 819

202 Acute Leak Following Bariatric Surgery: Endoscopic Stent

Part XXIV Pregnancy and General Surgery

204 Pregnancy and Cholelithiasis�� 845

Algorithmic Approach and pelvis with IV contrast or full body PET/

Algorithmic Approach D. The lesion should be risk stratified to deter-

Algorithmic Approach If excision is not possible, the patient should

Algorithmic Approach should be reassessed for possible resection

Algorithmic Approach patient should be started on broad-spectrum

Algorithmic Approach tongue, and posterior oropharyngeal wall.

Algorithmic Approach symptoms of infection including symptoms

Algorithmic Approach mass as well as its rate of growth are impor-

Algorithmic Approach C. Diagnostic imaging is warranted if there is a

Algorithmic Approach C. After the initial stabilization, a bronchos-

Algorithmic Approach thenia gravis, Horner syndrome, paraneoplas-