******ebook converter DEMO Watermarks*******

******ebook converter DEMO Watermarks*******

 COMPREHENSIVE REVIEW SERIES

CLINICALS

SURGERY

For PG Medical Entrance

******ebook converter DEMO Watermarks*******

Published by Delhi Academy of Medical Sciences (P) Ltd.

HEAD OFFICE
Delhi Academy of Medical Sciences (P.) Ltd.
4-B, Grovers Chamber, Pusa Road,
Near Karol Bagh Metro Station,
New Delhi-110 005
Phone : 011-4009 4009
http://www.damsdelhi.com
Email: [email protected]

ISBN: 978-93-89309-03-4

First Published 1999, Delhi Academy of Medical Sciences

© 2020 DAMS Publications

All rights reserved. No part of this book may be reproduced or transmitted in any form or by any means, electronic,
mechanical, including photocopying, recording, or any information storage and retrieval system without permission,
in writing, from the author and the publishers.
This book contains information obtained from authentic and highly regarded sources. Reprinted material is quoted
with permission. Reasonable efforts have been made to publish reliable data and information, but the authors and the
publishers cannot assume responsibility for the validity of all materials. Neither the authors nor the publishers, nor
anyone else associated with this publication, shall be liable for any loss, damage or liability directly or indirectly
caused or alleged to be caused by this book.
Neither this book nor any part may be reproduced or transmitted in any form or by any means, electronic or
mechanical, including photocopying, microfilming and recording, or by any information storage or retrieval system,
without permission in writing from Delhi Academy of Medical Sciences. The consent of Delhi Academy of Medical
Sciences does not extend to copying for general distribution, for promotion, for creating new works, or for resale.
Specific permission must be obtained in writing from Delhi Academy of Medical Sciences for such copying.
Trademark notice: Product or corporate names may be trademarks or registered trademarks, and are used only for
identification and explanation, without intent to infringe.
Typeset by Delhi Academy of Medical Sciences Pvt. Ltd., New Delhi (India).

******ebook converter DEMO Watermarks*******

 Contents

Chapter 1 Breast
Chapter 2 Thyroid and Parathyroid
Chapter 3 Urology
Chapter 4 GIT-1 - Esophagus, Stomach and Small and Large Intestine
Chapter 5 GIT-2 - Hepatobiliary System and Pancreas
Chapter 6 Adrenal Glands
Chapter 7 Salivary Glands
Chapter 8 Vascular Surgery
Chapter 9 Hernia
Chapter 10 Mediastinum
Chapter 11 General Surgery
Chapter 12 Plastic Surgery
Chapter 13 Bariatric surgery
Chapter 14 Fluid and Electrolytes
Chapter 15 Total Parenteral Nutrition
Chapter 16 Surgical Wounds and Infections
Chapter 17 Sutures
Chapter 18 Miscellaneous

******ebook converter DEMO Watermarks*******

Embryology and Functional Anatomy of the Breast:
Embryology:

At the fifth or sixth week of fetal development, two ventral bands of thickened ectoderm (mammary ridges,
milk lines) are evident in the embryo. In most mammals, paired breasts develop along these ridges, which
extend from the base of the forelimb (future axilla) to the region of the hind limb (inguinal area).
These ridges are not prominent in the human embryo and disappear after a short time, except for small portions
that may persist in the pectoral region.
Accessory breasts (polymastia) or accessory nipples (polythelia) may occur along the milk line when normal
regression fails.
Each breast develops when an ingrowth of ectoderm forms a primary tissue bud in the mesenchyme. The
primary bud, in turn, initiates the development of 15 to 20 secondary buds. Epithelial cords develop from the
secondary buds and extend into the surrounding mes enchyme. Major (lactiferous) ducts develop, which open
into a shallow mammary pit.
During infancy, a proliferation of mesenchyme transforms the mammary pit into a nipple. If there is failure of a
pit to elevate above skin level, an inverted nipple results. This congenital malformation occurs in 4% of infants.
At birth, the breasts are identical in males and females, demonstrating only the presence of major ducts.
Important: Enlargement of the breast may be evident and a secretion, historically referred to as witch’s milk,
may be produced. These transitory events occur in response to maternal hormones that cross the placenta.
The breast remains undeveloped in the female until puberty, when it enlarges in response to ovarian estrogen
and progesterone, which initiate proliferation of the epithelial and connective tissue elements. However, the
breasts remain incompletely developed until pregnancy occurs.
Absence of the breast (amastia) is rare and results from an arrest in mammary ridge development that occurs
during the sixth fetal week.
Poland’s syndrome consists of :hypoplasia or complete absence of the breastcostal cartilage and rib defects,
hypoplasia of the subcutaneous tissues of the chest wall, and brachysyndactyly.
Breast hypoplasia also may be iatrogenically induced before puberty by trauma, infection, or radiation therapy.
Symmastia is a rare anomaly recognized as webbing between the breasts across the midline.
Accessory nipples (polythelia) occur in <1% of infants and may be associated with abnormalities of the
urinary tract (renal agenesis and cancer), abnormalities of the cardiovascular system (conduction disturbances,
hypertension, congenital heart anomalies) and other conditions (pyloric stenosis, epilepsy, ear abnormalities,
arthrogryposis).
Supernumerary breasts may occur in any configuration along the mammary milk line but most frequently
occur between the normal nipple location and the symphysis pubis.
Turner’s syndrome(ovarian agenesis and dysgenesis) and Fleischer’s syndrome (displacement of the nipples
and bilateral renal hypoplasia) may have polymastia as a component. Accessory axillary breast tissue is
uncommon and usually is bilateral.

Functional Anatomy:

The breast is composed of 15 to 20 lobes , which are each composed of several lobules.
Fibrous bands of connective tissue travel through the breast (Cooper’s suspensory ligaments), insert
perpendicularly into the dermis. Cooper’s ligaments provide shape and structure to the breast as they course
from the overlying skin to the underlying deep fascia.
Because these ligaments are anchored into the skin, infiltration of these ligaments by carcinoma commonly
produces tethering, which can cause dimpling or subtle deformities on the otherwise smooth surface of the
breast.
******ebook converter DEMO Watermarks*******
 Extent: The mature female breast extends from the level of the second or third rib to the inframammary
fold at the sixth or seventh rib. It extends transversely from the lateral border of the sternum to the
anterior axillary line. The deep or posterior surface of the breast rests on the fascia of the pectoralis major,
serratus anterior, and external oblique abdominal muscles, and the upper extent of the rectus sheath. The retro-
mammary bursa may be identified on the posterior aspect of the breast between the investing fascia of the
breast and the fascia of the pectoralis major muscles.
The axillary tail of Spence extends laterally across the anterior axillary fold.
The upper outer quadrant of the breast contains a greater volume of tissue than do the other quadrants.
Shape: The breast has a protuberant conical form. The base of the cone is roughly circular, measuring 10 to 12
cm in diameter. Considerable variations in the size, contour, and density of the breast are evident among
individuals. The nulliparous breast has a hemispheric configuration with distinct flattening above the
nipple.
With the hormonal stimulation that accompanies pregnancy and lactation, the breast becomes larger and
increases in volume and density, whereas with senescence, it assumes a flattened, flaccid, and more pendulous
configuration with decreased volume.

Microscopic Anatomy:

The mature breast is composed of three principal tissue types: (1) glandular epithelium, (2) Fibrous stroma and
supporting structures and (3) adipose tissue.
The breast also contains lymphocytes and macrophages. In adolescents, the predominant tissues are epithelium
and stroma. In postmenopausal women, the glandular structures involute and are largely replaced by adipose
tissue.
The glandular apparatus of the breast is composed of a branching system of ducts, organized in a radial pattern
spreading outward and downward from the nipple-areolar complex. It is possible to cannulate individual ducts
and visualize the lactiferous duct. Each major duct has a dilated portion (lactiferous sinus) below the nipple-
areolar complex. These ducts converge through a constricted orifice into the ampulla of the nipple. lactiferous
sinus is lined with stratified squamous epithelium.
Each of the major ducts has progressive generations of branching and ultimately ends in the terminal ductules
or acini.
Acini are the milk-forming glands of the lactating breast and, together with their small efferent ducts or
ductules, are known as lobular units or lobules.
Lobules are the structural units of breast.
Terminal Duct Lobular Unit(TDLU) is the composite assembly of terminal portion of the duct and the
various lobules attached to it. TDLU are the Functional units of breast.
The entire ductal system is lined by epithelial cells, which are surrounded by specialized myoepithelial cells
that have contractile properties and serve to propel milk formed in the lobules toward the nipple. Outside the
epithelial and myoepithelial layers, the ducts of the breast are surrounded by a continuous basement membrane
containinglaminin, type IV collagen, and proteoglycans. The basement membrane layer is an important
boundary in differentiating in situ from invasive breast cancer. Continuity of this layer is maintained in ductal

******ebook converter DEMO Watermarks*******

 carcinoma in situ (DCIS), also termed noninvasive breast cancer. Invasive breast cancer is defined by
penetration of the basement membrane by malignant cells invading the stroma.

Inactive and Active Breast:

Each lobe of the breast terminates in a major (lactiferous) duct (2–4 mm in diameter), which opens through a
constricted orifice (0.4–0.7 mm in diameter) into the ampulla of the nipple. Major ducts are lined with two
layers of cuboidal cells, whereas minor ducts are lined with a single layer of columnar or cuboidal cells.
In the inactive breast, the epithelium is sparse and consists primarily of ductal epithelium. In the early phase of
the menstrual cycle, minor ducts are cord- like with small lumina. With estrogen stimulation at the time of
ovulation, alveolar epithelium increases in height, duct lumina become more prominent, and some secretions
accumulate. When the hormonal stimulation decreases, the alveolar epithelium regresses.
With pregnancy, the breast undergoes proliferative and developmental maturation. As the breast enlarges in
response to hormonal stimulation, lymphocytes, plasma cells, and eosinophils accumulate within the connective
tissues. The minor ducts branch and alveoli develop. Development of the alveoli is asymmetric, and variations
in the degree of development may occur within a single lobule. With parturition, enlargement of the breasts
occurs via hypertrophy of alveolar epithelium and accumulation of secretory products in the lumina of the
minor ducts.
Alveolar epithelium contains abundant endoplasmic reticulum, large mitochondria, Golgi complexes, and dense
lysosomes.
Two distinct substances are produced by the alveolar epithelium: (a) the protein component of milk,
which is synthesized in the endoplasmic reticulum (merocrine secretion); and (b) the lipid component of
milk (apocrine secretion), which forms as free lipid droplets in the cytoplasm.
Milk released in the first few days after parturition is called colostrum and has low lipid content but
contains considerable quantities of antibodies. The lymphocytes and plasma cells that accumulate within the
connective tissues of the breast are the source of the antibody component. With subsequent reduction in the
number of these cells, the production of colostrum decreases and lipid-rich milk is released.

Blood Supply, Innervation, and Lymphatics:

The breast receives its principal blood supply from:
a. Perforating branches of the internal mammary artery;
b. Lateral branches of the posterior intercostal arteries; and
c. Branches from the axillary artery, including the highest thoracic, lateral thoracic, and pectoral branches of the
thoracoacromial artery.

The second, third, and fourth anterior intercostal perforators and branches of the internal mammary
artery arborize in the breast as the medial mammary arteries.
The lateral thoracic artery gives off branches to the serratus anterior, pectoralis major and pectoralis minor, and
subscapularis muscles. It also gives rise to lateral mammary branches.
The veins of the breast and chest wall follow the course of the arteries, with venous drainage being toward the
axilla. The three principal groups of veins are:

a. Perforating branches of the internal thoracic vein.

b. Perforating branches of the posterior intercostal veins, and
c. Tributaries of the axillary vein.

Batson’s vertebral venous plexus, which invests the vertebrae and extends from the base of the skull to the
sacrum, may provide a route for breast cancer metastases to the vertebrae, skull, pelvic bones, and central
nervous system.
Lymph vessels generally parallel the course of blood vessels.
Lateral cutaneous branches of the third through sixth intercostal nerves provide sensory innervation of
the breast (lateral mammary branches) and of the anterolateral chest wall. These branches exit the
intercostal spaces between slips of the serratus anterior muscle.
Cutaneous branches that arise from the cervical plexus, specifically the anterior branches of the supraclavicular

******ebook converter DEMO Watermarks*******

 nerve, supply a limited area of skin over the upper portion of the breast.
Important: The intercostobrachial nerve is the lateral cutaneous branch of the second intercostal nerve
and may be visualized during surgical dissection of the axilla. Resection of the intercostobrachial nerve
causes loss of sensation over the medial aspect of the upper arm.

This is the most common nerve injured during mastectomy:

Six axillary lymph node groups recognized by surgeons are:
a. The axillary vein group (lateral), which consists of four to six lymph nodes that lie or posterior to the vein and
receive most of the lymph drainage from the upper extremity.
b. The external mammary group (anterior or pectoral group), which consists of five to six lymph nodes that lie along
the lower border of the pectoralis minor muscle contiguous with the lateral thoracic vessels and receive most of
the lymph drainage from the lateral aspect of the breast.
c. The scapular group (posterior or subscapular), which consists of five to seven lymph nodes that lie along the
posterior wall of the axilla at the lateral border of the scapula contiguous with the subscapular vessels and receive
lymph drainage principally from the lower posterior neck, the posterior trunk, and the posterior shoulder.
d. The central group, which consists of three or four sets of lymph nodes that are embedded in the fat of the axilla
lying immediately posterior to the pectoralis minor muscle and receive lymph drainage both from the axillary
vein, external mammary, and scapular groups of lymph nodes, and directly from the breast.
e. The subclavicular group (apical), which consists of six to twelve sets of lymph nodes that lie posterior and
superior to the upper border of the pectoralis minor muscle and receive lymph drainage from all of the other
groups of axillary lymph nodes.
f. The inter- pectoral group (Rotter’s lymph nodes), which consists of one to four lymph nodes that are interposed
between the pectoralis major and pectoralis minor muscles and receive lymph drainage directly from the breast.
The lymph fluid that passes through the interpectoral group of lymph nodes passes directly into the central and
subclavicular groups.
Levels of Lymph Nodes:
Levels are decided according to their anatomic relationship to the pectoralis minor muscle.

Lymph nodes located lateral to or below the lower border of the pectoralis minor muscle are referred to as level
I lymph nodes, which include the axillary vein, external mammary, and scapular groups.
Lymph nodes located superficial or deep to the pectoralis minor muscle are referred to as level II lymph nodes,
which include the central and interpectoral groups.
Lymph nodes located medial to or above the upper border of the pectoralis minor muscle are referred to as level
III lymph nodes, which consist of the subclavicular group. group of lymph nodes.
The axillary lymph nodes usually receive >75% of the lymph drainage from the breast. The rest is derived
primarily from the medial aspect of the breast, flows through the lymph vessels that accompany the perforating
branches of the internal mammary artery, and enters the parasternal (internal mammary) group of lymph nodes.

Gynecomastia (very important topic for exams):

Gynecomastia refers to an enlarged breast in the male.

In the nonobese male, breast tissue measuring at least 2 cm in diameter must be present before a
diagnosis of gynecomastia may be made:
Physiologic gynecomastia usually occurs during three phases of life:

a. The neonatal period, : Neonatal gynecomastia is caused by the action of placental estrogens on neonatal
breast tissues.
b. Adolescence: in adolescence, there is an excess of estradiol relative to testosterone.
c. Senescence: with senescence, the circulating testosterone level falls, which results in relative
hyperestrinism.
Common to each of these phases is an excess of circulating estrogens in relation to circulating testosterone.

In gynecomastia, the ductal structures of the male breast enlarge, elongate, and branch with a concomitant
******ebook converter DEMO Watermarks*******
 increase in epithelium.
During puberty, the condition often is unilateral and typically occurs between ages 12 and 15 years. In contrast,
senescent gynecomastia is usually bilateral.
Mammography and ultrasonography are used to differentiate breast tissues.
Dominant masses or areas of firmness, irregularity, and asymmetry suggest the possibility of a breast
cancer, particularly in the older male.
Gynecomastia generally does not predispose the male breast to cancer. However, the hypoandrogenic state
of Klinefelter’s syndrome (XXY), in which gynecomastia is usually evident, is associated with an increased
risk of breast cancer.
Grading: (Gynecomastia is graded based on the degree of breast enlargement, the position of the nipple with
reference to the inframammary fold and the degree of breast ptosis and skin redundancy):

Grade 1: mild breast enlargement without skin redundancy.

Grade IIa: moderate breast enlargement without skin redundancy.
Grade IIb: moderate breast enlargement with skin redundancy.
Grade 3: marked breast enlargement with skin redundancy and ptosis.

******ebook converter DEMO Watermarks*******

Treatment:
Liposuction is the treatment of choice.
Liposuction can be USG assisted or Vaccum assisted.
Surgery is indicated for GRADE 3 gynaecomastia or Klinefelter syndrome.
Wide local excision is preferred.
Mastectomy is indicated for Klinefelter Syndrome (as it is associated with increased risk of malignancy).
Bacterial Infection
(Mastitis and Abscess):

******ebook converter DEMO Watermarks*******

Staphylococcus aureus and Streptococcus species are the organ- isms most frequently recovered from nipple
discharge from an infected breast.
Typically breast abscesses are seen in staphylococcal infections and present with point tenderness, erythema, and
hyperthermia. When these abscesses are related to lactation they usually occur within the first few weeks of
breastfeeding. If there is progression of a staphylococcal infection, this may result in subcutaneous, subareolar,
interlobular (periductal), and retromammary abscesses (unicentric or multicentric).

USG guided Aspiration and Antiobiotics is the preferred initial management.

Operative drainage is now reserved for those cases which don’t resolve with repeated aspiration and antibiotic
therapy or if there is some other indication for incision and drainage (e.g. thinning or necrosis of the overlying
skin).
Preoperative ultrasonography is effective in delineating the required extent of the drainage procedure
While Staphylococcal infections tend to be more localized and may be situated deep in the breast tissues,
streptococcal infections usually present with diffuse superficial involvement. They are treated with local wound
care, including application of warm compresses, and the administration of IV antibiotics (penicillins or
cephalosporins).
Biopsy of the abscess cavity wall should be considered at the time of incision and drainage to rule out
underlying breast cancer in patients where antibiotics and drainage have been ineffective.
Epidemic puerperal mastitis is initiated by highly virulent strains of methicillin-resistant S. aureus that are
transmitted via the suckling neonate and may result in substantial morbidity and occasional mortality.
Purulent fluid may be expressed from the nipple. In this circumstance, breastfeeding is stopped, antibiotics are
started, and surgical therapy is initiated. (remember that breast feeding is not stopped in mastitis. In case of
abscess formation,feeding is stopped from the involved breast.
Nonepidemic (sporadic) puerperal mastitisrefers to involvement of the interlobular connective tissue of the
breast by an infectious process. The patient develops nipple fissuring and milk stasis, which initiates a
retrograde bacterial infection.

Emptying of the breast using breast suction pumps shortens the duration of symptoms and reduces the incidence of
recurrences. The addition of antibiotic therapy results in a satisfactory out- come in >95% of cases.
Zuska’s disease, also called recurrent periductal mastitis, is a condition of recurrent retroareolar infections and
abscesses.

Smoking has been implicated as a risk factor for this condition.This syndrome is managed symptomatically by
antibiotics coupled with incision and drainage as necessary. Attempts to obtain durable long-term control by
wide débridement of chronically infected tissue and/or terminal duct resection have been reported and can be
curative but equally can be frustrated by postopera-tive infections.

Mycotic Infections:

Fungal infections of the breast are rare and usually involve blastomycosis or sporotrichosis.
Intraoral fungi that are inoculated into the breast tissue by the suckling infant initiate these infections, which
present as mammary abscesses in close proxim- ity to the nipple-areola complex. Pus mixed with blood may be
expressed from sinus tracts.
Antifungal agents can be administered for the treatment of systemic (noncutaneous) infections. This therapy
generally eliminates the necessity of surgical inter- vention, but occasionally drainage of an abscess, or even
partial mastectomy, may be necessary to eradicate a persistent fungal infection.

Hidradenitis Suppurativa:

Hidradenitis suppurativa of the nipple-areola complex or axilla is a chronic inflammatory condition that
originates within the accessory areolar glands of Montgomery or within the axillary sebaceous glands.
Women with chronic acne are predisposed to developing hidradenitis.Antibiotic therapy with incision and
drainage of fluctuant areas is appropriate treatment.
Excision of the involved areas may be required. Large areas of skin loss may necessitate coverage with
advancement flaps or split-thickness skin grafts.
******ebook converter DEMO Watermarks*******
Mondor’s Disease:

Mondor’s disease is a variant of thrombophlebitis that involves the superficial veins of the anterior chest wall
and breast.
In 1939, Mondor described the condition as “string phlebitis,” a thrombosed vein presenting as a tender, cord-
like structure.
Frequently involved veins include the lateral thoracic vein, the thoracoepigastric vein, and, less commonly, the
superficial epigastric vein.
Typically, a woman presents with acute pain in the lateral aspect of the breast or the anterior chest wall. A
tender, firm cord is found to follow the distribution of one of the major superficial veins.
This benign, self-limited disorder is not indicative of a cancer.
Therapy for Mondor’s disease includes the liberal use of anti-inflammatory medications and application of
warm compresses along the symptomatic vein. The process usually resolves within 4 to 6 weeks.
When symptoms persist or are refractory to therapy, excision of the involved vein segment may be
considered.

Tuberculosis of the breast:

Tuberculosis of the breast is usually associated with active pulmonary tuberculosis or tuberculous cervical
adenitis.
Tuberculosis of the breast occurs more often in parous women and usually presents with multiple chronic
abscesses and sinuses and a typical bluish, attenuated appearance of the surrounding skin.
The diagnosis rests on bacteriological and histological examination.
Treatment is with anti-tuberculous chemotherapy. Healing is usual, although often delayed, and mastectomy
should be restricted to patients with persistent residual infection.

Duct ectasia/periductal mastitis (important topic for exams):

Pathology:

This is a dilatation of the breast ducts, which is often associated with periductal inflammation.
The pathogenesis is obscure and almost certainly not uniform in all cases, although the disease is much more
common in smokers.
The classical description of the pathogenesis of duct ectasia asserts that the first stage in the disorder is a
dilatation in one or more of the larger lactiferousducts, which fill with a stagnant brown or green
secretion. This may discharge.
These fluids then set up an irritant reaction in surrounding tissue leading to periductal mastitis or even abscess
and fistula formation. In some cases, a chronic indurated mass forms beneath the areola, which mimics a
carcinoma.
Fibrosis eventually develops, which may cause slit-like nipple retraction.
An alternative theory suggests that periductal inflammation is the primary condition and, indeed, anaerobic
bacterial infection is found in some cases. A marked association between recurrent periductal inflammation and
smoking has been demonstrated.
It is certainly clear that cessation of smoking increases the chance of a long-term cure.

Clinical Features:
1. Nipple discharge (of any colour).
2. A subareolar mass, abscess.
3. Mammary duct fistula and/or nipple retraction are the most com- mon symptoms.
Treatment:

In the case of a mass or nipple retraction, a carcinoma must be excluded by obtaining a mammogram and
negative cytology or histology. If any suspicion remains the mass should be excised.
Surgery is often the only option likely to bring about cure of this notoriously difficult condition.
******ebook converter DEMO Watermarks*******
 This consists of excision of all of the major ducts (Hadfield’s operation). It is particularly important to
shave the back of the nipple to ensure that all terminal ducts are removed. Failure to do so will lead to
recurrence.

The Nipple:
Nipple retraction:

Retraction occurring at puberty, also known as simple nipple inversion, is of unknown aetiology (benign
horizontal inversion).
In about 25 per cent of cases it is bilateral. It may cause problems with breastfeeding and infection can occur,
especially during lactation, because of retention of secretions. Recent retraction of the nipple may be of
considerable pathological significance.
A slit-like retraction of the nipple may be caused by duct ectasia (most common) and chronic periductal
mastitis.
Circumferential retraction, with or without an underlying lump, may well indicate an underlying carcinoma.

Treatment:
Treatment is usually unnecessary and the condition may spontaneously resolve during pregnancy or lactation.
Simple cosmetic surgery can produce an adequate correction but has the drawback of dividing the underlying ducts.
Mechanical suction devices have been used to evert the nipple, with some effect.
Discharges from the nipple:

Discharge can occur from one or more lactiferous ducts.

Management depends on the presence of a lump (which should always be given priority in diagnosis and
treatment) and the presence of blood in the discharge or discharge from a single duct.
Mammography is rarely useful except to exclude an underlying impalpable mass.
Cytology may reveal malignant cells but a negative result does not exclude a carcinoma or in situ disease.
A clear, serous discharge may be ‘physiological’ in a parous woman or may be associated with a duct
papilloma or mammary dysplasia.
Multiduct, multicoloured discharge is physiological and the patient may be reassured.
A blood-stained discharge may be caused by duct ectasia, a duct papilloma or carcinoma.
A duct papilloma is usually single and situated in one of the larger lactiferous ducts; it is sometimes
associated with a cystic swelling beneath the areola. It is most common cause of bloody discharge from
single duct.
A black or green discharge is usually the result of duct ectasia and its complications

******ebook converter DEMO Watermarks*******

Treatment:

Exclude a carcinoma by occult blood test and cytology.

Simple reassurance may then be sufficient but, if the discharge is proving intolerable, an operation to remove
the affected duct or ducts can be performed. Microdochectomy It is treatment of choice for Single Duct
Pathology.
A tennis racquet incision can be made to encompass the entire duct or a periareolar incision used and the nipple
flap dissected to reach the duct.
The duct along with its TDLU is then excised.

Cone excision of the major ducts (also known as Hadfield’s excision) (subareolar resection): Indicated if the duct of
origin of nipple bleeding is uncertain or when there is bleeding or discharge from multiple ducts

The entire major duct system can be excised for histological examination without sacrifice of the breast form.
A periareolar incision is made and a cone of tissue is removed with its apex just deep to the surface of the
nipple and its base on the pectoral fascia.

Page and Dupont classification:

******ebook converter DEMO Watermarks*******

Lobular Carcinoma in Situ (LCIS) RR, Relative Risk:

Ratio of observed incidence over the incidence in women without proliferative disease.
Fibrocystic change with no, usual, or mild hyperplasia.
Fibrocystic change with hyperplasia greater than mild or usual, papilloma, papillomatosis,
sclerosingadenosis, radial scar, and other findings.

Any diagnosis of atypical ductal or lobular hyperplasia, or both.

Fibrocystic Changes and Breast Pain:

The condition previously referred to as fibrocystic disease represents a spectrum of clinical, mammographic,
and histologic findings and is common during the fourth and fifth decades of life, generally lasting until
menopause.
Fibrocystadenosis is defined as cyclical mastalgia with Nodularity.
An exaggerated response of breast stroma and epithelium to various circulating and locally produced hormones
and growth factors is frequently characterized by the constellation of breast pain, tenderness and nodularity.
Symptomatically, the condition manifests as premenstrual cyclic mastalgia, with pain and tenderness to touch.
Pain without other signs or symptoms of breast cancer is uncommon, occurring in only approximately 5% of
patients with breast cancer.
In women with breast pain and an associated palpable mass, the presence of the mass is the focus of evaluation
and treatment.
Normal ovarian hormonal influences on breast glandular elements frequently produce cyclic mastalgia, pain
generally in phase with the menstrual cycle.
Noncyclic mastalgia is more likely idiopathic and difficult to treat.
Women 30 years and older with noncyclic mastalgia should undergo breast imaging with mammography and
ultrasonography in addition to a physical examination.
If examination reveals a mass, this should become the focus of subsequent evaluation. Occasionally, a simple
******ebook converter DEMO Watermarks*******
 cyst may cause noncyclic breast pain, and aspiration of the cyst usually resolves the pain. Most patients with
simple cysts do not require further evaluation.
Patients with complex cysts with solid intracystic components require additional evaluation including biopsy of
the solid components.
Fibrocystic changes are usually seen on mammography as diffuse or focal radiologically dense tissue. On
ultrasonography, cysts are seen in one third of all women 35 to 50 years old.

Treatment: Fibrocystadenosis is to be managed conservatively.

Danazol is the only FDA approved drug.
Others:
Evening Primrose oil.
NSAIDs.
Indications for surgery:
1. Intractable mastalgia.
2. Florid epitheliosis.
3. Bloodgood cyst (if its recurs after repeated aspirations).
Sub cutaneous mastectomy is preferred.
Bluedome Cyst of Bloodgood solitary cystic enlargement out of the multiple cysts/nodules.

Features: Tense ; fluctuant ; non tender with a Bluish Capsule.

TOC: aspiration is the preffered

Schimmelbusch Disease: Multiple ; diffuse cystic enlargement.

Fibroadenoma (important exam topic):
These usually arise in the fully developed breast between the ages of 15 and 25 years, although occasionally they
occur in much older women.
They arise from hyperplasia of a single lobuleusually grow up to 2–3 cm in size.
A fibroadenoma does not require excision unless associated with suspicious cytology, it becomes very large or the
patient expressly desires the lump to be removed.
Giant fibroadenomas occasionally occur during puberty. They are over 5 cm in diameter and are often rapidly
growing but, in other respects, are similar to smaller fibroadenomas and can be enucleated through a submammary
incision. They are more common in the Afro-Caribbean population.
Fibroadenomas:

Fibroadenomas are benign solid tumors composed of stromal and epithelial elements. They are disorder of
Lobule proliferation.
Fibroadenoma is the second most common tumor in the breast (after carcinoma) and is the most common tumor
in women younger than 30 years.
In contrast to cysts, fibroadenomas most often arise during the late teens and early reproductive years.
Fibroadenomas are rarely seen as new masses in women after age 40 or 45 years.
Clinically, fibroadenomas manifest as firm masses that are easily movable and may increase in size over
several months. They slide easily under the examining fingers (hence known as Breast Mouse).
May be lobulated or smooth.
They are surrounded by a well-marked capsule and can thus be enucleated through a cosmetically appropriate
incision.
Mammography is of little help in discriminating between cysts and broadenomas. Popcorn Calcification is
seen.
IOC: Ultrasonography and guided FNAC.
Fibroadenomas are benign tumors, although neoplasia may develop in the epithelial elements within them. 50%
******ebook converter DEMO Watermarks*******
 of neoplasias that involve fibroadenomas are LCIS, 35% are invasivefi carcinomas, and 15% are intraductal
carcinoma.
When a tissue diagnosis con rms that the breast mass is a fibroadenoma, the patient can be reassured, and
surgical excision is not needed.
If the patient is bothered by the mass or it continues to grow, the mass can be excised.

Two subtypes of fibroadenoma are recognized on HPE:

Intracanalicular: large and soft, mainly cellular. Stroma with distorted duct.
Pericanalicular: small and hard, mainly fibrous. Stroma with normal duct.

Giant fibroadenoma is a descriptive term applied to a fibroadenoma that attains an unusually large size (typically
>5 cm).
Juvenile fibroadenoma refers to a large fibroadenoma that occasionally occurs in adolescents and young adults and
histologically is more cellular than the usual fibroadenoma.

Although these lesions may display remarkably rapid growth, surgical removal is curative.

Complex fibroadenoma: Is a variant of fibroadenoma with fibrocystic changes like

1. Apocrine metaplasia.
2. Cyst formation.
3. Sclerosing adenosis.

Occasionally it may turn into malignancy unlike usual fibroadenomas. Core biopsy is needed to confirm the
condition.

Indications for surgery in FA:

1. Size > 3cm.
2. Giant FA.
3. Complex FA.
4. Recurrent FA.
5. Cosmesis.
Surgeries done: Enculeation (preferred) or Excision.
Phyllodes tumour:

Also known as sero- cystic disease of Brodie or cystosarcoma phyllodes.

These are usually benign tumours.
Occur in women over the age of 40 years but can appear in younger women.
They present as a:
Large, sometimes massive, tumour
Unevenly bosselated surface.
Occasionally, ulceration of overlying skin occurs because of pressure necrosis.
Remain mobile on the chest wall.

They metastasise via the bloodstream.

On HPE: Cystic spaces with leaf like arrangements.
Treatment:
Treatment for the:

Benign type is enucleation in young women or wide local excision.

Massive tumours, recurrent tumours and those of the malignant type will require simple mastectomy.

******ebook converter DEMO Watermarks*******

Carcinoma Breast:
Risk Factors:

******ebook converter DEMO Watermarks*******

Risk Assesment Scores:
1. Gail Model.
2. Brca Pro.
3. Tyrer Cuzick.
4. Couch.
5. Clauss.

******ebook converter DEMO Watermarks*******

 Candidates with relative risk >1.67% should be started with Tamoxifen.

Classification of Primary Breast Cancer:

******ebook converter DEMO Watermarks*******
 Ductal carcinoma is the most common variant with lobular carcinoma occurring in up to 15 per cent of
cases.
There are subtypes of lobular cancer including the classical type, which carries a better prognosis than the
pleomorphic type.
Occasionally, the picture may be mixed with both ductal and lobular features.
There are different patterns of spread depending on histological type.
If there is doubt whether a tumour is predominantly lobu lar in type, immune-histochemical analysis using
the e-cadherin antibody, which reacts positively in lobular cancer, will help in diagnosis.
Rarer histological variants, usually carrying a better prognosis, include colloid or mucinous carcinoma, whose
cells produce abundant mucin.
Medullary carcinoma, with solid sheets of large cells often associated with a marked lymphocytic reaction.
Tubular carcinoma is a low grade variant which has best prognosis.
Invasive lobular carcinoma is commonly multifocal and/or bilateral, hence the increasing use of MRI for
assessment.
Cases detected via the screening programme are often smaller and better differentiated than those presenting to
the symptomatic service and are of a special type.

Inflammatory carcinoma is a fortunately rare:

Highly aggressive cancer that presents as a painful, swollen breast, which is warm with cutaneous oedema.
This is the result of blockage of the subdermal lymphatics with carcinoma cells.
Inflammatory cancer usually involves at least one-third of the breast and may mimic a breast abscess.
A biopsy will confirm the diagnosis and show undifferentiated carcinoma cells.
It used to be rapidly fatal but with aggressive chemotherapy and radiotherapy and with salvage surgery the
prognosis has improved considerably.

In situ carcinoma:

It is preinvasive cancer that has not breached the epithelial basement membrane.
This was previously a rare, usually asymptomatic, finding in breast biopsy specimens but is becoming
increasingly common because of the advent of mammographic screening.
It now accounts for over 20 per cent of cancers detected by screening.
******ebook converter DEMO Watermarks*******
 In situ carcinoma may be ductal (DCIS) or lobular (LCIS), the latter often being multifocal and bilateral.
Both are markers for the later development of invasive cancer, which will develop in at least 20 per cent of
patients.
Although mastectomy is curative, this constitutes overtreatment in many cases. The best treatment for in situ
carcinoma is the subject of a number of ongoing clinical trials.
DCIS may be classified using the Van Nuys system, which combines the patient’s age, type of DCIS and
presence of microcalcification, extent of resection margin and size of disease.
Patients with a high score benefit from radiotherapy after excision, whereas those of low grade, whose tumour
is completely excised, need no further treatment.
Staining for estrogen and progesterone receptors is now considered routine, as their presence will indicate the
use of adjuvant hormonal therapy with tamoxifen or an aromatase inhibitor.
Tumours are also stained for c-erbB2 (also known as HER-2/neu) (a growth factor receptor) as patients who are
positive can be treated with the monoclonal antibody trastuzumab (Herceptin®), either in the adjuvant or
relapse setting.

Breast cancer site:

Triple assessment:

In any patient who presents with a breast lump or other symptoms suspicious of carcinoma, the diagnosis
should be made by a combination of clinical assessment, radiological imaging and a tissue sample taken for
either cytological or histological analysis, the so-called triple assessment.
The positive predictive value (PPV) of this combination should exceed 99.9 per cent.

******ebook converter DEMO Watermarks*******

Breast Imaging:
A. Screening for breast cancer:
Screening mammogram:

Mammography delivers radiation of 0.1 cGy (10 rads). X- ray chest gives 25% radiation as compared to
mammography.
Its sensitivity is lower for dense breast so not useful for younger patient (< 35 years).
Mammography is more accurate than clinical examination in detecting early breast cancer with accuracy of
90%.
Screening mammogram lowers mortality from breast cancer by 33%.
It is performed in the asymptomatic patient and consists of two standard views, mediolateral oblique (MLO)
and craniocaudal (CC).
MLO view images of greater volume, including upper outer quadrant and axillary tail.
CC gives better visualization of Medial aspect and allows greater breast compression.
The current US recommendation is annual screening mammography for women aged 40 to 70 years.
Breast lesions on mammograms are classified according to the American College of Radiology by BI-RADS
(Breast Imaging Reporting and Database System) scores:
0 = Needs further imaging; assessment incomplete.
1 = Normal; continue annual follow-up (risk of malignancy: 1/2,000).
2 = Benign lesion; no risk; continue annual follow-up (risk of malignancy: 1/2,000).
3 = Probably benign lesion; needs 4- 6 months follow-up (malignancy risk: 1% to 2%).
4 = Suspicious for breast cancer; biopsy recommended (risk of malignancy: 25% to 50%).
5 = Highly suspicious for breast cancer; biopsy required (75% to 99% are malignant).
6 = Known biopsy- proven malignancy.

******ebook converter DEMO Watermarks*******

 Malignant mammographic findings:
Ill-defined margin, irregular stellate speculation.
Comet tail, high density.
Heterogenous mass.
Wide halo.
Microcalcification (< 0.5 mm size).
Architectural distortion.
Thickened skin.
Thickened cooper ligament,
Obiterated subcutaneous retromammary space.
New or spiculated masses
Clustered microcalcifications in linear or branching array
Architectural distortion.
Benign mammographic findings
Radial scar. Generally due to fibrocystic breast condition (FBC) associated with proliferative epithelium in
the center of the fibrotic area in approximately one third of cases. Appearance often mimics malignancy;
so biopsy is needed to rule out malignancy.
Fat necrosis. Results from local trauma to the breast. It may resemble carcinoma on palpation and on
mammography. The fat may liquefy instead of scarring, which results in a characteristic oil cyst. A biopsy
may be needed to rule out malignancy.
Milk of calcium. Associated with FBC; caused by calcified debris in the base of the acini. Characteristic
microcalcifications appear discoid on craniocaudal view and sickle-shaped on mediolateral oblique view.
These are benign and do not require biopsy.
Curvilinear – Fat necrosis of breast
Cysts cannot be distinguished from solid masses by mammography; ultrasound is needed to make this
distinction.
******ebook converter DEMO Watermarks*******
 Screening in high-risk patients:
For patients with known BRCA mutations, annual mammograms and semiannual physical examinations
should begin at age 25 to 30 years.
In patients with a strong family history of breast cancer but undocumented genetic mutation, annual
mammograms and semiannual physical examinations should begin 10 years earlier than the age of the
youngest affected relative and no later than age 40 years.
Magnetic resonance imaging (MRI)is recommended for screening in selected high-risk patients with:
A lifetime risk of breast cancer greater than 20% as defined by available risk assessment tools (e.g.,
BRCAPRO, Gail, Claus, and Tyrer-Cuskick models).
BRCA mutations.
A first-degree relative (parent, sibling, child) with a BRCA1 or BRCA2 mutation.
History of radiation to the chest wall between the ages of 10 to 30 years (e.g., Hodgkin lymphoma
patients).
Li-Fraumeni, Cowden or Bannayan-Riley-Ruvalcaba syndromes.

B. Diagnostic imaging:

Diagnostic mammogram has a false-negative and false-positive rates of approximately 10%.

A normal mammogram in the presence of a palpable mass does not exclude malignancy and further workup
should be performed with ultrasound, MRI, and/or biopsy.
Ultrasonography is used to further evaluate any lesion identified by physical examination or mammography. It
can determine whether a lesion is solid or cystic and can define the size, contour, or internal texture of the
lesion. Though it is not a useful screening modality (due to significant false-positive rates) but when used as an
adjunct with mammography, ultrasonography may improve diagnostic sensitivity of benign findings to greater
than 90%, especially among younger patients for whom mammographic sensitivity is lower due to denser
breast tissue.
MRI is useful with mammography to determine extent of disease, multicentric disease in the dense breast, to
assess the contralateral breast, and axillary metastases and an unknown primary, and in patients in whom
mammogram, ultrasound, and clinical findings are inconclusive. It is also useful for assessing chest wall
involvement.

Breast Biopsy:
A. Palpable masses:

Fine-needle aspiration biopsy (FNAB) is reliable and accurate, with sensitivity greater than 90%. FNAB can
determine the presence of malignant cells and estrogen and progesterone receptor status but does not give
information on tumor grade or the presence of invasion. Nondiagnostic aspirates require an additional biopsy,
either surgical or core needle biopsy.
Core biopsy is preferred over FNAB as it can distinguish between invasive and noninvasive cancer and
provides information on tumor grade as well as receptor status. For indeterminate specimens, a surgical biopsy
is necessary.
Excisional biopsy should primarily be used when a core biopsy cannot be done. It is performed in the operating
room and incision is planned so that they can be incorporated into a mastectomy if required.
Incisional biopsy is indicated for large breast mass (where complete excision is not possible) suspicious for
malignancy but for which a definitive diagnosis cannot be made by FNAB or core biopsy. For inflammatory
breast cancer with skin involvement, an incisional biopsy can consist of a skin punch biopsy.

B. Nonpalpable lesions:
Any image-detected abnormality requires minimally invasive breast biopsy for pathologic diagnosis and correlation
between pathology results and imaging findings is mandatory.

Patients with histologically benign findings on percutaneous biopsy do not require open biopsy if imaging and
pathological findings are concordant.
Patients with high-risk lesions on image-guided biopsy (ADH, ALH, lobular carcinoma in situ, radial scar) may
have malignancy at the same site and should undergo a surgical biopsy.
Stereotactic core biopsy is used for nonpalpable mammographically detected lesions, such as
******ebook converter DEMO Watermarks*******
 microcalcifications, which cannot be seen with ultrasonography.
Contraindications are: lesions close to the chest wall or in the axillary tail and thin breasts. Superficial
lesions and lesions beneath the nipple-areola complex are also not approachable with stereotactic
techniques.
Nondiagnostic and insufficient specimens should undergo needle-localized excisional biopsy.
Ultrasound-guided biopsy is the preferred method if a lesion can be visualized with ultrasound because it
is generally easier than a stereotactic core biopsy. Lesions with a cystic component are better visualized
with ultrasound, and ultrasound-guided biopsy can be used to aspirate the cyst as well as to provide core
biopsy specimens.

Ultrasonography:
Main indications USG in breast is to differentiation between cystic and solid lesions:

Evaluation of a palpable lesion in a mammographically dense breast (for example young, pregnant or lactating
patient).
Evaluation of a lesion detected at mammography or mammographic asymmetry.
Detection of an abscess in an infectious breast.
Evaluation after breast cancer treatment and breast augmentation.
Evaluation of axillary lymph nodes and guidance for interventional procedures
Ultrasound can detect mammographically occult cancers, but it is generally accepted that US.

Microcalcifications with no associate mass are not usually reliably detectable at US.
Magnetic Resonance Imaging:

Most sensitive method for the detection of invasive breast cancer (based on lesion enhancement after contrast
agent administration and lesion morphology).

Diagnostic Criteria:

Well-defined margins indicate benignity, while ill-defined or spiculated lesions are suggestive of malignancy.
Internal septations, if seen, are specific for fibroadenomas.
Enhancement in benign lesions is homogeneous and proceeds centrifugally.
Benign lesions also usually enhance less and do so more slowly than malignant lesions.
In malignant lesions enhancement is often inhomogeneous or rim-like and tends to proceed centripetally.
Enhancement kinetics can also be analyzed by the shape of time-signal intensity curve: a continuous increase in
signal intensity is considered a benign finding, a rapid increase followed by a washout phenomenon is
considered malignant.

Main indications of MRI:American Cancer Society Guidelines for Magnetic Resonance Imaging Screening.

Annual Mri Recommended Based on Evidence:

BRCA mutation.
Untested first degree relative of BRCA carrier.
Lifetime risk of breast cancer 20% to 25%.
Annual Mri Recommended Based on Expert Opinion:
Radiation to chest between age 10 and 30.
Li-Fraumeni syndrome and first-degree relatives.
Cowden and Bannayan-Riley-Ruvalcaba syndromes and first-degree relatives.
Insufficient Evidence to Recommend for or Against MRI:
Lifetime breast cancer risk 15% to 20%.
Lobular carcinoma in situ.
Atypical hyperplasia (lobular or ductal).
Extremely or heterogeneously dense breasts on mammogram.

Other Imaging Modalities:

******ebook converter DEMO Watermarks*******
 Computed tomography has not been recommended for breast imaging, mainly because of high radiation dose. It
has been successfully used in regional staging of small breast cancer before breast conserving surgery.
Electrical impedance scanning is a new technique, which is based upon the principle that malignant cells
exhibit altered local dielectric properties and show measurably higher conductivity values.

Staging:
American Joint Committee on Cancer TNM Staging for Breast Cancer:

American Joint Committee on Cancer Classification for Breast Cancer:

******ebook converter DEMO Watermarks*******

A bone scan if the alkaline phosphatase or calcium level is elevated.

CT scan of the liver if liver function panel is abnormal.

Patients with clinical stage III disease should undergo bone scan and CT scan due to a high probability of
distant metastases.

Manchester Staging systems for Breast Cancer:

Stage I Tumour confined to the breast with skin involvement less than the size of the tumour.
Stage II Tumour confined to the breast with palpable mobile axillary lymph nodes.
Stage III Locally advanced breast cancer with skin fixation larger than the tumour. Cutaneous ulcers or fixity
to pectoralis fascia may be present. Peau d’orange or satellite chest wall nodules. Fixed axillary nodes,
supraclavicular nodal involvement.
Stage IV Distant metastases.

III Tumor Biomarkers and Prognostic Factors:

Tumor size and grade are the most reliable pathologic predictors of outcome for patients without axillary
nodal involvement.
The Nottingham score combines:
Histologic grade based on glandular differentiation.
Mitotic count.
Nuclear grade.
Hormone receptors: Expression of estrogen receptors (ERs) and progesterone receptors (PRs) should be
evaluated by immunohistochemistry. Intense ER and PR staining is a good prognostic factor.PR status may be
more sensitive than ER status in determining which patients are likely to respond to hormonal manipulation. Up
to 80% of patients with metastatic PR-positive tumors improve with hormonal manipulation.
Her2/neu (ERB2): Her2/neu is a member of the epidermal growth factor family and is involved in cell growth
regulation. Overexpression is seen in approximately 30% of patients with breast cancer. Overexpression of
Her2/neu is a poor prognostic factor.
Other negative markers include those tumors that do not express any tumor biomarkers (“triple negative”), the
presence of lymphovascular invasion, and other indicators of a high proliferative rate (>5% of cells in the S
phase; >20% Ki-67).

IV. Noninvasive:
Noninvasive (in situ) breast cancer: DCIS (ductal carcinoma in situ) or LCIS (lobular carcinoma in situ) are lesions
with malignant cells that have not penetrated the basement membrane of the mammary ducts or lobules,
respectively.
******ebook converter DEMO Watermarks*******
DCIS (Ductal Carcinoma In Situ):
Classification of DCIS is based on the microscopic characters of:
1. Architecture (growth pattern).
2. Nuclear features.
Classification of DCIS by the Predominant Architecture:

Papillary/Micropapillary type:
Multiple isolated papillary projections, most of which lack fibrovascular stalks.
Papillae become fused to form Roman bridges and arches giving the impression of rigidity.
Cribriform type:
Tumor cells are arranged in a sieve-like pattern, multiple small round glands growing in a larger gland or
duct. These glands are confluent without fibrous walls.
Most tumor cells have low nuclear grade.
Solid type:
Tumor cells fill the ducts and ductules as solid sheets.
Nuclear grade is predominantly intermediate or high grade. Necrosis is usually focal.
Comedo type (May present with lump or discharge):
Central necrosis of the involved ducts is a prominent feature.
Calcification occurs within the necrosis.
High nuclear grade in most tumors.

Prognosis of DCIS (by pathological analysis):

Nuclear grade is more important than architecture (growth) pattern.

Status of surgical margin.
Lesion size.

DCIS is treated as a malignancy because DCIS has the potential to develop into invasive cancer.

It is usually detected by mammography as clustered pleomorphic calcifications.

Physical examination is normal in the majority of patients.
It may advance in a segmental manner, with gaps between disease areas.
It can be multifocal within the same index quadrant or multicentric (in different quadrants).
Up to 20% of high-grade DCIS lesions or large lesions (>4 cm) will harbour invasive carcinoma.
Histology: There are five architectural subtypes: papillary, micropapillary, solid, cribriform, and comedo. (It
can also grouped as comedo versus noncomedo).
Treatment:
Surgical excision alone with 1 cm margin is associated with a local recurrence rate of 14% at 12 years.
The addition of adjuvant radiation reduces the local recurrence rate to 2.5%. Approximately half of the
recurrences present as invasive ductal carcinomas. Surgical options depend on the extent of disease, grade,
margin status, multicentricity of disease, and patient age.
Partial mastectomy: For unicentric lesions.
Needle localization is required to identify the area to be excised.

******ebook converter DEMO Watermarks*******

 Mastectomy: Total mastectomy is recommended for:
Multicentric lesions.
Extensive involvement of the breast (disease extent relative to breast size).
Persistently positive margins.
Size >4 cm.
Assessment of axillary lymph nodes: Axillary dissection is not performed for pure DCIS.
Sentinel lymph node biopsy may be considered when there is a chance of finding invasive cancer. (e.g., >4
cm, palpable, or high grade).
A positive sentinel node indicates invasive breast cancer and; a completion axillary dissection is then
indicated.
Adjuvant therapy:
For pure DCIS, there is no benefit from systemic chemotherapy.
ER-positive DCIS, adjuvant tamoxifen can reduce the risk of breast cancer recurrence by 37% over 5
years. However, there is no survival benefit. Aromatase inhibitor is used in postmenopausal patients
though bone loss is a significant side effect of this long-term treatment
Adjuvant radiation is indicated in partial mastectomy especially in younger women with close margins or
large tumors. However, there is no survival benefit.
The Van Nuys Prognostic Index is based on lesion size,Micro Calcfication margin, tumor grade, presence
of necrosis, and age and is used to determine greatest risk of recurrence.
The low-scoring group may be treated with partial mastectomy alone. The intermediate-scoring group has
been shown to benefit from adjuvant radiation therapy, and the high-scoring group should undergo
mastectomy because the risk of recurrence with partial mastectomy with or without radiation is high.

Van Nuys Scoring System:

******ebook converter DEMO Watermarks*******

LCIS (Lobular Carcinoma In Situ):
LCIS is not considered a preinvasive lesion but rather an indicator for increased cancer risk of approximately 1% per
year (20% to 30% at 15 years) and is not treated as a breast cancer.

Only seen in female breast.

It may be multifocal and/or bilateral (MCQ).
The cancer that develops may be invasive ductal (more common) or lobular and may occur in either breast.
LCIS has loss of E-cadherin (involved in cell–cell adhesion).
Pleomorphic LCIS is a particularly aggressive subtype of LCIS that is treated more like DCIS; it tends to have
less favorable biological markers.
Characterized by cytoplasmic mucoid globules and distortion of terminal ductal lobular unit.
Invasive lobular cancer cell invade in linear strands (Indian filing pattern).
Presents with Vague discreet ill-defined mass
Invasive lobular cancer Metastasize to Peritoneal surface and meninges.
Neighborhood calcification is a unique feature of LCIS.
Treatment options are.
Lifelong close surveillance.
Bilateral total mastectomies with immediate reconstruction for high risk women (mastectomy gives
protection by 90%).
prophylaxis with tamoxifen (In ER/PR positive patient)- Gives protection by 50%.
Invasive Breast Cancer:
Histology consists of five different subtypes: infiltrating ductal (75% to 85%), infiltrating lobular (5% to
10%), medullary (5% to 7%), mucinous (3%), and tubular (1% to 2%).

******ebook converter DEMO Watermarks*******

 Invasive breast cancer with productive fibrosis (Schirrous - NST) is most common type- 80%.
Lymph nodes involvement is seen in 60%.
Breast lump (Solitary, hard) is most common presentation.

Medullary cancer – 4%:

Although, the majority of medullary carcinomas are not associated with germline BRCA1 mutations,
hypermethylation of the BRCA1 promoter is observed in 67% of cases.
Gross- The tumor is soft, fleshy (medulla is Latin for “marrow”), and well circumscribed and hemorrhagic.
Bulky, deep with rapid increase in size.
Dense lymphoreticular infiltrate of lymphocytes and plasma cells with large pleomorphic nuclei.
HER2/neu overexpression is not observed and Lymph node metastases are infrequent.
Medullary carcinomas have a slightly better prognosis than do NST carcinomas.

Mucinous (colloid) – 2%:

Seen in elderly, Bulky.

The tumor is soft or rubbery and has the consistency and appearance of pale gray-blue gelatin.
The borders are pushing or circumscribed.
The tumor cells are arranged in clusters and small islands of cells within large lakes of mucin.
Lymph node involvement is seen in 1/3rd of cases. Prognosis is better than NOS.

Papillary cancer – (2%):

Seen in 7th decade, more common in non-white females

Usually small, papilla having fibrovascular stalk and multi-layered epithelium
Invasive papillary carcinomas are usually ER positive and have a favorable prognosis.
In contrast, invasive micropapillary carcinomas are more likely to be ER negative and HER2 positive. Lymph
node metastases are very common, and the prognosis is poorer than tubular.

Tubular carcinoma (2%)- Best prognosis (MCQ):

Smaller than 1 cm in size

Well-formed tubules and are sometimes mistaken for benign sclerosing lesions.
However, the myoepithelial cell layer is absent, placing the tumor cells in direct contact with the stroma
******ebook converter DEMO Watermarks*******
 More than 95% of all tubular carcinomas are diploid, ER positive, and HER2/neu negative.

Surgical options for stage I and II Breast Cancer:

Mastectomy with or without reconstruction.

Modified radical mastectomy (MRM) involves total (simple) mastectomy and axillary lymph node
dissection. It is indicated for patients with clinically positive lymph nodes.
Total (simple) mastectomy with SLNB is for patients with a clinically negative axilla. A skin-sparing
mastectomy (preserves skin envelope and inframammary ridge) may be performed if immediate
reconstruction is planned to improve cosmesis.
Immediate reconstruction at the time of mastectomy can be done. Options include latissimus dorsi
myocutaneous flaps, transverse rectus abdominis myocutaneous flaps, and inflatable tissue expanders
followed by exchange for saline or silicone implants. Immediate reconstruction has been shown not to
affect patient outcome adversely.
Follow-up after mastectomy:
Physical examination every 3 to 6 months for 3 years, then every 6 to 12 months for the next 2 years,
and then annually.
Mammography of the contralateral breast should continue yearly.
Regular gynaecologic follow-up is recommended for all women (tamoxifen increases risk of
endometrial cancer).
Breast conservation therapy (BCT): Partial mastectomy and SLNB (or axillary lymph node dissection)
followed by breast irradiation.
Several trials have demonstrated that BCT with adjuvant radiation therapy has similar survival and
recurrence rates to those for MRM.
Contraindications for BCT: A patient who may be unreliable with follow-up or radiation therapy
(may involve radiation treatment 5 days a week for 5 to 6 weeks); when the extent of disease prevents
adequate negative margins; a high tumor-to-breast size ratio, which prevents adequate resection
without major deformity; persistently positive margins on re-excision partial mastectomy; and
inability to receive adjuvant radiation (e.g., prior radiation to the chest wall; first- and second-
trimester pregnancy in which the delay of radiation to the postpartum state is inappropriate; collagen
vascular diseases such as scleroderma). (Pregnancy/ Multicentric tumour/ Previous irradiation/
Collagen vascular disease/ tumour > 4 cm in size/ N1 stage).
Adjuvant radiotherapy decreases the recurrence rate from 30% to less than 7% at 5 years and is a
required component of BCT.
Follow-up after BCT. Physical examination every 3 to 6 months for 3 years, then every 6 to 12
months for the next 2 years, and then annually A post treatment mammogram of the treated breast is
performed at 6 months after completion of radiation therapy. Mammograms are then performed every
6 to 12 months after the new baseline mammogram until the surgical changes stabilize and then
annually. Contralateral breast mammography remains on an annual basis. Regular gynecologic
follow-up is recommended.
Management of the axilla. Approximately 30% of patients with clinically negative exams will have
positive lymph nodes in an axillary lymph node dissection (ALND) specimen. Thus, sentinel lymph node
biopsy was developed to provide sampling of the lymph nodes without needing an ALND.
SLNB involves injection of blue dye (either Lymphazurin or methylene blue) or technetium-labeled
sulfur colloid. The combination of blue dye and radioisotope provides higher node identification rates
and increases the sensitivity of the procedure (95%).
The SLN is identified by its blue color, and/or by high activity detected by a handheld gamma probe.
Palpable nodes are also sentinel nodes, even if not blue or radioactive.
The first 2–5 blue nodes identified are considered the sentinel nodes
20% to 30% of the time more than one SLN is identified.
If the SLN is positive for metastasis (micrometastasis 0.2 mm or larger, not isolated tumor cells-
considered N0), a standard ALND is performed. Or Radiation therapy can be administered to the
axilla if the patient refuses ALND.
ALND. Patients with clinically positive lymph nodes, with positive SLN should undergo ALND for
local control. ALND involves:
Removal of level I and level II nodes and, if grossly involved, possibly level III nodes. Motor
******ebook converter DEMO Watermarks*******
 and sensory nerves are preserved unless there is direct tumor involvement.
An ALND should remove 10 or more nodes.
Patients with 4 or more positive lymph nodes should undergo adjuvant radiation to the axilla.
Selective patients with 1 to 3 positive nodes may also benefit from radiation therapy.
Most frequent postoperative complications:
Wound infections and seromas. Persistent seroma may be treated with repeated aspirations or
reinsertion of a drain.
Pain and numbness in the axilla and upper arm, impaired shoulder mobility.
Lymphedema: Seen in 10% to 40% of women undergoing axillary dissection; radiation to the
axilla increases the risk of this complication. It is managed by early intervention with intense
physiomassage; graded pneumatic compression devices and a professionally fitted compression
sleeve. Lymphedema itself increases the risk of developing angiosarcoma (Stewart Trevis
sundrome).
Adjuvant chemotherapy is indicated in:
All node-positive patients.
Typical regimens comprise of combination of cyclophosphamide and an anthracycline, followed by a
taxane.
Patients with ER-positive tumors receive adjuvant hormonal therapy for 10 years. Tamoxifen is given to
premenopausal women, and aromatase inhibitors are given to postmenopausal women (aromatase
inhibitors are not used in premenopausal women- MCQ).
In postmenopausal women older than 70 years, chemotherapy is performed less frequently. In
postmenopausal women with tumors with ER or PR positivity, tamoxifen or an aromatase inhibitor is
frequently the sole adjuvant medical therapy.
In patients with Her2/neu-positive tumors, Trastuzumab is a recombinant monoclonal antibody that binds
to Her2/neu receptor to prevent cell proliferation. It is usually administered intravenously monthly for 12
months. The most serious toxicity is cardiac failure.
Node-negative patients:
Up to 30% of node-negative women die of breast cancer within 10 years if treated with surgery alone.
Node-negative patients who are at high risk and benefit the most from adjuvant chemotherapy
include those with tumors greater than 1 cm, higher tumor grade, Her2/neu expression, aneuploidy,
Ki-67 expression, increased percentage in S phase, lymphovascular invasion, and ER/PR-negative
tumors.
Polychemotherapy plus tamoxifen is superior to tamoxifen alone in increasing disease-free and
overall survival, especially in ER-negative patients, regardless of tumor size.
Patients who have node-negative disease and whose tumors are < 1 cm and ER-positive may be
spared adjuvant chemotherapy but still may benefit from tamoxifen.
Adjuvant radiation.
Indications for adjuvant radiation to the chest wall and axilla:
T3 and T4 tumours/ fixed to the pectoral fascia/ positive surgical margins or skin involvement/ involved
internal mammary nodes/ inadequate or no axillary dissection/ four or more positive lymph nodes/ and
residual tumor on the axillary vein.
Presence of one to three positive axillary nodes is a relative indication.
Adjuvant whole-breast radiation after BCT decreases the breast cancer recurrence rate from 30% to less than
7% at 5 years.
Complications:
Skin changes/ and rarely interstitial pneumonitis, spontaneous rib fracture, breast fibrosis, pericarditis,
pleural effusion, and chest wall myositis.
Radiation to the axilla can increase the incidence of lymphedema and axillary fibrosis.
Angiosarcoma (MCQ) can occur as a late complication.
Vascular Endothelial Growth Factor (VEGF)
Bevacizumab (Avastin) is a monoclonal antibody directed against VEGF. This growth factor stimulates
endothelial proliferation and neoangiogenesis in cancer. It was approved for Breast cancer bu FDA but its
approval for breast cancer was revoked in 2011.

Locally Advance Breast Cancer (LABC) comprises T3 or T4, N1 or greater, and M0 cancers (stages IIIA and
IIIB).
******ebook converter DEMO Watermarks*******
 Noninflammatory LABC (chest wall or skin involvement, skin satellites, ulceration, fixed axillary nodes)
Patients should receive neoadjuvant chemotherapy (often cyclophosphamide combined with an
anthracycline and taxane),
followed by surgery (MRM) and radiation.
Adjuvant radiation to the chest wall and regional nodes and adjuvant chemotherapy follow surgery.
Approximately 20% of patients with stage III disease present with distant metastases after appropriate
staging has been performed.
Inflammatory LABC (T4d)
This is characterized by erythema, warmth, tenderness, and edema (peau d’orange).
It represents 1% to 6% of all breast cancers.
Skin punch biopsy confirms the diagnosis.
Approximately 30% of patients have distant metastasis at the time of diagnosis.
Inflammatory breast cancer requires aggressive multimodal therapy because median survival is
approximately 2 years, with a 5-year survival of only 5%.
Follow-up. Due to higher risk for local and distant recurrence, patients should be examined every 3 months by
all specialists involved in their care.
Locoregional recurrence. Patients with locoregional recurrence should have a metastatic workup to
exclude visceral or bony disease and should be considered for systemic chemotherapy or hormonal
therapy.
Recurrence in the breast after BCT requires total (simple) mastectomy. Provided margins are
negative.
Recurrence in the axilla requires surgical resection followed by radiation to the axilla and systemic
therapy.
In recurrence in the chest wall after mastectomy one third of these patients have distant metastases at
the time of recurrence, and greater than 50% will have distant disease within 2 years. Multimodal
therapy is essential.

Breast Cancer Follow-Up:

******ebook converter DEMO Watermarks*******

Chemoprevention:

Women taking tamoxifen achieved an overall reduction in the risk of developing invasive breast carcinoma of
49% and noninvasive breast cancer of 50%.
In subgroups of women with a history of LCIS and with a history of ADH, tamoxifen reduced the risk of
developing invasive breast cancer by 65% and 86%, respectively.
The toxicities of the drug include an increased risk of endometrial cancer, thrombotic vascular events, and
cataract development. Women on tamoxifen also reported increased vasomotor symptoms (hot flashes)
and vaginal discharge.
Tamoxifen also provided a significant reduction in hip fractures in women older than 50 years of age.
There was no difference noted in the incidence of ischemic heart disease for women taking tamoxifen.
Tamoxifen has been approved by the Food and Drug Administration (FDA) for (1) The treatment of
metastatic breast cancer, (2) adjuvant treatment of breast cancer, and (3) Chemoprevention of invasive or
contralateral breast cancer in high-risk women.
The dosage for chemoprevention is 20 mg/day for 5 years.
The Study of Tamoxifen and Raloxifene (STAR) trial compared tamoxifen to raloxifene (a selective
estrogen receptor modulator). Raloxifene has not been FDA approved for chemoprevention, but was
shown to provide equal risk reduction for the development of invasive breast cancers as tamoxifen.
It was not as effective at reducing the risk of developing noninvasive breast cancer. As its side effect
profile is somewhat different than that of tamoxifen, so it can be considered in patients with relative
contraindications to tamoxifen.

Special Considerations:
I. Breast Conditions During Pregnancy:
A. Bloody nipple discharge:
Bloody nipple discharge may occur in the second or third trimester. It results from epithelial proliferation under
hormonal influences and usually resolves by 2 months postpartum. If it does not resolve, standard evaluation of
pathologic nipple discharge should be performed.
B. Breast masses:
******ebook converter DEMO Watermarks*******
Breast masses occurring during pregnancy include galactoceles, lactating adenoma, simple cysts, breast infarcts,
fibroadenomas, and carcinoma. Fibroadenomas may grow during pregnancy due to hormonal stimulation.

Masses should be evaluated by ultrasound, and a core needle biopsy should be performed for any suspicious
lesion.
Though Mammography can be performed with uterine shielding but is rarely helpful due to increased breast
density.
If a breast lesion is diagnosed as malignant, the patient should be given the same surgical treatment options,
stage for stage, as a nonpregnant woman, and the treatment should not be delayed because of the pregnancy.

C. Breast cancer during pregnancy:

It occurs in approximately 1 in 3-5,000 gestations.

Most common presentation is painless lump, node involvement is seen in 75%.
Standard preoperative staging workup is performed.
Laboratory values such as alkaline phosphatase may be elevated during pregnancy.
For advanced-stage disease, MR scan or ultrasound may be used in lieu of CT scan for staging. Excisional
biopsy can be safely performed under local anesthesia if there is some contraindication to the preferred core
needle biopsy.
MRM has been the standard surgical modality for pregnant patients with breast cancer.
The radiation component of BCT cannot be applied during pregnancy, so it is usually not recommended to
patients in their first or second trimester. For patients in the third trimester, radiation can begin after delivery.
Chemotherapy may be given by the mid-second trimester.
Prognosis stage to stage is same to that of non-pregnant patient.

II. Paget's Disease of the Nipple:

Paget's Disease of the Nipple is characterized by eczematoid changes of the nipple, which may involve the
surrounding areola.

Burning, pruritus, and hypersensitivity may be prominent symptoms.

Paget disease is almost always accompanied by an underlying malignancy, either invasive ductal carcinoma or
DCIS.
Palpable masses are present in approximately 60% of patients.Of these 90% have underlying infiltrating ductal
carcinoma. 10-28% have no clinical lesion.
Mammography should be performed to identify other areas of involvement.
If clinical suspicion is high, a pathologic diagnosis should be obtained by wedge biopsy of the nipple and
underlying breast tissue.
The diagnosis is made by finding large cells with pale, vacuolated cytoplasm, round to oval large nuclei,
prominent nucleoli migrating through the epidermis.
Paget’s cells are positive with mucicarmine stain, and express CEA, epithelial membrane antigen, milk fat
globule by immunohistochemistry.
Paget’s disease is not associate with lobular carcinoma.
Treatment is mastectomy but rarely BCT with excision of the nipple-areolar complex (sometimes called a
central lumpectomy), followed by radiation therapy can be done.

Point to Remember about Paget’s Disease:

Paget’s disease is chronic eczematous eruption of nipple commonly associated with underlying breast cancer.
Paget’s cells are Large, pale staining cells with round nuclei and large nucleoli.
Paget’s cell remain above basement membrane (Equal to Ca in Situ).
******ebook converter DEMO Watermarks*******
 Underlying cancer should always be ruled out by clinical examination (Mass in >50%) an mammography.
Most are ER/PR negative.
Positive with mucicarmine stain, and express CEA, epithelial membrane antigen, milk fat globule by
immunohistochemistry.
Treatment is based on underlying breast cancer stage. If it is isolated paget’s then it is treated by simple
mastectomy.

III. Breast Cancer in Men:

It accounts for less than 1% of male cancers and less than 1% of all breast cancers.
BRCA2 mutations are associated with approximately 4% to 6% of these cancers.

Commonly seen in 6th decade and most are infiltrating ductal carcinoma.
Klinefelter syndrome and testicular feminization syndrome are two main risk factors. Excess exposure to
estrogen (exogenous or endogenous- Cirrhosis, infertility) increases risk of male breast cancer.
Gynaecomastia (except in Klinefelter syndrome) is not a risk factor.
Patients generally present with a nontender hard mass. This contrasts with unilateral gynecomastia, which is
usually firm, central, and tender.
Mammography can be helpful in distinguishing gynecomastia from malignancy. Biopsy of suspicious lesions is
essential, and core needle biopsy is preferred.
Modified radical mastectomy is traditionally the surgical procedure of choice.
85% percent of malignancies are infiltrating ductal carcinoma and are positive for ER (more than females).
35% are HER2/Neu positive.
Adjuvant hormonal, chemotherapy, and radiation treatment criteria are the same as in women. Overall survival
per stage is comparable to that observed in women, although men tend to present in later stages.
Stage to stage prognosis is same as female breast cancer.

IV. Phyllodes Tumors:

Phyllodes Tumors account for 1% of breast neoplasms.

They present as a large, smooth, lobulated (leaf like- Phylloid), bosselated,non-tender, mobile mass.
They can occur in women of any age, but most frequently between ages 35 and 55 years.
Skin ulcerations may occur secondary to pressure of the underlying mass.
FNAB cannot reliably diagnose these tumors; at least a core needle biopsy is needed.
Histologically, stromal overgrowth is the essential characteristic for differentiating phyllodes tumors from
fibroadenomas. There is biphasic proliferation of Strom and mammary epithelium.

Ninety percent are benign (Locally malignant); 10% are malignant. The biologic behavior of malignant tumors
is similar to that of sarcomas.
Most malignant phylloid contain liposarcomatous or Rhabdomyosarcomatous elements rather than
Fibrosarcomatous elements.
Treatment is wide local excision to tumor-free margins or total mastectomy. Axillary assessment with either
SLNB or ALND is not indicated unless nodes are clinically positive (which is rare< 1-2%). Blood born
metastasis (Lung, bone) is seen in < 5% cases
Currently, there is no role for adjuvant radiation; however, tumors greater than 5 cm in diameter and with
evidence of stromal overgrowth may benefit from adjuvant chemotherapy with doxorubicin and ifosfamide.
Patients should be followed with semiannual physical examinations and annual mammograms and chest
radiographs.

******ebook converter DEMO Watermarks*******

Breast Reconstruction After Mastectomy:

Common breast reconstruction techniques include synthetic implants and autologous tissue flaps (including the
latissimus dorsi flap and the transverse rectus abdominis myocutaneousflap). Procedures may be implemented
immediately following mastectomy or can be deferred until after adjuvant therapy is completed.
Techniques of Reconstruction:
Implant Reconstruction:
An implant consists of a silicone shell that contains saline or silicone gel and is available in a variety
of shapes and sizes. To replace missing breast volume, tissue expanders are inserted
submuscularly after the mastectomy. The expander is placed deep in relation to the pectoralis
major and serratus anterior. Initially, a minimally inflated tissue expander is placed; then it is slowly
inflated over a period of weeks, allowing the overlying tissues to stretch. After total expansion is
achieved and the tissues have been allowed to stretch (usually over a period of four to six months),
the expander is replaced with a permanent implant
Autologous Tissue Flaps:
Of several autologous flap options, the most common are the latissimus dorsi flap and the TRAM flap.
The latissimus dorsi flap utilizes the back muscle with its overlying tissue and skin, rotated around to the
mastectomy defect. The latissimus dorsi flap is appropriate for replacing small- to moderate-sized breasts;
candidates include women who smoke, have extensive abdominal scarring, or are morbidly obese. The
disadvantages: This surgery requires an additional scar on the patient’s back and occasionally diminishes
overhead strength.
The transverse rectus abdominis myocutaneous (TRAM) flap is currently considered the gold
standard of breast reconstruction. This autologous tissue transfer is well suited for immediate
reconstruction. The conventional TRAM flap or pedicle flap is supplied superiorly by the superior
epigastric artery and vein. The flap is elevated from an inferior to a superior position, leaving the top
portion of the muscle and the superior pedicle intact.
Free TRAM: In this procedure, the inferior blood supply, the deep inferior epigastric vessels, and the
superior pedicle are divided, then the entire flap is brought up to the mastectomy site. Fine suturing is used
to reattach or anastomose the inferior epigastric vessels microscopically into the recipient vessels - in most
cases, the thoracodorsal vessels.
The gluteal free flap is another option for autologous breast reconstruction. This technique may appeal to
many women with excess tissue in their buttocks; however, it is used rarely because of the technical
complexity of the flap. It also creates a significant donor defect for many women.

******ebook converter DEMO Watermarks*******

Haagensen and Stout Grave Signs of Breast Cancer:
a. Edema of the skin of the breast.
b. Skin ulceration.
b. Chest wall fixation.
d. An axillary lymph node >2.5 cm in diameter.
e. Fixed axillary lymph nodes.
Women with two or more signs had a 42% local recurrence rate and only a 2% 5–year disease-free survival rate
The Scarff–Bloom–Richardson cancer grade system:

Each of these features is assigned a score ranging from 1 to 3. The scores are then added together for a grade that
will range between 3 to 9. This value is then used to grade the tumor as follows:

3-5 Grade 1 tumor (well-differentiated). Best prognosis.

6-7 Grade 2 tumor (moderately-differentiated). Medium prognosis.
8-9 Grade 3 tumor (poorly-differentiated). Worst prognosis.

Molecular classifications:
Molecular classifications of this group of breast cancers (Based on DNA, RNA and protein).

******ebook converter DEMO Watermarks*******

Gene expression profiling, which can measure the relative quantities of mRNA for essentially every gene, has
identified five major patterns of gene expression in the NST group:
luminal A, luminal B, normal, basal-like, and HER2 positive. These molecular classes correlate with prognosis and
response to therapy, and thus have taken on clinical importance.

“Luminal A” (40% to 55% of NST cancers):

ER positive and HER2/neu negative. The majority are well- or moderately differentiated, and most occur
in postmenopausal women.
These cancers are generally slow growing and respond well to hormonal treatments.
Conversely, only a small number will respond to standard chemotherapy.
“Luminal B” (15% to 20% of NST cancers):
This group expresses ER but is generally of higher grade, has a higher proliferative rate, and
overexpresses HER2/neu.
They are sometimes referred to as triple-positive cancers.
They compose a major group of ER-positive cancers that are more likely to have lymph node metastases
and that may respond to chemotherapy.
“Normal breast–like” (6% to 10% of NST cancers):
This is a small group of usually well-differentiated ER-positive, HER2/neu-negative cancers characterized
by the similarity of their gene expression pattern to normal tissue.
It is not yet clear whether or not this is a specific tumor expression pattern.
“Basal-like” (13% to 25% of NST cancers):
Absnce of ER, PR, and HER2/neu and the expression of markers typical of myoepithelial cells (e.g., basal
keratins, P-cadherin, p63, or laminin), progenitor cells, or putative stem cells (e.g. cytokeratins 5 and 6).
“Basal” was chosen as a general term that covers all of these cell types.
Basal-like cancers are a subgroup of ER-PR-HER2/neu “triple-negative” carcinomas.
Members of this group include medullary carcinomas, metaplastic carcinomas (e.g., spindle cell
carcinomas or matrix-producing carcinomas), and carcinomas with a central fibrotic focus.
Many carcinomas arising in women with BRCA1 mutations are of this type.
These cancers are generally high grade and have a high proliferation rate.
They are associated with an aggressive course, frequent metastasis to viscera and the brain, and a poor
prognosis.
However, approximately 15% to 20% response to chemotherapy.
“HER2 positive” (7% to 12% of NST cancers):
This group comprises ER-negative carcinomas that overexpress HER2/neu protein.
In over 90% of HER2/neu positive cancers, overexpression is due to amplification of the segment of DNA on
17q21 that includes the HER2/neu gene.
These cancers are usually poorly differentiated, have a high proliferation rate, and are associated with a high
frequency of brain metastasis.

******ebook converter DEMO Watermarks*******

Embryology:

The thyroid gland arises as an outpouching of the primitive foregut around the third week of gestation.
It originates at the base of the tongue at the foramen cecum.
Endoderm cells in the floor of the pharyngeal anlage thicken to form the medial thyroid anlage that descends in
the neck anterior to structures that form the hyoid bone and larynx.
During its descent, the anlage remains connected to the foramen cecum via an epithelial-lined tube known as
the thyroglossal duct.
The epithelial cells making up the anlage give rise to the thyroid follicular cells.
The paired lateral anlages originate from the fourth branchial pouch and fuse with the median anlage at
approximately the fifth week of gestation.
The lateral anlages are neuroectodermal in origin (ultimobranchial bodies) and provide the calcitonin producing
parafollicular or C cells, which thus come to lie in the superoposterior region of the gland.
Thyroid follicles are initially apparent by 8 weeks, and colloid formation begins by the eleventh week of
gestation.

Developmental Abnormalities:
Thyroglossal Duct Cyst and Sinus:

Thyroglossal duct cysts are the most commonly encountered congenital cervical anomalies.
During the fifth week of gestation, the thyroglossal duct lumen starts to obliterate and the duct disappears by
the eighth week of gestation.
Rarely, the thyroglossal duct may persist in whole or in part.
Thyroglossal duct cysts may occur anywhere along the migratory path of the thyroid, although 80% are found
in juxtaposition to the hyoid bone.
They are usually asymptomatic but occasionally become infected by oral bacteria.
Thyroglossal duct sinuses result from infection of the cyst secondary to spontaneous or surgical drainage of the
cyst and are accompanied by minor inflammation of the surrounding skin.(thus they are always acquired)
Histologically, thyroglossal duct cysts are lined by pseudostratified ciliated columnar epithelium and squamous
epithelium, with heterotopic thyroid tissue present in 20% of cases.
The diagnosis usually is established by observing a 1- to 2-cm, smooth, well-defined midline neck mass that
moves upward with protrusion of the tongue.
Routine thyroid imaging is not necessary, although thyroid scintigraphy and ultrasound have been performed to
document the presence of normal thyroid tissue in the neck.
Treatment involves the “Sistrunk operation,” which consists of en bloc cystectomy and excision of the central
hyoid bone to minimize recurrence.
Approximately 1% of thyroglossal duct cysts are found to contain cancer, which is usually papillary (85%).

Lingual Thyroid:

Failure of the median thyroid anlage to descend normally and may be the only thyroid tissue present.
Intervention becomes necessary for obstructive symptoms such as choking, dysphagia, airway obstruction, or
hemorrhage.
Many of these patients develop hypothyroidism.
Medical treatment options include administration of exogenous thyroid hormone to suppress thyroid-
stimulating hormone (TSH)
TOC: Radioactive iodine (RAI) ablation followed by hormone replacement.
******ebook converter DEMO Watermarks*******
 Surgical excision is rarely needed

Ectopic Thyroid:

Normal thyroid tissue may be found any where in the central neck compartment, including the esophagus,
trachea, and anterior mediastinum,aortic arch, in the aortopulmonary window, within the upper pericardium, or
in the interventricular septum.
Thyroid tissue situated lateral to the carotid sheath and jugular vein, previously termed lateral aberrant
thyroid
LAT always represents metastatic thyroid cancer in lymph nodes, considered to be skip metastasis from
Papillary CA thyroid.

Thyroid Anatomy:

Brown in color and firm in consistency.

Located posterior to the strap muscles.
Weighs approximately 20 g.
The thyroid lobes are located adjacent to the thyroid cartilage and connected in the midline by an isthmus that
is located just inferior to the cricoid cartilage.
A pyramidal lobe is present in about 50% of patients.
The thyroid lobes extend to the midthyroid cartilage superiorly and lie adjacent to the carotid sheaths and
sternocleidomastoid muscles laterally.
The strap muscles (sternohyoid, sternothyroid, and superior belly of the omohyoid) are located
anteriorly and are innervated by the ansacervicalis (ansahypoglossi).
Enveloped by a loosely connecting fascia formed from the partition of the deep cervical fascia into anterior and
posterior divisions.
The true capsule of the thyroid is a thin, densely adherent fibrous layer that sends out septa that invaginate into
the gland, forming pseudolobules.
The thyroid capsule is condensed into the posterior suspensory or Berry’s ligament near the cricoid cartilage
and upper tracheal rings.
The superior thyroid arteries arise from the ipsilateral external carotid arteries and divide into anterior and
posterior branches at the apices of the thyroid lobes.
Inferior thyroid arteries arise from the thyrocervical trunk shortly after their origin from the subclavian arteries.
The inferior thyroid arteries travel upward in the neck posterior to the carotid sheath to enter the thyroid lobes
at their midpoint.
A thyroidea ima artery arises directly from the aorta or innominate in 1% to 4% of individuals to enter the
isthmus or replace a missing inferior thyroid artery.
The inferior thyroid artery crosses the recurrent laryngeal nerve (RLN), necessitating identification of the RLN
before the arterial branches can be ligated.
The venous drainage of the thyroid gland occurs via multiple small surface veins, which coalesce to form three
sets of veins – the superior, middle, and inferior thyroid veins.
The middle vein or veins are the least consistent.
The superior and middle veins drain directly into the internal jugular veins. The inferior veins often
form a plexus, which drains into the brachiocephalic veins.

******ebook converter DEMO Watermarks*******

Nerves:

The left RLN arises from the vagus nerve where it crosses the aortic arch, loops around the
ligamentumarteriosum, and ascends medially in the neck within the tracheoesophageal groove.
The right RLN arises from the vagus at its crossing with the right subclavian artery.
The nerve usually passes posterior to the artery before ascending in the neck, its course being more oblique
than the left RLN.
Along their course in the neck, the RLNs may branch, and pass anterior, posterior, or interdigitate with
branches of the inferior thyroid artery.
The right RLN may be nonrecurrent in 0.5% to 1% of individuals andoften is associated with a vascular
anomaly(associated with Dysphagia lusoria).
Nonrecurrent left RLNs are rare but have been reported in patients with situs inversus and a right-sided aortic
arch.
The last segments of the nerves often course below the tubercle and are closely approximated to the ligament of
Berry.
Branches of the nerve may traverse the ligament in 25% of individuals and are particularly vulnerable to injury
******ebook converter DEMO Watermarks*******
 at this junction.
The RLNs terminate by entering the larynx posterior to the cricothyroid muscle.
The RLNs innervate all the intrinsic muscles of the larynx, except the cricothyroid muscles, which are
innervated by the external laryngeal nerves. Injury to one RLN leads to paralysis of the ipsilateral vocal cord,
which comes to lie in the paramedian or the abducted position.
The paramedian position results in a normal but weak voice, whereas the abducted position leads to a hoarse
voice and an ineffective cough.
Bilateral RLN injury may lead to airway obstruction, necessitating emergency tracheostomy or loss of voice.
If both cords come to lie in an abducted position, air movement can occur, but the patient has an ineffective
cough and is at increased risk of repeated respiratory tract infections from aspiration.
The superior laryngeal nerves also arise from the vagus nerves.
The internal branch of the superior laryngeal nerve is sensory to the supraglottic larynx. Injury to this nerve is
rare in thyroid surgery, but its occurrence may result in aspiration.
The external branch of the superior laryngeal nerve lies on the inferior pharyngeal constrictor muscle and
descends alongside the superior thyroid vessels before innervating the cricothyroid muscle.
Injury to this nerve leads to inability to tense the ipsilateral vocal cord and hence difficulty “hitting high notes,”
difficulty projecting the voice, and voic fatigue during prolonged speech.
Sympathetic innervation of the thyroid gland is provided by fibers from the superior and middle cervical
sympathetic ganglia.

Hyperthyroidism:
Diffuse Toxic Goiter
(Graves’ Disease):

It is an autoimmune disease with a strong familial predisposition.

Female preponderance (5:1).
Peak incidence between the ages of 40 and 60 years.
Graves’ disease is characterized by thyrotoxicosis, diffuse goiter and extra- thyroidal conditions including
ophthalmopathy, dermopathy (pretibial myxedema), thyroid acropachy, gynecomastia, and other
manifestations.
Disease is associated with certain human leukocyte antigen (HLA) haplotypes, including HLA-B8, HLA-DR3,
and HLADQA1*0501.
HLA-DRB1*0701 is protective.
Polymorphisms of the cytotoxic T-lymphocyte antigen 4 (CTLA-4) gene also have been associated with
Graves’ disease development.Once initiated, the process causes sensitized T-helper lymphocytes to stimulate B
lymphocytes, which produce antibodies directed against the thyroid hormone receptor.
TSIs or antibodies that stimulate the TSH-R, as well as TSH-binding inhibiting immunoglobulins or antibodies,
have been described. The thyroid-stimulating antibodies stimulate the thyrocytes to grow and synthesize excess
thyroid hormone, which is a hallmark of Graves’ disease.
Macroscopically, the thyroid gland is diffusely and smoothly enlarged, with concomitant increase in
vascularity.
Microscopically, the gland is hyperplastic, and the epithelium is columnar with minimal colloid present.

Clinical Features:

******ebook converter DEMO Watermarks*******

 Hyperthyroid symptoms include heat intolerance, increased sweating and thirst, and weight loss despite
adequate caloric intake.
Symptoms of increased adrenergic stimulation include palpitations, nervousness, fatigue, emotional lability,
hyperkinesis, and tremors.
The most common GI symptoms include increased frequency of bowel movements and diarrhea.
Female patients often develop amenorrhea, decreased fertility, and an increased incidence of miscarriages.
Children experience rapid growth with early bone maturation.
Older patients may present with cardiovascular complications such as atrial fibrillation and congestive heart
failure.
Weight loss and facial flushing may be evident.
The skin is warm and moist and African American patients often note darkening of their skin.
Tachycardia or atrial fibrillation is present, with cutaneous vasodilation leading to a widening of the pulse
pressure and a rapid falloff in the transmitted pulse wave (collapsing pulse).
A fine tremor, muscle wasting, and proximal muscle groupweakness with hyperactive tendon reflexesoften are
present.
50% of patients with Graves’ disease also develop clinically evident ophthalmopathy.
Dermopathy occurs in 1% to 2% of patients.
It is characterized by deposition of glycosaminoglycans, leading to thickened skin in the pre- tibial region and
dorsum of the foot.
Eye symptoms include:
Lid lag (von Graefe’s sign).
Spasm of the upper eyelid revealing the sclera above the corneoscleral limbus (Dalrymple’s sign).
Stalwags infrequentstare, due to catecholamine excess.
True infiltrative eye disease results in periorbital edema.
Conjunctival swelling and congestion (chemosis).
Proptosis.
Limitation of upward and lateral gaze (from involvement of the inferior and medial rectus muscles,
respectively).
Keratitis, and even blindness due to optic nerve involvement.
Gynecomastia is common in young men.
Bony involvement leads to subperiosteal bone formation and swelling in the metacarpals (thyroid
acropachy).
Onycholysis, or separation of fingernails from their beds, is a commonly observed finding.
There may be an overlying bruit or thrill over the thyroid gland and a loud venous hum in the supraclavicular
space.

Diagnostic Tests:

Diagnosis of hyperthyroidism is made by a suppressed TSH with or without an elevated free T4 or T3 level.
If eye signs are present, other tests are generally not needed.
In the absence of eye findings, an 123 I uptake and scan should be performed.
An elevated uptake, with a diffusely enlarged gland, confirms the diagnosis of Graves’ disease.
Anti-Tg and anti-TPO antibodies are elevated in up to 75% of patients but are not specific.
Elevated TSH-R or thyroid-stimulating antibodies (TSAb) are diagnostic of Graves’ disease and are increased
in about 90% of patients.
MRI scans of the orbits is IOC for Graves’ ophthalmopathy.

******ebook converter DEMO Watermarks*******

Treatment:
Graves’ disease may be treated by any of three treatment modalities:
1. Antithyroid drugs.
2. Thyroid ablation with radioactive 131I.
3. Thyroidectomy.
Antithyroid Drugs:

Antithyroid medications generally are administered in preparation for RAI ablation or surgery.
The drugs commonly used are propylthiouracil andmethimazole.
The dose of antithyroid medication is titrated as needed in accordance with TSH and T4 levels
Most patients have improved symptoms in 2 weeks and become euthyroid in about 6 weeks
Treatment for curative intent is reserved for patients with
Small, nontoxic goiters less than 4 g.
Mildly elevated thyroid hormone levels.
Negative or low or titers of thyroid hormone receptor antibodies.
Rapid decrease in gland size with antithyroid medications.
******ebook converter DEMO Watermarks*******
 These drugs have the added effect of decreasing the peripheral conversion of T4 to T3.
Propranolol is the most commonly prescribed medication.

Caution should be exercised in patients with asthma.

Calcium channel blockers are useful for rate control in patients in whom blockers are contraindicated.

Radioactive Iodine Therapy (131I):

The major advantages of this treatment are the avoidance of a surgical procedure and its concomitant
risks, reduced overall treatment costs, and ease of treatment.
Antithyroid drugs are given until the patient is euthyroid and then discontinued to maximize drug uptake.
Most patients become euthyroid within 2 months.
RAI therapy is therefore most often used:
In older patients with small or moderate-sized goiters.
Those who have relapsed after medical or surgical therapy.
Total lobectomy can be performed on one side with a subtotal thyroidectomy on the other side (Hartley-
Dunhill procedure).
Based on the current evidence, recently published guidelines from the American Thyroid Association (ATA)
and the American Association of Clinical Endocrinologists (AACE) recommend: total or near-total
thyroidectomy as the procedure of choice for the surgical management of Graves’ disease.
Recurrent thyrotoxicosis is managed by radioiodine treatment.

Toxic Multinodular Goiter:

Toxic multinodular goiters usually occur in older individuals, who often have a prior history of a nontoxic
multinodular goiter.

The presentation often is insidious in that hyperthyroidism may only become apparent when patients are placed
on low doses of thyroid hormone suppression for the goiter.
Some patients have T3 toxicosis, whereas others may present only with atrial fibril- lation or congestive heart
failure.
Hyperthyroidism also can be precipitated by iodide-containing drugs such as contrast media and the
antiarrhythmic agent amiodarone (Jod-Basedow hyper- thyroidism).
Symptoms and signs of hyperthyroidism are similar to Graves’ disease, but extrathyroidal manifestations are
absent.

Diagnostic Studies:

Blood tests are similar to Graves’ disease with a suppressed TSH level and elevated free T4 or T3 levels.
RAI uptake also is increased, showing multiple nodules with increased uptake and suppression of the
remaining gland.

Treatment:

Hyperthyroidism must be adequately controlled.

Both RAI and surgical resection may be used for treatment.
When surgery is performed, near-total or total thyroidectomy is recommended to avoid recurrence
RAI therapy is reserved for elderly patients who represent very poor operative risks.
Because uptake is less than in Graves’ disease, larger doses of RAI often are needed to treat the
hyperthyroidism.

Toxic Adenoma:

Hyperthyroidism from a single hyperfunctioning nodule.

Occurs in younger patients.
Recent growth of a long-standing nodule along with the symptoms of hyperthyroidism.
******ebook converter DEMO Watermarks*******
 Toxic adenomas are characterized by somatic mutations in the TSH-R gene, although G-protein –
stimulating gene (gsp) mutations may occur also.
Physical examination usually reveals a solitary thyroid nodule without palpable thyroid tissue on the
contralateral side.
RAI scanning shows a “hot” nodule with suppression of the rest of the thyroid gland.
These nodules are rarely malignant.

Treatment:

Smaller nodules may be managed with antithyroid medications and RAI.

Surgery (lobectomy and isthmusectomy) is preferred to treat young patients and those with larger nodules.
Percutaneous ethanol injection (PEI) has been reported to have reasonable success rates but has not been
directly compared with surgery.

Thyroid Storm:

Thyroid storm is a condition of hyperthyroidism accompanied by fever, central nervous system agitation or
depression and cardiovascular and GI dysfunction including hepatic failure.
The condition may be precipitated by abrupt cessation of antithyroid medications, infection, thyroid or non
thyroid surgery and trauma in patients with untreated thyrotoxicosis.
Thyroid storm may result from amiodarone administration or exposure to iodinated contrast agents or following
RAI therapy.
Blockers are given to reduce peripheral T4 to T3 conversion and decrease the hyperthyroid symptoms. Oxygen
supplementation and hemodynamic support should be instituted.
Nonaspirin compounds can be used to treat pyrexia and Lugol’s iodine or sodium ipodate (intravenously)
should be administered to decrease iodine uptake and thyroid hormone secretion.

Thyroiditis:
Thyroiditis usually is classified into acute, sub acute, and chronic forms, each associated with a distinct clinical
presentation and histology.
Acute (Suppurative) Thyroiditis:

Infectious agents can seed it

via the hematogenous or lymphatic route.
via direct spread from persistent pyriform sinus fistulae or thyroglossal duct cysts.
As a result of penetrating trauma to the thyroid gland.
Due to immu-nosuppression.
Streptococcus and anaerobes account for about 70% of cases.
Acute suppurative thyroiditisis more common in children and often is preceded by an upper respiratory tract
infection or otitis media.
It is characterized by severe neck pain radiating to the jaws or ear, fever, chills, odynophagia, and dysphonia.
Complications such as systemic sepsis, tracheal or esophageal rupture, jugular vein thrombosis, laryngeal
chondritis, and perichondritis or sympathetic trunk paralysis may also occur.
Diagnosis is established by leukocytosis on blood tests and FNAB for Gram’s stain, culture, and cytology.
CT scans may help to delineate the extent of infection and identify abscesses.
A persistent pyriform sinus fistula should always be suspected in children with recurrent acute thyroiditis.
Treatment consists of parenteral antibiotics and drainage of abscesses. Thyroidectomy may be needed for
persistent abscesses or failure of open drainage.
Patients with pyriform sinus fistulae require complete resection of the sinus tract, including the area of the
thyroid where the tract terminates, to prevent recurrence.

Subacute Thyroiditis:

Subacute thyroiditis can occur in painful or painless forms.

******ebook converter DEMO Watermarks*******

 painful thyroiditis is thought to be viral in origin or result from a postviral inflammatory response.
Genetic predisposition may also play a role, as manifested by its strong association with the HLA-B35
haplotype.
Painful thyroiditis most commonly occurs in 30- to 40-year-old women and is characterized by the sudden or
gradual onset of neck pain, which may radiate toward the mandible or ear.
The gland is enlarged, exquisitely tender, and firm.
The disorder classically progresses through four stages. An initial hyperthyroid phase, due to release of
thyroid hormone, is followed by a second, euthyroid phase. The third phase, hypothyroidism, occurs in
about 20% to 30% of patients and is followed by resolution and return to the euthyroid state in > 90% of
patients.
In the early stages of the disease, TSH is decreased, and Tg, T4, and T3 levels are elevated due to the release of
preformed thyroid hormone from destroyed follicles.
The erythrocyte sedimentation rate is typically >100 mm/h.
RAIU also is decreased (<2% at 24 hours),
Painful thyroiditis is self-limited, and therefore, treatment is primarily symptomatic.
Aspirin and other nonste- roidal anti-inflammatory drugs are used for pain relief, but ste- roids may be
indicated in more severe cases. Short-term thyroid replacement may be needed and may shorten the duration of
symptoms. Thyroidectomy is reserved for the rare patient who has a prolonged course not responsive to
medical measures or for recurrent disease.

Painless thyroiditis:

Autoimmune in origin and may occur sporadically or in the postpartum period;(the latter typically occurs
at about 6 weeks after delivery in women with high TPO antibody titers in early pregnancy).
Painless thyroiditis also is more common in women and usually occurs between 30 and 60 years of age.
Laboratory tests and RAIU are similar to those in painful thyroiditis, except for a normal erythrocyte
sedimentation rate.

Chronic Thyroiditis / Lymphocytic (Hashimoto’s) Thyroiditis:

First described by Hashimoto in 1912 as strumalymphomatosa.

It is the most common inflammatory disorder of the thyroid and the leading cause of hypothyroidism.

Etiology, Pathogenesis and Pathology:

Autoimmune process that is thought to be initiated by the activation of CD4+ T (helper) lymphocytes with
specificity for thyroid antigens. Once activated, T cells can recruit cytotoxic CD8+ T cells to the thyroid.
Hypothyroidism results not only from the destruction of thyrocytes by cytotoxic T cells but also by
autoantibodies, which lead to complement fixation and killing by natural killer cells or block the TSH-R.
Antibodies are directed against three main antigens—Tg (60%), TPO (95%), and TSH-R (60%)–and, less
commonly, the sodium/iodine symporter (25%).
Apoptosis (programmed cell death) also has been implicated in the pathogenesis of Hashimoto’s thyroiditis.
Associated with specific chromosomal abnormalities such as Turner’s syndrome and Down syndrome.
Associations with HLA-B8, DR3, and DR5 haplotypes of the major histocompatibility complex .
On gross examination, the thyroid gland is usually mildly enlarged throughout and has a pale, gray-tan cut
surface that is granular, nodular, and firm.
On microscopic examination, the gland is diffusely infiltrated by small lymphocytes and plasma cells and
occasionally shows well-developed germinal centers. The follicles are lined by Hürthle or Askanazy cells,
which are charac- terized by abundant eosinophilic, granular cytoplasm.

Clinical Presentation:

Hashimoto’s thyroiditis is also more common in women (male:female ratio 1:10 to 20) between the ages of 30
and 50 years old.
The most common presentation is that of a minimally or moderately enlarged firm granular gland awareness of

******ebook converter DEMO Watermarks*******

 a painless anterior neck mass.
Hashimoto's Thyroiditis may be painful during the initial stage of lymphocytic infiltration.

Diagnostic Studies:

Elevated TSH and the presence of thyroid autoantibodies usually confirm the diagnosis.
FNAB with ultrasound guidance is indicated in patients who present with a solitary suspicious nodule or a
rapidly enlarging goiter.
Thyroid lymphoma is a rare but well-recognized, ominous complication of chronic autoimmune thyroiditis.

Treatment:

Thyroid hormone replacement therapy is indicated in overtly hypothyroid patients, with a goal of maintaining
normal TSH levels.
The management of patients with subclinical hypothyroidism (normal T4 and elevated TSH) is controversial. 3
The data for TSH levels of 5 to 10 mIU/L were less convincing. An evaluation of the 12 randomized controlled
trials in this area only showed a trend toward improvement of some lipid parameters, and none of the included
trials evaluated overall mortality or cardiac mor- bidity.
Surgery may occasionally be indicated for suspicion of malignancy or for goiters causing compressive
symptoms or cosmetic deformity.

Riedel’s Thyroiditis:

Riedel’s thyroiditis is a rare variant of thyroiditis also known as Riedel’s struma or invasive fibrous
thyroiditis that is characterized by the replacement of all or part of the thyroid parenchyma by fibrous tissue,
which also invades into adjacent tissues.
It has been reported to occur in patients with other autoimmune diseases. This association, coupled with the
presence of lymphoid infiltration and response to steroid therapy, suggests a primary autoimmune etiology.
The disease occurs predominantly in women between the ages of 30 and 60 years old.
It typically presents as a painless, hard anterior neck mass, which progresses over weeks to years to produce
symptoms of compression, including dysphagia, dyspnea, choking and hoarseness.
Patients may present with symptoms of hypothyroidism and hypoparathyroidism as the gland is replaced by
fibrous tissue. Physical examination reveals a hard, “woody” thyroid gland with fixation to surrounding
tissues.
The diagnosis needs to be confirmed by open thyroid biopsy, because the firm and fibrous nature of the
gland renders FNAB inadequate.
Surgery is the mainstay of the treatment. The chief goal of operation is to decompress the trachea by wedge
excision of the thyroid isthmus and to make a tissue diagnosis.
Some patients who remain symptomatic have been reported to experience dramatic improvement after
treatment with corticosteroids and tamoxifen. More recently, mycophenolate mofetil has been used to attenuate
the inflammatory process and led to dramatic symptom improvements in some patients.

Solitary thyroid nodule - Diagnostic Investigations:

Serum TSH, T3, T4 (indicates the thyroid status).
USG:

******ebook converter DEMO Watermarks*******

From Frates MC, Benson CB, Charboneau JW, et al: Management of thyroid nodules detected at US: Society of
Radiologists in Ultrasound consensus conference statement. Radiology 237:794-900, 2005. NPV, negative
predictive value; PPV, positive predictive value.
Fine-Needle Aspiration Biopsy:

FNAB has become the single most important test in the evaluation of thyroid masses and can be performed
with or without ultrasound guidance.
Ultrasound guidance is recommended for nodules that are difficult to palpate, for cystic or solid-cystic nodules
that recur after the initial aspiration and for multinodular goiters.
A 23-gauge needle is used.
Accordingly, optimum cytology specimens should have at least six follicles each containing at least 10 to
15 cells from at least two aspirates.

Management:

Thyroid Carcinoma:

******ebook converter DEMO Watermarks*******

FAP = familial adenomatous polyposis; FTC - follicular thyroid cancer PTC = papillary thyroid cancer.

Papillary Carcinoma Thyroid:

Papillary carcinoma accounts for 80% of all thyroid malignancies in iodine-sufficient areas and is the
predominant thyroid cancer in children.
Strongly associated with exposure to external radiation.
Papillary carcinoma occurs more often in women, with a 2:1 female-to-male ratio and the mean age at
presentation is 30 to 40 years.
Most patients are euthyroid and present with a slow-growing painless mass in the neck. Dysphagia, dyspnea
and dysphonia usually are associated with locally advanced invasive disease.
******ebook converter DEMO Watermarks*******
 Lymph node metastases are common, especially in children and young adults, and may be the presenting
complaint.
“Lateral aberrant thyroid” almost always denotes a cervical lymph node that has been invaded by metastatic
cancer.
Diagnosis is established by FNAB of the thyroid mass or lymph node. Once thyroid cancer is diagnosed on
FNAB, a complete neck ultrasound is strongly recommended to evaluate the contralateral lobe and for lymph
node metastases in the central and lateral neck compartments.
Distant metastases are uncommon at initial presentation, but may ultimately develop in up to 20% of patients.
The most common sites are lungs, followed by bone, liver, and brain.

Pathology:

PTCs generally are hard and whitish and remain flat on sectioning with a blade.
Macroscopic calcification, necrosis, or cystic change may be apparent.
The diagnosis is established by characteristic nuclear cellular features. Cells are cuboidal with pale,
abundant cytoplasm, crowded nuclei that may demonstrate “grooving,” and intranuclear cytoplasmic
inclusions (leading to the designation of Orphan Annie nucleiwhich allow diagnosis by FNAB.
Psammoma bodies, which are microscopic, calcified deposits representing clumps of sloughed cells, also may
be present.
Multifocality is common in papillary carcinoma and may be present in up to 85% of cases on microscopic
examination.
Multifocality is associated with an increased risk of cervical nodal metastases, and these tumors may rarely
invade adjacent structures such as the trachea, esophagus, and RLNs.

Minimal or occult/microcarcinoma:

Refers to tumors of 1 cm or less in size with no evidence of local invasiveness through the thyroid capsule or
angioinvasion, and that are not associated with lymph node metastases.

Prognostic Indicators:

PTC have an excellent prognosis with a >95% 10-year survival rate.

AGES scoring system, which incorporates Age, histologic Grade, Extrathyroidal invasion and metastases and
tumor Size to predict the risk of dying from papillary cancer.
MACIS. This scale incorporates distant metastases, Age at presentation (< 40 or > 40 years old), completeness
of original surgical resection, extrathyroidalInvasion, and size of original lesion (in centimeters).
AMES using age (men <40 years old, women < 50 years old), metastases, extrathyroidal spread, and size of
tumors (< or >5 cm).
TNM system (Tumor, Nodal status, Metastases).

Surgical Treatment:

Most authors agree that patients with Primary cancer > 1 cm ;high- risk tumors (judged by any of the
classification systems discussed earlier in Prognostic Indicators) or bilateral tumors should undergo total or
near-total thyroidectomy unless there are contraindications to the surgery.
Proponents of total thyroidectomy indicate that it enables the use of RAI to effectively detect and treat
residual thyroid tissue or metastatic disease and makes serum Tg level a more sensitive marker of
recurrent or persistent disease.
Patients with a nodule that is suspicious for papillary cancer should be treated by thyroid lobectomy,
isthmusectomy, and removal of any pyramidal lobe or adjacent lymph nodes.
If intraoperative frozen-section examination of a lymph node or primary tumor confirms carcinoma,
completion total or near-total thyroidectomy is performed.
Thyroid lobectomy alone is considered sufficient treatment for small (<1 cm), incidentally discovered,
low-risk, unifocal, intrathyroidal papillary carcinomas in the absence of prior head and neck irradiation
or radiologically or clinically involved cervical nodal metastases.

******ebook converter DEMO Watermarks*******

 During thyroidectomy, enlarged or obviously involved central neck nodes should be removed
(therapeutic central- compartment, level VI), along with nodes with known lateral neck metastases.
The 2009 ATA guidelines for thyroid cancer management suggest that prophylactic (ipsilateral or
bilateral) dissection may be performed in patients with advanced (T3 or T4) papillary thyroid
carcinoma, whereas the procedure is not needed for small (T1 and T2) tumors that are clinically node
negative.
Biopsy-proven lymph node metastases detected clinically or by imaging in the lateral neck in patients with
papillary carcinoma are managed with modified radical or functional neck dissection.
Prophylactic lateral neck node dissection is not necessary in patients with PTC, because these cancers do
not appear to metastasize systemically from lymph nodes, and micrometastases often can be ablated with
RAI therapy.

Follicular Carcinoma:

Follicular carcinomas account for 10% of thyroid cancers and occur more commonly in iodine-deficient areas.
Women have a higher incidence of follicular cancer, with a female-to-male ratio of 3:1, and a mean age at
presentation of 50 years old.
Follicular cancers usually present as solitary thyroid nodules, occasionally with a history of rapid size
increase, and long-standing goiter. Pain is uncommon, unless hemorrhage into the nodule has occurred
(very important for exams).
Cervical lymphadenopathy is uncommon.
Prefer hematogenous spread.
FNAB is unable to distinguish benign follicular lesions from follicular carcinomas. Therefore,
preoperative clinical diagnosis of cancer is difficult unless distant metastases are present.
Large follicular tumors (>4 cm) in older men are more likely to be malignant.

Pathology:

Follicular carcinomas usually are solitary lesions, and the majority are surrounded by a capsule.
Histologically, follicles are present, but the lumen may be devoid of colloid.
Malignancy is defined by the presence of capsular and vascular invasion.

Surgical Treatment and Prognosis:

Patients diagnosed by FNAB as having a follicular lesion should undergo thyroid lobectomy because at least
80% of these patients will have benign adenomas.
Total thyroidectomy is recommended by some surgeons in:
Older patients with follicular lesions >4 cm because of the higher risk of cancer in this setting (50%).
In patients with atypia on FNA.
A family historyof thyroid cancer, or
A history of radiation exposure.
Prophylactic nodal dissection is not needed because nodal involvement is infrequent.
Poor long-term prognosis is predicted by:
Age over 50 years old at presentation.
Tumor size > 4 cm, higher tumor grade.
Marked vascular invasion.
Extrathyroidal invasion.
Distant metastases at the time of diagnosis.

Hurthle Cell Carcinoma:

Account for approximately 3% of all thyroid malignancies and are considered to be a subtype of follicular
thyroid cancer.
Hurthle cell cancers also are characterized by vascular or capsular invasion and therefore, cannot be
diagnosed by FNAB.

******ebook converter DEMO Watermarks*******

 Tumors contain sheets of eosinophilic cells packed with mitochondria, which are derived from the oxyphilic
cells of the thyroid gland.
Hurthle cell tumors differ from follicular carcinomas in that they are more often multifocal and bilateral
(about 30%).
Usually do not take up RAI (about 5%).
Are more likely to metastasize to local nodes (25%) and distant sites.
Are associated with a higher mortality rate (about 20% at 10 years).

Management:

Lobectomy and isthmusectomy being sufficient surgical treatment for unilateral Hürthle cell adenomas.
Total thyroidectomy should be performed for Hurthle cell neoplasms.
These patients should also undergo routine central neck node removal, similar to patients with MTC,
and modified radical neck dissection when lateral neck nodes are palpable or identified by
ultrasonography.

Postoperative Management of Differentiated Thyroid Cancer (includes Papillary Ca thyroid

and Follicular Ca thyroid):
Radioiodine Therapy:
Screening with RAI is more sensitive than chest x-ray or CT scanning for detecting metastases; however, it is
less sensitive than Tg measurements for detecting metastatic disease in most differentiated thyroid cancers
except Hürthle cell tumors.

Metastatic differentiated thyroid carcinoma can be detected and treated by 131I in about 75% of patients.
Current ATA guidelines strongly recommend RAI
After total thyroidectomy only for patients with known distant metastases.
Patients with gross extrathyroidal extension of tumor (regardless of size) or
Tumors > 4 cm, even in the absence of other high-risk features.
Selected patients with 1- to 4-cm tumors with lymph node metastases or other high-risk features.

In contrast, RAI is not indicated for patients with unifocal cancers < 1 cm in diameter or patients with
multifocal tumors (all < 1 cm) without additional high-risk features. This latter indication is a major change
from the 2006 guidelines wherein RAI was recommended for all multifocal tumors.

Remnant ablation can be performed with either thyroid hormone withdrawal or recombinant TSH (rTSH)
stimulation
A “hot” spot in the neck after initial screening usually represents residual normal tissue in the thyroid bed.
The maximum dose of radioiodine that can be administered at one time without performing dosimetry is
approximately 200 mCi with a cumulative dose of 1000 to 1500 mCi.

******ebook converter DEMO Watermarks*******

External-Beam Radiotherapy and Chemotherapy:
External- beam radiotherapy is occasionally required to control unresectable, locally invasive or recurrent
disease and to treat metastases in support bones to decrease the risk of fractures.
It also is of value for the treatment and control of pain from bony metastases when there is minimal or no RAIU.
There is no role for routine chemotherapy.
Novel Therapies:
These therapies are directed at the pathways known to be involved in various thyroid cancers.Oncogenic kinase
inhibitors that inhibit the mutant V600E BRAF kinase.

(PLX4032) selectively or both the wild-type and mutant kinase (XL281) have shown promise in the treatment
of patients with metastatic papillary cancers.

Thyroid Hormone:

T4 is necessary as replacement therapy in patients after total or near-total thyroidectomy, and also has the
additional effect of suppressing TSH and reducing the growth stimulus for any possible residual thyroid cancer
cells.
TSH suppression reduces tumor recurrence rates.
Current guidelines advise maintaining TSH levels:
< 0.1 mU/ mL in patients with persistent disease.
in the low normal range (0.3 to 2 mU/mL) in patients who are clinically and biochemically free of disease
and at low risk for recurrence.
Between 0.1 to 0.5 mU/mL for high risk groups.

Follow-Up of Patients with Differentiated Thyroid Cancer

Thyroglobulin Measurement:

******ebook converter DEMO Watermarks*******

 Tg and anti-Tg antibody levels should be measured initially at 6-month intervals.
In low-risk patients, who have low suppressed Tg levels in the first year, serum Tg should be measured after T4
withdrawal or recombinant TSH stimulation approximately 12 months after ablation.
A single rTSH-stimulated Tg level of <0.5 ng/mL (with absent antibodies) has a 98% to 99.5% probability of
identifying patients completely free of disease on follow-up. A Tg level of >2 ng/mL following rTSH
stimulation is highly sensitive in identifying patients with recurrent tumor.

Imaging:

low-risk patients with negative TSH-stimulated Tg and cervical ultrasound do not require routine diagnostic
whole-body radioiodine scans.
Cervical ultrasound be performed to evaluate the thyroid bed and central and lateral cervical nodal
compartments at 6 and 12 months after thyroidectomy and then annually for at least 3 to 5 years, depending on
the patient’s risk for recurrent disease and Tg status.

Sonographically suspicious nodes >5 to 8 mm on the smallest diameter measurement should be biopsied for
cytology as well as Tg measurement in the aspirate washout.

FDG PT AND CT may be used as a prognostic tool in patients with metastatic disease and to evaluate the
response to treatment in patients with metastatic or locally advanced disease.

Medullary Carcinoma (very important for exams):

accounts for about 5% of thyroid malignancies.

arises from the parafollicular or C cells of the thyroid.
The female-to-male ratio is 1.5:1. Most patients present between 50 and 60 years old, although patients with
familial disease present at a younger age.
Most MTCs occur sporadically.
However, approximately 25% occur within the spectrum of several inherited syndromes such as familial MTC,
MEN2A, and MEN2B. All these variants are known to result secondary to germline mutations in the RET
proto-oncogene.
Patients with MTC often present with a neck mass that may be associated with palpable cervical
lymphadenopathy (15% to 20%).
Pain or aching is more common in patients with these tumors, and local invasion may produce symptoms of
dysphagia, dyspnea, or dysphonia.
Distant blood-borne metastases to the liver, bone (frequently osteoblastic) and lung occur later in the
disease.
Medullary thyroid tumors secrete not only calcitonin and carcinoembryonic antigen (CEA), but also other
peptides such as calcitonin gene–related peptide, histaminadases, prostaglandins E2 and F2, and
serotonin.
Patients with extensive metastatic disease frequently develop diarrhea, which may result from increased
intestinal motility and impaired intestinal water and electrolyte flux.
About 2% to 4% of patients develop Cushing’s syndrome as a result of ectopic production of
adrenocorticotropic hormone (ACTH).

Pathology:

MTCs typically are unilateral (80%) in patients with sporadic disease and multicentric in familial cases,
with bilateral tumors occurring in up to 90% of familial patients.
Familial cases also are associated with C-cell hyperplasia, which is considered a premalignant lesion.
Microscopically, tumors are composed of sheets of infiltrating neoplastic cells separated by collagen and
amyloid The presence of amyloid is a diagnostic finding, but immunohistochemistry for calcitonin is
more commonly used as a diagnostic tumor marker.
These tumors also stain positively for CEA and calcitonin gene–related peptide.

Diagnosis:
******ebook converter DEMO Watermarks*******
The diagnosis of MTC is established by history, physical examination, raised serum calcitonin, or CEA levels,
and FNAB cytology of the thyroid mass.
(Attention to family history is important because about 25% of patients with MTC have familial disease)

all new patients with MTC should be screened for RET point mutations, pheochromocytoma, and HPT.
Screening of patients with familial MTC for RET point mutations has largely replaced using provocative
testing with pentagastrin or calcium-stimulated calcitonin levels to make the diagnosis.
Calcitonin and CEA are used to identify patients with persistent or recurrent MTC.
Calcitonin is a more sensitive tumor marker, but CEA is a better predictor of prognosis.

Treatment:
The ATA published guidelines for the management of medullary cancers in 2009.

A neck ultrasound is recommended to evaluate the central and lateral neck compartments and the superior
mediastinum.
Serum calcitonin, CEA, and calcium levels should also be measured.
RET proto-oncogene mutation testing should be performed.
Pheochromocytomas need to be excluded. If patients are found to have a pheochromocytoma, this must be
operated on first. These tumors are gen- erally (>50%) bilateral.
Total thyroidectomy is the treatment of choice for patients with MTC because of the high incidence of
multicentricity, the more aggressive course, and the fact that (131I) therapy usually is not effective.
Central compartment nodes frequently are involved early in the disease process, so that a bilateral
prophylactic central neck node dissection should be routinely performed.
Lateral neck dissection is to be done if central neck lymph nodes are involved or if the primary tumor is
≥1.5 cm.
In the case of locally recurrent or widely metastatic disease, tumor debulking is advised not only to
ameliorate symptoms of pain, flushing, and diarrhea, but also to decrease risk of death from recurrent
central neck or mediastinal disease.
There is no effective chemotherapy regimen.
Various targeted therapies directed against the RET kinase have been investigated for the treatment of
MTC.Many of these also inhibit vascular endothelial growth factor receptor (VEGFR), due to their close
structural similarities. Sorafenib, sunitinib, lenvatinib (E7080) and cabozantinib (XL-184) are some such
multikinase inhibitors.
Vandetanib is used for the treatment of advanced and progressive MTC.

Postoperative Follow-Up and Prognosis:

Patients are followed by annual measurements of calcitonin and CEA levels

Prognosis is related to disease stage.
Prognosis is the worst (survival of 35% at 10 years) in patients with MEN2B.

Anaplastic Carcinoma:

Anaplastic carcinoma accounts for approximately 1% of all thyroid malignancies.

This tumor is one of the most aggressive thyroid malignancies, with few patients surviving 6 months beyond
diagnosis.
Women are more commonly affected, and the majority of tumors present in the seventh and eighth decade of
life.
The typical patient has a long-standing neck mass, which rapidly enlarges and may be painful.
Associated symptoms such as dysphonia, dysphagia, and dyspnea are common.
The tumor is large and may be fixed to surrounding structures or may be ulcer- ated with areas of necrosis.
Lymph nodes usually are palpable at presentation. Evidence of metastatic spread also may be present.
Diagnosis is confirmed by FNAB revealing characteristic giant and multinucleated cells.
Core or incisional biopsy occasionally is needed to confirm the diagnosis, especially when there is
necrotic material on the FNA.
******ebook converter DEMO Watermarks*******
Pathology:

Microscopically, sheets of cells with marked heterogeneity are seen.

The three main histologic growth patterns are spindle cell, squamoid, and pleomorphic giant cell.

Treatment and Prognosis:

All forms of treatment have been disappointing.

A total or near-total thyroidectomy with therapeutic lymph node dissection is recommended for patients with an
intrathyroidal mass.
If extrathyroidal extension is present, an en bloc resection should be considered if all gross disease can be
removed (R1).
Adjuvant radiation with should be offered to patients with a good performance status and no metastatic disease
who desire aggressive management.
Cytotoxic chemotherapy (with some combination of a taxane, anthracycline, and platinum) is typically given
concurrently although these agents are also being used in a neoadjuvant fashion particularly in patients with
unresectable disease.

Lymphoma:

Lymphomas account for <1% of thyroid malignancies.

Most are of the non-Hodgkin’s B-cell type.
Most thyroid lymphomas develop in patients with chronic lymphocytic thyroiditis.
Rapidly enlarging neck mass often is painless.
Patients may present with acute respiratory distress.
Ultrasound: lymphoma appears as a well-defined hypoechoic mass.
The diagnosis usually is suggested by FNAB, but FNAB can be nondiagnostic, particularly in the setting of
low-grade lympho- mas. Therefore, needle core or open biopsy may be necessary for definitivediagnosis.

Treatment:

Patients with thyroid lymphoma respond rapidly to chemotherapy (CHOP—cyclophosphamide, doxorubicin,

vincristine, and prednisone).
Combined treatment with radiotherapy and chemotherapy often is recommended.
Thyroidectomy and nodal resection are used to alleviate symptoms of airway obstruction in patients who do not
respond quickly to the above regimens or who have completed the regimen before diagnosis.

Complications of Thyroidectomy:
Hemorrhage:

A tension haematoma deep to the cervical fascia is usually due to reactionary haemorrhage from one of the
thyroid arteries; occasionally, haemorrhage from a thyroid remnant or a thyroid vein may be responsible.
This is a rare but desperate emergency which requires urgent decompression by opening the layers of the
wound, not simply the skin closure, to relieve tension before urgent transfer to theatre to secure the bleeding
vessel.
A subcutaneous haematoma or collection of serum under the skin flaps require evacuation in the
following 48 hours. This should not be confused with the potentially life- threatening deep tension haematoma.

Respiratory obstruction:

Most cases are due to laryngeal oedema.

This is very rarely due to collapse or kinking of the trachea (tracheomalacia).
The most important cause of laryngeal oedema is a tension haematoma. However, trauma to the larynx by
anaesthetic intubation and surgical manipulation are important contributory factors, particularly if the goitre is
******ebook converter DEMO Watermarks*******
 very vascular and may cause laryngeal oedema without a tension haematoma.
If releasing a tension haematoma does not immediately relieve airway obstruction, the trachea should be
intubated at once. An endotracheal tube can be left in place for several days; steroids are given to reduce
oedema and a tracheostomy is rarely necessary.

Recurrent laryngeal nerve paralysis and voice change:

Recurrent laryngeal nerve injury may be unilateral or bilateral, transient or permanent.

Patients, particularly those who use their voice professionally, must be advised that any thyroid operation will
result in change to the voice even in the absence of nerve trauma. Fortunately, for most patients, the changes
are subtle and only demonstrable on formal voice assessment.

Thyroid insufficiency:

Following subtotal thyroidectomy this usually occurs within two years. With longer follow up it is clear that the
majority of patients will eventually develop thyroid failure.

Parathyroid insufficiency:

This is due to removal of the parathyroid glands or infarction through damage to the parathyroid end artery;
often, both factors occur together.
Vascular injury is probably far more important than inadvertent removal.
Most cases present dramatically 2–5 days after operation but, very rarely, the onset is delayed for 2–3 weeks or
if a patient with marked hypocalcaemia is asymptomatic.

Thyrotoxic crisis (storm):

This is an acute exacerbation of hyperthyroidism. It occurs if a thyrotoxic patient has been inadequately
prepared for thyroidectomy and is now extremely rare.

Wound infection.
Hypertrophic or keloid scar.
Stitch granuloma.
Postoperative care:

About 25 per cent of patients develop transient hypocalcaemia and oral calcium may be necessary (1 g three to
four times daily).
If associated symptoms are severe, and serum calcium less than 1.90 mmol/L, 10 mL intravenous calcium
gluconate 10 per cent (10 mL equivalent to 8.9 mg or 2.3 mmol calcium) should be given and alfacalcidol (1–2
μg oral daily) may be required to maintain normocalcaemia.
To screen for parathyroid insufficiency, the serum calcium should be measured at the first review
attendance 4–6 weeks after operation.

Parathyroid:

Primary hyperparathyroidism is commonly a sporadic rather than familial condition associated with
hypercalcaemia and inappropriately raised serum PTH levels due to enlargement of one or more glands and
hypersecretion of PTH.
The normal response to hypercalcaemia is PTH suppression.

Epidemiology:

Sporadic primary hyperparathyroidism increases with age.

affects women more than men.
******ebook converter DEMO Watermarks*******
 Familial hyperparathyroidism occurs as part of the following genetically determined conditions:
MEN1 (Multiple endocrine neoplasia type 1: Werner’s syndrome).
MEN2A (Sipple syndrome), rarely in MEN2B.
Familial hyperparathyroidism.

Pathology:
The majority (85 per cent) of patients with sporadic primary hyperparathyroidism have a single adenoma,
approximately 13 per cent have hyperplasia affecting all four glands and about 1 per cent will have more
than one adenoma or a carcinoma.

ADENOMA: Single enlarged gland with three small normal glands is characteristic of a single adenoma
regardless of the histology which may show considerable overlap between a hyperplastic and adenomatous
gland.
Multiple adenomas occur more frequently in older patients.
Parathyroid hyperplasia: by definition affects all four glands.
Parathyroid carcinomas: Are large tumours and typically more adherent or invasive than large adenomas.
Histology demonstrates a florid desmoplastic reaction with dense fibrosis and capsular and vascular
invasion.

Clinical Manifestations:

Patients formerly presented with the “classic” pentad of symptoms (i.e., kidney stones, painful bones,
abdominal groans, psychic moans, and fatigue over- tones).
Currently, most patients present with weakness, fatigue, polydipsia, polyuria, nocturia, bone and joint pain,
constipation, decreased appetite, nausea, heartburn, pruritus, depression, and memory loss.

Renal Disease:

Approximately 80% of patients with PHPT have some degree of renal dysfunction or symptoms.
Kidney stones were previously reported in up to 80% of patients but now occur in about 20% to 25%.
Nephrocalcinosis, polyuria, polydipsia, and nocturia; hypertension has been reported to occur in patients with
PHPT.

Bone Disease:

Bone disease, including osteopenia, osteoporosis and osteitis fibrosa cystica, is found in about 15% of patients
with PHPT.
The skull also may be affected and appears mottled with a loss of definition of the inner and outer cortices.
Brown or osteoclastic tumors and bone cysts also may be present.

Gastrointestinal Complications:

PHPT has been associated with peptic ulcer disease.

An increased incidence of pancreatitis; cholelithiasis also has been reported in patients with PHPT.

Neuropsychiatric Complications:

Severe hypercalcemia may lead to various neuropsychiatric manifestations such as florid psychosis,
obtundation, or coma.
Other findings such as depression, anxiety, and fatigue are more commonly observed in patients with only mild
hypercalcemia.

Other Features:
PHPT also can lead to fatigue and muscle weakness, which is prominent in the proximal muscle groups.

******ebook converter DEMO Watermarks*******

Physical Findings:

A palpable neck mass in a patient with PHPT is more likely to be thyroid in origin or a parathyroid cancer.
Patients also may demonstrate evidence of band keratopathy, a deposition of calcium in Bowman’s membrane
just inside the iris of the eye.

Differential Diagnosis:

Diagnostic Investigations - Biochemical Studies:

The presence of an elevated serum calcium and intact PTH or two-site PTH levels, without hypocalciuria,
establishes the diagnosis of PHPT with virtual certainty.

Management:

Partial parathyroidectomy; also known as three and half gland parathyroidectomy.

******ebook converter DEMO Watermarks*******
 Total parathyroidectomy with auto implantation into brachioradialis of non dominant forearm or
sternomastoid.

Important: USG and SESTAMIBI scan are useful tests for preoperative localization of the parathyroid.
Secondary Hyperparathyroidism:
Elevated PTH levels may also occur as a compensatory response to hypocalcemic states resulting from chronic renal
failure or GI malabsorption of calcium.
Secondary hyperparathyroidism also occurs in vitamin D-deficient rickets, malabsorption and
pseudohypoparathyroidism
Tertiary Hyperparathyroidism:
Autonomous hyperfunction of the parathyroids even after the correction of the causes for hypocalcemia is termed
tertiary hyperparathyroidism.

******ebook converter DEMO Watermarks*******

Anatomy:

The Kidneys are paired, reddish brown, solid organ, measuring 10-12 cm.
In vertical dimension, 5-7 cm. in transverse width and approximately 3cm. in AP thickness.
In males the normal kidney weighs approx. 150 gm, while in females it is 135 gm.

The kidneys receive about 20% of total cardiac output.

At birth Kidneys are irregular in contour with multiple “fetal lobations”, which disappear in first year of life.
Some times a focal bulge persists in the mid-lateral contour of the kidney on either side referred to as
“DROMEDARY HUMP”.
This occurs much more frequently on the left side.
The renal parenchyma is divided into Cortex and medulla.
The medulla is not contiguous but consists of multiple conical segments,
The RENAL PYRAMIDS, which points centrally into the renal sinus, where it is cupped by an individual
minor calyx, thus the number of pyramids corresponds with the number of minor calyx.
The renal cortex covers the pyramids not only peripherally but also extends between the pyramids to renal sinus
forming “renal columns of BERTIN”. Through these columns renal vessel enter and leave the kidney.

The kidney is divided into cortex and medulla. The medullary areas are pyramidal, more centrally located, and
separated by sections of cortex. These segments of cortex are called the columns of Bertin.
Orientation of the kidney is greatly affected by the structures around it. Thus the upper poles are situated more
medially and posteriorly than the lower poles. Also, the medial aspect of the kidney is more anterior than the
lateral aspect.
Gerota fascia envelops the kidney on all aspects except interiorly where it is not closed but instead remains an
******ebook converter DEMO Watermarks*******
 open potential space.
From anterior to posterior, the renal hilar structures are the renal vein, renal artery, and collecting system.
The renal artery splits into segmental branches. Typically, the first branch is the posterior segmental artery which
passes posterior to the collecting system. There are generally three to four anterior segmental branches that pass
anteriorly to supply the anterior kidney.
The progression of arterial supply to the kidney is as follows: renal artery → segmental artery → interlobar artery
→ arcuate artery → interlobular artery → afferent artery.
The venous system anastomoses freely throughout the kidney. The arterial supply does not. Thus occlusion of a
segmental artery leads to parenchymal infarction, but occlusion of a segmental vein is not problematic because
there are many alternate drainage routes.
Anatomic variations in the renal vasculature are common, occurring in 25% to 40% of kidneys.
Each renal pyramid terminates centrally in a papilla. Each papilla is cupped by a minor calyx. A group of minor
calyces join to form a major calyx. The major calyces combine to form the renal pelvis. There is great variation in
the number of calyces, calyceal size, and renal pelvis size. The only way to determine pathologic from normal is
by evidence of dysfunction.

Renal Vasculature:

Each kidney is supplied by an artery (a branch of aorta) and drains into a renal vein (to IVC). The renal vein
lies most anterior and pelvis is most posterior, renal artery lies in between (VAP).
The right renal artery passes behind the IVC, while the left renal vein is anterior to the aorta. Sometimes renal
vein divides and sends one limb anterior and one limb posterior to the aorta forming “RENAL COLLAR”.
The main renal artery divides into four or more segmental arteries.
The first and most consistent division is posterior branch.
The remaining anterior division branches into apical, upper, middle and lower anterior segmental arteries.
******ebook converter DEMO Watermarks*******
 Each segmental artery is end artery.

Renal lymphatics:

There are often 2 or more lymph nodes at the renal hilum, (first site of metastatis).
From the left kidney, lymphatic trunk then drains into para-aortic nodes.
From the right kidney, lymphatics drain into inter-aortocaval and para-caval nodes.
Some lymphatics may cross over from right to left and drain primarily into para-aortic nodes.

******ebook converter DEMO Watermarks*******

Intrarenal Arterial Anatomy:
Renal Innervation:

Sympathetic preganglionic nerves originate from the T8 to L1 and then travel to the celiac and aorticorenal
ganglia.
From here, postganglionic fibers travel to the kidney via the autonomic plexus surrounding the renal artery.
Parasympathetic fibers originate from the vagus nerve and travel with the sympathetic fibers to the autonomic
plexus along the renal artery.
The primary function of the renal autonomic innervation is vasomotor, with the sympathetics inducing
vasoconstriction and the parasympathetics causing vasodilation.
Despite this innervation, it is important to realize that the kidney functions well even without this neurologic
control, as evidenced by the successful function of transplanted kidneys.

******ebook converter DEMO Watermarks*******

Ureter:

In the adult, length of ureter is generally 24 to 30 cm.

It is lined by Transitional epithelium.
Beneath the epithelium is a layer of connecting tissue, the lamina propria.
In a collapsed state, ureteral mucosa lies in longitudinal folds. Mucosa is covered by inner longitudinal muscle
and outer circular and oblique muscles.
The adventitial layer contains extensive plexus of ureteral blood vessels and lymphatics.
The ureter receives its blood supply by multiple feeding branches along its course.
In the abdomen, feeding vessels come from renal artery, gonadal artery, aorta and common iliac artery. In the
pelvic cavity, additional branches come from internal iliac artery or its branches, mainly the vesical and uterine
and also from middle rectal and vaginal.
The right ureter is related to the terminal ileum, cecum, appendix and ascending colon with their mesentery.
The left ureter is related to the descending colon and sigmoid colon along with their mesentery.
Within the female pelvis, ureters are closely related to the uterine cervix and are crossed anteriorly by the
uterine arteries.

Ureteral Innervation:

Normal ureteral peristalsis does not require outside autonomic input but it originates and is propagated from
intrinsic smooth muscle pacemaker sites located in the minor calyces of the renal collecting system.
The ureter receives preganglionic sympathetic input from the T10 to L2 spinal segments.
Postganglionic fibers arise from several ganglia in the aorticorenal, superior, and inferior hypogastric
autonomic plexuses.
Parasympathetic input is received from the 2nd through 4th sacral spinal segments.

Pain Perception and Somatic Referral:

Renal pain fibers are stimulated by tension (distention) in the renal capsule, renal collecting system or ureter.
Direct mucosal irritation in the upper urinary tract may also stimulate nociceptors.
******ebook converter DEMO Watermarks*******
 Signals travel with the sympathetic nerves and result in a visceral-type pain referred to the sympathetic
distribution of the kidney and ureter (eighth thoracic through second lumbar).
Pain and reflex muscle spasm are typically produced over the distributions of the subcostal, iliohypogastric,
ilioinguinal, and/or genitofemoral nerves, resulting in flank, groin, or scrotal (or labial) pain and hyperalgesia,
depending on the location of the noxious visceral stimulus.

Adrenals:

The adrenals are embriologically and functionally distinct from the kidneys, thus in cases of renal ectopia, the
adrenals are found at its normal location.
In the normal adults weighs approx. 35 gm. and measures 3-5 cm, in greatest dimension.
The right gland is pyramidal in shape while left one in crescentic and rests more medial to the upper pole. The
right adrenal thus lie more superior than the left adrenal.
Each adrenal has two separate distinct elements; Cortex and Medulla. The central medulla consists of
chromaffin cells derived from the neural crest.
It is closely related to the sympathetic nervous system.
The adrenal cortex is mesodermally derived and forms the bulk of the gland; 80-90%. Cortex consists of (GFR)
Zona glomerulosa, which produces aldosterone in response to renin angiotensin system; Zona fasciculata
and Zona reticularis, which produce glucocorticoids and sex steroids respectively.
Vascular supply:
Each adrenal is supplied by three arteries and one vein:
Superior branches from inferior phrenic.
Middle branches from the aorta.
Inferior branches from the ipsilateral renal artery.

A single large vein exits from the adrenal hilum and drains into the IVC on the right side and renal vein on the left
side.
The adrenal lymphatics drain into the para-aortic lymph nodes.
The adrenal cortex is believed to receive no innervations.
Embryology of the Genitourinary System:
The nephric system develops progressively from3 distinct entities:
Pronephros:

It is the earliest nephric stage and extends from 4th to 14thsomites and consists of 6-10 pairs of tubules.
It disappears completely by 4th week.

Mesonephros:
******ebook converter DEMO Watermarks*******
 It is the principle excretory organ during early embryonic life (4-8 weeks).
Though it gradually disintegrates, part of its duct system forms male reproductive organ.
In mesonephros, primitive glomeruli are present.
Its primary nephric duct is called mesonephric duct and it opens distally into the cloaca.

Metanephros:
It originates both from mesonephric duct and intermediate mesoderm.
It forms the main kidney, while a ureteral bud (a branch of mesonephric duct) forms ureter, pelvis and collecting
duct.

Main features of development are:

The 3 successive units of the system develop from the intermediate mesoderm.
The tubules of all levels appear as independent primordial and only secondarily unite with the duct system.
The nephric system is laid down as the duct of the pronephros and develops from the union of the ends of the
anterior pronephric tubules.
The pronephric duct serves later on as mesonephric duct and gives rise to ureter.
The embryonic ureter is an outgrowth of the nephric duct, yet the kidney tubules differentiate from the adjacent
metanephricblastema.

Anomalies of the Nephric System:

Failure to ascend leads to ectopic kidney (1 in 1000). An ectopic kidney may be on the proper side but low
(simple ectopia), or on the opposite side (crossed ectopia), with or without fusion.
Failure to rotate during ascent causes malrotated kidney.
Fusion of the paired metanephric masses leads to various anomalies; most common of which is horseshoe
kidney.
The ureteral bud from the mesonephric duct may bifurcate, causing bifid ureter. An accessory ureter may
develop from the mesonephric duct, thereby forming a duplicated ureter. Rarely such bud has a separate
metanephric mass resulting in supernumerary kidney.
Lack of development of a ureteral bud results in a solitary kidney and a hemitrigone, (Renal agenesis =
1: 1400)
In the double ureteral buds, the main ureter bud, which is first to appear, drains upper moiety and is more
caudal on the mesonephric duct, reaches the bladder first. It then moves upwards and laterally. The 2nd bud is
more caudal in bladder. The double ureter always cross (Weigert-Meyer Law).

Horseshoe Kidney:

The horseshoe kidney is the most common type of renal fusion anomaly.
It consists of 2 distinct, functioning kidneys on each side of the midline, connected at the lower poles by an
isthmus of functioning renal parenchyma or fibrous tissue.
Horseshoe kidney occurs in 1 in 400 live births. It is twice as common in males.

Pathophysiology:

The horseshoe kidney does not by itself produce symptoms.

There are higher rates of hydronephrosis stone formation, infection.
The most common associated finding in horseshoe kidney is ureteropelvic junction obstruction.
It causes the majority of problems. Obstruction is due to the high insertion of the ureter into the renal pelvis.
The crossing of the ureter over the isthmus may also contribute to obstruction.

Clinical:

******ebook converter DEMO Watermarks*******

 Nearly one-third of patients with a horseshoe kidney remain asymptomatic.
Symptoms, when present, are usually due to obstruction, stones, or infection.
In children urinary tract infection and in adults, pain is the most common presenting symptom.
Rovsing’s sign is abdominal pain, nausea, and vomiting with hyperextension of the spine.

Relevant Anatomy:

The kidneys may be lower than normal as the isthmus is tethered during renal ascent by the inferior mesenteric
artery.
The isthmus usually lies anterior to the great vessels at the level of the 4th lumbar vertebra.
The vascular supply is variable and originates from the aorta, the iliac arteries and the inferior mesenteric
artery.
The collecting system has a characteristic appearance on intravenous urogram due to an incomplete
inward rotation of the renal pelvis, which faces anterior.

Investigations:
An IVP is the best initial radiological study to determine anatomy and relative renal function. Lowermost
calyx is pointing caudally and medially (Hand joining sign or hand shake sign).
High insertion of ureter draping over midline isthmus (Flower vase sign).
CT scan or renal ultrasound is helpful to screen for the presence of stones, masses, or hydronephrosis.
Surgical therapy: Surgical treatment is based on the disease process and standard surgical indications.
e.g. Ureteropelvic junction obstruction, Kidney stones, renal Tumors, abdominal aneurysmectomy.
Prognosis:

The horseshoe kidney does not complicate pregnancy or delivery.

Presence of the horseshoe kidney alone does not affect survival.
The horseshoe kidney does have a higher propensity to become diseased. Survival is therefore dependent on the
disease process that the horseshoe kidney may harbor.

Points To Remember:
Most common type of renal fusion anomaly.
May be associated with PUJ obstruction (50%) and VUR (33%), Anorectal malformation, Uni-bicornuate
uterus or undescendend testis.
Kidney is low lying, Isthmus is at L4.
IVP shows hand joining sign or Flower vase sign.
Treatment is based on treating complications e.g. Pyeloplasty, pyelolithotomy etc. Isthmus not divided.
Preferred surgical approach is left retroperitoneal approach.

Radiology of the Urinary Tract

Radiography:
X-rays are electromagnetic waves with photon energies that fall between those of gamma rays and ultraviolet
radiation in electromagnetic spectrum.
The basic radiological studies commonly used are: Plain KUB, IVP, RGU, AGP, MCU, RGU and Angiogram.
These studies can be enhanced by digital radiographic subtraction.
1. Plain Film:
Plain film provides soft tissue shadow of the kidney and gives a rough idea of size (Shrunken kidney in renal failure
indicate medical rather than surgical cause), number and location of kidney.

******ebook converter DEMO Watermarks*******

It demonstrates the: foreign body, bones, abnormal calcifications (stone, calcified aneurysm/ hydatid cyst, calcified
ovarian cyst and calcified lymph nodes). Renal stone in lateral view overlies the spine.
2. Intravenous Pyelography (Intravenous urography):
In this procedure after aninitial plain film, films are taken at timed interval after the IV injection of iodine containing
contrast media, which is promptly excreted by the kidney.
It is commonly used for obstruction and to delineate lesions like. papillary necrosis, medullary sponge kidney,
tumours etc.
Contrast media are ionic (Iodopyracet, Acetrizoate, Diatrizoate, Iothalmate and dimer of iothalmic acid) and non-
ionic (Metrizamide, Iopamidol, Iohexol and ioxalate).
Non-ionic contrast media are safer in cases of history of allergy to iodinated compounds.
3. Retrograde Urogram:
It is helpful in cases of unsatisfactory excretory urogram; history of adverse reaction to IV contrast media or other
method of imaging is unavailable.
It may precipitate urinary tract infection.
4. Antigrade Pyelography:
It is occasionally done when urinary tact imaging is necessary but excretory or retrograde urography has failed or is
contraindicated or when there is nephrostomy tube in place and delineation of upper tract is desired.
5. Micturating Cystourethrography / Retrograde Urethrography:
Cystogram is obtained by instilling a radiographic contrast media in the bladder and obtain an x-ray film. This
outlines the bladder. Then the patient is asked to micturate and voiding films are taken and this is called MCU.
The urethra can be imaged by obtaining an x-ray while retrograde injection of the contrast in urethra.
MCU is required in lesions of the posterior urethra (PU valves), RGU is more helpful for examining the anterior
urethra.
Sonography:
Basic Principles:

Sound frequency greater than 20 KHz in called ultrasound.

The frequencies, commonly used in medical practice are between 3.5-10 MHz.

Clinical Application:

Ultrasound is commonly used for the evaluation of bladder, prostate, kidney, testis and penis.
In kidney it evaluates size, cortical thickness, echogenicity, cortico-medullary differentiation, mass lesion and
cyst.
In the bladder it helps in detecting stone, tumour bladder wall thickness and residual urine.
Prostatic size, calculi and echogenicity can be determined with the help of USG.
Stones appear as hyperechoic (white) while malignancy as hypoechoic (black) shadow.
Higher the frequency, better is the resolution and poor penetration, thus for superficial examination (e.g.
testis) higher frequency probes are used.

Computerized Axial Tomography

Basic Principle:

In CT scan a thin X-ray film is passed through the patient and absorbed in a linear array of solid-state or gas
detector.
Digital computers assemble and integrate the collected X-ray transmission data to reconstruct a cross sectional
******ebook converter DEMO Watermarks*******
 image (Tomogram).
CT works on density difference. The unit of CT number is HU (Hounsfield unit). This relative density scale of
numbers assigns a value of 0 for water, -1000 for air and +1000-2000 for bone.

Magnetic Resonance Imaging:

Basic Principles:
The nucleus of the H+ atom consists of a single proton. Any atom containing an odd number of proton and neutron
has a nuclear property to spin. Normally the axis of spin of hydrogen nuclei is randomly oriented. If the body is
placed in a strong magnetic field the hydrogen nuclei wobble like a spinning top around the line of Magnetic field.
If hydrogen nuclei are additionally stimulated by very short pulse of radio waves, they absorb the energy and invert
their orientation.
Once the short radio wave is terminated the hydrogen nuclei return at various speed to their (low energy) state,
emitting energy. This phenomenon is called nuclear magnetic resonance.
This emitting energy is collected and transformed with various computer programs into cross sectional area.
Clinical Application:

MRI in urological diseases gives more or less same information as CT scan, but MR angiography, which does
not require contrast media is useful in evaluating renal transplant vessels and renal vein, tumour, thrombus and
renal artery stenosis.
Contrast used in MRI is gadolinium. It is contraindicated in cases of metallic prosthesis.

Urolithiasis / Kidney Stones:

Epidemiology:

Approximately 10% of Caucasian men will develop a kidney stone by the age of 70. Within 1 year of a
calcium oxalate stone, 10% of men will form another calcium oxalate stone, and 50% will have formed
another stone within 10 years.
The prevalence of renal tract stone disease is determined by factors intrinsic to the individual and by extrinsic
(environmental) factors. A combination of factors often contributes to the risk of stone formation.

Intrinsic factors:
1. Age: Peak incidence of stones occurs between the ages of 20 and 50 years.
2. Sex: Males are affected 3 times as frequently as females. Testosterone may cause increased oxalate production
in the liver (predisposing to calcium oxalate stones) and women have higher urinary citrate concentrations
(citrate inhibits calcium oxalate stone formation).
3. Genetic: Kidney stones are relatively uncommon in Native Americans, Black Africans and U.S. Blacks, and more
common in Caucasians and Asians. About 25% of patients with kidney stones report a family history of stone
disease (the relative risk of stone formation remaining high after adjusting for dietary calcium intake).
4. Familial renal tubular acidosis (predisposing to calcium phosphate stones) and cystinuria (predisposing to cystine
stones) are inherited.
Extrinsic (environmental) factors:

Geographical location, climate and season:

Renal stone disease is more common in hot climates, some endogenous populations of hot climates have a low
incidence of stones (e.g., Black Africans, Aborigines), and many temperate areas have a high incidence of
stones (e.g., Northern Europe and Scandinavia). This may relate to Western lifestyle–excess food, inadequate
fluid intake, limited exercise combined with a genetic predisposition to stone formation.
Ureteric stones become more prevalent during the summer.
The highest incidence occurs a month or so after peak summertime tem- peratures, presumably because of
higher urinary concentration in the summer (encourages crystallization). Concentrated urine has a lower pH,
******ebook converter DEMO Watermarks*******
 encouraging cystine and uric acid stone formation.
Exposure to sunlight may also increase endogenous vitamin D production, leading to hypercalciuria.
Water intake: Low fluid intake (<1200 mL/day) predisposes to stone formation.
Increasing water hardness (high calcium content) may reduce risk of stone formation, by decreasing
urinary oxalate.
Diet: High animal protein intake increases risk of stone disease (high urinary oxalate, low pH, low urinary
citrate).

High salt intake causes hypercalciuria. Contrary to conventional teaching, low-calcium diets predispose to calcium
stone disease, and high–calcium intake is protective.
Occupation: Sedentary occupations predispose to stones more than manual work.
Pathogenesis Of Stone Formation (very important for exams):
Steps leading to stone formation:

Calcium and oxalate concentration < solubility product = No Stone Formation.

Metastable calcium and oxalate concentrations =NO STONE FORMATION
Calcium and oxalate concentrations > formation product = Stone Formation
In the urine of subjects who do not form stones, the concentrations of most stone components are between Ksp
and KF.
Urine is said to be saturated with, for example, calcium and oxalate, when the product of the concentrations of
calcium and oxalate exceeds the solubility product (Ksp). Below the solubility product, crystals of calcium and
oxalate will not form and the urine is undersaturated.
Above the solubility product, crystals of calcium and oxalate should form, but they do not because of the
presence of inhibitors of crystal formation.
However, above a certain concentration of calcium and oxalate, inhibitors of crystallization become ineffective,
and crystals of calcium oxalate start to form. The concentration of calcium and oxalate at which this is
reached (i.e., at which crystallization starts) is known as the formation product (Kf), and the urine is said
to be supersaturated with the substance or substances in question at concentrations above this level.
Urine is described as being metastable for calcium and oxalate at concentrations between the solubility product
of calcium and oxalate and the formation product .
The ability of urine to hold more solute in solution than can pure water is due partly to the presence of various
inhibitors of crystallization .
The earliest phase of crystal formation is known as nucleation. Crystal nuclei usually form on the surfaces of
epithelial cells or on other crystals.
Crystal nuclei form into clumps a process known as aggregation. Citrate and magnesium inhibit not only
crystallization but also aggregation.

******ebook converter DEMO Watermarks*******

Inhibitors of stone formation:
1. Citrate(most important).
2. Magnesium.
3. Tamm Horsefall Proteins.
4. Uropontin.
5. Osteopontin.
6. Polyanions like mucopolysaccharides and glycosaminoglycans.

Key Points: Physicochemistry:

Urine must be supersaturated for stones to form.
Supersaturation alone is not sufficient for crystallizationto occur in urine, owing to the presence of urinary
inhibitors.
Nephrocalcin, uropontin, and Tamm-Horsfall protein are important inhibitors of crystal nucleation, growth,
or aggregation.
Urinary calcium and oxalate are equal contributors to urinary saturation of calcium oxalate.
Common calcium stones may originate from subepithelial plaques composed of calcium apatite that serve as
an anchor on which calcium oxalate stones can grow.
The noncrystalline component of stones is matrix, which is composed of a combination of mucoproteins,
proteins, carbohydrates, and urinary inhibitors.

Composition and percentage distribution of renal stones

******ebook converter DEMO Watermarks*******

Radiodensity on X-ray:
Three broad categories of stones are described, based on their X-ray appearance.
Radio-opaque:

Opacity implies presence of substantial amounts of calcium within the stone.

Calcium phosphate stones are the most radiodense stones, almost as dense as bone. Calcium oxalate stones are
slightly less radiodense.

Relatively radiolucent:
Cystine stones are relatively radiodense because they contain sulfur . Magnesium ammonium phosphate (struvite)
stones are less radiodense than calcium-containing stones.
Completely radiolucent:
Uric acid, triamterene, xanthine, indinavir stones are in this category (cannot be seen even on CTU).
Factors predisposing to specific stone types:
Calcium oxalate (~85% of stones) : Sub types: i. Monohydrate ii. Dihydrate

Most common type.

Shape: Calcium Monohydrate: Dumbell shaped

Calcium Dihydrate: Envelope shaped.

Also known as JACK STONES or MULBERRY STONES

Are reddish brown in color as they are associated with HEMATURIA

Associated with:
Hypercalciuria:
Excretion of >7 mmol of calcium per day in men and >6 mmol/day in women.
About 50% of patients with calcium stone disease have hypercalciuria.
There are three types of hypercalciuria:

Absorptive–Increased intestinal absorption of calcium.

Renal–Renal leak of calcium.
Resorptive–Increased demineralization of bone (due to hyperparathyroidism).

Hypercalcemia:Almost all patients with hypercalcemia who form stones have primary hyperparathyroidism. Of
hyperparathyroid patients, about 1% form stones (the other 99% do not because of early detection of
hyperparathyroidism by screening serum calcium).
Hyperoxaluria:
This is due to the following:

******ebook converter DEMO Watermarks*******

 Altered membrane transport of oxalate leading to increased renal leak of oxalate.
Primary hyperoxaluria–increased hepatic oxalate production (rare).
Increased oxalate absorption in short bowel syndrome or malabsorption (enteric hyperoxaluria)–the colon is
exposed to more bile salts and this increases its permeability to oxalate.

Hypocitraturia:
This is low urinary citrate excretion. Citrate forms a soluble complex with calcium and prevents complexing of
calcium with oxalate to form calcium oxalate stones.
Hyperuricosuria:
High urinary uric acid levels lead to formation of uric acid crystals, on the surface of which calcium oxalate crystals
form.
Uric acid (~5–10% of stones) : Shape: Amorphous shards.
Colour: Orange.

Human urine is supersaturated with insoluble uric acid.

Uric acid exists in two forms in urine–uric acid and sodium urate. Sodium urate is 20 times more soluble than
uric acid.
At a urine pH of 5, <20% of uric acid is present as soluble sodium urate. At urine pH 5.5, half the uric acid is
ionized as sodium urate (soluble) and half is nonionized as free uric acid (insoluble). At a urine pH of 6.5,
>90% of uric acid is present as soluble sodium urate. Thus, uric acid is essentially insoluble in acid urine
and soluble in alkaline urine. Human urine is acidic (because the end products of metabolism are acid)
and this low pH, combined with supersaturation of urine with uric acid, predisposes to uric acid stone
formation.

Approximately 20% of patients with gout have uric acid stones. Patients with uric acid stones may have the
following:

Gout: 50% of patients with uric acid stones have gout. The chance of forming a uric acid stone if you have
gout is of the order of 1% per year from the time of the first attack of gout.
Myeloproliferative disorders: Particularly following treatment with cytotoxic drugs, cell necrosis results in
release of large quantities of nucleic acids that are converted to uric acid. A large plug of uric acid crystals may
form in the collecting system of the kidney, in the absence of ureteric colic, causing oliguria or anuria.
Idiopathic uric acid stones (no associated condition)

Phosphate stones:

Also known as TRIPLE PHOSPHATE STONES

Composition: Calcium phosphate + Calcium Oxalate.
Shape : COFFIN LID.
Colour : WHITE.
Associated with alkaline urine.
These stones occur in patients with renal tubular acidosis (RTA), a defect of renal tubular H+ secretion resulting
in impaired ability of the kidney to acidify urine. The urine is thus of high pH, and the patient has a metabolic
acidosis. The high urine pH increases supersaturation of the urine with calcium and phosphate, leading to their
precipitation as stones.
Types of renal tubular acidosis:
Type 1 or distal RTA: The distal tubule is unable to maintain a proton gradient between the blood and the
tubular fluid. Of such patients, 70% have stones. Urine pH is >5.5, the patient has a metabolic acidosis and
hypokalemia, urinary citrate is low, and hypercalciuria is present.
Type 2 or proximal RTA is due to failure of bicarbonate resorption in the proximal tubule. There is
associated increased urinary citrate excretion, which protects against stone formation.
Type 3: A variant of type 1 RTA
Type 4 is seen in diabetic nephropathy and interstitial renal disease. These patients do not make stones.

******ebook converter DEMO Watermarks*******

If urine pH is >5.5, use the ammonium chloride loading test. Urine pH that remains above 5.5 after an oral dose of
ammonium chloride = incomplete distal RTA.
Struvite:

also known as infection or triple phosphate stones (2–20% of stones)

These stones are composed of magnesium, ammonium, and phosphate. They form as a consequence of urease-
producing bacteria that produce ammonia from breakdown of urea (urease hydrolyses urea to carbon dioxide
and ammonium).
STRUVITE stones occupying atleast 20% pelvicalceal system are known as Staghorn Calculus.
Under alkaline conditions, crystals of magnesium, ammonium, and phosphate precipitate.

Cystine: (1% of all stones):

Shape : Polyhedral.
Colour : Blue (contains sulphur).
ESWL resistant.
These stones occur only in patients with cystinuria–an inherited (autosomal recessive) disorder of
transmembrane cystine transport, resulting in decreased absorption of cystine from the intestine and in the
proximal tubule of the kidney.
Cystine is very insoluble, so reduced absorption of cystine from the proximal tubule results in supersaturation
with cystine and cystine crystal formation.
Cystine is poorly soluble in acid urine

Kidney stones: Presentation and Diagnosis:

Kidney stones may present with symptoms or be found incidentally during investigation of other problems.
Presenting symptoms include pain or hematuria (microscopic or occasionally macroscopic).
Silent Calculus:
Renal failure may be the first indication of bilateral silent calculi, although secondary infection usually produces
symptoms first.
Struvite staghorn calculi classically present with recurrent UTIs. Malaise, weakness and loss of appetite can also
occur.
Less commonly, struvite stones present with infective complications (pyonephrosis, perinephric abscess, septicemia,
xanthogranulomatous pyelonephritis).
Diagnostic Tests:
Plain abdominal radiography (1st line investigation): Calculi that contain calcium are radiodense. Sulfur-containing
******ebook converter DEMO Watermarks*******
stones (cystine) are relatively radiolucent on plain radiography.

Radiodensity of stones in decreasing order is calcium phosphate > calcium oxalate > struvite (magnesium
ammonium phosphate) >>cystine.
Completely radiolucent stones (e.g., uric acid, triamterene, indinavir) are usually suspected on the basis of
patient’s history and/or urine pH (pH <6: gout; drug history: triamterene, indinavir) and the diagnosis may be
confirmed by ultrasound, Computed Tomographic Urography (CTU), or Magnetic Resonance Urography
(MRU).
Renal Ultrasound: Its sensitivity for detecting renal calculi is ~95%. A combination of plain abdominal
radiography and renal ultrasonography is a useful screening test for renal calculi.
IVP is increasingly being replaced by CTU. IVP is useful for patients with suspected indinavir stones (which
are not visible on CT).
CTU is a very accurate method of diagnosing all but indinavir stones. It allows accurate determination of stone
size and location and good definition of pelvicalyceal anatomy.
MRU cannot visualize stones but is able to demonstrate the presence of hydronephrosis.

Management of Renal Stones:

Conservative Management:
Calculi smaller than 0.5 cm pass spontaneously unless they are impacted. Surgical intervention should be avoided.
Small renal calculi may cause symptoms by obstructing a calyx or acting as a focus for secondary infection. Most
can be safely observed until they pass.
Dissolution Therapy:

Uric acid and cystine stones are potentially suitable for dissolution therapy. Calcium within either stone type
reduces the chances of successful dissolution.

Interventional Management of Renal Stones:

1. ESWL (Extra Corporeal Shock Wave Lithrotripsy).
2. PCNL.
3. Flexible Uretroscopy and Laser.
Stone Fragmentation Techniques - Extracorporeal Lithotripsy (ESWL):

The technique of focusing externally generated shock waves at a target (the stone) was first used in humans in
1980. The first commercial lithotriptor, the Dornier HM3, became available in 1983.ESWL revolutionized
kidney and ureteric stone treatment.

Three methods of shock wave generation are commercially available: electrohydraulic, electromagnetic and
piezoelectric.
Electrohydraulic:
Application of a high-voltage electrical current between 2 electrodes about 1 mm apart under water causes discharge
of a spark. Water around the tip of the electrode is vaporized by the high temperature, resulting in a rapidly
expanding gas bubble. The rapid expansion and then rapid collapse of this bubble generates a shock wave that is
focused by a metal reflector shaped as a hemi-ellipsoid.
This method was used in the original Dornier HM3 lithotriptor.
Electromagnetic:
Two electrically conducting cylindrical plates are separated by a thin membrane of insulating material. Passage of an
electrical current through the plates generates a strong magnetic field between them, the subsequent movement of
which generates a shock wave. An acoustic lens is used to focus the shock wave.
Piezoelectric:
******ebook converter DEMO Watermarks*******
A spherical dish is covered with about 3000 small ceramic elements, each of which expands rapidly when a high
voltage is applied across them. This rapid expansion generates a shock wave. X-ray, ultrasound, or combinations of
both are used to locate the stone on which the shock waves are focused.
Older machines required general or regional anaesthesia because the shock waves were powerful and caused severe
pain.
Newer lithotriptors generate less powerful shock waves, allowing ESWL with oral or parenteral analgesia in many
cases, but they are less efficient at stone fragmentation.
Efficacy of ESWL:

The likelihood of fragmentation with ESWL depends on stone size and location, anatomy of renal collecting
system, degree of obesity and stone composition.
ESWL is most effective for stones <2 cm in diameter, in favorable anatomical locations.
It is less effective for stones >2 cm diameter, in lower-pole stones in a calyceal diverticulum (poor
drainage) and those composed of cystine or calcium oxalate monohydrate (very hard).

Stone-free rates for solitary kidney stones are 80% for stones <1 cm in diameter, 60% for those between 1 and 2 cm
and 50% for those >2 cm in diameter. Lower stone-free rates as compared with open surgery or PCNL are accepted
because of the minimal morbidity of ESWL.
Side effects of ESWL:

ESWL causes a certain amount of structural and functional renal damage

Haematuria (microscopic, macroscopic) and edema are common,
Perirenal hematomas less so (0.5% detected on ultrasound with modern machines, although reported in as many
as 30% with the Dornier HM3).
Effective renal plasma flow (measured by renography) has been reported to fall in ~30% of treated kidneys.
There are data suggesting that ESWL may increase the likelihood of development of hypertension.
Acute renal injury may be more likely to occur in patients with pre- existing hypertension, prolonged
coagulation time, coexisting coronary heart disease, or diabetes and in those with solitary kidneys.

Contraindications to ESWL:
Absolute contraindications are pregnancy and uncorrected blood clotting disorders (including anticoagulation).
Procedure-specific consent form: Potential complications after ESWL.
Common:

Bleeding on passing urine for short period after procedure.

Pain in the kidney as small fragments of stone pass after fragmentation.
UTI from bacteria released from the stone, needing antibiotic treatment.
Occasional - Stone will not break as too hard, requiring an alternative treatment - Repeated ESWL treatments
may be required.
Recurrence of stones: Rare.
Kidney damage (bruising) or infection, needing further treatment.
Stone fragments occasionally get stuck in the tube (ureter) between the kidney and the bladder, requiring
hospital admission and sometimes surgery to remove the stone fragmenrt.

******ebook converter DEMO Watermarks*******

 Severe infection requiring intravenous antibiotics and sometimes drainage of the kidney by a small drain placed
through the back into the kidney. Alternative therapy: Endoscopic surgery, open surgery, or observation can be
used to allow spontaneous passage.

Important:

The first mechanism by which a stone might break is through spall fracture.

The second mechanism for stone breakage, termed squeezing-splitting or circumferential compression, occurs
because of the difference in sound speed between the stone and the surrounding.

The third mechanism is shear stress.

The fourth mechanism for stone breakage, superfocusing, is the amplification of stresses inside the stone due
to the geometry of that stone.
The fifth potential mechanism for SWL stone breakage is cavitation.
The final mechanism of stone fragmentation to be considered defines stone breakage in terms of a dynamic
fracture process, in which the damage induced by SWL accumulates during the course of the treatment, leading
to the eventual destruction of the stone.

Intracorporeal techniques of stone fragmentation (fragmentation within the body):

******ebook converter DEMO Watermarks*******
Electrohydraulic lithotripsy (EHL):

EHL was the first technique developed for intracorporeal lithotripsy. A high voltage applied across a concentric
electrode under water generates a spark. This vaporizes water, and the subsequent expansion and collapse of
the gas bubble generates a shock wave.
EHL is an effective form of stone fragmentation. The shock wave is not focused, so the EHL probe must be
applied within 1 mm of the stone to optimize stone fragmentation.
EHL has a narrower safety margin than that of pneumatic, ultrasonic, or laser lithotripsy and should be
kept as far away as possible from the wall of the ureter, renal pelvis, or bladder to limit damage to these
structures and at least 2 mm away from the cystoscope, ureteroscope, or nephroscope to prevent lens
fracture.

Principal uses are for bladder stones (wider safety margin than in the narrower ureter).
Pneumatic (ballistic) lithotripsy:

A metal projectile contained within the handpiece is propelled backward and forward at great speed by bursts
of compressed air. It strikes a long, thin, metal probe at one end of the handpiece at 12Hz (12 strikes/second)
transmitting shock waves to the probe, which, when in contact with a rigid structure such as a stone, fragments
the stone.
This technique is used for stone fragmentation in the ureter (using a thin probe to allow insertion down a
ureteroscope) or kidney (a thicker probe may be used, with an inbuilt suction device—Lithovac—to remove
stone fragments).
Pneumatic lithotripsy is very safe, since the excursion of the end of probe is about a millimeter, and it
bounces off the pliable wall of the ureter. Ureteric perforation is therefore rare.
The device is low cost and requires low maintenance. However, its ballistic effect has a tendency to cause stone
migration into the proximal ureter or renal pelvis, where the stone may be inaccessible to further treatment. The
metal probe cannot bend around corners, so it cannot be used for ureteroscopic treatment of stones within the
kidney or with a flexible ureteroscope.

Its principal use is for ureteric stones.

Ultrasonic lithotripsy:

An electrical current applied across a piezoceramic plate located in the ultrasound transducer generates
ultrasound waves of a specific frequency (23,000–25,000 Hz). The ultrasound energy is transmitted to a hollow
metal probe, which in turn is applied to the stone.
The stone resonates at high frequency and this causes it to break into small fragments (the opera singer
breaking a glass) that are then sucked out through the center of the hollow probe. Soft tissues do not resonate
when the probe is applied to them and thus are not damaged.
This technique can only be used down straight, rigid instruments.
Principal uses include fragmentation of renal calculi during PCNL.

Laser lithotripsy:

The holmium:YAG laser is principally a photothermal mechanism of action, causing stone vaporization. It has
minimal shock-wave generation and therefore less risk of causing stone migration. The laser energy is
delivered down fibers that vary in diameter from 200 to 360 microns. The 200-micron fiber is very flexible and
can be used to gain access to stones even within the lower pole of the kidney.
The zone of thermal injury is limited to 0.5–1 mm from the laser tip. No stone can withstand the heat generated
by the Ho:YAG laser. Laser lithot- ripsy takes time; however, since the thin laser fiber must be “painted” over
the surface of the stone to vaporize it.
Principal uses are for ureteric stones and small intrarenal stones.

******ebook converter DEMO Watermarks*******

Flexible ureteroscopy and laser treatment:

The holmium:YAG laser has a minimal effect on tissues at distances of 2–3 mm from the laser tip and so
collateral tissue damage is minimal with this laser type.
Flexible ureteroscopy and laser fragmentation offers a more effective treatment option than ESWL, with a
lower morbidity than PCNL, but usually requires a general anaesthetic (some patients will tolerate it with
sedation alone).
It can also allow access to areas of the kidney where ESWL is less efficient or where PCNL cannot reach. It is
most suited to stones <2 cm in diameter.

Indications for flexible ureteroscopic kidney stone treatment:

ESWL failure.
Lower pole stone (reduces likelihood of stone passage post- ESWL— fragments have to pass uphill).
Cystine stones.
Obesity such that PCNL access is technically difficult or impossible (nephroscopes may not be long enough to
reach stone).
Obesity such that ESWL is technically difficult or impossible. BMI >28 is associated with lower ESWL
success rates. Treatment distance may exceed focal length of lithotriptor.
Musculoskeletal deformities such that stone access by PCNL or ESWL is difficult or impossible (e.g.,
kyphoscoliosis).
Stone in a calyceal diverticulum (accessing stones in small diverticulae in upper and anterior calyces is difficult
and carries significant risks).
Stenosis of a calyceal infundibulum or tight angle between renal pelvis and infundibulum. The flexible
ureteroscope can negotiate acute angles and the laser can be used to divide obstructions.
Bleeding diathesis where reversal of this diathesis is potentially dangerous or difficult.
Horseshoe or pelvic kidney. ESWL fragmentation rates are only 50% in such cases due to difficulties of shock-
wave transmission through overlying organs (bowel). PCNL for such kidneys is difficult because of bowel
proximity and variable blood supply (blood supply derived from multiple sources).
Patient preference.

Percutaneous nephrolithotomy (PCNL):

******ebook converter DEMO Watermarks*******
Technique:

PCNL is the removal of a kidney stone via a track developed between the surface of the skin and the collecting
system of the kidney. The first step requires inflation of the renal collecting system (pelvis and calyces) with
fluid or air instilled via a ureteric catheter inserted cystoscopically.
Once the nephrostomy needle is in the calyx, a guide wire is inserted into the renal pelvis to act as a guide over
which the track is dilated . An access sheath is passed down the track and into the calyx and through this a
nephroscope can be advanced into the kidney .An ultrasonic lithotripsy probe is used to fragment the stone and
remove the debris.
A posterior approach is most commonly used, below the 12th rib (to avoid the pleura and far enough away
from the rib to avoid the intercostals, vessels, and nerve). The preferred approach is through a posterior calyx,
rather than into the renal pelvis, because this avoids damage to posterior branches of the renal artery that are
closely associated with the renal pelvis.
General anaesthesia is usual, though regional or even local anaesthesia (with sedation) can be used.

Indications for PCNL:

PCNL is generally recommended for stones >2cm in diameter and those that have failed ESWL and/or an
attempt at flexible ureteroscopy and laser treatment.
It is the first-line option for staghorn calculi, with ESWL and/or repeat PCNL being used for residual stone
fragments.

For stones 2–3 cm in diameter, options include ESWL (with a JJ stent in situ), flexible ureteroscopy and laser
treatment, and PCNL. PCNL gives the best chance of complete stone clearance with a single procedure, but this
is achieved at a higher risk of morbidity.
Outcomes of PCNL:
For small stones, the stone-free rate after PCNL is on the order of 90–95%. For staghorn stones, the stone-free rate
of PCNL, combined with postoperative ESWL for residual stone fragments, is on the order of 80–85%.

******ebook converter DEMO Watermarks*******

Open stone surgery:
Indications:

Complex stone burden (projection of stone into multiple calyces, such that multiple PCNL tracks would be
required to gain access to all the stone).
Failure of endoscopic treatment (technical difficulty gaining access to the collecting system of the kidney).
Anatomic abnormality that precludes endoscopic surgery (e.g., retrorenal colon).
Body habitus that precludes endoscopic surgery (e.g., gross obesity, kyphoscoliosis open stone surgery can be
difficult).
Patient request for a single procedure where multiple PCNLs might be required for stone clearance.
Nonfunctioning kidney: When the kidney is not working, the stone may be left in situ if it is not causing
symptoms (e.g., pain, recurrent urinary infection, hematuria). However, staghorn calculi should be removed,
unless the patient has comorbidity that would preclude safe surgery, because of the substantial risk of
developing serious infective complications.
If the kidney is nonfunctioning, the simplest way of removing the stone is to remove the kidney.
Functioning kidneys with either an open or laparoscopic technique options for stone removal:
Small to mediumsized stones:
Pyelolithotomy.
Radial nephrolithotomy.
Staghorn calculi:
Anatrophic (avascular) nephrolithotomy.
Done via BRODELS avascular zone located in between the posterior and lateral lobes of kidney. Any
surgery via this plane is a bloodless surgery.
Extended pyelolithotomy with radial nephrotomies (small incisions over individual stones): Also
known as GIL VERNETT surgery.
Excision of the kidney, to remove the stones, andautotransplantation:
Also known as “BENCH procedure”
Specific complications of open stone surgery:
Wound infection (the stones operated on are often infection stones).
Flank hernia.
Wound pain. (With PCNL these prob- lems do not occur; blood transfusion rate is lower, analgesic
requirement is less, mobilization is more rapid, and discharge is earlier all of which account for
PCNL having replaced open surgery as the mainstay of treatment of large stones).

Ureteric Calculus:
A stone in the ureter usually comes from the kidney. Most pass spontaneously.
Clinical features:
A stone passing down the ureter often causes intermittent attacks of ureteric colic.
Ureteric colic:
******ebook converter DEMO Watermarks*******
 The waves of agonising loin pain are typically referred to the groin, external genitalia and the anterior surface
of the thigh.
As the stone enters the bladder, the pain can be referred to the tip of the penis.

Impaction:

There are five sites of narrowing where the stone may be arrested.
An impacted stone causes a more consistent dull pain, often in the iliac fossa and increased by exercise and
lessened by rest.
Distension of the renal pelvis due to obstruction may cause loin pain. The stone may become embedded as the
adjacent ureteric wall becomes eroded and oedematous as a result of pressure ischaemia. Perforation of the
ureter and extravasation of urine is a rare complication.
Severe renal pain subsiding after a day or so suggests complete ureteric obstruction. If obstruction persists after
1–2 weeks, the calculus should be removed to avoid pressure atrophy of the renal parenchyma.

Haematuria:
Almost all ureteric colic is associated with transient microscopic haematuria. Serious bleeding is uncommon and
should suggest clot colic.
Treatment:
Pain:
Non-steroidal anti-inflammatory drugs, such as diclofenac and indomethacin, have replaced opiates as the first line
of treat- ment for renal colic. The value of smooth muscle relaxants, such as propantheline (Pro-Banthine), is
debatable.
Removal of the stone:
Expectant treatment is appropriate for small stones likely to pass naturally. If the patient is not disabled by recurrent
attacks of colic, progress can be followed by x-rays every 6–8 weeks.

******ebook converter DEMO Watermarks*******

 Many ureteric stones are 4 mm in diameter or smaller and most such stones (90%+) will pass spontaneously,
given a few weeks of watchful waiting, with analgesics for exacerbations of pain.
Average time for spontaneous stone passage for stones 4–6 mm in diameter is 3 weeks.
Stones that have not passed in 2 months are much less likely to do so, though large stones do sometimes drop
out of the ureter at the last moment.

Emergency treatment of an obstructed, infected kidney:

The rationale for performing percutaneous nephrostomy rather than JJ stent insertion for an infected, obstructed
kidney is to reduce the likelihood of septicemia occurring as a consequence of showering bacteria into the
circulation. It is thought that this is more likely to occur with JJ stent insertion than with percutaneous
nephrostomy insertion.
JJ stent insertion or percutaneous nephrostomy tube can be done quickly, but the stone is still present . It
may pass down and out of the ureter with a stent or nephrostomy in situ, but in many instances it simply
sits where it is and subsequent definitive treatment is still required.
While JJ stents can relieve stone pain, they can cause bothersome irritative bladder symptoms (pain
in the bladder, frequency, and urgency). JJ stents do make subsequent stone treatment in the form
of ureteroscopy technically easier by causing passive dilatation of the ureter.
The patient may elect to proceed to definitive stone treatment by immediate ureteroscopy (for stones at
any location in the ureter) or ESWL (if the stone is in the upper and lower ureter ESWL cannot be used for
stones in the mid-ureter because this region is surrounded by bone, which prevents penetration of the
shock waves).

Treatment options for ureteric stones:

ESWL: in situ or after push-back into the kidney (i.e., into the renal pelvis or calyces), or after JJ stent
insertion.
Ureteroscopy.
PCNL.
Open ureterolithotomy.
Laparoscopic ureterolithotomy.
Dormia Basketing of stones (blind or under radiographic control) are historical treatments (the potential
for serious ureteric injury is significant).
The ureter can be divided into two halves (proximal and distal to the iliac vessels) or in thirds (upper
third from the UPJ to the upper edge of the sacrum; middle third from the upper to the lower edge of the
sacrum; lower third from the lower edge of the sacrum to the VUJ).
Proximal ureteric stones – <1 cm diameter: ESWL (in situ, push-back).
>1 cm diameter: ESWL, ureteroscopy, PCNL.
JJ stent insertion does not increase stone-free rates and is therefore not required in routine cases. It is indicated
for pain relief, relief of obstruction, and in those with solitary kidneys.

Distal ureteric stones:

******ebook converter DEMO Watermarks*******
 Both ESWL and ureteroscopy are acceptable options.

Open ureterolithotomy and laparoscopic ureterolithotomy are used when ESWL or ureteroscopy have been
tried and failed or were not feasible.
Bladder stones:
Definition:
A primary bladder stone is one that develops in sterile urine; it often originates in the kidney. A secondary
stone occurs in the presence of infection, outflow obstruction, impaired bladder emptying or a foreign body.
Composition:
Bladder stones consist of:

Struvite (i.e., they are infection stones).

Uric acid (in noninfected urine).
IMPORTANT: mixed uric acid/urate stones are the most common type.
These stones are RADIOPAQUE. (isolated uric acid stones are radiolucent).

Adults:
Bladder calculi are predominantly a disease of men aged >50 and with bladder outlet obstruction due to benign
prostatic enlargement (BPE). They also occur in the chronically catheterized patient (e.g., spinal cord injury
patients), in whom the chance of developing a bladder stone is 25% over 5 years (similar risk whether urethral or
suprapubic location of the stone).1
Children:
Bladder stones are still common in Thailand, Indonesia, North Africa, the Middle East, and Burma. In these endemic
areas they are usually composed of a combination of ammonium urate and calcium oxalate. A low-phosphate diet in
these areas (a diet of breast milk and polished rice or millet) results in high peaks of ammonia excretion in the urine.
Symptoms:
Bladder stones may be symptomless (incidental finding on KUB X-ray or bladder ultrasound or on cystoscopy) and
is the common presentation in spinal patients who have limited or no bladder sensation.
In the neurologically intact patient, suprapubic or perineal pain, hematuria, urgency and/or urge incontinence,
recurrent UTI, and LUTS (hesitancy, poor flow) may occur.
Diagnosis:
If you suspect a bladder stone, it will be visible on KUB X-ray or renal ultrasound.
Treatment:

Most stones are small enough to be removed cystoscopically (endoscopic cystolitholapaxy), using stone-
fragmenting forceps for stones that can be engaged by the jaws of the forceps and
EHL or pneumatic lithotripsy for those that cannot.
Large stones can be removed by open surgery (open cystolitholapaxy).

Hypospadias:
Definition:

Hypospadias is a congenital deformity in which the opening of the urethra (the meatus) occurs on the
underside (ventral) part of the penis, anywhere from the glans to the perineum.
It is often associated with a hooded foreskin and chordee (ventral curvature of the penile shaft).
It occurs in 1 in 250 live male births.
There is an 8% incidence in offspring of an affected male, and a 14% risk in male siblings.
******ebook converter DEMO Watermarks*******
Classification:
Hypospadias can be classified according to the anatomical location of the urethral meatus.

Anterior (or distal) glandular, coronal, and subcoronal (~50%).

Middle distal penile, midshaft, and proximal penile (~30%).
Posterior (or proximal) penoscrotal, scrotal and perineal (~20%).

Etiology:

Hypospadias results from incomplete closure of urethral folds on the underside of the penis during
embryological development. This is related to a defect in production or metabolism of fetal androgens, or the
number and sensitivity of androgen receptors in the tissues.
Chordee is caused by abnormal urethral plate development, and
The hooded foreskin is due to failed formation of the glandular urethra and fusion of the preputial folds
(resulting in a lack of ventral foreskin but an excess of dorsal tissue).

Diagnosis:
Patients with absent testes and severe hypospadias should undergo chromosomal and endocrine investigation
to exclude intersex conditions.
Treatment:
Surgery is indicated:

Where deformity is severe, interferes with voiding,

OR is predicted to interfere with sexual function.

Surgery is now performed between 6 and 12 months of age. Local application of testosterone for 1 month
preoperatively can help increase tissue size.
Isolated CHORDEE correction can be done as early as 6 months

The aim of surgery is to correct penile curvature (orthoplasty).

Reconstruct a new urethra.
Bring the new meatus to the tip of the glans using urethroplasy, glanuloplasty, and meatoplasty techniques.
Severe cases may require staged procedures.
Common operations for anterior hypospadias include :
Meatal advancement and glanuloplasty (MAGPI):
Meatal-based flaps (Mathieu procedure)
Tubularization of the urethral plate.
Posterior defects require free grafts (buccal mucosa), on-lay grafts, and preputial transfer flaps.

******ebook converter DEMO Watermarks*******

Epispadias:
In epispadias, the urethra opens onto the dorsal surface of the penis, any- where from the glans, penile shaft,
or most commonly, the penopubic region.

An incomplete urethral sphincter mechanism results in a high risk of incontinence.

Epispadias is also associated with dorsal chordee (causing an upward curvature of the penis), with incomplete
foreskin dorsally.
Epispadias is part of the exstrophy–epispadias complex (which also includes bladder exstrophy and cloacal
exstrophy).

Associated anomalies:

Diastasis of the symphysis pubis results in splaying of the corpora cavernosa and shortening of the penile shaft.
Females have a bifid clitoris and poorly developed labia and demonstrate a spectrum of urethral deformities
ranging from a patulous urethral orifice to a urethral cleft affecting the entire length of the urethra and
sphincter.
There is a 40% risk of vesicoureteric reflux (VUR).

Incidence:

Epispadias affects 1 in 117,000 males.

It is rarely seen in females (male– female ratio is 5:1).

Management:

This involves urethroplasty with functional and cosmetic reconstruction of the external genitalia (penile
lengthening and correction of chordee) at 6–12 months.
The modified Cantwell–Ransley technique is commonly used in males. It describes mobilizing the urethra to
the ventral aspect of the penis, with advancement of the urethral meatus onto the glans with a reverse MAGPI
(meatal advancement-glanduloplasty). The corporal bodies are separated and rotated medially above the urethra
and re-approximated.
From age 4–5 years, when children can be toilet trained, bladder neck reconstruction can be performed
(Young–Dees–Leadbetter procedure). This achieves continence, and any bladder residuals may then be
emptied by urethral catheterization.

If this surgery fails, insertion of artificial urinary sphincters or collagen injections of the sphincter may be tried.
Posterior urethral valves:
******ebook converter DEMO Watermarks*******
Definition:
Posterior urethral valves (PUV) are abnormal congenital mucosal folds in the prostatic (posterior) urethra causing
lower urinary tract obstruction.
Incidence is 1 in >5000 males.
Classification:

Type I (90–95%): Membranes arise from the distal lateral aspect of the verumontanum,which extend distally
and anteriorly to fuse in the midline.
Type II: Longitudinal folds extending from the verumontanum to bladder neck (of historical interest only).
Type III (5%): A ring-like membrane found distal to the verumontanum.

Etiology:

Normal male urethra has small, paired lateral folds (plicae colliculi) found between the lateral, distal edge
of verumontanum and lateral urethral wall.
PUVs probably represent a congenital overgrowth of these folds from abnormal insertion of Wolffian ducts into
the posterior urethra during fetal development.

Presentation:
Prenatal US features: These include.

Bilateral hydroureteronephrosis.
Dilated bladder with elongated ectatic posterior urethra.
Thick-walled bladder.
Oligohydramnios (reduced amniotic fluid).
Renal dysplasia.

Early features are associated with poor prognosis.

Newborn and infants These children have:

Respiratory distress (secondary to pulmonary hypoplasia).

Palpable abdominal mass (hydronephrotic kidney or distended bladder).
Ascites.
UTI.
Electrolyte abnormalities.
Failure to thrive.

Older children:

Milder cases may present later with recurrent UTI, poor urinary stream, incomplete bladder emptying, poor
growth and incontinence.
There is a risk of renal failure, vesicoureteric reflux and voiding dysfunction (overactive or underactive
bladder), also described as valve bladder syndrome.
Associated features:

POP-off valve syndrome:

Seen in 20%.
It describes mechanisms by which high urinary tract pressure is dissipated to allow normal renal development.
It includes leaking of urine from small bladder or renal pelvis ruptures (urinary ascites), reflux into a
nonfunctioning kidney (vesicoureteral reflux with renal dysplasia [VURD] and formation of bladder diverticuli.

******ebook converter DEMO Watermarks*******

Investigation:

Ultrasound scan of kidneys and bladder.

VCUG shows distended and elongated posterior urethra (shield shaped; partially filled anterior urethra; bladder
neck hypertrophy; lucencies representing valve leaflets; thick-walled bladder (±diverticuli); incomplete bladder
emptying; reflux (50%).
Isotope renal scan (MAG-3, DMSA) assesses renal function.
Videourodynamics allows diagnosis of associated voiding dysfunction.

Management:

Commence prophylactic antibiotics immediately.

Check serum electrolytes and drain the bladder with a paediatric feeding tube.
If there is improvement, cystoscopy and transurethral ablation of valve (cuts at 5 and 7 o’clock with
electrocautery) is recommended (complications include urethral strictures).
If upper tracts remain dilated with raised creatinine after bladder drainage, a temporary cutaneous
vesicostomy is indicated (communicating stoma between the bladder dome and suprapubic abdominal wall,
allowing free drainage of urine).
An alternative is ureterostomy drainage.
Valve ablation is performed at a later stage.

Prognosis:
35% of patients will have poor renal function; 20% develop end-stage renal failure.
Undescended testes:
The testes descend into the scrotum in the third trimester (passing through the inguinal canal at 24–28
weeks). Failure of testicular descent results in cryptorchidism (or undescended testes).
Incidence:
Incidence is 3% at birth (unilateral > bilateral).
Approximately 80% will spontaneously descend by 3 months. The incidence at 1 year is 1%.
Thus, in most cases SPONTANEOUS DESCENT of the testis occurs upto 5 months, failing which the descent is
unlikely.
Classification:

Retractile: An intermittent active cremasteric reflex causes the testis to retract up and out of the scrotum.
Ectopic (<5%): Abnormal testis migration below the external ring of the inguinal canal (to perineum, base of
penis or femoral areas).
Incomplete descent (~95%): Testis may be intra-abdominal, inguinal, or prescrotal.
Atrophic/absent.

Risk factors:
These include:

Preterm infants.
Low birth weight, small for gestational age.
Twins.

Etiology:
This includes:

Abnormal testis or gubernaculum (tissue that guides the testis into the scrotum during development).

******ebook converter DEMO Watermarks*******

 Endocrine abnormalities (low level of androgens, human chorionic gonadotrophin [hCG], luteinizing hormone
(LH), calcitonin gene–related peptide).
Decreased intra abdominal pressure (prune-belly syndrome, gastroschisis).

Pathology:
There is degeneration of Sertoli cells, loss of Leydig cells, and atrophy and abnormal spermatogenesis.
Long-term complications:

Relative risk of cancer is 40-fold higher in the undescended testis.

Most are seminomas; carcinoma in situ represents a small percentage (~2%). There is a slightly increased risk
of cancer in the contralateral, normally descended testis.
Reduced fertility.
Increased risk of testicular torsion.
Increased risk of direct inguinal hernias (due to a patent processus vaginalis).

Management:

Full examination is required to elucidate if the testis is palpable and to identify location.
Assess for associated congenital defects.
If neither testis is palpable, consider chromosome analysis (to exclude an androgenized female) and hormone
testing (high LH and FSH with a low testosterone indicates anorchia).

Benign renal masses:

The most common (70%) are simple cysts, present in >50% of >50-year- olds.
SIMPLE CYSTS: Rarely symptomatic, treatment by aspiration or laparoscopic unroofing is seldom considered.
The two most clinically important are oncocytoma and angiomyolipoma.

Important:
Solid masses <2.0 cm are benign in up to 30% of cases, therefore a period of observation is reasonable as many of
these may not grow, especially in older or debilitated patients.
Oncocytoma:

This is uncommon, accounting for 3–7% of renal tumors.

Males are twice as commonly affected as females.
They occur simultaneously with renal cell carcinoma in 7–32% of cases.

Pathology:

Oncocytomas are spherical, capsulated (PSEUDOCAPSULE) and brown/tan in color, with a mean size of 4–6
cm.
Half contain a centralstellate scar.
They may be multifocal and bilateral (4–13%) and 10–20% extend into perinephric fat.
Histologically, they comprise aggregates of eosinophilic cells, packed with mitochondria. Mitoses are rare.
They are considered benign, not known to metastasize.
It is often difficult to distinguish oncocytoma from chromophobe RCC. There is often loss of the Y
chromosome.

Presentation:

Oncocytomas often (83%) present as an incidental finding or with flank pain or hematuria.

Investigations:
******ebook converter DEMO Watermarks*******
 Oncocytoma cannot often be distinguished radiologically from RCCs; it may coexist with RCC.
They exhibit a spoke-wheel pattern on angiography and stellate scar on CT scanning. Percutaneous biopsy
is not recommended since it often leads to continuing uncertainty about the diagnosis.
HALE COLLOIDAL IRON stain differentiates Oncocytoma (negative) from Chromophobe RCC (positive).

Treatment:

Partial nephrectomy (open or laparoscopic) is indicated whenever technically feasible, based on lesion size and
location.
Radical nephrectomy is rarely indicated unless the lesion is very large and partial nephrectomy is not possible
or diagnosis is uncertain. Ablation is used in selected cases.
No aggressive follow-up is usually necessary.

Angiomyolipoma (AML):

Eighty percent of these benign clonal neoplasms (hamartomas) occur sporadically, mostly in middle-aged
females.
20% are in association with tuberous sclerosis (TS)—an autosomal dominant syndrome characterized by
mental retardation, epilepsy, adenoma sebaceum and other hamartomas.
Half of TS patients develop AMLs.
The mean age is 30 years, and 66% of patients are female.
Frequently, AMLs are multifocal and bilateral.

Pathology:

AML is composed of blood vessels, smooth muscle and fat.

They are always considered benign, although extrarenal AMLs have been reported in venous system and hilar
lymph nodes.
Macroscopically, it looks like a well-circumscribed lump of fat.
Solitary AMLs are more frequently found in the right kidney.

Important:
HMB-45 is a monoclonal antibody against a melanoma-associated antigen seen in AML and can differentiate
cases from other renal cortical neoplasms.
Presentation:

AMLs frequently present as incidental findings (>50%) on ultrasound or CT scans.

They may present with flank pain, palpable mass, or painless hematuria.
Massive and life-threatening retroperitoneal bleeding occurs in up to 10% of cases, is known as Wunderlich
syndrome).
Pregnant women appear to be at an increased risk for hemorrhage.

Investigations:

Ultrasound reflects from fat, hence it has a characteristic bright echo pattern. This does not cast an acoustic
shadow beyond, helping to distinguish an AML from a calculus.
CT shows fatty tumor as low-density (Hounsfield units <-20) in 86% of AMLs. If the proportion of fat is
low, a definite diagnosis cannot be made.
Measurement of the diameter is relevant to treatment.
On MRI, adipose tissue has high signal intensity on T1-weighted images and lower on T2-weighted images.

Treatment:

In studies, 52–82% of patients with AML >4 cm are symptomatic compared with only 23% with smaller
******ebook converter DEMO Watermarks*******
 tumors.
Therefore, asymptomatic AMLs can be followed with serial ultrasound if <4 cm,
While those bleeding or >4 cm should be treated surgically or by embolization(preferred 1st line
management).
Emergency nephrectomy or selective renal artery embolization may be life saving.
In patients with TS, in whom multiple bilateral lesions are present, conservative treatment should be
attempted.

******ebook converter DEMO Watermarks*******

Renal cell carcinoma:
Epidemiology:
Renal cell carcinoma (RCC)—also known as hypernephroma (since it was erroneously believed to originate in the
adrenal gland), clear cell carcinoma and Grawitz tumor—is an adenocarcinoma.
It is the most common of renal tumors, accounting for 85% of renal malignancies and 2% of all cancer deaths.
RCC is considered the most lethal of all urological tumors, approximately 40% of patients dying of the condition.

It occurs in sporadic (most common) and hereditary (rare) forms.

RCC is adenocarcinoma of the renal cortex, believed to arise from proximal convoluted tubule.
It is usually tan colored and solid; 7–20% are multifocal, 10–20% contain calcification, and 10–25% contain
cysts or are predominantly cystic.
Smaller lesions are rarely grossly infiltrative.
They are usually circumscribed by a pseudocapsule of compressed tissue.

Etiology:

Males are affected twice as commonly as females.

Peak incidence of sporadic RCC is the sixth to eighth decade.

Environmental:
Studies have shown associations with the following:

Analgesic phenacetin use.

Asbestos exposure.
Family history in a first- or second-degree relative (relative risk of 2.9).
Hypertension (1.4- to 2-fold risk) (2nd mc cause).
Low socioeconomic status.
Obesity.
Renal failure and dialysis (30-fold risk).
Tobacco (most common cause).
Urban dwelling.
Nutrition is considered important: Asian migrants to Western countries are at increased risk of RCC; vitamins
A, C, E, and fruit and vegetable consumption are protective.
Anatomical risk factors: Polycystic and horseshoe kidneys.

Genetics:
******ebook converter DEMO Watermarks*******
Clear cell renal cell carcinoma: This is associated with deletion of chromosome 3p and/or mutations of the VHL
gene.
Von Hippel–Lindau (VHL) syndrome Half of individuals with this autosomal dominant syndrome are
characterized by:

Pheochromocytoma.
Renal and pancreatic cysts.
Cerebellar hemangioblastoma, (mc lesion associated).
RCC, often bilateral and multifocal. (clear cell carcinoma is mc type).

Patients typically present in third, fourth, or fifth decades.

VHL syndrome occurs from loss of both copies of a tumor suppressor gene on chromosome 3p25–26; this
and other genes on 3p are also implicated in causing the common sporadic form of RCC.
Inactivation of the VHL gene leads to effects on gene transcription, including dysregulation of hypoxia
inducible factor 1 (HIF-1), an intracellular protein that plays an important role in the cellular response to
hypoxia and starvation.
This results in upregulation of vascular endothelial growth factor (VEGF), the most prominent angiogenic
factor in RCC, which is why some RCCs are highly vascular.

Chromophobe RCC is a result of loss of chromosome 17.

Non-hereditary papillary RCC is linked with changes in Trisomy of 7 and 17.
Hereditary papillary RCC (HPRCC):

HPRCC arises from mutation and activation of the met proto-oncogene on chromosome 7p34.
Has an autosomal dominant familial component.
c-MET encodes the receptor tyrosine kinase for hepatocyte growth factor, which regulates epithelial
proliferation and differentiation in a wide variety of organs, including the normal kidney.

Birt-Hogg-Dubé (BHD) Syndrome:

Rare.
Autosomal dominant disorder caused by germline mutations in the BHD (FLCN) gene within the
chromosomal band 17p11.2.
Encodes for a tumor-suppressor protein, folliculin.
BHD syndrome is characterized by the cutaneous triad of fibrofolliculomas (hamartoma of the hair follicle),
trichodiscomas and skin tags, along with a propensity for renal tumors.
The renal tumors are often chromophobe RCC or oncocytoma or hybrids of these tumors.
A substantial number of patients will develop clear cell tumors as well. These tumors are more likely to be
multiple and bilateral.

Spread:

By direct extension to adrenal gland (7.5% in tumors >5 cm), through the renal capsule.
Into the renal vein (5% at presentation), inferior vena cava (IVC), right atrium.
By lymphatics to hilar and para-aortic lymph nodes.
Hematogenous to lung (75%), bone (20%), liver (18%), and brain (8%).

Histological classification of RCC:

Conventional (Clear cell carcinoma) (70–80%):

Arise from the proximal tubule.

Highly vascular; cells clear (glycogen, cholesterol) or granular (eosinophilic cytoplasm, mitochondria).

******ebook converter DEMO Watermarks*******

Papillary (10–15%):

papillary, tubular, and solid variants.

40% multifocal; small incidental tumors could equate with Bell’s legendary “benign adenoma”

Chromophobe (5%):

arises from the cortical portion of the collecting duct;

possess a perinuclear halo of microvesicles

Collecting duct (Bellini): Rare; young patients; poor prognosis

Medullary cell:

Rare; arises from calyceal epithelium.

Young, Black.
Associated with sickle-cell sufferers;
(important).
Poor prognosis.

The term sarcomatoid is used to describe an infiltrative, poorly differentiated.

Grading is by the Fuhrman system
(1 = well-differentiated; 2 = moderately differentiated; 3 and 4 = poorly differentiated) based on nuclear size,
outline, and nucleoli.
Pathologic stage has proved to be the single most important prognostic factor for RCC.

******ebook converter DEMO Watermarks*******

Robson staging:
International TNM Staging System for Renal Cell Carcinoma:

******ebook converter DEMO Watermarks*******

Modified from Edge SB, Byrd DR, Compton CC, et al, editors. AJCC cancer staging manual. 7th ed. New York:
Springer; 2010.
Presentation and Investigations:

More than 50% of RCCs are now detected incidentally on abdominal imaging carried out to investigate vague
or unrelated symptoms.
50% of patients present with haematuria.
40% with flank pain.
30% of patients notice a mass.
25% have symptoms or signs of metastatic disease (night sweats, fever, fatigue, weight loss, hemoptysis).
Less than 10% patients exhibit the classic triad of hematuria, flank pain, and mass.
Acute varicocele due to obstruction of the testicular vein by tumor within the left renal vein (5%).
Bilateral lower limb edema due to venous obstruction.

Paraneoplastic syndromes due to ectopic hormone secretion by the tumor occur in 10–40% of patients; these may
be associated with any disease stage.

******ebook converter DEMO Watermarks*******

Important: Non metatstatic liver dysfunction is known as Stauffer Syndrome
Investigations:
IOC for RCC is CECT. (In patients with contrast sensitivity MRI is done).

Radiological evaluation of hematuria, flank pain, and renal masses.

Needle biopsy of renal masses is not recommended, since the result may be misleading and complications
include hemorrhage and seeding of the biopsy tract.
Urine cytology and culture should be normal.
Full blood count may reveal polycythemia or anemia.
Any suggestion of renal vein or IVC involvement on CT may be further investigated with MRI. Thus , MRI is
IOC for evaluation of IVC invasion(important recent question)
Angiography may be helpful in planning partial nephrectomy or surgery for horseshoe kidneys.
Contralateral kidney function is assessed by uptake and excretion of CT contrast and the serum creatinine. If
doubt persists, an isotope renogram is obtained.

Active surveillance:

There are no currently established protocols.

Active surveillance is a reasonable option for patients with limited life expectancy or for those who are unfit for
or do not desire intervention, especially those with T1a renal masses (<4 cm). These masses grow slowly
growth and very infrequently progress to metastasis.

Surgical treatment:

Surgery is the mainstay of treatment for RCC.

Localized disease radical nephrectomy.

Open approach:

The aim is to excise the kidney with the perinephris (Gerota fascia), perhaps with ipsilateral adrenal gland (for
tumors >5 cm, upper pole tumors or with evidence of adrenal invasion) and regional nodes (controversial),
******ebook converter DEMO Watermarks*******
 removing all tumor with adequate surgical margins.
Surgical approach is usually transperitoneal (subcostal, midline), which provides good access to hilar and major
vessels or thoracoabdominal (for very large or T3c tumors).
Smaller masses can be approached retroperitoneally through a flank incision.
Following renal mobilization, the ureter is divided. Ligation and division of the renal artery or arteries should
ideally take place prior to ligation as well as division of the renal vein to prevent vascular swelling of the
kidney.
Complications include mortality up to 2% from bleeding or embolism of tumor thrombus; and bowel,
pancreatic, splenic, or pleural injury.

Laparoscopic Approach:

Well accepted for treating benign disease.

Masses of up to 10–12 cm can usually be removed by this approach, with larger masses requiring advanced
skills.
Approaches are either transperitoneal or retroperitoneal.
Advantages over open surgery include less pain, reduced hospital stay, and quicker return to normal activity.

Localized disease partial nephrectomy:

Nephron-sparing surgery is the best option for multifocal, bilateral tumors, particularly if the patient has VHL
syndrome or single functioning kidney
PN is now standard of care for the management of clinical T1 renal masses in the presence of a normal
contralateral kidney, presuming that the mass is amenable to this approach
Acceptable to treat small (<4 cm) tumors, even with a normal contralateral kidney, unless the tumor is close to
the pelvicaliceal or hilar vessels. Arteriography or three-dimensional CT/MRI reconstructions are helpful to the
surgeon.
laparoscopic partial nephrectomy is now standard.
Specific complications include failure of complete excision of the tumor(s) leading to local recurrence in up to
10% of cases, and urinary leak from the collecting system.
Postoperative follow-up.
The aim is to detect local or distant recurrence (incidence is 7% for T1N0M0, 20% for T2N0M0, and 40% for
T3N0M0) to permit additional treatment if indicated.
After partial nephrectomy, concern will also focus on recurrence in the remnant kidney.
Stage-dependent 6-monthly clinical assessment and annual CT imaging of chest and abdomen for 3–10 years.
Localized disease—tumor ablation therapy.
These technologies include thermal ablation by heating (radiofrequency ablation, or RFA) and cryotherapy and
may be accomplished by open, laparoscopic, or percutaneous approaches.
Cryoablation and RFA represent valid treatment alternatives for many older patients or those with substantial
comorbidities, presuming judicious patient selection and thorough patient counseling.
Cryoablation is associated with a lower rate of incomplete ablation (4.8%) than RFA (14.2%).
Technologies that employ novel techniques (HIFU, radiosurgicalablation; “Cyberknife” and microwave and
laser interstitial therapy remain investigational.

Lymphadenectomy:

Lymph node involvement in RCC is a poor prognostic factor.

Incidence ranges from 6% in T1–2 tumors, 46% in T3a, to 62–66% in higher-stage disease.
Lymphadenectomy at the time of nephrectomy may add prognostic information, but therapeutic benefit remains
unclear.

Treatment of Local Recurrence:

Surgical excision remains the preferred treatment choice, provided there are no signs of distant disease.
Local recurrence is more common after partial nephrectomy, where it can be treated by a further partial or total

******ebook converter DEMO Watermarks*******

 nephrectomy.

Adjuvant treatment:
Important: RCC is radioresistant. It may be used to palliate distant metastasis.
Molecular targeted therapies:

Sunitinib: Targets VEGF receptor tyrosine kinase and other pathways and is FDA approved for treatment of
metastatic clear cell carcinoma.

Noteworthy are reports of microangiopathic hemolytic anemia (MAHA) when used with bevacizumab.

Sorafenib: Targets multiple tryosine kinases (VEGF receptor PDGF) receptor, fibroblast growth factor
receptor-1 [FGFR-1], others).

It is FDA approved for metastatic clear cell RCC.

Temsirolimus: FDA approved for first-line therapy in poor-risk patients and offers approximately a 3-month
improvement in survival.
Everolimus: Approved for advanced RCC after failure of sunitinib or sorafenib. It is an mTOR (mammalian
target of rapamycin) inhibitor.
Bevacizumab is a VEGF inhibitor .It is not currently FDA approved for RCC.

Metastatic RCC:
Nephrectomy has long been indicated for palliation of symptoms (pain, hematuria) in patients with metastatic RCC
(if inoperable, arterial embolization can be helpful).
A recent study demonstrated a median survival benefit of 10 months for patients with good performance
status treated with cytoreductive nephrectomy prior to immunotherapy (interferon A-2B) and has further
expanded the indications for surgery in RCC.
Resection of solitary metastases is an option after nephrectomy and can extend survival.
Bladder cancer:
Epidemiology and etiology.
Bladder cancer is the second-most common urological malignancy.
Risk factors:

******ebook converter DEMO Watermarks*******

 Men are 2.5 times more likely than women.
Age increases risk; most commonly diagnosed in the eighth decade and is rare in those <50 years of age.
Race: Black people have a lower incidence than that of White people.
Environmental carcinogens, found in urine, are the major cause of bladder cancer.
Chronic inflammation of bladder mucosa: Bladder stones, long-term catheters and the ova of
Schistosomahaematobium(bilharziasis) are implicated in development of squamous cell carcinoma of the
bladder.
Smoking is the major cause of bladder cancer: in the developed world.

Cigarette smoke contains the carcinogens 4-aminobiphenyl (4-ABP) and 2-naphthylamine which appears to increase
urinary carcinogenic exposure of the urothelium.
Smokers have a 2- to 5-fold risk compared to that of non-smokers of developing bladder cancer and subsequent
recurrences.
Estimates suggest that 30–50% of bladder cancer is caused by smoking.
Passive exposure to cigarette smoke is also implicated.

Occupational exposure to carcinogens, in particular aromatic hydrocarbons like aniline, is a recognized cause
of bladder cancer.

A latent period of 25–45 years exists between exposure and carcinogenesis.

Drugs: Phenacetin and cyclophosphamide.

Pelvic radiotherapy for other malignancies such as prostate, rectal or cervical cancer.

Pathology:

Benign tumors of the bladder, including inverted papilloma and nephrogenic adenoma, are uncommon. The
vast majority of primary bladder cancers are malignant and epithelial in origin.
>90% are transitional cell carcinoma (TCC); urothelial carcinoma (UC) is now the preferred term.
1–7% are squamous cell carcinoma (SCC).
75% are SCC in areas where schistosomiasis is endemic.
2% are adenocarcinoma.
Rarities include pheochromocytoma, melanoma, lymphoma, and sarcoma arising within the bladder muscle.
Secondary bladder cancers are mostly metastatic adenocarcinoma from gut, prostate, kidney, or ovary.

Tumor spread:

Direct tumor growth to involve the detrusor, the ureteral orifices, prostate, urethra, uterus, vagina, perivesical
fat, bowel, or pelvic side walls.
Implantation into wounds/percutaneous catheter tracts.
Lymphatic infiltration of the iliac and para-aortic nodes.
Hematogenous, most commonly to liver (38%), lung (36%), adrenal gland (21%), and bone (27%). Any other

******ebook converter DEMO Watermarks*******

 organ may be involved.

Histological grading: Grading is divided into well, moderately, and poorly differentiated (abbre- viated to G1, G2,
and G3, respectively).

Primary UC is considered clinically as superficial or muscle invasive: 70% of tumors are papillary.
Papillary UC is most often superficial, confined to the bladder mucosa (Ta) or submucosa (T1). 10% of patients
subsequently develop muscle-invasive or metastatic disease.

Carcinoma in situ (CIS). is poorly differentiated carcinoma, but confined to the epithelium and associated with an
intact basement membrane.

Half of CIS lesions occur in isolation; the remainder occur in association with muscle-invasive UC.
CIS usually appears as a flat, red, velvety patch on the bladder mucosa.
From 40% to 83% of untreated CIS lesions will progress to muscle- invasive UC, making CIS the most
aggressive form of superficial UC.

Metastatic node disease is found in 0% Tis, 6% Ta, 10% T1, 18% T2 and T3a, and 25–33% T3b and T4 UC.
Squamous cell carcinoma (SCC):

SCC is usually solid or ulcerative and muscle invasive at presentation.

SCC accounts for only 2–5% of bladder cancers.
SCC in the bladder is associated with chronic inflammation (bladder calculi, indwelling catheters) and
urothelial squamous metaplasia, rather than CIS.
Bilharzial SCC is the most common form of bladder cancer in East Africa and the Middle East and is related to
infection with Schistosomahematobium. In Egypt, 80% of SCC cases are induced by the ova of Schistosoma
hematobium.
Non-bilharzial SCC is the second-most common form of bladder cancer in North America and Europe. 5% of
paraplegics with long-term catheters develop SCC.
Smoking is also a risk factor for SCC.
The prognosis is better for bilharzial SCC than for non-bilharzial disease, probably because it tends to be lower
grade and metastases are less com- mon in these patients.

Adenocarcinoma:

Adenocarcinoma is rare.
******ebook converter DEMO Watermarks*******
 Usually solid/ulcerative and high grade, and carries a poor prognosis.
It is frequently advanced at initial presentation (muscle invasive or metastatic).
One-third of cases originate in the urachus, the remnant of the allantois, located deep to the bladder mucosa in
the dome of the bladder.
Adenocarcinoma is a long-term (10–20+ year) complication of bladder exstrophy and bowel implantation into
the urinary tract, particularly bladder substitutions and ileal conduits after cystectomy. There is association with
cystitis glandularis, rather than CIS.

Presentation:
Symptoms:

The most common presenting symptom (85% of cases) is painless total hematuria.
34% of patients >50 years of age and 10% under age 50 with macroscopic hematuria have bladder cancer.
History of smoking or occupational exposure should raise suspicion for UC in the setting of hematuria.
Asymptomatic microscopic hematuria is found on routine urine stick- testing.
Pain is unusual, even if the patient has obstructed upper tracts, since the obstruction and renal deterioration
arise gradually. Pain may be caused by locally advanced (T4 disease).
Malignant cystitis: Filling-type lower urinary tract symptoms, such as urgency or suprapubic pain associated
with microscopic or macroscopic hematuria.
Advanced cases may present with lower limb swelling due to lymphatic/venous obstruction, bone pain, weight
loss, anorexia, confusion, and anuria (renal failure due to bilateral ureteral obstruction).
Urachal adenocarcinomas may present with a blood or mucus umbilical discharge or a deep subumbilical mass
(rare).

Diagnosis:

Cystoscopy is the preferred 1st line investigation.

Important :CT urography (CTU) before and after IV contrast is becoming the first- line radiological
investigation of hematuria.
Urine for cytology. Overall, routine urine cytology has 50% sensitivity but a high 96% specificity and is most
reliable with high-grade UC and CIS.
Fluorescent in situ hybridization (FISH) (sensitivity 77%, specificity 98%) and other tests such as NMP-22
(sensitivity 56%, specificity 85%) may be helpful in the evaluation.
Transurethral resection of bladder tumor (TURBT).
TURBT usually provides definitive histological diagnosis. This is usually undertaken under general or spinal
anesthesia. Bimanual examination is mandatory before and after bladder tumor resection in order to assess size,
position, and mobility.
The pathologist should report on the tumor type, grade, and stage.
The prostatic urethra is biopsied if radical reconstructive surgery such as orthotopicneobladder is under
consideration.
Mitomycin C, given as a single dose within 24 hours of TURBT.

Staging investigations:
These are usually reserved for patients with biopsy-proven muscle invasive bladder cancer unless clinically
indicated, since superficial UC and CIS disease are rarely associated with metastases.

Pelvic CT or MRI may demonstrate extravesical tumor extension or pelvic lymphadenopathy, reported if >5–8
mm in maximal diameter.
Chest X-ray.
Isotope bone scan (positive in 5–15% of patients with muscle-invasive UC) is obtained in cases being
considered for radical treatment.
Staging lymphadenectomy (open or laparoscopic) may be indicated in the presence of CT-detected pelvic
lymphadenopathy if radical treatment is under consideration. However, this is most often performed at the time
of radical cystectomy.

******ebook converter DEMO Watermarks*******

Adjuvant Intravesical Chemotherapy and BCG:
Adjuvant intravesical chemotherapy:

Intravesical chemotherapy (e.g., mitomycin C [MMC])is used for G1–2, Ta or T1 tumors, and recurrent
multifocal UC.
MMC is an antibiotic chemo- therapeutic agent that inhibits DNA synthesis.
It is used either as a single dose within 24 hours of first TURBT, or weekly for 6 weeks com- mencing up to 2
weeks post-TURBT.
Thiotepa is no longer used in most centers for superficial UC.

Other potential agents include doxorubicin and gemcitabine.

Adjuvant intravesical BCG:

BacilleCalmette–Guérin (BCG) is an attenuated strain of Mycobacterium bovis. It acts as an immune stimulant,

up-regulating cytokines such as IL-6 and IL-8 in the bladder wall.
BCG is given as a 6-week course for G3T1 UC and for CIS, starting at least 2 weeks post-TURBT.

Contraindications to intravesical BCG:

Immunosuppressed patients.
Pregnant or lactating women.
Patients with hematological malignancy.
After a traumatic catheterization.
******ebook converter DEMO Watermarks*******
 Active UTI.

Muscle-invasive bladder cancer : Surgical management of localized (pT2/3a) disease

Untreated 5-year survival is 3%.

Options for a patient with newly diagnosed confined mus- cle-invasive bladder cancer are as follows:

Bladder preservation:

Radical transurethral resection of bladder tumor (TURBT) plus systemic chemotherapy, mostly done IN setting
of clinical trial at present or in patients clearly unfit for radical cystectomy. With hydronephrosis, bladder
preservation relative contraindication.
Palliative TURBT palliative radiotherapy (RT): for elderly/unfit patients.
Partial cystectomy in highly selected patients without evidence of CIS.
TURBT plus definitive RT: poor options for SCC and adenocarcinoma as they are seldom radiosensitive.

Radical cystectomy with:

Ileal conduit urinary diversion

Continent urinary diversion (orthotopicneoblader or catherizable stoma)

Partial cystectomy:

A good option for well-selected patients with small solitary disease located near the dome, and for urachal
carcinoma.

Important:
Radical cystectomy with urinary diversion is the most effective primary treatment for muscle-invasive UC, SCC,
and adenocarcinoma, and can be used as salvage treatment if bladder preservation has failed.
It is also a treatment for G3T1 UC and CIS, refractory to BCG.
Radical External Beam Radiotherapy (RT):
This is a good option for treating muscle-invasive (pT2/3/4) UC in patients who are not healthy or unwilling to
undergo cystectomy.
Locally advanced bladder cancer (pT3b/4)
Neoadjuvant RT:
Randomized studies have suggested improvements in local control using RT prior to cystectomy, but no survival
benefit has been demonstrated.
Adjuvant RT:
The rationale for post-cystectomy RT is that patients with proven residual or nodal disease may benefit from
locoregional treatment.
Post-treatment bowel obstruction occurs 4.5 times more commonly in RT patients.
Neoadjuvant chemotherapy:
Neoadjuvant chemotherapy with methotrexate, vinblastine, adriamycin, and cisplatin (MVAC) should be considered
in all patients with muscle-in- vasive (T2–4a) disease.
Adjuvant chemotherap:
The rationale for post-cystectomy chemotherapy is that patients with proven residual or nodal disease may benefit
from systemic treatment. Patients with T3–4a, or N+ disease are at high risk for relapse following radical
cystectomy.
******ebook converter DEMO Watermarks*******
Adjuvant MVAC or gemcitabine/cisplatin can be considered.
Metastatic bladder cancer:
Systemic chemotherapy:

This modality is routine for patients with unresectable, diffusely metastatic measurable disease.
The most active agents include cisplatin, taxanes (docetaxel, paclitaxel), gemcitabine, and cisplatin.

Radiotherapy:
Roles for RT include palliation of metastatic pain and spinal cord compression.
Surgery :There is no surgical role in treatment of extravesical metastatic disease.
Urinary diversion after cystectomy (important for exams).
Ureterosigmoidostomy:

Form of urinary diversion, whereby the ureters drain into the sigmoid colon, either in its native form or
following its detubularization and reconstruction into a pouch (Mainz II).
This diversion requires no appliance (stoma bag, catheter) so remains popular in developing countries.

Complications:
1. Upper UTI with the risk of long-term renal deterioration.
2. metabolichyperchloremic acidosis, and
3. loose, frequent stools.
4. long-term risk of colon cancer.
Ileal conduit:

Most popular form of urinary diversion.

A 15 cm segment of ofsubterminal ileum is isolated on its mes-entery, the ureters are anastomosed to the
proximal end, and the distal end is brought out in the right lower quadrant as a stoma.
The ileum is anastomosed to gain enteral continuity (ileoileostomy).

Complications:

Prolonged ileus • Urinary leak • Enteral leak • Pyelonephritis • Ureteroileal stricture • Stoma problems-skin
irritation, stenosis, and parastomal hernia
Metabolic complications are uncommon.

Continent diversion:

The advantage of such a diversion is the absence of an external collection device.

A neobladder (pouch) is fashioned from 60–70 cm of detubularized ileum or right hemicolon. The ureters drain
into the neobladder, usually through an antireflux submucosal tunnel.
This may be drained by the patient via a catheterizable stoma, such as the appendix or uterine tube (the
Mitrofanoff principle) brought out in the right iliac fossa.
Alternatively, the neobladder may be anastomosed to the patient’s urethra so that natural voiding can be
established.
Patients void by relaxing their external sphincter and performing a Valsalva maneuver.

Testicular cancer:
Epidemiology:

******ebook converter DEMO Watermarks*******

 Primary testicular cancer (TC) is the most common solid cancer in men ages 20–45 years.
It is rare below age 15 years and above 60 years.
Constituting 1–2% of all male cancers, the lifetime risk of developing testicular cancer is 1 in 500.
It is also considered the most curable cancer.

Age:

The most commonly affected age group is 20–45 years, with germ cell tumors;
Teratomas are more common at ages 20–35.
Seminoma is more common at ages 35–45 years.
Infants and boys below age 10 years develop yolk sac tumors,
50% of men >60 years with TC have lymphoma.

Race: White people are three times more likely to develop TC than are Black people.
Cryptorchidism:

10% of TC cases occur in undescended testes.

The risk increases by 4-6 times compared to men with normally descended testes.
5–10% of patients with a cryptorchid testis develop malignancy in the normally descended contralateral testis.

Intratubular germ cell neoplasia (IGCN):

Is carcinoma in situ, although the disease arises from malignant change in spermatogonia.
50% of cases develop invasive germ cell TC within 5 years.

Risk factors:

Cryptorchidism.
Extragonadal germ cell tumor.
Previous or contralateral TC (5%).
Atrophic contralateral testis.
45XO karyotype.
Infertility.
Maternal estrogen ingestion during pregnancy increases the risk of cryptorchidism and TC in the male
offspring.

Important: Bilateral testicular cancer occurs in 1–2% of cases.

Pathology:

Ninety percent of testicular tumors are malignant germ cell tumors (GCT), split into seminomatous and non-
seminomatous (NS) GCTs for clinical purposes.
Seminoma, the most common germ cell tumor, appears pale and homogeneous.
NSGCTs are heterogeneous and sometimes contain bizarre tissues such as cartilage or hair.
Metastases to the testis are rare, notably from the prostate (35%), lung (19%), colon (9%), and kidney (7%).
The right testis is affected slightly more commonly than the left.
TC spreads by local extension into the epididymis, spermatic cord, and, rarely, the scrotal wall.
Lymphatic spread occurs via the testicular vessels, initially to the para-aortic nodes.
Involvement of the epididymis, spermatic cord or scrotum may lead to pelvic and inguinal node metastasis.
Blood-borne metastasis to the lungs, liver, and bones is more likely once the disease has breached the tunica
albuginea.

An additional S category is appended for serum tumor markers.

WHO histopathological classification of testicular tumors.

******ebook converter DEMO Watermarks*******

Staging

******ebook converter DEMO Watermarks*******

Clinical Presentation:
Symptoms:

Most patients present with a scrotal lump, usually painless or slightly aching.
Occasionally (5%), acute scrotal pain may occur, due to intratumoral hemorrhage, causing diagnostic
confusion.
In 10%, symptoms suggestive of advanced dis-ease include weight loss, lumps in the neck, cough, and bone
pain.

Signs:

Hard, non-tender, irregular, non-transilluminable mass in the testis or replacing the testis.
Reactive hydrocele may be present if the tunica albuginea has been breached.
Supraclavicular lymphadenopathy, left-sided neck mass, chest signs, hepatomegaly, lower limb edema or
abdominal mass—all suggestive of metastatic disease.
Gynecomastia is seen in <5% of patients with TC and is due to endocrine manifestations of some tumors.

Investigations:
Scrotal ultrasound: 1st line investigation

******ebook converter DEMO Watermarks*******

Any hypoechoic area within the tunica albuginea should be regarded with suspicion.
Remember: Pre operative FNAC/BIOPSY is contraindicated as there is risk of tumor seeding and also alteration in
the lymphatics.
Abdominal and chest CT scans are usually obtained for staging purposes if the diagnosis of TC is confirmed, usually
following radical orchiectomy.
Serum tumor markers are measured prior to any treatment of a confirmed testicular mass.
Treatment:
Radical orchiectomy (high inguinal orchidectomy).

Radical orchiectomy should be performed for diagnosis and treatment of the primary tumor.
This involves excision of the testis, epididymis and cord, with their coverings, through a groin incision.
The cord is clamped, transfixed, and divided near the internal inguinal ring before the testis is manipulated into
the wound, preventing inadvertent metastasis.
Sperm cryopreservation should be offered to patients without a normal contralateral testis.

Contralateral testis biopsy should be considered in patients at high risk for IGC.
Serum markers:

These tumor markers (with the exception of PLAP) are useful in diagnosis, staging, prognostication and
monitoring of response to treatment
Currently, testicular cancer is the only malignancy to incorporate serum markers into the staging system.

α-Fetoprotein (AFP):

AFP is expressed by trophoblastic elements within 50–70% of teratomas and yolk sac tumors.
With respect to seminoma, the presence of elevated serum AFP strongly suggests a non-seminomatous element.
Serum half-life is 3–5 days; normal is <10 ng/mL. It can be elevated.
Human chorionic gonadotrophin, B subunit (β-hCG).
β-hCG is expressed syncytiotrophoblastic elements of choriocarcinomas (100%), teratomas (40%) and
seminomas (10%). Serum half-life is 24–36 hours. Assays measure the B subunit with the normal <5 mIU/mL.

Lactate dehydrogenase (LDH):

LDH is a ubiquitous enzyme, elevated in serum for various causes and is therefore less specific.
It is elevated in 10–20% of seminomas, correlating with tumor burden, and is most useful in monitoring
treatment response in advanced seminoma.

Other markers with limited current clinical use include PLAP, CD30, and GGTP.
Clinical use of tumor markers:

These markers are measured at presentation, 1–2 weeks after radical orchiectomy, and during follow-up to
assess response to treatment and residual disease.
Normal markers prior to orchiectomy do not exclude metastatic disease.
Normalization of markers post orchiectomy cannot be equated with absence of disease and persistent elevations
of markers post- orchiectomy may occur with liver dysfunction and hypogonadotrophism, but usually indicate
metastatic disease.

Management of non-seminomatous germ cell tumors

(NSGCT)
Following radical orchiectomy and formal staging, the patient may require a retroperitoneal lymph node dissection
(RPLND)..

******ebook converter DEMO Watermarks*******

Stage I:

Surveillance is appropriate for reliable patients with no risk factors. It has a 25–30% relapse rate.
Requires close follow-up with imaging (CT, CXR), tumor markers, and physical examination
RPLND: modified unilateral template/nerve-sparing. (optional).
RPLND offers cure in 95% with negative nodes and may cure low volume nodal disease. Only 5% will relapse.
Chemotherapy with two cycles of bleomycin, etoposide, cisplatin (BEP) yields 95% survival.

Stage IIa/II:
RPNLD with modified bilateral template, RPNLD + adjuvant chemotherapy, or primary chemotherapy.

RPNLD and no tumor found: observe

RPNLD and positive nodes: two cycles of adjuvant chemotherapy
RPNLD and high-volume disease or residual tumor left behind: three cycles adjuvant chemotherapy

Stage IIC or III:

RPLND + Chemotherapy is the initial treatment, with either 3 or 4 cycles depending on risk.
Complete responses are frequently observed.
Management of seminoma:

Of all seminomas, 75% are confined to the testis at presentation and are cured by radical orchiectomy;
10–15% of patients harbor regional node metastasis, and 5–10% have more advanced disease.
RPLND is not used for seminoma. Unlike NSCGT, radiation therapy is a therapeutic modality in seminoma.
Subdiaphragmatic radiation is the traditional method: 35 Gy to the para-aortic and ipsilateral iliac lymph nodes
following radical orchiectomy using a “hockey stick” template.

Stage I:

Radical orchiectomy and adjuvant radiation therapy in low-stage disease is the most common treatment.
Close surveillance with imaging and serial tumor markers instead of radiation therapy for low-stage seminoma
is gaining acceptance.

Stage IIA:

Non-bulky retroperitoneal nodes: RT 35 Gy or chemotherapy

Stage IIB/IIC:

Bulky lymphadenopathy (>5–7 cm nodes) or visceral metastasis with standard NSCGT: platinum-based
chemotherapy (BEP/EP)

Stage III:

Chemotherapy; with brain metastasis, brain radiation therapy +/ excision.

Cure rate All stages have at least a 90% cure rate:

Stage I is 98–100%.
Stage II (B1/B2 non-bulky) is 98–100%.
Stage II (B3 bulky) and stage III have a >90% complete response to chemotherapy and an 86% durable
response rate to chemotherapy.

Management of intratubular germ cell neoplasia (IGCN):

******ebook converter DEMO Watermarks*******
 Observation or orchiectomy for unilateral disease.
Radiotherapy for unilateral disease in the presence of a contralateral tumor
Radiotherapy for bilateral disease, to preserve Sertoli cells
Frozen sperm storage must be offered.

Penile neoplasia:
Benign tumors and lesions:
Non cutaneous:

Congenital and acquired inclusion cysts

Retention cysts
Syringomas (sweat gland tumors)
Neurilemoma
Angioma, lipoma
Iatrogenic pseudotumor following injections
Pyogenic granuloma following injections

Cutaneous

Pearly penile papules (normal in 15% of postpubertal males).

Zoon balanitis (shiny, erythematous plaque on glans or prepuce)
Lichen planus (flat-topped violaceous papule)

Viral-related lesions:
Condyloma acuminatum:

Also known as genital warts.

Related to human papillomavirus (HPV) infection.
Soft, multiple benign lesions on the glans, prepuce, and shaft.
they may occur elsewhere on genitalia or perineum.
A biopsy is worthwhile prior to topical treatment with podophyllin.
5% have urethral involvement, which may require diathermy.
HPV infection (particularly types 16 and 18) is potentially carcinogenic.
Condylomata have been associated with penile SCC.

Bowenoid papulosis:

Resembles carcinoma in situ, but with a benign course.

Multiple papules appear on the penile skin, or a flat glanular lesion.
These should be biopsied.
HPV is the suspected cause.

Kaposi sarcoma:

This reticuloendothelial tumor has become the second-most common malignant penile tumor.
It presents as a raised, painful, bleeding violaceous papule or as a bluish ulcer with local edema.
It is slow growing, solitary, or diffuse.
It occurs in immunocompromised men, particularly in gay men with HIV/AIDS.
Urethral obstruction may occur.
Treatment is palliative, with intralesional chemotherapy, laser, cryoablation, or radiotherapy.

Premalignant cutaneous lesions:

******ebook converter DEMO Watermarks*******

Cutaneous horn:

Rare, solid skin overgrowth;

Extreme hyperkeratosis,
The base may be malignant.
Treatment is wide local excision.

Pseudoepitheliomatous micaceous and keratotic balanitis:

Unusual hyperkeratotic growths on the glans.

They require excision.
Histological examination, and follow-up, as they may recur.

Balanitis xerotica obliterans (BXO):

Also known as lichen sclerosus et atrophicus.

This is a common sclerosing condition of glans and prepuce.
It occurs at all ages and most commonly presents as non-retractile foreskin (phimosis).
The meatus and fossa navicularis may be affected, causing obstructed and spraying voiding.
Important:The histological diagnosis is usually made after circumcision, with epithelial atrophy, loss of rete
pegs, and collagenization of the dermis.
BXO occurs in association with penile SCC, but most pathologists would regard the lesion as benign unless
epithelial dysplasia is present.

Leukoplakia:

Solitary or multiple whitish glanular plaques that usually involve the meatus.
Leukoplakia is associated with in situ SCC.
Treatment is excision and histology.

Erythroplasia of Queyrat:

Also known as carcinoma in situ of the glans, prepuce, or penile shaft.

A red, velvety, circumscribed painless lesion
May ulcerate, resulting in discharge and pain.
Treatment is excision biopsy if possible;
Histology reveals hyperplastic mucosal cells with malignant features.
radiotherapy, laser ablation, or topical 5-fluorouracil may be required.
Bowen disease: this is carcinoma in situ of the remainder of the keratinizing genital or perineal skin. Treatment
is wide local excision, laser, or cryoablation.

Buschke–Löwenstein tumor:

Also known as verrucous carcinoma or giant condylomaacuminatum.

Aggressive locally invasive tumor of the glans.
Metastasis is rare, but wide excision is necessary to distinguish it from SCC.

Penile cancer:
Epidemiology:

Squamous cell carcinoma (SCC) is the most common penile cancer, accounting for 95% of penile
malignancies.
Others include Kaposi sarcoma and, rarely, basal cell carcinoma, melanoma, sarcoma, and Paget disease.
Metastases to the penis are occasionally seen from the bladder, prostate, rectum, and other primary sites.

******ebook converter DEMO Watermarks*******

 Incidence.
Penile cancer is rare, representing 1% of male cancers.
Risk factors for SCC:
Age: penile cancer incidence rises during the sixth decade and peaks in the eighth decade.
Premalignant lesions: 42% of patients with penile SCC are reported to have had a pre-existing penile
lesion.
A prepuce (foreskin): penile cancer is rare in men circumcised at a young age.
Smegma that forms from desquamated epithelial cells is thought to be a primary instigating factor in
penile cancer; good hygiene and circumcision limit smegma accumulation.
Geography: Highest incidence worldwide is in Brazil. It is virtually non-existent in Israel.
Human papilloma virus (HPV) genital wart infection, especially with types 16, 18, and 21.
Multiple sex partners.
Smoking and tobacco products.

Pathology:
SCC starts as a slow-growing papillary, flat or ulcerative lesion on the glans (48%), prepuce (21%), glans and
prepuce (9%), coronal sulcus (6%) or shaft (2%). The remainder are indeterminate.

******ebook converter DEMO Watermarks*******

Presentation:

A hard, painless lump on the glans penis is the most common presentation.
15–50% of patients delay presentation for >1 year because of embarrassment, personal neglect, fear, or
ignorance.
A bloody discharge may be confused with hematuria.
Groin mass or urinary retention are presenting symptoms.
The inguinal lymph nodes are examined. They may be enlarged, fixed, or even ulcerate overlying skin.

Investigations:
A biopsy is indicated.
Treatment:

******ebook converter DEMO Watermarks*******

Primary tumor:

The first-line treatment of penile cancer, regardless of the inguinal node status, is surger.
Circumcision is appropriate for preputial lesions, but local recurrence is observed in 22–50%.
Penis-preserving wide excision with 2 cm tumor free margins of glanular lesions with skin graft glanular
reconstruction may be suitable for smaller G1–2 Ta–1 tumors, giving good cosmetic and functional results.
Alternatives to surgery include laser or cryoablation, radiotherapy or brachytherapy, photodynamic therapy, or
topical 5-fluorouracil. Mohs cutaneous surgery has been used with mixed results.
For G3T1 and more advanced tumors, partial or total penile amputation is required, depending on the extent of
the tumor.
Partial amputation is preferable, provided a 2 cm margin of palpably normal shaft can be obtained.
The patient must be prepared for poor cosmetic and functional results: inability to have sexual intercourse and
need to sit to void urine.
Local recurrence occurs in 10% if the excision margin is positive.
Total amputation involves excision of the scrotum and its contents, with formation of a perineal urethrostomy.
The most common complication is urethral meatal stenosis.
Radiotherapy remains an alternative, but disadvantages include radio-resistance, leading to reported recurrence
rates of 30–60%; and
tissue necrosis and damage leading to urethral stricture, fistula, and pain.
Patients with M1 disease are offered palliative surgery.

Prophylactic lymphadenectomy:

This is currently practiced in for tumors:

exhibiting vascular invasion,
high grade, or
3. stages T2–4.
Lymph node sampling (either sentinel node biopsy or modified inguinal dissection) may be offered for patients
with palpably normal inguinal nodes and T2 or above lesion to avoid the morbidity of formal inguinal
lymphadenectomy.

Urethral cancer:

Primary urethral cancer is rare, occurring in elderly patients.

It is 4 times more common in women than in men.
Risk factors:
Urethral stricture and sexually transmitted disease are implicated.
Direct spread from tumor in the bladder or prostate is more common.

Pathology:

Seventy-five percent of cases are SCC, occurring in the anterior urethra;

15% are UC, occurring in the posterior/prostatic urethra.
8% are adenocarcinoma.
Urethral cancer metastasizes to the pelvic lymph nodes from the posterior urethra and to the inguinal nodes
from the anterior urethra in 50% of patients.

Presentation:
This is often late; many patients have metastatic disease at presentation.

Painless hematuria; initial, terminal, or a bloody urethral discharge.

Voiding-type LUTS (less common).
Perineal pain (less common).
Periurethral abscess or urethrocutaneous fistula (rare).
Past history of sexually transmitted or stricture disease.
******ebook converter DEMO Watermarks*******
Treatment:

For localized anterior urethral cancer, radical surgery or radiotherapy are the options.
Results are better with anterior urethral disease .
Male patients would require perineal urethrostomy.
Postoperative incontinence due to disruption of the external sphincter mechanism is minimal unless the bladder
neck is involved, but the patient would need to sit to void.
For posterior/prostatic urethral cancer, cystoprostatourethrectomy should be considered for men in good overall
health,
Anterior pelvic exenteration (excision of the pelvic lymph nodes, bladder, urethra, uterus, ovaries, and part of
the vagina) should be considered for women.

Prostate:
Acute Bacterial Prostatis (ABP):
Characterized by; Fever, chills, low back and perineal pain, Myalgia and varying degree of irritative and bladder
outlet obstruction features. Rectal examination reveals hot and tender prostate.
Caused by E. coli (commonest), Proteus, Klebsiella, Enterobactor, Pseudomonas, Serratia and other less common
gram negative organism. (Most infections are caused by a single pathogen).
Treatment:
Antibiotics and symptomatic (analgesics) treatment.
Septran (TMP-SMX): Should be given for 30 days to prevent CBP, Ciprofloxacin, Norfloxacin, Ofloxacin or
ampicillin with gentamicin (IV). Urethral instrumentation should be avoided in acute phase.
Prostatic Abscess:
Coliform (mainly E. coli) is the main causative organism (>70%). Presentation is like acute prostatitis, which fails to
respond to antibiotics, (commonest presenting symptom is acute urinary retention and fever > 35%). On PR
examination prostate is tender with an area of fluctuation. Main diagnostic tools are TRUS and CT scan. Treatment
is: drainage under antibiotic cover. Drainage is done by transurethral route, percutaneous aspiration, or perineal
incision.
Chronic Bacterial Prostatitis (CBP):
It may evolve from ABP, but many men with CPB have no prior history of ABP. It mainly presents with irritative
voiding symptoms. Postejaculatory pain or hemospermia may be found. Hallmark of CBP is recurrent UTI, caused
by same pathogen. Prostatic exprassates show excessive WBC and fat laden macrophages and fewer bacteria.
Treatment is mainly medical: Antibiotic therapy, Septran (for 4 to 16 weeks), Carbenicillin, erythromycin,
minocyclin, Doxycyclin and cephalexin. Fluoroqunolone: ciprofloxacin, norfloxacin and ofloxacin are also
effective. Those who do not respond to medical therapy are candidate for surgical therapy (TURP).
Nonbacterial Prostatitis
(Abacterial Prostatitis, Prostatosis):
It is an inflammatory condition of unknown cause. Usually presents with irritative voiding symptom and pain /
discomfort in pelvis, suprapubic region genitals, perineal or postejaculatory. In NBP culture is negative despite the
presence of excessive leukocytes and macrophages.
Exact causative organism is not known but Staphylococcus epidermidis, Ureaplasma urealyticum, Mycoplasma and
Chlamydia tracomatis are probable pathigen.
As the causative organism is not known, when culture is negative an empirical trial of tetracycline, erythromycin,
minocyclin or doxycyclin is given.
Prostatodynia (Pd):

******ebook converter DEMO Watermarks*******

Patient with PD has symptoms of prostatitis but no H/O UTI, culture is negative and typically normal Prostatic
secretion. A typical patient of prostatodynia is young or middle aged with variable sign and symptom of urinary
flow, irritative voiding and pain.
It is diagnosed by normal urine findings, normal EPS, sterile culture but abnormal urodynamic study (Decreased
UFR, decreased relaxation of the sphincter, increased urethral pressure).
Treatment includes: Sitz bath, alpha-1 blockers, sedative and analgesics.
Summary:

Benign Hyperplasia of Prostate

Mc Neal’s 4 zones
1. Peripheral zone.
2. Transitional zone(periurethral zone).
3. Central zone.
4. Anterior fibro muscular stromal.

BHP typically affects transitional zone.

Most common benign tumour in men.

Seen in 50% between 50-60 years and 90% in ninth decade.

Symptoms:
A- Irritative symptoms
1:Frequency, urgency, nocturia, urge incontinence, nocturnal enuresis.
B- Bladder outlet obstruction
1. Poor flow
2. Hesitancy in initiating urine
3. Intermittency (double voiding)
4. Sense of incomplete emptying
5. Inability to terminate micturition abruptly with post micturition dribbling.
Secondary effects: The urethra gets compressed laterally and is elongated causing bladder outlet obstruction e.g.
trabaculation/ secculation/ diverticuli. Later on there may be stone formation or Vesico-ureteral reflux.
Pathology:
Characterized by adenosis, epitheliosis and stromal proliferations. It mainly involves the central part and lateral part
gets compressed. With enlarging prostate middle lobe develops, which projects into the base of bladder.
Some important points regarding pathogenesis of BPH.

******ebook converter DEMO Watermarks*******

 Previously held notions that the clinical symptoms of BPH (prostatism) are due simply to a mass-related
increase in urethral resistance are too simplistic. It is now clear that a significant portion of LUTS is due to age-
related detrusor dysfunction
The precise molecular etiology of this hyperplastic process is uncertain. The observed increase in cell number
may be due to epithelial and stromal proliferation or to impaired programmed cell death leading to cellular
accumulation.
The prostate, unlike other androgen-dependent organs, maintains its ability to respond to androgens throughout
life
Intraprostatic DHT concentrations are maintained but not elevated in BPH.
Two steroid 5α-reductase enzymes have been discovered. The role of type 1 5α-reductase in normal and
abnormal prostate growth remains to be defined but the type 2 enzyme is critical to normal development of the
prostate and hyperplastic growth later in life.
Immunohistochemical studies with type 2 5α-reductase specific antibodies show primarily stromal cell
localization of the enzyme. Epithelial cells uniformly lack type 2 protein, and some basal epithelial cells stain
positively.

Clinical presentation:
Frequency is the earliest symptom, which initially is only nocturnal. Bladder symptoms are divided in irritative
symptoms (e.g. Frequency, urgency, urge incontinence, nocturia) or obstructive symptoms (e.g. hesitancy, thin
stream of urine, terminal dribbling and retention). Other features are recurrent UTI (due to increased residual urine)
hematuria, or renal failure due to backpressure changes.
Examination may reveal bladder lump and on PR examination enlarged prostate (feature on BPH are: non nodular
enlarged prostate with firm consistency, prominent median sulcus).
It is important to examine nervous system also to exclude the presence of neurogenic bladder.
Investigations: Complete hemogram and urinalysis, blood urea and serum creatinine.
USG is the investigation of choice:
PSA (prostate specific antigen) is helpful in excluding carcinoma. It is a glycoprotein (mol.wt: 33,000), its normal
value is 0- 4 ng/dl.
Urodynamic study (uroflowmetry, cystometrogram, and urethral pressure profile) is also helpful. A value of <10
ml/sec in UFR is suggestive of obstruction. Cystometrogram is helpful in differentiating between BPH and
neurogenic bladder (indicated when patient presents with mainly irritative symptoms).

AUA Symptom score:

Mild-0-7.
Moderate-8-19.
Severe-20-35.
******ebook converter DEMO Watermarks*******
 For mild symptoms, “Wait and watch” is recommended.
For moderate and severe symptoms, intervention is required.

Treatment:
1 Medical: By A) Androgen deprivation with LHRH agonists; Progestational compounds; Antiandrogens
(cyproterone acetate, flutamide); 5 alpha reductase inhibitor (finastride: it prevents conversion of testosterone to
dihydrotestosterone). B) alpha 1 blockers prazocine, terazocin (long acting).
2 Surgical: Open prostatectomy (suprapubic- Freyer’s, transpubic- Millin’s, perineal- Young’s) and Trans urethral
prostatectomy. 2 main complications of TURP are; Dilutional hyponatremia (when distilled water is used for
irrigation) and hyperammonical state (with glycein).
Indications for surgery:

Refractory retention (failed on ecatheter free trial).

Recurrent UTI.
Rcurrent haematuria.
Bladder stone.
Non responders of medical treatment.

Absolute Indications for surgery:

Bladder decompensation with overflow incontinence.

Hydronephrosis.

Alpha blockers: Relaxes smoth muscle, decreases urethral resistance.

5 Alpha reductase inhibitors: Prevents conversion to testosterone to Dihydro-testosterone by blocking 5 Alpha
reductase thus reducing size.

Maximum effect is seen in 6 months. Glad size reduction is achived by 20%. Mainly used for prostate size > 40
gm and PSA value is reduced by 50%.

Surgical:

TURP is gold standard.

Best irrigating fluid is Glycine 1.5% (Saline contains ions so not compatible with electrocautery). But now a
days bipolar cautery is also being used where saline can be used as an irrigant.
******ebook converter DEMO Watermarks*******
 TURP is most important proximal land mark in TURP (TURP below this will cause incontinence).
Complications are: Bleeding (Earliest commonest- bleeding from Badenoch’s artery), Injury to ureter or
bladder, TURP syndrome (Glycine metabolized to Glycolic acid and ammonia causing encephalopathy like
symptoms- and visual toxicity). Late complications are – Retrograde ejaculation (commonest 75%), Bladder
neck contracture (Mainly seen in small prostate <20 gms, where TUIP is better than TURP), impotence,
incontinence.
For big prostate- HO:YAG Laser is better (HoLEP).

Newer techniques are:

TUVP (Transurethral vaporization of prostate).

Water-Induced Thermotherapy.
Transurethral Ethanol Ablation of the Prostate.
Rotoresection of the Prostate.
Prostatic urethral lift.

Carcinoma of the Prostate:

Commonest malignant condition in men over 65 years. It usually originates from lateral lobes (Lowsley) / peripheral
zone (Mcneal).Porsterior lobe 70%, central lobe – 15-20%, Transitional lobe – 10-15%.Histologically commonest
type is Adenocarcinoma.
Risk Factor:
Afro-American > Whites. Less common in Asians.
Avancing age with Incraesed fat intake. Defeciency of Lycopene, Vitamin A, E and Selenium.
Most common type – Adenocarcinoma
Best screening modality – PSA and DRE (95% accuracy).
70% aise from peripheral zone and 80% are multicentric.
Genes:

The products of two hypermethylated genes that have been evaluated in prostate cancer development are
glutathione S-transferase P1 (GSTP1- Chromosome 11) and ras association domain family protein isoform A
(RASSF1A).Hypermethylation of regulatory sequence at the glutathione S transferase pi (GSTP 1) gene,
located at chromosome 11,found in > 90% of prostate cancer (Most common gene alteration - MCQ).
The α-methylacyl coenzyme A racemase (AMACR) gene, located on chromosome 5, has been found to be
upregulated in prostate cancer tissues
Hereditary- Gene called hereditary prostate cancer 1 (HPC 1) or Prostate cancer susceptibility 1 (PCS 1). This
gene is situated on Chromosome 1, specifically on Chromosome 1q24-q25.

Pathology:

Carcinoma shows glandular crowding, smaller glands, Lack of branching, papillary infolding.
Absence of basal layer.

Spread:
Local: initially capsule and the denonvillier’s fascia prevent its spread. Later on there is spread to SV, ureter,
bladder base, urethra or rectum.
Hematogenous: Ca prostate spreads to bones through periprostatic venous plexus. Prostate is the most common site
of origin to bone mets.“Oteoblastic” Secondary is mostly to lower vertebra, pelvis, Femur, ribs and skull. Other than
bones, breast, kidney, lungs or thyroid may be secondarily involved.
Lymphatic: First to involve is obturautor nodes then it spreads to internal iliac nodes, external iliac nodes, and later
on to retroperitoneal node, mediastinal and supraclavicular node.
******ebook converter DEMO Watermarks*******
Common presenting symptoms are: Features of bladder outlet obstruction, retention, haematuria or incontinence.
This may be an incidental finding found by raised PSA, palpable nodule in PR examination, histologically detected
Ca in TURP chips.Occasionally bone mets. (Pain, neurological symptoms due to cord compression, or pathological
fracture) may be a presenting symptom.
PR examination reveals hard irregular nodular enlarged prostate with loss of median sulcus.
Diagnosis is confirmed by ultrasound guided needle biopsy of mass lesion (80% prostate cancer appear as
hypoechoic lesion in USG).
Other important investigations are complete hemogram, LFT, acid phosphatase (raised in 70% cases of bone mets),
PSA (other then diagnosis it is also helpful in detecting recurrence after radical prostatectmy), x-ray chest and pelvis
(to rule out mets, bone secondary in Ca prostate is sclerotic: D/D; pegets disease), CT scan (to see the extent of
disease in advanced cases) MRI (to locate the neuro-vasculer bundle, if nerve sparing prostatectomy is to be done),
Bone scan (Technitiun 99-m labeled methylene diphosphonate is used).
PSA:

It’s a glycoprotein Serine protease,

Normal value is 0-4 ngm/ ml,
Causes liquefaction of serum.
Value > 20 is diagnostic of Cancer,
Half life is 2.5 days.
PSA Density is PS/ Prostate volume and should be < 0.15,
PSA velocity is rate of increase of PSA pe year and it shoul not be > 0.75 ng/ml/year (assessed every 18
months)
The combination of DRE and serum PSA is the most useful first-line test for assessing the risk of prostate
cancer being present in an individual
Serum PSA elevations may occur as a result of disruption of the normal prostatic architecture that allows
PSA to diffuse into the prostatic tissue and gain access to the circulation. This can occur in the setting of
prostate disease (BPH, prostatitis, prostate cancer) and with prostate manipulation (e.g., prostate massage,
prostate biopsy, transurethral resection)
PSA elevations may indicate the presence of prostate disease, but not all men with prostate disease have
elevated PSA levels. Furthermore, PSA elevations are not specific for cancer.
5α-Reductase inhibitors that are used for treatment of BPH have been shown to lower PSA levels by about
50% after 12 months of treatment. Both type 2 isoenzyme inhibitors (finasteride) and dual type 1 and 2
isoenzyme inhibitors (dutasteride) lower PSA to the same extent

Newer tumour markers:

1. α-Methylacyl Coenzyme A Racemase:The α-methylacyl coenzyme A racemase (AMACR) gene, located on
chromosome 5, has been found to be upregulated in prostate cancer tissues.
2. PCA3/ DD3: Expression of the DD3PCA3 protein has been localized to prostatic tissue and has been found in
95% of prostate cancer and prostate metastasis specimens.
3. Prostate Breast Overexpressed Gene1 (PBOV1) or UROC28:overexpressed in prostate, breast, and bladder
carcinomas. This gene is on chromosome 6q23-24, and the expression product protein is measurable in serum.
4. Hepsin.
5. NMP 48 (50.8 kD).
Imaging:

Endorectal MRI: Most optimal imaging for Postate anatomy.

MR spectroscopy: Prostate cancer is associated with lower lvels of Citrate and higher levels of choline and
creatine.
Comined MRI and MR spectroscopy allows more accurate assessment of cncerlocaion and stage.
CT canis best to assess LN metastasis
IV supermagnetc nanoparticles in high resolution MRI improves small node visualization.

******ebook converter DEMO Watermarks*******

Gleason score: Is most commonly used Histological grading system for prostate cancer.
The Gleason system is based on the glandular pattern of the tumor as identified at relatively low magnification.
Cytologic features play no role in the grade of the tumor.
Both the primary (predominant) and the secondary (second most prevalent) architectural patterns are identified and
assigned a grade from 1 to 5, with 1 being the most differentiated and 5 being the least differentiated.
Gleason grade ranges for 1 – 5 and Gleason score ranges from 1 – 1 0.
There resulted a Gleason sum or score obtained by the addition of the primary and secondary grades. If a tumor has
only one histologic pattern, then for uniformity, the primary and secondary patterns are given the same grade.
Gleason scores range from 2 (1 + 1 = 2), which represents tumors uniformly composed of Gleason pattern 1 tumor,
to 10 (5 + 5 = 10), which represents totally undifferentiated tumors.
Gleason score > 7 is considered anaplastic (High grade).
Prostate Cancer Staging Systems:

Treatment:

******ebook converter DEMO Watermarks*******

A. Surgery: Radical prostatectomy or TURP to relieve outflow obstruction.

Early complications include hemorrhage; rectal, vascular, ureteral, or nerve injury; urinary leak or fistula;
thromboembolic and cardiovascular events; urinary tract infection; lymphocele; and wound problems.
The most common late complications of radical prostatectomy are erectile dysfunction, urinary incontinence,
inguinal hernia, and urethral stricture.

B. Radiation Therapy: Doses of 76 to 80 Gy or more have been shown to improve cancer control.
a External beam radiotherapy of 6800-7000 rads to prostate and 4500-5000 rads to pelvic nodes.
b: Intrestitial implants: I 125 is used to deliver high dose (10,000 to 17,000 rads) to prostate without
damaging surrounding tissue.

The outcomes of radiation therapy corrected for anatomic disease extent and other prognostic factors have been
reported to be roughly comparable to those of radical prostatectomy.
Complications of radiothery are: Inestinal sequelae (rectal bleed, tenesmus, mucous discharge, diarrhoea, fecal
incontinence, intestinal obstruction, and rectal stricture), Urological (frequency, dysuria, cystitis, hematuria,
and urethral stricture, and recto-vesical fistula) and other rare complications like; impotency, pedal edema.
Approximately one third of patients experience acute symptoms of proctitis or cystitis during the course of
radiotherapy, usually after the dose exceeds 50 Gy.
A prior transurethral resection of the prostate is a relative contraindication to brachytherapy and external beam
radiation therapy.
Another relative contraindication is inflammatory bowel disease.
Combined External Beam Radiation Therapy and Hormone Therapy for Locally Advanced Prostate Cancer:
patients with high PSA value, high Gleason score, or large-volume tumor benefit from androgen deprivation
therapy in conjunction with radiotherapy, whereas those with lower risk tumors do not.

Cancer control After Radiotherapy: Followed up by PSA:

Declining PSA to leass than 0.5 ng/ml.

Patients with detectable PSA (>0.1 ng/mL) after radical prostatectomy usually have persistent cancer.
The level of PSA nadir achieved following radiation therapy reflects the pattern of failure. Specifically, a nadir
greater than 2 ng/mL is associated with distant failure.
The PSA doubling time following radiation therapy also predicts the pattern of failure. Specifically, a PSA
doubling time less than 3 months is almost always associated with distant failure.
Phoenixdefinition: Rise in PSA > 2ngm/ ml higher than lowest PSA achieved.

C. Hormone manipulation:
1. Estrogen: DES has comparable efficacy with orchidectomy, but complication rate is higher.
2. Orchidectomy.
3. LHRH agonist: Complications are like DES e.g. hot flashes, gynacomastia etc.
******ebook converter DEMO Watermarks*******
4. Antiandrogens.Inhibitors of androgen synthesis includes aminoglutethimide, ketokonazole and spironolactone.
Ketoconazole is a P450 inhibitor, which inhibits both adrenal and testicular androgen synthesis. Side effects are
severe; GI intolerance, hepatotoxicity, gynacomastia and hypocalcemia. It is rapid acting and is useful in bone pains
or impending spinal cord compression.
D. Chemotherapy:
It is a relatively chemoresistanttumour. Some agents (e.g. adramycin, 5-FU) have shown some effects (about 10%
objective response). Suramin, by blocking growth factors (Beta FGF, EGF), direct cytotoxicity and
adrenocorticolytic activity has shown 40 % response rate.
E. Palliative therapy:
Painful Bone mets are managed with RT (2000-3000 rads). Strontium 89 (a beta emoting compound) has a affinity
for new bone activity and is effective in bone secondary. TURP is done to relieve outflow obstruction.
Newer drugs in prostate canceer metastasis: Cabazitaxel and Sipuleucel T.
Sipuleucel- T:
It is a cell-based cancer immunotherapy for prostate cancer.
A course of sipuleucel-T treatment consists of three basic steps:
A patient’s own white blood cells, primarily antigen-presenting cells (APCs), also called dendritic cells, are
extracted in a leukapheresis procedure.

The blood product is sent to a production facility and incubated with a fusion protein (PA2024) consisting of
two parts.
the antigen prostatic acid phosphatase (PAP), which is present in 95% of prostate cancer cells, and
an immune signaling factor granulocyte-macrophage colony stimulating factor (GM-CSF) that helps the
APCs to mature.
The activated blood product (APC8015) is returned from the production facility to the infusion center and re-
infused into the patient to cause an immune response against cancer cells carrying the PAP antigen.

A complete sipuleucel-T treatment repeats three courses, with two weeks between successive courses.

Wilm's Tumor:
Wilms tumor (WT) is the fifth most common pediatric malignancy and the most common renal tumor in children.
Incidence is approximately 0.8 cases per 100,000 persons.
Etiology: The tumor may arise in 3 clinical settings, (1) sporadic, (2) association with genetic syndromes, and (3)
familial. The etiology essentially remains unknown.
Sporadic Wilm's tumor:

******ebook converter DEMO Watermarks*******

 Beckwith-Wiedemann syndrome (macroglossia, gigantism, and umbilical hernia).
Hemihypertrophy.
Congenital aniridia.
WT, aniridia, genitourinary malformations, and mental retardation (WAGR syndrome).
Denys-Drash syndrome (WT, pseudohermaphroditism, and glomerulopathy).
Trisomy 18 mutation.

Pathophysiology: The pathophysiology of WT is characterized by an abnormal proliferation of the

metanephricblastema.
Clinical: The mean age at diagnosis is 3.5 years.

The most common feature at presentation is an abdominal mass.

Abdominal pain occurs in 30-40% of cases.
Other signs and symptoms include hypertension, fever from tumor necrosis, hematuria, and anemia.

Major congenital anomalies include genitourinary anomalies (WAGR and Denys-Drash syndromes- 5%); ectopic,
solitary, horseshoe kidney; hypospadias and cryptorchidism; hemihypertrophy&organomegaly (Beckwith-
Wiedemann syndrome- 2%); aniridia (1%).
Imaging Studies:
Ultrasound:

Initial diagnosis of a renal or abdominal mass, possible renal vein or inferior vena cava (IVC) thrombus.
Information regarding liver and other kidney.

Computed tomography scan:

Differential diagnosis of a kidney tumor versus adrenal tumor (neuroblastoma).

Liver metastases.
Status of opposite kidney.
Lymph node assessment.
Status of chest with respect to metastases.

Chest x-ray - As a baseline for pulmonary metastases.

Bone scan - Necessary for children with clear cell sarcoma of the kidney.
Magnetic resonance imaging:

Typically, these tumors appear inhomogeneous when using gadolinium-enhanced MRI, while the nephrogenic
rests (which sometimes are precursors of WT) appear as homogeneous masses.

Histologic Findings: WT arises from the primitive embryonal renal tissue and contains epithelial, stromal, and
blastemal elements.
Favorable histology (FH): 90% of cases. All 3 histological elements are present. The cure rate is close to 90%.
Occasionally, foci of cartilaginous, adipose, or muscle tissue may appear (ie, teratoid WT).
Unfavorable histology (UH): 10% of the cases. Focal or diffuse anaplasia, clear cell carcinoma of the kidney (bone-
metastasizing renal tumor of childhood), and rhabdoid tumor of the kidney are present.
Staging:

Stage I: The tumor is limited to the kidney and is excised completely.

Stage II: The tumor extends beyond the kidney but is excised completely. Capsular penetration, renal vein
involvement, and renal sinus involvement also may be found. A biopsy of the tumor is performed, and local
spillage occurs.
Stage III: Residual intra-abdominal tumor (nonhematogenous) exists after the completion of surgery. Lymph
******ebook converter DEMO Watermarks*******
 node findings are positive, or peritoneal implants are found. The resected specimen has histologically positive
margins, or the tumor has been spilled into the abdominal cavity.
Stage IV: Hematogenous or lymph node metastasis has occurred outside the abdomen or pelvis.
Stage V: Synchronous bilateral involvement has occurred. Each side is assigned a stage from I to III, and
histology is based on biopsy findings.

Treatment:
Surgical therapy: According to the NWTSG protocol, the first step in the treatment of WT is surgical staging
followed by radical nephrro-uretrectomy and regional lymph node dissection or sampling are performed (If the
disease is unilateral).
If bilateral disease is diagnosed, nephrectomy is not performed but biopsy specimens are obtained.
If the tumor is unresectable, biopsies are performed and the nephrectomy is deferred until after chemotherapy, which
will shrink the tumor in most cases.
Contiguous involvement of adjacent organs frequently is overdiagnosed.
With bilateral WT (5% of cases), surgical exploration, biopsies from both sides, and accurate surgical staging
(including lymph node biopsy of both sides) are performed. This is followed by 6 weeks of chemotherapy that is
appropriate to the stage and histology of the tumor. Then, reassessment is performed using imaging studies,
followed by definitive surgery with (1) unilateral radical nephrectomy and partial nephrectomy on the contralateral
side; (2) bilateral partial nephrectomy; and (3) unilateral nephrectomy only, if the response was complete on the
opposite side. This approach dramatically reduces the renal failure rate following bilateral WT therapy.
Postoperative details: Postoperative chemotherapy and radiotherapy protocols are based on the surgical staging and
follow the guidelines of the NWTSG.
Stage I FH and UH or stage II FH:

Nephrectomy.
Postoperative vincristine and actinomycin D (18 wk).

Stage II focal anaplasia or stage III FH and focal anaplasia:

Nephrectomy.
Abdominal radiation (1000 rad)
Vincristine, actinomycin D, and doxorubicin (24 wk).

Stage IV FH or focal anaplasia:

Nephrectomy.
Abdominal irradiation according to local stage.
Bilateral pulmonary irradiation (1200 rad) with Bactrim prophylaxis for Pneumocystis carinii.
Chemotherapy with vincristine, actinomycin D, and doxorubicin.

Stage II and stage IV diffuse anaplasia:

Nephrectomy.
Abdominal irradiation.
Whole lung irradiation for stage IV.
Chemotherapy for 24 months with vincristine, actinomycin D, doxorubicin, etoposide, and cyclophosphamide.

Prognosis: With the advent of multimodal therapy, the overall cure rate approaches 80-85%. Cases with diffuse
anaplasia and stage III or IV that recur in spite of the complex therapy have a bad prognosis.

******ebook converter DEMO Watermarks*******

******ebook converter DEMO Watermarks*******
Oesophagus:
Esophagus a 25 cm long tube, originates at the level of the C6, posterior to the cricoid cartilage and ends at T11 after
terminating at fundus. About 2–4 cm of esophagus is below the diaphragm.
Three anatomic areas of narrowing occur in the esophagus:
1. At the level of the cricoid cartilage (upper esophageal sphincter) at C6,
2. In the mid thorax, from compression by the aortic arch and the left main stem bronchus.
3. At the level of the esophageal hiatus of the diaphragm (lower esophageal sphincter).
The distance from upper incisor teeth.

to the cricopharyngeus muscle is 15–20 cm.

to the aortic arch, 20–25 cm.
to the inferior pulmonary vein, 30–35 cm.
And to the gastroesophageal junction, approximately 45 cm.

Cardinal Rules for Cardinal Symptoms of Esophageal Pathology:

Dysphagia:

Assume malignancy until proven otherwise.

Obtain barium swallow to rule out structural abnormality (tumor, stricture, hernia, diverticulum).
Perform upper GI endoscopy and obtain a biopsy sample to detect malignancy, esophagitis, Barrett’s
******ebook converter DEMO Watermarks*******
 metaplasia.

Chest pain:

Rule out cardiac causes (EKG, stress EKG, thallium scan, etc.).
Perform esophageal manometry to detect motility disorder.
Perform 24-h pH monitoring to correlate acid reflux with chest

Pain:

Perform Bernstein acid perfusion test in selected patients.

Heartburn:

Perform upper GI endoscopy and obtain a biopsy sample toassess esophagitis and presence of Barrett’s
metaplasia and dysplasia.
24h pH monitoring to assess frequency of reflux and correlation with heartburn.

Points to Remember:
3 points of constriction in Ba swallow:
15 cm from inscisor, at C6, at crycopharynx.
25 cm from inscisor, at T4 (Arch of aorta and “Left bronchus”).
40 cm , at T10 (diaphragm).

LES pressure- 10-25 cm (Measured by Dent Sleeve).

Esophageal Diverticula:
The three most common sites of esophageal diveticulum are:
1. Pharyngoesophageal (Zenker’s).
2. Parabronchial (midesophageal).
3. Epiphrenic (supradiaphragmatic- lower).

True diverticula(also known as traction diverticula) involve all layers of the esophageal wall, including mucosa,
submucosa, and muscularis.
Traction, or true, diverticula result from external inflammatory mediastinal lymph nodes adhering to the
esophagus as they heal and contract, pulling the esophagus during the process usually seen in mid-esophagus.
A false diverticulum(also known as Pulsion diverticulae) consists of mucosa and submucosa only. Pulsion
diverticula are false diverticula that occur because of elevated intraluminal pressures generated from abnormal
motility disorders.
These forces cause the mucosa and submucosa to herniate through the esophageal musculature.
Both a Zenker’s diverticulum and an epiphrenic diverticulum (Lower) are pulsion diverticula.
Zenker’s diverticulum (also known as cricopharyngeal achalasia) is due to increased pressure in the
oropharynx during swallowing against a closed upper esophageal sphincter.
Zenker diverticula are an acquired pulsion-type of diverticula in the posterior hypopharynx from a defect
in the muscular wall, between the inferior pharyngeal constrictor muscle (thyropharyngeus) and the
cricopharyngeal sphincter (Killian triangle).
It usually presents in older patients in the 7th decade of life and as the diverticulum enlarges, the mucosal
and submucosal layers dissect down the left side of the esophagus into the superior mediastinum,
posteriorly along the prevertebral space.
Retention of undigested food in large diverticula occasionally results in regurgitation (of undigested old
putrified food), nocturnal cough, halitosis and aspiration pneumonia.
Most common complaint is Dysphagia (Intermittent).
Most common complication is “lung abscess”.
******ebook converter DEMO Watermarks*******
Investigations:

Diagnosis of Zenker diverticulum is made best by barium swallow.

Chest x-rays and CT scans may also manifest large diverticula as air or fluid-filled structures communicating
with the esophagus.
Endoscopy, in the presence of a cricopharyngeal diverticulum is potentially dangerous with the risk of
diverticular perforation. (cricopharynx or upper esophagus is the most common site of perforation in Upper GI
endoscopy).

Treatment:
Medical Care is only partially successful and only directed to the motility disorder when feasible e.g.pneumatic
dilation, botulinum toxin injection into esophageal sphincter.
Surgical Care: Treatment of Zenker diverticulum is surgical.
Surgical options include:
1. Diverticulectomy with cricopharyngeal myotomy (Postoperative complications include fistula formation, abscess,
hematoma, recurrent nerve paralysis, difficulties in phonation, and Horner’s syndrome).
2. Diverticular suspension (diverticulopexy) with cricopharyngeal myotomy.
3. Cricopharyngeal myotomy.

If the diverticulum is small (<2 cm), resection may be unnecessary or just botulinum Toxin may suffice.
Larger diverticula (2–4cm) may be suspended upside-down through sutures to the retropharyngeal fascia after
the myotomy (Diverticulopexy).
Diverticula larger than 4 cm are best excised using a stapler following myotomy.

4. Dohlman’s procedure: Effective for large diverticula (> 3 cm).

Is an endoscopic procedure.
A double-lipped oesophagoscope is used.
Wall between the diverticulum and oesophageal wall is exposed.
Hypopharyngeal bar divided with diathermy or laser.

Points to remember:
Acquired pulsion diverticulum, old males, left side, through Killians dehiscence.
Defect in hypopharynx (midline posteriorly) but sweeling in left neck.
Commonest sympt- Dysphagia> Regurgitation.

******ebook converter DEMO Watermarks*******

 Commonest complication- aspiration pneumonitis> lung abscess.
IOC- Barium swallow.
Treatment- Cricopharngomyotomy + diverticulectomy.

Gastroesophageal Reflux Disease:

Pathophysiology:

The lower esophageal sphincter (LES) must have normal length and pressure and a normal number of
episodes of transient relaxation.
The GE junction must be located in the abdomen so that the diaphragmatic crura can assist the action of the
LES functioning. (The presence of a hiatus hernia disrupts this synergistic action and can promote reflux).
Esophageal clearance (motility) must be able to neutralize the acid refluxed (mechanical clearance is by
peristalsis; chemical clearance is due to saliva).
The stomach must empty properly.

A functional (frequent transient LES relaxation) or mechanical problem of the LES (hypotensive LES) is
the most common cause of GERD. Certain foods (coffee, alcohol), medications (calcium channel blockers,
nitrates, β blockers), or hormones (progesterone) can decrease LES pressure. Obesity is a contributing factor.
Physiologic reflux in normal subjects is more common when they are awake and in the upright position than
during sleep in the supine position.

History:
Typical symptoms include the following:

Heartburn/ Regurgitation/ Dysphagia:

Atypical symptoms include the following:

Cough and/or wheezing/ Hoarseness/ Chest pain:

Imaging Studies:
1. Barium esophagogram: It is particularly important for patients who experience dysphagia and shold be the first
investigation. A barium study before esophagoscopy can tell about potential danger of cervical vertebral
osteophyte, esophageal diverticulum, a deeply penetrating ulcer, or carcinoma.
2. Esophagogastroduodenoscopy: To decide presence and severity of esophagitis, barrett esophagus and also
excludes the presence of other diseases like peptic ulcer.
Other Tests:
3. Esophageal manometry.
******ebook converter DEMO Watermarks*******
 This study defines the function of the LES and esophageal body (peristalsis).

4. Ambulatory 24-hour pH monitoring: Measured 5cm above the upper border of LES.

This test is the criterion standard for diagnosis of GERD, with a sensitivity of 96% and a specificity of 95%.
A Demeester score is derived based on the frequency of reflux episodes and the time required for the
esophagus to clear the acid.

5. Radionuclide measurement of gastric emptying.

Medical Care:
Lifestyle modifications include the following:

Lose weight (if overweight). Avoid alcohol, chocolate, citrus juice, and tomato-based products. Avoid large
meals. Wait 3 hours after a meal before lying down.
Elevate head of bed 8 inches.

Pharmacological therapy.

Antacids.
Histamine-2 receptor antagonists.
Proton pump inhibitors: used only when GERD has been objectively documented.
Prokinetic agents:

Complications:
1. Esophagitis:

The squamous epithelium responds to acid by summoning lymphocytes and neutrophils to the sub-epithelial
layers.
The resulting mucosal damage and loss of the surface squamous cells then promotes frank ulceration, fibrosis
and stricture formation.

Grades of Esophagitis:

Grade I esophagitis is, a as small, circular, nonconfluent erosions.

Grade II esophagitis is, a linear erosions lined with granulation tissue that bleeds easily when touched.
Grade III esophagitis is, a advanced stage, in which the linear erosions coalesce into circumferential loss of
the epithelium and the mucosa may take a “cobblestone” appearance.
Grade IV esophagitis is, the presence of a stricture.

2. Barrett’s esophagus:

The chronic regurgitation of gastric contentscauses replacement of squamous mucosa of the esophagus by
numerous goblet cells, the so called intestinal metaplasia (MCQ).
Over several years, it will evolve into a true dysplasia and then to adenocarcinoma (50% risk of malignancy
reported with intestinal metaplasia).

Non-surgical eradication of Barrett’s Esophagus:

Laser ablation of the unwanted columnar intestinal metaplasia can be accomplished in 60-80 % cases

Treatment options include:

Surveillance endoscopy.
Antireflux surgery with or without continued surveillance endoscopy.
Ablative therapy, endoscopic mucosal resection, and esophageal resection.
******ebook converter DEMO Watermarks*******
 In general, gastroenterologists advocate aggressive surveillance programs with high-dose acid suppression, and
surgeons advocate antireflux surgery to correct the dysfunctional LES.
Yearly surveillance endoscopy is recommended in all patients with a diagnosis of Barrett’s esophagus,
regardless of the length of the segment.
Surveillance be extended to every 2 to 3 years for individuals in whom there is no evidence of dysplasia
on two consecutive yearly endoscopic exams.
For patients with low-grade dysplasia, surveillance endoscopy is performed at 6-month intervals for the
first year and then yearly thereafter if there has been no change.
Studies have demonstrated regression of metaplasia to normal mucosa up to 57% of the time in patients
who have undergone antireflux surgery.
Furthermore, antireflux surgery encourages regression of low-grade dysplasia to intestinal metaplasia, or
Barrett’s esophagus though adequate surveillance is not possible after a fundoplication.
Ablative therapy is proposed mostly for patients with high-grade dysplasia.
Photodynamic therapy (PDT) is the most common ablative method used.
Complications include persistent metaplasia in more than 50% as well as esophageal strictures in up to 34% of
patients.
Endoscopic mucosal resection (EMR) is gaining favor for the treatment of Barrett’s esophagus with low-grade
dysplasia(remember: till present : endoscopic follow up is the standard protocol for LOW grade dysplasia)
Because of an increase in stricture rate with larger resections, it is not advocated for long-segment Barrett’s
esophagus.
It is acceptable in patients with high-grade dysplasia who are not fit candidates for esophageal resection or have
an isolated focus of Barrett’s with dysplasia.

Esophageal resection for Barrett’s esophagus is recommended only for patients in whom high-grade dysplasia is
found. Pathologic data on surgical specimens demonstrate a 40% risk for adenocarcinoma within a focus of high-
grade dysplasia.
Surgical Care:
Indications for fundoplication include the following.

Patients not controlled by proton pump inhibitor.

The presence of Barrett esophagus with high grade dysplasia or adenocarcinoma is an indication for surgery.
Symptoms like (1) respiratory manifestations (cough, wheezing, aspiration); (2) ear, nose, and throat
manifestations (hoarseness, sore throat, otitis media); and (3) dental manifestations (enamel erosion).

Points to remember:
Classical triad – Heart burn (commonest symptom), epigastric pain, regurgitation.
Complications- Ulceration, stricture, Barrett’s.
LES plays primary role in preventing reflux.
Increased abdominal pressure is protective.
Gold standard test is 24 hrs pH monitoring – (DeMeester score <1 4.7)
Nissen Fundoplication is treatment of choice.

Hiatus Hernia:
Types:

TYPE I or sliding hiatal hernia (90%).

TYPE II or paraesophageal hernia or rolling (<5%).
TYPE III or Mixed(combination of I and II) (>5%).
Type IV.
congenitally short esophagus (not a true hernia).

******ebook converter DEMO Watermarks*******

Associated with:
1. Diverticulosis (25%).
2. Gallstones (18%).
3. Esophagitis (25%).
4. Duodenalulcer (20%).
(Saint’s triad: Hiatus hernia + diverticulosis + Gall stones).

Sliding Hiatal Hernia:

******ebook converter DEMO Watermarks*******
Also known as “Axial hernia,” or “Concentric hernia”:

Gastroesophageal junction herniates through the hiatus.

It is the mc type.
Not a true paraesophageal hernia.
Incidence: increases with age.
Presentation is reflux.

Findings:

UGI: Epiphrenic bulgedistance between B ring and hiatal margin > 2cmtortuous esophagusgastroesophageal
reflux 6 thick gastric folds within suprahiatal pouch.
CT: Dehiscence of diaphragmtic crura > 15 mm.
Pseudomonas within/above distal esophagus.
Fat (omemtum) surrounding distal esophagus.

Paraesophageal Hiatal Hernia:

“Rolling hiatal hernia,”“parahiatal hernia”

5% of hiatal hernias.
Portion of stomach superiorly displaced into through hiatus with the esophagogastric junction remaining in the
subdiaphragmatic position.

Findings:

Herniation of portion of the stomach anterior to esophagus.

Frequently nonreducible, may be associated with gastric ulcer of lesser curvature at level of diaphragmatic
hiatus.

Symptoms and Signs:

A paraesophageal hiatus hernia is generally asymptomatic but may present with pain and dysphagia, or it may
incarcerate and strangulate.
Occult or massive GI hemorrhage may occur is more common in rolling type.

Ischemic ulcer in rolling type seen in fundus is called: “CAMERON ulcers”

Diagnosis and Treatment:

X ray chest: RETRO-CARDIAC air fluid level in rolling type.

A barium study is IOC (especially in trendelenberg position).

Endoscopy:

Hiatal hernia is diagnosed easily using upper gastrointestinal endoscopy and it also permits biopsy of any
abnormal or suspicious area.

The operation is indicated in:

1. Patient who have > 10 yrs life expetancy and need and require chronic life long therapy or non responders.
2. Patients with respiratory symptoms (cough, asthma, aspiration pneumonia, pulmonary fibrosis).
3. Patients with vocal cord damage, and
4. Patients with Barrett esophagus. (Recent evidence suggests that an effective antireflux operation may promote
regression of the columnar epithelium in up to 50% of patients who have a short segment of Barrett esophagus < 3
cm).

******ebook converter DEMO Watermarks*******

Surgery:
A laparoscopic Nissen fundoplication (360°) is considered today the procedure of choice.
1. Dissection of the esophagus and make lower 3–4 cm of esophagus free below the diaphragm.
2. Division of the short gastric vessels in order to create a “floppy” fundoplication.
3. Approximation of the esophageal crus to decrease the size of the esophageal hiatus (Allison repair).
4. Construction of a 360° fundoplication over a 56–60 French bougie.
Gas bloat syndrome (difficult belching) and occasional dysphaga are two complicaions (10%) of Nissen’s
fundoplication.
Partial fundoplication whether performed posteriorly (Toupet) or anteriorly (Dor, Watson), has fewer short-term
side-effects.

Surgeries in nutshell:
******ebook converter DEMO Watermarks*******
1. Nissen’s Fundoplicaion: 360 degree wrap.
2. Belsay maak IV- 270 degree posterior wrap.
3. Toupet- 270 degree post wrap.
4. Dor is 180 degree anterior.
5. Watson- 90 degree anterior.
6. Allison-Narrowing the crus of diaphragm.
7. Collis Gastroplasy: Done for short esophagus (Aquired shortening due to refulx or scleroderma).

Points to rememebr:

Most comon type Sliding 90% > mixed > rolling.

Slding hernia presents with GERD (heart burn) while rolling presents with Pain and dysphagia.
Only symptomatic and large sliding hernias are operated while rolling is always an indiaction for suergry.
Rolling is more common in females.
Ba meal is investigation of choice.

Oesophageal Perforation:
Causes, Incidence, and Sites of Esophageal Perforation:
1. Iatrogenic (54%).

Instrumentation (44%).
Intraoperative (8%).
Radiation, sclerotherapy, and other therapies (2%).

2. Traumatic (19%).

Penetrating.
Blunt.
Ingestion of caustic substance.

3. Spontaneous, also known as Boerhaave syndrome (16%).

4. Ingestion of foreign body (7%).
5. Tumor (4%).
Boerhaave Syndrome:

Boerhaave syndrome is a spontaneous transmural perforation of the esophagus resulting from a sudden rise in
intraluminal pressure caused by an uncoordinated act of forceful vomiting against a glottis.
> 90% of perforations seen in the left posterolateral wall of the lower 1/3rd of esophagus.
The syndrome can also occur after other spontaneous Valsalva-like maneuvers, such as childbirth, coughing,
straining during a bowel movement, or heavy lifting.
After repeated episodes of retching and vomiting patient feels a sudden onset of severe chest pain in the lower
thorax and upper abdomen (MCQ).
The pain typically radiates to the back or left shoulder as a result of mediastinitis.
Other symptoms are neck pain, dysphagia, odynophagia, respiratory distress, and fever.
O/E, nonspecific findings may be present: tachycardia, diaphoresis, fever, hypotension, and generalized
abdominal tenderness with guarding and rebound.
The Mackler triad seen in Boerhaave syndrome comprises of:
Vomiting.
Lower chest pain.
Subcutaneous emphysema (50%).
Hamman’s sign (Hammond’s sign or Hammond’s crunch) is a crunching, rasping sound, synchronous with
the heartbeat, heard over the precordium in spontaneous mediastinal emphysema produced by the heart beating
******ebook converter DEMO Watermarks*******
 against air-filled tissues.

Investigations:
1. CXR:

left pleural effusion, (remember: this is an important clue on backgroung of vomiting and chest pain).
mediastinal widening, or free mediastinal air.
Radiographic signs of pneumomediastinum are-
pneumopericardium.
continuous diaphragm sign.
continuous left hemidiaphragm sign.
Naclerio’s V sign.
V sign at confluence of brachiocephalic veins.
ring-around-the-artery sign.
thymic spinnaker-sail sign.
extrapleural air sign.

2. Water-soluble contrast esophagraphy (IOHEXOL > BARIUM)to reveal the location and extent of
extravasation IS THE INVESTIGATION OF CHOICE.
3. If a leak is not detected, the barium swallow test or endoscopy may be required.
Endoscopy has a limited role, as small tears are difficult to visualize on this study.
In addition, the insufflation of air required for the procedure can result in the extension of the perforation.
Treatment:

Early detection and surgical repair within the first 24 hours results in 80% to 90% survival; after 24 hours,
survival decreases to less than 50%.
Initial management includes- NPO, administration of broad-spectrum antibiotics, fluid resuscitation,
nasogastric decompression.
Depending on the location of the tear, a chest or abdominal approach to repair the perforation is performed, and
parenteral nutrition is required.
Since the perforation is usually on the left side of the lower esophagus, the lesion can be visualized through a
left-sided thoracotomy.
Early operation to close perforation with reinforcement (within 24 hours).
(pleura, omentum, stomach) and establish adequate drainage.
Establish enteral or parenteral nutrition
Late perforation: After 24 hours.
Options: Simple drainage, debridement, esophageal exclusion, esophagostomy (for saliva drainage),
feeding jejunostomy and delayed repair by Colonic interposition.
The pathophysiology of Boerhaave’s syndrome closely resembles that of Mallory-Weiss syndrome (occurs
after persistent retching), which is characterized by upper gastrointestinal bleeding due to a mucosal tear (no
perforation) at the esophago-gastric junction.
Forceful vomiting may lead to either entity, but in Boerhaave’s syndrome, the tear extends through all layers of
the esophageal wall and in Mallory-Weiss syndrome it is partial thickess tear.

******ebook converter DEMO Watermarks*******

 Points to remember:

Barotrauma, Vertical tear, commonest site of tear- left posterolateral, commonest space of tear- Pleural
space.
Symptom- Mackler’s triad.
Sign- Hamman’s Crunch.
Xray- Continuous diaphragm sign and Naclerio V sign.
Immediate surgery (<24 hours) and repair.

Rings and Webs

Vascular Rings and Pulmonary Artery Slings:

The most common aortic arch anomaly ring is when the right subclavian artery (MCQ) arises from the
descending aorta and travels behind the esophagus (partial ring) called dysphagia lusoria.
Anomalous formation of a right aortic arch with a left ligamentum arteriosum and a resultant retroesophageal
left subclavian artery will form a complete ring that will also cause posterior esophageal compression.
A pulmonary artery sling is anomalous left pulmonary artery arising from the right pulmonary artery instead of
from the main pulmonary artery trunk. Then it traverses between the trachea and the esophagus and causes
significant anterior compression of the esophagus.
Both vascular rings and pulmonary artery slings cause dysphagia. Recurrent respiratory infections and
difficulty breathing are also common symptoms.
Aberrant right subclavian anomalies cause mild dysphagia to solids but not liquids.
This radiographic study will reveal extrinsic anterior or posterior compression of the esophagus.
IOC- high-resolution contrast CT (HRCT).

Treatment:
In symptomatic patients, both vascular rings and pulmonary artery slings are repaired. Asymptomatic patients with
aberrant right subclavian artery anomalies need not undergo repair. Pulmonary artery slings all require repair so as to
avoid narrowing of the left pulmonary artery and tracheal stenosis that develop with time. The results of surgery are
usually good, and the dysphagia resolves nearly 100% of the time.

Points to remmeber:
Most commonvascular compression is Dysphagia lusoria (Aberrent right subclavian artery).
Dysphagia Aortica is compession of esophagus by Thoracic aortoc aneurysm.
Ortner’s syndrome also called cardio-vocal syndrome associated with recurrent laryngeal nerve palsy
caused by compression of dilated left atrium due to mitral stenosis.
******ebook converter DEMO Watermarks*******
 Kommerell diverticula is outpouching of descending aorta at the origin of aberrant right subclavian artery
(which is aberrant and arising from descending aorta).

Oesophageal Webs
Webs may be congenital or acquired:

Congenital webs are rare, found in young children and more commonly are found in the lower two thirds of the
esophagus. Esophageal webs are thin, membranous structures.
Acquired esophageal webs are more common and are usually found in the cervical esophagus at postcricoid
area.
They are covered on both sides with squamous epithelium and are usually a thin mucosal fold and frequently
asymmetrical.
These webs are seen in patients with Plummer-Vinson or Paterson-Brown-Kelly syndrome (edentulous,
middle-aged, malnourished women with atrophic oral mucosa, glossitis, spoon-shaped fingernails, and iron
deficiency anemia), pemphigoid, and ulcerative colitis. They are also associated with a slight increase in
squamous cell cancer of the esophagus.

Treatment:
Acquired webs regress spontaneously with treatment of the anaemia.
But congenital and refractory web requires treatment. Thin webs are treated with membranous disruption through an
endoscope or bougie. Balloon dilation and Laser lysis has gained popularity in the recent decade. Surgical mucosal
resection is reserved for patients with thick rings that are refractory to bougienage.
Oesophageal Ring:
Two types of oesophageal ring have been described.
1. The Schatzki ring is a symmetrical, submucosal, fibrous thickening, at the squamo-columnar junction at the
lower end of the oesophagus. It consists of esophageal mucosa above and gastric mucosa below, with variable
amounts of muscularis mucosae, connective tissue, and submucosal fibrosis in between. It does not have a
component of true esophageal muscle, nor is it associated with esophagitis (Unlike peptic ulcer, here no ulcer is
seen in mucosa). It is often accompanied by a small hiatal hernia. The ring is present above the diaphragmatic
indentation.

Main presentation is dysphagia is usually to solid foods only and comes on abruptly with nearly complete
obstruction (often called episodic aphagia).
Diagnosis of a Schatzki’s ring is made with a barium study in prone position.
An endoscopy is indicated if the patient presents with foreign body obstruction or if the barium esophagram is
equivocal.

Treatment:

Incidentally found asymptomatic patients require no treatment.

Patients presenting with acute obstruction require immediate attention.
Administration of oral papain 2.5% solution as proteolytic digestion of impacted protein food.
Intravenous (IV) meperidine (25-50 mg) may also be used in small doses to encourage spontaneous
dislodgment of the impacted food bolus.
Esophagoscopy, either rigid or flexible to safely extract impacted food.
In symptomatic patients treatment is disruption of the ring by bougienage.
Surgery can cause devastating esophageal strictures thus Surgical intervention is reserved for patients who fail
or have intractable reflux.
In these few circumstances, intraoperative bougienage followed by a Nissen fundoplication is recommended,
but excision of the ring is not indicated.

2. The other type of ring occurs just proximal to the site of the Schatzki ring, at the junction of the distal oesophagus
******ebook converter DEMO Watermarks*******
and the uppermost part of the LES and is thought to be muscular.
Manometrically, this corresponds to a high pressure zone and is frequently associated with oesophageal motor
disorders and diffuse oesophageal spasm.

Points to remember:
Schatzki ring (B-ring)- is at lower esophagus involving mainly submucosa (and mucosa but not muscle).
Its at Squamo-columnar junction, associated with small hiatus hernia.
It is either asymptomatic or presents with mild to moderate episodic dysphagia only for solids.
Generally Balloon dilatation suffices.

Motility Disorder:

Achalasia of the Cardia:

The incidence is 6 per 100,000 persons per year.

The term achalasia cardia is a disorder of esophageal motility characterized by
Impaired relaxation of the lower esophageal sphincter.
Decreased or absent peristalsis of the esophageal body.
Increased pressure in the esophagus.

Allgrove syndrome is:

Achalasia cardia.
Alacrimia.
ACTH resistant Adrenal insufficiency.
Pathogenesis: Selective loss of Inhibitory Myenteric ganglion in Auerbach’s plexes (that normally scretes VIP and
Nitric oxide).
Achalasia can be divided into primary and secondary forms.

Primary achalasia is due to loss of ganglion cells in the myenteric plexus of Auerbach (more common).
Secondary achalasia, which may be due to malignancy, diabetes, or Chagas disease (Trypanosoma cruzi).
The commonest age range at presentation is 25-50 years, with no predilection for sex.
Patients with this condition have an increased incidence of malignancy; approximately 5% developing
squamous cell carcinoma, usually in the mid-esophagus (MCQ- not in lower narrowed part).
Dysphagia is the cardinal symptom (More for liquids). Regurgitation, weight loss, and chest pain or discomfort
are other symptoms.
Classical triad of Achalasiais: Dysphagia, regurgitation and weight loss (Patients with achalasia may have
chronic aspiration pneumonia involving Mycobacterium fortuitum-chelonei.).

A subgroup of patients with otherwise typical features of classic achalasia has simultaneous contractions of their
esophageal body that can be of high amplitude. This manometric pattern has been termed vigorous achalasia, and
chest pain episodes are a common finding in these patients.

******ebook converter DEMO Watermarks*******

Investigations:
1. Initial examination may be chest radiography, which can demonstrates

A homogeneous, usually right-sided, paramediastinal soft-tissue opacity.

Mediastinal widening/ air-fluid levels/ absence of a gastric air bubble (due to a water-seal effect)/
complications such as aspiration pneumonia or lung abscess.

2. Fluoroscopic barium swallow demonstrates failure of the contrast agent to enter the stomach when the patient is in
the recumbent position, nonpropulsive tertiary esophageal contractions (MCQ), various degrees of dilatation,
and the bird-beak sign
3. Manometry is the gold standardfor diagnosis. Typical achalasia has 5 classic findings:
a. The LES will be hypertensive with pressures usually above 35 mm Hg
b. It will fail to relax with deglutition
c. The body of the esophagus will have a pressure above baseline.
d. Simultaneous mirrored contractions with no evidence of progressive peristalsis.
e. Low-amplitude waveforms indicating a lack of muscular tone.
4. CT findings are nonspecific and insensitive, with esophageal dilatation usually present.

Symmetric wall thickening helps to distinguish achalasia from pseudoachalasia of malignancy, in which
mucosal irregularity or mass effect at the cardia is usually present.

5. Endoscopy can yield biopsy samples to exclude malignancy and permit direct visualization of esophagitis or
ulcers.
6. Mecholyl test is Hypersensitive (Not done now a days) Mecholyl test is to demonstrate “denervation
supersensitivity” in achalasia (Its not positive in Achalasia not in Hypertensive LES).
Therapeutic interventions includes:
1. Pneumatic balloon dilation (which is 70% effective with 5% perforation rate).

The initial success rate is between 70% and 80%, but it decreases to 50% at 10 years.
The perforation rate is 2–5%.
The incidence of postdilatation gastroesophageal reflux is about 25–35%.
Patients who fail pneumatic dilatation are usually treated by a Heller myotomy.

2. botulinum toxin injection (Injections of botulinum toxin (Botox) directly into the LES blocks acetylcholine
release, preventing smooth muscle contraction, and effectively relaxes the LES but symptoms recur more than 50%
of the time within 6 months and only 30% of patients have continued relief at 1-year follow-up).
3. Heller myotomy: modified laparoscopic Heller myotomy is now the operation of choice. The operation consists

******ebook converter DEMO Watermarks*******

of a controlled division of the muscle fibers (myotomy) of the lower esophagus (6 cm) and proximal stomach (2
cm), followed by an anterior or a posterior partial fundoplication to prevent reflux.The addition of a partial
antireflux procedure, such as a Toupet or Dor fundoplication can restore postoperative symptoms of reflux.
Drug therapies like calcium channel blockers and nitrates, are effective in a few patients (10%) and may be
tried in patients with contraindications to pneumatic dilation or surgery.

Points to remember:
Allgrove syndrome: Achalasia, Alacrimia, ACTH resistant Adrenal insufficiency.
Achalasia is failure of relaxation of LES
Classical triad is – Dysphagia, weight loss, regurgitation.
Onstruction is gradual so Maximum dilatation is seen in achalasia (Sigmoid esophagus).
IOC – Barimium swallow (bird beak sign). Gold standard: Manometry.
Treatment of choice: Heller’s Myotomy (Laparoscopic)+ antireflux surgery.

Diffuse Esophageal Spasm (DES):

In DES, coordinated esophageal peristalsis is lost and, a large segment of the esophagus contracts at once,
generating very high luminal pressures.
The distal half of the esophagus is often affected.
The primary symptom is severe spastic pain (squeezing retrosternal pain), normally at night. Dysphagia,
regurgitation, and weight loss are also common.
Both upper and lower sphincters function is normal and relax appropriately.
A significant association exists between DES and epiphrenic diverticulum, due to high intraluminal pressures
may contribute to the development of this pulsion-type diverticulum.
Gold standard test is manometry.
Barium swallow shows:
Cork screw or Rosarybead esophagus, segmental spasm or pseudo-diverituculam.
Tertiary contraction (on Fluoroscopy).
Manometry: Simultaneous multipeak contractions of high amplitudes (> 200 mm Hg) of long duration (>2.5
secs).
Medical Treatment with calcium channel blockers and nitrates can reduce the amplitude of the esophageal
contractions but usually is not beneficial.
Surgical treatment consists of a long esophagomyotomy, extending from the stomach to the aortic arch, and
often a concomitant antireflux procedure.

Nutcracker Esophagus:

******ebook converter DEMO Watermarks*******

The disorder, termed nutcracker or supersqueezer esophagus, hypertensive peristalsis or high-amplitude peristaltic
contractions is the most common of the primary esophageal motility disorders (MCQ).
It is characterized manometrically by prolonged, high-amplitude peristaltic waves associated with chest pain that
may mimic cardiac symptoms.
Unlike achalasia, LES relaxation is normal.

Manometry is diagnostic: showing peristaltic wave of pressure > 180 mmHg and duration more than > 6
seconds
Treatment with calcium channel blockers and long-acting nitrates has been helpful.
Esophagomyotomy is of uncertain benefit.

Patients may require treatment for GERD.

Hypertensive Lower Esophageal Sphincter:
In this condition LES pressure is above normal while esophageal body pressure may by hyperparistatic or normal.
Though LES pressrure is high but relaxation is Normal.
Esophageal body is hyperparistaltic or normal.
Treatment is:

Botulinium toxin injection for temporary relief.

Balloon dilatation for long term relief.
Modifid Heller’s myotomy for severe symptoms or when other modalities fail.

Esophageal Atresia and Tracheoesophageal Fistula

(EA and TEA):
The prevalence of EA or TEF is 2.6 to 3 per 10,000 births, with a slight male predominance.
Embryology:

The esophagus and trachea derive from the primitive foregut.

During the fourth and fifth weeks of embryologic development, the trachea forms as a ventral diverticulum
from the primitive pharynx (caudal part of the foregut).
A tracheoesophageal septum develops at the site and divides the foregut into a ventral portion, the
laryngotracheal tube and a dorsal portion (the esophagus).
Esophageal atresia results if the tracheoesophageal septum is deviated posteriorly.
This deviation causes incomplete separation of the esophagus from the laryngo-tracheal tube and results in a
concurrent tracheoesophageal fistula.
Esophageal atresia as an isolated congenital anomaly may occur due to failure of the recanalization of the
esophagus during the eighth week of development and is not associated with tracheoesophageal fistula.

******ebook converter DEMO Watermarks*******

Types:

Type C is the most common variantwhich consists of a blind esophageal pouch with a fistula between the
trachea and the distal esophagus- 85% (MCQ),.
The fistula often enters the trachea close to the carina.
The second most common anomaly is Type A ;pure atresia without tracheoesophageal fistula.

Associated Anomalies:

Polyhydroamnios is seen in 50%.

One third children have low birth weight.
Half to two third have anomalies can be remembered by the acronym VACTERL syndrome.
Vertebral: Mainly lumber vertebra.
Ano-rectal.
Cardiac- 20-25%. Most commonest (VSD > PDA > TOF).
Tracheo-esophgeal.
Renal.
******ebook converter DEMO Watermarks*******
 Limb- Radial Hyperplaisa>Hypoplasia (commonest) (ref:Schwartz ; 10th edi. Pg 1609).

Clinical Presentation:

Infants presents with excessive salivation and drooling.

Coughing, choking or cyanosis during the first oral feeding (MCQ).
A maternal history of polyhydramnios is often present.
Acute gastric distention due to air entering the stomach with each inspired breath.
Reflux of gastric contents into the distal esophagus will traverse the TEF and spill into the trachea, resulting in
cough, tachypnea, apnea, or cyanosis.

Diagnosis:

The inability to pass a nasogastric tube into the stomach of the neonate is a cardinal feature for the diagnosis of
EA (tube gets stuck at 10 cm- MCQ). If gas is present in the gastrointestinal tract below the diaphragm, an
associated TEF is confirmed.
On the other hand, inability to pass a nasogastric tube in an infant with absent radiographic evidence for
gastrointestinal gas is virtually diagnostic of an isolated EA without TEF.
For H type TEF most accurate is CECT.

Preoperative Evaluation and Management:

The immediate care includes decompression of the proximal EA pouch and will prevent spillover of oral
secretions into the trachea.
Bag-mask ventilation or positive-pressure ventilationis not appropriate since it may cause acute gastric
distention requiring emergency gastrostomy.
In these circumstances, manipulation of the endotracheal tube distal to the TEF (e.g., right main-stem
intubation) may minimize the leak and permit adequate ventilation.
Perform a thorough physical examination especially for VACTERL anomalies.
A preoperative echocardiogram is essential to evaluate the presence or absence of congenital heart disease as
well as to define the side of the aortic arch. A right thoracotomy is typically done for the repair of TEF in
patients with a normal left-sided aortic arch. However, for infants with a right-sided arch, a left
thoracotomy would be preferred, and a higher incidence of aortic arch anomalies (vascular rings) and
postoperative complications must be anticipated.

Surgical Management:
Surgical treatment involves an extrapleural thoracotomy through the right fourth intercostal space.
Gastrostomy for gastric decompression is reserved for use in patients with significant pneumonia or atelectasis, to
prevent reflux of gastric contents through the fistula and into the trachea.
Healthy infants without pulmonary complications or other major anomalies usually can undergo primary repair in
the first few day. The TEF is dissected circumferentially and then ligated using interrupted, nonabsorbable sutures.
The proximal esophageal pouch is then mobilized as high as possible to afford a tension-free esophageal
anastomosis (distal segment is more prone for ischemia with mobilization due to poor blood supply). The
anastomosis is performed using either a single- or double-layer technique. The rates of anastomotic leak are slightly
higher with the single-layer anastomosis, whereas the rates of esophageal stricture are higher with the double-layer
technique.
Fitness of the patient is determined by Waterston criteria (Weight and pneumonia):

Weight > 5.5 pounds and no Pneumonia- Can be operated as single stage thoracotomy.
Weight between 4-5.5 Pounds / no or minimal pneumonia- Should be made fit by IV fluid, and supportive
treatment prior to surgery.
Weight < 4 pounds or severe pneumonia – First do feeding gastrostomy then thoracotomy should be done after
few weeks.

******ebook converter DEMO Watermarks*******

Postoperative complications unique to EA or TEF include esophageal motility disorders, gastroesophageal reflux
(25%-50%), anastomotic stricture (15%-30%), anastomotic leak (10%-20%), and tracheomalacia (8%-15%).

Points to remember:
Most common type is proximal atresia with distal fistula, second most common is pure atresia.
Polyhydroamnios is 50%.
Commonest VACTERAL anomaly is Cardiac (VSD).
Symptoms- Excessive drool of saliva with aspiration on feeding.
Inability to pass NG tube beyond 10 cm first investigation.
Waterston criteria is used to decide whether single stage thoracotomy or first feeding gastrostomy should be
done.
Right Thoracotomy is preferred.

Caustic Ingestion:
Liquid alkali solutions cause most of the serious caustic esophageal and gastric injuries, producing coagulation
necrosis in both organs. Acid ingestion is more likely to cause isolated gastric injury.
Three Phases of Tissue Injury from Alkali Ingestion:

Endoscopic Grading and Treatment of Corrosive Esophageal and Gastric Burns:

Initial management:

Hemodynamic stabilization and evaluation of the airway and extent of injury.

Airway compromise can occur from burns of the epiglottis/ larynx requiring tracheostomy.
Fluid resuscitation and broad-spectrum antibiotics should be instituted.
Within the first hour of ingestion, neutralization is attempted. Alkalis (including lye) are neutralized with half-
strength vinegar or citrus juice. Acids are neutralized with milk, egg whites, or antacids.

******ebook converter DEMO Watermarks*******

 Emetics and sodium bicarbonate must be avoided as they increase the chance of perforation
Vomiting should not be induced.
Steroids are of no proven benefit.

Evaluation:
Water soluble contrast study and gentle Upper GIscopy should be done early to judge the severity and extent of
injury and to rule out esophageal perforation/ gastric necrosis.
Early investigation – Endospcoy (to assess severity)
IOC for caustic stricture- Ba swallow
Management:

Without perforation, management is supportive, with acute symptoms generally resolving over several days.
Perforation, unrelenting pain, or persistent acidosis mandate surgical intervention.
A transabdominal approach is recommended to allow evaluation of the patient’s stomach and distal esophagus.
If it is necrotic, the involved portion of the patient’s stomach and esophagus must be resected, and a cervical
esophagostomy must be performed.
A feeding jejunostomy is placed for nutrition, and reconstruction is performed 90 or more days later.
Late problems include the development of strictures and an increased risk of esophageal carcinoma (1,000
times that of the general population).

Esophageal Tumors:
Benign Esophageal Neoplasms:

The most common esophageal tumor is Leiomyoma (GIST followed by polyps. hemangioma and granular cell
myoblastoma are rare).
Leiomyoma are well encapsulated, single, oval and common in lower esophagus.
Esophageal GIST consititute 10% of GI GIST.
Leiomyoima are slightly more common in males and common in 4th or 5th decade.
Many leiomyomas are asymptomatic but may present with Dysphagia or pain.
IOC- Barium swallow – Smooth, well-definedpunched out defect, noncircumferential mass with distinct
borders.
Endoscopic biopsy is avoided because subsequent mucosal adherence to the mass increases the chance of a
mucosal perforation during surgical resection.

Treatment:
Treatment for all symptomatic or enlarging tumors is surgical removal (Enucleation).

Intraluminal tumors can be removed successfully via endoscopy, but if they are large and vascular, they should
be resected via thoracotomy and esophagostomy.
Intramural tumors usually can be enucleated from the esophageal muscular wall without entering the mucosa.
This is done via a video-assisted thoracoscopic or open thoracotomy approach. Laparoscopic resection may be
appropriate for distal lesions.

Esophageal Carcinoma:

Incidence of esophageal carcinoma is 15-20 cases per 100,000.

Overall esophageal carcinoma has a dismal 5 year survival rate of less than 10-15%. With surgical treatmentan
approximate 20%, or less may survive 5 years.
The male-to-female ratio for squamous cell cancer is 3:1.

Squamous cell carcinoma is still most common esophageal cancers (mailnly in mid esophagus):

******ebook converter DEMO Watermarks*******

 Adenocarcinoma is a disease affecting white men and lower esophagus (Adenocarcinoma is more common
with- Increasing incidence of GERD, Western diet, Increased use of acid-suppression medications), whereas
squamous cell carcinoma predominantly affects African American men.

Points to remember:
Progressive dysphagia, initially for solids and later for liquids.
Progressive weight loss.
Diagnosis established by endoscopy and biopsies.
Staging established by endoscopic ultrasound (best for T stage- depth), computed tomography of chest and
abdomen (for staging and operability), and positron emission tomography (18 FDG PET for mets).
Bronchoscopy indicated for cancer of the mid thoracic esophagus.

Risk Factors:

Cigarette smoking and alcohol drinking are the two major etiological factors. Incidences of heavy smoking and
heavy drinking combined, increases the risk from 25 to100 folds.
Diets low in beta-carotene, vitamins A, C, B, magnesium, molybdenum and zinc have been associated with
cancer. Also, reduced consumption of fruits, vegetables, fresh meat, fresh fish and dairy products resulted in a 2
fold increased incidence of esophageal carcinoma.
In addition, high level exposure to asbestos, ionizing radiation, and drinking exceptionally hot beverages has
also been found to be positively correlated.

Predisposing Conditions:
1. Tylosis.
2. Achalasia.
3. Caustic Injury.
4. Esophageal Webs (Plummer Vinson Syndrome).
5. HPV.

For adenocarcinoma: Barrett’s Esophagus, obesity, high fat diet and scleroderma.

Genetic alterations accounting for cellular and molecular changes (as in the p53 gene) have been associated with an
increased risk for esophageal cancer.
Lymphatic Drainage:

The lymphatic drainage of the esophagus is conducted by a vast network in the mucosal plexus which
communicates with a submucosal plexus.
These two then coalesce with lymph channels of the muscularis layer.
Because of the rich and complex lymphatic drainage, fluids from any part of the esophagus can travel to any
other portion of the esophagus.
Therefore, when resection of the tumor is carried out, ample distance from the actual tumor (10 cm)
must also be resected.
The lymph channels eventually drain into a number of lymph nodes: internal jugular, cervical, supraclavicular,
paratracheal, hilar, subcarinal, paraesophageal, para-aortic, paracardial, lesser curvature, left gastric, and celiac.

Normal Histology:

The esophagus consists of three layers: mucosa, submucosa, and muscularis.

The mucosa consists of epithelial lining containing nonkeratinizing, stratified squamous epithelium with a layer
of basal and parabasal cells.
Squamous cell carcinoma is derived from this layer.
The sublayer, lamina propria, contains vessels, connective tissue, lymphatics, inflammatory cells and
******ebook converter DEMO Watermarks*******
 esophageal cardiac glands which are mucus secreting glands.
Adenocarcinomas arise from these glands.
Without the serosa covering, neoplasms that arise in the esophagus can spread unimpeded to other tissues.

Squamous Cell Carcinoma (Epidermoid Carcinoma):

Squamous cell carcinoma has been the commonest cell-type of esophageal cancers. However, in the last
decade, the incidence of adenocarcinoma has increased at roughly 10% per year. It is no longer the leading
form of esophageal cancer in US white males.
Tumors of epidermoid carcinoma are located mainly in the thoracic esophagus. Neoplasms can be of four major
types:

1. Fungating-type: Predominantly intraluminal growth with surface ulceration and extreme friability. This type
frequently invades mediastinal structures.
2. Ulcerating-type: Characterized by a flat based ulcer with slightly raised edges; hemorragic and friable and
surrounding induration and erythema.
3. Infiltrating-type: A dense firm logitudinal and circumferential intramural growth pattern.
4. Polypoid: Intraluminal polypoid growth with a smooth surface on a narrow stalk. A five year survival of 70%
(best) is seen here as compared to less than 15% five year survival for other types.

Adenocarcinoma:
Adenocarcinoma has been the second most common cell type of esophageal cancer, but now is the leading cell type
of this type of cancer. Adenocarcinoma is derived from glandular tissue or tumor cells form recognizable glandular
structures.
Arises from:

Superficial and deep glands of the esophagus such as mucous glands.

embryonic remnants of glandular epithelium.
Metaplastic glandular epithelium.
Less common malignant esophageal tumors include small-cell carcinoma, melanoma, leiomyosarcoma,
lymphoma and esophageal involvement by metastatic cancer.

Symptoms of Esophageal Carcinoma:

The most common symptoms are dysphagia and weight loss.

Because of the elasticity of the esophagus, two-thirds of the lumen must be obstructed to produce dysphagia. .
Dysphagia is initially for solids but eventually it progresses to liquids.
Other symptoms: Vomiting or Regurgitation, Pain, Cough or Hoarseness usually due to invasion of the right or
left recurrent laryngeal nerves with paralysis of the ipsilateral vocal cord, Cachexia, Dyspnea.
Respiratory symptoms may be due to regurgitation and aspiration of undigested food or to invasion of the
tracheobronchial tree, with development of a tracheoesophageal fistula.

Diagnosis:
Chest radiograph:

Abnormal azygoesophageal recess.

widening of the mediastinum.
Posterior tracheal indentation.

A barium esophagram is recommended for any patient presenting with dysphagia and in cancer classic finding of
an apple-core (Classical lesion) lesion is recognized easily.
Endoscopy is 1st investigation and it determines the extent of the tumor and its location.
******ebook converter DEMO Watermarks*******
Endoscopy (IOC): The diagnosis of esophageal cancer is made best from an endoscopic biopsy. The accuracy of
brush cytology alone is about 85-97% and biopsy alone ranging from 83-90%.
The accuracy for the combination brush cytology and biopsy is 97-100%.
Computed Tomography: Used for accurate staging and to assess the length of the tumor, thickness of the
esophagus and stomach, regional lymph node status (including cervical, mediastinal, and celiac lymph nodes),
distant disease to the liver and lungs.
It is also helpful in determining T4 lesions where the lesion is invading surrounding structures. It may identify a
fistula or other anatomic variations such as a deviated trachea.
Although a CT scan is helpful, its accuracy is only 57% for T staging, 74% for N staging, and 83% for M staging. It
is most inaccurate for T stage as it usually over-stages the T stages.

A positron emission tomography (PET)- 18 FGD PET scan is best to assess Metastasis.
For evaluation of lymph node disease, PET has a accuracy (76%) on par with what CT can offer.
PET is now the best staging tool available.

Magnetic Resonance Imaging is not performed routinely and helps to identify involvement of vascular and neural
tissues. It is accurate for T4 lesions and metastatic lesions in the liver but it overstages T and N status.
Endoscopic Ultrasound:
Best to assess depth (T stage) of tumour:

EUS is best to identify the depth of the tumor, the length of the tumor, the degree of luminal compromise, the
status of regional lymph nodes, and involvement of adjacent structures.
In addition, biopsy samples can be obtained of the mass and lymph nodes in the paratracheal, subcarinal,
paraesophageal, celiac, lesser curvature, and gastrohepatic regions.
EUS tends to overstage T status and understage N status.
The accuracy of EUS for T staging correlates directly with increasing T stage. For T1 lesions, EUS is 84%
accurate, and it approaches 95% accuracy in estimating T4 lesions.

Endoscopic Mucosal Resection:

EMR is performed with a double-channel endoscope and may also be used as diagnostic or a therapeutic
modality for premalignant and early malignant conditions.
Endoscopic submucosal dissection (ESD) is a technique that uses hook cautery and scissors to resect a lesion
down to the level of the muscularis propria.

******ebook converter DEMO Watermarks*******

Staging:
Techniques in radiology, such as barium esophogram, CT, and magnetic resonance imaging (MRI) are often used as
a basis for clinical staging. EUS is the method of choice to determine depth of tumor invasion and regional nodal
disease and involvement of adjacent structures.
Tumor-Node-Metastasis (TNM) Staging of Esophageal Carcinoma:

******ebook converter DEMO Watermarks*******

Siewert classification: Siewert-Stein classification is anatomical classification of the location of Esophago-gastric
junction (OGJ) tumours.
Type I: adenocarcinoma of distal part of the oesophagus (centre located within between 1-5cm above the anatomic
OGJ)
Type II: adenocarcinoma of the real cardia (within 1cm above and 2cm below the OGJ)
Type III: adenocarcioma of the is subcardial stomach (2-5cm below OGJ)
Treatment:
85 to 95% of patients have lymph node involvement at the time of surgical resection and with lymph node
involvement, less than 10% survive five years.
Curative Treatment:
Approach to Cervical Tumors:

Most tumors of the upper esophagus above the level of the carina are squamous cell carcinomas.
Surgical excision with immediate reconstruction significantly improves survival over radiation therapy alone
for patients with upper esophageal tumors.
Tumors that do not invade the trachea, spine, larynx, or vessels are resected primarily.
Tumors upper esophagus or the larynx are treated with two to three cycles of chemotherapy and up to 3500 cGy
before surgical resection.
Squamous cell tumors are more sensitive to chemoradiotherapy and are treated aggressively with nonsurgical
therapy; adenocarcinomas are not as sensitive to chemoradiotherapy and are often imbedded in long segments
of Barrett’s esophagus, necessitating a more aggressive surgical approach.

Surgery:
Only 50% of patients can undergo a curative resection.
There are three main types:

A: Transthoracic Esophagectomy .
B: Transhiatal esophagectomy .
C: Tri Incisional esophagectomy.

Regardless of technique, because of the unusual lymphatic system of the esophagus, malignant cells can be found a
number of centimeters away (10 cm) from the primary lesion.
Therefore, when the esophagectomy is performed, a generous margin is included and lymph node dissection is
carried out.

Margin for mid esophagus- 10 cm on either sides. For lower esophagus- Proximal margin- 10 cm and distal 5

******ebook converter DEMO Watermarks*******

 cm

Transhiatal esophagectomy (THE)- (Orringer): Most common procedure today for early stages:

The transhiatal esophagectomy though not a typical “cancer” surgery, with a cervical gastroesophageal
anastomosis.
THE carries a low operative mortality of 2-6% and a low anastomotic leak rate of 5-8%.
ontraindications are surrounding structre involvement or fixity (pericardium, aorta).
Transhiatal esophagectomy is done through first an abdominal incision and then an cervical incision
allowing complete removal of the esophagus without a thoracotomy.

Transthoracic esophagectomy:

The transthoracic esophagectomy is considered the “standard”.

It allows a meticulous lymph node dissection, complete resection of tumor mass and adjacent tissue, and
appropriate staging of the tumor which allows for a better chance of cure.
Two of the main risks for this operation are a anastomotic leak and respiratory complications.
Because of the relative location of the esophagus within the mediastinum, a left side thoracotomy is performed
if the tumor in the distal third of the esophagus and a right side thoracotomy if it is in the middle and upper
thirds.

Ivor Lewis Esophagectomy:

Ivor Lewis esophagectomy is chosen for patients that have tumors of esophageal cancer of the middle and lower
third of the esophagus. Done through first aparotomy then right thoracotomy, then resection of cancer followed by
gastric pull up is done.
Total Thoracic Esophagectomy:
This procedure also begins with a laparotomy as do all esophagectomies to mobilize the conduit of choice. The
conduit, whether it be the stomach (best for cancer) or the colon, is placed retrosternally and a cervical anastomosis
is performed. A right thoracotomy is then made and the esophagus is resected.
En Bloc Esophagectomy: Mckeown Esophagectomy or Tri incisional esophagectomy.
Because many patients present with metastases to regional lymph nodes as well as to the surrounding tissue and
organs a more radical resection has been advocated; the en bloc esophagectomy. An envelope of normal tissue is
removed along with the spleen, celiac nodes, posterior pericardium, azygous vein, thoracic duct, and adjacent
diaphragm.
Thorascopic Esophagectomy:
This procedure is the only thoracotomy excluding technique that allows for a complete esophagectomy and a full
lymphadenectomy.
Vagal-Sparing Esophagectomy:
This technique varies from THE only in the method of removing the esophagus without severing the vagus nerves
facilitating a limited nodal dissection and is advocated for treatment of intramucosal tumors. The esophageal
resection is performed by stripping the esophagus away from the vagus nerves, performing a highly selective
vagotomy, and preserving the function of the pylorus so that a pyloroplasty is not needed.
Reconstruction After Esophagectomy:
The stomach, colon and jejunum have all been successfully used.

The stomach appears to be the conduit of choice because of its ease in mobilization and tremendous
vascular supply.
The colon is used if the patient has undergone a partial or total gastrectomy previously or if metastases has
spread to the stomach.
Jejunal loops has restricted mobility and possibly limited vascular supply soit is used for short segmen
******ebook converter DEMO Watermarks*******
 reconstruction.
Anastomosis can be performed in the chest just below the arch of the aorta (intrathoracic anastomosis), or the
rest of the esophagus can be resected and a cervical anastomosis can be performed in the neck.

Radiation Therapy:
External Beam Radiation:

External beam radiation therapy may be used alone in the treatment of esophageal carcinoma but is not
considered curative. For curative attempts, chemotherapy and/ or surgery generally accompanies this technique.
Used alone, there is a 5-10% 5 year survival.
Radiation therapy is contraindicated in the presence of a fistula or likely fistula formation.
The target includes a 5 cm margin on either side of the tumor. In addition to irradiating the tumor, lymph node
stations are irradiated as well to treat possible metastatic disease. The supraclavicular and celiac lymph nodes
are targets if the tumor is in either the upper or lower esophagus respectively.
The dosage of radiation used depends upon several factors. One is the choice of treatment.
Treatment can be given in a hyperfractionation (small fractions 2-3 times a day), accelerated fractionation
(normal-sized fractions given more than once a day), or conventionally (normal-sized fractions (180-250 rads)
once a day). The range is between 5000 cGy in 20 treatments over 4 weeks to 6600 cGy in 33 treatments over 7
weeks.
Definitive radiotherapy such as this commonly results in a median survival of less than 12 months and 5 year
survival of less than 20%.

Intracavitary Radiation
(Intraluminal Brachytherapy):

Intracavitary radiation is a technique that involves implanting a radiation source in or around the tumor.
Only used for small tumors as source delivers about 1000 cGy doses approximately 1-1.5 cm in diameter.
It is used most often as a boost before or after external beam radiation therapy.
Contraindications include stenosis, fistula, or deep ulceration.

Chemotherapy:
It is used more commonly in conjunction with radiation and/or surgery.
Chemotherapy is used as Neo-adjuvant or Adjuvant therapy either alone or combined with radiation to treat
micrometastases and to reduce the size of the tumor in order to improve resectability rates. Also, as palliate if
surgery is not an option.
Chemotherapy is typically given in a combination of two or more chemotherapy drugs. The most prescribed drug is
cisplatin.
It has been most commonly combined with 5-fluorouracil (5-FU), vindesine, or bleomycin.

Cisplatin and 5-FU is the most commonly prescribed combination.

Chemotheraputic agents fall into five descriptive categories based on activities, source, and resultant toxicities:
1. Antibiotics include bleomycin, mitomycin, idarubicin and amonafide, deoxorubicin (Adriamycin) and
methotrexate. The antibiotics’ side effects include pulmonary toxicity such as fibrosis and decreased carbon
monoxide diffusing capacity.
2. Antimetabolites include 5-fluorouracil, methotrexate, dichloromethotrexate, aminothidiazole, and trimetrexate.
Toxicity to the gastrointestinal mucosa and bone marrow increases with the dosage.
3. Cisplatin and carboplatin are heavy metals whose dosage related and potentially reversible side effects include
nephrotoxicity, ototoxicity, and peripheral neuropathy.
4. Plant alkaloids are vindesine, etoposide, taxol, and navelbine. They may cause myelosuppression,
hypersensitivity reactions, cardiac arrhythmias.
5. Ifosphamide is in the alkylating agent group. Other general side effects of chemotherapy agents include nausea,
******ebook converter DEMO Watermarks*******
 vomiting, alopecia, stomatitis and diarrhea.

Palliative treatment:
Palliation is appropriate when patients are too malnourished or debilitated to undergo surgery, have a T4 tumor,
recurrence of resected or irradiated tumor, and/or due to metastases. Most of these patients have impassible tumor or
painful swallowing.
Dependent upon the life expectancy, relief is carried out by surgery, radiotherapy with or without chemotherapy,
intubation, dilation, photodynamic therapy, and/or laser therapy.
Surgery:
Esophagectomy:
The entire esophagus or a region of the esophagus is removed. As an alternative conduit, the stomach, colon or
jejunum, may be used. This technique is preferable for low risk patients with good life expectency.
Bypass:
A palliative bypass may be useful when a tumor is unresectable and severe dysphagia or tracheoesophageal fistula
has occurred after radiochemotherapy. Tracheoesophageal fistula (TE) has a survival of weeks to months. Bypass
should be proposed for younger, healthier patients. A bypass can be performed by: presternal, or retrosternal routes.
The reterosternal route offers the most direct route to the neck.
Endothoracic Endoesophageal Pull-Through
The operation consists of stripping the esophagus of its mucosal layer and tumor and using the muscular tube of the
esophagus as a sleeve inside which the stomach is pulled through. The operation is less risky than a bypass and
achieves the same results. Normal swallowing and normal diet is achieved in almost 80% of the patients.
Radiotherapy:

External Beam Radiation.

For surgically unfit patients. Dysphagia is satisfactorily relieved in approximately 80% of the patients that
undergo radiation therapy.
However, the relief achieved from radiotherapy is many times short lived for abut 6 months.
Intracavitary Radiation.
Good for debilitated patients. Dosages ranging from 10-20 Gy with length of relief of dysphagia
increasing as the dosage increases.
Doses of greater than 20 Gy will cause severe damage to the esophagus and this level should not be
exceeded.

Laser Therapy:
A neodymium: yttrium-aluminum-garnet (ND:YAG) laser is used for small obstructive tumors of the middle to
lower thirds of the esophagus.
Photodynamic Therapy:

Malignant tumor cells have a unique vascular and lymphatic system and that the photosensitizer used will
absorb light and produce oxygen radicals.
A photosensitizer such as, dihematoporphyrin ether, is given intravenously.
After 2 or 3 days (40-50 hours) it is retained in the malignancies in a much higher concentration than in normal
tissue of the body.
Then, a low power laser light (620-630 nm) is delivered to the tumor by a flexible endoscope.
The photosensitizer absorbs the red light and produces oxygen radicals which destroys the tumor.
Two to three days after PDT, esophagoscopy is repeated and the necrotic tumor tissue is removed.

Stents – SEMS (SELF EXPANDING METALLIC STENTS): Palliative treatment of choice:

******ebook converter DEMO Watermarks*******

 Success rate 95% in palliation.
Duration of relief- 5-6 months.
10% complication rate (mainly stent migration and tmour ingrowth). Silicon and PU (Polyurethane) coating is
done to prevent tumor ingrowth.
Palliation of choice in malignant TE fistula.
Stents protruding above cricopharynx has poor tolerance and going below GE junction (by migration) causes
severe GERD and acid reflux.

The flexible self-expanding stent is made up of two layers of superalloy monofilament wire with a layer of silicon in
between. The addition of the polymer in between the layers of mesh wire is a relatively new addition that preventing
tumor overgrowth through the holes in the wire mesh.
Patients are placed under local or general anesthesia and the stricture is dilated to 42-45F using a flexible
gastroscope and Savary bougies. The lesion is marked and insertion of the stent is carried out under fluoroscopic
control. The procedure is very often successful, >90% and patients can begin the routine of eating normal foods.
Patients complain of chest pain in almost 100% of the cases because of the stretching of the stricture. Also
hematemesis and nausea are possible complications.

Points to remember:
SCC and most common esophagus cancr. Adeno Ca is common in lower esophagu in white males.
Dysphagia is a late yet first symptom (For solids).
First Investigation – Ba swallow (commonest finding – Apple core).
IOC- Endoscopy.
Best Investigation for depth or nodes (T and N stage)- EUS (Endoscopy USG).
Best for staging- PET-CECT.
Best for metastasis- 18 FDG PET scan.
Three surgeries.

1. Ivor Lewis esophagectomy (Best for mid or lower esophagus)- done by Laaparotomy followed by
thoracotomy. Gastric pull up done. Main cause of mortality is “Anastomotic leak”!
2. McKeown Esophagectomy- Three stage (laparotomy, thoracotomy then neck incision), Complete
esophagetomy done with gastric tube placement.
3. Orringer- (Trans-hatal esophagectomy)- Done by Laparotomy then neck incision. No thoracotomy done (so
less pulmonary complications). More chances of Anastomotic Leak and bleeding (Though Leak is more here
but mortlity due to leak is more in Ivor lewis).
Best Palliative treatment is- Self Expanding Stents (SEMS).

For malignant TE fistula – best palliation stents. Other modalities are Radio, Chemo, laser and
Photodynamic therapy. Submucosal resection (SMR) is for early stage cancer not palliation.
Best chemotherapy- Cisplatins.

Few more Esophageal points for MCQ:

Killian Jamieson Diverticula:

A pulsion type of acquired diverticulum below cricopharynx on left side.

Usually asymptomatic but may cause reflux and aspiration.
Ba swallow is the IOC.
Surgical excision is best treatment but only reserved for symptomatic patients!

Crow – Fukase syndrome:Also known as POEMS syndrome or Takatsuki disease:

It is defined as the combination of a plasma-cell proliferative disorder (typically myeloma), polyneuropathy,

and other organ systems.

******ebook converter DEMO Watermarks*******

 It begins in middle age and more common in males.

Main features: POEMS

Polyneuropathy (peripheral nerve damage).

Organomegaly (abnormal enlargement of organs).
Endocrinopathy (damage to hormone-producing glands) or Edema.
M-protein.
Skin abnormalities (including hyperpigmentation and hypertrichosis).
Treatments for demyelinating neuropathy, such as steroids, intravenous immunoglobulin and plasma
exchange, are ineffective;
treatment must be aimed at the haematological disorder.
Prednisolone and alkylating agents are the most commonly used.
Role of Bevacizumab (against VEGF) is documented but risk of Capillary leak syndrome is very high.

Diaphragm:
The diaphragm is a musculotendinous, dome-shaped structure attached posteriorly to L1 to L3 vertebrae, anteriorly
to the lower sternum and laterally to the costal arches.
It has 3 opening.
Through the aortic hiatus, at T12 level aorta, the thoracic duct, and the azygos venous passes through it.
The esophageal hiatus is immediately anteriorly and slightly to the left at the level of T10, is separated from the
aortic hiatus by the decussation of the right crus of the diaphragm. Through this hiatus pass the esophagus and the
vagus nerves.
At the level of the T8 and slightly to the right of the esophageal hiatus is the vena caval foramen, which allows
passage of the inferior vena cava and Right phrenic nerve.
The phrenic arteries arising from the aorta supply the diaphragm along with the lower intercostal arteries and the
terminal branches of the internal mammary arteries.
Diaphragmatic hernia:
Incidence: 1:2500
Two types:
1. Parasternal or Retrosternal (Foramen of Morgagni) Hernia - Right retrosternal anterior.
2. Pleuroperitoneal (Foramen of Bochdalek) Hernia (Congenial diaphragmatic hernia is used for Bochdalek hernia)-
Left posterolateral posterior.
Failure of fusion of the sternal and costal portions of the diaphragm anteriorly in the midline creates a defect
(foramen of Morgagni- 80% on rightside) through which hernias can occur (Morgagni Hernia)- 20%. Superior
epigastric vessels may pass through this space. Also called “Larry’s hernia”
Posterolaterally on left side, failure of fusion of the pleuroperitoneal canal creates a defect through which viscera
may herniate to produce a foramen of Bochdalek hernia- 80%

Symptoms in the Morgagni hernia usually appear in middle age. This type of hernia is more frequent in
women. These hernias are mostly right-sided and have a hernia sac. The most common contents are the
omentum, the colon, and the stomach.
The Bochdalek hernia occurs on the left side and may cause severe respiratory distress at birth, requiring an
emergent operation.

Classical clinical triad is: Scaphoid abdomen, respiratory distress, Dextrocardia.

Polyhydroamnios may be present.

Routine chest films show Gastric bubble of bowel loops in thorax retrosternally in Morgagni hernia or in
posterolateral thorax in a Bochdalek hernia.
******ebook converter DEMO Watermarks*******
 Pneumothorax if develops is always seen in contralateral side.
Early repair adds to physiological stress so child is prepared for 24-72 hrs before surgery.
For repair transabdominal approach is preferred, though laparoscopic or thoracoscopic approach can also be
used. Defect is closd by interrupted non absorbable sutures and for large defect a prosthetic mesh can be usd for
tension free repair.
Pulmonary hypoplasia and pulmonary arterial hypertension are main cause of death.

Points to remember:
Early repair does not improve survival.
Bag Mask ventilation is contraindicated.
Pulmonary Hypertension is most important prognostic factor. Pulmonary hypoplasia is major determinant of
survival.
Most common content of Morgagni hernia is Colon.
Prenatal USG can be diagnostic where peristalsis in thorax can be seen.

Traumatic Diaphragmatic Hernia:

Traumatic rupture occurs in the tendinous portion of the diaphragm, most often on the left side (MCQ).
Abdominal viscera may immediately herniate through the defect or may gradually insinuate themselves into the
thorax over a period of months or years.
Patient may have respiratory or bowel symptoms.
Plain films of the chest show a radiopaque area (omentum) or occasionally an air-fluid level if hollow viscus.
If the stomach has entered the chest, the abnormal path of a nasogastric tube may be diagnostic.
Ultrasonography, CT scan, and MRI may demonstrate the diaphragmatic rent.
Common complications are haemorrhage and obstruction or finally strangulation.
Treatment.
For acute ruptures, a trans abdominal route preferred. Chronic injuries can be repaired by either approach.
Asymptomatic tears of the diaphragm should always be repaired because the risk of strangulating obstruction is
high.

Tumors of the Diaphragm:

Primary tumors of the diaphragm are rare, most common is benign lipomas.
Pericardial cysts is seen between the heart and the diaphragm and are usually unilocular and on the right side.
Fibrosarcoma, the most common primary malignant diaphragmatic tumor.
All diaphragmatic lesions should be excised through thoracotomy or thoracoabdominal approach.

Stomach:

The cardia is located at the gastroesophageal junction.

The fundus is the portion of the stomach that lies cephalad to the gastroesophageal junction.
The corpus (Body) is the capacious central part; division of the corpus from the pyloric antrum is marked
approximately by the angular incisure, a crease on the lesser curvature just proximal to the “crow’s-foot”
terminations of the nerves of Latarjet.
The pylorus is the boundary between the stomach and the duodenum.
The cardiac gland area is the small segment located at the gastroesophageal junction. Histologically, it
contains principally mucus-secreting cells, though a few parietal cells are sometimes present.
The oxyntic gland area is the portion containing parietal (oxyntic) cells and chief cells. The boundary between
this region and the adjacent pyloric gland area is reasonably sharp, since the zone of transition spans a segment
of only 1–1.5 cm.
The pyloric gland area constitutes the distal 30% of the stomach and contains the G cells that manufacture
gastrin. Mucous cells are common in the oxyntic and pyloric gland areas.
As in the rest of the gastrointestinal tract, the muscular wall of the stomach is composed of an outer
******ebook converter DEMO Watermarks*******
 longitudinal and an inner circular layer. An additional incomplete inner layer of obliquely situated fibers is
most prominent near the lesser curvature but is of less substance than the other two layers.

Motility:

Storage, mixing, trituration, and regulated emptying are accomplished by the muscular apparatus of the
stomach. Peristaltic waves originate in the body and pass toward the pylorus. The thickness of the smooth
muscle increases in the antrum and corresponds to the stronger contractions. The pylorus behaves as a
sphincter, though it normally allows a little to-and-fro movement of chyme across the junction.
An electrical pacemaker situated in the fundal musculature near the greater curvature gives rise to
regular (3/min) electrical impulses (pacesetter potential, basic electrical rhythm) that pass toward the pylorus
in the outer longitudinal layer. Every impulse is not always followed by a peristaltic muscular contraction, but
the impulses determine the maximal peristaltic rate. The frequency of peristalsis is governed by a variety of
stimuli. Each contraction follows sequential depolarization of the underlying circular muscle resulting from
arrival of the pacesetter potential.
Peristaltic contractions are more forceful in the antrum than the body and travel faster as they progress distally.
Gastric chyme is forced into the funnel-shaped antral chamber by peristalsis; the volume of contents delivered
into the duodenum by each peristaltic wave depends on the strength of the advancing wave and the extent to
which the pylorus closes. Most of the gastric contents that are pushed into the antral funnel are propelled
backward as the pylorus closes and pressure within the antral lumen rises. Five to 15 mL enter the duodenum
with each gastric peristaltic wave.
The volume of the empty gastric lumen is only 50 mL. By a process called receptive relaxation, the stomach
can accommodate about 1000 mL before intraluminal pressure begins to rise. Receptive relaxation is an active
process mediated by vagal reflexes and abolished by vagotomy. Peristalsis is initiated by the stimulus of
distention after eating. Various other factors have positive or negative influences on the rate and strength of
contractions and the rate of gastric emptying. Vagal reflexes from the stomach have a facilitating influence on
peristalsis. The texture and volume of the meal both play a role in the regulation of emptying; small particles
are emptied more rapidly than large ones, which the organ attempts to reduce in size (trituration). The
osmolality of gastric chyme and its chemical makeup are monitored by duodenal receptors. If osmolality is
greater than 200 mosm/L, a long vagal reflex (the enterogastric reflex) is activated, delaying emptying. Gastrin
causes delay in emptying. Gastrin is the only circulating gastrointestinal hormone to have a physiologic effect
on emptying.

Hypergastrinemia:
Causes of Hypergastrinemia:

H pylori:

Association with ulcer proved by “Warren and Marshall” (received Nobel Prize in 2005).
Gastric antrum is most common site of localization (Also in- Proximal esophagus, Gastric metaplasia in
******ebook converter DEMO Watermarks*******
 duodenal mucosa, Meckel’s diverticulum and heterotropic Gastric mucosa in rectum).
Most infections are acquired in childhood (inversely related to socio-economic group) probably through faeco-
oral spread. Spontaneous remission is unlikely.
Mechanism: Stimulation of gastric release by ammonia (released by urease), Suppression of somatostatic
(which is inhibitory to Gastrin), Interrupts inhibitory vagal reflux and inhibits gastro-duodenal bicarbonate
secrtion.
Helicobacter-associated pangastritis is also a very common manifestation of infection (So H pylori may
involve Antrum or pangastritic but corpus alone does not seem to be involved).
Patients with pangastritis seem to be most prone to the development of gastric cancer.
Intestinal metaplasia is associated with chronic pangastritis with atrophy.
Although intestinal metaplasia per se is common, intestinal metaplasia associated with dysplasia has
significantmalignant potential.
Reinfection following successful eradication appears rare but incomplete eradication is a bigger clinical
problem.

Features:

Spiral, gram negative, motile (Lophotrichous flagella).

UREASE, catalase, oxidase positive.
Grows at 37° at microaerophilic conditions.
Skirrow and Chocolate medium can be used.

Pathogenesis:

Optimal pH for growth- 6-7 (can’t survive at normal gastric pH). It grows deep in the mucous layer, near
epithelial surface where urease provide ammonia to buffer acid.
H pylori produces Cag A and Vac A (Vacuolatingcytotixin) two toxin. Gene CagPal.
Associated with Duodenal ulcer (90%), Gastric ulcer (75%), Gastric carcinoma and MALToma.
H pylori and H. bilis is also associated with increased risk of Ca Gall Bladder
In extra intestinal manifestation it may cause Ischemic heart disease and Cerebrovascular disease Also
associated with SCC of esophagus

Diagnosis:

Gold standard- Histological Visualization (Gold standard)- Silver, Giemsa, Genta or “Warthin Silver starry
stain” .
During endoscopy (IOC)- Rapid urease test.
Initial investigation (endoscopy no required)- Serology.
In follow up after treatment to see eradication (4 weeks after therapy)- Urea breath test.

Treatment:
Metronidazole, Amoxycillin, Clarithromycin
Gastritis:
Acute Gastritis:

Acute gastritis is often the result of toxic injury by drugs (NSAID or alcohol).
Haemorrhage is most common presentation which may be both generalized and profuse.
Endoscopically shows multiple small superficial erosions against a hyperaemia.
Barium is rarely needed, it may then show small, shallow erosions in which a central pit of barium is
surrounded by a lucent halo.
Acute gaTypes:
erosive (eg, hemorrhagic erosions, superficial erosions, deep erosions).
nonerosive (generally caused by Helicobacter pylori).

******ebook converter DEMO Watermarks*******

 Causes:
Drugs (Nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin, ibuprofen, and naproxen);
alcohol.
Bacterial (H pylori gastritis typically starts in the antrum- It is also responsible for as many as 80% of
gastric ulcers and is associated with a transient increase in gastric acid secretion).
Viral (Cytomegalovirus can cause gastritis in immunocompromised- Gastric-fold thickening may be
confined to the antrum).
Fungal infections(Candida albicans and histoplasmosis. C albicans rarely involves the gastric mucosa).
Acute stress (shock); radiation; and direct trauma.

Other Tests:
For H pylori, the urea breath test (sensitivity and specificity of the urea breath test is greater than 90%).
Treatment:
Medical Care:

Treatment is dependent on the pathology and cause of gastritis. No specific therapy is indicated. Discontinue
use of drugs known to cause gastritis, eg, NSAIDs and alcohol.

Surgical Care: Rarely needed for phlegmonous gastritis.

Chronic atrophic gastritis, which usually spares the antrum, is associated with parietal cell antibodies, reduced
secretion of acid and intrinsic factor, and hence with pernicious anaemia and other autoimmune disorders.
By contrast, in chronic gastritis associated with Helicobacter pylori infection, activity is maximal in the
antrum, acid secretion is not much affected, and B12 malabsorption is not found.

Special Forms of Gastritis:

1. Eosinophilic gastritis affects the distal stomach. Dyspepsia is common, and thickened antral folds may cause
pyloric obstruction. Bleeding, protein-losing enteropathy, and eosinophilic ascites are also described. Other sites
in the gastrointestinal tract may also be affected, and peripheral eosinophilia is usually present. The diagnosis is
generally made from gastric histology; the changes are most marked in the antrum, where there is submucosal
oedema and a variable eosinophilic infiltrate.
******ebook converter DEMO Watermarks*******
2. Granulomatous gastritis is a diagnosis of exclusion. Macroscopically there may be ulceration, infiltration, and
thickening of the mucosa responsible for pyloric narrowing, or changes resembling linitisplastica.
3. Giant-cell granulomas within the mucosa - with or without an associated gastritis - are seen histologically.
4. Reflux gastritis

This is caused by enterogastric reflux (common after gastric surgery or biliary surgery).
Bile-chelating or prokinetic agents may be useful in treatment and as a temporising.
Surgery is reserved for the most severe cases.

Menetrier’s disease - giant hypertrophic gastritis – (hypoproteinemic hypertrophic

gastropathy):

Acquired, premalignant disease characterized by massive gastric folds in the fundus and corpus of the stomach,
giving the mucosa a cobblestone or cerebriform appearance.
Histologic examination reveals foveolar hyperplasia (expansion of surface mucous cells) with absent parietal
cells.
The condition is associated with

a. Protein loss from the stomach,

b. Excessive mucus production, and
c. Hypochlorhydria or achlorhydria.

The cause of Ménétrier’s disease is unknown, but it has been associated with.

a. Cytomegalovirus infection in children.

b. H. pylori infection in adults.

Additionally, increased transforming growth factor-α has been noted in the gastric mucosa of patients with
the disease.
Main presentation is epigastric pain, vomiting, weight loss, anorexia, peripheral edema, skin rash.
Typical gastric mucosal changes can be detected by radiographic or endoscopic examination. Biopsy should be
performed to rule out gastric carcinoma or lymphoma.
Twenty-four-hour pH monitoring reveals hypochlorhydria or achlorhydria, and a chromium-labeled albumin
test reveals increased GI protein loss.
Medical treatment has yielded inconsistent results; however, some benefit has been shown through the use of
anticholinergic drugs, octreotide, and H. pylori eradication.
Total gastrectomy should be performed in patients who continue to have massive protein loss despite optimal
medical therapy or if dysplasia or carcinoma develops.
In children the disease characteristically is self-limited and benign. There is an increased risk of
adenocarcinoma of the stomach in adults with Ménétrier disease.
Now a days Cetuximab is also being tried for this condition.

Points to remember
TPE A Gastritis:

Autoimmune, Proximal part, Cancer risk, Parietal cell loss, Decreased acid enzyme production.

Type B Gastritis:

Hpylori, Antrum.

Menetrier’s Disease:

Involves proximal part.

Thickened Mucosal, Giant Rugal fold, Deep crypts, fovolar hypertrophy.

******ebook converter DEMO Watermarks*******

 Decreased enzyme and acid production and increased mucus (protein) loss.
Associated with- CMV (adults) and H.Pylori (children).
Overexpression of TGF alpha.

Peptic Ulcer Disease (PUD):

Peptic Ulcer Disease (PUD) characterized by ulceration of the stomach or proximal duodenum due to an imbalance
between acid secretion and mucosal defense mechanisms.
Benign gastric ulcer is commonest on the lesser curve away from acid-secreting epithelium.
Pathogenesis:
Four etiologic factors are responsible for the vast majority of PUD.

Helicobacter pylori (H. pylori) infection is associated with 90% to 95% of duodenal ulcers and 70% to 90% of
gastric ulcers. Infection produces chronic antral gastritis, increased acid and gastrin secretion, and decreased
mucosal resistance to acid.
NSAID use confers an 8-fold increase in risk of duodenal and a 40-fold increase in risk of gastric ulcers due to
suppression of prostaglandin production.
Cigarette smoking.
Acid hypersecretion occurs in the majority of patients with duodenal ulcers.

******ebook converter DEMO Watermarks*******

Presentation:

Presentation in uncomplicated ulcer disease is usually burning, intermittent epigastric pain that is relieved by
food or antacid ingestion for duodenal ulcers but exacerbated by intake for gastric ulcers.
Pain may be accompanied by nausea, vomiting, and mild weight loss.

Histology:

The intact gastric mucosa extends to the margins of the ulcer crater, the base of the ulcer consisting mainly of
granulation tissue. With chronicity, fibrosis may completely replace the gastric muscle, seen then only at the
margins of the lesion.
Ulcers on the greater curve are more often malignant than ulcers elsewhere in the stomach, but other benign
ulcers at greater curvature are “sump ulcer” which occurs in the most dependent part of the stomach and is
often associated with ingestion of anti-inflammatory drugs and “stomal ulcer”.
Juxtapyloric ulcers are invariably benign and can be considered with duodenal ulcers, which they resemble in
clinical behaviour.

Types of Gastric Ulcer: Three main types of gastric ulcer have been described.

Johnson’s Classification:
1. Type I, the most common type of gastric ulcer, occurs at the lesser curvature and is typically found in the
transitional mucosa between the body of the stomach and the antrum.
2. Type II is a gastric ulcer that coexists with a duodenal ulcer.
3. Type III is a prepyloric or pyloric channel ulcer that seems to be associated with gastric acid hypersecretion.
Type IV is at fundus and
Type V is at greater curvature/ Diffuse (Associated with NSAID).
(Types 2 and 3 are associated with acid hypersecretion).

******ebook converter DEMO Watermarks*******

Diagnosis: Diagnosis can be made by:

Barium contrast radiography: Barium meal examination showing an ulcer crater with radiating mucosal folds
reaching to its rim strongly suggests that the ulcer is benign.Hypotonicduodenography was done in past for DU.
Upper gastrointestinal endoscopy: more sensitive and specific than contrast examination. In addition, EGD
offers therapeutic options (ligation of bleeding vessels) and diagnostic options (biopsy for malignancy, antral
biopsy for H. pylori).

Investigations for causes:

H. pylori infection detected by:

noninvasively
radiolabeled urea breath test
serologic antibody testing.
Antral tissue biopsy can be subjected to
direct histologic examination or
rapid urease testing using the cod liver oil (CLO) test.
Fasting serum gastrin levels should be done where no history of NSAID use and are H. pylori-negative or who
have recurrent ulcers despite adequate treatment, multiple ulcers, ulcers in unusual locations (such as the 2nd
and 3rd part of the duodenum), or complicated PUD (hemorrhage, perforation, obstruction). Such atypical
presentations suggest the possibility of Zollinger-Ellison syndrome.
Endoscopic biopsy of gastric ulcers is mandatory to exclude malignancy.

Treatment:
Treatment of PUD has changed dramatically with the development of antisecretory drugs.
[histamine2-receptor blockers and proton-pump inhibitors (PPIs)], and H. pylori –eradication.
regimens have greatly diminished the role of elective surgery for PUD.
Medical therapy:

H. pylori eradication is the cornerstone of medical therapy for PUD. These regimens are 85% to 90% effective
in eradicating H. pylori. Antisecretory therapy is then continued until ulcer healing is complete.
NSAID-associated PUD is treated by discontinuing the offending medication.
Smoking cessation greatly facilitates ulcer healing, but compliance rates are low.
Follow-up endoscopy to ensure healing is essential for gastric ulcers because up to 3% harbor malignancy.

Recommendations for Helicobacter pylori Treatment:

Patients with active peptic ulcer disease who are H. pylori positive

******ebook converter DEMO Watermarks*******

 Use of nonsteroidal anti-inflammatory drugs should not alter treatment.
Document eradication in those with complications.
Ulcer patients in remission who are H. pylori positive, including patients on maintenance H2-receptor
antagonist therapy
H. pylori–positive patients with mucosa-associated lymphoid tissue (MALT) lymphoma
Controversial issues in H. pylori–positive patients
First-degree relatives of gastric cancer patients
Immigrants from countries with high prevalence of gastric cancer
Individuals with gastric cancer precursor lesions (intestinal metaplasia)
Non–ulcer dyspepsia patients who insist on eradication (benefit vs. risk)
Patients on long-term antisecretory therapy for reflux disease.

Surgical therapy:
Indications for elective operation for PUD include failure of medical therapy and inability to exclude malignancy.
Duodenal ulcers are treated by one of three acid-reducing operations:
1. Truncal vagotomy with pyloroplasty.
2. Truncal vagotomy with antrectomy and Billroth I (gastroduodenostomy) or Billroth II (gastrojejunostomy)
reconstruction.
3. Highly selective vagotomy (HSV).
Truncal vagotomy with antrectomy yields maximal acid suppression with lowest ulcer recurrence rates (1%– MCQ)
but carries the highest postoperative morbidity and mortality. HSV has the lowest postoperative morbidity and
mortality rates but is technically demanding to perform and has higher recurrence rates (5% to 15%).

******ebook converter DEMO Watermarks*******

Gastric ulcers are typically treated with either wedge excision or antrectomy with inclusion of the ulcer, depending
on ulcer location. Concurrent truncal vagotomy is reserved for patients who are known to have refractory ulcer
disease despite maximal medical management; this is rare today.

******ebook converter DEMO Watermarks*******

Gastric vs. Duodenal Ulcer:
Important clinical differences exist between a gastric and a duodenal ulcer.

In cases of gastric ulcer one must rule out the diagnosis of carcinoma. The incidence of benign gastric ulcer
turning malignant is extraordinarily small (<1.0%). Chances that a benign ulcerating gastric lesion may be
malignant, however, are much higher.
Bleeding from a gastric ulcer may be more serious and less likely to stop spontaneously compared to a
duodenal ulcer.

******ebook converter DEMO Watermarks*******

 Gastric outlet obstruction is more a feature of duodenal ulcer than of gastric ulcer.
Perforation is more common with a duodenal ulcer and, at surgery, whenever possible, a gastric ulcer must be
excised or removed with the gastrectomy specimen to be sure it is not malignant. In gastric ulcers located high
in the body of the stomach, where resection of the ulcer is not feasible, four-quadrant biopsy is necessary.

The response to surgical therapy of the two types of ulceration is different.

1. While proximal gastric vagotomy is a highly satisfactory operation for duodenal ulcer, it is less effective for
treating gastric ulcer. In prepyloric ulcers proximal gastric vagotomy is associated with ulcer recurrence rates
of 25% to 40%.
2. When surgery is indicated, the majority of gastric ulcers are most reliably treated by antrectomy and Bilroth I
anastomosis, thus removing the ulcer completely.

Points to remember:
GU is associated with decreased mucosal resisitance and DU with hyperacidity.
H pylori associated with 90% DU and 75% GU.
Johnson types 2 and 3 are associated with hyperacidity so treated by Vagotomy.
Treatment of GU (Johnson type 1)- Billroth 1 (or Billroth II with reconstruction)

1. For Johnson 2 and 3- (increased acid production).

2. Treatment for chronic / intractable DU- HSV.
3. Treatment of recurrent DU- TV and antrectomy (This surgery sis associated with lowest recurrence rate)
4. For type IV- Pouchet/ KellingMedlener/ Csendes procedure. Shoemaker surgery
Barium Meal Benign ulcer:

Mucosal folds reaching upto edge- sponge like manner

Ulcer collar, Hampton line ulcer nd Penetraing sign

Barium Meal Malignant ulcer:

Mucosal folsa are amputated, clubbed not reaching upto ulcer edge
Carman meniscus sign, Intraluminal crater, Kirklinccomplex

Complications of Gastric Ulcers

A. Haemorrhage

20% of all Peptic ulcer have risk of bleeding.

Petic ulcer is most common cause of Upper GI bleed
Hemorrhage is the leading cause of death due to PUD (5% to 10% mortality).
Spontaneous cessation of bleeding occurs in 70% of patients.
endoscopic therapy using thermal coagulation (with or without epinephrine) is warranted in individuals
who present with hemodynamic instability, need for continuing transfusion, hematemesis or red stool, age
> 60 years, and serious medical comorbidities.
Indications for surgery:
repeated episodes of bleeding.
Old patients.
Active sever bleeding in Endoscopy.
Hb< 8 gm% at admission.
continued hemodynamic instability.
ongoing transfusion requirement of more than 5 units of packed red blood cells over 24 hours.
more than two unsuccessful endoscopic intervention.
Continued slow bleeding with a transfusion requirement exceeding 3 units/day.
Bleeding duodenal ulcers are usually located on the posterior duodenal wall within 2 cm of the pylorus and
******ebook converter DEMO Watermarks*******
 typically erode into the gastroduodenal artery. Bleeding is controlled by duodenotomy and oversewing of the
bleeding vessel (MCQ).
In Gastric ulcer bleeding is not as common as DU but ulcer situated proximally on lesser curvature r prone to
bleed (From left gastric artery)
Stable patients may be candidates for antrectomy and vagotomy.
Possibility of recurrence is more in Gasric ulcer.
Blood group O has more tendencu to bleed.
Rarely, a bleeding vessel will be found in the proximal stomach with no apparent ulcer: the so-called Dieulafoy
lesion.

B. Perforated peptic ulcer:

Perforated peptic ulcer typically presents with peritonitis.

Abdominal x-ray reveals free subdiaphragmatic gas in 80% to 85% of cases.
After aggressive fluid resuscitation and broad-spectrum antibiotics prompt operative repair is indicated.
Perforated duodenal ulcers are best managed by simple omental patching and peritoneal débridement, followed
by H. pylori eradication. An acid-reducing procedure (preferably truncal vagotomy and pyloroplasty) should be
added in stable patients who are known to be H. pylori-negative or have failed medical therapy.
Perforated gastric ulcers are best treated by simple wedge resection to eliminate the perforation and exclude
malignancy. If wedge resection of the ulcer cannot be performed due to its juxtapyloric location, multiple
biopsies of the ulcer are taken and omental patching is performed.

Posterior Duodenal perforation signs: (Valentino Sign and Veiled kidney sign):
1. Valentino’s syndrome is pain presenting in the right lower quadrant of the abdomen caused by duodenal ulcer
with retroperitoneal perforation presenting with pain in the right lower quadrant mimicking appendicitis.
2. Accumulated air around right kidney gives Veiled Kidney sign.

******ebook converter DEMO Watermarks*******

Surgery in Nutshell:

Named Procedures:
Hill And Backer Procedure: Posterior Truncal Vagotomy With Anterior Hsv
Taylor Procedure: Posterior Truncal Vagotomy With Anterior Seromyotomy (Best For Laparoscopy).
C. Gastric outlet obstruction (Most common cause of GOO is Maliganancy).

Gastric outlet obstruction can occur as a chronic process due to fibrosis and scarring of the pylorus from
chronic ulcer. Mosly Ulcer situated in first part of duodenum cause GOO.
Patients present with recurrent vomiting of poorly digested food, dehydration, and hypochloremic hypokalemic
******ebook converter DEMO Watermarks*******
 metabolic alkalosis.
Management consists in correction of volume and electrolyte abnormalities, nasogastric suction, and
intravenous antisecretory agents.
Endoscopy is necessary to rule out malignancy, and endoscopic hydrostatic balloon dilation can be performed
at the same time.
It is feasible in up to 85% of cases, but < 40% have sustained improvement at 3 months.
Indications for surgical therapy include persistent obstruction after 5 – 7 days of nonoperative management and
recurrent obstruction.
Antrectomy to include the ulcer and truncal vagotomy is the ideal operation for most patients.
In exceptional instances, truncal vagotomy with gastrojejunostomy may be preferred in those patients
whose pyloroduodenal inflammation precludes safe management with Billroth I or II reconstructions.

Surgical Treatment Recommendations for Complications Related to Peptic Ulcer Disease:

Complications of Surgery:
A Vagotomy: Diarrhoea, Atony and Incomplete vagotomy:
1. Post vagotomy Diarrhoea: 30%.

Mild and in most patients with postvagotomydiarrhea have their symptoms resolve over time.
Medical management is cholestyramine. Surgery is only done after 1 year for continuing symptom.
The operative procedure of choice is to interpose a 10-cm segment of reverse jejunum 70 to 100 cm from the
******ebook converter DEMO Watermarks*******
 ligament of Treitz.

2. Gastric atony:

After vagotomy, gastric emptying is delayed (In truncal and selective vagotomies but not in HSV).
Due to loss of antral pump function and loss of receptive relaxation in the proximal stomach, which regulates
liquid emptying.
The diagnosis of gastroparesis is confirmed on scintigraphic assessment of gastric emptying.
(D/D-Diabetes mellitus, electrolyte imbalance, drug toxicity, and neuromuscular disorders)
Medical management - prokinetic agents such as metoclopramide (Dopamine antagonist and acetylcholine
release) and erythromycin (Binds to Motilin receptor).

3. Recurrenec (Incomplete transaction)

B Secondary to Gastric resection- Dumping syndrome and Metabolic:
1. Dumping Syndrome:
Dumping syndromeoccurs when normal pyloric sphincter mechanism has become disrupted.
Early Dumping:

Occurs within 20 to 30 minutes after ingestion of a meal.

The GI symptoms include nausea and vomiting, a sense of epigastric fullness, eructations, cramping abdominal
pain, and often explosive diarrhea.
The cardiovascular symptoms are- palpitations, tachycardia, diaphoresis, fainting, dizziness, flushing, and
occasionally blurred vision.
More common after partial gastrectomy with the Billroth II reconstruction (50-60%), far less common
following Billroth I gastrectomy or after vagotomy and drainage procedures.
It occurs because of the rapid passage of food of high osmolarity from the stomach into the small intestine.
Hypertonic food bolus passing into the small intestine leads to shift of extracellular fluid into the intestinal
lumen.
Several humoral agents, such as serotonin, bradykinin-like substances, neurotensin, and enteroglucagon are
released.
Most patients experience spontaneous relief and require no specific therapy.
For continuing symptoms octreotide acetate has been shown to be highly effective in controlling both
vasomotor and GI symptoms.
Surgery is required in < 1% cases.
Interposition of antiperistalticjejunal segment orcreation of a long-limb Roux-en-Y anastomosis to delay gastric
emptying are two surgical approaches.

Late Dumping:

It appears 2 to 3 hours after a meal and is far less common than early dumping. Its caused by hypoglycaemia
which occurs due to large amounts of insulin release to control the rising blood sugar (of early dumping).
Presets with diaphoresis, tremulousness, light-headedness, tachycardia, and confusion. The symptom complex
is like insulin shock.
Treatment - Frequent small meals and to reduce their carbohydrate intake.
Adding acarbose, an α-glucoside hydrolase inhibitor that delays carbohydrate absorption.Surgery is rarely
required.

2. Metablic:

More common and serious after partial gastrectomy than after vagotomy.
The incidence much greater if a Billroth II as opposed to a Billroth I
The severity is directly related to the extent of gastric resection (Like Dumping).
The most common metabolic defect appearing is anemia. Corrected by dietary supplementation.
Anaemia:
Both type seen- Iron and B12 deficiency. Iron def more common (30%). B12 def due to lack of Intrinsic
******ebook converter DEMO Watermarks*******
 factors (requiring IM injection of Cyanocobalamine every 3-4 months).
Folate def is rare. Dietary suppimentation suffices
Impared absorption of fat.
Steatorrhea may be seen after a Billroth II may occur due to duodenal bypass. If this occurs, a deficiency
in uptake of fat-soluble vitamins may also occur.
Osteoporosis and osteomalacia caused by deficiencies in calcium.
Treatment of this disorder usually requires calcium supplements (1-2 g/day) in conjunction with vitamin D
(500-5000 units daily).

C. Secondary to Gastric reconstruction: Afferent and efferent loop syndrome, Alkaline reflux gastritis,
Retained ntrum syndrome
1. Afferent Loop Syndrome:

Afferent loop syndrome is due to partial obstruction of the afferent limb and improper emptying. The syndrome
usually occurs when the afferent limb is greater than 30 to 40 cm in length and has been anastomosed to the
gastric remnant in an antecolic fashion.
Improper drainage leads to accumulation of pancreatic and hepatobiliary secretion leading to distention,
epigastric discomfort and cramping, bilious vomiting often projectile
In the setting of complete obstruction, necrosis and perforation of the loop can occur or Duodenal blow out
(common on fourth day).
Surgery is required for acute or chronic Afferent loop syndrome. Either Billroth II is converted to Billroth I
anastomosis, or an enteroenterostomy below the stoma can be done.

2. Efferent Loop Obstruction:

Rare.
The most common cause of efferent loop obstruction is herniation of the limb behind the anastomosis in a
right-to-left fashion.
This can occur with both antecolic and retrocolicgastrojejunostomies.
50% of cases develop symptoms within the first postoperative month.
Presentation is pain, bilious voting and distension.
Diagnosis is confirmed by Ba study
Operative intervention is almost always necessary and consists of reducing the retroanastomotic hernia and
closing the retroanastomotic space to prevent recurrence

3. Alkaline Reflux Gastritis:

******ebook converter DEMO Watermarks*******
 After gastrectomy, reflux of bile is fairly common.
In few patients it may cause severe epigastric abdominal pain , bilious vomiting and weight loss and iron def
anaemia.
On endoscopy, the mucosa is frequently friable and beefy-red, and superficial mucosal ulcerations may be
apparent on microscopic examination.
Medical management is rarely beneficial and most require surgical correction which is done by Billroth II
anastomosis into a Roux-en-Y gastrojejunostomy.

4. Retained Antrum Syndrome

Antral mucosa may extend past the pyloric muscle for a distance of 0.5 cm, the syndrome of retained gastric
antrum may occur after partial gastrectomy even if the resection is carried beyond the pyloric sphincter.
So retained antrum syndrome if residual antrum is left in the duodenal stump continuously bathed in alkaline
pH from the duodenal, pancreatic, and biliary secretions release of large amounts of gastrin with a resultant
increase in acid secretion and recurrence.
A technetium scan may prove helpful in diagnosing retained antrum.
Medical management is: H2-receptor blockade or proton pump inhibitors
If ineffective, either conversion of the Billroth II to a Billroth I reconstruction or excision of the retained antral
tissue in the duodenal stump is indicated.

Gastrojejunocolic and Gastrocolic Fistula:

A deeply eroding ulcer may occasionally produce a fistula between the stomach and colon mainly seen in
recurrent peptic ulcer after gastrojejunal anastomosis.
Pain followed by severe diarrhea (watery, 8-10 ims per day and contains undigested food) and weight loss are
the presenting symptoms in over 90% of cases.
An upper gastrointestinal series reveals the marginal ulcer in only 50% of patients and the fistula in only 15%.
Barium enema unfailingly demonstrates the fistulous tract.
After fluid and electrolyte imbalance correctionthe involved colon and ulcerated gastrojejunal segment should
be excised and colonic continuity re-established.
Vagotomy, partial gastrectomymay be required to prevent recurrence

Bleed Risk Classification:

Bleeding (Ongoing)
Low systolic BP (< 100 mm Hg)
Elevated PT (>1.2 times from control)
Altered mental status
Uncontrolled co-morbidity

Forrest Classification of Endoscopic Findings and Rebleeding Risks in Peptic Ulcer Disease:

Common Causes of Upper Gastrointestinal Hemorrhage:

******ebook converter DEMO Watermarks*******
Dieulafoy’s Gastric Lesion:

A rare cause of upper GI hemorrhage from a abnormally large (1-3 mm), tortuous submucosal arteryleading
to erosion of the superficial mucosa.
The mucosal defect is 2 - 5 mm in size, surrounded by normal-appearing gastric mucosa within 6 cm from the
GE junction generally in the fundus near the cardia at lesser curvture.
Dieulafoy’s lesions are more common in men, with the peak incidence in the fifth decade.
The classic presentation is sudden massive, painless, recurrent hematemesis with hypotension.
Esophagogastroduodenoscopy is the diagnostic modality of choice.
Because of the intermittent nature of the bleeding, repeated endoscopies may be needed.
Lesion istreatedendoscopicallyby electrocoagulation, heater probe, noncontact laser photocoagulation, injection
sclerotherapy, band ligation, or endoscopic hemoclipping.
Surgery is now reserved for patients in whom other modalities have failed.
The surgical management consists of gastric wedge resection to include the offending vessel.

Gastric Antral Vascular Ectasia: Also known as “watermelon stomach”

gastric antral vascular ectasia (GAVE) is characterized by a collection of dilated venules appearing as linear red
streaks converging on the antrum in longitudinal fashion.
Histologically, gastric antral vascular ectasia is characterized by dilated mucosal blood vessels that often
contain thrombi, in the lamina propria
Mucosal fibromuscular hyperplasia and hyalinization often are present resembling Portal gastropathy though
later involves fundus while here Antrum is involved.
Most patinets are old women having autoimmune connective tissue disorder or chronic liver disease (25%).
Acute severe hemorrhage is rare in GAVE, and most patients present with persistent, iron deficiency anemia
from continued occult blood loss.
Endoscopic therapy is indicated for persistent, transfusion-dependent bleeding and has been reportedly
successful in up to 90% of patients.
The preferred endoscopic therapy is argon plasma coagulation.
Those failing endoscopic therapy are considered for antrectomy.

Gastric Polyps:

Gastric polyps may be hamartomatous and, regenerative or hyperplastic, or true neoplasms (adenomas).
It is probably only the adenomathat have malignant potential, with a higher risk when multiple and diameter
exceeds 2cm.
Gastric polyps occur predominantly in the elderly. Those located in the distal stomach are more apt to cause
symptoms.
Gastric polyps can be classified histologically as hyperplastic, adenomatous, or inflammatory.
Hyperplastic polyps constitute 80% of cases; they are not true neoplasms and have no relationship to
gastric cancer.
About 30% of adenomatous polyps contain a focus of adenocarcinoma, and adenocarcinoma can be found
elsewhere in the stomach in 20% of patients with a benign adenomatous polyp. The incidence of cancer in an
adenomatous polyp rises with increasing size. Lesions with a stalk and those less than 2 cm in diameter are
usually not malignant. About 10% of benign adenomatous polyps undergo malignant change during prolonged
******ebook converter DEMO Watermarks*******
 follow-up.
Excision with a snare through the endoscope can be performed safely for most polyps. Otherwise, laparotomy
is indicated for polyps greater than 1 cm in diameter or when cancer is suspected. Single polyps may be excised
through a gastrotomy and a frozen section performed. If the polyp is found to be carcinoma, an appropriate type
of gastrectomy is indicated. Partial gastrectomy should be performed for multiple polyps in the distal stomach.
If 10–20 polyps are distributed throughout the stomach, the antrum should be removed and the fundic polyps
excised. Total gastrectomy may be required for symptomatic diffuse multiple polyposis.
These patients should be followed because they have an increased risk of late development of pernicious
anemia or gastric cancer. Recurrent polyps are uncommon.
Barium studies demonstrate apparently translucent filling defects, and at endoscopy, adenomatous polyps are
more obviously distinct from surrounding mucosa than the commoner hyperplastic type.
Polyps in the duodenum are common in familial adenomatous polyposis, but unusual proximal to the ampulla
of Vater.

Gastric Carcinoma:

Gastric cancer is the second most common cause of cancer-related death in the world.
The site of the lesion is classified based on the long axis of the stomach. Approximately 50% of cancers
develop in the proximal part (includes cardia and GE junction), 35% in the lower part,and 15% in the mdddle
stomach. Incidenece of proximal cancer (GE junction is increasing).
Gastric cancer afflicts more men than women (2:1).
The median age at diagnosis is 65 years (range 40 – 70 y).

Factors Associated with Increased Risk for Developing Stomach Cancer:

******ebook converter DEMO Watermarks*******

Genetically gastric cancr is associated with:

Blood group A, HNPCC, Pernicious anaemia, Li- Fraumeni syndrome

Tumour Markers:CEA, CA19-9, CA 125, CA 72-4, beta HCG

ONCOGENES: p-53, Cox- 2 gene.
Different classifications for gastric cancer
Japanese Classification:
The Japanese classification is used to describe early stomach cancer, which is a tumour that is limited to the
mucosa or submucosa of the stomach wall. Tumours are described as they appear during an upper gastrointestinal
endoscopy.
Early carcinoma is defined as limited to mucosa and/or submucosa (lymph nodes may or may not be involved).

I Protuberant
IIa Flat, superficially elevated
IIb Flat, not elevated
IIc Flat, slightly depressed
III Excavated (full thickness of submucosa)- Commonest

******ebook converter DEMO Watermarks*******

Borrmann classification (gross):

I Polypoid
II Fungating, ulcerated with sharp raised margins
III Ulcerated with poorly defined infiltrative margins.
IV Infiltrative, predominantly intramural lesion, poorly demarcated.

WHO classification (microscopic):

******ebook converter DEMO Watermarks*******

Lauren classification (microscopic):

Lauren Classification System:

History:

Early disease has no associated symptoms. Most symptoms of gastric cancer reflect advanced disease.
The earliest symptom is usually vague postprandial abdominal heaviness, pain.
Patients may complain of indigestion, nausea or vomiting, dysphagia, postprandial fullness, loss of appetite,
and weight loss.
Late complications include pathologic peritoneal and pleural effusions; obstruction of the gastric outlet,
gastroesophageal junction, small bowel; intrahepatic jaundice caused by hepatomegaly; extrahepatic jaundice;
and Chacexia.

Physical:
All physical signs are late events. Signs may include a palpable enlarged stomach with succussion splash; primary
mass (rare); and enlarged liver, Virchow node’s (ie, left supraclavicular Level IV), Lef axillary node (Irish node),
Sister Mary Joseph’s node, Blumer’s shelf (pouch of Doughlas) and Ovary (Krukenbertumour).
Some patients have signs of weight loss. Other patients may have pallor from bleeding and anemia.
Two cutaneous syndromes of Gastric cancer:
1. Leser-Trelat Sign (Mainly in Gastric cancer):

Multiple pruritic seborrheic keratoses (Sudden onset).

Due to cytokine/growth factor production by tumor.

******ebook converter DEMO Watermarks*******
Commonly seen at the time of cancer diagnosis.

Associated with:
Adenocarcinoma (1/3) Mainly with stomach cancer.
Lymphproliferative disorders (1/5).

2. Tripe palms:

Tripe palms are characterized by thickened and velvety palms associated with Scaling and thickening of the
Palm skin {most commonly associated with underlying malignancy (stomach (35%) or lung (11%)}.
In over 40% of patients, tripe palms are the first sign of an undiagnosed cancer.
Tripe palms are frequently seen along with acanthosis nigricans.
There is no specific treatment for tripe palms.
Only 30% of cases resolve once the underlying cancer is treated.

Imaging Studies:

Esophagastroduodenoscopy: (IOC for diagnosis) This procedure also is the primary method for obtaining a
tissue diagnosis of suspicious lesions.
Double-contrast upper GI series: An upper gastrointestinal barium swallow detects large primary tumors but
only occasionally detects their spread to the esophagus and duodenum (particularly if the tumor is small and
submucosal).
Chest radiograph evaluates for metastatic lesions.
CT scan (IOC for staging) or MRI of the chest, abdomen, and pelvis
PET (IOC for metastasis). If PET is not available then CECT is better than MRI for Metastasis.
Endoscopic ultrasound: For depth.

Histologic Findings:
Adenocarcinoma of the stomach comprises between 90-95% of all gastric malignancies. The second most common
******ebook converter DEMO Watermarks*******
malignancies are lymphomas. Leiomyosarcomas now called GIST (2%), carcinoids (1%), adenoacanthomas (1%),
and squamous cell carcinomas (1%) are the remaining histologies.
Intestinal metaplasia:

Intestinal metaplasia is defined by the replacement of the gastric mucosa with epithelium that resembles small
bowel mucosa.
This replacement is effected by gastric stem cells by persistent irritation (most commonly from H. pylori
infection).
Intestinal metaplasia can be subclassified into complete type I and the incomplete types (II, III).
The types differ based on the patterns of mucin core protein (MUC) expression as well as cell type
composition.
Type I is characterized by the presence of absorptive cells, Paneth cells, and goblet cells secreting sialomucins,
whereas the incomplete types II / III are characterized by the presence of columnar and goblet cells secreting
sialomucins, sulfomucins, or both.
The risk for progression from intestinal metaplasia to gastric cancer is higher in the type III > Type I.
Molecular alteration: overexpression of cyclooxygenase-2 and cyclin D2, p53 mutations, microsatellite
instability, decreased p27 expression, and alterations in transcription factors like CDX1 and CDX2.

Staging of gastric carcinoma:

Requires a combination of preop investigations and intraoperative assessment.

OGD confirms diagnosis, site and extent of tumour.
Endoscopic ultrasound may allow assessment of intramural tumour penetration.
CT will assess nodal spread and extent of metastatic disease.
Laparoscopy will identify peritoneal seedlings.

Peritoneal lavage will identify free tumour cells.

******ebook converter DEMO Watermarks*******

Birmingham Staging System
Clinicopathological system:
******ebook converter DEMO Watermarks*******
 Does not require detailed lymph node status
Stage 1 Disease confined to muscularispropria
Stage 2 Muscularis and serosal involvement
Stage 3 Gastric and nodal involvement
Stage 4a Residual disease
Stage 4b Metastatic disease
Staging: 1997 TNM classification system (AJCC) for staging gastric carcinoma.

Treatment:

Surgery is the only prospective of cure

Antral tumours may be suitable for a partial gastrectomy usually with Polya reconstruction
Other Proxiamltumours will need a total gastrectomy with oesophagojejunal anastomosis and Roux-en-Y
biliary diversion
A tumour is considered resectable if confined to stomach or N1 or N2 nodes involved
Nodes less than 3 cm from tumour = N1 nodes
Nodes greater than 3 cm from tumour = N2 nodes
If tumour and N1 nodes resected = D1 gastrectomy
If tumour and N2 nodes resected = D2 gastrectomy
Evidence to support the use of D2 gastrectomy is incomplete but today ecommmneded surgery is D2
gastrectomy
Splenectomy is only done for proximal cancers (Fundus).
Even in patients with incurable disease surgery may palliate symptoms
Palliative resection is usually indicated if the stomach is still movable and life expectancy is estimated to be
more than 3 months mainly for distal cancer.
Adjuvant chemotherapy post surgery a may reduce relapse and improve survival

Surgical Care:
Type of surgery:

Total gastrectomy (if required for negative margins), an esophagastrectomy for tumors of the cardia and
gastroesophageal junction, and a subtotal gastrectomy for tumors of the distal stomach.

******ebook converter DEMO Watermarks*******

Adjuvant therapy:

The pattern of failure prompted a number of investigations into adjuvant therapy. The rationale behind
radiotherapy is to provide additional local-regional tumor control. Adjuvant chemotherapy is used either as a
radiosensitizer or as definitive treatment for presumed systemic metastases.
Adjuvant radiotherapy.
Moertel and colleagues randomized postoperative patients with advanced gastric cancer to 40 Gy
radiotherapy or 40 Gy radiotherapy with 5-FU as a radiosensitizer, and demonstrated improved survival
associated with the combined modality therapy.
A series from the Mayo Clinic randomized patients to postoperative radiotherapy with 5-FU versus
surgery alone and demonstrated improved survival in the patients receiving adjuvant therapy (23% vs 4%).
Intraoperative radiotherapy.
Some centers suggest that intraoperative radiotherapy (IORT) shows promising results.
The National Cancer Institute randomized patients with grossly resected stage III/IV gastric cancer to
either 20 Gy IORT or 50 Gy postoperative external beam.
Chemotherapy
Numerous randomized clinical trials comparing combination chemotherapy in the adjuvant setting to
surgery alone did not demonstrate a consistent survival benefit.
The most widely studied regimen is 5-fluorouracil, doxorubicin, and mitomycin C. The addition of
methyl-CCNUR, leucovorin, or triazinade did not increase response rates.

Ascitis, Jaundice, palpable mass and distant metastasis ae inoperable disease.

Recurrence:

Recurrence rate is high (40-80%), mostly within 3 years.

Most common site of local recurrence is Anastomosis (20-25%) and Gastric bed (20%).
Most common vascular metastasis – Liver > lung.

Surveillance:

Complete History examination 4 monthly in first year, then 6 monthly for 2 year then annually.
Yearly endoscopy in indicated in partial gastrectomy.

Best prognostic incators are: Depth (for meatstais) and node

Survival:

Prognosis if generally very poor.

Overall 5 year survival is approximately 5-20% (12%).
Survival is 70%, 30%, 10% and 0-3% for Stages 1,2,3 and 4 respectively.

Points to Remember:
Gastric cancer shift is observed from disal to proximal part.
Incidence has reduced to one third in last 50 years in west.
Refrigeration is protective.
Superficial spreding type does not involve muscle (may involve nodes).
Main presenting symptom- pain.
Enscopic biopsy is best for Diagnosis, CECT for Staging and PET for Metastasis.
Main treatment is total gastrectomy- Partial gastrectomy with Billroth II reconstruction preferred for distal
cancer while total gastrectomy with Roux en Y esophago-jejunostomy suitable for proximal cancer.
CT is routinely used as Adjuvant therapy, RT reduces local recurrence.

Other Gastric Tumours:

******ebook converter DEMO Watermarks*******
Gastric Leiomyomas& Gastrointestinal Stromal Tumor (GIST):

2- 3% of gastric cancer.
GIST arises from intretstitial cell of Cajal, autonomic nerve–related GI pacemaker cells (mesenchymal
components) from Muscularispropria.
Most common sites: Stomach (50-60%)> Small Intestine, ileum (25%) > Rectum/ Esophagus.
Most are positive for CD117 (>90%), CD 34 (>70%), BCL 2.
5% associated with plate derived growth factor alpha- 5% (PDGF α has good prognosis).
Other tumor markers are DOG1 and protein kinase C theta.
Two types: Spindle cell (70% most common), epitheloid (30%) and rarely pleomorphic.
Age of presentation after 4th decade, usually around 60 years.
Main presenting symptom: Bleeding (75%) > Pain and dyspepsia. Mass in 60%.

Malignant potential is decided by:

1. Size > 5 cm.
2. metastasis: Vascular or peritoneal (Lymphatic involvement is very rare- 5%-10%).
3. Mitotic Index: > 5 / HPF.
4. Cellular atypia, necrosis, or local invasion.
5. c-kit mutations occur predominantly in malignant GISTs and are an unfavorable prognostic marker. Most c-kit
mutations occur in exon 11 and result in activation of c-kit.
Investigation:

CECT: for primary tumour.

PET: For secondary.

Treatment: Surgical resection (segmental with 1 cm margin).

Node involvement is very rare (so lymphadenectomy is not usually required).

Imatinibmesylate (selective inhibitor of type 3 tyrosine kinase KIT) is best adjuvant therapy. Used in CD117
positive unrespectable tumour or metastatic GIST.
PET is best investigation to assess response of Imatinib
Sunitinib is used in Imatinib resistance cases.
This tumor is radio resistant.

Tumour size is predominant factor for survival.

Most common site of disease failure in Liver.
Carney Triad:

GIST.
Paraganglioma.
Pulmonary Chondroma.

Points to Remember
Commonest site is stomach. Arises from cell of Cajal
Tumour marker – CD 117
Main symptom- hemorrhage
CECT for primary and PET for secondary
Treatment is Surgery (Imatinib for inoperable of adjuvant therapy)

Gastric Lymphoma:
******ebook converter DEMO Watermarks*******
 Stomach is the commonest extranodal primary site for non-Hodgkin’s lymphomain GIT.
Accounts for approximately 4% of gastric malignancies mainly seen in atrum.
The most common gastric lymphoma is diffuse large B-cell lymphoma (DLCL- 55%), followed by extranodal
marginal cell lymphoma (MALT) (40%), Burkitt’s lymphoma (3%), and mantle cell and follicular lymphomas
(each <1%).
Common between 50-70 years.
Males > females.
Clinically presents similar to gastric carcinoma:Pain (Commonest, early satiety and fatigue).
Anaemia is common due to occult bleesing (Overt bleeding is rare).
70% of tumours are resectable.
5-year survival is approximately 25%
Both adjuvant radiotherapy and chemotherapy may be useful.
Gastric lymphoma may be an isolated lesion or part of a disseminated process; it is being seen with increased
frequency in AIDS.
Malignant lymphomas of mucosa-associated lymphoid tissue (MALTomas), comprising diffuse sheets of
maturing lymphocytes are the most common, but the full range of cytological patterns may occur, including
Hodgkin’s disease.
A rare form of ‘so-called’ benign, follicular, lymphoid hyperplasia (pseudolymphoma) has now been shown by
immunocytochemistry to be monoclonal and hence a true lymphoma

Treatment:
Low grade MALtoma:

only in gastric wall (no t 11:18 translocation)- H pylori eradication then re-evaluation afer 1 year.
Node involvement or t 1: 18 translocation- H pylori eradication then re-evaluation after 3-6 months. If persists
then RT for stage 1 and CT + surgery for stage II
Stage III or IV: H pylori eradication followed by CT (+/- RT)

High grade MAlToma:

Stage I, II, III, IV- CT + RT

Residual tumour: Further CT and surgery

Chemotherapy- CHOP (Cyclophosphamide, Doxorubicin, Vincristine, Predinisolone) + Rituximab.

(Rituximab is monoclonal Ab aginst CD 20).
Indications of surgery are:

Hemorrhage
Obstruction
Perforation
Rsidual disease after CT
Confined to bowel wall (Ann Arbor stage IE)

The presence of transmural tumor extension, nodal involvement, transformation into a large cell phenotype,
t(11;18), or nuclear Bcl-10 expression all predict failure after H. pylori eradication alone
Hodgkin’s Lymphoma:

<1% of all primary. Stomach is most common site.

Associated with EB Virus
LMP1 (Latent membrane protein 1), an essential EBV protein commonly expressed.
Hodgkins confined to stomach is treated by Surgery (+/- post op CT).
Prognosis – poor (40-50% death within 1 year).

******ebook converter DEMO Watermarks*******

 Points to remember about Gastric lymphoma
Stoamch most common site for GI lymphoma
Most are Diffuse large cell lymphoma >MALToma
Pain main presenting smptom
Treated by H plorieradiaction and CT (CHOP)

Congenital Hypertrophic Pyloric Stenosis:

This condition is second only to inguinal hernia as a reason for surgical intervention during the first year of life.
Results in hypertrophy and hyperplasia of pyloric sphincter in neonatal period.
Mainly affects circular muscle fibres of pylorus.
Associated anomay (5-20%)- Esophageal atresia, Hirschprung’s disease, ARM and malrotation.
More common in whites (Scandinavian), least common in Asians, blacks and Chinese.
Erythromycin given within 1 week after birth increases he chnces of CHPS.
Pylorus becomes elongated and thickened (? Due to failure of nitric oxide synthesis).
Results in gastric outflow obstruction, vomiting and dehydration.
Affects 3 per 1000 live births.
Male: female 4:1. Most common in first born males.
Usually presents between 4 and 6 weeks (3-8) of age.
Child hungry and often feeds immediately after vomiting.
Biochemically a hypochloraemic alkalosis exists (with hypokalemia and paradoxical aceduria).
Non-bilious vomiting, associated with weight loss and dehydration, are typical.
The upper abdomen may be distended with visible gastric peristalsis and a palpable olive shaped lump
representing the hypertrophied pylorus.
Features of protein energy malneutrition is absent.
confirmation of the diagnosis is by ultrasound scanning. – Length of pylorus > 16 mm, width > 4 mm has a
accuracy of > 95%. Stomach is empty doesn’t have food residue.
First electrolyte imbalance correction is must by – N/2 (.45% saline) with 2.5% dextrose and Kcl or RL.
At laparotomy, pyloromyotomy (Ramstedt’s operation) is the usual procedure done through right
hypochondrial transverse muscle splitting incision.
Aropin is medical management (Not preferred).

Gastric Outlet Obstruction:

Clinical entities that can result in GOO generally are categorized into 2 groups of causes—benign and malignant.
Etiology:
The major benign causes of GOO are PUD, gastric polyps pyloric stenosis, congenital duodenal webs, gallstone
obstruction (Bouveret syndrome), pancreatic pseudocysts, and bezoars.
Within the pediatric population, pyloric stenosis constitutes the most important cause of GOO.
Pancreatic cancer is the most common malignancy causing GOO (may occur in 10-20% of pancreatic carcinoma).
Other tumors that may obstruct the gastric outlet include ampullary cancer, duodenal cancer, cholangiocarcinomas,
and gastric cancer.
Clinical:

Nausea and vomiting, usually nonbilious, and it characteristically contains undigested food particles. Early
satiety and epigastric fullness are common. Weight loss is frequent. Abdominal pain is not frequent and usually
relates to the underlying cause, eg, PUD, pancreatic cancer.
Physical examination demonstrates the presence of chronic dehydration and malnutrition. A dilated stomach
may be appreciated as a tympanitic mass in the epigastric area.
Patients with GOO due to benign ulcer disease may be treated medically if results of imaging studies or
endoscopy determine that acute inflammation and edema are the principle causes of the outlet obstruction (as
******ebook converter DEMO Watermarks*******
 opposed to scarring and fibrosis, which may be fixed).
If medical therapy conducted for a reasonable period fails to alleviate the obstruction, then surgical intervention
becomes appropriate. The choice of surgical procedure is vagotomy and antrectomy, against which the efficacy
of other procedures should be measured.

Diagnostic Procedures:

Upper endoscopy can help visualize the gastric outlet and may provide a tissue diagnosis when the obstruction
is intraluminal.
Nuclear gastric emptying studies measure the passage of orally administered radionuclide over time.
Unfortunately, both the nuclear test and saline load test may produce abnormal results in functional states.
Barium upper GI studies are very helpful because they can delineate the gastric silhouette and demonstrate the
site of obstruction.

Treatment:
Medical therapy:
Initial management of GOO should be the same regardless of the primary cause i.e. hydration and correction of
electrolyte abnormalities. Sodium chloride solution should be the initial IV fluid of choice. Potassium deficits are
corrected after repletion of volume status, and after the chloride has been replaced.
Surgical therapy:
Management of benign disease:
The most common surgical procedures performed for GOO related to PUD are vagotomy and antrectomy, vagotomy
and pyloroplasty, truncal vagotomy and gastrojejunostomy, pyloroplasty, and laparoscopic variants of the
aforementioned procedures. Of these, vagotomy and antrectomy with Billroth II reconstruction
(gastrojejunostomy) seems to offer the best results. A combination of balloon dilatation and highly selective
vagotomy has been described, but it is associated with gastroparesis and a high recurrence rate.
Management of malignant disease:
Of patients with periampullary cancer, 30-50% present with nausea and vomiting at the time of diagnosis. Most of
these tumors are unresectable (approximately 40% of the gastric cancers and 80-90% of the periampullary cancers).
Gastrojejunostomy remains the surgical treatment of choice for GOO secondary to malignancy. Traditionally an
antecolic anastomosis has been performed to prevent further obstruction by advancing tumor growth, recent studies
favoursretrocolic anastomosis.
Gastric Volvulus:
Gastric volvulus is defined as an abnormal rotation of the stomach of more than 180°, creating a closed loop
obstruction that can result in incarceration and strangulation.
According to the axis of rotation, gastric volvulus is classified into the following:

Organo-axial: The stomach rotates around an axis that connects the gastroesophageal junction and the pylorus.
This is the most common type in both children and adults. It is usually associated with diaphragmatic defects.
Strangulation and necrosis occurs commonly with this type and has been reported in 5-28% of cases.
Mesenterico-axial: Here the axis bisects both the lesser and greater curvatures. The antrum rotates anteriorly
and superiorly so that the posterior surface of the stomach lies anteriorly. The rotation is usually incomplete
and occurs intermittently. Vascular compromise is uncommon. This type usually presents without
diaphragmatic defects and usually manifests with chronic symptoms.
Combined: This is a rare form in which the stomach twists both mesentero- and organo-axially and usually is
seen in patients with chronic volvulus.

Etiology:
According to etiology, gastric volvulus can be classified as either type 1 or 2.
******ebook converter DEMO Watermarks*******
Type 1, or idiopathic: This type comprises two-thirds of cases and is presumed to be due to abnormal laxity of the
gastro-splenic, gastro-duodenal, gastro-phrenic, and gastro-hepatic ligaments. This allows approximation of the
cardia and pylorus when the stomach is full, predisposing to volvulus. This type is more common in adults.
The most common cause of gastric volvulus in adults is diaphragmatic defects. In cases of paraesophageal hernias,
the gastroesophageal junction remains in the abdomen while the stomach ascends adjacent to the esophagus,
resulting in an upside-down stomach. Gastric volvulus is the most common complication of paraesophageal hernias.
Clinical:
Acute gastric volvulus:

Intra-abdominal gastric volvulus presents most commonly with the sudden onset of severe epigastric or left
upper quadrant pain.
Intra-thoracic gastric volvulus presents with sharp chest pain radiating to the left side of the neck, shoulder,
arms, and back.
Occasionally, some patients present with hematemesis secondary to mucosal ischemia and sloughing.
Borchardt’s triad (pain, retching, and inability to pass a nasogastric tube) is diagnostic of acute volvulus
and has been reported to occur in 70% of cases.

Chronic gastric volvulus:

Intermittent epigastric pain and abdominal fullness following meals.

Early satiety, dyspnea, and chest discomfort.
Dysphagia may occur if the gastroesophageal junction is distorted.

Imaging Studies:
Chest x-ray: A retrocardiac gas-filled viscus in cases of intrathoracic stomach confirms the diagnosis.
Plain abdominal radiograph reveals a massively distended viscus in the upper abdomen.
Barium study may be valuable in chronic volvulus with the stomach lying horizontal or upside down.

Treatment:
Medical therapy:
******ebook converter DEMO Watermarks*******
Endoscopic reduction can be attempted in selected cases.
Surgical therapy:
Emergent surgical intervention is indicated for acute gastric volvulus. With chronic gastric volvulus, surgery is
performed to prevent complications.
The strategy of surgery is as follows:

Reduction of the volvulus

Assessment of gastric viability with resection of the gangrenous portions by segmental, subtotal, or total
gastrectomy
Prevention of recurrence by anterior gastropexy, which is most often accomplished with a gastrostomy tube

Complications:
Strangulation and necrosis occur most commonly with organo-axial gastric volvulus and occur in 5-28% of cases.
Gastric perforation occurs secondary to ischemia and necrosis. It can also complicate endoscopic reduction.
Duodenum:
The duodenum is approximately 25cm long and its configuration divides it into four parts: the duodenal bulb, and
the descending, horizontal and ascending portions.
In the submucosa, and peculiar to the duodenum, are Brunner’s glands. These mucus-secreting glands are most
numerous in the first part of the duodenum. Paneth cells and cells of the APUD system are also found within the
crypts.
Duodenal Diverticula:
Duodenal diverticula may be acquired or congenital. Acquired or Pseudodiverticula are the product of the scarring
of chronic peptic ulceration in the proximal duodenum. Congenital examples typically arise from the second part of
the duodenum; the ampulla of Vater is usually closely adjacent, and may lie within the diverticulum. There is a
definite but unexplained asssociation between common bile duct stones and duodenal diverticula. Stasis within large
and/or multiple diverticula of the duodenum.Treatment is by choleduodenostomy.
Congenital Duodenal Obstruction:
Congenital obstruction of the duodenum varies from a simple stenosis or diaphragm partially obstructing the lumen,
to a complete block with a gap between the two ends of bowel (duodenal atresia). The site of obstruction is distal to
the ampulla of Vater in about 80% of cases. Vomiting, which occurs within a few hours of birth, is of bile-stained
fluid. Its common with Down’s syndrome(25-33%). (Anomaly associated with down synd. are: 11 or 13 ribs,
duodenal atresia / stenosis, tracheo-esophageal fistula, Hirschsprungdis)

******ebook converter DEMO Watermarks*******

The typical double bubble’ sign with absence of distal abdominal gas may be seen on abdominal radiograph (Other
less common causes of the “double bubble” include annular pancreas, and peritoneal bands). Obstetric ultrasound
shows polyhydramnios (in 50%).
Management varies accordingly to the type of stenosis: Ladd’s bands are lysed. Pure stenosis is opened
longitudinally and closed transversely (Heineke-Mickulicz). Membranous stenosis is resected. Duodeno-
duodenostomy is the procedure of choice for annular pancreas. Duodenojejunostomy has higher risk of long-term
complications.
Annular pancreas: A congenital anomaly characterized by a ring of normal pancreatic tissue encircling and
sometimes obstructing the descending part of the duodenum. The annulus represents the ventral part of the pancreas
that remains fixed to the duodenum. In the extramural type, the annulus is drained by ducts running around the
duodenum to join the main pancreatic duct. Associated features with Annular pancreas are: Absent spleen,
Duodenal stenosis, Ectopic spleen, Polysplenia, Situs inversus-abdominal, Small bowel atresia/ obstruction.
Superior mesenteric artery (SMA) syndrome (also known as Wilkie syndrome):
Is a clinical entity characterized by compression of the third, or transverse, portion of the duodenum against the
aorta by the SMA, resulting in chronic, intermittent, or acute, complete or partial duodenal obstructions More
common in thin females of young age with less amount or retroperitoneal fat to support duodenum.
Main causes are.

Thin body build.

Exaggerated lumbar lordosis.
Anorexia nervosa.

CT-Angiography or magnetic resonance angiography (MRA) enables visualization of vascular compression of the
duodenum and measurement of aortomesenteric distance precisely.

The superior mesenteric artery usually forms an angle of approximately 45° (range, 38-56°) with the abdominal
aorta and aortomesenteric distance is normally 10-20 mm.
CT criteria for the diagnosis of superior mesenteric artery syndrome include an aortomesenteric angle of less
than 22 degrees and an aortomesenteric distance of less than 8-10 mm. In children, an angle of less than 20° has
been correlated with superior mesenteric artery syndrome.

At least 70% of cases can typically be treated with medical treatment, The goal of medical treatment for SMA
Syndrome is resolution of underlying conditions and weight gain
In case surgical treatment is required then duodenojejunostomy is effective in the majority of patients.

******ebook converter DEMO Watermarks*******

Intestine:
Anatomy:
Gross Anatomy:

The small intestine in an adult is 5–6 m long from the ligament of Treitz to the ileocecal valve.
The upper two fifths of the small intestine distal to the duodenum is termed the jejunum, and the lower three
fifths is the ileum.
There is no sharp demarcation between the jejunum and the ileum; however, as the intestine proceeds distally,
the lumen narrows, the mesenteric vascular arcades become more complex, and the circular mucosal folds
become shorter and fewer.
The mesentery contains fat, blood vessels, lymphatics, lymph nodes, and nerves.
The arterial blood supply to the jejunum and ileum derives from the superior mesenteric artery.
Branches within the mesentery anastomose to form arcades, and small straight arteries travel from these arcades
to enter the mesenteric border of the gut.
The antimesenteric border of the intestinal wall is less richly supplied with arterial blood than the mesenteric
side, so when blood flow is impaired, the antimesenteric border becomes ischemic first.
Venous blood from the small intestine drains into the superior mesenteric vein and then enters the liver through
the portal vein.
Submucosal lymphoid aggregates (Peyer patches) are much more numerous in the ileum than in the jejunum.
Lymphatic channels within the mesentery drain through regional lymph nodes and terminate in the cisterna
chyli.
Parasympathetic nerves from the right vagus and sympathetic fibers from the greater and lesser splanchnic
nerves reach the small intestine through the mesentery.
******ebook converter DEMO Watermarks*******
 Both types of autonomic nerves contain efferent and afferent fibers, but intestinal pain appears to be
mediated by the sympathetic afferents only.

Microscopic Anatomy:

The wall of the small intestine consists of four layers: mucosa, submucosa, muscularis, and serosa.

Mucosa:

The absorptive surface of the mucosa is multiplied by circular mucosal folds termed plicae circulares (valvulae
conniventes) that project into the lumen.
They are taller and more numerous in the proximal jejunum than in the distal ileum.
On the surface of the plicae circulares are delicate villi less than 1 mm in height, each containing a central
lacteal, a small artery and vein, and fibers from the muscularis mucosae that lend contractility to the villus.
Villi are in turn covered by columnar epithelial cells that have a brush border consisting of microvilli 1 µm in
height.
The presence of villi multiplies the absorptive surface about 8 times, and microvilli increase it another 14–24
times; the total absorptive area of the small intestine is 200–500 m2.
The major cell types in the epithelium of the small intestine are absorptive enterocytes, mucous cells, Paneth
cells, endocrine cells, and M cells.
Absorptive enterocytes are responsible for absorption; they arise from continually proliferating undifferentiated
cells in the crypts of Lieberkühn and migrate to the tips of villi over a 3–7-day period. Peptide growth factors
regulate this process.
The life span of enterocytes in humans is 5–6 days.
Mucous cells originate in crypts and migrate to the tips of villi also; mature mucous cells are termed goblet
cells.
Paneth cells are found only in the crypts; their function is unknown but may be secretory. Endocrine cells have
abundant cytoplasmic granules that contain 5-hydroxtryptamine and various peptides.
Enterochromaffin cells are the most numerous; N cells (containing neurotensin), L cells (glucagon), and other
******ebook converter DEMO Watermarks*******
 cells containing motilin and cholecystokinin are also present. M cells are thin membranous cells that cover
Peyer patches.
They have the ability to sample luminal antigens such as proteins and microorganisms. Mucosal T lymphocytes
of several phenotypes play an important role in mucosal cell–mediated immunity. Mast cells in the lamina
propria are closely applied to nerve fibers, thus providing an anatomic basis for communication between these
two structures in disease processes such as inflammation.

Other Layers:

The submucosa is a fibroelastic layer containing blood vessels and nerves.

Submucosa is the strongest component of bowel wall and must be included in intestinal sutures.
The muscularis consists of an inner circular layer and an outer longitudinal coat of smooth muscle.
The serosa is the outermost covering of the intestine.

Intestinal Obstruction:
Mechanical obstruction may be :
1. Within the lumen.
2. Within the bowel wall.
3. Extrinsic (as in the case of adhesions and herniae), and it has to be differentiated from functional causes or
pseudo-obstruction, and from the paralytic ileus.
Pathophysiology:

The small bowel proximal to a point of obstruction distends with gas and fluid.
Swallowed air is the major source of gaseous distention, at least in the early stages, because nitrogen is not
well absorbed by mucosa.
When bacterial fermentation occurs later on, other gases are produced; the partial pressure of nitrogen within
the lumen is lowered, and a gradient for diffusion of nitrogen from blood to lumen is established.
Enormous quantities of fluid from the extracellular space are lost into the gut and from the serosa into the
peritoneal cavity.
Fluid fills the lumen proximal to the obstruction, because the bidirectional flux of salt and water is disrupted
and net secretion is enhanced. Mediator substances (eg, endotoxin, prostaglandins) released from proliferating
bacteria in the static luminal contents are responsible.
Reflexly induced vomiting accentuates the fluid and electrolyte deficit.
Hypovolemia leads to multiorgan system failure and is the cause of death in patients with nonstrangulating
obstruction.

Causes:

Postoperative adhesionsis the most common cause overall. Can cause acute obstruction within 4 weeks;
chronic obstruction may occur decades later.
******ebook converter DEMO Watermarks*******
 Other etiologies include malignant tumors (20%), hernias (10%), inflammatory bowel disease (5%), volvulus
(3%), and miscellaneous causes (2%).
The causes of small bowel obstruction in pediatric patients include congenital atresia, pyloric stenosis, and
intussusception.

Presentation:

The associated clinical features vary according to the site of obstruction: if the obstruction is high, then pain
and bilious vomiting with little distension will predominate.
lower small bowel obstruction is more often associated with distension and faeculent vomiting.
Peristalsis may be visible in thin patients until motility becomes impaired, and examination may demonstrate
the cause of the obstruction.
The plain radiograph taken in supine view is usually confirmatory.
Dilated small bowel loops with air fluid levels indicate SBO.
Small bowel distension is differentiated radiologically from colonic distension by the outlining of the valvulae
conniventes by intestinal gas, and by the distribution of distended loops, which mainly occupy the centre of the
abdomen.
in small bowel obstruction of mechanical cause there is usually little or no colonic gas. Absent or minimal
colonic gas indicates SBO.
CECT is sometimes indicated and may reveals both the anatomical site of obstruction and its etiology.
Obstruction is present if the small bowel loop is greater than 3 cm in diameter dilated proximal to a distinct
transition zone of collapsed bowel less than 1 cm in diameter.
A smooth beak indicates simple obstruction without vascular compromise; a serrated beak may indicate
strangulation.

Treatment:

Partial small bowel obstruction can be treated expectantly (non-surgically) as long as there is continued passage
of stool and flatus.
Plain abdominal x-rays show gas in the colon, and small bowel contrast x-rays prove the diagnosis.
Decompression with a nasogastric tube is successful in 90% of such patients.
Operation may be required if obstruction persists for several days even though it is incomplete.
Complete obstruction of the small intestine is treated by operation after a period of careful preparation.
The compelling reason for operation is that strangulation cannot be excluded with certainty, and strangulation
is associated with high rates of complications and death.
There are exceptions to the general rule that operation must be performed promptly: Incomplete
obstruction, postoperative obstruction, a history of numerous previous operations for obstruction,
radiation therapy, inflammatory bowel disease, and abdominal carcinomatosis are situations demanding
mature judgment, and judicious nonoperative management may be in the patient’s best interests. A long
intestinal tube (eg, Miller-Abbott tube) may be passed in these cases to decompress the intestine.

Preparation:
The risk of strangulation must be weighed against the severity of fluid and electrolyte abnormalities and the need for

******ebook converter DEMO Watermarks*******

evaluation and treatment of associated systemic diseases.

Nasogastric Suction.
Fluid and Electrolyte Resuscitation.
Operation.
Operation may commence when the patient has been rehydrated and vital organs are functioning
satisfactorily.
Laparoscopic adhesiolysis may be performed in carefully selected patients by surgeons skilled in this
procedure. Generally, however, an open procedure is performed through an incision that is partly dictated
by the location of scars from previous operations.
Details of the operative procedure vary according to the cause of obstruction. Adhesive bands causing
obstruction should be lysed; an obstructing tumor should be resected; and an obstructing foreign body
should be removed through an enterotomy. Gangrenous intestine must be resected, but it may be difficult
to determine whether obstructed bowel is viable or not. The loop should be wrapped in a warm saline-
soaked pack and inspected for color, mesenteric pulsation, and peristalsis several minutes later.
Intraoperative use of Doppler ultrasound is a method of determining viability of obstructed intestine. The
qualitative fluorescein test may be helpful; 1000 mg of fluorescein is injected into a peripheral vein over
30–60 s, and the bowel is then inspected under ultraviolet (Wood) light. If the loop appears nonviable,
resection with end-to-end anastomosis is the safest course.
Extirpation of the obstructing lesion is not possible in some patients with carcinoma or radiation injury.
Anastomosis of proximal small bowel to small or large intestine distal to the obstruction (bypass) may be
the best procedure in these patients. Rarely, adhesions are so dense that the intestine cannot be freed and
bypass cannot be accomplished. Prolonged decompression through a gastrostomy or jejunostomy tube and
provision of nutrition via the parenteral route may allow spontaneous resolution over a period of a few
weeks.
Decompression of massively dilated small bowel loops facilitates closure of the abdomen and may shorten
the time for recovery of bowel function postoperatively. Decompression is accomplished by threading
down a long tube passed orally or by needle aspiration through the bowel wall.
Attempts to prevent uncontrolled adhesion formation by suturing loops of bowel so that they are fixed in a
suitable relation to one another (Nobel plication procedures) are unsuccessful. However, another
procedure in which a long tube is inserted through a gastrostomy or jejunostomy for 10 days to provide
intraluminal stenting has some proponents. Adhesion prevention with a hyaluronic acid methylcellulose
bioabsorbable barrier has proved effective in decreasing adhesion formation and decreasing reoperative
times. Studies proving efficacy in reducing the incidence of small bowel obstructions have shown a small
benefit.

Small Bowel Carcinoma:

Adenocarcinomas of the small bowel constitute less than 2% of all gastrointestinal malignancies.
The duodenum is most often affected, and 90% of carcinomas occur within 20cm of the ligament of Treitz.
A number of conditions predispose to intestinal carcinoma, the most important of which are coeliac disease and
familial adenomatous polyposis,Peutz-Jeghers syndrome and Crohn’s disease.
******ebook converter DEMO Watermarks*******
 Presentation is usually in the 6th and 7th decades.
Bleeding is common.
The diagnosis may be apparent from barium studies or CT scan.
Macroscopically, a polypoid pattern is commonest, and this often leads to bleeding or intussusception, but
sessile, stenosing, and ulcerating tumours are also seen. The histological appearances are those of
gastrointestinal adenocarcinoma.

Peutz-Jeghers Syndrome:

Peutz-Jeghers syndrome is inherited as an autosomal dominant condition.

Multiple hamartomatous polyps occur throughout the gastrointestinal tract and are accompanied by
pigmentation of the lips and buccal mucosa.
Symptoms are relatively unusual, but are most likely from small bowel polyps which may bleed or cause
obstruction or intussusception.
Histologically, the lesions have a lobulated surface with a core of muscle fibres (derived from the muscularis
mucosae) which arborizes around the crypts and mucosal glands, thinning out towards the surface.
There is an associated increased risk of malignancy in the small intestine and other sites.

Meckel’s Diverticulum:

The Meckel’s diverticulum is the most common congenital anomaly of the gastrointestinal tract, and affects
approximately 2% of normal caucasians.
Meckel’s diverticulum is a true intestinal diverticulum that results from the failure of the vitelline duct to
obliterate during the fifth week of fetal development.
It arises from the antemesenteric border of the ileum, 50-100cm proximal to the ileocaecal valve.
It is usually about 2-5 cm in length and wide-mouthed. In approximately 50% of cases, the mucosa is ileal, but
duodenal, colonic, pancreatic, and particularly gastric mucosa may be present.
Ectopic tissue, found in approximately 50 percent of cases of Meckel’s diverticulum, is most commonly gastric
in origin.
Most remain asymptomatic but bleeding and intestinal obstruction, do occur.
Bleeding is usually the result of ulceration in gastric mucosa and this allows the possibility of diagnosis by
scintigraphy which will usually identify tissue containing parietal cells.

Diagnosis:

The diagnosis cannot be made with plain radiographs, and arteriography is not always diagnostic because
arterial supply is not always abnormal.
Contrast studies such as upper gastrointestinal series with small bowel follow-through are of limited value
because the layers of barium-filled intestine will obstruct the view of the diverticulum. Computed tomographic
scans are often nonspecific but occasionally helpful.
The most useful method of detection of a Meckel’s diverticulum is technetium-99m pertechnetate
scanning. However, the technetium scan depends on uptake by heterotopic gastric mucosa.
Not all diverticula contain ectopic tissue; because complications such as bleeding are often caused by ectopic
gastric tissue, diagnosis may be assisted in symptomatic cases. The accuracy of the scan can be improved with
the use of pentagastrin. Cimetidine improves diagnostic accuracy by inhibiting the intraluminal release of
technetium, and glucagon does so as an antiperistaltic.

Complications and Treatment:

Bleeding is the most common complication: from ileal ulcers

Others:

Obstruction.
intussusception.
diverticulitis.
******ebook converter DEMO Watermarks*******
 perforation may also occur, especially in adults.

Intestinal obstruction is a dangerous complication, since torsion and gangrene can be fatal if early operation is not
done.

A bleeding diverticulum with an indurated area in the adjacent ileum requires resection of this section of the
bowel and the diverticulum.
Small, asymptomatic diverticula encountered incidentally at laparotomy need not be removed.
Whenever a normal appendix is found during an exploration for appendicitis, Meckel’s diverticulum should be
suspected.

Hereditary Haemorrhagic Telangiectasia (Osler-Weber-Rendu Syndrome):

It is an autosomal dominant condition with high penetrance. Lesions usually appear in childhood. Telangiectases
are commonly found on the lips, tongue, and oral mucosa and less often on conjunctivae, ears, and digits.
Lesions in the gastrointestinal mucosa often lead to a low-grade iron deficiency, and may be responsible for
troublesome haemorrhage, because their number and extensive distribution throughout the intestine makes a surgical
approach impractical.
Carcinoid Tumours:
Carcinoid tumours occur most often in the ileum and appendix, and are usually chance findings at appendicectomy.
The tumour arise from the argentaffin of Kulchitzky cells which lie deep in the crypts of Lieberkühn and are derived
from neural crest tissue. Like other APUD tumours, carcinoids contain numerous neurosecretory granules.
Large tumours may cause obstruction or intussusception, but symptoms are otherwise rare unless metastases to the
liver are responsible for the carcinoid syndrome (seen only in 1% of cases). This syndrome is due to the
excessive production of a variety of gut hormones - particularly 5-hydroxytryptamine, but also histamine, kinins,
catecholamines, and prostaglandins.
Typically, patients present with episodic facial flushing, watery diarrhoea, and abdominal cramps, and may
have pellagra-like skin lesions as a result of niacin deficiency. Involvement of the right side of the heart is also
well recognized, and there may be bronchial lesions. Auscultation may indicate pulmonary and/or tricuspid valve
lesions.
Macroscopically, the primary tumours are generally small and extra-appendiceal; and the liver may be almost
completely replaced by metastases. Intestinal biopsy shows solid groups of regular polyhedral cells spreading
through the submucosa and muscle; the specific neuroendocrine characteristics may be determined from
immunocytochemical stains.
Surgical resection is the standard curative modality. If the primary tumor is localized and resectable, 5-year survival
rates are excellent (70%-90%). Radiation therapy has a minor role in patients with regionally unresectable disease
and may palliate the pain of bone metastasis. Patients with carcinoid syndrome can usually be effectively
palliated by injections of somatostatin analogue.
Malrotation of the Gut:

During 6-12 week of gestation, the intestine undergoes evisceration, elongation, and eventual return to the
abdominal cavity in a 270 degree counterclockwise rotation with fixation.

Malrotation frequently causes abnormalities of fixation, which can result in neonatal volvulus, but more often it
causes recurrent abdominal pain in childhood, culminating in obstruction from intestinal volvulus or fibrous bands.
Up to 70% of children with intestinal malrotation also have another congenital (present at birth) malformation.
These include the following:

Abdominal wall defects and digestive system abnormalities, including gastro. schisis, omphalocele, congenital
diaphragmatic hernia, intestinal atresia, Hirschsprung’s disease, gastroesophageal reflux, intussusception, and
anorectal malformations.
Cardiac (heart) abnormalities.

******ebook converter DEMO Watermarks*******

 Abnormalities of the liver or spleen.

Ladds bands extend from the colon to the duodenum, causing duodenal obstruction and biliary emesis.
Obstructive bilious vomiting in a newborn is malrotation until proven otherwise.
Midgut volvulus refers to the narrow based mesentery twisting around the SMA (usually clockwise).
This results in obstruction and vascular compromise.
Most develop symptoms in first month of life.
If patient stable do UGI series (contrast study is gold standard).
See bird’s beak in third part of duodenum.
Ligament of Treitz is right of midline.
Immediate exploration to avoid loss of small bowel and resultant SBS, death.
Surgical treatment is the Ladd’s procedure.
This consists of division of bands, correction of malrotation, restoration of broad based mesentery,
appendectomy (because now it it is in the wrong place in LUQ and may cause confusion in future).

Normal Rotation:

******ebook converter DEMO Watermarks*******

Intussusception:
It occurs when one part of bowel invaginates (intussusceptum) into an adjacent section (intussuscipiens) and results
in intestinal obstruction and venous compression which if uncorrected it can result in arterial insufficiency and
necrosis. Peak incidence is between 6 and 9 months. Frequently occurs after a recent upper respiratory infection, by
Adenovirus type 3 that causes a reactive lymphoid hyperplasia that act as lead point (of Peyer’s patch).5% are due to
Meckel’s diverticulum, polyps, Henoch’s Schonlein purpura, hematoma, lymphoma, foreign bodies, and
duplications. Commonest site involved is the ileocaecal junction
Three types of intussusception can occur:

Ileocolic – the small intestine invaginates into the right colon; this is the most common intussusception
Ileoileal – the small intestine invaginates into itself

******ebook converter DEMO Watermarks*******

 Colocolic – the large intestine invaginates into itself

Clinical features:

Intermittent colicky abdominal pain and vomiting.

Passage of blood - ‘red currant jelly’ per rectum.
Sausage shaped abdominal mass.
Diagnosis confirmed with water soluble contrast enema or ultrasound.

Treatment:

Resuscitation with intravenous fluids and nasogastric tube.

Attempt reduction with air or contrast enema under radiological guidance.
Failure of hydrostatic reduction requires urgent operation through a right lower quadrant horizontal incision.
The intussusception is reduced by pushing on the distal bowel like a tube of toothpaste rather than pulling the
proximal bowel.
If peritonitis, shock or failed reduction requires surgery.
If bowel necrosis requires resection with primary anastomosis.

Meconium Ileus:
Meconium Ileus (MI) is the earliest clinical manifestation of CF and occurs in approximately 10-15% of neonates
with CF2. It occurs due to the increased viscidity of the meconium associated with its high protein and low
carbohydrate content. The most common presentation is abdominal distension with or without bilious vomiting and
a failure or delay in passing meconium after birth. Occasionally it is diagnosed prenatally on ultrasound scans.
MI can be categorised as either simple or complicated. Complicated MI includes those with volvulus, atresia,
perforation or giant cystic peritonitis. Abdominal x-ray is likely to show several loops of dilated small bowel
without air-fluid levels; and possibly a soap bubble appearance in the right lower quadrant, the so-called Neuhauser
sign. Complicated cases may present with greater bowel dilatation and air-fluid levels. Perforation or giant cystic
meconium peritonitis calcifications are often noted. Most babies then undergo barium enema to confirm the
diagnosis. Since the publication of Noblett’s report on the use of gastrograffin enemas (GGE) in the treatment of
simple MI, non-operative management has become increasingly important. All neonates with complicated and those
with 2 unsuccessful GGE require operative intervention.
The surgical management options of MI are varied and include:
1. Enterotomy / appendicectomy with irrigation.
2. Enterostomy with/out resection.
3. Resection with primary anastomosis.
Treatment is Enterotomy and irrigation for simple MI as they had a 20% leak rate with Bishop-Koop (End-to-distal
side ileal anastomosis with a distal end ileostomy which allows post-operative irrigation of the meconium pellets) or
Santulli (Side-to-end anastomosis with a proximal enterostomy after resection of the dilated segment of bowel)
ileostomies.
Acute Mesenteric Ischemia:
AMI is a syndrome in which inadequate blood flow through the mesenteric circulation causes ischemia and eventual
gangrene of the bowel wall. The syndrome can be classified generally as arterial or venous disease. Arterial disease
can be subdivided into nonocclusive mesenteric ischemia (NOMI) and occlusive mesenteric arterial ischemia
(OMAI).
Practically, AMI is divided into 4 different primary clinical entities: acute mesenteric arterial embolus (AMAE),
acute mesenteric arterial thrombosis (AMAT), NOMI, and mesenteric venous thrombosis (MVT). OMAI includes
both AMAE and AMAT.
Anatomy:
Typically the celiac artery (CA) supplies the foregut, hepatobiliary system, and spleen; the superior mesenteric
artery (SMA) supplies the midgut (ie, small intestine and proximal mid colon); and the inferior mesenteric artery
******ebook converter DEMO Watermarks*******
(IMA) supplies the hindgut (ie, distal colon and rectum. Venous drainage is through the superior mesenteric vein
(SMV), which joins the portal vein.
AMI arises primarily from problems in the SMA circulation or its venous outflow. Collateral circulation from the
CA and IMA may allow sufficient perfusion if flow in the SMA is reduced because of occlusion, low-flow state
(NOMI), or venous occlusion. The inferior mesenteric artery seldom is the site of lodgment of an embolus. Only
small emboli can enter this vessel because of its smaller lumen. When lodgment occurs, the embolus lodges at the
site of division of the inferior mesenteric artery into the left colic, sigmoidal, and superior hemorrhoidal arteries. In
such instances, collateral flow from the middle colic and middle hemorrhoidal arteries (through the vascular arcades
of the inferior mesenteric artery distal to the embolus) may sustain the perfusion of the left colon.
Pathophysiology: Embolic phenomena account for roughly 50% of all cases (MCQ), arterial thrombosis for
about 25%, NOMI for roughly 20%, and MVT for less than 10%. Hemorrhagic infarction is the common pathologic
pathway whether the occlusion is arterial or venous.
The mucosal barrier becomes disrupted as the ischemia persists, and bacteria, toxins, and vasoactive substances are
released into the systemic circulation. This can cause death from septic shock, cardiac failure, or multisystem organ
failure before bowel necrosis actually occurs. As hypoxic damage worsens, the bowel wall becomes edematous and
cyanotic. Fluid is released into the peritoneal cavity, explaining the serosanguinous fluid sometimes recovered by
diagnostic peritoneal lavage. Bowel necrosis can occur in 8-12 hours from the onset of symptoms. Transmural
necrosis leads to peritoneal signs and heralds a much worse prognosis.
NOMI is precipitated by a severe reduction in mesenteric perfusion, with secondary arterial spasm from such causes
as cardiac failure, septic shock, hypovolemia, or the use of potent vasopressors in patients in critical condition.
History: The most important finding is pain disproportionate to physical examination findings. Typically, pain is
moderate to severe, diffuse, nonlocalized, constant, and sometimes colicky.
Onset varies from type to type. Nausea and vomiting are found in 75% of affected patients. Anorexia and diarrhea
progressing to obstipation are also common. Abdominal distension and GI bleeding are the primary symptoms in up
to 25% of patients. Pain may be unresponsive to narcotics. As the bowel becomes gangrenous, rectal bleeding and
signs of sepsis (eg, tachycardia, tachypnea, hypotension, fever, altered mental status) develop. This syndrome has a
catastrophic outcome if not properly and rapidly treated.
Physical: Early in the course of the disease, in the absence of peritonitis, physical signs are few and nonspecific.
Tenderness is minimal to nonexistent. Stool may be guaiac positive. Peritoneal signs develop late, when infarction
with necrosis or perforation occurs. Signs reflecting risk factors for AMI may be noted. Patients with embolic AMI
may have atrial fibrillation or heart murmurs. Those with thrombotic AMI or NOMI may have an abdominal
murmur or a scar from a recent abdominal aortic repair with or without reimplantation of the SMA. Those with
MVT may have evidence of tumor, cirrhosis, DVT, or recent abdominal surgery.
Lab Studies:

CBC count may be within the reference range initially, but the WBC count eventually rises as the disease
progresses. Leukocytosis and/or leftward shift are observed in over 50% of cases. The hematocrit is elevated
initially from hemoconcentration due to third spacing, but it decreases with GI bleeding.
Amylase levels are moderately elevated in over 50% of patients.
Phosphate levels has a sensitivity of only 25-33%.
Lactate is elevated late in the clinical course. Levels that are persistently within the reference range strongly
indicate a diagnosis other than AMI (sensitivity 96%, specificity 60%).

Imaging Studies:
Plain abdominal films:

Plain films are warranted to exclude identifiable causes of abdominal pain such as perforated viscus with free
intraperitoneal air.
Positive findings are usually late and nonspecific and include ileus, small bowel obstruction,
edematous/thickened bowel walls, and paucity of gas in the intestines. More specific signs, such as
pneumatosis intestinalis, ie, submucosal gas; thumbprinting of bowel wall; and portal vein gas, are late
******ebook converter DEMO Watermarks*******
 findings.

Computed tomography scan:

This technique has a sensitivity of 71% and specificity of 92%. It is not as useful as angiography, but it is
noninvasive and preferred for MVT (90% sensitivity).
CT scan may show pneumatosis intestinalis, portal vein gas, bowel wall and/or mesenteric edema, abnormal
gas patterns, thumbprinting, and streaking of mesentery. Bowel wall edema is the most common finding on CT
scan.

Angiography:

Sensitivity is reported to be 88% for AMI.

An embolus appears as a sharp cutoff of flow near the origin of the middle colic artery. Thrombus appears as a
more tapered occlusion near the origin of the SMA. NOMI is characterized by narrowing of the origins of
multiple SMA branches, alternating dilation and narrowing of the intestinal branches (ie, “string of sausages”
sign), spasm of the mesenteric arcades, and impaired filling of the intramural vessels.
Angiography is actually a second-line study in patients with a strong suspicion of MVT because false-negative
findings are common. Findings with MVT include thrombus in the SMV, reflux of contrast into the aorta,
prolonged arterial phase with accumulation of contrast and thickened bowel walls, extravasation of contrast
into bowel lumen, and filling defect in the portal vein or complete lack of venous phase.

Ultrasonography:

Duplex sonography studies are highly specific (92-100%) but not as sensitive (70-89%) compared to
angiography.

Magnetic resonance imaging and/or magnetic resonance angiography:

MRI and MRA provide findings similar to CT scan in AMI. Sensitivity of MRA is 100% and specificity is
91%. MRA is particularly effective for evaluating MVT.

Other Tests:

Intraoperative fluorescein administration: During laparotomy, 1 g of fluorescein is infused. Viable bowel

fluoresces brightly under a Wood lamp.
ECG may show myocardial infarction or atrial fibrillation.

Treatment:
Medical Care: Make all efforts to improve patients’ cardiovascular status. Provide oxygen at 100% or by intubation
if needed. Fluid resuscitation is accomplished with isotonic sodium chloride solution, and blood products are
provided as needed. Adequacy of resuscitation can be monitored by urinary output, central venous pressure, or
Swan-Ganz pressure monitoring. Insert a nasogastric tube, and optimize cardiac status by treating arrhythmia, CHF,
or myocardial infarction. Start broad-spectrum antibiotics early. Provide pain control while maintaining stable blood
pressure.
Angiographically infused papaverine:

Papaverine infused in the affected vessel is useful for all arterial forms of AMI. It relieves reactive vasospasm
in occluded arterial vessels and is the only treatment for NOMI other than resection of gangrenous bowel.

Angiographically infused thrombolytics:

Thrombolytics infused through the angiogram catheter can be a life-saving therapy for selected patients with
embolic AMI.
******ebook converter DEMO Watermarks*******
 Bleeding is the main complication. Thrombolytic administration is risky and should only be undertaken if
peritonitis or other signs of bowel necrosis are absent. It must be started within 8 hours of symptom onset.
If symptoms do not improve within 4 hours or if peritonitis develops, stop the infusion and perform surgery.

Angioplasty after thrombolysis:

A very select group of patients who have atherosclerotic plaques at the origin of the SMA after thrombolysis are
eligible for angioplasty.
Heparin for MVT:

Heparin anticoagulation is the main treatment for MVT.

Administer heparin as a bolus of 80 U/kg, not to exceed 5000 U, and then as an infusion at 18 U/kg/h until full
conversion to oral warfarin. Appropriate monitoring of anticoagulation using activated partial thromboplastin
time (aPTT) is mandatory.

Surgical Care: Prompt laparotomy is indicated in patients with suspected AMI when expeditious angiography is not
available. A second-look procedure is indicated whenever bowel of questionable viability is not resected.
Preoperative care: Stabilize patients using IV fluids, antibiotics covering the colonic flora, nasogastric tube
decompression, and bladder catheterization, with heparin or papaverine administered as indicated. Blood should be
available.
Operative care: All types of AMI may require resection of necrotic bowel if signs of peritonitis develop.
Differentiation of nonviable versus viable bowel can be enhanced by intraoperative fluorescein use. Because of fat
absorption, fluorescein can be used only once. Most patients can benefit from a 24- to 48-hour second-look
operation to assess for viability of remaining bowel.
Gastrointestinal Stromal Tumors:
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract.
Overall, GISTs are third in prevalence after adenocarcinomas and lymphomas among the histologic types of
gastrointestinal tract tumors.
Historically, these lesions were classified as leiomyomas or leiomyosarcomas because they possessed smooth
muscle features when examined under light microscopy but it lacks ultrastructural and immunohistochemical
features characteristic of smooth muscle differentiation, as are seen in leiomyomas and leiomyosarcomas. Therefore,
the determination was made that GISTs do not arise from smooth muscle cells, but from another mesenchymal
derivative such as the progenitors of spindle and epithelioid cells and the actual cell of origin of GISTs is a
pluripotential mesenchymal stem cell programmed to differentiate into the interstitial cell of Cajal.
Pathophysiology: They are submucosal lesions, which most frequently grow endophytically in parallel with the
lumen of the affected structure. Approximately 50-70% of GISTs originate in the stomach. The small intestine is the
second most common location, with 20-30% of GISTs arising from the jejunoileum. Less frequent sites of
occurrence include the colon and rectum (5-15%) and esophagus (<5%).
Outcomes in patients with GISTs are highly dependent on the clinical presentation and the histopathological features
of the tumor. The overall 5-year survival rate ranges from 28-60%. This can be stratified for patients presenting with
localized primary disease and those presenting with metastatic or recurrent disease. The median survival rate in the
former group is 5 years, while the median survival rate in the latter group is approximately 10-20 months.
Tumors can be classified into high- and low-risk categories based on size and mitotic activity.
GISTs are most commonly diagnosed in 55-65 yrs.
Causes:

Gain-of-function mutations in exon 11 of the c-kit proto-oncogene are associated with most GISTs. These
mutations lead to constitutive overexpression and autophosphorylation of c-Kit, provoking a cascade of
intracellular signaling that propels cells toward proliferation or away from apoptotic pathways.
The c-kit proto-oncogene is located on chromosome arm 4q11-12. It encodes KIT, which is a transmembrane
tyrosine kinase.
******ebook converter DEMO Watermarks*******
 A small minority of GISTs are associated with hereditary syndromes.
GISTs occur with a higher than expected frequency in patients with type 1 neurofibromatosis.
GISTs are also a feature of the rare Carney triad, which is observed predominantly in young women. This
triad consists of epithelioid gastric stromal tumors, pulmonary chondromas, and extra-adrenal
paragangliomas.

Histologic Findings: The morphologic features that appear to be most predictive of outcome and biological
behavior are tumor size and the mitotic rate. Unfortunately, no absolute determinations can be made because even
small lesions with low mitotic rates can metastasize or behave in a locally aggressive fashion. GISTs typically stain
intensely for the CD117 molecule, which is an epitope of KIT. In contrast, desmoids, schwannomas (S-100–
positive, KIT-negative), leiomyomas, and leiomyosarcomas (desmin-positive, KIT-negative) do not. In GISTS,
according to Fletcher et al, CD117 appears diffusely in the cytoplasm in a punctate or Golgilike pattern. CD34
staining results are also positive in approximately 60% of GISTs.
Treatment:
The only effective, specific, nonsurgical therapy for GISTs is imatinib mesylate.
Surgery is the definitive therapy for patients with GISTs. Radical and complete surgical extirpation offers the only
chance for cure.
Certain Important points about GUT
Causes of mechanical Bowel obstruction:

Types: Based on site and physiology

1. Acute- Small bowel obstruction (most common cause- Post operative adhesion then hernia).
2. Subacute (Acute on chronic)- S I obstruction (Most common cause again is adhesion)
3. Chronic- Large bowel obstruction (most common cause- malignancy)
Simple obstruction: obstruction at one site. No vascular compromise
Strangulated obstruction:Obstruction at two points. Vascular compromise between 2 points.
There is dilation proximal to obstruction due to:
1. Gases - Swallowed air (maximum contribution) mainly nitrogen as C02 is absorbed.
H2S produced by bacteria.
2. Fluid-Fluid secretion / Decreased absorption / (Volume produced in GIT-Saliva- 1000-1500 ml, gastric- 1500-
2500 ml, Bile- 1000 ml, Pancreas- 1500 ml, Succus enterricus- 3000 ml).
Clinical feature:
Acute: 1st sympt- Pain, (Other- vomiting, distention, constipation, dehydration)
Chronic/ Large bowel- 1st sympt- Distention and constipation (Other- pain and vomiting are delayed features,
distention is peripheral).
With strangulation, pain becomes constant. With perforation, features of peritonitis seen (rebound tenderness is
pathognomic sign of peritonitis) and finally shock and Hippocartic face (seen in terminal phase of perotinitis).
******ebook converter DEMO Watermarks*******
Investigations:
X ray abdomen:
1. Presence of multiple air-fluid level. – Step ladder pattern.
2. Double bubble sign (duodenum atresia).
3. 3-5 levels Jejunum.
4. Multiple air fluid level- Ileum.
5. Concertina effect- Jejunal loop.
Supine view gives diagnostic site and level of obstruction.
Differences:
1. Jejunal- Volvular coniventes.
2. Ileum- Characterless.
3. Colon- Haustra.
Normal air-fluid level:
1. Duodenal cap.
2. Terminal ileum.
3. Gastric fundus.
Distinction between small and large bowel dilatation:

Paralytic Ileus:
Small multiple air fluid levels/ Gas seen till rectum/ No site of obstruction seen.
X-ray abdomen erect to see gas under right diaphragm - 75% accurate, (X ray chest is 80% accurate). In abdominal
X rays, Left lateral decubitus is more accurate than Erect view.
Treatment:
Sub acute obstruction (SAO)/ partial obstruction – is managed conservatively by NPO, Naso-gastric aspiration by
Ryles tube, Broad spectrum anti-biotics, IV fluids and monitoring.
Surgery is done when patient fails to pass flatus/ faeces for 12 hours or strangulation develops.

Duodenal atresia is most common congenital site of obstruction.

Intussusception is most common cause of paediatric obstruction.
Meckel’s is most common congenital GI anomaly.
Treatment of uncomplicated Muconium ileus- Gastrograffic enema (2 attempts permissible).
Treatment of complicated meconium ileus- Surgery (Bishop Koop operation)
Malrotation- Is intrinsic with Omphalocele, gastroschisis and diaphragmatic hernia.

Reduction of bowel sequence is- First duodenum then ileum than jejunum.
Gene with muconiun ilus- delta F 501 (Cystic fibrosis).

******ebook converter DEMO Watermarks*******

Peritoneum: Mesentry: Omentum

Largest cavity in body is peritoneal cavity

Organ failure in following order- Lung> Kidney > Heart > GIT Sensitive- Anterior parietal peritoneum is most
sensitive and pelvic peritoneium is leas sensitive.
Healing in parietal peritoneum is by metamorphosis of mesenchymal cells.
Organism in peritonitis- Aerobic commonest- E. coli. Anaerobic commonest- Bacteroids (Bacteroids are
commonest in perforated appendix).
IOC of intra-abdominal abscess is CT scan (Gallium 67 helps in locatization but takes more time).
Spontaneous bacterial peritonitis:
In adults mainly due to cirrhosis (commonest now) and in children due to nephrotic syndrome (commonest
previously).
Organism- Coliform (Now). Pneumococci (Previously).
Peritonitis due to peritoneal dialysis- Staph aureus an epididymitis.
Typhoid perforation- Usually 3rd week, Typhoid ulcers are longitudinally placed, Ileal. Bleeding seen in 20%.
Periodic peritonitis- treated by colchicine.
Intra-abdominal abscess:
Anaerobic environment.
IOC- CT scan
Commonest site intraperitoneal- Pelvic
Commonest (Supracolic- intra-abdominal)- Subphrenic
Pelvic abscess preents wih muous diarrheoa- treated by perrectal drainage.

Subphrenic abscess- 4 intraperitoneal, 3 extraperitoneal.

a. Left subphrenic.
b. Left posterior intra-peritoneal (Lesser sac)- Commonest cause- Pseudopancreatic cyst.
c. Right subphrenic- Cmmonest cause- ruptured subphrenic abscess.
d. Right subhepatic- (Morrison pouch).
e. Right/ left extraperitoneal.
f. Midline extraperitoneal.
Preferred treatment of intraperitoneal abscess is CT guided percutaneous drainage if fails then open drainage.
Signs of peritonitis is masked by steroid, children and elderly.
Meig’s syndrome- Ascitis and pleural effusion.
Small Bowel/ Mesentery/ Retroperitoneum/ Appendix:
Muconeum peritonitis (Asceptic peritonitis)- remains sterile 3 hours after birth
Pseudomyxoma pritonii-

Due to ruptured mucocele of appendix or pseudomucinous cyst of ovary.

More common in females.
Distended abdomen with no fluid thrill.
Surgery required though recurrence possible.
Seat belt injury- mesenteric tear.
Shifting tenderness- Sign of mesenteric lymphadinitis.
Disease of mistake- Brucellosis.
Commonest mesenteric cyst- Chylolymphatic.
Chylolymphatic- Thin walled, unilocular, separate blood sully from GUT, can be enucleated.
Enterogenous- Thick walled, same blood supply, Resection of bowel required.
Commonest benign mesenteric tumours- Chylous lymphatic cyst > lipoma.
Commonest malignant mesenteric tumours- Lymphoma > Fibrosarcoma.
Most common retroperitoneal cyst- Remnant of Wolffian cyst.

Ideopathic retroperitoneal fibrosis- Non suppurative fibromatosis.

******ebook converter DEMO Watermarks*******
Associated with- Riedel’s thyroidistis/ Sclerosing cholangitis/ Medistinal fibrosis/ Desmoid tumour/ orbital
pseudotumour.
Primary- Idiopathic, called Ormond’s disease.
Inflammatory condition- hr. Pancreatitis, Actinomycosis, TB, Histolasmosis.
Drugs causing RPF- Methysergide (Main), Methyl Dopa, Hydralazine, B-Blocker, bromocriptine, Phenacitine,
Amphetamine.
Cancer association with- breast, stomach an colon, Ca prostate, Carcinoid.
Autoimmune: SLE, PAN, Ankylosing spondylitis.
Causes B/L periureteral fibrosis leading to Anuria (also aorta and IVC).
Age: 40-60 yrs.
Early symptoms are vague (Pain, weight loss, Malaise and HT).
Most common symptom is Obstructive urinary symptom due to BlL ureteric obstruction (75%).
Best noninvasive investigation- CT scan
MRI is preferred if renal function is compromised.
IVP- Hydronephrosis with media deviation of lower ureter (maiden waist deformity).
Treatment:
For primary- ureteric stents and immunosuppression (Penicillamine, Azathioprine, Tamaoxifen, Steroid- PATS or
TAPS).
For ureteric obstruction/ Secondary- Ureterolysis through transperitoneal approach with intra-abdominal placement
of ureter after wrapping with omentum.
Strongest layer of abdominal wall:
Fascia Transversalis (laterally).
Anterior rectus sheath (Centrally).
Rectus sheath hematoma:
Traumatic/Spontaneous/Anti-coagulant therapy.
Bleeding from ruptured inferior epigastric artery.
Soft tender swelling below umbilicus.

Fothergill sign- Swelling does not cross midline and becomes prominent when rectus is contracted.

Usually managed conservatively.

Desmoid tumour:

Tumour of fibrous sheath (tumour of fibrous sheath).

Associated with Gardner’s syndrome.
It’s a locally malignant tumour (though behaves like benign tumour yet invades locally, no metastasis).
Unencapsulated so local recurrence is very common.
Treatment is surgery- wide excision. For recurrence radiotherapy is added.

Peritoneal mice- Asymptomatic, Normally seen in pouch of Douglous or hernia sac.

Source is either Appendix epiploica and Sub-acute pancretitis.

Most common solid tumour of omentum- Metastasis.

Mesentery is 6 inches, extending from DJ to L2 (sacroiliac junction).

******ebook converter DEMO Watermarks*******

Superior Mesenteric artery syndrome (Wilkie syndrome or CAST syndrome)-
3rd par of duodenum gets compressed between Aorta and Supeior mesenteric artery due to narrow angle.
Mesenteric ischemia:

Acute Ishemia- Embolus is the most common cause. SMA is most common artery involved (at the origin) then
middle colic artery (Jejunal branches are spared).
S/S- Pain (symptoms are out of proportion to signs).
IOC- Angiogram.
Treatment – immediate laparotomy.

Chronic Ischemia- Abdominal angina:

Chronic occlusion of mesenteric artery due to atherosclerosis when two of the three major vessels are involved.
Usually narrowing at SMA/IMA.
S/S- Abdominal pain few hours after eating (usually 3 hours).
Malabsorption.
IOC- CECT.
Treatment- Thrombo-endarterectomy/ Bypass graft.
Commonest Abdominal Aneurysm- Aortic Aneurysm.
Commonest Splanchnic vessel aneurysm- splenic (60%) – common in females (associated with pregnancy,
Rupture risk <10%).
Commonest cause of mesenteric Lymphadinitis- Yarsinia.

Small Bowel:

Largest endocrine tumour of the body- Small GUT.

Length- Duodenum- 20 cm, Jejunum 00 cm, Ileum 150 cm.
Jejunal-ileal length is α to body length (1.6 times).
Seninel loop of acute pancreatitis is jejunal loop.

Pain-Visceral efferent fibers in sympathetic ganglions

Secretion and motility- Xth cranial nerve.
Strongest layer of S.I is submucosa (Catgut is made from submucsa of sheep intestine).
New epithelization sarts from crypts and march upward.
Jejunum- 5-7 days.
Ileum – 3 days.
Pacemaker potential of small bowel- Duodenum.
Proximal bowel (Duodenum/ Jejunum absorbs- Iron, calcium, Folate, fat).
Distal Bowel absorbs- Bile salts, Vit B12 and fat soluble vitamines.
Total bile salt pool in man- 5 grams. Recirculation 6 liters per day. Resynthesized from cholesterol. 0.5 gm lost
per day.
All ingested fat is absorbed, small amount that appears in stool is from sloughed C cells and bacteria.
Secretin- Released by duodenum bulb after acidification- Inceases water and bicarbonate secretion from
pancreas.
Secretin decreases gastric secretion (Except in ZE syndrome).
Bombesin releases all GUT peptides except secretin.
Small bowel mucosa is main source of Ig A

******ebook converter DEMO Watermarks*******

Carcinoid:
Originate from Enetrochromaffin cells (Kultchisky cells in crypts)
Secretory products- Serotonin/ Subs P/
Carcinoid syndrome-
Episodic attacks of cutaneous flushing broncospasm, diarrhea (due to serotonin) andvasomotor collapse (due to subs
P)
Seen in <5% of patients with malignant carcinoid.
Increased urinary 5 HIAA (in malignant GI carcinoid or Ovary, RP carcinoid)
Most common tumour of the bowel- (55%)
Classification- Foregut- (Breast, thymus, gut, Duodenum, pancreas)
Mid gut- (Jejunum, Ileum, Appendix, Ascending and proximal 2/3rd transverse colon)
Hind gut- (Left colon rectum)
Common age- 50-70 years.
Most common site- Ileum , Rectum, Appendix.
Usually single (33% multiple), Yellow grey appearance.
>2 cm size is usually associated with metastasis.
Secretory products:
Amines- Serotonin, Histamine, Dopamine.
Tachykinins- Subs P, Kallikrein
Peptide- Neurotensin/ Chromogranin
Prostaglandins
Investigations:
Increased serum serotonins.
Increased urinary HIAA (Normal value- 2-8 mg/ day).
CT/ Angio/ USG.
Treatment:
Surgery- Resection.
Carcinoid syndrome- 5% patients (from liver mets) Flushing, sweating, diarrhea, pain abdomen, bronchospasm,
Pellagra like symptom.
Alcohol induced symptoms.
Treatment – Surgery. Octreotide improves symptoms in > 85%.
Diverticula of Small Inetstine:
Most common site of acquired diverticula- Duodenum (Overall- Sigmoid).
Most common true diverticula- Meckel’s
Second Most common congenital diverticula – Duodenal 2nd part (Para vaterian).
Presentation- Obstruction leading to pancreatitis and cholangitis.
Meckel’s Diverticulum.
Most common congenital diverticulum (2%).
True diverticulum.A remnant of VitelloIntestinal duct (Remnanat of duct between Intetinal tract and yolk sac)
******ebook converter DEMO Watermarks*******
Rule of 2- 2 inches long, 2 feet proximal to Ileoceacal junction, 2% complication rate
Most common symptom- bleeding (seen in children) second most common Intussusception. In adults most common
symptom is obstruction.
Investigation: Tc 99 scan.
Treatment- Surgery Diverticulectomy or resection and anastomosis.
Blind Loop Syndrome:
Bacterial overgrowth in stagnant bowel contents.
Diagnosed by Schilling test.
Co60 labeled Vit B12 --> Increased urinary excretion of Vitamin B12.
(D/D- Pernicious anaemia, BLS).
Add intrinsic factor.

Short Bowel Syndrome:

Main causes: Mesenteric Ischemia/ Midgut volvulus/ Traumatic SMA tear.
Humans can tolerate 25% of resection of small bowel without ileo-caecal junction.
IN children < 45 Cm and in adults < 60 cm with ICJ.
Proximal bowel resection is better tolerated than distal. Ileum can take over function of jejunum but not vice-versa.
Intestinal adaption- Hyperplasia of cell, Increase in length of villi.
Hypergastrenemia occurs.
Presentation of SB syndrome:
Diarrhoea, Steatorrhoea, Malabsorption, Gall stones (cholesterol) and Vit B 12 def Anaemia and oxalate renal
stones.
Absorption:
Duodenum >Jejunum-
Water soluble and fat soluble vitamins (Except B12), Fatty acid absorption, Sodium, Calcium, Iron and chloride are
maximally absorbed.
Sugar, Amino acids, sulfates moderately absorbed.
Jejunum > Duodenum –
Mainly Sugar and Amino acids but Water soluble vitamins, Antibody in newborns, Fatty acids, Sodium, chloride
and iron is moderately absorbed.
Ileum- Bile salts, B12, Sodium, antibody in newborns > Sugar, Amino acids, > Fatty acids absorption.

Sodium absorption is dependent on Glucose.

Calcium is primarily absorbed in Jejunum (Facilitated by DHC).
Iron is mainly absorbed in duodenum (90%) mainly in ferrous state.
******ebook converter DEMO Watermarks*******
 Maximum absorption takes place in Duodenum and jejunum.
Principle storage form of Iron is ferritin
70% of iron in the body is in ferritin.
Principle transport protein in plasma is Transferrin (35% saturated with Fe).
Findings in Fe def anaemia- Increased plasma transferrin, decreased transferrin saturation, decreased ferritin
store.

Surgeries for Short Bowel syndrome:

1. The Bianchi Procedure

splits the bowel lengthwise

2 hemi-loops
puts the 1/2 loops end to end

2. STEP (serial transverse enteroplasty) :The bowel is cut and stapled in a zigzag patter

******ebook converter DEMO Watermarks*******

Appendix:
Positions- Retrocaecal (65%), Pelvic (31%), Least common- post ileal (<.5%)

The appendix first appears at the 8th week of gestation from the cecum and gradually rotates to a more medial
location as the gut rotates and the cecum becomes fixed in the right lower quadrant.
The appendiceal artery, a branch of the ileocolic artery, supplies the appendix.
Histologically, that goblet cells producing mucus, are scattered throughout the mucosa. The submucosa
contains lymphoid follicles, the lymphatics drain into the anterior ileocolic lymph nodes. In adults, the
appendix has no known function.
The base of the appendix is located at the convergence of the taeniae along the inferior aspect of the cecum, and
the tip of the appendix most commonly is location is retrocecal position but within the peritoneal cavity.

Appendicitis:

Appendectomy is the most common urgently performed surgical procedure.

The maximal incidence occurs in the second and third decades of life.
The male:female ratio of approximately 2:1 gradually shifts after age 25 years toward a 1:1 ratio.

Pathophysiology:
A. Appendiceal obstruction:

Appendiceal obstruction is the most common initiating event of appendicitis.

Hyperplasia of the submucosal lymphoid follicles of the appendix accounts for approximately 60% of
obstructions (most common in teens).
In older adults and children, the fecalith is the most common etiology (35%).

B. Intraluminal pressure due to the obstructed appendiceal lumen increases secondary to continued mucosal
secretion and bacterial overgrowth; the appendiceal wall thins, and lymphatic and venous obstruction occurs.
C. Necrosis and perforation due to ischemia.
Bacteriology:
The bacterial flora of the normal appendix is similar to that of the normal colon and remains constant throughout life
with the exception of Porphyromonas gingivalis. This bacterium is seen only in adults. Though Appendicitis is a
polymicrobial infection, the main bacteria of normal appendix, in acute appendicitis, and in perforated appendicitis
are Escherichia coli and Bacteroides fragilis.
******ebook converter DEMO Watermarks*******
Diagnosis:

The diagnosis of acute appendicitis is made by clinical evaluation.

Although laboratory tests and imaging procedures are helpful but mainly they are of secondary importance.

A score of 5 or 6 is compatible with the diagnosis of acute appendicitis. A score of 7 or 8 indicates a probable
appendicitis, and a score of 9 or 10 indicates a very probable acute appendicitis.
History: The classic history of anorexia and periumbilical pain followed by nausea, right lower quadrant (RLQ)
pain, and vomiting occurs in only 50% of cases.
Migration of pain from the periumbilical area to the RLQ is the most discriminating historical feature. When
vomiting occurs, it nearly always follows the onset of pain.
Physical: RLQ tenderness is present in 96% of patients but is a very nonspecific finding.
The most specific physical findings are rebound tenderness, pain on percussion, rigidity, and guarding. Rovsing sign
(ie, RLQ pain with palpation of the LLQ), obturator sign- in pelvic appendix (ie, RLQ pain with internal rotation of
the flexed right hip), and psoas sign, in retrocecal appendix (ie, RLQ pain with hyperextension of the right hip-
MCQ) are present in a minority of patients with acute appendicitis. A palpable mass in the RLQ suggests a
periappendiceal abscess or phlegmon (C/I for appendicectomy and now should be managed coservatively).
Lab Studies: Complete blood count: 80-85% of adults with appendicitis have a WBC count greater than 10,000 and
Neutrophilia greater than 75%.
C-reactive protein: C-reactive protein (CRP) is an acute-phase reactant synthesized by the liver in response to
bacterial infection. Serum levels begin to rise within 6-12 hours of acute inflammation. A normal CRP has a
negative predictive value of approximately 100% for the presence of appendicitis.
Imaging Studies: Computed tomography scan: It is superior to US in diagnosing appendicitis (sensitivity of 94%
and specificity of 95%) and is particularly useful in distinguishing between periappendiceal abscesses and
phlegmon.
Ultrasound: An outer diameter of greater than 6 mm, noncompressibility, lack of peristalsis, or presence of a
periappendiceal fluid collection characterizes an inflamed appendix. The normal appendix is not visualized in most
cases.
Abdominal x-rays: Visualization of an appendicolith in a patient with symptoms consistent with appendicitis is
highly suggestive, but this occurs in < 10% of cases.
Radionuclide scanning: Whole blood is withdrawn. Neutrophils and macrophages are labeled with technetium 99m
albumin and administered intravenously. Images of the abdomen and pelvis are obtained serially over 4 hours.
Localized uptake of tracer in the RLQ suggests appendiceal inflammation.

******ebook converter DEMO Watermarks*******

Treatment:
Open or laparoscopic appendectomy when no lump formation is there.
Conservative management (Ochsner Sherren Regime) after lump formation and appendicectomy is done after 6
weeks (interval appendicectomy).
Most patients with acute appendicitis are managed by prompt surgical removal.
In most patients, a transverse right lower quadrant incision (Davis-Rockey, Fowler-Weir) or an oblique incision
(McArthur-McBurney- grid iron) provides the best cosmetic appearance and allows easy extension medially for
greater exposure.
Patients with diffuse peritonitis or questionable diagnosis should be explored through a midline incision.

After entering the peritoneal cavity, obtain purulent fluid for Gram stain and culture.
Tumours of the Appendix:
Primary tumors of the appendix are rare. Although it was previously believed that carcinoid tumors were the most
common appendiceal neoplasms but newer database indicates that mucinous tumors of the appendix are more
common. Appendiceal carcinoids are neuroendocrine tumors that are usually of the enterochromaffin cell type. They
frequently contain sustentacular cells that express S-100. Usually, these tumors are benign and found either
incidentally at laparotomy or because they cause obstructive appendicitis. A small proportion of carcinoid tumors of
the appendix are malignant and may metastasize. Liver metastases may cause the carcinoid syndrome.
Most are adequately treated with appendectomy.

******ebook converter DEMO Watermarks*******

Right hemicolectomy may be required under three circumstances:
1. When the appendiceal carcinoid is 2 cm or larger.
2. When it is located at the base of the appendix.
3. When mesenteric lymph node spread is present.
Signs:
Most common complication after Appendicectomy- Ileus> Infection> Stump rupture.
Most common late complication- Intestinal adhesion.
Most common extrauterine acute emergency in pregnancy – acute appendicitis (Maternal mortality- 0 but fetal
mortality- 5%).

MANEC (Mixed Adeno NeuroEndocrine):

In appendix there are tumours ranging from exocrine (Aenocarcinoma) to neuroendocrine components
(Carcinoid).
Mixed exocrine-neuroendocrine carcinomas, are now renamed as mixed adenoneuroendocrine carcinomas
(MANECs), in which each component represents at least 30% of the lesion.
An adenocarcinoma showing scattered neuroendocrine cells, demonstrated using anti-chromogranin A
antibody, cannot be classified as a MANEC.
In the appendix, this carcinoma nomally arise by progression from a pre-existing goblet cell carcinoid. Either a
signet-ring cell type or poorly differentiated adenocarcinoma type may be encountered . The latter type
typically exhibits immunohistochemical expression of p53 and MUC1, together with loss of MUC2.

Typhoid Enteritis:
Commonest complication- Haemorrhage (20%)- Managed conservatively.
Perforation (2%)- Usually Single, in terminal ileum, ulcers are longitudinal, ulcerate through payer’s patches.
Treatment is Surgery.
Actinomycosis of Ileo-Caecal Area:
Now rare. Local abscess in retroperitoneal region involving parietal wall. Generally presents 3 weeks after
appendicectomy. Treated by Penicillin.
Most common cause of intussusception in children:
5 months to 1years- Enlarged Submucosal nodes (Payers patches) at weaning.
Ater 2 years- Meckel’s diverticulum.
In adults- Small bowel neoplasm.
Most common benign tumour of SI:
******ebook converter DEMO Watermarks*******
In autopsy- Adenoma.
Symptomatic- Leiomyoma.
Peutz Jegher Syndrome:

Autosomal dominant.
GI hamartomatus polyp (Jejunum > Duodenum > Colon).
Mucocutaneous pigmentation (Melanosis)- Circumoral, buccal mucosa, Palm, sole.
Chr- 19. Gene- STK11/ LKB1.
Now considered premalignant.

Colon:
Volvulus:

Volvulus is twisting of an air-filled segment of bowel about its mesentery.

Sigmoid volvulus an acquired condition, accounts for 80% - 90% of all volvulus and most Sigmoid volvulus is
substantially commoner in populations with a high fibre intake, and tends to affect elderly people caused by sigmoid
redundancy with narrowing of the mesenteric pedicle.

Patient presents with abdominal pain, striking distention, cramping, and obstipation.
Plain X-Ray films show a inverted-U, sausage-like shape of air-filled sigmoid pointing to the right upper
quadrant (inner bent tube). If gangrene is not suspected, water-soluble contrast enema usually shows a bird’s-
beak deformity at the obstructed rectosigmoid junction.
In the absence of peritoneal signs, treatment involves sigmoidoscopy (MCQ), with the placement of a rectal
tube beyond the point of obstruction (recurrence rate after decompressive approaches is 40%); therefore,
elective sigmoid colectomy should be performed.
If peritonitis is present, the patient should undergo laparotomy and Hartmann procedure (sigmoid
colectomy, end-descending colostomy, and defunctionalized rectal pouch). Or rarely in the stable patient
without significant fecal soilage of the peritoneal cavity is “sigmoidectomy, on-table colonic lavage, and
colorectal anastomosis with or without proximal fecal diversion (loop ileostomy).

Cecal volvulus occurs in a younger population due to rotation between a particularly mobile caecum and the
ascending colon, which may in turn be associated with malrotation.
Presentation is nausea, vomiting, abdominal pain, and distention.
Plain films show a coffee bean–shaped, air-filled cecum with the convex aspect extending into the left upper
quadrant.
Management involves:

Urgent laparotomy and right hemicolectomy with Ileocolonic anastomosis (Best).

Ileostomy and mucous fistula in unstable patient or bowel viability is questionable.
Cecopexy has an unacceptably high rate of recurrent volvulus.

Transverse volvulus is rare and has a clinical presentation similar to that of sigmoid volvulus. Diagnosis is made
based on the results of plain films which show a dilated right colon and an upright, U-shaped, dilated transverse
colon. Endoscopic decompression has been reported, but operative resection is usually required.

******ebook converter DEMO Watermarks*******

Colonic Obstruction:
Large bowel obstruction is caused by neoplasms or anatomic abnormalities such as volvulus, incarcerated hernia,
stricture, or obstipation.
Large bowel obstruction fpresents with colonic distention (first), constipation, pain (late), anorexia and feculent
vomiting (last). Distinguishing colonic ileus from organic obstruction is important.
Imaging Studies:

Flat and upright abdominal roentgenography demonstrates dilation of the small and/or large bowel and air fluid
levels.
Tracing colonic air around the colon, into the left gutter, and down into the rectum or demonstrating an abrupt
cut-off in colonic air suggests the anatomic location of the obstruction.
A dilated colon without air in the rectum is more consistent with obstruction. The presence of air in the
rectum is consistent with obstipation, ileus, or partial obstruction.
The characteristic bird’s beak of volvulus may be seen.

Flexible endoscopy preceded by rectal enema may be useful in evaluating left-sided colonic obstruction.
Procedures:

Endoscopic reduction of volvulus

Barium enema for reduction of intussusception
Cleansing enemas

Surgical Care:

Surgical care is directed at relieving the obstruction.

In most patients, the obstructing lesion is resected.
Because the colon has not been cleansed, anastomosis often is risky.
In patients with substantial comorbidity and surgical risk or in the presence of an unresectable tumor, a
diverting proximal colostomy or ileostomy may be performed without resection.

Ischaemic Colitis:

Ischaemic colitis arises from a failure of the blood supply to the colon and occurs most commonly in
conjunction with advanced atherosclerotic disease affecting at least two of the major branches of the aorta.
Acute occlusion of a major artery leads to a surgical emergency with colonic gangrene or to a less acute
syndrome typified by abdominal pain and bloody diarrhoea.
Arterial emboli, dissecting aortic aneurysm, and vasculitic cases may also be responsible.

******ebook converter DEMO Watermarks*******

 The most vulnerable areas of the colon are around the splenic flexure, and, to a lesser extent, the rectosigmoid
region, both of which lie in relatively poorly vascularized
The plain abdominal radiograph may show thumbprinting’, representing oedema of the mucosal folds.
Contrast radiology shows swollen mucosal folds, sawtooth irregularities and narrowing.
In acute ischaemia, the involved section of bowel undergoes infarction; it is dilated and darkly congested with a
friable wall, and is usually filled with blood.
Mucosal ulceration and intense submucosal oedema with haemorrhage and necrosis are seen.
Granulation tissue is later replaced by fibrosis which may lead to post-ischaemic stricturing.
In chronic ischaemia, it is common to find a stricture together with ulceration and granulation tissue.
Fibrosis of the submucosa and circular muscle coat is also characteristic. Where mucosa survives, iron-laden
macrophages are often prominent in the lamina propria.
CT with intravenous contrast is most useful. This modality also may permit visualization of the arterial supply
to the entire intestine.
Arteriography is not indicated unless it is believed that acute mesenteric ischemia involves the small intestine.
Arteriography does not change the management or outcome of clinically apparent colonic ischemia.
Treatment of CI depends on the presentation and severity of signs and symptoms.
Hospital admission, intravenous fluids, bowel rest, and general supportive measures until the patient is pain-
free usually are adequate treatment for mucosal ischemia.
Because loss of integrity of the mucosa may result in bacterial translocation, broad-spectrum antibiotics are
generally advocated for the treatment of CI.

Diverticular Disease:

Diverticula are acquired pouches of mucosa and submucosa herniating through the muscular layers of the
bowel occurring along the mesenteric aspect of the antimesenteric taenia where arterioles penetrate the
muscularis.
Diverticula are associated with a low-fiber diet and its incidence increases with age to a 75% prevalence after
age 80 years.
The sigmoid is affected in 95% but diverticula may occur throughout the colon.

Presentation:

Haemorrhage from a diverticulum (without inflammation or diverticulitus) is responsible for brisk and usually
self-limiting rectal bleeding.
A small minority of patients with diverticulosis develop diverticulitis. This results when an inflamed
diverticulum becomes, effectively, an abscess, which may subsequently perforates causing localized peritonitis.
Lower abdominal pain, tenderness with fever and leucocytosis are usual.

Hinchey’s classification for diverticulitis:

Hinchey I - localised abscess (para-colonic).

Hinchey II – pelvic, Intra-abdominal or retroperitoneal abscess.
Hinchey III – Generalized purulent peritonitis.
Hinchey IV - feculent peritonitis.

******ebook converter DEMO Watermarks*******

Modified Hinchey classification
(there is change in grade II):

Stage II a- Distant abscess amenable to surgical treatment

Stage II b- Complex abscess +/- Fistula

Investigation:
1. As diverticulosis is usually accompanied by marked thickening of the circular muscle layer of the colon, barium
studies show shortening and narrowing of the sigmoid segment as well as of the diverticula (Saw tooth
appearance).
2. Flexible sigmoidoscopy and/or colonoscopy is indicated for the diagnosis Haemorrhage from a diverticulum.
3. CT Scan is the investigation of choice in the presence of infection (Diverticulitis).

******ebook converter DEMO Watermarks*******

Complications:

Infection (diverticulitis) develops in 10% to 25% of patients and presents with left-lower quadrant pain, fever,
altered bowel habit, and urinary urgency.
On examination most common finding is localized left-lower-quadrant tenderness. The finding of a mass
suggests an abscess or phlegmon.
Evaluation by CT scan include segmental colonic thickening, focal extraluminal gas, and abscess formation.
Neither sigmoidoscopy nor contrast enema is recommended in the initial workup of diverticulitis because of the
risk of perforation or barium or fecal peritonitis respectively.

Complications of diverticular disease:

Diverticulitis.
Pericolic abscess.
Peritonitis.
Intestinal obstruction.
Haemorrhage.
Fistula formation.

Treatment is tailored to symptom severity.

Mild diverticulitis can be treated conservatively with clear liquid diet and broad-spectrum oral antibiotics.
Severe diverticulitis is treated with complete bowel rest, intravenous fluids, narcotic analgesics, and broad-
spectrum parenteral antibiotics (e.g., cipro and metronidazole). A colonoscopy or water-soluble contrast study
must be performed after 4 to 6 weeks to rule out colon cancer, inflammatory bowel disease, or ischemia as a
cause of the segmental inflammatory mass.
The likelihood of recurrence is 30% to50% therefore resection is considered 4 to 6 weeks after treatment of a
complicated initial attack of diverticulitis or after treatment of the first recurrence.
Elective resection for diverticulitis usually consists of a sigmoid colectomy on-table colonic lavage, and
colorectal anastomosis with or without proximal fecal diversion (loop ileostomy).
Generalized peritonitis results if diverticular perforation leads to fecal contamination. In most cases, resection
of the diseased segment is possible (two-stage procedure), and a Hartmann procedure is performed. The
colostomy can then be reversed in the future.

******ebook converter DEMO Watermarks*******

Fistulization secondary to diverticulitis may occur between the colon and other organs, including the bladder,
vagina, small intestine, and skin. Diverticulitis is the most common etiology of colovesical fistulas.

The presentation of enterovesical fistula includes frequent urinary tract infections or fecaluria or pneumaturia.
CT findings of air and solid material in a noninstrumented bladder confirm the diagnosis.
A colovaginal fistula is usually suspected based on the passage of air or gas per vagina.
Immediate treatment is for inflammatory mass of severe diverticulitis. Colonoscopy is performed after 6 weeks
to rule out cancer or inflammatory bowel disease and an elective sigmoid resection is performed. A colovaginal
fistula is managed in a similar fashion. It may be helpful to interpose omentum between the colorectal
anastomosis and the bladder or vaginal defect.

Obstruction of the Large Intestine:

Essentials of Diagnosis:

Constipation or obstipation.
Abdominal distention and sometimes tenderness.
Abdominal pain.
Nausea and vomiting (late).
Characteristic x-ray findings.

General Considerations:

Approximately 15% of intestinal obstructions in adults occur in the large bowel.

The obstruction may be in any portion of the colon but most commonly is in the sigmoid.
Complete colonic obstruction is most often due to carcinoma; volvulus, diverticular disease, inflammatory
disorders, benign tumors, fecal impaction, and miscellaneous rare problems.
Adhesive bands seldom obstruct the colon, and intussusception is uncommon in adults.

******ebook converter DEMO Watermarks*******

The pathophysiology of more distal colonic obstruction depends on the competence of the ileocecal valve.

In 10–20% of individuals, the ileocecal valve is incompetent, and colonic pressure is relieved by reflux into the
ileum.
If the colon is not decompressed through the ileocecal valve, a closed loop is formed between the valve and the
obstructing point. The colon distends progressively. If luminal pressure becomes very high, circulation is
impaired and gangrene and perforation can result. The wall of the right colon is thinner than that of the left
colon and its luminal caliber is larger, so the cecum is at greatest risk of perforation in these circumstances
(law of Laplace). In general, if the cecum acutely reaches a diameter of 10–12 cm, the risk of perforation is
great.

Clinical Findings:

Simple mechanical obstruction of the colon may develop insidiously.

Deep, visceral, cramping pain from obstruction of the colon is usually referred to the hypogastrium.
Lesions of the fixed portions of the colon (cecum, hepatic flexure, splenic flexure) may cause pain that is
felt immediately anteriorly.
Pain originating from the sigmoid is often located to the left in the lower abdomen.
Severe, continuous abdominal pain suggests intestinal ischemia or peritonitis.
Borborygmus may be loud and coincident with cramps.
Constipation or obstipation is a universal feature of complete obstruction, though the colon distal to the
obstruction may empty after the initial symptoms begin.
Vomiting is a late finding and may not occur at all if the ileocecal valve prevents reflux.
If reflux decompresses the cecal contents into the small intestine, the symptoms of small bowel as well as large
bowel obstruction appear.
Feculent vomiting is a late manifestation.
Physical examination discloses abdominal distention and tympany, and peristaltic waves may be seen if
the abdominal wall is thin.
High-pitched, metallic tinkles associated with rushes and gurgles may be heard on auscultation.
Localized tenderness or a tender, palpable mass may indicate a strangulated closed loop.
Signs of localized or generalized peritonitis suggest gangrene or rupture of the bowel wall.
Fresh blood may be found in the rectum in intussusception and in carcinoma of the rectum or colon.
Sigmoidoscopy may disclose a neoplasm.
Colonoscopy may be diagnostic and perhaps therapeutic in some patients with strictures or neoplasms.

Imaging Studies:

The colon can be distinguished from the small intestine by its haustral markings, which do not cross the entire
lumen of the distended colon.
A contrast enema or CT scan with rectal contrast will confirm the diagnosis of colonic obstruction and identify
its exact location.
Water-soluble contrast medium should be used if strangulation or perforation is suspected.
Barium should not be given orally in the presence of suspected colonic obstruction.
******ebook converter DEMO Watermarks*******
 A CT scan with rectal contrast is the most useful single test for large bowel obstruction because it can yield
information regarding the location and etiology of the bowel obstruction.

Differential Diagnosis:
Small versus Large Bowel Obstruction:

Large bowel obstruction is frequently slow in onset, causes less pain, and may not cause vomiting in spite of
considerable distention.
Elderly patients with no history of abdominal surgery or prior attacks of obstruction frequently have carcinoma
of the large bowel.
Plain abdominal x-rays and contrast studies are helpful in establishing the diagnosis.

Complications:

Cecal perforation, is a potentially lethal complication.

Partially obstructive lesions of the colon may be complicated by acute colitis in the bowel proximal to the
obstruction;
it is probably a form of ischemic colitis secondary to impaired mucosal blood flow in the distended segment.

Treatment:

An operation is almost always required.

The primary goals of treatment are resection of all necrotic bowel and decompression of the obstructed
segment. Removal of the obstructing lesion is a secondary goal, but a single operation to accomplish both
objectives is preferred whenever possible.
Obstructing lesions of the right colon are resected in one stage, with ileotransverse colostomy if the patient’s
condition is good.
If the patient’s condition is precarious or if the colon has perforated, the bowel is resected but no anastomosis is
done; an ileostomy is established, and anastomosis is performed at a second operation.
Unresectable lesions may be bypassed.
Obstructing lesions of the left colon are best treated by resection in patients who seem likely to tolerate this
procedure.
After resection has been achieved, anastomosis may be postponed and a temporary end colostomy created
(two-stage procedur). Alternatively, intraoperative colonic lavage has previously been advocated to cleanse the
colon well enough so that primary anastomosis can be performed safely. In this setting, subtotal colectomy
results in similar morbidity and mortality as on-table lavage and still permits a one-stage procedure using
healthy bowel.
If the proximal bowel is healthy, a primary anastomosis with a proximal defunctioning (diverting) loop
ileostomy may be possible.
Two other options may be entertained. Alternatively, in unfavorable circumstances, a diverting transverse
colostomy may utilized. However, a serious disadvantage is the need for three operations if this approach is
elected: (1) colostomy, (2) resection of the obstructing lesion with anastomosis, and (3) closure of the
colostomy.

Colo-Rectal Cancer:
Risk Factors:

Age: The incidence of colorectal cancer is relatively low in individuals up to age 50.
Diet: Frequency of colorectal cancer may be associated with high intakes of meat and fat.
Medical Factors: Colorectal cancer is more common among individuals with histories of: intestinal polyps
(noncancerous mushroom-shaped growths), chronic inflammatory bowel disease (ulcerative colitis or Crohn’s
colitis), and previous colorectal cancer.
Women who have had cancers of the breast, uterus or ovary also have a higher risk.
Ethnic Group: Another genetic defect, or mutation, called I1307K, has been found in Ashkenazi Jews (those
******ebook converter DEMO Watermarks*******
 of Eastern European descent).
This defect causes another mutation that can cause uncontrolled cell growth. This may lead to the development
of cancer.
Ulcerative colitis, Crohn colitis, schistosomal colitis, exposure to radiation, and the presence of an
ureterocolostomy are conditions that predispose to cancer of the large bowel.

It was previously thought that a prior history of cholecystectomy predisposed to the development of colorectal
cancer, but this has largely been disproved.
The main behavioral risk factors for the development of advanced colorectal neoplasia include cigarette smoking
and excessive alcohol consumption.

Additional contributing factors may include obesity, lack of dietary fiber, and excessive red meat consumption.
Mitigating factors include nonsteroidal anti-inflammatory drug (NSAID) use, vitamin D consumption, and
physical activity.

Increased intake of saturated fat, increased caloric intake, decreased dietary calcium, and decreased intake of fiber
are among the possible dietary influences.
Genetic or Family Predisposition: There are a number of genetic disorders which can predispose a person to
develop colorectal cancer. There are two classifications of these genetic disorder:

Familial Polyposis Syndromes.

Hereditary Non-Polyposis Colon Cancer(or HNPCC) Syndromes.

Familial Polyposis Syndromes:

Familial Adenomatous Polyposis: This is a relatively rare condition affecting approximately 1 in 8000 individuals.
Without intervention virtually all people with this condition will develop colon cancer. The condition is
characterized by multiple polyps throughout the entire colon. The polyps are not present at birth but develop over
time.
Gardner’s Syndrome: In this syndrome the entire large and small bowel may be affected by adenoma. Other
abnormalities may coexist such as desmoid tumors, lipomas and sebaceous cysts.
Oldfield Syndrome: This syndrome is composed of sebaceous cysts in association with polyposis and cancer
(specifically adenocarcinoma).
Turcot Syndrome: Here CNS tumors are associated with bowel polyposis.

Points to remember about Colonic polyp:

Juvenile Polyp is a benign polyp (Not juvenile polyposis which has malignant potential).
Hyperplastic polyps are not neoplastic and therefore do not become malignant.
Inflammatory polyps have no malignant potential.
Cancer developing in association with hamartomas is rare but has been reported. (Patients with the Peutz-
Jeghers hamartomatous polyposis syndromedo have an increased risk for colorectal cancer).
Adenomas are a premalignant lesion.
The malignant potential of an adenoma depends on size, growth pattern, and the degree of epithelial
atypia.
Cancer is found in 1% of adenomas under 1 cm in diameter, 10% of adenomas 1–2 cm in size, and up
to 45% of adenomas larger than 2 cm.
Most adenomas are tubular, tubulovillous, or villous. The three histologic patterns of adenoma are
variations of one neoplastic process; about 5% of tubular adenomas, 22% of tubulovillous adenomas,
and 40% of villous adenomas become malignant.
The potential for cancerous transformation rises with increasing degrees of epithelial dysplasia.
Sessile.

For MCQ purpose Malignant potential in increasing order:

******ebook converter DEMO Watermarks*******
Juvenile polyp (benign) > Cowden Syndrome (Malignant potential is questionable) > Peutz Jegher (Considered a
risk factor) > Juvenile polyposis > Ulcerative colitis/ Crohn’s disease.

Haggitt classification of Colonic polyp:

Level 0: Carcinoma in situ or intramucosal carcinoma; non-invasive.
Level 1: Carcinoma invades muscularis mucosa to submucosa; limited to head of polyp.
Level 2: Carcinoma invades neck of polyps.
Level 3: Carcinoma invades any part of the stalk.
Level 4: Carcinoma invades submucosa of bowel wall, below polyp stalk but above muscularis propria.

******ebook converter DEMO Watermarks*******

Hereditary Non-polyposis Colon Cancer (HNPCC) Syndromes:
A category of genetic defects where the DNA is unable to make self-repairs when copying itself. Patients are
classified as HNPCC when there is a strong family history of developing colorectal cancer at an early age – that is,
under 50. They are sometimes further divided into Lynch I and Lynch II groups depending on age and other cancers
to which they are predisposed.

Lynch I: In this condition successive generations develop colon cancer at an extremely early age (some studies
showed the average age to be 46).
Lynch II: In this syndrome there is a hereditary predisposition to the development of cancer (specifically of the
colon, ovary, pancreas, uterine and gastric systems).

Criteria for HNPCC syndrome (Amsterdam criteria):

At least one family member who has developed colorectal cancer by age 50.
Colorectal cancer involving at least two successive generations.
Histologically verified colorectal cancer in three or more relatives, one of whom is a first degree relative of
the other two.

There are four cardinal features of HNPCC:

1. Earlier average age (45 years) at onset of cancer than in the general population,
2. The presence of HNPCC-associated cancers within the pedigree.
3. Improved survival when compared stage for stage to sporadic cases.
4. The presence of a germline mutation in affected family members.

The gene responsible for this syndrome has been localized to chromosome 2p, and the genetic defect is in
DNA mismatch repair genes (MLH1, MSH2, MSH6, PMS1, and PMS2). The defects occur in the setting of
microsatellite instability.
******ebook converter DEMO Watermarks*******
 The presence of the Amsterdam criteria defines HNPCC by history alone and the presence of any one of the
Revised Bethesda criteria warrants further investigation for HNPCC.
However, all patients with early-onset colorectal cancer should be offered screening for a familial colorectal
cancer syndrome.
First-degree relatives of patients with sporadic colorectal cancer have a twofold to threefold increased risk of
large bowel cancer, and it is estimated that approximately 10% of cancers of the large bowel are due primarily
to an inherited genetic defect.

Essentials of Diagnosis of colorectal cancer:

Right Colon:

Unexplained weakness or anemia.

Occult blood in feces.
Dyspeptic symptoms.
Persistent right abdominal discomfort.
Palpable abdominal mass.
Characteristic x-ray findings.
Characteristic colonoscopic findings.

Left Colon:

Change in bowel habits.

Gross blood in stool.
Obstructive symptoms.
Characteristic x-ray findings.
Characteristic colonoscopic or sigmoidoscopic findings.

******ebook converter DEMO Watermarks*******

Rectum:

Rectal bleeding.
Change in bowel habits.
Sensation of incomplete evacuation.
Intrarectal palpable tumor.
Sigmoidoscopic findings.

Symptoms:
Approximately 50% of patients present with abdominal pain, 35% with altered bowel habits, 30% with occult
bleeding, and 15% with intestinal obstruction. Right-sided colon cancers tend to be larger and more likely to bleed,
whereas left-sided tumors tend to be smaller and more likely to be obstructing.
Feeling tired and weak; jaundice; pain in the abdomen; differences in the patient’s usual bowel habits: constipation,
diarrhea, narrowing of stool; feeling of fullness in bowel that is not relieved by a bowel movement; blood in stool;
and reduced appetite.
Diagnosis:
Carcinoembryonic antigen:
(1) CEA may be elevated for reasons other than colon cancer, such as pancreatic or hepatobiliary disease, and
******ebook converter DEMO Watermarks*******
elevation does not always reflect cancer or disease recurrence; (2) recurrence remains a possibility when CEA is not
elevated, even if CEA was elevated preoperatively. Findings of other tests, such as CT scans and colonoscopy, must
be incorporated in detection of recurrence.
Imaging Studies:
Chest x-ray: It may reveal metastatic spread to the lungs.
Computed tomographic scanning:

Abdominal/pelvic CT scans can be useful in diagnosis of colon cancer that has metastasized to lymph nodes
and liver.
CT scans also are very helpful in the follow-up of patients with resected, as well as metastatic, disease. Imaging
can diagnose recurrent disease and can document response to chemotherapy.

Procedures:
Flexible sigmoidoscopy is a screening tool that can detect polyps or cancers as far as 60 cm from the anus.
Colonoscopyallows examination of the entire colon, and can be used to obtain a biopsy of suggestive lesions or to
remove polyps.
Double-contrast barium enemas are an option for screening for colorectal cancer and can aid in establishing the
diagnosis of colon cancer.
Staging:

Modified Duke Staging System.

TNM Staging.
Stage Grouping.

Modified Duke Staging System:

Treatment:
Surgery:
This is the most common and usually the first treatment for patients who have colorectal cancer.
There are several forms of surgical treatment used for rectal cancer that don’t involve cutting into the abdomen:

Electrofulgeration: use of electricity to destroy tumors.

******ebook converter DEMO Watermarks*******
 Local excision: cutting off a layer of the rectum that contains cancer.
Local full-thickness resection: cutting out cancer from all layers of the rectum.

Chemotherapy, radiation therapy and biological therapy, either individually or in various combinations, may be used
after surgery.
Chemotherapy:

Eloxatin Eloxatin is a platinum-based anticancer drug used to treat colorectal cancer that has recurred or
advanced colorectal cancer. It is administered intravenously in combination with 5-fluorouracil plus leucovorin
(5FU/LV).

Chemotherapy for Colon cancer:

FOLFOX IV- 5 FU + Leucovorin (Folinic acid) + Oxaliplatin

Irinotecan, Bevacizumab (For VEG-F),and Cetuximb (For RAS +) are used for systemic metastatic disease
or stage IV

Temporary side effects include numbness or tingling in the fingers, toes, throat, and around the mouth.
Radiation Therapy:
Biological or Immunotherapy:
Molecular Biology in prognosis of Colo-rectal cancer:

DNA Sequence of microsatellite instability – good response with 5Fu chemo.

P21 protein mutant expressed – radioresistant
KRAS, DOC, and P53 – if +ve – poor prognosis
Microsatellite instability or low cox2 expression and P21 maker – if +ve – good prognosis

Anal Canal and Rectum:

Anatomy of the Anal Canal:
The anal canal is 3-4cm in length and runs from the anorectal junction to the anus verge. The line of the anal valves,
often called the dentate line, is approximately half way along the anal canal. Anal columns extend upwards from this
line.
The columnar epithelium of the rectum is replaced by mixed columnar and squamous epithelium in the upper anal
canal, corresponding to the zone of fusion between the embryological hindgut and the proctodeum. Between the line
of the anal valves and the lower border of the internal sphincter, the epithelium is stratified squamous and often
referred to as the pecten. At the anal margin, the epithelium becomes hair-bearing skin.
Sphincters: Two sphincters surround the anal canal: the internal sphincter, which is the expanded distal portion of
the circular smooth muscle; and the external sphincter, which is derived from the striated muscle of the pelvic floor
and becomes continuous with the puborectalis and the levator ani muscles.
Spaces: There are three important spaces in the area. The intersphincteric space contains the terminal fibres of the
longitudinal muscle of the gut and the anal intermuscular glands. These anal glands are important in the
pathogenesis of abscesses and fistulae. The ischiorectal fossa lies outside the external sphincter, and the supra-
levator space is between the rectum and the levator ani muscles.
Anatomy of the Anal Canal:
Cicular muscle longitudinal muscle supralevator space levator ani muscle anorectal junction puborectails transitional
zone line of anal valves intimal sphincter extemal sphincter intersphincteric space ansu pectin anal gland.

******ebook converter DEMO Watermarks*******

Congenital Anorectal Anomalies:
Classification system:

Malformations occur in approximately 1 in 5000 live births and affect males and females almost equally (a
slight male predominence).
The embryologic basis includes failure of descent of the urorectal septum.
Anorectal anomalies, varies from anal stenosis and imperforate anus to complete ano-rectal agenesis. Agenesis
accounts for over 75% of anorectal atresias and is often complicated by vaginal, vesical or urethral fistula. Anal
stenosis is usually manifest at birth with the presence of a small anal aperture containing a dot of meconium.
The abdomen may be distended, and defaecation - if possible - results in a ribbon-like stool.
The lesions may also be classified as low, intermediate, or high depending on whether the atresia is below, at
the level of, or above the puborectalis sling (levator ani).
Based on this classification system, in male patients with high imperforate anus the rectum usually ends as a
fistula into the membranous urethra. In females, high imperforate anus often occurs in the context of a
persistent cloaca. In both males and females, low lesions are associated with a fistula to the perineum.
Males: perineal fistula, rectourethral bulbar fistula, rectourethral prostatic fistula, rectovesical (bladder neck)
fistula, imperforate anus without fistula, rectal atresia and stenosis.
Females: perineal fistula, vestibular fistula, imperforate anus with no fistula, rectal atresia and stenosis,
persistent cloaca.

Classification of Congenital Anomalies of the Anorectum:

Diagnosis:
******ebook converter DEMO Watermarks*******
 Physical examination: Inspection of the perineum for the presence of meconium on the perineum within 24
hours of birth may signify a perineal fistula (low defect), which may be safely repaired without a colostomy.
Plain radiographs: obstructive series, invertogram, sacral abnormalities.
A lateral decubitus radiograph is taken, the anal dimple being marked with barium. The film should not
be taken before 12 hours (MCQ) to allow sufficient passage of air distally. In a low lesion air reaches the
perineum. In a high lesion the bowel ends above the pelvic sling and lies above the M line, a line running
horizontally through the junction of the lower 1/3 and upper 2/3 of the ischia and the sacrococcygeal
junction.
Contrast studies: mucous fistulogram.
The exact site of the blockage is determined by injecting contrast medium into the distal loop of the colon
after a colostomy (distal colonogram). It helps in assessing the length of distal colon and to identify a
fistula.
Ultrasound of the renal tract should be performed to rule out renal anomalies.
Magnetic resonance.

Management:

Define associated defects

VACTERL syndromes.
Cardiovascular anomaly (in 10 to 20%) with anorectal malformations.
The management of anal stenosis and imperforate anal membrane is generally with dilatation or simple surgical
incision (Cut back).
A high defect with probable rectourethral or rectovaginal fistula requires a colostomy and mucous fistula for
initial management.
Definitive operative repair
For perineal fistulas, primary repair may be performed without a protective colostomy.
For the more complex anorectal anomalies, a three-step methodology is advocated with a diverting
colostomy after birth (MCQ), posterior sagittal anorectoplasty (PSARP- Pens’s operation), and colostomy
closure.

Long term colonic complications of anorectal anomalies are:

faecal incontinence- due to deficiency of puborectalis and levator ani muscles.

Constipation: due to stenosis of the pull through or deficiency of the nerve supply to the pelvic floor
secondary to sacral nerve deficiency.

Low and High Ano-Rectal Agenesis:

******ebook converter DEMO Watermarks*******

Hemorrhoids:
Hemorrhoids are masses of normal vascular tissue in the anal canal arising from a plexus of dilated veins originating
from the superior and inferior hemorrhoidal veins and are located in the submucosal layer. The prevalence of
hemorrhoids is equal in both sexes.
Hemorrhoids are classified as internal or external, based on whether they arise above or below the dentate line.

Internal hemorrhoids arise from the superior (internal) hemorrhoidal vascular plexus, and their primary
locations are the 3, 7, and 11 o’clock positions corresponding to the end branches of the middle and superior
hemorrhoidal veins.
External hemorrhoids are dilations of the inferior (external) hemorrhoidal plexus and lie below the dentate line,
covered with squamous epithelium that contains numerous somatic pain receptors. . The main clinical
significance of external hemorrhoids is that they may produce painful thrombosis. External skin tags represent
residual excess skin associated with prior thrombosis of external hemorrhoids.

Internal and external hemorrhoids communicate with each other and drain into the internal pudendal veins.
Hemorrhoids may reside in proximity to rectal varices in patients with cirrhosis but are not more common in patients
with portal hypertension.
Hemorrhoids has been associated with increasing age, chronic diarrhea, pregnancy, pelvic tumors, prolonged sitting
and straining, and possibly chronic constipation.

Haemorrhoids usually present with the passage of bright red blood. Bleeding is usually self-limited but may be
associated perianal pain, prolapse, mucous discharge and pruritus.

Internal haemorrhoids that do not prolapse are known as 1st degree,

those that prolapse but reduce spontaneously as 2nd degree,
those that require digital reduction, 3rd degree.

Thrombosis may affect external or internal components.

******ebook converter DEMO Watermarks*******

A small area of involvement of the external plexus is often referred to as a perianal haematoma. These are painful
and may progress to ulceration and/or haemorrhage.
Haemorrhoids – Internal and External Components:

A. Medical treatment:
Medical treatment for first-degree and most second-degree hemorrhoids includes increased dietary fiber and water,
stool softeners, and avoidance of straining during defecation.
Refractory second- and third-degree hemorrhoids may be treated in the office by elastic ligation. The ligation must
be 1 to 2 cm above the dentate line to avoid pain and infection.
B. Excisional hemorrhoidectomy
Excisional hemorrhoidectomy is reserved for large third- and fourth-degree hemorrhoids, thrombosed, incarcerated
hemorrhoids with impending gangrene. After the procedure is performed the resulting elliptical defects are
completely closed with chromic suture (Ferguson hemorrhoidectomy) or can be left open for healing with secondary
intention (Milligan Morgan Hemorrhoidectomy). Complications include a 10% to 50% incidence of urinary
retention, bleeding, infection, sphincter injury, and anal stenosis.
Classification and Treatment of Symptomatic Internal Hemorrhoids:

Operations for Hemorrhoids:

1. Milligan Morgan Haemorrhoidectoy (Open).
2. Ferguson Haemorrhoidectomy (Closed).
3. Whitefield submucosal Hemorrhoidectomy.
4. Longo’s stapled Hemorroidectomy.
5. Doppler Guided haemorrhoid arterial ligation (DGHAL).
Complications of Haemorroidectomy:
******ebook converter DEMO Watermarks*******
Anal Fissures:
An anal fissure is a laceration in the lining of the anal canal distal to the dentate line, which most commonly occurs
in the posterior midline and is often caused by local trauma such as the passage of hard stool. Anal fissures can
occasionally be seen in patients with leukemia, tuberculosis, and Crohn’s disease. The internal anal sphincter
muscle, which is exposed beneath the tear, goes into spasm, pulling the edges of the fissure apart, impairing wound
healing and leading to further tearing of the anal mucosa with subsequent passage of bowel movements. In addition,
ischemia can contribute to the development of a chronic anal fissure. The patient describes a tearing pain with the
passage of hard stool, accompanied by bright red blood, which is usually limited to the surface of the stool or as a
stain on the toilet paper. A fresh laceration usually signifies an acute fissure, whereas a chronic fissure has raised
edges exposing the white horizontally oriented fibers of the internal sphincter at its base and is often accompanied
by external skin tags distally and hypertrophied anal papillae proximally.
Large or multiple fissures, and particularly those away from the midline, should always raise the suspicion of
Crohn’s disease.
Therapy for anal fissures is aimed at breaking the cycle of sphincter spasm and tearing of anal mucosa and
promoting subsequent healing of the fissure. Medical therapy is often successful which includes fiber
supplementation, stool softeners, and warm sitz baths. Topical anesthetic creams such as topical nitroglycerin can
often soothe the inflamed anoderm in the setting of an acute fissure. Stretching of anal sphincter causes anal
dilatation and cures anal fissures by reducing the anal canal pressure.
Botulinum toxin, which is a potent inhibitor of acetylcholine release from nerve endings, can be injected into the
anal sphincter. Botulin toxin is known to cause paresis of the sphincter and thus 2.5 to 5 units, is injected bilaterally
to the fissure. This causes sphincter paresis for about 3 months.
Surgical management can be performed by excising the anal fissure (fissurectomy) or by doing a lateral internal
sphincterotomy to relax and reduce internal sphincter pressure. This technique divides the internal anal sphincter
from its distal end for a distance equal to that of the fissure or up to the dentate line. The sphincterotomy heals best
if it is performed in the lateral position.

Commonest sit: Midline posteriorly (6’O clock).

Presents with pain and streak of blood
Females > Males.
Triad of fissure is: Anal Papilla, Fissure and sentinel pile.
Treated by breaking the cycle of pain, constipation and fissure.
Surgical treatment I for recurrent and chronic cases (Fissurectomy, Lord’s dilatation, Notara’s Lateral
sphincterotomy).

Abscess and Fistula:

Abscesses and fistulae begin as a nonspecific infection of the anal glands which may produce an abscess between
the two sphincters - the so-called intersphincteric abscess, but infection may then spread, to the anal margin gives
rise to a perianal abscess, upward spread produces either an intermuscular abscess or a supralevator abscess,
horizontal spread carries infection back into the anal canal across the internal sphincter, or across the striated muscle
into the ischiorectal fossa forming an ischiorectal abscess.If the primary track passes through the external sphincter,
it is termed trans-sphincteric, but if above the puborectalis, suprasphincteric. Circumferential spread carries infection
in the intersphincteric space, supralevator space or the ischiorectal fossa to the opposite side

******ebook converter DEMO Watermarks*******

Fistulae are classified according to the position of the primary track - intersphincteric if it lies between the
sphincters, trans-sphincteric when it crosses the external sphincter and suprasphincteric when it crosses above the
puborectalis muscle.
Perianal fistulae are a major feature of Crohn’s disease and are usually associated with rectal involvement; rarely do
they represent spread from more proximal bowel disease.
Anorectal fistulas can develop in other medical disorders such as anorectal malignancy, lymphogranuloma
venereum, radiation proctitis, actinomycosis, tuberculosis, and leukemia.
Classification of anorectal fistulas:

The goal of surgical management is to eradicate the fistula while preserving fecal continence. Intersphincteric
fistulas are managed by a primary fistulotomy; the base of the wound is then curetted and left open to heal by
secondary intention. Transsphincteric fistulas are divided into low and high fistulas. Low fistulas are managed by a
primary fistulotomy. High transsphincteric and anterior fistulas are managed with a conservative approach whereby
a cutting section is often performed. This procedure involves placing a reactive suture or elastic through the fistulous
tract and tightening it sequentially until it cuts through the tract. A relatively new therapy involves the injection of
fibrin glue.
Solitary Rectal Ulcer Syndrome (Mucosal prolapse syndrome):
SRUS is a benign condition and usually presents in women during the third and fourth decades of life. SRUS
pursues a chronic course of constipation, mucorrhea-associated rectal prolapse, rectal bleeding, and tenesmus. It is
characterized by an indolent, shallow, whiteish ulcer surrounded by hyperaemic mucosa on the anterior wall of the
rectum, typically 7-10 cm from the margin. There is usually some degree of intussusception of the anterior rectal
wall into the anal canal, and the ulceration is probably caused by trauma to the mucosa during excessive straining
against an actively contracting puborectalis. Once clinical symptoms are elucidated flexible sigmoidoscopy is
performed to confirm the diagnosis.
Management of SRUS is based on the presence of symptoms. Usually conservative therapy with bulk laxatives,
application of local steroids or 5-acetylsalicylic acid (ASA) and bowel retraining is attempted before considering
surgical options.

On anterior wall of rectum 5-10 cm from anal verge

Female > males. 20 – 40 yeas. Presents with bleeding P/R
Caused by: Internal intussusception, Anterior wall prolapse, increased intrarectal pressure
Histo- Mucosal hyperplasia, Hypercellular lamina propria, subepithelial fibrosis, thickened muscularis mucosae,
extension of muscle in mucosa.
Non operative repair is mainstay of treatment
******ebook converter DEMO Watermarks*******
Rectal Prolapse:
Rectal prolapse represents an intussusception of the rectum from a point typically some 8cm above the anus.
Essential defects may be poor rectal support generalized laxity of the pelvic floor and increased intra-abdominal
pressure (cystic fibrosis) and should be distinguished from prolapse of an adenoma or other neoplasm.
Anatomically, patients with rectal prolapse have:

deep rectovesical space (Douglas pouch).

lax levator muscles.
a weak puborectalis.
loss of the acute angle it produces between rectum and anus by pulling anteriorly.
poor fixation of the rectum to the sacrum posteriorly.
and poor support from the lateral ligaments.

Types:
Partial - only the mucosa protrudes out.

This is the more common type.

It is seen at the extremes of age.
Treatment in children is digital reposition, or rarely sub-mucosal injections of a mixture of phenol & almond oil
causing aseptic inflammation and later on fibrosis & adherence of mucosa to muscle.
Operative treatment is required only in the severe & recurrent cases where Thiersch’s operation can be
performed.

Total (procidentia)- entire wall of rectum protrudes out.

This is less common.

It is actually a hernia - en - glissade of the rectum, where it slides down, & may bring along the surrounding
structures or a peritoneal pouch along with it.
Commonly starts in anterior wall, where the support is weakest, especially in women. Anal sphincter is
patulous & gapes widely on straining.
It is usually >4 cm in length.
Concentric folds can be seen on mucosa (radial in partial).
Definitive treatment is for complete prolapse is surgery.

Perineal approach:

Delorme’s operation – Stripping of redundant mucosa + plication of muscle wall to create a ring + re-suturing
of anal mucosa to rectal mucosa.
Thiersch Purse string suturing.
Altemier Rectosigmoidectomy.

Abdominal approach – mostly “sling” procedures to hold rectum to a fixed structure.

Well’s operation - rectum held to sacrum by polyvinyl alcohol sponge - evokes a fibrotic reaction.
Lahaut’s - rectum passed through a rectus sheath.
Ripstein’s - the rectum is fully mobilized and anchored to the presacral fascia with a Teflon or Mersilene mesh,
may be useful for low-risk patients with incompetent anal sphincter. (Treatment of choice in most cases).
Lateral mesh plasty: Orr-Loygue oertion.
Resection rectopexy: Frykmann and Goldberg.
Anterior resection: Treatment of choice in long standing cases with severe prolapsed and chronic mucosal
changes due to prolonged exposure (ulcers etc).
Gracilis sling procedure - corrects the incontinence.

Pilonidal Sinus:
******ebook converter DEMO Watermarks*******
Pilonidal sinus is common in young adults (particularly males- MCQ). It comprises one or more openings in the
natal cleft. The subcutaneous component has a base of granulation tissue and often contains hairs (without hair
follicle). It is thought that pilonidal sinuses follow folliculitis and localized abscess formation within the
subcutaneous fat. The relevance of hair is probably secondary, being responsible, once trapped within the sinus, for
continued infection and a foreign body reaction. Treatment is mainly surgical, where whole of the excised along
with its shin margin is excised and tract is layed open to heal with secondary intention to prevent recurrence.

Surgeries for Pilonidal sinus:

Laying open of all tracks with primary closure or Marsupialization.
Excision of all tracks and closure by Limberg flaps, Karydakis flaps.
Boscom procedure.

Rectal Cancer:
Adenocarcinomas (98%).
70 years. Males > Females.
Clinical: Colorectal carcinoma:

Causes:

No specific risk factors: 75%.

With significant risk factors: 25% [(15-20% in a positive family history or a personal history of colorectal
cancer or polyps), rest in people with certain genetic predispositions, eg. hereditary nonpolyposis colorectal
cancer (HNPCC, 4-7%) and familial adenomatous polyposis (FAP, 1%) or in people with inflammatory bowel
disease (IBD, 1%).
Most related to environmental factors - dietary red fat and animal fat

Adenoma - carcinoma sequence

Of all adenomas - 70% tubular, 10% villous and 20% tubulovillous

Most cancers arise within pre-existing adenomas. Risk is greatest in villous type.
3% patients present with more than one tumour (synchronous tumours). Series of mutations results in epithelial
changes from normal → dysplasia to invasion
Important genes – APC, DCC, k-ras, p53.
******ebook converter DEMO Watermarks*******
 Genetic disorders.
Familial adenomatous polyposis:

An autosomal dominant syndrome

causes more than 100 adenomatous polyps and extraintestinal manifestations like osteomas (jaw), desmoid
tumors and brain tumors.
If left untreated, colorectal cancer develops in nearly 100% of these patients by age 40 years.
80% has a documented hereditary link, rest are caused by spontaneous mutation.

Hereditary nonpolyposis colorectal cancer

HNPCC is an autosomal dominant syndrome.

Patients have the same number of polyps as the general population, but their polyps are more likely to
become malignant. These patients also have a higher incidence of endometrial, gastric, thyroid, and
brain cancers.

Inflammatory bowel disease

Ulcerative colitis

After 10 years, the incidence of colorectal cancer in UC is approximately 1% per year.

Evaluate patients for dysplastic changes with annual colonoscopies.

Crohn disease

Colorectal cancer in Crohn disease is 4-20 times greater than in general population. Cancers often develop in
areas of strictures and in defunctionalized segments of intestine. Patients with Crohn colitis undergo the
same surveillance regimen as those with UC.

Workup:
Routine laboratory study & CEA.
Metastatic workup.

Liver function tests. Chest radiograph

Carcinoembryonic antigen test: A CEA higher then 100 ng/mL usually indicates metastatic disease and
warrants a thorough investigation.

Rigid proctosigmoidoscopy / Flexible sigmoidoscopy

This procedure is used to obtain biopsies of the lesion, assess ulceration, and determine the degree of fixation.

Colonoscopy:
Double contrast barium enema

In patients presenting with large bowel obstruction single contrast enema (after rigid sigmoidoscopy) is the
investigation of choice

Computed axial tomography scan generally is used to determine the presence or absence of metastases and to assess
regarding depth of penetration of the primary rectal tumor.
STAGING: Dukes classification: Developed by Cuthbert Duke in 1932.

Those limited to the rectal wall (Dukes A)

Those that extended through the rectal wall into extrarectal tissue (Dukes B)
Those with metastases to regional lymph nodes (Dukes C)

******ebook converter DEMO Watermarks*******

This system was modified to include subdivisions of stages B and C, as follows:

Stage B became B1 (tumor penetration into muscularis propria) and B2 (tumor penetration through muscularis
propria).
Stage C became C1 (tumors limited to the rectal wall with nodal involvement) and C2 (tumors penetrating
through the rectal wall with nodal involvement).
Stage D was added to indicate distant metastases.

Five year survival - 90%, 70% and 30% for Stages A, B and C respectively
TNM classification for cancer of the colon and rectum (AJCC):

Treatment: Medical Care:

Treatment of polyps:

Ten percent of polyps larger than 1 cm contain carcinoma.

Sessile polyps that contain invasive carcinoma require resection. Pedunculated, cancer-
containing polyps may be removed colonoscopically.
If the following requirements are not met, surgical resection is indicated: (1) the carcinoma must be well or
moderately differentiated with no venous or lymphatic invasion. (2) The carcinoma must not invade further
than the stalk of the polyp, and the margins of resection must not contain tumor.

******ebook converter DEMO Watermarks*******

Preoperative radiation therapy: Preoperative radiation therapy decreases tumor recurrence in patients with stage II
and III disease. In stage I disease, the morbidity and mortality are higher, and there is no proven benefit to giving
preoperative RT.
Neoadjuvant Therapy:

Upper rectal cancers no

Mid rectal cancers yes
Lower rectal cancers yes

Contra-indications to neo-adjuvant therapy:

Upper rectal cancers.

Early T1 lesions.
Obstruction.
Known stage IV disease.
Patient can not tolerate therapy.

T1 lesions should be approached with local excision +/- chemoradiation

T2 lesions should be may be treated with local excision and neo-adjuvant therapy, LAR is better if feasible.
T3 and T4 lesions are best treated with radical excision (LAR or APR) even if there is a good response to neo-
adjuvant therapy.
N1 and N2 lesions need radical excision.

Neo-adjuvant Therapy as an adjunct to Local Excision of Rectal Cancer:

Sphincter preservation.
Decreased or delayed local recurrence.
Increase survival.
Certainty of treatment.
Euoxic tissue levels.
Improved symptoms.

Criteria for Local Excision:

Tumor < 4 cm in size, < 9 cm from anal verge.

confined to less than one quadrant.
mobile, non fixed.
well differentiated, exophytic tumor.
absence of lymphovascular invasion.
T1 or T2, NO lesion by ultrasonographic exam.
no evidence of metastatic disease on CT scan.

Surgical Care:
Transanal excision:

******ebook converter DEMO Watermarks*******

 Patients with stage 0 or I cancer with a T1 lesion.
The lesion is excised with full thickness of the rectal wall, leaving a 1-cm margin.
Lesions that display unfavorable histology but are excised completely may be treated with adjuvant radiation
therapy.

Surgical options:

1 cm distal clearance of rectal lesions adequate if mesorectum resected.

Radial margin should be histopathologically free of tumour if possible.
Lymph node resection should be performed to the origin of the feeding vessel.
En Bloc resection of adherent tumours should be performed.
Depending on site of lesion surgical options are:
Upper rectum – Anterior resection (Consider defunctioning loop ileostomy is anastomosis <12 cm From anal
margin).
Lower rectum – Abdomino-perineal resection.

Sphincter-sparing procedures:
Low anterior resection.

Low anterior resection (LAR) procedure is performed generally for lesions in the middle and upper third of the
rectum,
A 2-cm margin distal to the lesion must be achieved.

Coloanal anastomosis.

Very distal rectal cancers, located just above the sphincters can be resected without the need for a permanent
colostomy. In this pelvic dissection is carried down to below the level of the levator ani muscles from within
the abdomen. A straight-tube coloanal anastomosis (CAA) can be performed.
An alternative to the straight-tube CAA is the creation of a colonic J pouch. The advantages of the J pouch
include decreased frequency and urgency of bowel movements because of the increased capacity of the pouch.

Abdominal perineal resection

Abdominal perineal resection (APR) is performed in patients with lower-third rectal cancers who cannot
undergo a sphincter-sparing procedure.

Surgeries: Mergins- 5 cm proximal/ 2 cm distal:

Any cancer which is > 5 cm from anal verge: Anterior resection (Sphincter preserved)
Cancer < 5 cm from Anal verge: Abdomino-perineal resection
In Emergency, a patient presenting with obstruction
Fit for major surgery Excision and Hartmann’ procedure
Unfit for major surgery Proximal defunctioning colostomy

Adjuvant radiotherapy:

Risk factors for local recurrence include:

Local extent of tumour.
Nodal involvement.
Circumferential margin status.
Risk of local recurrence can be reduced by radiotherapy.
Can be given either preoperatively or postoperatively.
Combination chemotherapy and radiotherapy may produce better outcome.

Adjuvant chemotherapy:
******ebook converter DEMO Watermarks*******
 Improves survival in Duke’s C tumours
Not required in Duke’s A tumours which already have a good prognosis.
Role in Duke’s B tumours remains to be defined.

Generally, patients with T1 or T2 disease can undergo local surgical excision. Patients with T3 or N1 disease benefit
from a course of preoperative neoadjuvant chemotherapy and radiation therapy prior to planned surgical resection,
which could include either a low anterior resection or abdominoperineal resection. Patients with T4 disease often
require palliative therapies including chemotherapy, radiation therapy, and surgery.

True anal cancers arise from the transitional mucosa between the rectal mucosa and the squamous epithelium of the
anal margin; most are squamous carcinomas or basaloid carcinomas, so termed because of their histological
resemblance to basal cell lesions, but a spectrum from squamous through basal and transitional epithelium to a
glandular pattern is recognized.
The clinical behaviour of most types is similar to that of a low rectal carcinoma. The malignant melanoma, from its
bluish/black polypoid appearance, is easily mistaken for a thrombosed external haemorrhoid. The tumour is highly
malignant and metastasizes rapidly.
Anal canal SCC is treated by Chemo-Radiotherapy (Nigro’s regime).

Cheemmotherpy 5FU + Cisplatin or Mitomyicn C.

Followed By radio-therapy.

******ebook converter DEMO Watermarks*******

Liver:
Introduction:

The liver is the largest organ in the body.

weighs 1.5 kg in the average 70-kg man.

The liver parenchyma is entirely covered by a thin capsule and by visceral peritoneum on all but the posterior
surface of the liver, termed the ‘bare area’.

The liver is divided into a large right lobe, which constitutes three-quarters of the liver parenchyma, and a
smaller left lobe.

Anatomy of the Liver:

Ligaments and peritoneal reflections:

The liver is fixed in the right upper quadrant by peritoneal reflections that form ligaments.

Left triangular ligament:

On the superior surface of the left lobe.

Dividing the anterior and posterior folds of this ligament allows the left lobe to be mobilised from the
diaphragm and the left lateral wall of the inferior vena cava (IVC) to be exposed.

Right triangular ligament:

fixes the entire right lobe of the liver to the undersurface of the right hemi-diaphragm.
Division of this ligament allows the liver to be mobilised from under the diaphragm and rotated to the left.

Falciformligament:

It is remnant of the umbilical vein.

Runs from the umbilicus to the liver between the left medial and left lateral , passing into the interlobar
fissure.
From the fissure, it passes anteriorly on the surface of the liver, attaching it to the posterior aspect of the
anterior abdominal wall.
Division of the superior leaves of the falciform ligament allows exposure of the suprahepatic IVC, lying within
a thin sheath of fibrous tissue.
The final peritoneal reflection is between the stomach and the liver.

Liver blood supply:

80 per cent being derived from the portal vein and 20 per cent from the hepatic artery.
The arterial blood supply in most individuals is derived from the coeliac trunk of the aorta, where the hepatic
artery arises along with the splenic artery.
After supplying the gastroduodenal artery, it branches at a very variable level to produce the right and left
hepatic arteries.
******ebook converter DEMO Watermarks*******
 The right artery supplies the majority of the liver parenchyma and is therefore the larger of the two arteries.
The blood supply to the right lobe of the liver may be partly or completely supplied by a right hepatic
artery arising from the superior mesenteric artery. This is known as replaced right hepatic artery.
Similarly, the arterial blood supply to the left lobe of the liver may be derived from the coeliac trunk via
its left gastric branch. This is known as replaced left hepatic artery.This vessel runs between the lesser
curve of the stomach and the left lobe of the liver in the lesser omentum.

Structures in the hilum of the liver:

The porta hepatis, a transverse fissure on the visceral surface of the liver, is the hilum of the liver.
The hepatic artery, portal vein and bile duct are present within the free edge of the lesser omentum or the
‘hepatoduodenal ligament’ and together with nerves and lymphatics enter the liver at the porta hepatis.
To expose these structures requires division of the overlying peritoneum followed by the division of small
vessels and the lymphatic plexus.
The usual anatomical relationship of these structures is for the bile duct to be within the free edge, the hepatic
artery to be above and medial, and the portal vein to lie posteriorly.
Within this ligament, the common hepatic duct is joined by the cystic duct at a varying level to form the
common bile duct.
The common hepatic artery branches at a variable level within the ligament to form two, or often three, main
arterial branches to the liver.
The right hepatic artery crosses the bile duct either anteriorly or posteriorly before giving rise to the
cystic artery.
The portal vein arises from the confluence of the splenic vein and the superior mesenteric vein behind the neck
of the pancreas.
It has some important tributaries, including the left gastric vein which joins just above the pancreas.

Division of structures at the hilum:

At the hilum, the major structures are divided into right and left branches.
The left duct has a longer extrahepatic course of approximately 2 cm.
Once within the liver parenchyma, the duct accompanies the branches of the hepatic artery and portal vein
within a fibrous sheath.
The portal vein often gives off two large branches to the right lobe, which are usually outside the liver for a
short length, before giving a left portal vein branch that runs behind the left hepatic duct.

Venous drainage of the liver:

The venous drainage of the liver is via the hepatic veins into the IVC.
The vena cava lies within a groove in the posterior wall of the liver.
Above the liver, it immediately penetrates the diaphragm to join the right atrium, whereas below the liver
parenchyma, there is a short length of vessel before the insertion of the renal veins.
The inferior hepatic veins are short vessels that pass directly between the liver parenchyma and the anterior
wall of the IVC.
The major venous drainage is through three large veins that join the IVC immediately below the diaphragm.
The right hepatic vein can be exposed fully outside the liver, but the middle and left veins usually join within
the liver parenchyma.
The right kidney and adrenal gland lie immediately adjacent to the retrohepatic IVC.
The right adrenal vein drains into the IVC at this level, usually via one main branch.

Segmental Anatomy of the Liver:

Couinaud, a French anatomist, described the liver as being divided into eight segments.
Each of these segments can be considered as a functional unit, with a branch of the hepatic artery, portal vein
and bile duct, and drained by a branch of the hepatic vein.
The overall anatomy of the liver is divided into a functional right and left ‘unit’ along the line between the gall

******ebook converter DEMO Watermarks*******

 bladder fossa and the middle hepatic vein (Cantlie’s line).
Liver segments (V–VIII), to the right of this line, are supplied by the right hepatic artery and the right branch of
the portal vein, and drain bile via the right hepatic duct.
To the left of this line (segments I–IV), functionally, is the left liver, which is supplied by the left branch of the
hepatic artery and the left portal vein branch, and drains bile via the left hepatic duct.
The main scissura contains the middle hepatic vein, which runs in an antero-posterior direction from the
gallbladder fossa to the left side of the vena cava. It divides the liver into right and left hemilivers.
The right liver is divided into anterior (segments V and VIII) and posterior (segments VI and VII) sectors by
the right scissura, which contains the right hepatic vein.
the right portal pedicle is composed of the right hepatic artery, portal vein, and bile duct. It splits into right
anterior and right posterior pedicles, which supply the segments of the anterior and posterior sectors.
The left liver has a visible fissure along its inferior surface called the umbilical ssure.
The ligamentumteres, containing the remnant of the umbilical vein, runs into this fissure.
The umbilical fissure is not a scissura and does not contain a hepatic vein; it contains the left portal
pedicle, which contains the left portal vein, hepatic artery, and bile duct.
The left liver is split into anterior (segments III and IV) and posterior (segment II, the only sector
composed of a single segment) sectors by the left scissura.
Note: there is difference between section and sectors. Left lobe can be divided into left medial
section(containingseg IV A and IV B.) and left lateral section (contains segment II and III). The right sections
and sectors are same.
The left scissura runs posterior to the ligamentumteres and contains the left hepatic vein.
The caudate lobe(segment I) lies to the left and anterior of the IVC and contains three subsegments: the Spiegel
lobe, the paracaval portion, and the caudate process.

Infections of the Liver:

******ebook converter DEMO Watermarks*******

Pyogenic Liver Abscess:

They may be single or multiple.

More frequently found in the right lobe of the liver.
The abscess cavities are variable in size and, when multiple, may coalesce to give a honeycomb appearance.
Approximately 40% of abscesses are monomicrobial, an additional 40% are polymicrobial, and 20% are
culture-negative.
The most common infecting agents are gram-negative bacteria; Escherichia coli is found in two thirds of cases,
and other common organisms include Streptococcus faecalis, Klebsiella, and Proteus vulgaris. Anaerobic
organisms such as Bacteroides fragilis also are seen frequently.
In patients with endocarditis and infected indwelling catheters, Staphylococcus and Streptococcus species are
more commonly found.
Patients commonly present with right upper quadrant pain and fever.
Jaundice occurs in up to one third of affected patients.
Leukocytosis, an elevated sedimentation rate, and an elevated AP level are the most common laboratory
findings.
Ultrasound examination of the liver reveals pyogenic abscesses as round or oval hypoechoic lesions with well-
defined borders and a variable number of internal echoes.
CT scan is highly sensitive in the localization of pyogenic liver abscesses, which appear hypodense with
peripheral enhancement and may contain air-fluid levels indicating a gas-producing infectious organism.
MRI of the abdomen can also detect pyogenic abscesses with a high level of sensitivity but plays a limited role
because of its inability to be used for image-guided diagnosis and therapy.
The current cornerstones of treatment include correction of the underlying cause and IV antibiotic therapy.
Percutaneous needle aspiration and culture of the aspirate may be useful in guiding subsequent antibiotic
therapy.
Surgical drainage either via the laparoscopic or open approach may become necessary if initial therapies fail.

Amebic Abscesses:

Amebic abscesses are the most common type of liver abscesses worldwide.
Entamoeba histolytica is the most common causative agent.
Amebiasis is most common in subtropical climates, especially in areas with poor sanitation.
E. histolytica exists as cysts in a vegetative form that are capable of surviving outside the human body. The
cystic form passes through the stomach and small bowel unharmed and then transforms into a trophozoite in the
colon.
In colon it invades the colonic mucosa forming typical flask-shaped ulcers, enters the portal venous system,
and is carried to the liver.
Amebae multiply and block small intrahepatic portal radicles with consequent focal infarction of hepatocytes.
They contain a proteolytic enzyme that also destroys liver parenchyma. The abscesses formed are variable in
size and can be single or multiple.
The amebic abscess is most commonly located in the superior-anterior aspect of the right lobe of the liver
near the diaphragm
Has a necrotic central portion that contains a thick, reddish brown, pus-like material. This material has
been linked to anchovy paste or chocolate sauce.
Amebiasis should be considered in patients who have traveled to an endemic area and present with right upper
quadrant pain, fever, hepatomegaly, and hepatic abscess.
Leukocytosis is common, whereas elevated transaminase levels and jaundice are unusual.
The most common biochemical abnormality is a mildly elevated AP level.
Even though this disease process is secondary to a colonic infection, the presence of diarrhea is unusual.
Most patients have a positive fluorescent antibody test for E. histolytica and test results can remain positive for
some time after a clinical cure. This serologic test has a high sensitivity, and therefore amebiasis is unlikely if
the test results are negative.
Ultrasound and CT scanning of the abdomen are both very sensitive but nonspecific for the detection of amebic
abscesses.
Amebic abscesses usually appear on CT as well- defined low-density round lesions that have
enhancement of the wall, somewhat ragged in appearance with a peripheral zone of edema. The central
******ebook converter DEMO Watermarks*******
 cavity may have septations as well as fluid levels. CT scanning is also useful in the detection of extrahe-
patic involvement.
Metronidazole 750 mg three times a day for 7 to 10 days is the treatment of choice and is successful in 95% of
cases.
Aspiration of the abscess rarely is needed and should be reserved for patients with large abscesses, those who
do not respond to medical therapy, or those who appear to be superinfected.
Furthermore, abscesses of the left lobe of the liver at risk for rupture into the pericardium should be
treated with aspiration and drainage.

Hydatid Disease:

Hydatid disease is due to infection by the tapeworm Echinococcus granulosus in its larval or cyst stage.
Scolices, contained in the cysts, adhere to the small intestine of dogs and become adult taenia, which attach to
the intestinal wall. Each worm sheds approximately 500 ova into the bowel.
The infected ova-containing feces of dogs contaminate grass and farmland, and the ova are ingested by
intermediate hosts such as sheep, cattle, pigs, and humans.
The ova have chitinous envelopes that are dissolved by gastric juice. The liberated ovum then burrows through
the intestinal mucosa and is carried by the portal vein to the liver, where it develops into an adult cyst.
Most cysts are caught in the hepatic sinusoids, and therefore 70% of hydatid cysts form in the liver.
A few ova pass through the liver and are held up in the pulmonary capillary bed or enter the systemic
circulation, forming cysts in the lung, spleen, brain, or bones.
Hydatid cysts commonly involve the right lobe of the liver, usually the anterior-inferior or posterior-
inferior segments.
The affected patient presents with symptoms such as dull right upper quadrant pain or abdominal distention.
Cysts may become secondarily infected, involve other organs, or even rupture, which leads to an allergic or
anaphylactic reaction.
The diagnosis of hydatid disease is based on the findings of an enzyme-linked immunosorbent assay (ELISA)
for echinococcal antigens, and results are positive in approximately 85% of infected patients.
Eosinophilia is seen in approximately 30% of infected patients.
Ultrasonography and CT scanning of the abdomen are both quite sensitive for detecting hydatid cysts.
Ultrasound is most commonly used worldwide for the diagnosis of echinococcosis because of its availability,
affordability, and accuracy.
Gharbi classification is the USG classification for hydatid cyst.

1. Type I has a pure fluid collection.

2. Type II has a fluid collection with a split wall (floating membrane).
3. Type III reveals a fluid collection with septa (honeycomb image).
4. Type IV has heterogenousechographic patterns
5. Type V has reflecting thick walls.

MRI of the abdomen may be useful to evaluate the pericyst, cyst matrix, and daughter cyst characteristics.
Treatment of hydatid disease is surgically based because of the high risk of secondary infection and rupture.
surgical resection involving excision (or pericystectomy), marsupialization procedures, leaving the cyst open,
drainage of the cyst, omentoplasty(also known as capitonization), and partial hepatectomy are the various
options.
conservative management is appropriate for:
older patients with small, asymptomatic, densely calcified cysts.
PAIR (puncture, aspiration, injection, and reaspiration) and has become more accepted in some centersfor :

1. Small cysts
2. Uncomplicated cyst
3. Pregnant females
Tumors of Liver:

******ebook converter DEMO Watermarks*******

Hemangioma:

Hemangiomas (also referred to as hemangiomata) are the most common solid benign masses that occur in the
liver.
They consist of large endothelial-lined vascular spaces.
Recent data states that there is no difference in the incidence of hemagioma in males and females.
A recent case-control study found no association between liver hemangioma and the patient’s
reproductive history or use of oral contraceptives.
Avereage size is between 1 and 2 cm in diameter.
lesions greater than 4 cm in size, are known as “GIANT HEMAGIOMA”
Most hemangiomas are discovered incidentally with little clinical consequence.
Large lesions can cause symptoms as a result of compression of adjacent organs or intermittent thrombosis,
which in turn results in further expansion of the lesion.
Kasabach-Merritt syndrome is another complication of large hemangiomas characterized by
thrombocytopenia and consumptive coagulopathy.Its pathophysiology is thought to involve activation of the
clotting cascade by platelets trapped within the hemangioma.
Spontaneous rupture (bleeding) is rare, but surgical resection can be considered if the patient is symptomatic.
Resection can be accomplished by enucleation or formal hepatic resection, depending on the location and
involvement of intrahepatic vascular structures and hepatic ducts.
The majority of hemangiomas can be diagnosed by liver imaging studies like CECt or MRI
On biphasic contrast CT scan, large hemangiomas show asymmetrical nodular peripheral enhancement
that is isodense with large vessels and exhibit progressive centripetal enhancement fill-in over time
On MRI, hemangiomas are hypointense on T1-weighted images and hyperintense on T2-weighted images.
Caution should be exercised in ordering a liver biopsy if the suspected diagnosis is hemangioma because
of the risk of bleeding from the biopsy site, especially if the lesion is at the edge of the liver.

Adenoma:

Hepatic adenomas are benign solid neoplasms of the liver.

They are most commonly seen in premenopausal women older than 30 years of age and are typically solitary,
although multiple adenomas also can occur.
Prior or current use of estrogens (oral contraceptives) is a clear risk factor for development of liver adenomas.
Diabetes and glycogen storage disorder(type 1) are another known risk factor.
On gross examination, they appear soft and encapsulated and are tan to light brown.
Histologically, adenomas lack bile duct glands and Kupffer cells, have no true lobules, and contain
hepatocytes that appear congested or vacuolated due to glycogen deposition.
On CT scan, adenomas usually have sharply defined borders . In venous phase contrast, they can look
hypodense or isodense in comparison with background liver, whereas on arterial phase contrast, subtle

******ebook converter DEMO Watermarks*******

 hypervascular enhancement often is seen.
On MRI scans, adenomas are hyperintense on T1-weighted images and enhance early after gadolinium
injection.
Adenoma show a negative sulphur colloid scan.
Hepatic adenomas carry a significant risk of spontaneous rupture with intraperitoneal bleeding.
The clinical presentation may be abdominal pain, and in 10% to 25% of cases, hepatic adenomas present with
spontaneous intraperitoneal hemorrhage.
Hepatic adenomas also have a risk of malignant transformation to a well-differentiated HCC.
Therefore, it usually is recommended that a hepatic adenoma (once diagnosed) be surgically resected.

Focal Nodular Hyperplasia:

FNH is a solid, benign lesion of the liver.

Believed to be a hyperplastic response to an anomalous artery.
More common in women of childbearing age,
Not related to oral contraceptive use .
On histology: they contain: a central stellate scar; proliferating bile duct elements and NO PORTAL
VENOUS STRUCTURES.
Biphasic CT scan usually is diagnostic of FNH, on which such lesions appear well circumscribed with a typical
central scar . They show intense homo- geneous enhancement on arterial phase contrast images and are often
isodense or invisible compared with background liver on the venous phase.
On MRI scans, FNH lesions are hypointense on T1-weighted images and isointense to hyperintense on T2-
weighted images.
They show positive Sulphur Colloid Scan.
FNH lesions usually do not rupture spontaneously and have no significant risk of malignant transformation.
Therefore, the management of FNH is usually reassurance and prospective observation irrespective of size.

Malignant Liver Tumors:

Malignant tumors in the liver can be classified as:
1. Primary (cancers that originate in the liver) or
2. Metastatic (cancers that spread to the liver from an extrahepatic primary site) .
Primary cancers in the liver that originate from hepatocytes are known ashepatocellular carcinomas (HCCs or
hepatomas), whereas cancers arising in the bile ducts are known as cholangiocarcinomas.
The most common tumor seen in the liver is metastatic colorectal cancer.
Hepatocellular Carcinoma:

HCC is the fifth most common malignancy worldwide, with an estimated 750,000 new cases diagnosed
annually.
Because of its high fatality, it is the third most common cause of cancer death worldwide.
Major risk factors are viral hepatitis (B or C), alcoholic cirrhosis, hemochromatosis, and NASH.

Pathology:

HCC is graded as well, moderately, or poorly di erentiated.

In gross appearance, the growth patterns of HCC have been classiffied as:

1. Hanging type of HCC is connected to the liver by a small vascular stalk and is easily resected without
sacrifice of a significant amount of adjacent non-neoplastic liver tissue.
2. Pushing type of HCC is well demarcated and often contains a fibrous capsule. It is characterized by growth
that displaces vascular structures rather than invading them. is type is usually resectable.
3. Infiltrative type of HCC, which tends to invade vascular structures, even at a small size. Resection of the
infiltrative type is often possible, but positive histologic margins are common.

******ebook converter DEMO Watermarks*******

Distinct variants of HCC:

Fibrolamellar HCCis a variant HCC with remarkably different clinical features.

tumor generally occurs in younger patients without a history of cirrhosis.
tumor is usually well demarcated and encapsulated and may have a central brotic area.
central scar can make distinguishing this tumor from FNH difficult.
Fibrolamellar HCC does not produce AFP but is associated with elevated neurotensin levels.
In general, brolamellar HCC has a better prognosis than HCC, probably related to high resectability rates, lack
of chronic liver disease, and a more indolent course.

Clinical Presentation:

More prevelant in male patients in 5-6th decade.

Most commonly, patients presentwithright upper quadrant abdominal pain and weight loss and have a palpable
mass.
Presentation at an advanced stage is often with vague right upper quadrant abdominal pain that sometimes
radiates to the right shoulder.
Nonspecic symptoms of advanced malignant disease, such as anorexia, nausea, lethargy, and weight loss, are
also common.
Another common presentation of HCC is hepatic decompensation in a patient with known mild cirrhosis or
even in patients with unrecognized cirrhosis.
HCC can rarely be manifested as a rupture, with the sudden onset of abdominal pain followed by hypovolemic
shock secondary to intraperitoneal bleeding.
Other rare presentations include hepatic vein occlusion (Budd-Chiari syndrome), obstructive jaundice,
hemobilia, and fever of unknown origin.
Less than 1% of cases of HCC are manifested with a paraneoplastic syndrome, usually hypercalcemia,
hypoglycemia, and erythrocytosis.

Diagnosis:

TRI PHASIC CECT is ioc for HCC.

HCCs are typically hypervascular with blood supplied predominantly from the hepatic artery. Thus, the
lesion often appears hypervascular during the arterial phase of CT studies and relatively hypodense
during the delayed phases due to early washout of the contrast medium by the arterial blood.
MRI imaging also is effective in characterizing HCC.
CT and MRI also evaluate the extent of disease in terms of peritoneal metastases, nodal metastases, and extent
of vascular and biliary involvement.
HCC has a tendency to invade the portal vein, and the presence of an enhancing portal vein thrombus is
highly suggestive of HCC.
AFP measurements can be helpful in the diagnosis of HCC.
But, AFP measurements have low sensitivity and specificity. False-positive elevations of serum AFP levels can

******ebook converter DEMO Watermarks*******

 be seen in in inflammatory disorders of the liver, such as chronic active viral hepatitis.
Furthermore, AFP is not specific to HCC and can be elevated with intrahepatic cholangiocarcinoma and
colorectal metastases.
AFP is largely used as an adjunctive test in patients with liver masses. AFP levels are particularly useful in
monitoring treated patients for recurrence after normalization of levels.
HCC does not require pre-operative biopsy unless the diagnosis is in question. Percutaneous ne-needle
aspiration of HCC does run a small risk of tumor cell pillage (estimated to be ≈1%) and rupture or bleeding,
especially in cirrhotic livers and subcapsular tumors.
Assessment of liver function is absolutely critical in considering treatment options for a patient with HCC.
Child-Pugh status is used most often. Child-Pugh class C patients are not candidates for resectional
therapy, whereas Child-Pugh class A patients can usually tolerate some extent of liver resection. Many
consider Child-Pugh class B patients to be candidates for operation, but they are generally borderline, and
therapy must be individualized

Child-Turcotte-Pugh (CTP) score:

portal hypertension, regardless of biochemical assessments, is highly predictive of postoperative liver failure
and death.
TNM staging system is not routinely used for HCC because it does not accurately predict survival; it
does not take liver function into account.
Okuda staging system :It adds up a single point for the presence of tumor involving more than 50% of the liver,
presence of ascites, albumin level less than 3 g/dL, and bilirubin level higher than 3 mg/dL.
Other scoring systems:Cancer of the Liver Italian Program (CLIP),. Chinese University Prognostic Index
(CUPI

Management:

For HCC, resection is the treatment of choice.

For patients with Child’s class A cirrhosis with preserved liver function and no portal hypertension, resection
also is considered.
If resection is not possible because of poor liver function and the HCC meets transplant criteria liver
transplantation is the treatment of choice.
Mazzaferro /Milan Criteria :
Following are desired candidayes for transplant: one tumor ≤5 cm, or up to three tumors no larger than 3 cm,
along with the absence of gross vascular invasion or extrahepatic spread.

******ebook converter DEMO Watermarks*******

Portal Hypertension:
Definition:
Portal hypertension is defined by a portal pressure higher than 10 mm Hg.
Etiology:

******ebook converter DEMO Watermarks*******

Pathophysiology:

Portal hypertension usually occurs because of increased portal venous resistance that is prehepatic, intrahepatic,
or posthepatic in location.
increased portal venous inflow secondary to a hyperdynamic systemic circulation and splanchnic hyperemia is
a major contributor to the maintenance of portal hypertension as portal systemic collaterals develop.
The most common cause of prehepatic portal hypertension is portal vein thrombosis. is accounts for
approximately 50% of cases of portal hypertension in children.
Isolated splenic vein thrombosis (left-sided portal hypertension) is usually secondary to pancreatic
inflammation or neoplasm.
most common cause of intrahepatic presinusoidal hypertension is schistosomiasis.
nonalcoholic cirrhosis result in presinusoidal portal hypertension.
In contrast, alcoholic cirrhosis, the most common cause of portal hypertension usually causes increased
resistance to portal fow at the sinusoidal (secondary to deposition of collagen in the space of Disse) and
postsinusoidal (secondary to regenerating nodules distorting small hepatic veins) levels.
Posthepatic or postsinusoidal causes of portal hypertension are rare; they include Budd-Chiarisyndrome
(hepatic vein thrombosis), constrictive pericarditis, and heart failure.

Etiology and Clinical Features of Portal Hypertension:

The most significant clinical finding associated with portal hypertension is the development of gastro-
esophageal varices, which are mainly supplied by the anterior branch of the left gastric (coronary) vein.
Portal hypertension also results in splenomegaly with enlarged, tortuous, and even aneurysmal splenic vessels.
Splenomegaly is frequently associated with functional hypersplenism, causing leukopenia, thrombocytopenia,
and anemia.
Ascites occurs in the setting of severe portal hypertension in combination with hepatocyte dysfunction.
The umbilical vein may recannulate and dilate, leading to visible collaterals on the abdominal wall.
Anorectal varices are present in approximately 45% of cirrhotic patients.

******ebook converter DEMO Watermarks*******

Management of Gastroesophageal Varices:

The most leading cause of morbidity and mortality related to portal hypertension is variceal bleeding.
Approximately 30% of patients with compensated cirrhosis and 60% of patients with decompensated cirrhosis
have esophageal varices.

Prevention of Variceal Bleeding:

Current measures aimed at preventing variceal bleeding include the administration of nonselective Beta
blockers and prophylactic endoscopic surveillance with variceal band ligation.
Variceal band ligation is recommended for patients with medium to large varices, performed every 1 to 2
weeks until obliteration, followed by esophagogastroduodenoscopy (EGD) 1 to 3 months later and
surveillance EGD every 6 months to monitor for recurrence of varices.

Management of Acute Variceal Hemorrhage:

Patients with acute variceal hemorrhage should be admitted to an ICU for resuscitation and management.
Cirrhotic patients with variceal bleeding have a high risk of devel- oping bacterial infections, which are
associated with increased risks of rebleeding and mortality.
Spontaneous bacterial peritonitis accounts for approximately half of these infections, with urinary tract
infections and pneumonias comprising the remainder.
Although vasopressin is the most potent available vasoconstrictor, its use is limited by its systemic
vasoconstrictive effects that can produce hypertension, myocardial ischemia, arrhythmias, ischemic abdominal
pain, and limb gangrene.
Octreotide, a somatostatin analog, has the advantage that it can be administered for 5 days or longer,
and it is currently the preferred pharmacologic agent for initial management of acute variceal bleeding.

Luminal Tamponade:

Balloon tamponade using a Sengstaken-Blakemore tube will control refractory bleeding in up to 90% of
patients.
Associated with complications, which include aspiration, airway obstruction, and esophageal perforation due to
overinflation or pressure necrosis. Therefore, the use of a Sengstaken-Blakemore tube should not exceed 36
hours to avoid tissue necrosis, and this treatment modality should only be considered a temporary bridge to
more definitive measures of variceal hemorrhage control.
Tube is decompressed every 12 hourly-24hourly to prevent pressure necrosis.

Transjugular Intrahepatic Portosystemic Shunt:

The TIPS procedure involves implantation of a metallic stent between an intrahepatic branch of the portal vein
and a hepatic vein radicle.
The needle track is dilated until a portal pressure gradient of ≤12 mmHg is achieved.
TIPS controls variceal bleeding in>90% of cases refractory to medical treatment.
complications include bleeding either intra-abdominally or via the biliary tree, infections, renal failure,
decreased hepatic function, and hepatic encephalopathy, which occur in 25% to 30% of patients after the TIPS
procedure.

Balloon-Occluded Retrograde Transvenous Obliteration:

The balloon-occluded retrograde transvenous obliteration (BRTO) procedure has been used for the specific
management of bleeding gastric varices in patients with spontaneous gastrorenal or splenorenal shunts shown on
contrast-enhanced cross-sectional imaging.
Surgical Shunting:

At this time, the recommendation is that surgical shunts be consid-ered only in patients who have MELD
******ebook converter DEMO Watermarks*******
 scores of <15, who are not candidates for hepatic transplantation, or who have limited access to TIPS
therapy and the necessary follow-up.
The aim of the surgical shunt is to reduce portal venous pressure, maintain total hepatic and portal blood flow,
and avoid the high incidence of complicating hepatic encephalopathy.
Patient survival is determined by hepatic reserve.
The portacaval shunt, as first described by Eck in 1877, either joins the portal vein to the IVC in an end-to-side
fashion(known as Eck’s fistula) and completely disrupts portal vein flow to the liver, or joins it in a side-to-side
fashion and thereby maintains partial portal venous flow to the liver.
The mesocaval shunt uses an 8- or 10-mm polytetrafluo- roethylene (PTFE) graft to connect the superior
mesenteric vein to the IVC.
The smaller caliber of the shunt avoids the deleterious effects of portal blood flow deprivation on hepatic
function.
Small-diameter portosystemic shunts have been reported to reduce the incidence of encephalopathy but at the
expense of increased risks of shunt thrombosis and rebleeding.
The surgical shunt currently used most often is the distal splenorenal or Warren shunt.
This shunt is technical the most difficult to perform. It requires division of the gastro- esophageal collaterals
and allows venous drainage of the stomach and lower esophagus through the short gastrosplenic veins into the
spleen, and ultimately decompresses the left upper quadrant by allowing the splenic vein to drain directly into
the left renal vein via an end-to-side splenic to left renal vein anastomosis.
This shunt has the advantages of being associated with a lower rate of hepatic encephalopathy and
decompensation and not interfering with subsequent liver transplantation.

Nonshunt Surgical Management of Refractory Variceal Bleeding:

In the patient with extrahepatic portal vein thrombosis and refractory variceal bleeding, the Sugiura procedure
may be considered.
The Sugiura procedure consists of extensive devascularization of the stomach and distal esophagus along with
transection of the esophagus, splenectomy, truncalvagotomy, and pyloroplasty.

Hepatic Transplantation:

hepatic transplantation must be considered in the patient with ESLD, because it represents the patient’s only
chance for definitive therapy and long-term survival.
Hepatic transplantation also can be considered for the patient with variceal bleeding refractory to all other
forms of management.

Gall Bladder Abd Bile Ducts:

Anatomy:

The gall bladder lies on the underside of the liver in relation to Segment 4B and V.
It is a pear-shaped structure,
7.5–12 cm long,
normal capacity of about 25–30 mL.
The anatomical divisions are a fundus, a body and a neck that terminates in a narrow infundibulum.
The muscle fibres in the wall of the gall bladder are arranged in a criss-cross manner, being particularly well
developed in its neck.
The segment of the cystic duct adjacent to the gallbladder neck bears a vari- able number of mucosal folds
called the spiral valves of Heister. They do not have any valvular function but may make cannulation of the
cystic duct difficult.
The mucous membrane contains indentations of the mucosa that sink into the muscle coat; these are the crypts
of Luschka.
The cystic duct is about 3 cm in length, but the length is variable.
The lumen is usually 1–3 mm in diameter.
The mucosa of the cystic duct is arranged in spiral folds known as the ‘valves of Heister’ and the wall is
surrounded by a sphincteric structure called the ‘sphincter of Lütkens’.
******ebook converter DEMO Watermarks*******
 The cystic duct joins the supraduodenal segment of the common hepatic duct in 80 per cent of cases; however,
the anatomy may vary and the junction may be much lower in the retroduodenal or even retropancreatic part of
the bile duct.
Occasionally, the cystic duct may join the right hepatic duct or even a right hepatic sectorial duct.
CALOT’S TRIANGLE : The common hepatic duct constitutes the left border of the triangle of Calot, the other
corners of which were originally described as the cystic duct below and the cystic artery above.
The commonly accepted working definition of the triangle of Calot recognizes, however, the inferior surface of
the right lobe of the liver as the upper border and the cystic duct as the lower border with common hepatic duct
as the lateral border. Dissection of the triangle of Calot is of key signicance during cholecystectomy, because in
this triangle runs the cystic artery,
The common hepatic duct is usually less than 2.5 cm long and is formed by the union of the right and left
hepatic ducts.
The common bile duct is about 7.5 cm long and formed by the junction of the cystic and common hepatic
ducts.
It is divided into four parts:

1. Supraduodenal portion. About 2.5 cm long, running in the free edge of the lesser omentum.
2. Retroduodenal portion.
3. Infraduodenal portion/ retro-pancreatic portionlies in a groove, but at times in a tunnel, on the
posterior surface of the pancreas.
4. Intraduodenal portion passes obliquely through the wall of the second part of the duodenum, where it is
surrounded by the sphincter of Oddi, and terminates by opening on the summit of the ampulla of Vater.

The cystic artery, a branch of the right hepatic artery, usually arises behind the common hepatic duct

Sphincter of Oddi: The sphincter of Oddi, a thick coat of circular smooth muscle, surrounds the common bile duct
at the ampulla of Vater. It controls the flow of bile, and in some cases pancreatic juice, into the duodenum

Sphincter of ODDI is complex structure with 4 sphincters:

1. Upper sphincter choledochus.

2. Lower sphincter choledochus.
3. Sphincter pancreaticus.
4. Sphincter ampullae.

******ebook converter DEMO Watermarks*******

Embryology:

The hepatic diverticulum arises from the ventral wall of the foregut and elongates into a stalk to form the
choledochus.
A lateral bud is given off, which is destined to become the gall bladder and cystic duct.
The embryonic hepatic duct sends out many branches which join up the canaliculi between the liver cells.
As is usual with embryonic tubular structures, hyperplasia obliterates the lumina of this ductal system;
normally, recanalisation subsequently occurs and bile begins to flow.
During early fetal life, the gall bladder is entirely intrahepatic.
CONGENITAL ABNORMALITIES OF THE GALL BLADDER AND BILE DUCTS.

Absence of the gall bladder:

Occasionally, the gall bladder is absent.
The Phrygian cap:

The Phrygian cap is present in 2–6 per cent of cholecystograms and may be mistaken for a pathological
deformity of the organ.
‘Phrygian cap’ refers to hats worn by the people of Phrygia, an ancient country of Asia Minor.

Floating gall bladder:

The gall bladder may hang on a mesentery, which makes it liable to undergo torsion.
Absence of the cystic duct:
This is usually a pathological, as opposed to an anatomical anomaly and indicates the recent passage of a stone or
the presence of a stone at the lower end of the cystic duct, which is ulcerating into the common bile duct.
Low insertion of the cystic duct:
Dissection of a cystic duct which is inserted low in the bile duct should be avoided, as removal will damage the
blood supply to the common bile duct and can lead to stricture formation.
An accessory cholecystohepatic duct:
Ducts passing directly into the gall bladder from the liver. Larger ducts should be closed, but before doing so the
precise anatomy should be carefully ascertained to ensure a right hepatic duct is not being ligated.

******ebook converter DEMO Watermarks*******

Extrahepatic Biliary Atresia:
Aetiology and physiology:

Atresia is present in approximately 1 per 12 000 live births.

affects males and females equally.
The extrahepatic bile ducts are progressively destroyed by an inflammatory process which starts around the
time of birth.
The aetiology is unclear.
Intrahepatic changes also occur and eventually result in biliary cirrhosis and portal hypertension.
Untreated, death from the consequences of liver failure occurs before the age of three years.
USG and HIDA scan are useful for diagnosis.

The inflammatory destruction of the bile ducts has been classified into three main types

Type I: atresia restricted to the common bile duct;

Type II: atresia of the common hepatic duct;
Type III: atresia of the right and left hepatic ducts.

******ebook converter DEMO Watermarks*******

Clinical features:

About one-third of patients are jaundiced at birth.

Liver function tests show an obstructive pattern with elevated bilirubin and alkaline phosphatase.
The meconium may be a little bile stained, but later the stools are pale and the urine is dark.
Prolonged steatorrhoea gives rise to osteomalacia (biliary rickets).
Pruritus is severe.
Clubbing and skin xanthomas, probably related to a raised serum cholesterol, may be present.

Treatment:

Roux-en-Y hepaticojejunostomy: for type I and II.

Kasai procedure (portoenterostomy) for type III and some cases of type II.
Liver transplantation should be considered in children in whom a portoenterostomy is unsuccessful.

Gallstones (Cholelithiasis):

Gallstones are the most common biliary pathology.

SAINT’s triad: Gall stones; Hiatus hernia and Diverticulosis.

Causal factors in gallstone formation:

Gallstones can be divided into three main types:
1. Cholesterol stones.
2. Pigment (brown/black).
3. mixed stones (cholesterol and pigment).

In the United States and Europe, 80 per cent are cholesterol or mixed stones, whereas in Asia, 80 per
cent are pigment stones.
Cholesterol or mixed stones contain 51–99 per cent pure cholesterol plus an admixture of calcium salts,
bile acids, bile pigments and phospholipids.
When bile is supersaturated with cholesterol or bile acid concentrations are low, unstable unilamellar
phospholipid vesicles form, from which cholesterol crystals may nucleate, and stones may form.
Cholestrol stones are solitaire in shape and yellow in colour.
Cholesterol stones are usually solitary.
Mixed stones are Mullbery in shape and usually multiple in number
Obesity, high-caloric diets and certain medications (e.g. oral contraceptives) can increase secretion of
cholesterol and supersaturate the bile increasing the lithogenicity of bile.
Resection of the terminal ileum, which diminishes the enterohepatic circulation, will deplete the bile acid pool
and result in cholesterol supersaturation.
Pigment stone is the name used for stones containing less than 30 per cent cholesterol.

******ebook converter DEMO Watermarks*******

 They are Multifaceted in shape and multiple in number.
There are two types – black and brown.
Black stones are largely composed of an insoluble bilirubin pigment polymer mixed with calcium phosphate
and calcium bicarbonate.
Overall, 20–30 per cent of stones are black.
The incidence rises with age.
Black stones are associated with haemolysis, usually hereditary, spherocytosis or sickle cell disease.
For reasons that are unclear, patients with cirrhosis have a higher instance of pigmented stones.
Brown pigment stones contain calcium bilirubinate, calcium palmitate and calcium stearate, as well as
cholesterol.
Brown stones are rare in the gall bladder.
They form in the bile duct and are related to bile stasis and infected bile.
Stone formation is related to the deconjugation of bilirubin deglucuronide by bacterial glucuronidase. Insoluble
unconjugated bilirubinate precipitates.
Brown pigment stones are also associated with the presence of foreign bodies within the bile ducts, such as
endoprosthesis (stents), or parasites, such as Clonorchis sinensis and Ascaris lumbricoides.

Clinical presentation:

Gallstones may remain asymptomatic, being detected incidentally as imaging is performed for other symptoms.
If symptoms occur, patients typically complain of right upper quadrant or epigastric pain, which may
radiate to the back.
This may be described as colicky, but more often is dull and constant.
Murphy’s sign: jerk of breath on palpation of right upper quadrant
Other symptoms include dyspepsia, flatulence, food intolerance, particularly to fats, and some alteration in
bowel frequency.
Biliary colic is typically present in 10–25 per cent of patients. This is described as a severe right upper quadrant
pain which ebbs and flows associated with nausea and vomiting.
Pain may radiate to the chest.
The pain is usually severe and may last for minutes or even several hours. Frequently, the pain starts during the
night and wakes the patient.
Dyspeptic symptoms may coexist and be worse after such an attack.
Jaundice may result if the stone migrates from the gall bladder and obstructs the common bile duct.

******ebook converter DEMO Watermarks*******

 Rarely, a gallstone can lead to bowel obstruction (gallstone ileus)
Mc site for stone impaction in bowel is terminal ileum.
Riglers triad: ectopic gall stone ;pneumobilia and intestinal obstruction.
When the symptoms do not resolve, but progress to continued pain with fever and leukocytosis, the diagnosis
of acute cholecystitis should be considered.

Complications of gall stones:

Diagnosis:

IOC for gall stones is USG.

A diagnosis of gallstone disease is based on the history and physical examination with confirmatory
radiological studies, such as transabdominal ultrasonography and radionuclide scans.
A mass may be palpable as the omentum walls off an inflamed gall bladder.

Treatment:

Most consider that it is safe to observe patients with asymptomatic gallstones, with cholecystectomy
reserved for patients who develop symptoms or complications.
However, prophylactic cholecystectomy may be considered for diabetic patients, those with congenital
haemolyticanaemia and those patients who are undergoing bariatric surgery for morbid obesity as it has been
found in these groups that the risk of developing symptoms is increased.
For patients with biliary colic or cholecystitis, cholecystectomy is the treatment of choice if there are no
medical contraindications.
Non-operative treatment is based on four principles:
Nil per mouth (NPO) and intravenous fluid administration until the pain resolves.
Administration of analgesics.
Administration of antibiotics.
Subsequent management. When the temperature, pulse and other physical signs show that the inflammation is
subsiding, oral fluids are reinstated followed by regular diet. Ultrasonography is performed to confirm the
diagnosis.
If jaundice is present, an MRCP is performed to exclude choledocholithiasis.
If there is any concern regarding the diagnosis or presence of complications, such as perforation, a CT should
be performed.
Cholecystectomy may be performed on the next available list, or the patient may be allowed home to return
later when the inflammation has completely resolved.
Conservative treatment must be abandoned if the pain and tenderness increase; depending on the status
of the patient, either operative intervention and cholecystectomy should be performed or if the patient
has comorbid conditions, a percuta-neouscholecystostomy can be performed by a radiologist under
ultrasound control.
Early cholecystectomy during acute cholecystitis appears to be safe and shortens the total hospital stay.

******ebook converter DEMO Watermarks*******

 Provided that the operation is undertaken within 5–7 days of the onset of the attack, the surgeon is
experienced and excellent operating facilities are available, good results are achieved.
If an early operation is not indicated, one should wait approximately 6 weeks for the inflammation to
subside before operating.

Empyema of the Gall Bladder:

Empyema may be a sequele of acute cholecystitis or the result of a mucocoele becoming infected.
The gall bladder is distended with pus.
The optimal treatment is drainage (cholecystostomy and, later, cholecystectomy.

Acalculouscholecystitis:

Acute and chronic inflammation of the gall bladder can occur in the absence of stones and give rise to a clinical
picture similar to calculous cholecystitis.
Some patients have non-specific inflammation of the gall bladder, whereas others have one of the
cholecystoses.
Acute acalculouscholecystitis is par- ticularly seen in critically ill patients and those recovering from major
surgery, trauma and burns.

The Cholecystoses (Cholesterosis, Polyposis, Adenomyomatosisand Cholecystitis Glandularis

Proliferans):

This is a relatively uncommon group of conditions affecting the gall bladder, in which there are chronic
inflammatory changes with hyperplasia of all tissue elements.

Cholesterosis (‘strawberry gall bladder’):

submucous aggregations of cholesterol crystals and cholesterol esters(yellow specks like seeds of strawberry).
the interior of the gall bladder looks some- thing like a strawberry.
It may be associated with cholesterol stones.

Cholesterol polyposis of the gall bladder:

associated with cholesterol polyposis or adenomatous change.

surgery is advised only if there is a change in size over time or are larger than 1 cm.

Diverticulosis of the gall bladder:

Diverticulosis of the gall bladder is usually manifest as black pigment stones impacted in the outpouchings of
the lacunae of Luschka.
Diverticulosis of the gall bladder may be demonstrated by cholecystography, especially when the gall bladder
contracts after a fatty meal.
There are small dots of contrast medium just within and outside the gall bladder. A septum may also be present
to be distinguished from the Phrygian cap.
The treatment is cholecystectomy.

Typhoid infection of the gall bladder:

Salmonella typhior S. typhimurium can infect the gall bladder.

Acute cholecystitis can occur.
More frequently, chronic cholecystitis occurs, the patient being a typhoid carrier excreting the bacteria in the
bile.
Gallstones may be present.
Salmonellae can, however, frequently be cultured from these stones. Treatment with ampicillin/ quinolones and
******ebook converter DEMO Watermarks*******
 cholecystectomy are indicated.

Bile Duct Stones (Choledocholithiasis):

Essentials of Diagnosis:

Biliary pain.
Jaundice.
Episodic cholangitis.
Gallstones in gallbladder or previous cholecystectomy.

General Considerations:

Approximately 15% of patients with stones in the gallbladder have CBD stones.
Common duct stones are associated with gallbladder stones but in 5% of cases, the GB is empty.
There are two possible origins for common duct stones. The evidence suggests that most cholesterol stones
develop within the gallbladder and reach the duct after traversing the cystic duct. These are called secondary
stones. Pigment stones may have a similar pedigree or, more often, develop de novo within the common duct.
These are called primary common duct stones.
About 60% of common duct stones are cholesterol stones and 40% are pigment stones. The latter are generally
associated with more severe clinical manifestations.
Patients may have one or more of the following principal clinical findings: biliary colic, cholangitis, jaundice,
and pancreatitis. It seems likely, however, that as many as 50% of patients with choledocholithiasis remain
asymptomatic.
Biliary colic is the result of rapid rises in biliary pressure whether the block is in the common duct or neck of
the gallbladder. Gradual occlusion of the duct—as in cancer—rarely produces the same kind of pain as
gallstone disease.
Choledocholithiasis may be asymptomatic or may produce sudden toxic cholangitis.
Choledocholithiasis should be strongly suspected if intermittent chills, fever, or jaundice accompanies biliary
colic.
The patient may be icteric and toxic, with high fever and chills. A palpable gallbladder is unusual in patients
with obstructive jaundice from common duct stone because the obstruction is transient and partial, and scarring
of the gallbladder renders it inelastic and nondistensible.
Tenderness may be present in the right upper quadrant but is not often as marked as in acute cholecystitis,
perforated peptic ulcer, or acute pancreatitis. Tender hepatic enlargement may occur.

Laboratory Findings:

In cholangitis, leukocytosis of 15,000/mL is usual, and values above 20,000/mL are common. A rise in serum
bilirubin often appears within 24 hours after the onset of symptoms. The absolute level usually remains under
10 mg/dL, and most are in the range of 2–4 mg/dL. The direct fraction exceeds the indirect. Bilirubin levels do
not ordinarily reach the high values seen in malignant tumors because the obstruction is usually incomplete and
transient. In fact, fluctuating jaundice is very characteristic of choledocholithiasis.
The serum alkaline phosphatase level usually rises and may be the only chemical abnormality in patients
without jaundice. When the obstruction is relieved, the alkaline phosphatase and bilirubin levels should return
to normal within 1–2 weeks.
Mild increases in AST and ALT are often seen with extrahepatic obstruction of the ducts; rarely, AST levels
transiently reach 1000 units.

Imaging Studies:

Radiopaque gallstones may be seen on plain abdominal films or CT scans (but very few are RO).
Ultrasound scans will usually show gallbladder stones and, depending on the degree of obstruction, dilatation
of the bile duct. Ultrasound and CT scans are insensitive in the search for stones in the common duct.
Ultrasonography, commonly the first test, can document stones in the gallbladder and estimate the diameter
of the common bile duct. A dilated bile duct (>8 mm in diameter) on ultrasonography in a patient with
******ebook converter DEMO Watermarks*******
 gallstones, jaundice and biliary pain is highly suggestive of choledocholithiasis
MRCP (IOC) provides excellent anatomic detail, with sensitivity and specificity of 95% and 98%,
respectively, for common bile duct stones, avoids the need for invasive ERCP.
ERCP has diagnostic value but considering its invasive nature and potential side effects it is only used now for
its therapeutic role.

Complications:

Longstanding ductal infection can produce intrahepatic abscesses. Hepatic failure or secondary biliary cirrhosis
may develop in unrelieved obstruction of long duration. Acute pancreatitis, a fairly common complication of
calculous biliary disease. Hemorrhage (hemobilia) is also a rare complication.

Treatment:

Patients with acute cholangitis should be treated with systemic antibiotics and other supportive measures this
usually controls the attack within 24–48 hours. If the patient’s condition worsens or if marked improvement is
not observed within 2–4 days, endoscopic sphincterotomy or surgery and common bile duct exploration should
be performed.
Patients with common duct stones are best treated by endoscopic sphincterotomy with stone extraction
(ERCP). Using a side-viewing duodenoscope, the ampulla is cannulated, and a 1-cm incision is made in the
sphincter with an electrocautery wire. The opening created in the sphincter permits stones to pass from the duct
into the duodenum.
Prompt cholecystectomy after endoscopic clearance of the common bile duct should be performed during the
hospital admission if the patient is fit for surgery.
Endoscopic sphincterotomy is unlikely to be successful in patients with large stones (eg, > 2 cm), and it is
contraindicated in the presence of stenosis of the bile duct proximal to the sphincter. If ERCP clearance is not
possible because of multiple stones, intrahepatic stones, impacted stones, difficulty with cannulation, duodenal
diverticula, or biliary stricture Laparoscopic CBD exploration or Laparotomy and common duct exploration is
required.

Choledochal Cyst:

Choledochal cyst is congenital cystic dilatation of the common bile duct.

Todani classified choledochal cysts into 5 types and several subtypes.

Type I lesions consist of concentric dilatations of the common bile or cystic duct, which may be diffuse or cysti:
type IA, in which cystic dilatation involves nearly the entire common bile duct (CBD); type IB, in which there is
more segmental dilatation of the CBD; and type IC, in which dilatation is diffuse throughout the CBD and
common hepatic duct. Type I is the most common variant of bile duct cyst.
Type II lesions involve a supraduodenal eccentric dilatation.
Type III cysts, or choledochoceles, are dilatations of the CBD within the muscular portion of the duodenal wall.
Type IVA cysts consist of multiple cystic dilatations that affect both the intra- and extrahepatic ducts; this type
accounts for 18% of reported cases. Type IVB cysts affect the extrahepatic ducts and are much less common.
Type V, or classical Caroli’s disease describes cysts of only the intrahepatic ducts. Caroli’s syndrome applies to
Caroli’s disease in association with congenital hepatic fibrosis. Caroli’s syndrome is transmitted as an autosomal
recessive trait and is associated with adult polycystic renal disease.

******ebook converter DEMO Watermarks*******

 Choledochal cysts has incidence of 1/1-1.5 lacs live births.
Although their etiology is not known, one hypothesis involves anomolous pancreaticco biliary ductal union
(APBDU), which impairs sphincter of Oddi function and permits reflux of pancreatic juice into the bile duct,
with subsequent weakening of the biliary system.
Another hypothesis considers ductal plate anomalies (DPA). This mechanism may involve partial or complete
arrest of remodeling of the ductal plate of the larger intrahepatic bile ducts.
Symptoms present mostly in childhood or early adulthood. In infancy, painless or intermittent jaundice with
acholic stools is common. Infants may present with nausea, vomiting, and failure to thrive as a result of
luminal compression or with a smooth, palpable abdominal mass in the right hypochondrium.
Complications associated with choledochal cysts are largely due to obstruction and include jaundice, stone
formation, recurrent cholangitis, hepatic abcess, portal vein thrombosis, pancreatitis, cyst rupture, secondary
biliary cirrhosis, and carcinoma.
Malignant change is estimated to occur in 3%-20% of cases.
Abdominal ultrasound and CT scan are valuable. ERCP is a definitive method for diagnosing choledochal cyst.
In the absence of any unrelated findings such as mass, lymphadenopathy, or filling defects, no other diagnostic
test is needed.
Treatment is usually surgical.

Classification and the Surgical Treatment of Choledochal Cysts:

******ebook converter DEMO Watermarks*******

Cholangitis:

Cholangitis is an acute infection of the biliary tree. compounded by presence of bacteria.

Common bile duct (CBD) stones are the most common cause of obstruction.
Bile stasis may be caused by strictures, stenosis, tumors, or endoscopic manipulation of the CBD.
Partial obstruction is more likely to cause cholangitis than complete obstruction.

History:

In 1877, Charcot described cholangitis as a triad of findings of right upper quadrant pain, fever, and
jaundice.
Reynold pentad adds mental status changes and sepsis to the triad.
Charcot triad of fever, RUQ pain, and jaundice is found in 70% of patients presenting with cholangitis. Most
patients complain of right upper quadrant pain.

Imaging Studies:

Sonograms followed by CT scan are used most commonly.

ERCP or percutaneous cholangiogram images and treats the obstruction directly.
Ultrasonography is fairly sensitive for intrahepatic and extrahepatic, including CBD dilation; however, it is
not reliable for choledocholithiasis. Ultrasound can differentiate intrahepatic from extrahepatic obstruction and
image dilated ducts.

CT scan: Dilated intrahepatic and extrahepatic ducts and inflammation of the biliary tree are imaged. Gallstones
are visualized poorly with CT scan.
Biliary scintigraphy (HIDA and DISIDA) are functional studies of the gallbladder.
Obstruction of the common bile duct causes nonvisualization of the small intestine. A HIDA scan with complete
biliary obstruction does not visualize the biliary tree.
Advantages include its ability to assess function, and positive results may appear before the ducts are
sonographically enlarged.
Treatment:
Standard therapy for cholangitis is broad-spectrum antibiotics with close observation to determine the need for
emergency decompression.
From the surgical literature, in patients with mild cholangitis, 70-85% of patients will respond to medical therapy.
Approximately 15% will not respond and will require immediate surgical or endoscopic decompression. In severely
ill patients, treatment is immediate biliary decompression by percutaneous or endoscopic drainage.
******ebook converter DEMO Watermarks*******
Cholecystectomy or ERCP is best after resolution of the cholangitis.
Recurrent Pyogenic Hepatitis (Oriental Cholangiohepatitis):

It is characterized by formation of intrahepatic pigmented stones with recurrent exacerbation and remission of
abdominal pain, frequently associated with jaundice, chills, and fever.
The cause is secondary to infections with coliform bacteria or parasites such as Clonorchissinensis, causing
pigmented stone formation by inducing the precipitation of bilirubin, acting as nidus for stone formation, or
causing biliary strictures that lead to further biliary stasis.
The hallmark of the disease is presence of soft pigmented bilirubinate stones within dilated intra- and
extrahepatic ducts.

Clinical presentation:

Characterized by recurrent attacks of right upper quadrant pain, fever, chills, and jaundice

Laboratory findings:

Increased polymorphonuclear leukocytes,

Elevated levels of alkaline phosphatase, and
Excretion of urobilinogen in urine.

MRCP is the gold standard for evaluation for RPC:

Direct cholangiography--such as endoscopic retrograde cholangiography, percutaneous transhepatic

cholangiography, operative cholangiography, and T-tube cholangiography--demonstrates the full spectrum of
ductal changes and stones.
Treatment of this disease focuses on management of acute cholangitis, followed by either drainage and removal
of stones using endoscopic, radiologic, or surgical methods, or hepatic resection for focal disease.

Haemobilia:

Pain, jaundice and gastrointestinal haemorrhage (Quinke’s triad) are the classical presenting symptoms of
bleeding into the biliary tree.
This may be caused by trauma, an aneurysm, or a hepatic tumour, but it is most commonly associated with
liver biopsy.
Retrograde cholangiography may reveal clots in the bile duct, but hepatic angiography will define the site of
haemorrhage.
Selective hepatic angiography with embolization of the bleeding vessel is an effective means of treatment
which obviates the necessity for laparotomy.

Bile Duct Strictures:

Most benign bile duct strictures are iatrogenic, resulting from operative trauma.
Laparoscopic cholecystectomy is most common iatrogenic cause.

Other conditions leading to benign strictures are.

pancreatitis bile duct stones.

primary sclerosing cholangitis (PSC).
HIV cholangiopathy (Cryptosporidium and Cytomegalovirus may be responsible).
Mirizzi syndrome (Pressure on the common hepatic duct due to a gallstone impacted in the Hartmann pouch
or cystic duct results in jaundice and cholangitis).
Choledochal cyst, Recurrent pyogenic cholangitis. Bile duct strictures can cause ascending cholangitis, liver
abscess, and secondary biliary cirrhosis.
Periampullary carcinoma is the most common cause of malignant biliary strictures.
******ebook converter DEMO Watermarks*******
History:

Most patients with biliary strictures remain asymptomatic until the lumen of the bile duct is sufficiently
narrowed.
Occasionally, patients may have intermittent episodes of right upper quadrant pain (biliary colic) or features of
obstructive jaundice; pruritus, yellow discoloration of skin, and steatorrhea.
With chronic cholestasis, xanthomas appear around the eyes, chest, back, and on extensor surfaces.
Patients presenting with cholangitis also may have fever and right upper quadrant tenderness in addition to
jaundice (ie, Charcot triad),hypotension, and altered mental status (ie, Reynold pentad).

Lab Studies:

Elevated serum alkaline phosphatase and gamma-glutamyltranspeptidase.

increases in total and conjugated bilirubin. Alkaline phosphatase levels are increased to more than 3-times
normal.
In malignant strictures causing only partial obstruction (eg, Klatskin tumor), a rise in the alkaline phosphatase
level may not be accompanied by a rise in the bilirubin level.

Imaging Studies:

The initial radiological study should be an ultrasound.

If it shows dilated bile ducts but do not provide clues to the site or cause of the obstruction, magnetic resonance
cholangiopancreatography (MRCP) or abdominal CT scans should be performed next.
In some patients, endoscopic retrograde cholangiopancreatography (ERCP) may be needed for definitive
diagnosis and has the advantage of being therapeutic.
Hepatic iminodiacetic acid scan: HIDA scan is commonly used for the diagnosis of biliary leaks. HIDA
scanning can help determine the clearance of bile across strictures and surgical anastomosis.
HIDA scan findings suggest complete biliary obstruction if the small intestine is not visualized in 60
minutes. HIDA scanning also is useful for distinguishing cholangitis from cholecystitis.

Staging:
Bismuth proposed an anatomic classification based on location, into 5 types:

Type 1: Low common hepatic duct stricture. 2 cm of the hepatic duct is intact.
Type 2: Mid common hepatic duct stricture. The hepatic duct stump is < 2 cm.
Type 3: This is a hilar stricture. The common hepatic duct is not involved, but the confluence of right and
left hepatic ducts is intact.
Type 4: In this type, the hilar confluence is destroyed. The right and left hepatic ducts are separated.
Type 5: The aberrant right sectorial duct is involved, alone or with the CBD.

Strasberg Classification:

******ebook converter DEMO Watermarks*******

Treatment:
Medical Care:
Medical treatment consists of managing complications of bile duct strictures until definitive therapy can be
instituted.
Surgical Care:
1. endoscopic or percutaneous balloon dilatation and insertion of an endoprosthesis (Sphincterotomy and
endoscopic balloon dilation, Endoscopic biliary stenting, PTC and biliary stenting).
2. surgery:Operative treatment: Surgical management consists of restoration of biliary enteric continuity, which
******ebook converter DEMO Watermarks*******
 usually is achieved with a defunctionalized Roux-en-Y jejunal loop by hepaticojejunostomy,
choledochojejunostomy or intrahepatic cholangiojejunostomy.
Carcinoma of the Gallbladder:

It occurs in elderly patients and is associated with gallstones in 70-90% of cases with risk of malignancy
directly proportional to the length of time gallstones have been present.
The tumor is twice as common in women as in men.
Most primary tumors of the gallbladder are adenocarcinomas that appear histologically to be scirrhous (60%),
papillary (25%), or mucoid (15%).
Dissemination of the tumor occurs early by direct invasion of the liver and hilar structures and by metastases to
the common duct lymph nodes, liver, and lungs.
Though occasionally carcinoma is an incidental finding after cholecystectomy for gallstone disease where the
tumor is confined to the gallbladder as a carcinoma in situ but more often carcinoma have spread by the time of
surgery, and spread is virtually certain if the tumor has progressed to the point where it causes symptoms.
Nevin’s classification for Gall bladder cancer:

Stage I: mucosa only (45% 2yr survival)

Stage II: muscularismuscularis (15%)
Stage III: all layers (4%)
Stage IV: lymph nodes (2%)
Stage V: liver invasion, adjacent organs, distant metastasis
Treatment:

In the few cases where cancer has not penetrated the muscularis mucosae, cholecystectomy alone should
suffice.
If a localized carcinoma of the gallbladder is recognized at laparotomy, cholecystectomy should be performed
along with en bloc wedge resection of an adjacent 3–5 cm of normal liver and dissection of the lymph nodes in
the hepatoduodenal ligament.
If a small invasive carcinoma overlooked during cholecystectomy for gallstone disease is later discovered by
the pathologist, reoperation is indicated to perform a wedge resection of the liver bed plus regional
lymphadenectomy. Some surgeons also recommend that the common duct be included routinely (ie, even in the
absence of gross invasion) in the lymph node dissection for any lesion that involves the full thickness of the
gallbladder wall. More extensive hepatectomies (eg, right lobectomy) are not worthwhile.
Lesions that invade the bile duct and produce jaundice should be resected if possible. When not a stent should
be inserted endoscopically or percutaneously. There is little that surgery can offer in cases with hepatic
metastases or more distant spread.

Prognosis:

Radiotherapy and chemotherapy are not effective palliative measures. About 85% of patients are dead within a
year after diagnosis. Mean survival time for nonoperable patient is less than 6 montths.
The 10% of patients who presently survive more than 5 years consist of those whose carcinoma was an
incidental finding during cholecystectomy for symptomatic gallstone disease and those in whom an aggressive
resection has removed all gross tumor.

Pancreas:

The pancreas is a lobulated gland lying retroperitoneally, measuring 12-15cm in length, with a head and an
uncinate process, which lie within the curve of the duodenum.

Embryological development:

The pancreas develops from separate ventral and dorsal buds that arise from the junction of the primitive
foregut and midgut.
******ebook converter DEMO Watermarks*******
 The dorsal bud enlarges towards the left, and forms the main bulk of the adult gland.
The ventral bud, which is closely associated with the developing common bile duct is brought into apposition
with the dorsal system only in the seventh week of intrauterine growth, following its rotation.
Both parts of the primitive pancreas contain axial ducts, the dorsal duct arising from the duodenal wall, and the
ventral duct from the common bile duct.
When they fuse, the ventral duct (of Wirsung) becomes continuous with the dorsal duct (of Santorini) to form
the main pancreatic duct.
The common bile duct and pancreatic ducts therefore enter the duodenum at the main papilla, while the portion
of the dorsal duct within the head of the pancreas enters the duodenum proximally to the main papilla, through
a small accessory, or minor papilla.
Complete failure of the two duct systems to fuse results in a pancreas divisum (Fig 2- 5%) and may
predispose to pancreatitis.
Failure of the body of the ventral bud to rotate may give rise to an annular pancreas surrounding the second part
of the duodenum. This may be responsible for duodenal obstruction.

Pancreatitis:
Acute pancreatitis is an inflammatory process in which pancreatic enzymes autodigest the gland.
The gland can heal without any impairment of function or any morphologic changes.
It can recur intermittently, contributing to the functional and morphologic loss of the gland.
Recurrent attacks are referred to as chronic pancreatitis.
Pathophysiology:

Since the pancreas is located in the retroperitoneal space with no capsule, inflammation can easily spread. In
acute pancreatitis, parenchymal edema and peripancreatic fat necrosis occur first, called acute edematous
pancreatitis.
When necrosis involves the parenchyma, accompanied by hemorrhage and dysfunction of the gland, the
inflammation evolves into hemorrhagic or necrotizing pancreatitis.
Pseudocysts and pancreatic abscesses can result from necrotizing pancreatitis, due to enzymes being walled off
by granulation tissue (pseudocyst formation) or bacterial seeding of pancreatic or peripancreatic tissue
(pancreatic abscess formation).
The inflammatory process can cause systemic effects due to the presence of cytokines such as bradykinins and
phospholipase A. These cytokines may cause vasodilation, increase in vascular permeability, pain, and
leukocyte accumulation in the vessel walls. Fat necrosis causes hypocalcemia. Pancreatic B cell injury may
lead to hyperglycemia.
Trypsin and chymotrypsin are the initiating enzymes; their release can in turn result in the release and
activation of other proenzymes (including proelastase, procollagenase and phospholipases). Trypsin damages
endothelial cells and mast cells, resulting in the release of histamine. This major inflammatory mediator
enhances vascular permeability, leading to edema, hemorrhage and the activation of the kallikrein system,
which in turn results in the production of vasoactive peptides or kinins. The latter are thought to cause pain and
further aggravate the inflammatory response. The other released enzymes destroy the supporting matrix of the
gland and the plasma membrane of the acinar cell, precipitating further release of digestive enzymes, which in

******ebook converter DEMO Watermarks*******

 turn leads to further damage. Lysolecithin, which is released by the action of phospholipase on lecithin (a
phospholipid found in bile), has also been implicated in pancreatic damage, because of its cytotoxic and
hemolytic properties. When the pancreas is inflamed but remains viable, the condition is termed interstitial
pancreatitis; this may occur in up to 80% of cases. In the remaining cases, there is significant pancreatic
necrosis resulting from disruption of the microcirculation, destruction of the pancreatic parenchyma and
peripancreatic.

History:

Epigastric pain or right upper quadrant pain radiating to the back.

Nausea/vomiting.
Fever.
The patient should be asked about recent surgeries and invasive procedures (i.e., ERCP) or family history of
hypertriglyceridemia.
There is frequently a history of previous biliary colic, and binge alcohol consumption, the major causes of acute
pancreatitis.

Physical:

Tachycardia/ Tachypnea/ Hypotension/ Fever.

Abdominal tenderness, distension, guarding and rigidity. In severe cases, there may be a Grey Turner sign (i.e.,
bluish discoloration of the flanks) and Cullen’s sign (i.e., bluish discoloration of the periumbilical area) due to
the retroperitoneal leak of blood from the pancreas in hemorrhagic pancreatitis and Fox sign: Discolouration
along inguinal ligamnet.
Mild jaundice.
Diminished or absent bowel sounds.
Basal rales in lungs, (especially in the left due to contiguous spread of inflammation).
In the extremities, muscular spasm may, be noted secondary to hypocalcemia.
Multisystem organ failure (ARDS, renal failure from ATN), shock, DIC and hemorrhage.
Pleural effusions, pneumonia, and atelectasis.
Formation of pancreatic fluid collections (pseudocysts and abscesses) account for 70% to 80% of mortality.
Ileus, colonic obstruction, CNS hypoperfusion with confusion, etc.

Causes:

The major causes are long-standing alcohol intake or biliary stone disease.
Medications: azathioprine, corticosteroids, sulfonamides, thiazides, furosemides, NSAIDs, mercaptopurine,
methyldopa, tetracyclines DDI (dideoxycytosine), DDC (dideoxyinosine), azathioprine, valproic acid,
acetaminophen, and others.
Endoscopic retrograde cholangiopancreatography (ERCP).
Hypertriglyceridemia: When the triglyceride level exceeds 1000mg/U.
Peptic ulcer disease.
Abdominal or cardiopulmonary bypass surgery: insulting the gland by ischemia.
Trauma to the abdomen or to the back.
Carcinoma of the pancreas, which may lead to pancreatic outflow obstruction.
Viral infections: cytomegalovirus, hepatitis virus, EBV, CMV and paramyxovirus [mumps], togavirus
[rubella], cytomegalovirus, adenovirus, HIV, coxsackie B.
Bacterial infections, such as Mycoplasma, Campylobacter, Legionella, Mycobacterium tuberculosis, M.
avium complex.
Intestinal parasites: Ascaris, Opisthorchis [clonorchiasis], which blocks outflow.
Pancreas divisum.
Black Scorpion and snake bites.
Vascular factors, such as ischemia or vesculitis, Connective-tissue disorders (SLE, polyarteritisnodosa,
sarcoidosis).
Drugs: 5- Aminosalicylic acid, Azathiopine, 6 Mercaptopeurine, Thiazide, steroid, Pentamedine,
Metronidazole, Valproic acid, L-Asperginase, Zidovudine.
******ebook converter DEMO Watermarks*******
Objective measurements such as Ranson’s criteria, show a good correlation with the risk of major complications and
death.
Ranson’s criteria:
Ranson developed a series of different criteria for the severity of acute pancreatitis.

Poor prognostic indicators (Ranson’s criteria 1978, modified by Hollander 1983):

Workup:
Lab Studies:
A complete blood count will show leukocytosis (WBC > 12000) with the differential being shifted towards the
segmented polymorphs.
BUN, Cr and electrolytes (Na, K, Cl, Carbon dioxide, P, Mg) should be ordered. Patient may have hypocalcemia
caused by “soap” formation (saponification of triglycerides and calcium). Frequently have hypomagnesemia.
Amylase:
Preferably the Amylase P: Levels more than 3 times the norm strongly suggest the diagnosis of acute pancreatitis.
Amylase is elevated in 80% of those with pancreatitis and is more sensitive early on.
Lipase:
Is more sensitive if symptoms have been present for more than 24 hours. Both amylase and lipase levels may be
normal in a patient with CT-proved pancreatitis.
Lipase remains high for 12 days.

******ebook converter DEMO Watermarks*******

In patients with chronic pancreatitis (usually due to alcohol abuse), lipase may be elevated in the presence of a
normal serum amylase level.
Liver function tests (alkaline phosphatase, SGPT, SGOT, G-GT) and bilirubin should be ordered, particularly with
biliary origin Pancreatitis.
Extrapancreatic conditions may cause hyperamylasemia:
Abdominal conditions that may cause elevations in the serum amylase includes:
Perforated peptic ulcer/ Cholecystitis/ Generalised peritonitis/ Intestinal obstruction.
Mesenteric infarction/ Ruptured AAA/ Ruptured ectopic pregnancy.
Two-thirds of diabetic ketoacidosis is associated with hyperamylasemia and may present a confusing clinical
picture, particularly when it is associated with shock
It has been estimated that as many as 2-5% of cases of hyperamylasemia are due to macroamylasemia, a benign
condition resulting from the binding of amylase to macro-molecules too large to be filtered through the kidneys.
Serum lipase also rises rapidly, within hours after an attack:
Reference range for serum lipase is, 130 U/l
Lipase is measured via turbidimetric method:
Sensitivity of lipase for acute pancreatitis approaches 100%
Lipase lacks specificity and may be elevated in:
Acute cholecystitis.
Choledocholithiasis.
Mesenteric infarction.
Intestinal obstruction.
Urine amylase:
Urine amylase rises a few hours after the rise sin serum amylase and lipase.
Urine amylase remains elevated for approximately seven to ten days.
The rise in urine amylase is secondary to an increase in the renal clearance of amylase.
Amylase clearance is used to differentiate between a patient with macroamylasemia secondary to hyperamylasemia
from a patient with pancreatitis.
Normal ratio for amylase clearance is between 2-5%.
In macroamylasemia, the ratio is decreased.
In pancreatitis, the ratio is increased.
Amylase clearance= Urine amylase concentration x Serum creatinine concentration / Serum amylase
concentration x Urine creatinine concentration.
Imaging Studies:
Plain KUB (Kidney/Ureter/Bladder):
Perform in the upright position to exclude viscus perforation (air under the diaphragm). In case of a recurrent
episode of chronic pancreatitis, peripancreatic calcifications may be noted. Radiography may reveal a “sentinel
loop,’’ a localized ileus in the midepigastric region. Pleural effusions may also be present.
Ultrasound can be used as a screening test:

******ebook converter DEMO Watermarks*******

CT scan is the most reliable imaging modality in the diagnosis of acute pancreatitis. The criteria for the
diagnosis are divided by Balthazar and colleagues into 5 grades.

Grade A: Normal pancreas.

Grade B: Focal or diffuse gland enlargement.
Grade C: Intrinsic gland abnormality seen by haziness on the scan.
Grade D: Single ill-defined collection or phlegmon.
Grade E: Two or more ill-defined collections or the presence of gas in or nearby, the pancreas.

Treatment:
Emergency Department Care: 80% patients respond to conservative treatment.
Fluid Resuscitation:
Accurate intake/output and electrolyte balance of the patient should be monitored
Crystalloids are used but other infusions, such as packed red blood cells (PRBCs), are occasionally administered,
particularly in the case of hemorrhagic pancreatitis
Patients should be kept as NPO and a nasogastric tube should be inserted to assure an empty stomach and to keep
the GI at rest.
Parenteral nutrition should be started if the prognosis is poor and the patient is going to be kept in the hospital for
more than 4 days.
Analgesics: Meperidine is preferred on morphine due to the greater spastic effect of the latter on the sphincter of
Oddi.

NSAID of choice: Metamizole

Opiate of choice: Buprenorphine.

******ebook converter DEMO Watermarks*******

Antibiotics are used in severe cases associated with septic shock or when a phlegmon of the pancreas has evolved as
seen by CT scan. Other conditions, such as biliary pancreatitis associated with cholangitis, also need antibiotic
coverage.
The preferred antibiotics are the ones secreted by the biliary system, such as ampicillin and third generation
cephalosporins.
Continuous oxygen saturation should be monitored by pulse oxymetry and acidosis should be corrected. When
tachypnea and pending respiratory failure develops, intubation should be performed.
General Surgery should be consulted in the following cases:

Phlegmon of the Pancreas:

Infected peripancreaic fluid collection: Percutaneous drainage.
Culture proven Infected Necrotising pancreatitis.
Patients who fail to improve despite optimal medical treatment, or patients who push the Ranson score
even further.
Biliary Pancreatitis: Sphincterotomy would relieve the obstruction. A cholecystectomy may be performed to
clear the system from biliary stones.

Complications:
Most common cause of death is Multiorgan dysfunctional syndrome
First sign of Mutisystem organ failure is ARDS.

Infected pancreatic necrosis ia due to seeding of bacteria into the inflammation.

An acute pseudocyst is an effusion of pancreatic juice that is walled off by granulation tissue after an episode of
acute pancreatitis.
Hemorrhage into the GI tract retroperitoneum or the peritoneal cavity is possible.
Intestinal obstruction or necrosis.
CBD obstruction by a pancreatic abscess, pseudocyst or biliary stone.
Internal pancreatic fistula from pancreatic duct disruption or a leaking pseudocyst.

******ebook converter DEMO Watermarks*******

Chronic Pancreatitis:
Chronic pancreatitis is defined as a continued inflammation characterized by irreversible morphologic changes.
These changes include fibrosis, ductal abnormality, calcification and cellular atrophy. Alcohol is the major etiologic
factor, accounting for about 75% of the cases.
Repeated attacks of gallstone-related pancreatitis can rarely cause chronic pancreatitis. Other causes include
diabetes, protein-calorie malnutrition, hereditary pancreatitis, cystic fibrosis and idiopathic causes.
Alcohol presumably causes pancreatic injury by the intraductal formation of protein plugs secondary to increased
protein concentration and precipitation, with or without calcification. These plugs lead to obstruction and secondary
pancreatic damage.
Classification:

Chronic pancreatitis is divided into two clinical types: Chronic pancreatitis and chronic relapsing pancreatitis In
both, regardless of the cause, the gland is permanently damaged, morpho-logically and functionally causing
recurrent painful attacks resembling acute pancreatitis.
In contrast, the type referred to simply as chronic pancreatitis is often illustrated by idiopathic disease, in
which the gradual destruction of the gland, with resulting pancreatic insufficiency, often proceeds without
discrete painful exacerbation.

Pathology:

A cardinal feature of chronic pancreatitis is the presence of protein plugs and calcifications.
Proteinaceous material precipitates in the ducts and ductules, initially consisting mainly of pancreatic enzyme
protein and a glycoprotein.
In late stages, calcium carbonate is added to the precipitates, giving rise to stones (pancreatic calculi).
Protein plugs and calculi are rare in chronic obstructive pancreatitis.
Chronic obstructive pancreatitis is the result of occlusion of main pancreatic duct.
Any obstructing lesion--a tumor, a scar resulting from trauma, papillary inflammation, a congenital structure--is
a potential cause.
Fibrosis is accompanied by uniform acinar atrophy.
Exocrine insufficiency ensues, but it’s partially reversible if the obstruction is removed.

Clinical Manifestations:
The clinical picture of chronic pancreatitis is dominated by three features; abdominal pain, maldigestion, and
diabetes (loss of exocrine and endocrine pancreatic function). The pain is localized to the upper abdomen, with
radiation to subcostal regions and to the back. The pain is aggravated by meals and improves with fasting.
When more than 90% of exocrine pancreatic function is lost, maldigestion and malabsorption ensue. This is
manifested by steatorrhea (fat malabsorption) associated with diarrhea and bloating, azotorrhea (protein
malabsorption) and progressive weight loss. These patients frequently present with loss of adipose tissue, judged by
hanging skin folds, and more objectively by demonstrating that the skin fold at the mid-triceps is less than 8 mm in
males and less than 12 mm in females. Latent fat-soluble vitamin deficiency (vitamins A, D, E and K) in addition to
deficiencies of magnesium, calcium and essential fatty acids may occur and are closely related to dysfunction of fat
digestion. Endocrine insufficiency presenting as diabetes mellitus may present at the same time as exocrine
insufficiency or a few years later.
Complications:

Pseudocyst.
Common Bile Duct Obstruction.
Pancreatic Ascites.
Pancreatic Pleural Effusion.
Splanchnic Venous Obstruction.

******ebook converter DEMO Watermarks*******

Diagnostic and radiographic evaluation:
Radiological evidence such as calcification (in up to 30%) seen exclusively in the ductal system on plain
radiographic abdominal films, by Endoscopic ultrasonography or on computerized tomography suggests chronic
pancreatitis. Abnormalities of the ducts associated with chronic pancreatitis can also be demonstrated by ERCP
(Chain of lake appearance). Examination may reveal a tortuous main duct containing stones or protein plugs, or
obstruction of the CBD.
The only tests that appear to accurately measure pancreatic function in chronic pancreatitis are the direct tube tests
that measure the response of the pancreas to various stimuli. The commonest manifestation is a decreased
bicarbonate concentration (<50 mEq/L) and decreased volume of secretion.

Tests of pancreatic function:

Measurement of pancreatic exocrine function: The severity of maldigestion, its contribution to weight loss, &
efficacy of pancreatic enzymes can be assessed.
Pancreatic function may be inferred by direct measurement of the components of pancreatic secretion.
Enzyme activity may be estimated by the ability of the pancreas to cleave a given substance.
Pancreatic integrity may be reflected by the level of pancreatic enzymes or hormones secreted in the
bloodstream or by recovering enzymes from the stool.
Pancreatic dysfunction can be appreciated by the amount of undigested nutrients recovered in the stool.

1. Invasive Tests:

The Secretin Test.

The Lundh Test.

2. Noninvasive Tests:

The NBT-PABA Test (Bentiromide Test).

Fecal Fat.

Treatment:
Goals are: To alleviate pain, maintain adequate nutritional status, and reduce symptoms associated with steatorrhea
such as abdominal pain, bloating and diarrhea.
Pain management: Abstinence from alcohol may decrease the frequency and severity of painful attacks. Large
meals with foods rich in fat should be avoided. Analgesics should be given prior to meals. Large doses of pancreatic
extracts may reduce the frequency and severity of the pain. Patients with more severe disease, whose peak
bicarbonate output is > 50 mEq/L, tend not to respond to this regimen. Patients with intractable pain who fail to
respond to medical therapy may benefit from surgical intervention.

When there is a dilated pancreatic duct with obstructive areas, longitudinal pancreatojejunostomy (modified
Pustow operation) may relieve pain.
An alternative to surgical drainage may be achieved by endoscopic insertion of an an endoprosthesis (stent)
into the pancreatic duct.
Octreotide, a long-acting somatostatin analogue, appears to decrease the pain of chronic pancreatitis.
******ebook converter DEMO Watermarks*******
Surgeries for Chronic Pancreatitis:

Denervation:Denervation procedures aim at interrupting the transmission of pain from the pancreas through the
sympathetic nerve fibers.

Since the majority of sensory nerves to the pancreas transverse the celiac ganglia and splanchnic nerves, both
transthoracic splanchnicectomy and ganglionectomy have been used. They offer variable degrees of pain relief.

Endoscopic Therapy:
Considerable progress has been achieved in applying endoscopic techniques to the management of chronic
pancreatitis.

It is possible to remove stones in the pancreatic duct, directly by use of a basket.

Stents may be placed in strictured areas of the pancreatic duct.
In cases of chronic pancreatitis associated with pancreas divisum, endoscopic sphincterotomy of the minor
papilla and stent placement across the papilla are successful in decreasing the frequency and severity of the
attacks

Malabsorption: Administration of high-potency, enteric-coated pancreatic enzymes remains the main therapy for
the treatment of steatorrhea in the majority of patients with idiopathic and alcoholic pancreatitis. This will improve
fat digestion, increase absorption and allow weight gain, although it will not correct the steatorrhea completely.
Azotorrhea is more easily reversed than steatorrhea, as trypsin is more resistant to acid inactivation than lipases.
Treatment with these enzymes is life long. Pancreatic enzymes are inactivated by pH 4 or below; hence, enteric-
coated preparations may be appropriate. In patients who do not respond well, the use of histamine H2-receptor
antagonists (cimetidine, ranitidine or famotidine) or antacids with meals may overcome the detrimental effect of
acid.
Hypersensitivity to pancreatic enzymes has been reported in patients who have hypersensitivity to pork proteins.
Hyperuricosuria may occur in patients receiving high doses of pancreatic extracts. It appears that oral pancreatic
enzymes may bind to folic acid, thereby impairing its absorption. Fat-soluble vitamins (e.g., vitamins A and E) are
poorly absorbed when steatorrhea exceeds 20 g of fat loss per day. Vitamin D and calcium malabsorption leads to
osteopenia and tetany. Vitamin K is also malabsorbed, but bleeding is rare. This malabsorption is thought to be due
to the failure of R factor to cleave from the vitamin B12-intrinsic factor complex, resulting in failure to absorb
vitamin B12.
Diabetes:

Exogenous insulin, unopposed by glucagon (low or absent in pancreatic disease), may cause hypoglycemia, so
tight glycemic control is potentially dangerous.
Since the development of diabetic vasculopathy is infrequent, it is unnecessary to maintain blood sugar within
the normal range.
Plasma glucose value of 200 to 250 mg/dl throughout the day is a desirable target.
Therapy should control symptoms, principally polydypsia and polyuria and prevent the excessive loss of
calories in the urine.

Hereditary Pancreatitis and Shwachman’s Syndrome:

Hereditary Pancreatitis is probably inherited as an autosomal dominant condition with a bimodal incidence with
******ebook converter DEMO Watermarks*******
peaks at about 10 and 17 years of age. Pancreatic calcification develops early, in the first decade, but in most
respects, clinical course is similar to that of chronic pancreatitis of other causes. Children are from an affected
family or have a structural abnormality in the pancreas or biliary tree (particularly pancreas divisum or choledochal
cyst).
The association of pancreatic malabsorption, steatorrhoea, and failure to thrive, with metaphyseal dysplasia,
neutropenia and other haematological abnormalities, is known as Shwachman’s syndrome and accounts for a high
proportion of children presenting with painless pancreatic insufficiency. It is probably inherited as an autosomal
recessive condition. With appropriate dietary modification and enzyme supplements, the prognosis is good.
Complications of Pancreatitis:
Edema of pancreatic head may be responsible for bile duct obstruction and cholestasis.
Pancreatic Pseudocyst:
Pancreatic pseudocysts are a relatively common complication of pancreatitis, especially alcoholic pancreatitis.
Pseudocysts are localized collections of fluid and contain concentrations of pancreatic enzymes; they are usually
confined to the retroperitoneal areas by a fibrous membrane that is devoid of endothelial lining. They develop after
15% to 50% of all attacks of acute pancreatitis, many resolving spontaneously, and are seen in 20% to 40% of
patients with chronic pancreatitis. They typically result from disruption of the pancreatic duct with extravasation of
pancreatic enzymes. Pseudocysts are prone to cause complications, including hemorrhage, infection, pancreatic
ascites, obstruction, and, infrequently, fistula formation to other viscera in the gastrointestinal (GI) tract.
Although outside the peritoneal cavity, pseudocysts may migrate to the mediastinum or pelvis, and associated
pleural effusions also occur. Ultrasound has demonstrated that such cysts represent a very common complication
although rarely it warrants intervention.
Most lesions of 5cm or less resolve spontaneously. When larger, pseudocysts may cause pain, gastrointestinal
obstruction or a palpable mass.

Carcinoma of the Pancreas:

Among cancers of the gastrointestinal tract, it is the third most common malignancy and the fifth leading cause of
cancer-related mortality. Of pancreatic tumors, 95% develop from the exocrine portion of the pancreas, including the
ductal epithelium, acinar cells, connective tissue, and lymphatic tissue. Approximately 75% of all pancreatic
carcinomas occur within the head or neck of the pancreas. Approximately 15-20% occur in the body of the pancreas,
and 5-10% occur in the tail. Pancreatic cancer typically first metastasizes to regional lymph nodes, then to the
liver and, less commonly, to the lungs.
The male-to-female ratio for pancreatic cancer is 1.2-1.5:1.
History: Patients typically report the gradual onset of anorexia, malaise, nausea, and midepigastric abdominal pain.
Significant weight loss is a characteristic feature of pancreatic cancer. Pain is the most common presenting featur
in patients with DISTAL(TAIL)pancreatic cancer. Back radiation of the pain indicating retroperitoneal invasion
of the splanchnic nerve plexus by the tumor.

******ebook converter DEMO Watermarks*******

The most characteristic sign of head pancreatic carcinoma, is obstructive jaundice.
Patients with jaundice may have a palpable gallbladder (ie, Courvoisier sign) and may have evidence of excoriations
from pruritus.
Causes: Overall, 40% of pancreatic cancer cases are sporadic in nature. Another 30% are related to smoking, and
20% are associated with dietary factors. Only 5-10% are hereditary. Fewer than 5% of all pancreatic cancers are
related to chronic pancreatitis.
Factors Associated with Increased Risk of Pancreatic Cancer:
Advancing age.
African American males.
Low socioeconomic status.
Native female Hawaiians.
Ashkenazic Jewish heritage.
Cigarette smoking.
Six genetic syndromes.
Diabetes mellitus.
Chronic pancreatitis.
Cirrhosis.
Obesity.
Increased height.
Low level of physical activity.
High-fat and cholesterol diet.
Occupational exposure to carcinogens (PCBs, DDT, NNK, benzidine).
PCBs, polychlorinated biphenyls; DDT, dichlorodiphenyltrichloroethane; NNK,
4-(methylnitrosamino)-1- (3-pyridyl)-1-butanone.
Genetic Syndromes and Gene Alterations Associated with Familial Pancreatic Cancer:

Familial atypical multiple mole melanoma syndrome with germline mutations in the p16 gene. Carriers of p16
germline mutations have a 12- to 20-fold increased risk of developing pancreatic cancer, as well as an increased
risk of melanoma.
The Peutz-Jeghers syndrome, characterized by mucocutaneous melanocytic macules and hamartomatous polyps
of the gastrointestinal tract. Patients with the Peutz-Jeghers syndrome have a greater than 100-fold increased
risk of developing pancreatic cancer.
The hereditary nonpolyposis colorectal cancer syndrome, characterized by germline mutations in one of the
DNA mismatch repair genes (hMSH1, hMSH2, etc.).
Hereditary pancreatitis with germline mutations in the PRSS1 (cationic trypsinogen) gene. Patients develop
******ebook converter DEMO Watermarks*******
 severe pancreatitis at a young age (often children and adolescents) and have a 50-fold excess risk of developing
pancreatic cancer.
Ataxia-telangiectasia, a rare autosomal recessive inherited disorder, characterized by cerebellar ataxia,
oculocutaneoustelangiectasias, and cellular and humoral immune deficiencies. The gene, ATM, is also
associated with an increased risk of leukemia, lymphoma, and cancers of the breast, ovaries, biliary tract,
stomach, and, occasionally, the pancreas.
Pancreatic cancer, pancreatic insufficiency, and DM have been described in a family (called Family X), and the
phenotype has been linked to chromosome 4q32-34.

Lab Studies:
General laboratory studies:
Tumor markers:

Carcinoembryonic antigen (CEA) is a high molecular weight glycoprotein found normally in fetal tissues. The
reference range is < 2.5 mg/mL. Only 40-45% of patients with pancreatic carcinoma have elevations in CEA
levels.
CA 19-9 is a murine monoclonal antibody originally made against colorectal cancer cells. The reference range
of CA 19-9 is < 37 U/mL. In pancreatic carcinoma, 75-85% have elevated CA 19-9 levels. In the absence of
biliary obstruction or benign pancreatic disease, a CA 19-9 value greater than 100 U/mL is highly specific for
malignancy, usually pancreatic.

Imaging Studies:

Computed tomography scan : Standard abdominal CT scan can detect 70-80% of pancreatic carcinomas. CT
scans can be used to direct fine-needle aspiration of masses.
Percutaneous ultrasound : Percutaneous abdominal ultrasonography is useful for patients with pancreatic
cancer who present with jaundice, by detecting intrahepatic bile duct dilation/extrahepatic biliary obstruction.
Cholangiopancreatography (MRCP) should usually be performed to definitively diagnose the source of
extrahepatic obstruction.
Endoscopic ultrasound: EUS has detection rates of 99-100% for pancreatic carcinomas.
Endoscopic retrograde cholangiopancreatography : Brush cytology and forceps biopsy at the time of ERCP
have been used to histologically diagnose pancreatic carcinoma.
Magnetic resonance imaging.

Histologic Findings:
80% are adenocarcinomas of the ductal epithelium. Only 2% of tumors of the exocrine pancreas are benign.
Treatment:
The only therapy that has definitively been shown to increase the survival of patients with pancreatic cancer
is surgical resection. The mean survival for patients with unresectable disease remains 4-6 months.
Chemotherapy:

Pancreatic carcinoma has been markedly resistant to chemotherapeutic regimens, either alone or in combination
therapy. The most active agents have been 5-fluorouracil (5-FU) and the more recently gemcitabine and
Erlotinib.

Surgical Care: Only 20% of patients present with resectable disease.

Pancreaticoduodenectomy (Whipple operation).

The standard operation for carcinoma of the head of the pancreas is a pancreaticoduodenectomy (ie, Whipple
procedure). This operation involves en bloc resection of the pancreatic head; the first, second, and third
portions of the duodenum; the distal antrum; and the distal common bile duct.

******ebook converter DEMO Watermarks*******

 The patient’s gastrointestinal tract is reconstructed with a gastrojejunostomy.
The common bile duct and residual pancreas are anastomosed into a segment of small bowel.
A more recent variation of the operation spares the pylorus, allowing for a more natural physiologic emptying
of the stomach. Some surgeons prefer total pancreatectomy to avoid the risks of anastomotic leaks and
pancreatic fistulas. This has the disadvantage of leaving the patient with brittle diabetes postoperatively.

Whipple’s operation:

Pylorus preserving pancreatectomy (Longmire Traverso operation):

Celiac Plexus Block and Splanchnic nerve block:

******ebook converter DEMO Watermarks*******

 Typically, the celiac plexus is located anterolateral to the aorta, immediately caudal to the celiac artery’s
origin, at the cephalad border of the L1 vertebral body.
Neurolytic Celiac Plexus Block: NCPB is an injection of medication that helps relieve upper abdominal
pain, commonly due to cancer or chronic pancreatitis. It can be performed with a variety of techniques by
either posterior or anterior approaches with use of absolute alcohol or phenol.

Splanchnic nerve block is a useful alternative to coeliac plexus block in the management of patients with chronic
upper abdominal pain. The predictable relationship of the splanchnic nerves to other structures allows for accurate
needle placement hence Radiofrequency ablation (RFA) can be used with low risk of iatrogenic damage. It
produces predictable and accurate lesions and hence is useful alternative to more conventional phenol and alcohol
neurolytic methods.
Complications:
1. Hypotension and diarrhoea may occur shortly after the intervention, but are easy to treat.
2. There is always the possibility of a collapsed lung.
3. Phrenic nerve injury leading to elevated diaphragm and dyspnoea.
4. Thoracic injury is present when a yellowish, turbid fluid is aspired.
5. Paraplegia is rare, but there have been case reports of alcohol injections damaging Adamkiewicz’s arteries.

******ebook converter DEMO Watermarks*******

Endocrine Pancreatic Tumours:
Subsequent to initial description of insulinoma syndrome, 4 other classic pancreatic endocrine tumor syndromes
have been described. The first is Zollinger-Ellison syndrome (also termed gastrinoma syndrome); second types
comprise a group of 3 tumor syndromes, termed Verner-Morrison syndrome, WDHA (watery diarrhea,
hypokalemia, and achlorhydria) syndrome, and pancreatic cholera (also termed [VIP]–releasing tumor or VIPoma);
The third is glucagonoma syndrome. The fourth is somatostatinoma syndrome.
The cells in pancreatic endocrine neoplasms are termed amine precursor uptake and decarboxylation (APUD) cells
because they have a high amine content, are capable of amine precursor uptake, and contain an amino acid
decarboxylase. The tumors arise from APUD stem cells, which are pluripotential neuroendocrine cells located
within the ductular epithelium of the exocrine pancreas and elsewhere in the distal foregut.
Insulinoma: Insulinomas are insulin-secreting tumors associated with the Whipple triad. The triad includes (1)
******ebook converter DEMO Watermarks*******
symptoms of fasting hypoglycemia, (2) documented fasting hypoglycemia with a serum glucose < 50 mg/dL, and
(3) relief of hypoglycemic symptoms after glucose administration (Hypoglycemic symptoms typically occur when
glucose levels are < 50 mg/dL; concurrent serum insulin levels often exceed 25 mU/mL).
Gastrinoma: The classic triad of Zollinger-Ellison syndrome includes (1) severe gastrointestinal ulcerative disease,
(2) gastric acid hypersecretion, and (3) nonbeta islet cell tumors of the pancreas (Zollinger, 1955). Abdominal pain
and peptic ulceration of the upper gastrointestinal tract are the most common symptoms and are observed in 90-95%
of patients with Zollinger-Ellison syndrome. (Fasting serum gastrin test: Levels greater than 200 pg/mL are
suggestive of gastrinoma, and levels greater than 1000 pg/mL are virtually diagnostic of gastrinoma).
VIPoma: Symptoms of Verner-Morrison or WDHA syndrome (ie, watery diarrhea, hypokalemia, achlorhydria,
acidosis) are the result of the physiologic effects of overproduction of VIP by pancreatic endocrine neoplasms. The
primary symptom of patients with a VIPoma is watery diarrhea. Abdominal cramps are common, and flushing
episodes may occur. (The level of serum VIP ranges from 225-1850 pg/mL. The normal serum VIP level is <170
pg/mL).
Glucagonoma: Glucagonomas secrete excessive amounts of glucagon and cause a syndrome characterized by
dermatitis, stomatitis, weight loss, and anemiaThe dermatitis associated with glucagonoma syndrome is termed
necrolytic migratory erythema. This dermatitis is characterized by the cyclic migration of erythematous patches
that spread serpiginously and then reveal central points of healing. Patients with glucagonoma syndrome have
secondary thromboembolic phenomena; therefore, they may have a history consistent with deep venous thrombosis
and/or pulmonary embolism. (Serum glucagon levels >1000 pg/mL are diagnostic, levels less than 150 pg/mL are
normal).
Somatostatinoma: Patients often have diabetes mellitus, which is probably secondary to the inhibitory action of
somatostatin on insulin and glucagon release. Inhibition of the action of cholecystokinin by somatostatin causes
relative biliary stasis and the formation of gallbladder calculi. Patients may also have diarrhea and/or steatorrhea.

MEN 1 syndrome, or Wermer syndrome, is an autosomal dominant disorder. The syndrome is characterized by
hyperparathyroidism, adenomas of pituitary, and neoplasms of endocrine pancreas. In pituitary; prolactin-
secreting tumors are most common type.The pancreatic tumors in these patients tend to be multiple and usually
secrete multiple hormonally active products.

Imaging Studies:
High-resolution contrast-enhanced spiral CT scanning with thin sections is the initial imaging technique used to
localize most neoplasms of the endocrine pancreas.
Magnetic resonance imaging.
Somatostatin receptor scintigraphy: Radiolabeled octreotide is a somatostatin analogue that preferentially binds to
somatostatin receptors; the intravenous administration of octreotide can be used to identify such tumors.

******ebook converter DEMO Watermarks*******

Medical Care:

Upon initial presentation, patients with insulinoma may require immediate potassium replacement and dextrose
administration.
The primary initial concern in the treatment of a patient who presents first with VIPoma-associated diarrhea is
the replacement of volume losses and the correction of acid-base and electrolyte abnormalities.
Patients with glucagonomas requires preoperative control of hyperglycemia.

Surgical Care:
Surgical management of the primary tumor is similar for the different types of pancreatic endocrine neoplasms:

Small benign lesions remote from the main pancreatic duct can be enucleated.
Tumors deep in the substance of the pancreatic gland, and therefore close to the main duct, have ill-defined
capsules, and tumors larger than 2 cm in diameter should be treated with regional pancreatectomy.
Tumors in the tail of the pancreas can be managed with distal pancreatectomy
Lesions in the head or uncinate process of the pancreas can be resected with pancreaticoduodenectomy.

Periampullary Carcinoma:
Obstructive jaundice is the usual presenting feature, and ultrasound (especially endoscopic) or a CT scan may
demonstrate the lesion. At ERCP, the endoscopic appearance is immediately suggestive of a malignant tumour at the
ampulla, and the absence of extension along the pancreatic duct or bile duct helps in the distinction from an
infiltrating pancreatic carcinoma or extension of a cholangiocarcinoma.
Biopsies, taken at endoscopy or ERCP, should confirm the diagnosis.
Ampullary neoplasms are usually papillary or solid tumours which invade locally. The usual histological pattern is
of moderately well-differentiated adenocarcinoma.
The periampullary region is anatomically complex, representing the junction of 3 different epithelia, pancreatic
ducts, bile ducts, and duodenal mucosa. Carcinomas originating in the ampulla of Vater by gross inspection can
arise from 1 of 4 epithelial types, (1) terminal common bile duct, (2) duodenal mucosa, (3) pancreatic duct, or (4)
ampulla of Vater. In general, ampullary cancers produce sialomucins, whereas periampullary tumors secrete sulfated
mucins.
History: Patients with carcinoma of the ampulla of Vater often complain of anorexia, nausea, vomiting, jaundice,
pruritus, or weight loss, abdominal pain. Diarrhea may be associated with an absence of lipase in gut because of
pancreatic duct obstruction.
Physical: Some patients might demonstrate a distended, palpable Courvoisier gallbladder (ie, palpable gall bladder
in a patient with jaundice). A rising bilirubin level due to obstructive jaundice often is the sole presenting
symptom.
Ultrasound of the abdomen is the initial study (dilation of these ducts essentially is diagnostic for extrahepatic
obstruction).
CT scan often demonstrates a mass but is not helpful in differentiating ampullary carcinoma from tumors of the head
of the pancreas or periampullary region. Both CT scan and ultrasound findings can help reveal metastatic disease in
the liver or regional lymph nodes
ERCP can show irregular pancreatic duct narrowing, displacement of the main pancreatic duct, destruction or
displacement of the side branches of the duct, and pooling of contrast material in necrotic areas of tumor.
Lab Studies: Routine laboratory studies include a complete blood cell count, electrolyte panel, liver function studies
(prothrombin time, bilirubin [direct and indirect], transaminases, alkaline phosphatase), CEA, and CA 19-9 (CA 19-
9 and CEA is often is elevated in pancreatic malignancies and might have a role in assessing response to therapy or
predicting tumor recurrence).

Staging: Martin proposed a 4-stage system, as follows:

Stage I - Vegetating tumor limited to the epithelium with no involvement of the sphincter of Oddi.
******ebook converter DEMO Watermarks*******
 Stage II - Tumor localized in the duodenal submucosa without involvement of the duodenal
muscularispropria but possible involvement of the sphincter of Oddi.
Stage III - Tumor of the duodenal muscularispropria.
Stage IV - Tumor of the periduodenal area or pancreas, with proximal or distal lymph node involvement.

Surgical Care: The standard surgical approach is pancreaticoduodenal resection (Whipple procedure). The
procedure involves en bloc resection of the gastric antrum and duodenum; a segment of the first portion of
the jejunum, gallbladder, and distal common bile duct; the head and often the neck of the pancreas; and
adjacent regional lymph nodes.
For patients with unresectable disease, endoscopic stenting to achieve biliary decompression is an appropriate
palliative procedure.

******ebook converter DEMO Watermarks*******

Adrenal Pheochromocytoma:

Pheochromocytoma is a catecholamine-secreting tumor derived from chromaffin cells.

Tumors arising outside the adrenal are termed extra-adrenal pheochromocytomas/ paragangliomas.
The clinical manifestations of pheochromocytoma result from excessive catecholamine secretion by the tumor.
Catecholamines typically secreted, either intermittently or continuously, include norepinephrine and
epinephrine but rarely dopamine.
Unlike the healthy adrenal medulla, pheochromocytomas are not innervated, and catecholamine release is not
precipitated by neural stimulation.
Most pheochromocytomas contain norepinephrine predominantly, in comparison with the normal adrenal
medulla, which is comprised of roughly 85% epinephrine.
Familial pheochromocytomas are exception as they secrete mainly epinephrine.
Most common site of extra adrenal pheochromocytoma is “Organ of Zuckerkandl”.
Generally called 10% tumour- 10% are B/L, familial, malignant, children, extra-adrenal and Bilateral.
Mainly unilateral, solitary and benign but SDBH mutation is associated with higher likelihood of extra-adrenal
and malignant tumours.
Histology: Polygonal or spindle chromaffin cells clusters in small nets or alveoli, rich vascular network
(Zelballen).

Pheochromocytomas may be familial, seen in-

Multiple endocrine neoplasia (MEN) 2A and 2B.

Neurofibromatosis.
Von Hippel-Lindau (VHL) disease.
Sturge weber syndrome.
Tuberus sclerosis.

Clinical:
Male : Female ratio is equal, usually age of presentation is 3rd to 5th decade.
4 cardinal symptoms of pheochromocytoma are, headaches (Commonest symptom), palpitations, and
diaphoresis in association with severe hypertension (Commonest sign, Episodic in 50%).
Symptoms:

Headache.
Diaphoresis.
Palpitations.
Tremor.
Nausea.
Weakness.
Anxiety, sense of doom, Epigastric pain, Flank pain, Constipation, Weight loss

(Diarrhea is not a symptom of pheochromocytoma).

Clinical signs:

Hypertension (paroxysmal in 50% of cases).

Postural hypotension – Resulting from volume contraction.
******ebook converter DEMO Watermarks*******
 Hypertensive retinopathy.
Weight loss, Pallor, Fever, Tremor.
Neurofibromas.
Cafe au lait spots: These are patches of cutaneous pigmentation, which vary in size from 1-10 mm and occur
any place on the body. Characteristic locations include the axillae and intertriginous areas (groin). Their color
varies from light to dark brown, hence the name cafe au lait.
Tachyarrhythmias, Pulmonary edema, Cardiomyopathy, Ileus.

Pheochromocytoma occur in certain familial syndromes. These include MEN 2A and 2B, neurofibromatosis (Von
Recklinghausen disease), and VHL disease. Neurofibromatosis has a 1% incidence of pheochromocytoma. The
VHL syndrome is associated with pheochromocytomas, cerebellar hemangioblastomas, and RCC.

MEN 2A (Sipple syndrome) is comprised of medullary thyroid carcinoma, hyperparathyroidism,

pheochromocytoma, and Hirschsprung disease. > 95% of cases of MEN 2A are associated with mutations in
the Ret proto-oncogene affecting 1 of 5 codons in exons 10.
Medullary thyroid carcinoma, pheochromocytoma, mucosal neurofibromatosis, intestinal ganglioneuromatosis,
Hirschsprung disease, and a marfanoid body habitus characterize MEN 2B.
Other neuroectodermal disorders associated with pheochromocytoma include tuberous sclerosis (Bourneville
disease, Epiloia) and Sturge-Weber syndrome.

Laboratory features:

Hyperglycemia.
Hypercalcemia.
Erythrocytosis.

Lab Studies:

A 24-hour urine collection for creatinine, total catecholamines, vanillylmandelic acid (VMA), and
metanephrines. Metanephrines are considered the most sensitive and specific test for pheochromocytoma, while
VMA is the least specific test.
Best test for diagnosis: Fractionated plasma Metanephrines.
Most sensitive: Urinary metaneprines.

Imaging Studies:

Over 90% of pheochromocytomas are located within the adrenal glands and 98% within the abdomen. Extra-
adrenal pheochromocytomas develop in paraganglion chromaffin tissue of the sympathetic nervous system.
Common locations for extra-adrenal pheochromocytomas include organ of Zuckerkandl (close to origin of the
inferior mesenteric artery), bladder wall, heart, mediastinum, and carotid and glomus jugulare bodies.
MRI has a sensitivity of 100% in detecting adrenal pheochromocytomas. MRI also is superior to computed
tomography (CT) scanning in detecting extra-adrenal pheochromocytomas.
CT scans of the abdomen have an accuracy of 85-95% in detecting adrenal masses with a spatial resolution of 1
cm or greater.
Dopa - PET scan in investigation of choice for extra adrenal Pheochromocytoma.
A scan with iodine-131 (131I)–labeled metaiodobenzylguanidine (MIBG) is reserved for cases when a
pheochromocytoma is confirmed biochemically but CT scan or MRI fail to visualize a tumor. The molecular
structure of iodine-123 (123I) MIBG resembles norepinephrine and concentrates within adrenal or extra-adrenal
pheochromocytomas. This isotope has a short half-life and is very expensive.

Procedures:
Rarely indicated due to the high sensitivity of MRI and CT scanning.

Selective venous sampling seldom is performed to localize pheochromocytomas but occasionally has been
utilized to detect extra-adrenal pheochromocytomas.
******ebook converter DEMO Watermarks*******
 Arteriography rarely is indicated and provides little additional information compared to an MRI or CT scan.
Biospy/ FNAC is contraindicated as it can precipitate Hypertensive crisis.

Pharmacological tests:

Not diagnostic but helpful (Biochemical tests must).

Phentolamine reduces BP (35/25 mm Hg). Glucagon release catecholamine and cause paroxysm of HT.

Histologic Findings:
Pheochromocytomas vary in size from 2 g to 3 kg but on average weigh 100 g. These tumors are well encapsulated,
highly vascular, and appear reddish brown on cut section.
Histologically, the tumor cells are arranged in balls and clusters separated by endothelial-lined spaces; this classic
pattern characteristic of pheochromocytoma is termed zellballen.
Staging:
Approximately 10% of pheochromocytomas are malignant.
Direct invasion of surrounding tissue or the presence of metastases determines malignancy.
Treatment:
Surgical resection of the tumor is the treatment of choice and usually results in cure of the hypertension.
Careful treatment with alpha- and beta-blockers is required preoperatively to control blood pressure and prevent
intraoperative hypertensive crises.

Start alpha blockade with phenoxybenzamine 7-10 days preoperatively to allow for expansion of blood volume.
Initiate a beta-blocker only after adequate alpha blockade. If beta blockade is started prematurely, unopposed
alpha stimulation could precipitate a hypertensive crisis.
Administer the last doses of oral alpha- and beta-blockers on the morning of surgery.

Surgical Care:

An anterior midline abdominal approach was utilized in the past; however, now laparoscopic adrenalectomy is
the preferred procedure for small to moderate lesions (< 5cm).

The 5-year survival rate for people with nonmalignant pheochromocytoma is greater than 95%. In malignant
pheochromocytomas, the 5-year survival rate is less than 50%.

Malignant metastatic pheochromocytoma:

Risk of malignancy is proportional to size of tumour.

Malignanttumours are likely to express- P53, Bcl-2, and have activated telomerase.
Capsular and vascular invasion seen in benign tumour too.
******ebook converter DEMO Watermarks*******
 Dopamine and HVA secretion is more in malignant tumour.
Mote common site of metastasis is Bone > Liver > nodes.

Should be treated with α and β blockers and with metyrosine (Tyrosine hydroxylase inhibitor). Metyrosine inhibits
tyrosine hydroxylase, which catalyzes the first transformation in catecholamine biosynthesis. Thus, levels of VMA
and BP fall. BP can be controlled even though the tumor growth continues and will eventually cause death.
Combination chemotherapy using cyclophosphamide, vincristine, and dacarbazine is the best treatment for
metastases. Experimentally, 131I-MIBG has been used to treat large metastases. Radiotherapy may reduce bone pain
but is generally ineffective.

Hyperaldosteronism:

Primary aldosteronism, also termed Conn syndrome.

It is clinically characterized by hypertension and hypokalemia.
The cause of primary aldosteronism is an adrenal adenoma in 80% and adrenal gland hyperplasia in 20%.
Adrenal carcinoma is an extremely rare cause.
In 75-90% of patients with a solitary aldosterone-producing tumor.

Pathophysiology:
Aldosterone promotes the excessive preservation of sodium at the expense of potassium loss. Sodium retention
promotes water retention, hypertension, and a suppression of renin production. Excessive potassium loss causes
hypokalemic alkalosis, which may be associated with complications including muscular weakness, tetany, and
abnormal electrocardiographic findings.
The diagnosis of primary aldosteronism is based on the typical biochemical findings of hypokalemia,
hypernatremia, depletion of magnesium, elevated bicarbonate levels, alkaloses, and elevated aldosterone
levels in both the serum and urine.
The demonstration of suppressed renin levels is vital to the diagnosis. A sodium chloride suppression test can be
used that involves administration of large amounts of sodium chloride over 3-5 days, which causes hypokalemia in

******ebook converter DEMO Watermarks*******

80-90% of patients with primary aldosteronism. This response is associated with muscle weakness, cardiac
arrhythmia, carbohydrate intolerance, and nephrogenic diabetes insipidus. Hypertension associated with primary
aldosteronism is usually benign, malignant hypertension is rare.
Sex: The male-to-female ratio is 1:2.
Age: Primary aldosteronism occurs in patients aged 30-50 years.
Clinical Details:
Primary aldosteronism is characterized by:

Moderate-to-severe hypertension without edema.

Biochemically, the condition is associated with hypokalemia, metabolic alkalosis, and hyperaldosteronism not
appropriately suppressed during volume expansion and depression of plasma renin activity.
With hypokalemic alkalosis, muscular weakness, polydipsia, polyuria, nocturia, paresthesia, tetany, headaches,
and abnormal electrocardiographic features may develop.
Other associated reported abnormalities include subarachnoid hemorrhage, postural hypotension, and
bradycardia.

Preferred Examination:
The workup starts with appropriate biochemical analysis, after which thin-collimation CT is performed. If CT
findings are equivocal, radionuclide studies and MRI should be performed.
Limitations of Techniques:
Adrenal hypersecreting glands may appear to be normal in size. The adrenal glands also vary in size and weight as a
result of illness or stress. This size discrepancy is a particular problem with APAs because they are often small and
difficult to detect. With the use of current scanners, the sensitivity is 82-88%.
MRI:
APAs are isointense or hypointense relative to the liver on T1-weighted images and slightly hyperintense on T2-
weighted images.
A sensitivity of 70-100% and a specificity of 64-100% have been reported in the detection of APAs with MRI.
Ultrasound:
Sonograms may reveal a significantly sized APA, but because APAs tend to be small, the overall sensitivity of
sonography is poor.
Nuclear Medicine:
Findings: Iodine 131-6-β-iodomethylnorcholesterol (NP-59) is a cholesterol analog that binds to low-density
lipoprotein receptors of the adrenal cortex and is the primary radionuclide used to image the adrenal cortex. Imaging
is usually performed after dexamethasone suppression to reduce high background tracer uptake by the zona
fasciculata. The normal glands (which show uptake of the radionuclide) are identified on day 5 or thereafter.
Bilateral early depiction of the glands (before day 5) implies adrenal hyperplasia, whereas unilateral early depiction
implies an APA.
Angiography:
Because APAs are small and not usually vascular, selective adrenal angiography is seldom helpful. However,
adrenal phlebography has a useful role in the investigation of APA because the splaying of veins around APAs can
help in identifying even small tumors. If contrast medium is refluxed into the veins of the APA, a wheel-spoke
pattern is seen in the intratumoral veins.
The most useful technique in the investigation of primary aldosteronism is adrenal venous sampling.
Intervention:
Although primary aldosteronism accounts for 0.05-2% of cases of hypertension in the general population,
recognition of the disease is important because patients readily respond to the removal of the adrenal gland tumor.
******ebook converter DEMO Watermarks*******
In 75-90% of patients with a solitary APA, surgical adrenalectomy corrects hypertension and hypokalemia.
In idiopathic hyperaldosteronism associated with bilateral adrenal hyperplasia; surgery rarely cures hypertension.
Patients with idiopathic hyperaldosteronism are usually treated medically; therefore, differentiating primary
aldosteronism caused by APAs from idiopathic hyperaldosteronism is essential.
Adrenal Carcinoma:
Adrenocortical masses are common; autopsy studies show that approximately 5-15% of the general adult population
may have adrenal incidentalomas. Adrenal incidentalomas are biochemically and clinically asymptomatic adrenal
masses found incidentally as a result of unrelated imaging such as abdominal CT or MRI scans. Only a small
number of adrenal tumors are functional and an even smaller number (about 1%) are malignant.

All nonfunctional adrenal tumors larger than or equal to 6 cm should be removed because of the significant
potential cancer risk.
Nonfunctional adrenal tumors (<3 cm) have a very low probability of being adrenal cancer; therefore, they can
be removed safely.
The management strategy for adrenal masses larger than 3-6 cm is disputed.

These criteria do not apply to children, who generally have smaller ACs.
Incidence rate of malignancy is small (<0.03%) in all adrenal incidentalomas that are 1.5-6 cm. However, this rate
increases considerably with tumors larger than 6 cm (up to 15%).
Classifying adrenal tumors:
Adrenal tumors are classified in several ways.

Functional and nonfunctional,

Older reports suggest that approximately 50-80% of ACs are functional, and patients mainly present with
Cushing syndrome.
More recent reports suggest that nonfunctional ACs may be more common than previously suggested.
Virtually all feminizing adrenal tumors in men are malignant.
Sporadic and syndromic variants.
The syndromic variants occur with Gardner, Beckwith-Wiedemann (associated with hemihypertrophy),
and Li-Fraumeni syndromes.
On the cellular origin of the neoplasm.
Primary adrenocortical carcinomas
Primary adrenal lymphomas
Soft-tissue sarcomas of the adrenal
Malignant pheochromocytomas
Secondary metastatic adrenal tumors (common primaries are the breast, kidney, lung, ovary,
melanoma, leukemia, lymphoma).

Pathophysiology:
The role of tumor suppressor gene mutations is suggested by their association with Li-Fraumeni syndrome, which is
characterized by inactivating germline mutations of the TP53 gene (a vital tumor suppressor gene or antioncogene)
on chromosome 17. This syndrome also is associated with a predisposition to other malignancies, including breast
carcinoma, leukemias, osteosarcomas, and soft-tissue sarcomas.
A few reports describe an association between AC and familial adenomatous polyposis, which also is due to a
germline inactivating mutation of a tumor suppressor gene (in this case, the adenomatous polyposis coli gene, APC).
Incidence: The incidence is approximately 0.6-1.67 cases per million persons per year.
Race: AC has no specific racial predilection.
Sex: The female-to-male ratio is 2.5-3:1. Male patients tend to be older and have a worse overall prognosis than
female patients.
Age: AC occurs in 2 major peaks:
******ebook converter DEMO Watermarks*******
 In the first decade of life and again in the fourth to fifth decades.
Approximately 75% of the children with AC are younger than 5 years.
Functional tumors also are more common in children, while nonfunctional tumors are more common in adults.

History: Most patients with AC present with advanced disease that is characterized by multiple abdominal or extra-
abdominal metastatic masses (stage IV disease).
Nonfunctional variants:

These typically present with fever, weight loss, abdominal pain and tenderness, back pain, abdominal fullness,
or symptoms related to metastases.
In other cases, mass is found incidentally, during radiological imaging.

Endocrine syndromes

Approximately 30-40% of patients present with the typical features of Cushing syndrome, while 20-30%
present with virilization syndromes.
In children, however, virilization (in girls) or precocious puberty (in boys) is the most common endocrine
presentation of a functional AC.
Other modes of presentation include profound weakness, hypertension, and/or ileus from hypokalemia related
to hyperaldosteronism and hypoglycemia.

Physical:

Patients may present with features of Cushing syndrome, including truncal obesity, striae, severe acne, malar
flushing, supraclavicular and dorsocervical fat pads, Conn syndrome (hypertension with weakness and ileus
resulting from hypokalemia), virilization in girls, or precocity and feminization (rarely) in boys.
In nonfunctional tumors, the major finding is an abdominal mass, in a flank.

Lab Studies:

The best screening tests for Cushing syndrome are the standard 1-mg dexamethasone suppression test and the
24-hour urinary cortisol excretion test.
Screen for hyperaldosteronism by using simultaneous aldosterone and renin levels to compute aldosterone-to-
renin ratios.
Screen for virilization syndromes using serum adrenal androgens (androstenedione, dehydroepiandrosterone,
dehydroepiandrosterone sulfate), serum testosterone, and 24-hour urinary 17 ketosteroids.
Plasma estradiol and/or estrone tests can help screen for feminization syndromes.
The evaluation of adrenal masses also must include screening for possible pheochromocytoma, including, at a
minimum, a 24-hour urinary estimation of catecholamines (epinephrine, norepinephrine, dopamine) and
metabolites (particularly metanephrines and normetanephrines).

Imaging Studies:
CT scans and MRI:

Adrenal CT scans and MRI are the imaging studies of choice. The typical case is characterized by a large
unilateral adrenal mass with irregular edges. The presence of contiguous adenopathy serves as corroborating
evidence.

Ultrasonography:

This test has less sensitivity in detecting adrenal tumors

It has particular utility, in the follow-up of previously detected incidentalomas.

Other Tests:
******ebook converter DEMO Watermarks*******
Because the histologic analysis of these masses may be unreliable, fine and/or core tissue needle aspiration biopsies
(percutaneous route) generally are not recommended.
Histological Findings:
Macroscopic features suggesting malignancy include a weight > 500 g, presence of areas of calcification or necrosis,
and a grossly lobulated appearance.
Distinction between adrenocortical and adrenomedullary tumors:
These have distinctive histologic appearances and immunohistochemical staining patterns. While adrenomedullary
tumors stain positive for neuroendocrine markers (eg, synaptophysin, neuron-specific enolase, chromogranin A),
adrenocortical cells stain positive for D11. ACs virtually always are unilateral.
Staging:
Staging for adrenal carcinoma according to Sullivan and colleagues.
Tumor criteria:

TI – Tumor diameter smaller than or equal to 5 cm with no local invasion.

T2 – Tumor diameter larger than 5 cm with no local invasion.
TI – Tumor of any size with local extension but not involving adjacent organs.
T4 – Tumor of any size with local invasion of adjacent organs.

Lymph node criteria:

NO – No regional lymph node involvement.

NI – Positive regional nodes.

Metastasis criteria:

MO – No distant metastasis.
MI – Distant metastasis.

Stages:

Stage 1 - TI, NO, MO.

Stage 2 - T2, NO, MO.
Stage 3 - TI or T2, NI, MO.
Stage 4 - Any T, any N + M1 or T3, NI or T4.

Treatment: Medical Care:

Mitotane:

It is a relatively specific to adrenocortical cytotoxin.

At best, only 20-25% of patients respond to mitotane. Therapy may be required for at least 3 months before
deciding the response of mitotane.
Mitotane apparently causes adrenal inhibition without cellular destruction. The.

Suramin: Although a few reports suggest the potential utility of suramin as an additional chemotherapeutic agent in
the treatment of AC, this drug is not recommended for AC.
Gossypol:

Gossypol also has been tried for metastatic adrenal cancer.

It was originally developed as a spermatotoxin and was derived from cottonseed oil. It has been used widely in
China as a male contraceptive with few adverse effects. While the exact mechanism for its action is unclear, it
******ebook converter DEMO Watermarks*******
 is known to cause selective mitochondrial destruction by the uncoupling of oxidative phosphorylation.

Cisplatin-based chemotherapy:

In cases where mitotane fails, chemotherapeutic regimens containing cisplatin alone or in combination often are
used.
Cyclophosphamide, Adriamycin, and cisplatin (CAP), 5-fluoro uracil, Adriamycin, and cisplatin (FAP), and
cisplatin with VP-16 have been tried.

Surgical Care:

When feasible, total resection remains the management modality of choice for the definitive management of
AC. It also remains the only potentially curative therapeutic modality.

Cushing syndrome:
Cushing’s Syndrome is due to excessive levels of glucocorticoids causing non-specific symptoms such as obesity,
muscle weakness and depression.
Pathophysiology & Etiology:
The glucocorticoid cortisol is secreted from the zona fasciculata and reticularis of the adrenal gland under the
stimulus of adrenocorticotropin (ACTH) from the pituitary gland. ACTH in turn is secreted in response to
corticotropin releasing hormone (CRH) and vasopressin from the hypothalamus. Cortisol exerts negative feedback
control on both CRH and vasopressin in the hypothalamus, and ACTH in the pituitary. In normal individuals,
cortisol is secreted in a circadian rhythm. It is the loss of this circadian rhythm, together with loss of the normal
feedback mechanism of the hypothalamo-pituitary-adrenal (HPA) axis, which results in chronic exposure to
excessive circulating cortisol levels and that gives Cushing’s syndrome.
The etiology of Cushing’s syndrome can broadly be divided into two categories; ACTH-dependent and ACTH-
independent (Table). Of the ACTH-dependent forms, pituitary-dependent Cushing’s syndrome, Cushing’s disease, is
the most common, accounting for 60-80% of all cases.
Ectopic sources of ACTH derive from multiple tumor types, the most frequent being small-cell lung carcinoma.
Excessive autonomous cortisol secretion can occur from an adrenal adenoma or carcinoma.
In addition, rarer forms of Cushing’s syndrome include ectopic CRH production, macronodular adrenal hyperplasia,
adrenal hyperplasia secondary to abnormal hormone receptor expression.

******ebook converter DEMO Watermarks*******

Clinical Features
The classical impression of the disease are:
Gross obesity of the trunk with wasting of the limbs, facial rounding and plethora, hirsutism with frontal balding,
muscle weakness, spontaneous bruising, vertebral fractures, hypertension and diabetes mellitus.
Other symptoms include lethargy, depression, acne, easy bruising, loss of libido and menstrual irregularity.
The signs are : myopathy, thin skin and easy bruising.
Severe hirsutism and virilisation strongly suggest an adrenal carcinoma.
Biochemical Confirmation
Circadian rhythm assessment:
Loss of the normal circadian rhythm of cortisol secretion in Cushing’s syndrome, with elevated nocturnal levels.
At the NIH in the United States, an awake midnight cortisol of greater than 207 nmol/l was claimed to show 94%
sensitivity and 100% specificity for the differentiation of Cushing’s syndrome from pseudo-Cushing’s states.
Urinary free cortisol:
Measurement of urinary free cortisol (UFC) is a non-invasive test and is widely used. Under normal conditions, 10%
of plasma cortisol is ‘free’ or unbound and physiologically active. Unbound cortisol is filtered by the kidney, with
the majority being reabsorbed in the tubules, and the remainder excreted unchanged.
UFC measurement have a sensitivity of 95% for the diagnosis.
Low-dose dexamethasone suppression test:
In normal individuals administration of an exogenous glucocorticoid results in suppression of the HPA axis, whilst
patients with Cushing’s syndrome are resistant, at least partially, to negative feedback.
Dexamethasone is a synthetic glucocorticoid that is 30 times more potent than cortisol.
Treatment of Cushing’s Syndrome:
Surgical Management
Transphenoidal surgery:
Transsphenoidal surgery is widely regarded as the treatment of choice for Cushing’s disease. The overall remission
rate in various large series is in the order of 70-75%, although higher rates of approximately 90% can be achieved
with microadenomas.
******ebook converter DEMO Watermarks*******
Adrenalectomy:
Adrenalectomy is the definitive treatment for all cases ACTH-independent Cushing’s syndrome. This is either
unilateral in the case of an adrenal adenoma or carcinoma, or bilateral in cases of bilateral hyperplasia. In adrenal
adenomas cure following surgery in skilled hands is 100%. Bilateral adrenalectomy is also an important therapeutic
option in patients with ACTH-dependent Cushing’s syndrome not cured by other techniques. However, the
development of Nelson’s syndrome in patients with ACTH-secreting pituitary adenomas occurs in between 8% and
38% of cases.
Patients undergoing bilateral adrenalectomy will require lifelong mineralocorticoid and glucocorticoid replacement.
Surgery for the ectopic ACTH syndrome:
If the ectopic ACTH-secreting tumor is benign and amenable to surgical excision, such as in a lobectomy for a
bronchial carcinoid tumor, the chance of cure of Cushing’s syndrome is high. However, if significant metastatic
disease is present, surgery is unlikely to be of benefit.
ADDISON’S DISEASE (adrenal insufficiency, hypocortisolism):
Addison’s disease is characterized by weight loss, muscle weakness, fatigue, low blood pressure, and sometimes
darkening of the skin in both exposed and nonexposed parts.
Addison’s disease occurs when the adrenal glands do not produce enough of the hormone cortisol and, in some
cases, the hormone aldosterone.
Causes:
The problem may be due to a disorder of the adrenal glands themselves (primary adrenal insufficiency) or to
inadequate secretion of ACTH by the pituitary gland (secondary adrenal insufficiency).
Primary Adrenal Insufficiency:

Addison’s disease affects about 1 in 100,000 people.

Most cases are caused by the gradual destruction of the adrenal cortex immune system.
About 70 percent of reported cases of Addison’s disease are autoimmune.
Adrenal insufficiency occurs when at least 90 percent of the adrenal cortex has been destroyed.
As a result, often both glucocorticoid (cortisol) and mineralocorticoid (aldostertone) hormones are lacking.
Sometimes only the adrenal gland is affected, as in idiopathic adrenal insufficiency; sometimes other glands
also are affected, as in the polyendocrine deficiency syndrome.

Polyendocrine Deficiency Syndrome:

The polyendocrine deficiency syndrome is classified into two separate forms, referred to as type I and type II.
Type I occurs in children, and adrenal insufficiency may be accompanied by

Underactive parathyroid glands.

Slow sexual development.
Pernicious anemia.
Chronic candida infections.
Chronic active hepatitis.
Hair loss (in very rare cases).

Type II, often called Schmidt’s syndrome, afflicts young adults. Features of type II are:

An underactive thyroid gland.

Slow sexual development.
Diabetes.
Vitiligo.
Loss of pigment on areas of the skin.

Tuberculosis:
******ebook converter DEMO Watermarks*******
Tuberculosis (TB), accounts for about 20 percent of cases of primary adrenal insufficiency in developed
countries.
Other Causes:

Chronic infection, mainly fungal infections.

Cancer cells spreading from other parts of the body to the adrenal glands.
Amyloidosis.
Surgical removal of the adrenal glands.

Secondary Adrenal Insufficiency:

This form of adrenal insufficiency is more common than primary adrenal insufficiency. Without ACTH to stimulate
the adrenals, the adrenal glands’ production of cortisol drops, but not aldosterone.
A temporary form of secondary adrenal insufficiency may occur when a person who has been receiving a
glucocorticoid hormone.
Glucocorticoid hormones block the release of both corticotropin-releasing hormone (CRH) and ACTH. Normally,
CRH instructs the pituitary gland to release ACTH. If CRH levels drop, the pituitary is not stimulated to release
ACTH, and the adrenals then fail to secrete sufficient levels of cortisol.
Less commonly, adrenal insufficiency occurs when the pituitary gland either decreases in size or stops producing
ACTH. These events can result from.

Tumors or infections of the area.

Loss of blood flow to the pituitary.
Radiation for the treatment of pituitary tumors.
Surgical removal of parts of the hypothalamus.
Surgical removal of the pituitary gland.

Symptoms:
The symptoms of adrenal insufficiency usually begin gradually.

Chronic, worsening fatigue.

Muscle weakness.
Loss of appetite.
Weight loss.
Nausea.
Vomiting.
Diarrhea.

Other symptoms include:

Low blood pressure that falls further when standing, causing dizziness or fainting.
Skin changes in Addison’s disease, with areas of hyperpigmentation, or dark tanning, covering exposed and
nonexposed parts of the body; this darkening of the skin is most visible on scars; skin folds; pressure points
such as the elbows, knees, knuckles, and toes; lips; and mucous membranes.
Addison’s disease can cause irritability and depression.
Hypoglycemia, or low blood glucose, is more severe in children than in adults.
In women, menstrual periods may become irregular or stop.
Because the symptoms progress slowly, they are usually ignored until a stressful event like an illness or an
accident causes them to become worse. This is called an addisonian crisis, or acute adrenal insufficiency. In
about 25 percent of patients, symptoms first appear during an addisonian crisis.

Symptoms of an addisonian crisis include:

******ebook converter DEMO Watermarks*******

 Sudden penetrating pain in the lower back, abdomen, or legs.
Severe vomiting and diarrhea.
Dehydration.
Low blood pressure.
Loss of consciousness.

Diagnosis:
ACTH Stimulation Test:
This is the most specific test for diagnosing Addison’s disease. In this test, blood cortisol, urine cortisol, or both
are measured before and after a synthetic form of ACTH is given, and measurement of cortisol in blood is repeated
30 to 60 minutes after an intravenous ACTH injection. The normal response after an injection of ACTH is a rise in
blood and urine cortisol levels. Patients with either form of adrenal insufficiency respond poorly or do not respond at
all.
CRH Stimulation Test:
When the response to the short ACTH test is abnormal, a “long” CRH stimulation test is required to determine the
cause of adrenal insufficiency. In this test, synthetic CRH is injected intravenously and blood cortisol is measured
before and 30, 60, 90, and 120 minutes after the injection. Patients with primary adrenal insufficiency have high
ACTHs but do not produce cortisol. Patients with secondary adrenal insufficiency have deficient cortisol responses
but absent or delayed ACTH responses. Absent ACTH response points to the pituitary as the cause; a delayed
ACTH response points to the hypothalamus as the cause.
Addisonian crisis must be teeated with injections of salt, fluids, and glucocorticoid hormones immediately.
Treatment:
Cortisol is replaced orally with hydrocortisone tablets, a synthetic glucocorticoid, taken once or twice a day.
If aldosterone is also deficient, it is replaced with oral doses of fludrocortisone acetate. Patients with secondary
adrenal insufficiency do not require aldosterone replacement therapy.

******ebook converter DEMO Watermarks*******

Salivary Gland:
A. Parotid Gland:
1. Located on side of face, anterior to mastoid tip and external auditory canal, inferior to zygomatic arch, and
superior to the lower border of the angle of the mandible. Anteriorly, it overlaps the masseter muscle.
2. Stenson’s duct enters oral cavity through buccal mucosa opposite upper 2nd molar.
3. Parasympathetic secretory afferents to the parotid leave the inferior salivary nucleus with the glossopharyngeal
nerve and travel via Jacobson’s plexus in the middle ear to synapse in the otic ganglion. Post-synaptic fibers are
distributed to the parotid by the auriculotemporal nerve.
4. Facial nerve passes through this gland.
B. Submandibular Gland:
1. Beneath floor of the mouth, inferior to mylohyoid muscles and superior to digastric muscle.
2. Marginal mandibular branch of the facial nerve travels in the fascia on the lateral surface of this gland.
3. Parasympathetic secretory afferents to the submandibular gland arise from the superior salivatory nucleus, and
leave the brainstem in the facial nerve. They exit the facial nerve at the geniculate ganglion and travel via the
chorda tympani to the lingual nerve. Fibers synapse in the submandibular ganglion, and post-synaptic fibers then
enter the gland.
4. The lingual and hypoglossal nerves lie deep to this gland.
5. Wharton’s duct enters the floor of the mouth near the lingual frenula.
C. Sublingual Glands:
Located below the mucous membrane of the floor of the mouth, adjacent to mandible and mylohyoid muscle. Ten to
twelve small caliber ducts drain the gland, some emptying into the submandibular duct, and others draining directly
into the floor of the mouth.
D. Minor Salivary Glands:
small collections of salivary gland tissues are scattered throughout the oral mucosa, and can also be seen in the
pharynx, supraglottis, nose and sinuses.
Parotid Gland:

Parotid gland appears on the 4th week of gestational life from the epithelium of the oro-pharynx.
Agenesis of the parotid glands is rare; may be associated with other facial abnormalities.
Cyst arising from the first branchial cleft may be located within the parotid gland.
Largest of the salivary glands and it overlaps the masseter muscle.
VIIthNr. passes through and divides the gland into a superficial and deep lobe
The deep surface of the gland lies alongside the back of the throat, near the tonsils.
Stenson’s duct enters oral cavity through buccal mucosa opposite upper 2nd molar.
Parasympathetic secretory afferents to the parotid leave the inferior salivary nucleus with the glossopharyngeal
nerve and travel via Jacobson’s plexus .in the middle ear to synapse in the otic ganglion. Post-synaptic fibers
are distributed to the parotid by the auriculotemporal nerve.

******ebook converter DEMO Watermarks*******

Submandibular Gland:

Beneath floor of the mouth, inferior to mylohyoid muscles and superior to digastric muscle.
Marginal mandibular branch of the facial nerve travels in the fascia on the lateral surface of this gland.
Parasympathetic secretory afferents to the submandibular gland arise from the superior salivatory nucleus, and
leave the brainstem in the facial nerve. They exit the facial nerve at the geniculate ganglion and travel via the
chorda tympani to the lingual nerve. Fibers synapse in the submandibular ganglion, and post-synaptic fibers
then enter the gland.
The lingual and hypoglossal nerves lie deep to this gland.
Wharton’s duct enters the floor of the mouth near the lingual frenula.

Sublingual Glands:
Located below the mucous membrane of the floor of the mouth, adjacent to mandible and mylohyoid muscle. Ten to
twelve small caliber ducts drain the gland, some emptying into the submandibular duct and others draining directly
into the floor of the mouth.
Minor Salivary Glands:
Small collections of salivary gland tissues are scattered throughout the oral mucosa, and can also be seen in the
pharynx, supraglottis, nose and sinuses. Minor glands are muco-serous only Ebner gland (posterior lingual gland)
like parotid, is pure serous.
Trauma:
A: Laceration: Parenchymal damage only – usually heals by it self.
Injury to Stenson’s duct – should be repaired over a small catheter.
Injury to facial nerve – should be repaired within 72 hours.

Injury to Stenson’s duct may cause chronic salivary fistula.

Acute obstruction or ligation of parotid duct causes complete atrophy of the gland.

B: Any foreign body should be removed.

Sialoadinitis:

In viral infection Mumps is the most common infection. Its treatment is symptomatic.

******ebook converter DEMO Watermarks*******

 Low grade bacterial infection of salivary gland is usually self limiting.
Stenson’s duct obstruction by Stone/stricture also causes intermittent painful swelling →Sialogram should be
done →obstruction should be relieved by →

1. Transorally (if the stone is near the duct end).

2. By external incision (If the stone is deep).
3. Parotidectomy (If multiple stone / stricture is present).
Acute Suppurative Parotitis:

Characterized by presence of pus and seen in debilitated/ dehydrated/ or in patients with poor oral hygiene.
Commonest causing organism is Staph. aureus.
Initial treatment is proper hydration/ antibiotics/ improving oral hygiene.
If abscess develops then it is drained by giving a J shaped incision (see 1st diagram).

Salivary Gland Tumors:

******ebook converter DEMO Watermarks*******

 Tumors of the salivary glands are grouped into epithelial, nonepithelial, and metastatic categories. Benign
epithelial tumors include pleomorphic adenoma (80%), Warthin tumor, monomorphic adenoma, intraductal
papilloma, oncocytoma etc.
Benign nonepithelial tumors (mesenchymal origin) include lipoma, hemangioma, lymphangioma (cystic
hygroma), and neural sheath tumors.
Pleomorphic adenomas make up 70% of parotid gland tumors and 50% of submandibular gland tumors.
Of minor SGTs, 50% are malignant.
Mucoepidermoid cancer is the most common parotid malignancy.
Overall, adenoid cystic carcinoma is the most common malignant tumor of all minor salivary glands and,
specifically, the submandibular gland.

Benign Epithelial Tumors:

1. Pleomorphic adenoma:

Commonly located at the superficial lobe.

Lesions arising from the deep lobe develop primarily within the parapharyngeal space and present late with
symptoms related to pharyngeal compression
It consists of epithelial and connective tissue. It is round, smooth, and freely mobile.
The term pleomorphic adenoma describes its multiples histological components, including myxoid, mucoid,
chondroid, and other element.
Malignant mixed tumour has a tendency for perineural and perivascular invasion and significant cellular atypia.
It has a thin, delicate capsule with occasional projections into the surrounding parotid tissue, so obtaining clean
margins is mandatory to minimize recurrence.
If the parotid is the gland involved, superficial parotidectomy with standard facial nerve dissection and
preservation is the procedure of choice.

2. Warthin tumor (ie, papillary cystadenoma lymphomatosum, cystic papillary adenoma, adenolymphoma):

It is the second most common benign tumour of the parotid gland.

In gross appearance, it is a smooth, soft parotid mass.
It is well encapsulated and contains multiple cysts.
Histologically, it has a heavy lymphoid stroma and aciniform epithelial cells lining the cystic areas with
papillary projections.
Malignant transformation has not been observed. The recurrence rate is 5%.
The Warthin tumor tends to be bilateral (10% of cases).

3. Lymphoepithelial hyperplasia (Mikulicz disease):

Manifest as a diffuse enlargement of the parotid gland, or it may manifest as a discrete mass.
Histologically, the lesion is composed of a diffused, well-organized lymphoid tissue and lymphocytic
interstitial infiltrate.
More frequent in females, with peak incidence in the fourth and fifth decades.
Growth of this tumor is slowly progressive, and it gives rise to pain around the ear or the retromandibular area.

4. Intraductal papilloma:

Intraductal papilloma is a small, smooth lesion that is found in the submucosal layer. Microscopically, it
consists of a
Cystically dilated duct partially lined with a cuboidal epithelium with complex anatomizing papillary fronds
filling the cystic area.

5. Oxyphil adenoma (oncocytoma):

Oncocytomas of the salivary glands are very uncommon.

Such neoplasms occur in women, after fifth decade (female-to-male ratio of 2:1), and the superficial lobe of the
parotid is commonly involved.

******ebook converter DEMO Watermarks*******

Kuttner tumor:

Is known as- chronic sclerosingsialadenitis, is a chronic inflammatory disease of the salivary gland.
Mainly involves “Submandibular glands”.
It produces a firm swelling of the glands and may be difficult to distinguish from neoplasia.
Heavy infiltration of the glandular tissue by lymphocytes (predominantly activated B-cells and helper T-cells)
as well as plasma cell with atrophy of acini is common finding.
The diagnosis can only be made histologically.
Excision of the mass, usually carried out diagnostically, is adequate treatment.

Staphne bone cyst is- Ectopic submandibiular gland in mandible bone

Bilateral cystic parotitisgiving Swiss cheese appearance is seen in HIV
Benign Nonepithelial Tumors
Hemangiomas:

Two forms, capillary and cavernous, develop in the major salivary glands.
The capillary type is the most prevalent tumor in the first year of life.
Capillary hemangiomas are rapidly growing, lack a capsule and are formed by purple, spongy, lobular masses
that infiltrate salivary gland tissue.
Observation is recommended in children.
Cavernous hemangiomas, which present in an older age group, rarely show spontaneous regression.
Recurrent ulceration or bleeding may require conservative surgical resection.

Lymphangioma (cystic hygroma):

They manifest as painless masses that may involve parotid, submandibular, or both. Diagnosis is made based on
clinical findings. Surgical excision with preservation of the vital structures is the treatment of choice.
Lipoma:
These tumors manifest as soft, mobile, painful masses and peak in the fifth and sixth decades, with a male-to-female
ratio of 10:1. They are slow-growing tumors with an average diameter of 3 cm. Treatment is surgical excision.
Metastatic disease of the parotid gland:
Melanoma (46%), squamous cell carcinoma (37%), and a variety of tumors (17%) are included in this category.
Malignant Tumours:
Includes :
1. Mucoepidermoid carcinoma.
2. Adenoid cystic carcinoma.
3. Adenocarcinoma.
4. Malignant mixed tumour.
5. Acinic cell carcinoma.
6. epidermoid carcinoma.
7. Squamous Cell carcinoma.
Acinic cell carcinoma:

It is a rare, low grade malignancy commonly seen in parotid.

Infrequently invade the facial nerve and are late to metastasize (to lung).

Mucoepidermoid carcinoma:

Most common Major salivary gland tumour.

******ebook converter DEMO Watermarks*******
 Most common malignancy in infancy.
Associated with radiation exposure.
It can be of low grade or high grade type.
On HPE: low grade contain mucus cells Intermediate grade contain clear cells high grade contain
epitheloid cells
High grade version is locally aggressive and prone for invasion of nerves and vessels and to early metastasis (to
regional nodes).
Treatment of generous primary excision with regional node dissection(only for high grade) followed by
radiotherapy.

Adenocarcinoma:

Less than 7-8% incidence.

It again has a low grade and high grade type and high grade has a very poor prognosis.

Adenoid cystic carcinoma:

Most common SG malignancy for Submandibular and Sublingual Glands.

It is more common in minor salivary glands than parotid.
they are low grade and slow growing tumors.
high propensity for perineural invasion.
It makes up one fourth of malignant salivary gland tumours.
Lung is most common metastatic site but it is known for its prolonged natural history (eg. Pt. may live for 10-
15 years even after lung metastasis).
When visceral or bone metastasis occurs prognosis is poor.
Treatment is wide surgical excision with radiation therapy.

Clinical features:

SGTs manifest as a painless mass on the face (parotid), the angle of the jaw (parotid tail, submandibular), neck
(submandibular), or a swelling at the floor of mouth (sublingual).
New onset of pain, rapid growth of the mass, facial nerve weakness, paresthesias, and hoarseness of the voice
are indicators of possible underlying malignancy.
Trismus usually represents invasion to the masseter or pterygoid muscles. Skin involvement and fixation to the
mastoid tip are also signs of malignancy.

Etiology:
An associated long-standing history of smoking and a strong family history may be risk factors. SGTs are indolent,
painless, and well-circumscribed tumors.
Treatment:
Medical therapy:

Inflammatory, infectious masses (eg, reactive, fungal), and lymphoma should be treated medically.
Salivary gland excision is also sometimes done for symptomatic, recurrent chronic gland infection, refractory
to conservative treatments.

Surgical therapy:

Standard in the management of SGTs is surgical therapy.

Treatment of the benign neoplasm is complete surgical excision of the affected gland – “Superficial
parotidectomy” with nerve preservation.
Benign tumour involving deep lobes is treated by “Total parotidectomy with preservation of facial nerve (Total
conservative parotidectomy)”.

******ebook converter DEMO Watermarks*******

 Tumor spillage of a pleomorphic adenoma is undesirable because it can lead to tumor recurrence and should be
avoided.
Excision of the tumor with clear margins is the aim for malignant tumours.
Parotic tumours are radio-responsive but chemo-resistant
In case of Facial nerve injury : best substitute for reconstruction is SURAL NERVE, other options being
auriculo-temporal nerve.

Complications of parotid gland surgery:

Complications of parotid gland surgery include:

Haematoma formation.
Infection.
Temporary facial nerve weakness.
Transection of the facial nerve and permanent facial weakness.
Sialocoele.
Facial numness.
Permanent numbness of the ear lobe associated with great auricular nerve transection.
Frey’s syndrome.

Frey’s syndrome:

Frey’s syndrome (gustatory sweating) is now considered an inevitable consequence of parotidectomy, unless
preventative measures are taken .
It results from damage to the autonomic innervation of the salivary gland with inappropriate regeneration of
parasympathetic nerve fibres that stimulate the sweat glands of the overlying skin.
The clinical features include sweating and erythema over the region of surgical excision of the parotid gland as
a consequence of autonomic stimulation of salivation by the smell or taste of food.
The symptoms are entirely variable and are clinically demonstrated by a starch iodine test.
This involves painting the affected area with iodine, which is allowed to dry before applying dry starch, which
turns blue on exposure to iodine in the presence of sweat.
Sweating is stimulated by salivary stimulation.
The management of Frey’s syndrome involves the prevention as well as the management of established
symptoms.

Prevention:
There are a number of techniques described to prevent Frey’s syndrome following parotidectomy. These include:

Sternomastoid muscle flap.

Temporalis fascial flap.
Insertion of artificial membranes between the skin and the parotid bed.

All these methods place a barrier between the skin and the parotid bed to minimise inappropriate regeneration of
autonomic nerve fibres.
Management of established Frey’s syndrome:
Methods of managing Frey’s syndrome include:

Antiperspirants, usually containing aluminium chloride.

Denervation by tympanic neurectomy;
The injection of botulinum toxin into the affected skin.

The last is the most effective and can be performed as an outpatient.

Cysts:

******ebook converter DEMO Watermarks*******

 Minor mucous retention cysts develop in the floor of the mouth either from an obstructed minor salivary gland
or from the sublingual salivary gland.
The term ‘ranula’ should be applied only to a mucous extravasation cyst that arises from a sublingual gland.
It produces a characteristic translucent swelling that takes on the appearance of a ‘frog’s belly’ (ranula).
A ranula can resolve spontaneously, but many also require formal surgical excision of the cyst and the affected
sublingual gland.
Incision and drainage, however tempting, usually results in recurrence.

Plunging ranula:

Plunging ranula is a rare form of mucous retention cyst that can arise from both sublingual and submandibular
salivary glands.
Mucus collects within the cyst, which perforates through the mylohyoid muscle diaphragm to enter the neck.
Patients present with a dumb-bell-shaped swelling that is soft, fluctuant and painless in the submandibular or
submental region of the neck.
Diagnosis is made on ultrasound or magnetic resonance imaging (MRI) examination.
Excision is usually performed via a cervical approach removing the cyst and both the submandibular and
sublingual glands.
Smaller plunging ranulas can be treated successfully by transoral sublingual gland excision, with or without
marsupialisation.

******ebook converter DEMO Watermarks*******

Deep Vein Thrombosis:
Essentials of Diagnosis:

Pain in the thigh or calf sometimes accompanied by edema. Half of patients are asymptomatic.
History of recent surgery, trauma, cancer, prolonged immobilization, or oral contraceptive use.
Clinical impression is accurate in 50% of cases.
Venous duplex ultrasound is the diagnostic modality of choice.

General Considerations.
The Virchow triad (stasis, vascular injury, and hypercoagulability) is cornerstone for DVT.
Acquired risk factors include:

In men, pancreatic and colorectal cancers have thrombotic risk, while hematological malignancies carry a
lower risk.
Other cancers are pancreas, ovary, and brain and breast cancer, especially during its chemotherapy treatment.
Endothelial injury leads to platelet aggregation, degranulation, and formation of thrombus as well as
vasoconstriction and activation of the coagulation cascade.
******ebook converter DEMO Watermarks*******
 Hypercoagulable states may also be inherited: Genetic causes are
Hematologic disorders associated with DVT are

Hematologic disorders associated with DVT are:

Disseminated intravascular coagulation.

Heparin-induced thrombocytopenia.
Antiphospholipid antibody syndrome.
Thrombotic thrombocytopenic purpura.
Hemolytic uremic syndrome.
Polycythemiavera, and essential.
Thrombocythemia.

Inflammatory bowel disease, systemic lupus erythematosus, and obesity are additionally associated with DVT.

DVT occurs most frequently in the calf veins, though it may arise in the femoral or iliac veins.
Thrombi originate in soleal sinusoids or in valve sinuses, where there are flow eddies.
Isolated calf vein thrombosis is associated with a low risk of pulmonary embolism.
25% progresses to proximal deep veins of the leg, 25% causes chronic venous and 10% pulmonary embolism
is.
Most common site of DVT- Calf veins
M/C site of thrombosis for pulmonary embolus- Femoropopliteal.

Clinical Findings:

Only 50% have symptoms.

Pain and mild edema.
Fever (Low grade) tachycardia- rare.
Homan sign: Pain on dorsiflexion of ankle (little value).
Moses Sign: Calf tenderness on direct pressure on calf (Little value).
Pratt’s sign: Calf tenderness on squeezing of calf (Little value).

Iliofemoral venous thrombosis can result in phlegmasia.

In phlegmasia alba dolens, the leg is pulseless, pale, and cool, which may progress.
Phlegmasia cerulean dolens, characterized by cyanosis of the limb and a precursor to gangrene.

Surgical Risk of DVT:

******ebook converter DEMO Watermarks*******

Investigations:

Historically, the standard for diagnosis was ascending phlebography

Duplex ultrasound has now become the test of choice (95% sensitive and specific).
Acute thrombus is anechoic while chronic thrombus is echogenic.
Dx criteria:
Absence of spontaneous flow.
Loss of flow variation with respiration.
Failure to increase flow velocity after distal augmentation.
In chronic venous thrombosis: Veins are narrowed, and there are prominent nearby collaterals. Chronic thrombi
are highly echogenic, while acute thrombi are anechoic (and therefore not visible) on the B-mode image.
Magnetic resonance venography is 100% sensitive and 96% specific. The injection of gadolinium is useful
for determining the age of the thrombus.
CT imaging, especially as part of a pulmonary embolism protocol CT image, may also be a good alternative to
establish the diagnosis.
D-dimer levels is too nonspecific but has high negative predictive value so helps in R/O.
Radiolabeled fibrinogen is too sensitive in the pelvis for use in the acute clinical setting and carries with it a
risk of transmission of infectious disease. It is no longer used.
Older tests like impedance plethysmography and venous pressure measurements are obsolete.

Complications:

Treatment:

Treatment is aimed at reducing the incidence of complications associated with DVT.

The primary treatment of DVT is systemic anticoagulation (risk of pulmonary embolism, extension of venous
thrombosis and rate of recurrent DVT reduced by 80%).
Systemic anticoagulation does not directly lyse thrombi but stops propagation and allows natural fibrinolysis.
Once the diagnosis of VTE has been made, antithrombotic therapy should be initiated promptly. If clinical
suspicion for VTE is high, it may be prudent to start treatment while the diagnosis is objectively confirmed.
The goals of VTE treatment are the prevention of mortality and morbidity associated with PE and the
prevention of the postthrombotic syndrome (PTS).
Treatment regimens may include:

1. antithrombotic therapy,
2. temporary or permanent vena cava filter placement,
3. catheter-directed or systemic thrombolytic therapy, and
4. operative thrombectomy.
Antithrombotic Therapy:

******ebook converter DEMO Watermarks*******

 Most often, antithrombotictherapy for VTE is initiated with IV or subcutaneous (SC) unfractionated heparin or
SC low molecular weight heparin.
Fondaparinux, a synthetic pentasaccharide, is sometimes also used as an alternative to heparin to initiate
therapy.
An oral vitamin K antagonist, usually sodium warfarin, is begun shortly after initiation of IV or SC therapy.
Either SC or IV therapy is continued until effective oral anticoagulation with warfarin is achieved as indicated
by an international normalized ratio (INR) ³2 for 24 hours.
A minimum of 5 days of heparin or fondaparinux therapy is recommended.
Recently, the U.S. Food and Drug Administration (FDA) has also approved alternative oral anticoagulants for
both treatment and prophylaxis for VTE.
Hemorrhage is the primary complication of UFH therapy.
For patients with UFH-related bleeding complications, cessation of UFH is required, and anticoagulation may
be reversed with protamine sulfate.
Low molecular weight heparins (LMWHs) bind to antithrombin via a specific pentasaccharide sequence to
expose an active site for the neutralization of fac- tor Xa.
Fondaparinuxcurrently is a synthetic pentasaccharidesequence that binds and activates antithrombin, causing
specific inhibition of factor Xa.
Direct thrombin inhibitors (DTIs) include recombinant hirudin, argatroban, and bivalirudin.
These antithrombotic agents bind to thrombin, inhibiting the conversion of fibrinogen to fibrin as well as
thrombin-induced platelet activation.
In patients with established HIT, DTIs should be administered for at least 7 days, or until the platelet count
normalizes.
Warfarin may then be introduced slowly, overlapping therapy with a DTI for at least 5 days.
Vitamin K antagonists, which include warfarin and other coumarin derivatives, are the mainstay of long-term
antithrombotic therapy in patients with VTE.
Warfarin inhibits the carboxylation of vitamin K–dependent procoagulants (factors II, VII, IX, and X) and
anticoagulants (proteins C and S), resulting in formation of less functional proteins.
Warfarin usually requires several days to achieve full effect because normal circulating coagulation proteins
must first undergo their normal degradation.
Factors X and II have the longest half-lives, in the range of 36 and 72 hours, respectively. A steady-state
concentration of warfarin is usually not reached for 4 to 5 days.
Warfarin therapy is monitored by measuring the INR.
The therapeutic target INR range is usually 2.0 to 3.0.
The recommended duration of warfarin antithrombotic therapy is stratified based on whether the DVT was
provoked or unprovoked, whether it was the first or a recurrent episode,where the DVT is located, and whether
malignancy or thrombophilia is associated.
In patients with VTE related to malignancy, increasing evidence suggests that longer-term therapy with LMWH
(up to 6 months) is associated with a lower VTE recurrence than treatment using conventional vitamin K
antagonists.
The primary complication of warfarin therapy is hemorrhage, and the risk is related to the magnitude of INR
prolongation.
The ACCP recommendation, therefore, is that 3 months of anticoagulation are sufficient to prevent recurrent
VTE in patients with DVT occurring around the time of a transient risk factor (e.g., hospitalization, orthopedic
or major general surgery).

******ebook converter DEMO Watermarks*******

DVT prophylaxis:

Thrombolysis: Preferred in Iliac vein thrombosis especially early in course with extremely swollen limb
IVC filters to prevent Pulmonary embolus (Greenfield) are recommended in patients when anticoagulation is
contraindicated or recurrent embolism.

Abdominal aortic aneurysms:

An AAA is an increase in aortic diameter by greater than 50% of normal (aortic diameter of greater than 3 cm
diameter).
In men, the infrarenal aorta is normally between 14 and 24 mm, and in women, it is between 12 and 21 mm.
Therefore, an abdominal aortic aneurysm (AAA) is diagnosed if the diameter is 3 cm or larger in a man or 2.6
cm or larger in a woman.
Classification of abdominal aortic aneurysms.
infrarenal.
juxtarenal.
pararenal.
suprarenal.
More prevalent in elderly men. Male : female ratio is 4:1.
Risk factors – hypertension, peripheral vascular disease, family history (15-25%).
Other causes of aortic aneurysms include:
******ebook converter DEMO Watermarks*******
 Genetic: There is a familial tendency to aortic aneurysms. Connective tissue disorders such as Ehlers-
Danlos syndrome and Marfan’s syndrome.
Post-traumatic.
Arteritis, e.g.Takayasu disease, giant cell arteritis, and polychondritis.
Congenital malformation of the aorta (aneurysms tend to develop just beyond the narrowing of a
coarctation of the aorta).
End-stage (tertiary) syphilis, which tends to affect the ascending aorta and arch of the aorta.
Mycotic: infective (immunodeficiency, IV drug abuse, valve surgery).
Degenerative aneurysms account for more than 90% of all infrarenal AAAs.
Most cases of AAA begin below the renal arteries and end above the iliac arteries.
They generally are spindle shaped (fusiform).

Natural history:

In general, AAAs gradually enlarges (0.2-0.8 mm/y) and eventually rupture.

5 year risk of rupture:
5.0 – 5.9 cm = 25%
6.0 – 6.9 cm = 35%
More than 7 cm = 75%
Risk Factors for Rupture of Abdominal Aortic Aneurysm:

Pathophysiology:

The aortic wall contains smooth muscle, elastin, and collagen arranged in concentric layers.
The number of medial elastin layers from the proximal thoracic aorta to the infrarenal aorta is markedly
reduced, with medial thinning and intimal thickening.
Elastin is the principal load-bearing element in the aorta. Elastin fragmentation and degeneration are observed
in aneurysm walls.
It is coupled with the histological changes of this matrix protein in aneurysms.

Clinical features:
75% are asymptomatic.

Possible symptoms include.

Epigastric pain.
Back pain.
Malaise and weight loss (with inflammatory aneurysms).
Rupture presents with:
Sudden onset abdominal pain.
Hypovolaemic shock.
Pulsatile epigastric mass.
Rare presentations include.
Distal embolic features: may cause livedoreticularis (blue toe syndrome).
Acute aortic occlusion: Occasionally, small AAAs thrombosis, producing acute claudication.

******ebook converter DEMO Watermarks*******

 Aorto-caval fistula (symptoms include tachycardia, congestive heart failure (CHF), leg swelling,
abdominal thrill, machinery-type abdominal bruit, renal failure, and peripheral ischemia).
Primary aorto-intestinal fistula (AAA may rupture into the fourth portion of the duodenum and present
with a herald upper gastrointestinal bleed).

Indication for operation:

Rupture.
Symptomatic aneurysm; any size.
Rapid expansion.
Asymptomatic > 6 cm – exact lower limit controversial.

Contraindications:
COPD/ severe cardiac disease/ active infection/ and medical problems that preclude operative intervention. These
patients may benefit best from endovascular stenting of the aneurysm. Severe life-threatening comorbidities include
advanced cancer, end-stage lung disease, or cardiac disease.
Approach:

Monitor patients if AAA is smaller than 4 cm with ultrasound every 6 months, offer surgical intervention if the
aneurysm expands or becomes symptomatic.
Patients with an AAA of 5-6 cm in diameter benefit from repair, especially if they have other contributing
factors like hypertension, continued smoking, or COPD.
For patients at higher risk, the threshold for repair may be 6-7 cm in diameter.

Pre-operative investigation:

Need to determine.
Extent of aneurysm.
Fitness for operation.
Ultrasound, conventional CT and more recently spiral CT.
Determines – aneurysm size, relation to renal arteries, involvement of iliac vessels.
Angiography: It is indicated only(not in all cases) when associated renal or visceral involvement, peripheral
occlusive disease, or aneurysmal disease exists.
Most significant post op morbidity and mortality related to cardiac disease
Cardiac revascularisation required in up to 10% of patients.

Treatment:
Medical therapy: Smoking cessation/ aggressively control hypertension.
Surgical therapy: Operative approach is through the traditional open laparotomy approach or, by the placement of
endovascular stents.
Prevention of distal embolization: The patient is heparinized prior to aortic cross clamping. If significant
intraluminal debris, juxtarenal thrombus, or prior peripheral embolization exists, the distal arteries are clamped first,
followed by aortic clamping.

******ebook converter DEMO Watermarks*******

Endovascular aneurysm repair:

Morbidity of conventional open aneurysm surgery related to:

Exposure of infra-renal aorta/ Cross clamping of aorta.
Endovascular repair achieved by transfemoral or transiliac placement of prosthetic graft.
Proximal and distal cuffs / stents anchor graft.
Exclude aneurysm from circulation.
Only ~40% of aneurysms suitable for this type of repair.
Major problem related to placement and leakage around stent.

Complications:

Graft migration/ Endovascular leak/ Graft kinking/ Graft occlusion.

Aortic dissection:

Commonest aortic emergency.

Incidence is twice that of ruptured abdominal aortic aneurysm.
Most commonly seen between 50 and 70 years.
Associated with hypertension, Marfan’s syndrome, bicuspid aortic valve.

Pathology:

Intimal tear results in blood splitting the aortic media.

Rupture can occur back into the lumen or externally in pricardium/ mediastinum.
External rupture often results in fatal pericardial tamponade.
Commonest site of intimal tear is within 2-3 cm of aortic valve.
Dissection can result in occlusion of aortic branches.
Most commonly involved are renal, spinal, coronary or iliac arteries.
******ebook converter DEMO Watermarks*******
Classification:

Clinical features:

Usually presents with tearing chest pain radiating to back associated with collapse.
Examination may show.
Reduced or absent peripheral pulsed.
Soft early diastolic murmur.
Chest x-ray usually shows a widened mediastinum.
If aortic branches occluded there may clinical evidence of:
Acute renal failure.
Paraplegia.
Acute limb ischaemia.
Cerebrovascular accident.
Inferior myocardial infarction.

Management:

All patients require urgent management of associated hypertension.

Type A dissections usually require surgical intervention.
Dissection excised and aorta replaced with graft.
Aortic valve is preserved if possible.
An evolving CVA or established renal failure are contraindications to surgery.
Type B dissections may be treated without surgery.
Requires fastidious blood pressure control.
Surgery should be considered if evidence of aortic expansion.
Surgery for Type B dissections is associated with significant risk of paraplegia.
Without operation the prognosis for Type A dissections is poor.
40% die within 24 hours and 80% die within 2 weeks.

Popliteal artery aneurysms:

Defined as a popliteal artery diameter greater than 2 cm.

Account for 80% of all peripheral aneurysms.
50% are bilateral. 50% are associated with an abdominal aortic aneurysm. 50% are asymptomatic.
Symptomatic aneurysms present with features of:
Compression of adjacent structures (veins or nerves).
Rupture.
Limb iscahemia due to emboli or acute thrombosis.

******ebook converter DEMO Watermarks*******

 Treatment is by proximal and distal ligation.
Revascularisation of the leg with a femoropopliteal bypass.
With a symptomatic popliteal aneurysm 20% patients will undergo an amputation.

Arterial assessment
Clinical Assessment:
Claudication:

Calf or thigh pain precipitated by exercise. Usually occurs after predictable distance. Relieved by rest
Progression of symptoms is important - worsening or improvement
Need to differentiate form spinal stenosis: Also cause exercise induced leg pain; Usually associated with
neurological symptoms and relieved by spinal flexion
Peripheral pulses can be present in patients with intermittent claudication

Critical limb ischaemia:

Characterised by rest pain.

Occurs when foot is elevated (e.g. in bed).
Improved with foot dependent.
May be associated with ulceration or gangrene.
Foot pulses are invariably absent.

Non-invasive testing of arterial patency:

Hand-held doppler:

Reflection of an ultrasound wave off a stationary object does not change its frequency. Reflection off a moving
object results in a change of frequency.
The change in frequency is proportional to velocity or blood flow.
Hand held 8 MHz doppler probe is used to assess arterial system.
Can be used to measure arterial pressures (at rest and after exercise).
In normal individual lower limb pressures are greater than upper limb
Ankle-brachial pressure index (ratio of best foot systolic to brachial systolic Pr).

******ebook converter DEMO Watermarks*******

Toe pressures:

Provides accurate assessment of distal circulation.

Not influenced by calcification in pedal vessels.
Normal toe pressures are 90-100 mmHg.
Toe pressure less than 30 mmHg suggests critical limb ischaemia.

Duplex ultrasound:

Combined pulsed doppler and real time B mode ultrasound.

Allows imaging of vessels and any stenotic lesion.
Flow and pressure wave form can be also be assessed.
Duplex ultrasound has sensitivity of 80% and specificity of 90%.

Pulse generated run off:

Proximal occlusion often causes poor filling of crural vessels on arteriography.

Rapid cycling of a proximal cuff generates arterial pulse wave.
P GR allows functional testing of distal arterial patency.

Magnetic resonance angiography:

No contrast required.
Invasive Vascular Assessment:
Angiography:

Usually performed using digital subtraction techniques.

Femoral artery is commonest site of venous access.
Potential complications include.
Contrast-related: Anaphylactic reaction / Toxic reactions. Deterioration in renal function.
Technique-related : Haematoma/ Arterial spasm / Sub-intimal dissection/ False aneurysm/ Arteriovenous
fistula/ Embolisation/ Infection.

CT angiography:

Required intravenous contrast and ionising radiation.

Spiral CT and reconstruction can provide detailed images.
Particularly useful for the assessment of aneurysmal disease.

Acute limb ischaemia:

Effects of sudden arterial occlusion depends on state of collateral supply.

Aetiology of acute limb ischaemia:

Embolism.
Left atrium in patients in atrial fibrillation.
Mural thrombus after myocardial infarct.
Prosthetic and diseases heart valves.
Aneurysm or atheromatous stenosis.
Tumour, foreign body, paradoxical.
Thrombosis.
Trauma.
Dissecting aneurysm.
******ebook converter DEMO Watermarks*******
 Raynaud’s Syndrome.

Clinical features of limb ischaemia:

Clinical diagnosis depends on the 6 ‘p’ s.

Pain/ Paraesthesia/ Pallor/ Pulselessness/ Paralysis/ Perishing with cold
Objective sensory loss requires urgent treatment.
Need to differentiate embolism from thrombosis.
Important clinical features include.
Rapidity of onset of symptoms.
Features of pre-existing chronic arterial disease.
Potential source of embolus.
State of pedal pulses in contralateral leg.

Management of acute ischaemia:

Initial:

Heparinise& analgesia. Treat associated cardiac disease.

Treatment options are:
Embolic disease - embolectomy or intra-arterial thrombolysis.
Thrombotic disease - intra-arterial thrombolysis / angioplasty or bypass surgery.

Emergency embolectomy:
Can be performed under either general or local anaesthesia.

Transverse artereotomy performed over common femoral artery.

Fogarty balloon embolectomy catheters used to retrieve thrombus.
If embolectomy fails – on-table angiogram and consider.
Bypass graft or intraoperative thrombolysis.

Intra-arterial thrombolysis:

Arteriogram and catheter advanced into thrombus. Streptokinase 5000u/hr + heparin 250u/hr.
Alternative thrombolytic agents are urokinase/ tissue plasminogen activator (tPA).
Repeat arteriogram at 6 -12 hours
Advance catheter and continue thrombolysis for 48 hours or until clot lysis.
Angioplasty of chronic arterial stenosis may be necessary.

Buerger Disease (Thromboangiitis Obliterans):

Thromboangiitis obliterans is a nonatherosclerotic, segmental, inflammatory, vasoocclusive disease that affects the
small and medium-sized arteries and veins of the upper and lower extremities. It is strongly associated with heavy
tobacco use.
Male-to-female ratio = 3:1and majority of patients are aged 20-45 years.
History:
Because a firm diagnosis of thromboangiitisobliterans is difficult to establish, a number of different diagnostic
criteria have been proposed:

Age younger than 45 years.

Current (or recent) history of tobacco use.
Presence of distal-extremity ischemia (indicated by claudication, pain at rest, ischemic ulcers, or gangrene)
documented by noninvasive vascular testing.
Exclusion of autoimmune diseases, hypercoagulable states, and diabetes mellitus by laboratory tests.

******ebook converter DEMO Watermarks*******

 Exclusion of a proximal source of emboli by echocardiography and arteriography
Consistent arteriographic findings in the involved and noninvolved limbs.

Patients also may present with claudication of the feet, legs, hands, or arms and often describe Raynaud
phenomenon of sensitivity of the hands and fingers to cold.
Physical:

The diseased hands and feet are usually cool and mildly edematous.
Superficial thrombophlebitis is often migratory (in 50%). Paresthesias (numbness, tingling, burning,
hypoesthesia) of the feet and hands.
Impaired distal pulses in the presence of normal proximal pulses.

Imaging Studies:

Other Tests:

An abnormal Allen test indicating distal arterial disease and establishing involvement of the upper extremities
in addition to the lower extremities helps to differentiate thromboangiitisobliterans from atherosclerotic disease.

Treatment:
Absolute discontinuation of tobacco use.
Treatment with intravenous iloprost(a prostaglandin analogue), has been shown to improve symptoms, accelerating
resolution of distal extremity trophic changes.
Surgical Care:
Given the diffuse segmental nature and that the disease affects primarily small and medium-sized arteries; surgical
revascularization is usually not feasible.
Autologous vein bypass of coexistent large-vessel atherosclerotic stenoses should be considered in patients with
severe ischemia who have an acceptable distal target vessel.

Other proposed surgical treatments for thromboangiitisobliterans are:

Omental transfer.
Sympathectomy.

******ebook converter DEMO Watermarks*******

 Spinal cord stimulator implantation
Distal limb amputation for nonhealing ulcers, gangrene, or intractable pain may be required.

Varicose veins:
Varicose veins are veins that have dilated under the influence of increased venous pressure.
Vein diameter > 3 mm in upright position.

Varicose veins affect: 20-25% of adult females.10-15% of adult males.

Etiology:

Intrinsic pathological conditions and extrinsic environmental factors combine to produce a wide spectrum of
varicose disease.
Most varicose disease is caused by elevated superficial venous pressures.
Some people have an inborn weakness of vein walls.
Reflux at the saphenofemoral junction (SFJ).
Prolonged standing leads to increased hydrostatic pressures that can cause chronic venous distention and
secondary valvular incompetence.
If proximal junctional valves become incompetent, high pressure passes from the deep veins into superficial
veins and the condition rapidly becomes irreversible.

******ebook converter DEMO Watermarks*******

History:
Common symptoms include, leg heaviness, exercise intolerance, pain or tenderness along the course of a vein,
pruritus, burning sensations, restless legs, night cramps, edema, skin changes, and paresthesias.

Pain caused by venous insufficiency often is improved by walking in contrast to the pain of arterial
insufficiency, which is worse with ambulation and elevation.
Acute varicose complications are variceal bleeding, dermatitis, thrombophlebitis, cellulitis, and ulceration.
Ulcers are mainly on the medial side of calf (Above medial malleolus).
Poor correlation exists between symptoms and signs.
If history of DVT need preoperative investigation with duplex scanning.

Examination:

Identify distribution of varicose veins - long saphenous vs short saphenous.

Pigmentation is due to deposition of Hemosiderin and melanin.
Ankle flare is suggestive of varicose vein.
Champaign bottle leg.
Confirm with tourniquet testing and hand held-doppler probe (5 MHz).
Indications for duplex scanning.
Suspected short saphenous incompetence.
Recurrent varicose veins.
Complicated varicose veins (e.g. ulceration, lipodermatosclerosis).
History of deep venous thrombosis.
Varicosities in Thigh: Long Saphenous.
Varicosities in Back of leg: Short Saphenous incompitence.

Perthes maneuver:
The Perthes maneuver is a traditional technique intended to distinguish antegrade flow from retrograde flow in
superficial varices. Antegrade flow in a variceal system indicates that the system is a bypass pathway around a deep
venous obstruction. This is critically important because if deep veins are not patent, superficial varices are an
important pathway for venous return and must not be sclerosed or surgically removed.
If the Perthes maneuver is positive and the distal varices have become engorged, the patient is placed supine with
the tourniquet in place and the leg is elevated (Linton test). If varices distal to the tourniquet fail to drain after a few
seconds, deep venous obstruction must be suspected.
Trendelenburg test:
The Trendelenburg test often can distinguish patients with superficial venous reflux from those with incompetent
deep venous valves.
Morrissey test:
Cough impulse test.
Schwartz test:
In long saphenous varicosity if lower part is tapped, impulse felt at saphenous opening.
Fegan’s test:

******ebook converter DEMO Watermarks*******

Palpation to find the fascial defect of incompetent perforators.
Treatment:

Elastic compression stockings.

Bisgard’s Regimen: Limb Elevation + Compression stockings + Massage for varicose.

Indications for varicose vein surgery:

Most surgery is cosmetic or for minor symptoms.

Absolute indications for surgery :
Lipodermatosclerosis leading to venous ulceration.
Recurrent superficial thrombophlebitis.
Bleeding from ruptured varix.

Contraindications:

Venous outflow obstruction and during pregnancy.

DVT is absolute C/I for varicose vein surgery.

LSV surgery:

Trendelenberg position with 20 – 30° head down.

Saphenofemoral junction (SFJ) found 2 cm below and lateral to pubic tubercle.
Essential to identify SFJ before performing flush ligation of the LSV.
Individually divide and ligate all tributaries of the LSV.
Superficial circumflex iliac vein.
Superficial inferior epigastric vein.
Superficial and deep external pudendal vein.
Check that femoral vein clear of direct branches for 1 cm above and below SFJ.
Do fluch ligation of LSV at SF junction.
Stripping of LSV reduces risk of recurrence. Only strip to upper calf if needed.
Stripping to ankle is associated with increased risk of saphenous neuralgia.
Post-operative care: Elevate foot of bed for 12 hours. Varix stocking should be worn for at least 2
weeks.

SSV surgery:

Patient prone with 20-30° head down.

Saphenopopliteal junction (SPJ) has very variable position.
Identify and preserve the sural nerve.
Need to identify the Sapheno-popliteal Junction.
Stripping associated with risk of sural nerve damage.
Subfascial ligation inadequate.

Perforator surgery:

Perforator disease may be improved by superficial vein surgery.

Perforator surgery (e.g. Cockett’s and Todd’s procedure) associated with high morbidity.
Subfascial endoscopic perforator surgery (SEPS) recently described.
May have a role in addition to saphenous surgery in those with venous ulceration
Sclerotherapy.

Sclerotherapy “Varicose veins: sclerotherapy”:

******ebook converter DEMO Watermarks*******

 Sodium tetra-decylsulphate is most commonly used.
It causes thrombosis, fibrosis and sclerosis by destroying lipid membrane of endothelial cells.
Not good for major saphenous incompitenece.
Only suitable for below knee varicose veins.
Need to exclude SFJ or SPJ incompetence
Main use is for persistent or recurrent varicose veins after adequate saphenous surgery.
Sclerosants.
5% Ethanolamine oleate/ 0.5% Sodium tetradecylsulphate (Most commonly used).
Needle placed in vein when full with patient standing.
Empty vein prior to injection.
Apply immediate compression and maintain for 4-6 weeks.

Complications of sclerotherapy:

Extravasation causing pigmentation or ulceration.

Deep venous thrombosis.

Other Modalities:

Endovenous laser: Endovenous laser therapy is a thermal ablation technique that uses a laser fiber placed
inside the vein to destroy the vascular endothelium.
Radiofrequency ablation: Radiofrequency (RF) ablation is a thermal ablation technique. This tissue heating
causes protein denaturation, collagenous contraction, and immediate closure of the vessel.
Ambulatory phlebectomy: The stab-avulsion technique allows removal of short segments of varicose and
reticular veins through tiny incisions.

TIPP (Transilluminated powered phlebectomy) by TRIVEX:

Irrigatedtransilluminatorispasseddeep to varicositiesand using a powersuctionresectorveinissucked, morcellated and
removed bysuction.

Recurrent varicose veins “Varicose veins: recurrent”.

15 - 25 % of varicose vein surgery is for recurrence.

Reasons for recurrence:

Inaccurate clinical assessment.

Confusion as to whether varicosities are in LSV or SSV distribution.
Inadequate primary surgery.
10% cases SFJ not correctly identified.
20% cases tributaries mistaken for LSV.
Failure to strip LSV.
Injudicious use of sclerotherapy.
70% of SF incompetence treated with sclero-therapy will have recurrence.
Neovascularisation.
With recurrent varicose vein need to image with duplex or varicography.

Complications:
Deep vein thrombosis and pulmonary embolism are the most serious complications. Other complications are
dysesthesias from injury to the sural nerve or the saphenous nerve, subcutaneous haematoma, infection and
arterial injury.
Thoracic Outlet Syndrome:
Definitions: Thoracic outlet syndrome is a disease of extrinsic compression of the artery, vein, or nerve at the
thoracic outlet.

******ebook converter DEMO Watermarks*******

 The specific structures compressed are usually the nerves of the branchial plexus and occasionally the
subclavian artery or subclavian vein.
The compressing structures include the clavicle, the first rib, subclavian muscles, costoclavicular ligament and
the anterior scalene.

The predisposing factors are fibromuscular bands, bony protuberances and long or larger transverse processes, this
together with the tendinous or cartilaginous muscular insertions are responsible for the compression of the
neurovascular structures at the thoracic outlet.

Symptoms:

******ebook converter DEMO Watermarks*******

Compression can be of different magnitude in each of these structures. For example, the subclavian vein can be the
only compressed structure and this patient might have a thrombosis of the vein that was called in the past effort
thrombosis, or a swelling of the fingers. The subclavian artery can also be compressed with symptoms of temporary,
arterial, positional insufficiency of the upper extremity. When they are present for a long time, aneurysm and
thrombosis of the subclavian artery may develop with distal embolization. Nerve compression of the brachial plexus
is very common and is or not associated with venous or arterial compression.

Paget Schroetter Syndrome: This is the name given to the subclavian vein thrombosis (beneath the clavicle)
which results in pain, swelling, blue discoloration, and congestion of the arm. It is commonly caused by
compression of the vein between the clavicle and the first rib, and is considered one of the venous manifestations
of TOS.

Physical Examination:

Posture.
The White Hand Sign.
C7-C8-T1 Testing.
Sweating, Swelling.
Selmonosky Triad.
Tenderness in the supra clavicular area.
Hand paleness and/or paresthesias on elevation.
Adduction and abduction weakness of fingers 4 & 5. (C8 - T1 testing).
The Adson sign is the loss of radial pulse by rotating the head to the ipsilateral side and inspiring.

Investigations:
Imaging Studies:
Chest x-ray:
Cervical ribs or rudimentary first ribs often can be identified with a CXR.
CT scan:

CT scans with 3-dimensional reconstruction have become popular for evaluating the thoracic outlet.
CT scan angiography and venography.

Standard MRI:
Dynamic MRI with gadolinium infusion also provides detail of the thoracic outlet and may be helpful when
evaluating for compression.
Angiography with dynamic positioning.
Venography with dynamic positioning.
Other Tests:
Electromyography (EMG) and nerve conduction studies are useful in the workup of patients suspected of having
neurogenic TOS. A reduction in nerve conduction velocity < 85 m/s of either ulnar or median nerves across the
thoracic outlet corroborates the diagnosis of neurogenic TOS.
Indications:
Failure of conservative treatment in a patient with severe disability.
Treatment:
Physical therapy:
Postural exercises, stretching, abdominal breathing, and medications used to relieve muscular tension and pain are
******ebook converter DEMO Watermarks*******
beneficial.
No satisfactory medical treatment for arterial TOS exists.
Surgical therapy:

Arterial TOS requires prompt surgical intervention to treat or prevent acute thromboembolic events.
Treatment for venous TOS-related effort thrombosis relies on anticoagulation and arm elevation leaves.

Venous thoracic outlet obstruction: Surgical treatment of venous TOS consists of releasing the extrinsic
compression and restoring luminal patency. After thrombolysis, surgeons wait one month before decompressive
treatment surgery is undertaken. Surgical decompression of veins within the scalene triangle is achieved by
anterior rib resection, anterior scalene release and in some cases clavicular resection.
Neurogenic/arterial thoracic outlet obstruction
Thoracic outlet decompression can be performed through an axillary, supraclavicular, or posterior approach.
Thoracic outlet decompression may entail anterior and middle scalenectomy, first rib resection, or scalenectomy
plus first rib resection.
Vascular trauma:

Vascular trauma can result from either blunt or penetrating injury.

Types of vascular injury:

Contusion / Puncture / Laceration / Transection.

Pathophysiology:

Haemorrhage is the prime consequence of vascular injury.

Bleeding may be obvious, with visible arterial haemorrhage, or it may be concealed.
Ischaemia results from an acute interruption of flow of blood to a limb or organ.
Peripheral nerves are more sensitive to ischaemia, and prolonged neurological deficit may result from relatively
short periods of tissue ischaemia.
If arterial supply is restored to ischaemia tissue, the sudden release of inflammatory mediators, lactic acid,
potassium and other intracellular material into the circulation can cause profound myocardial depression,
generalised vasodilatation and initiate a systemic inflammatory response.

Clinical features:

Depends on site, mechanism and extent of injury.

Signs classically divided into ‘hard’ and ‘soft’ sign.

Hard signs of vascular injury:

Pulsatile bleeding.
Expanding haematoma.
Absent distal pulses, cold, pale limb- Distal ischaemia.
Audible Bruit or palpable thrill.
Active haemorrhage.

The presence of hard signs of vascular injury mandates immediate operative interventionwithout prior
investigation.
Soft signs of vascular injury:

Haematoma.
******ebook converter DEMO Watermarks*******
 History of haemorrhage at site of accident.
Unexplained hypotension
Peripheral nerve deficit
Decreased pulse compared to the contralateral extremity
Bony injury or in proximity penetrating wound

Softer signs require close observation and monitoring. If the ABI is higher than 0.9, close observation is advocated,
but if the ABI is lower than 0.9, further evaluation is warranted.
Investigation

Arteriography should be considered:

To confirm extent of injury in stable patient with equivocal signs
To exclude injury in patient without hard signs but strong suspicion of vascular injury

Diagnostic Adjuncts:
Pulse Oximetry:
A reduction in oximeter readings from one limb, as compared to another is suggestive of significant vascular injury.
Doppler Ultrasound:
The diagnosis of a significant (ie. requiring intervention) vascular injury has been shown to be related to the
presence or absence of a palpable pulse. Similarly, a reduction in the ankle-brachial pressure index (ABPI) in the
presence of a palpable pulse does not indicate the presence of a vascular injury requiring intervention. Doppler
ultrasound is therefore adds little to careful clinical examination.
Duplex Ultrasound:
Duplex imaging is a non-invasive examination combining B-mode and Doppler ultrasound. Duplex can detect
intimal tears, thrombosis, false aneurysms and arteriovenous fistulae.
Angiography: Angiography remains the gold-standard investigation for the further investigation and
delineation of vascular injury. Proximal control may be possible with an angioplasty catheter prior to transfer to
the operating room.
Management:

The priorities of vascular injury are arrest of haemorrhage and restoration of normal circulation.
Airway control and respiratory assessment take priority over management of the circulation.

Immediate Haemorrhage Control:

By direct pressure or where haemorrhage is welling up from a deep knife or gunshot track, control may be
temporarily achieved by passing a urinary catheter into the track as far as possible, inflating the balloon. If
angiography is performed prior to surgery, it may be possible to obtain proximal control by passing an angioplasty
balloon catheter into the proximal vessel and inflating the balloon.
Volume resuscitation:
Prior to haemorrhage control, minimal fluids should be administered. Raising the blood pressure will increase
haemorrhage from the vessel injury. No inotropes should be given to the hypovolaemic patient as this will
effectively deplete myocardial tissue oxygen and increase myocardial work. Once haemorrhage control is achieved,
aggressive volume resuscitation is done.
Operative Strategy:

The basic principle of vascular repair is to gain proximal and distal control of the relevant vessel before
investigating the site of injury.
Proximal control is best achieved through a separate incision away from the site of injury.
******ebook converter DEMO Watermarks*******
 Distal control similarly is best achieved via a second incision.
Once proximal and distal control is achieved, the site of injury can be explored and control made closer to the
injury site.
Once the vessel injury is identified, the first step is debridement of devitalized tissue and definition of the edges
of the wound.
Next an assessment of inflow and outflow is made. If it is inadequate, a balloon (Fogarty) catheter is passed
proximally and distally to extract any thrombus.
Following extraction, heparinized saline is instilled proximally and distally to locally antcoagulate the vessel.
Small, clean, transverse wounds to vessels that involve only part of the circumference can be repaired with a
direct suture technique.
A vein or synthetic patch may be required where there is a larger defect in the vessel wall where direct suturing
may lead to narrowing of the vessel lumen.
While vein grafts probably have a longer patency, the graft infection rates are the same for both vein and
synthetic grafts, regardless of wound contamination.

Compartment syndrome:

Prolonged interruprion of blood flow to a limb leads to cellular ischaemia, activation of cellular and humoral
inflammatory responses and alterations in vascular permeability. Subsequent reperfusion of the limb leads to
generalised tissue oedema.
When this occurs in a limited, enclosed space - such as the fascial compartments of the lower limb, the pressure
in the compartment may rise above capillary and venous pressure and cause vascular stasis, cellular ischaemia
and death.
The pressure in the compartments is rarely above arterial pressure and distal pulses are preserved.
If the patient is awake, there is intense, disproportionate pain in the limb, worsened by passive flexion of the
muscle groups.
In measurement of compartment pressures values over 30mmHg are diagnostic of compartment
syndrome.
Fasciotomy is best performed at the time of initial surgery, rather than as a subsequent procedure for a second
episode of limb ischaemia.

Aims of surgery are to:

Control life-threatening haemorrhage.

Prevent limb ischaemia.
If surgery is delayed more than 6 hours, revascularisation is unlikely to be successful.

Vascular repair:

Usually performed after gaining proximal control and wound debridement.

Options include :
Simple suture of puncture hole or laceration.
Vein patch angioplasty.
Resection and end-to-end anastomosis.
Interpositional graft.
Contralateral saphenous vein is the ideal Interpositional graft.
Prosthetic graft material may be used if poor vein or bilateral limb trauma.

Complications of vascular injury:

Thrombosis of the graft remains the most common complication of vascular injury.
Narrowing of the vessel with primary repair or kinking of the graft, may require revision of the repair.

“False aneurysm”:

******ebook converter DEMO Watermarks*******

 Most commonly occurs following catheterisation of femoral artery.
Often presents with pain, bruising and a pulsatile swelling.
Diagnosis can be confirmed by doppler ultrasound.
Suturing of puncture site/ Vein patching may be required.

Arteriovenous fistula:

Often presents several weeks after the injury.

Patient complains of a swollen limb with dilated superficial veins.
Machinery type bruit (continuous) often present throughout cardiac cycle.
Diagnosis can be confirmed by angiography.
Fistula can be divided an both the vein and artery sutured.
Flap of fascia can be interposed between vessels to reduce risk of recurrence.

Klipple Trenaunay Syndrome:

Congenital AV fistula, Absence of Deep venous system and valves, Varicose veins, Cutaneous haemangiomas and
limb hypertrophy.
Kasabach Meritt Syndrome:
Big hemagiomas or AV malformations causing intravascular coagulation (consumption of coagulation factors),
platelet trapping, thrombocytopenia, Microangiopathic hemolytic anaemia.

******ebook converter DEMO Watermarks*******

Groin Hernia:
Groin hernias are the most common type of hernias.

Anatomy:

******ebook converter DEMO Watermarks*******

The myopectineal orfice of Fruchamds.
The passageway for the great vessels to the lower extremity, and for the testicle to reach the scrotum.
The MPO is divided anteriorly by the inguinal ligament, nad posteriorly by the iliopublic tract. It is bounded
medially bu the lateral border of the rectus muscle, superiorly by the arching fibers of the transversuabdominus and
the internal oblique muscles, laterally by the iliopsoas muscle and inferiorly by the Cooper ligament.
Myopectineal orifice of Fruchaud:

The MPO is divided anteriorly by the inguinal ligament, and posteriorly by the iliopubic tract. It is bounded
medially by the lateral border of the rectus muscle, superiorly by the arching fibers of the
transversusabdominus and the internal oblique muscles, laterally by the iliopsoas muscle and inferiorly by the
Cooper ligament.
The MPO is perforated in its superior pane by the spermatic cord, and through its inferior pane by the femoral
vessels.
The MPO is protected only by the combined lamina of the aponeurosis of the transversusabdominus and the
transversalis fascia.

Myopectineal orifice of Fruchaud a region of the groin is bounded anteriorly by the inguinal ligament, and
posteriorly by the iliopubic tract, Superiorly by the internal oblique and trans versus abdomen is muscles, medially
by the rectus abdominismuscl, laterally by theiliopsoasmuscle, and inferiorly by Cooper ligament and the pubis. It
transmits the spermatic cord and femoralvessels; all inguinalhernias beginas weak are as in this orifice.

The ‘Myopectineal Orifice Of Fruchaud:

In 1956, Henry Fruchaud espoused the theory that all groin (inguinofemoral) hernia originate in a single weak
area called the Myopectineal orifice. This oval, funnellike, ‘potential’ orifice formed by the following structures,
forms the ‘Myopectineal orifice of Fruchaud’.

Superiorly Internal oblique and transverses abdominis muscles.

Inferiorly Superior pubic ramus.
Medially Rectus muscle sheath.
******ebook converter DEMO Watermarks*******
 Laterally Iliopsoas muscle.
Weakness through this area leads to inguinofemoral hernia. Proper exposure of the area is essential during a
preperitoneal (posterior) repair!
To avoid missing small hernia in addition to.
To achieve adequate fixation.

The orifice is divided through the Iliopubic tract and the inguinal ligament into an ‘inguinal’ defect and a ‘femoral
defect’.

Classification of Groin Hernias:

Gilbert designed a classification for primary and recurrent inguinal hernias done through an anterior approach . It is
based on evaluating 3 factors:

presence or absence of a peritoneal sac.

size of the internal ring.
integrity of the posterior wall of the canal.

Types 1, 2 and 3 are indirect hernias; types 4 and 5 are direct.

Type 1 hernias have a peritoneal sac passing through an intact internal ring that will not admit 1 fingerbreadth
(ie,<1 cm.); the posterior wall is intact.
Type 2 hernias (the most common indirect hernia) have a peritoneal sac coming through a 1-fingerbreadth
internal ring (ie, </=2 cm.); the posterior wall is intact.
Type 3 hernias have a peritoneal sac coming through a 2-fingerbreadth or wider internal ring (ie, >2 cm.).
(Type 3 hernias frequently are complete and often have a sliding component. They begin to break down a
portion of the posterior wall just medial to the internal ring).
Type 4 hernias have a full floor posterior wall breakdown or multiple defects in the posterior wall. The internal
ring is intact, and there is no peritoneal sac.
Type 5 hernias are pubic tubercle recurrence or primary diverticular hernias. There is no peritoneal sac and the
internal ring remains intact. In cases where double hernias exist, both types are designated (eg, Types 2/4).
Descriptors such as L, Sld., Inc., Strang. Fem. are used to designate lipoma, sliding component, incarceration,
strangulation and femoral components.

In 1993,Rutkow and Robbins added a type 6 to the Gilbert classification to designate double inguinal hernias and a
type 7 to designate a femoral hernia.

Nyhus developed a classification designed for the posterior approach based on the size of the internal ring and the
integrity of the posterior wall.

Type 1 is an indirect hernia with a normal internal ring.

Type 2 is an indirect hernia with an enlarged internal ring.
Type 3a is a direct inguinal hernia.
******ebook converter DEMO Watermarks*******
 Type 3b is an indirect hernia causing posterior wall weakness.
Type 3c is a femoral hernia.
Type 4 represents all recurrent hernias.

Treatment:
Most groin hernias are clinically important and should be repaired electively.
Anesthesia:
Local anesthesia; Regional anesthesia: Subarachnoid block or spinal anesthesia.
Other options: Such as caudal anesthesia or paravertebral block. General anesthesia provides complete relaxation
and calms the patient’s fears however.
The Evolution of Hernia Repair.
EdoardoBassini: The Father of Modern Day Hernia Surgery.
Bassini’s operation epitomized the essential steps for an ideal tissue repair. He opened the external oblique
aponeurosis through the external ring, and then resected the cremasteric fascia to expose the spermatic cord.
He then divided the canal’s posterior wall to expose the preperitoneal space and did a high dissection and
ligation of the peritoneal sac in the iliac fossa. Bassini then reconstructed the canal’s posterior wall in 3
layers. He approximated the medial tissues, including the internal oblique muscle, transversusabdominus
muscle and transversalis fascia to the shelving edge of the inguinal ligament with interrupted sutures. He then
placed the cord against that newly constructed wall and closed the external oblique aponeurosis over it,
thereby restoring the step-down effect of the canal and reforming the external inguinal ring.

******ebook converter DEMO Watermarks*******

There have been numerous modifications of Bassini’s original technique eg. Introduction of relaxing incisions by
Tanner- Tanner’s slide.
Stoppa used the posterior approach to implant an impermeable barrier around the entire peritoneal bag,
demonstrating that permanent repair of groin hernias does not require closure of the abdominal wall defect per se.
Without having stated it, their repair used a tension-free technique InStoppa’s approach, the mesh is held in place by
intra-abdominal pressure, an application of Pascal’s principle.

******ebook converter DEMO Watermarks*******

Contemporary Classical Repairs:
Modified Bassini. Bassini’s original repair yielded outstanding results for a pure tissue technique, but, as noted
above, problems occurred when surgeons failed to open the posterior wall. This operation became known as the
“modified” or “North American” Bassini. By not opening the posterior wall, the wall tissue was damaged in its most
medial portion by sutures placed under tension, and recurrences resulted, primarily in the pubic tubercle area.
Shouldice repair: It applies the principle of an imbricated posterior wall closure with continuous monofilament
suture. At the Shouldice hospital, continuous stainless-steel wire is used for all layers of the repair. The
Shouldice repair remains an excellent option, however, and has produced the bsest and most enduring results of any
other pure tissue repair.

Tension-Free Hernia Repair:

The most important advance in hernia surgery has been the development of tension-free repairs. Usher opened the
posterior wall and sutured a swatch of Marlex mesh to the undersurface of the medial margin of the defect (which he
described as the transversalis fascia and the conjoined tendon) and to the shelving edge of the inguinal ligament.
The most common prosthetic open repairs done today are the Kugel patch repair, the Lichtenstein onlay patch
repair, the PerFix plug and patch repair, and the PROLENE Hernia Systembilayer patch repair.

******ebook converter DEMO Watermarks*******

The Stoppa-Rives giant prosthetic repair of the visceral sac is also an important tension-free technique done through
an open posterior approach.
Complications Associated with Hernia Surgery:
Infection: Before the use of prosthetics, hernia repair was considered a clean, low-risk operation that did not require
prophylactic antibiotics. Because the use of prosthetics later was erroneously associated with increased infection risk

******ebook converter DEMO Watermarks*******

Seroma: A seroma is a collection of serum in a surgical wound. The size of the collection relates to the amount of
dissection done between tissue planes and the amount of dead space remaining in the wound.
The wound appears raised but is not inflamed or tender. The mass is fluctuant and the fluid ballotable.
Ultrasonography confirms the clinical diagnosis. Treatment consists primarily of observation and expectation.
Aspiration is rarely necessary, and in most cases the seroma will completely reabsorb in 2 to 3 weeks. Early
aspiration is futile, as the fluid will reaccumulate within a day or 2.
Hematoma: Opening the wound, evacuating the hematoma, and allowing it to close by secondary intention best
treats bleeding into the wound.
Postoperative Neuralgia: Symptoms are pain or a burning sensation in the inguinal region, which may radiate to
the genitalia and the upper thigh. It is aggravated by activity and relieved by hip flexion. Tinel’s sign helps in
identifying a trigger point causing the problem.If localized anesthetic blocks confirm the diagnosis of a specific
postoperative neuralgia.Treatment is resection of the nerve trunk carried as far proximal as possible.
Pediatric Inguinal Hernias:
Inguinal hernias in the pediatric age group are almost always indirect, the result of persistent patency of the
processusvaginalis.Theprocessusvaginalis is still open in most newborns.It normally becomes fibrosed during
infancy, and by age 2 most are completely obliterated. The persistent processus in itself does not indicate the
presence of a hernia.Bowelor other intra-abdominal contents must come into the processus for it to clinically
become a hernia. The processus may close inconsistently, leading to a funicular hernia, a scrotal hernia, or, a
hydrocele.

The persistence of the processusvaginalis seems to be more common on the right side.
Once the diagnosis of an inguinal hernia is made in a child it should be repaired, there is a higher incidence of
incarceration or strangulation in these young children.
Repair of Pediatric Hernias:
Repair of most pediatric hernias requires ligation of the true neck of the sac through the internal ring.The sac should
be examined to rule out the presence of a sliding component. This is especially important in female patients, as it
may contain a Fallopian tube or ovary that could inadvertently be ligated.
In general, prosthetics should not be used in small children. However, hernias in full-grown teenagers can be safely
repaired with mesh.
Laparoscopic Hernia Repair:
Intraperitoneal onlay mesh technique (IPOM):
The IPOM technique focused on the placement of an intra-abdominal piece of a prosthetic biomaterial fixed with

******ebook converter DEMO Watermarks*******

some type of stapling device); the repair did not involve the dissection of the peritoneum. The advantages of this
repair were the lack of significant dissection of the preperitoneal space and the rapid placement of the prosthesis.
The recurrence rate, however, was somewhat higher that that of the more widely adopted repairs developed later.
Trans abdominal preperitoneal (TAPP) repair method:
In this approach, the preperitoneal tissue is removed from the fascial layer by directly entering the intra-abdominal
cavity. This is similar to the IPOM approach, except that TAPP involves more dissection of the preperitoneal space.
With TAPP, the prosthesis is placed into the preperitoneal space following dissection, fixed with a stapling device or
a spiral tacking device, and covered.
Totally extraperitoneal approach (TEP):
The TEP approach generally employs a preperitoneal dissection balloon that is introduced via a subumbilical
incision. The balloon is inflated, creating the preperitoneal space for the hernia repair. Two or 3 additional working
ports are then placed to complete the necessary surgical dissection in which to adequately expose the inguinal floor
and the myopectineal orifice. Once the hernia defects are visualized, a polypropylene biomaterial is then inserted
and secured to the transversalis fascia and/or Cooper’s ligament with tacks or staples.The material is of a size that
completely covers the myopectineal orifice.
In both the TAPP and TEP approaches, the hernia sac is reduced, and a large piece of mesh is placed over the area in
order to cover any indirect, direct, or femoral hernia found. The mesh can be held in place using staples, or by the
pressure of the peritoneum alone (after removing the balloon).
The laparoscopic approach has several advantages over previous repairs. They include smaller incisions, less pain
and disability, a quicker return to work, and less recurrence.
Other terminology and types of hernias:

Maydel’s hernia (Hernia in W): Strangulated loop of W lies within the abdomen and local symptoms of
strangulation are not marked.
Sliding hernia (Hernia engissade): The posterior wall of sac is also formed by cecum (right), Sigmoid colon
(left) or by a portion of bladder (either side).
Spigelian hernia: Occurs commonly at the level of arcute line.
Lumber hernia: Exits through inferior lumber triangle of Petit (formed by iliac crest; external oblique and
latissmusdorsi), or rarely through superior lumber triangle (formed by sacrospinalis; lower border of 12th rib
and posterior border of internal oblique).
Obturator hernia: it occurs through the obturator canal. Swelling is covered by pectinius but becomes more
apparent on flexion abduction and external rotation of the limb.
Gluteal hernia: passes through greater sciatic foramina either above or below the piriformis.
Schiatic hernia: passes through the lesser schiatic foramen.
Incisional hernia occurs in a previous scar and it is the most common hernia in a female.
Velpeau hernia: A hernia in the groin in front of the femoral blood vessels. Named for the 19th-century Paris
surgeon Alfred Armand Louis Marie Velpeau (1795-1867).
Holt·house’s hernia: An inguinal hernia with extension of the loop of the intestine along the inguinal
ligament.
Gibbon hernia: Hernia with hydrocele
Berger: Hernia in Pouch of Douglas.
Beclard: Femoral hernia through saphenous opening.
Serofini’s hernia - Behind femoral vessels.
Ogilive: Hernia through a congenital circular defect in the conjoined tendon
Stammer: Through Transverse colon window after retro-coloic gastro-jejunostomy.
Peterson hernia: Hernia through mesenteric defects of Roux-en-Y gastric bypass procedure. The mesenteric
defect in such cases, called Petersen’s defect, is located between the transverse colon and the mesentery of
Jejunum.
Amyand’s hernia is an inguinal hernia in which the hernia sac contains a appendix.
Cooper’s hernia: a femoral hernia with two sacs, the first being in the femoral canal, and the second passing
through a defect in the superficial fascia and appearing almost immediately beneath the skin.
Grynfelt-Lesshaft hernias: occurs through “superior lumbar triangle”, bordered by.
******ebook converter DEMO Watermarks*******
 Inferior aspect of the 12th rib.
The internal oblique muscle.
The quadratus lumborum.
Petit hernias occur in the “inferior lumbar triangle”, whose borders are
The external oblique muscle.
The latissimus dorsi muscle.
The iliac crest.

The Triangle of Doom is an anatomical triangle defined by the vas deferens medially, spermatic vessels laterally
and peritoneal folds covering external iliac vessels inferiorly. This triangle contains external iliac artery and vessels,
the deep circumflex iliac vein, the genital branch of genitofemoral nerve and hidden by fascia the femoral nerve.

The “triangle of pain” is an inverted “V” shaped area with its apex at the internal (deep) inguinal ring. It is bound
anteriorly by the iliopubic tract / inguinal ligament and by the testicular (spermatic) vessels posteromedially. It
contains.

Lateral femoral cutaneous nerve

Femoral nerve.
Genitofemoral nerve.

******ebook converter DEMO Watermarks*******

Corona mortis” or crown of death:
The “corona mortis” is an anatomical variant, an anastomosis between the obturator and the external iliac or inferior
epigastric arteries or veins. It is located behind the superior pubic ramus at a variable distance from the symphysis
pubis. The name “corona mortis” or crown of death testifies to the importance of this feature, as significant
hemorrhage may occur if accidentally cut and it is difficult to achieve subsequent hemostasis. It is important for
surgery for anterior approach to the acetabulum or femoral hernia.Yellow triangle in 2nd photo is Hasselbach’s
triangle (direct hernia) and area just media to femoral vein is site of femoral ring (origin of femoral hernia). One can
see that this artery comes in field of femoral hernia surgery.

******ebook converter DEMO Watermarks*******

“Cave of Retzius” or “Retzius’ space”:
Is Retropubic space, a extra-peritoneal space between the pubic symphysis and urinary bladder. It is separated from
the anterior abdominal wall by the transversalis fascia and extends to the level of the umbilicus. (Important space in
TEP laparoscopic hernia repair).

******ebook converter DEMO Watermarks*******

Space of Borgos- Retro-inguinal space:

A triangular space between the peritoneum and transversalis fascia, at the lower angle of which is the inguinal
ligament.
It contains the lower portion of the external iliac artery.
This ‘preperitoneal space’ is split into two by the posterior lamina from the transversalis fascia.
The posterior compartment continues to be now termed as the ‘Space of Bogros.
The anterior space has been referred to as the ‘Vascular Space’.
In some places the posterior lamina is deficient, there the peritoneum adheres towards the anterior lamina.
Medially it’s continuous with the space of Retzius.

Synonym:
spatiumretroinguinale.

******ebook converter DEMO Watermarks*******

Traumatic Abdominal Wall Hernias:

Type I hernias are small defects caused by blunt trauma (handlebar injury)
Type II result from high-velocity traumas (motor vehicle accidents, falls) and tend to be larger
Type III, which typically result from deceleration injuries and involve herniation of intra abdominal contents

Femoral Hernia:
Epidemiology:

Accounts for 5% (5-10%)of Groin Hernias (96% are inguinal)

More common in elderly women (F: M = 3:1).

Anatomy:

Femoral canal – 1.25 cm long from the femoral ring above to the saphenous opening below.

Pathophysiology:

Associated with increased intra-abdominal pressure

Hernia sac bulges into femoral canal, which is continuation of femoral sheath
Femoral canal lies immediately medial to femoral vein

Mechanism:

Hernia is narrow in the canal

No resistance at the saphenous opening - expands upwards towards abdomen, because the deep fascia of the
abdomen is attached lower to the saphenous opening to the fascia lata.
May form inverted retort shape - may traverse above Ing. lig.
Predisposes to strangulation d.t. tortuous course, narrow canal, fixed rigid ring.

Symptoms and Signs:

Groin Pain and tenderness often absent, strangulation occurs often without pain
Hernia sac neck location palpable lateral and inferior to pubic tubercle
Large femoral hernias may bulge over inguinal ligament

Differential Diagnosis:

Inguinal Hernia, Inguinal Lymphadenopathy, Varix of Saphenous Vein (Thrill on palpation; Fills on standing
and empties while supine).
Infectious Bubo (Chancroid, Syphilis, Lymphogranuloma venereum).

Varieties:
Laugier’s Femoral hernia - Occurs through a defect in the lacunar ligament (of Gimbernat). A small hernia in a very
medial position. Almost always presents as strangulated.
Narath’s femoral hernia - Seen in Congenital dislocation of hip. Occurs due to lat displacement of the psoas.
Cloquet’s femoral hernia - Occurs behind the pectineus muscle. The hernia is behind the femoral vessels.
Pre-vascular femoral hernia - Occurs in front of the inguinal ligament and the femoral vessels. Has a wide neck and
less tendency to strangulate.
Treatment:
No role for conservative management e.g. truss
******ebook converter DEMO Watermarks*******
Operations:
The hernia is reduced, & repair done by stitching the conjoint tendon to the Cooper’s ligament.
1. Low Approach (LOCKWOOD):
Groin crease incision/ high risk of injury to abnormal obturator Artery/ not used in strangulation as intestine not well
approached.
2. High Approach (McEVEDY):
Vertical incision over femoral canal extended over the ing. lig. up to the abdomen/ Good control over abnormal
obturator Artery/ Useful in strangulated hernias/ higher risk of incisional hernia.
3. Inguinal Approach (LOTHEISSEN):
Incision over inguinal canal/ Good control over abnormal A.

******ebook converter DEMO Watermarks*******

Umbilical Hernia:
Definition:
A condition caused by a small defect in the periumbilical musculature of the anterior abdominal wall resulting in
protrusion of the umbilicus.
Epidemiology:

Age of onset: at birth or during the first year of life.

Risk factors: blacks > whites/ low birth weight/ hypothyroidism/ chromosomal anomalies (i.e., Trisomy 13)/
Mucopolysaccharidoses (i.e., Hurler Syndrome) / Beckwith-Wiedemann Syndrome.

Pathogenesis:

In normal embryogenesis, the intestines exit the abdominal cavity, return, rotate, then become fixed to the
posterior abdominal wall.
An umbilical hernia results from the failure of this process and due to an imperfect closure or weakness of the
umbilical ring, a small portion of the intestine remains in the umbilical coelom producing a small sac
protruding up through the base of the umbilical cord.
******ebook converter DEMO Watermarks*******
 This sac (hernia) may contain omentum or portions of the small intestine.

Clinical Features:
1. Protuberant Umbilicus: usually varies from 1-5 cm in diameter/ easily reduced when the infant is relaxed/ is
soft, non-tender, and covered by normal skin.
2. Complications: incarceration (irreducible umbilical hernia)/ strangulation of the intestine within the hernia/
perforation of the hernia.
Management:
1. Supportive.

observe as most hernias close spontaneously before 5 years of age.

most hernias that appear before 6 months of age disappear by 1 year of age.

2. Surgery: indications for surgery:

Complications (incarceration, strangulation, perforation).

If the hernia persists to 3-4 years of age
A large hernia (defects larger than 2 cm in diameter are less likely to close spontaneously).
The hernia becomes progressively larger after 1-2 years of age or cosmetic reasons.

Congenital diaphragmatic hernia:

Occurs in 1 in 2500-4000 live births.

Results from failure of closure of the pleuro-peritoneal canals.
The herniation occurs in the 8-10th week of gestation.
95% occur through the posterior foreman of Bochdalek (occur on the left).
Less than 5% occur through the anterior foreman of Morgagni.
The midgut herniates into the chest impairing lung development.
Abnormalities of the pulmonary vasculature results in pulmonary hypertension.
Usually associated with gastrointestinal malrotation.

Anatomy:

The diaphragm is composed of muscle and fascia that separates the chest from the abdominal cavity.
It is composed of three muscles parts about the rim that lead to a central tendinous portion.
The muscle parts are:
The sternal portion that attaches to the breastbone area.
The costal (rib) portion that attaches along the ribs.
The lumbar portion that attaches along the back.
The tissue formed by the fusion of the various parts of the diaphragm is called the pleuroperitoneal membrane.
The are three openings in the diaphragm that allow passage of:
The inferior vena cava.
The esophagus.
The aorta.
The diaphragm is covered on both sides by a membranous layer of fascia. The transversalis fascia covers the
abdominal side, and the endothoracic fascia covers the thoracic side.
The phrenic nerves control the muscles of the diaphragm.

******ebook converter DEMO Watermarks*******

Fryns syndrome is an autosomal recessive disorder with variable features, including diaphragmatic hernia,
cleft lip or palate, and distal digital hypoplasia.

******ebook converter DEMO Watermarks*******

Clinical features:

Often presents with cyanosis and respiratory distress soon after birth.
Prognosis is related to the time of onset and degree of respiratory impairment.
The abdomen to flat and Air entry is reduced on the affected side.
Heart sounds are often displaced.
Chest x-ray will confirm the presence of gastrointestinal loops in the chest.
Occasionally presents with respiratory distress of intestinal obstruction later in life.

Management:

Respiratory support with intubation and ventilation is usually required/ A Ryle’s tube should be passed/ Gas
exchange and acid-base status should be assessed/ Acidosis may need correction with bicarbonate infusion/
Surgery should be considered early after resuscitation/ Hernial content are usually reduced via and abdominal
approach/ Hernial sac is excised and diaphragm repaired with nonabsorbable suture or a Gortex patch/ A
Ladd’s procedure may be required for malrotation/ Early respiratory failure is associated with a poor prognosis.

Incisional Hernia:
It occurs through a weak surgical or traumatic wound. It is a type of Ventral Hernia. Usually, the incisional hernia
presents as a bulge near a previous wound. The condition is often asymptomatic but occasionally, presents with pain
or strangulation.
Pathophysiology:

Develops in scar of prior laparotomy or drain site.

Risks for post-operative hernia development.
Vertical scar more commonly affected than horizontal/ Wound infection/ Wound dehiscence/
******ebook converter DEMO Watermarks*******
 Malnutrition/ Obesity/ Tobacco abuse/ Presence of drains in wounds/ DM, jaundice, renal failure,
immunosuppression/ Malignant disease.

Incisions commonly affected:

Lower midline.
Subcostal.
Lateral muscle splitting incisions.

C/F: Like any other hernia. May get obstructed and strangulated.
Treatment:
Smaller incisional hernias (< 3 cm.) can be repaired with primary tissue approximation. Repair of larger defects
generally requires the use of prosthetic materials, which allows for a tension free repair. Laparoscopic
techniques may also be used.
Operative:

Layer to layer repair - where defect is small to moderate without much tissue loss.
Keel repair (MAINGOT’S) – Scar is excised. The peritoneum and the layers are invaginated into the cavity
and successive sutures taken.
Mesh – Used especially with large defects and tissue loss.

Obturator hernia:nickname- “little old lady’s hernia”:

Obturator hernia proceeds through the obturator foramen situated at the anterolateral pelvic wall, interiorly to
the acetabulum. The obturator artery, vein and nerve pass through this tunnel protected by extraperitoneal
connective tissue and fat.
Typically obturator hernias occur in elderly women or patients with chronically raised intra-abdominal pressure
(e.g. ascites, COPD, chronic cough). It has been suggested that the female predominance of these hernias is the
result of pregnancy, which leads to relaxation of the pelvic peritoneum and a wider and more horizontal
obturator canal.
Female : Male - 6:1. Right : Left – 3:1.
The Howship–Romberg sign: Seen in 50%.Intermittent, acute, and severe hyperesthesia or pain in the medial
thigh or in the region of the greater trochanter, usually relieved by thigh flexion and worsened by medial
rotation, adduction, or extension at the hip.
The Hannington–Kiff sign, a clinical sign in which there is an absent adductor reflux in the thigh (more
specific).
Other symptoms include acute or intermittent small bowel obstruction with high risk of strangulation.
Abdominal X-ray may show nonspecific pattern of small-bowel obstruction, or occasionally intraluminal air
******ebook converter DEMO Watermarks*******
 bubbles in close proximity to the superior ramus of the pubic bone or gas shadows in the obturator foramen
area will be diagnostic.
Generally approached abdominally and often amenable to laparoscopic repair; mesh closure is necessary for a
tension-free repair.
Strangulated obturator hernias carry the highest mortality rate of all abdominal hernias.

Sciatic Hernia- Hernia through Sciatic foramina:

Sciatic hernia - Tender mass in the gluteal area that is increasing in size (Right Gluteal mass visible in 2nd
photo).
Sciatic neuropathy and symptoms of intestinal or ureteral obstruction can also occur.
Expanding, yet reducible, right gluteal mass, indicative of a sciatic hernia.
A transperitoneal approach is used in the event of incarceration; a transgluteal repair can be used if the
diagnosis is established and the intestine is clearly viable.

******ebook converter DEMO Watermarks*******

Lumber triangle and Hernias:
Major Lumber Triangle- Grynfeltt Hernia.
Minor Lumber Triangle- Petit Hernia.

******ebook converter DEMO Watermarks*******

******ebook converter DEMO Watermarks*******
Mediastinum:
Mediastinum - that part of the thorax contained between the two pleural cavities.
Superior compartment - above the aortic arch.

Thyroid / Aneurysms / Oesophagus / Neurogenic tumors.

Anterior - is bordered by the sternum anteriorly, the pericardium posteriorly and the mediastinal pleura laterally. (4
T’s).

Thymus / Thyroid/ Teratoma (and other germ cell tumours)/ Terrible Lymphoma and other lymphoid diseases
(sarcoid, Castlemann’s NSCLC, SCLC).
(also pericardial cyst and Morgagni’s hernia inferiorly).

Middle (visceral) compartment - from the anterior pericardium back to the pre-vertebral fascia and bounded by both
pleura, includes the heart, trachea, main bronchi and oesophagus.

Bronchogenic carcinoma / Lymphoma/ Aneurysms (and other vascular, including cardiac tumours)/
Bronchogenic cyst.

Posterior compartment - better referred to as the paravertebral sulci, includes those structures medial to the pleura
but excluding the vertebral column.

Neurogenic / Oesophageal (duplication and para-oesophageal hernia)/ Bone/ Aneurysms.

Presentation:

Mediastinal tumours in children are usually symptomatic with respiratory symptoms such as cough, stridor and
dyspnoes.
Malignant lesions are often accompanied by lethargy, fever, malaise and chest pain.
In adults many lesions are asymptomatic, found incidentally on routine chest radiographs.
However, obstructive symptoms do occur when the tumour impresses on the superior vena cava, oesophagus or
tracheo-bronchial tree and cardiac tamponade can be caused by large anterior compartment tumours.
Invasion of phrenic, recurrent laryngeal or sympathetic chain nerves may cause breathlessness, hoarseness or
Horner’s syndrome.

Diagnosis:

HRCT is the IOC for mediastenial masses.

CT scan or MRI scan outline the exact site of the lesion and will give clues to the diagnosis, a variegated
appearance suggesting teratoma.
Scans will also give an indication of malignant invasion of adjacent structures and pleural metastases which in
the case of thymoma produce a “droplet” pattern.
Fine needle aspiration cytology is frequently inadequate to differentiate thymoma from lymphoma and almost
never provides enough tissue to differentiate between types of lymphoma.
Thymictumours are associated with a number of paraneoplastic or “parathymic” syndromes. The rare
paraganglionic neurogenic tumours may be functional in that they produce biogenic amines resembling
phaeochromocytoma.
Vanillylmandelic acid or homovanillic acid may be detectable in the urine. Haematological markers of germ
******ebook converter DEMO Watermarks*******
 cell tumours (beta-HCG and alpha feto-protein) should be sought.

Neurogenic tumours of the mediastinum:

Neurogenic tumours account for 20-30% of all mediastinal tumours rising to 50-60% in children. Von
Recklinhausen’s neurofibromatosis is associated with an increased incidence of neurogenic tumours of all
histological types.
Most tumours are benign neurilemmomas (also known as schwannomas) or ganglioneuromas (classification) and are
completely cured by excision.
Treatment:

Benign – resection.
Complete resection also leads to high cure rates in the intermediate malignancy ganglioneuroblastoma and even
frankly malignant neuroblastoma and paraganglionoma.
Malignant schwannoma can rarely be excised completely and leads to death within a year of diagnosis:
incompletely excised paraganglionoma usually proves fatal regardless of adjuvant therapy though survival is
substantially longer.
Neuroblastoma is more sensitive to chemotherapy and when used in combination with radiotherapy even
incompletely excised tumours can achieve reasonable long-term survival.
Radiotherapy can also reduce local recurrence of incompletely excised neurilemmoma, neurofibroma and
ganglioneuroblastoma

Tumours of the thymus:

The thymus is a bilobed lymphoid organ sited in the superior mediastinum in adults. It is relatively large at birth and
enlarges to a maximal size during puberty. In adulthood, it regresses as its functional tissue is replaced by fatty
connective tissue.
The thymus is thought to have a central role in the development of immune function.
There are three general categories of thymictumours: thymoma, thymic carcinoma and tumours of other thymic
elements. Thymomas are one of the most common mediastinal neoplasms - 90% are benign.
Thymoma:

Thymomas are of particular fascination because of their unusual para-neoplastic associations.

The best known of these is myasthenia gravis but the number of syndromes associated with thymoma is
extensive (Parathymic syndromes).
The tumour contains both epithelial and lymphocytic elements which can also make differentiation from
lymphoma difficult.

Types:
Three main types are:

Benign - the most common, accounting for 80-90% of thymomas. Characterized by a diffuse proliferation of
neoplastic thymic epithelial cells and an abundance of lymphocytes. There is no capsular invasion.
Malignant type I - cytologic features are identical to the benign thymoma but with additional invasion of the
capsule. Occasionally, there may be metastases to the lungs and bone.
Malignant type II - known as thymic carcinoma. There is capsular invasion and cytologicpleomorphism. The
tumour often resembles a squamous cell carcinoma.

Classification:
Masoaka staging systemcategorisesthymoma purely on encapsulation and invasion of local tissues, and the Muller-
Hermelink morphological classification.
Clinical features:
******ebook converter DEMO Watermarks*******
 mean age of patients with thymomas is 50 years; rare in children where they are associated with a poor
prognosis.
males : females = 1:1.
radiographic mass - most common in anterosuperior mediastinum.
variable clinical presentation dependent upon the aggressiveness of the lesion. Basic patterns include:
asymptomatic.
features attributable to local pressure effects e.g. cough, dyspnoea, dysphagia and superior venal caval
obstruction.
associated systemic disorders.

Associated conditions:

myasthenia gravis - the most common association but less often associated with more aggressive thymomas.
haematologiccytopenias e.g. aplastic anaemia.
hypogammaglobulinaemia.
collagen vascular diseases e.g. systemic lupus erythematous.
non-thymic malignancies.

Di George syndrome:

The DiGeorge syndrome is an example of a selective T-cell deficiency caused by the failure of development of
the third and fourth pharyngeal pouches.
These pouches give rise to the following structures:
Thymus/ parathyroids/ aortic arch / portions of the lips and ears.
Consequently, DiGeorge syndrome may present with as immune deficiency state - usually T cells, but
sometimes B cells, and also aberrant calcium metabolism, congential heart disease and abnormal facies.

Nezelof syndrome:
Nezelof syndrome is congenital hypoplasia of the thymus with retention of normal parathyroid function.
It should be contrasted to DiGeorge syndrome in which there is absence of the parathyroids.
Thymic Carcinoma:
Thymic carcinoma is exceedingly rare and are of squamous histology and most have metastases at the time of
diagnosis and follows an aggressive course.
Treatment consists of chemotherapy and radiotherapy appropriate to the corresponding histological type.
Parathymic Conditions:

******ebook converter DEMO Watermarks*******

Dermoid (mediastinal):

Dermoid cysts arise from ectodermal tissue, whereas teratomas are germ cell tumours that contain ectodermal,
mesodermal and endodermal tissue.
These teratodermoidtumours occur in the mediastinum mainly in 20 to 30 year age group, most often in the
anterior mediastinum.
In general, dermoids are cystic and benign, whereas teratomas are solid and malignant.

Management:

thoracotomy may be considered for diagnosis and removal.

radiotherapy may may achieve long-term control in malignant teratoma.

Germ cell tumours of the mediastinum:

25% of anterior compartment mediastinal tumours are of germ cell origin and fall into three groups: benign
teratoma, malignant seminoma and nonseminomatous malignant germ cell tumours.
Teratomas are the most common mediastinal germ cell neoplasms and are located most commonly in the
anterosuperior mediastinum.
Treatment depends on histology.
For benign tumors of large size or with involvement of adjacent mediastinal structures such that complete
resection is impossible, partial resec- tion has led to resolution of symptoms, frequently without relapse. For
malignant teratomas, chemotherapy and radiation therapy, combined with surgical excision, are individualized
for the type of malignant components contained in the tumors. e overall prognosis is poor for malignant
teratomas.
They are due to primary tumours arising from residual germ cells which have migrated along the embryonic
urogenital ridge.
The malignant germ cell tumours, have a preponderance in males and are associated with chromosomal
abnormalities such as Klinefelter’s syndrome and other blood dyscrasias.
Benign teratomas are cured by complete surgical excision.
Seminomas are very radiosensitive and chemo-radiotherapy is the mainstay of treatment.
Bulky tumours may respond to induction therapy with cisplatin, bleomycin and etoposide before radiotherapy.
Non seminomatoustumours cisplatin based chemotherapy can produce complete remission in over 50% of
cases.
Surgery is indicated for residual disease in the mediastinum or for lung metastases if serum tumour markers
have reverted to normal.

******ebook converter DEMO Watermarks*******

Cardiac tumours:

Cardiac tumours are rare and most are benign.

Myxomas, 90% of which occur in the left atrium, are the commonest of the benign tumours.

Presentation:

Cardiac tumours present with syncopal attacks due to obstruction of flow within cardiac chambers, valve
incompetence due to impairment of valve closure, symptoms of embolisation, rarely arrythmia or constitutional
symptoms such as fever, weight loss, finger clubbing, Raynaud’s syndrome or myalgia.

Investigation:

Echocardiography outilines the intracardiacdisease.

Cardiac catheterisation is usually contraindicated because of increased risk of inducing embolisation,.
CT scan will outline the extent of extracardiac disease.
There is no non-invasive method of distinguishing between a benign from malignant cardiac lesion. Therefore
surgical exploration is required for any symptomatic or clinically suspicious intra-cardiac mass.

Treatment and prognosis:

Surgical resection by open heart surgery under cardio-pulmonary bypass is curative for the majority of atrial
myxomas and other benign tumours.
Malignant neoplasms are rarely cured with surgery alone though patients whose tumours have been resected
have a median survival of twenty-four months compared to eleven months for patients with
unresectabletumours.

Mediastinal Tumours:
Superior compartment - above the aortic arch.

Thyroid/ Aneurysms/ Oesophageal/ Neurogenic.

Anterior – is bordered by the sternum anteriorly, the pericardium posteriorly and the mediastinal pleura laterally. (4
T’s)

Thymus (Commonest).
Thyroid.
Teratoma (and other germ cell tumours).
Terrible Lymphoma and other lymphoid diseases (sarcoid, Castlemann’s NSCLC, SCLC).
Also pericardial cyst and Morgagni hernia inferiorly.

Middle (visceral) compartment - from the anterior pericardium back to the pre-vertebral fascia and bounded by both
pleura, includes the heart, trachea, main bronchi and oesophagus. (BLAB).

Bronchogenic carcinoma.
Lymphoma.
Aneurysms (and other vascular, including cardiac tumours).
Bronchogenic cyst.
Tracheal tumours.

Posterior compartment - better referred to as the paravertebral sulci, includes those structures medial to the pleura
but excluding the vertebral column. (NOBA)

Neurogenic.
Oesophageal (duplication and para-oesophageal hernia).
Bone.
******ebook converter DEMO Watermarks*******
 Aneurysms.

Commonest in anterior- Thymoma/ Middle- Pleuropericardiaal cyst/ Post- Neurogenic

Commonest in Children- Neurogenic.

******ebook converter DEMO Watermarks*******

Burns:

Depths:
First degree: injury localized to the epidermis.
Superficial second degree: injury to the epidermis and superficial dermis.
Deep second degree: injury through the epidermis and deep into the dermis.
Third degree: full-thickness injury through the epidermis and dermis into
subcutaneous fat.
Fourth degree: injury through the skin and subcutaneous fat into underlying
muscle or bone.

Burn Depth:

depth of burn varies by the degree of tissue damage.

Burn depth is classified into degree of injury in the epidermis, dermis, subcutaneous fat, and underlying
structures.

******ebook converter DEMO Watermarks*******

First-degree burns:

by definition, injuries confined to the epidermis.

painful and erythematous and blanch to the touch with an intact epidermal barrier.
Examples include sunburn or a minor scald from a kitchen accident. They do not result in scarring, treatment is
aimed at comfort with the use of topical soothing salves with or without aloe and oral nonsteroidal anti-
inflammatory agents.

Second-degree burns :

Are divided into two types: superficial and deep.

All second-degree burns have some degree of dermal damage, by definition, and the division is based on the
depth of injury into the dermis.
Superficial dermal burns are erythematous and painful, blanch to touch, and often blister.
Examples include scald injuries from overheated bathtub water and ash flame burns.
These wounds spontaneously re-epithelialize from retained epidermal structures in the rete ridges, hair follicles,
and sweat glands in 1 to 2 weeks.
After healing, these burns may have some slight skin discoloration in the long term.
Deep dermal burns into the reticular dermis :

1. appear more pale and mottled,

2. do not blanch to touch,
3. remain painful to pinprick.

These burns heal in 2 to 5 weeks by re-epithelialization from hair follicles and sweat gland keratinocytes, often
with severe scarring as a result of the loss of dermis.

Third-degree burns:

Are full thickness through the epidermis and dermis.

Characterized by a hard, leathery eschar that is painless and black, white, or cherry red.
No epidermal or dermal appendages remain.
These wounds must heal by re-epithelialization from the wound edges.
Deep dermal and full- thickness burns require excision with skin grafting from the patient to heal the wounds.

Fourth-degree burns:

involve other organs beneath the skin, such as muscle, bone, and brain.

New technologies, such as the multisensor laser Doppler flowmeter, hold promise for quantitative determination of
burn depth.
Zones of burn:

******ebook converter DEMO Watermarks*******

 area of cutaneous or superficial injury has been divided into three zones:
zone of coagulation, zone of stasis, and zone of hyperemia.
zone of coagulation :
the necrotic area of burn where cells have been disrupted and irreversibly damaged at the time of injury.

zone of stasis:

area immediately surrounding the necrotic zone has a moderate degree of insult with decreased tissue
perfusion.
depending on the wound environment, can either survive or go on to coagulative necrosis.
zone of stasis is associated with vascular damage and vessel leakage.
Thromboxane A2, , is present in high concentrations in the zone of stasis.
Treatment directed at the control of local inflammation immediately after injury may spare the zone of stasis.

zone of hyperemia:

characterized by vasodilation from inflammation surrounding the burn wound.

contains the clearly viable tissue from which the healing process begins and is generally not at risk for further
necrosis.

Assessment of burn depth:

Burn size is generally assessed by the “rule of nines”

PALM RULE:Another method of estimating smaller burns is to equate the area of the open hand (including the
palm and the extended fingers) of the patient to be approximately 1% TBSA and then to transpose that
measurement visually onto the wound for a determination of its size. This is method is crucial in evaluating
burns of mixed distribution.
Children have a relatively larger portion of the body surface area in the head and neck (approx. 18 %), which is
compensated for by a relatively smaller surface area in the lower extremities.
Infants have 21% of the TBSA in the head and neck and 13% in each leg.
Berkow formula is used to accurately determine burn size in children.
Lund and Broader chart is most specific for adults.

******ebook converter DEMO Watermarks*******

Systemic effects of burns:

Metabolic changes post burn:

(important):

Postburn metabolic phenomena occur in a timely manner, suggesting two distinct patterns of metabolic
regulation after injury.
EBB Phase:
First phase occurs within the 1st 48 hours of injury and has classically been called the ebb phase,
characterized decreases in cardiac output, oxygen consumption, and metabolic rate as well as impaired glucose
tolerance associated with its hyperglycemic state.
FLOW /PLATEAU Phase:
******ebook converter DEMO Watermarks*******
 next phase of metabolic changes is known as FLOW Phase.
The metabolic variables gradually increase within the first 5 days after injury to a plateau phase ,
associated with hyperdynamic circulation and the hypermetabolic state.
hypermetabolic response to burn injury may last for more than 12 months after the initial event.
hypermetabolic alterations after a burn, indicated by persistent elevations of total urine cortisol levels, serum
cytokines, catecholamines, and basal energy requirements, were accompanied by impaired glucose metabolism
and insulin sensitivity that persisted for up to 3 years after the initial burn injury.

Effects on the Gastrointestinal System

Mucosal atrophy, changes in digestive absorption, and increased intestinal permeability.

Atrophy of the small bowel mucosa occurs within 12 hours of injury in proportion to the burn size and is
related to increased epithelial cell death by apoptosis.
Stress gastritis associated with burn is more common in the PROXIMAL STOMACH, which is due to the
ischemia mediated injury. These ulcers are known as “CURLING ULCERS”

Management of the burn patients:

Hospital care:
The principles of managing an acute burn injury are the same as in any acute trauma case:

A, Airway control.
B, Breathing and ventilation.
C, Circulation.
D, Disability – neurological status.
E, Exposure with environmental control.
F, Fluid resuscitation.

The Criteria for acute admission to a burns unit:

Suspected airway or inhalational injury.

Any burn likely to require fluid resuscitation.
Any burn likely to require surgery.
Patients with burns of any significance to the hands, face, feet or perineum.
Patients whose psychiatric or social background makes it inadvisable to send them home.
Any suspicion of non-accidental injury.
Any burn in a patient at the extremes of age.
Any burn with associated potentially serious sequelae, including high-tension electrical burns and concentrated
hydrofluoric acid burns.

Airway:

Because burns can result in massive edema, the upper airway is at risk for obstruction.
Signs of obstruction may initially be subtle until the patient is in crisis; therefore, early evaluation of the need
for endotracheal intubation is essential.
Factors that increase the risk for upper airway obstruction are:
increasing burn size and depth, burns to the head and face.
inhalation injury, and burns inside the mouth.
Burns localized to the face and mouth(cause more localized edema and pose a greater risk for airway
compromise).
Children: Because their airways are smaller, children are at higher risk for airway problems.

******ebook converter DEMO Watermarks*******

Stop the Burning Process:

All clothing should be removed to stop the burning process.

Any clothing that was burned by chemicals should be removed carefully.
The patient then should be covered with warm, clean, dry linens to prevent hypothermia.
after primary survey and initial care of the patient, the next step is the calculation of TBSA and Resuscitation of
the patient.

Any patient with burns over more than 20% of the body surface requires fluid resuscitation.

Venous access is best attained through short peripheral catheters in unburned skin.
In children younger than 6 years, experienced practitioners can use intraosseous access in the proximal tibia
until intravenous access is accomplished.
Lactated Ringer solution without dextrose is the fluid of choice except in children younger than 2 years, who
should receive 5% dextrose in lactated Ringer solution.
initial rate can be rapidly estimated by multiplying the TBSA burned by the patient’s weight in
kilograms and then dividing by 8.

Thus, the rate of infusion for an 80-kg man with a 40% TBSA burn would be
80kg × 40%TBSA 8 = 400mL hr .
PARKLAND’s Formula:
total percentage body surface area × weight (kg) × 4 = volume (mL).
Half this volume is given in the first 8 hours and the second half is given in the subsequent 16 hours.

Colloid solutions should not be used in the 1st 24 hours until capillary permeability returned closer to normal.
Hypertonic saline(HAS: Human Albumin Solution) solutions have theoretical advantages in burn resuscitation.
these solutions decrease net fluid intake, decrease edema, and increase lymph flow, probably by the transfer of
volume from the intracellular space to the interstitium. When these solutions are used, hypernatremia must be
avoided, and it is recommended that serum sodium concentrations not exceed 160 mEq/dL.
Resuscitation is easily monitored in burned patients with normal renal function by following the volume of
urine output, which should be at 0.5 mL/hr in adults and 1.0 mL/ kg/hr in children.
Changes in intravenous fluid infusion rates should be made on an hourly basis determined by the response of
the patient to the particular fluid volume administered.
For pediatric burn;Galveston formula uses 5000 mL/TBSA + 2000ml/m^2 (in 1st 24 hrs) for maintenance, it is
given in the chart.

Escharotomies:

When deep second- and third-degree burn wounds encompass the circumference of an extremity, peripheral
circulation to the limb can be compromised.
Development of generalized edema beneath a non yielding eschar impedes venous outflow and eventually
affects arterial in flow to the distal beds.
******ebook converter DEMO Watermarks*******
 This is can be recognized by numbness and tingling in the limb and increased pain in the digits.
Extremities at risk are identifed either on clinical examination or on measurement of tissue pressures higher
than 40 mm Hg.

Options for topical treatment of deep burns.

1% silver sulphadiazine cream.

0.5% silver nitrate solution.
Mafenide acetate cream.
Serum nitrate, silver sulphadiazine and cerium nitrate.
The simplest method of treating a superficial wound is by exposure. The initial exudate needs to be managed by
frequent changes of clean linen around the patient but, after a few days, a dry eschar forms, which then
separates as the wound epithelialises.
A similar method of managing these types of burn is to place a Vaseline-impregnated gauze (with or without an
antiseptic, such as chlorhexidine) over the wound. (recent question asked in AIIMS).
Early debridement and grafting is the key to effectively treat- ing deep partial- and full-thickness burns in a
majority of cases.

Inhalation Injury:

Toxic gases ; smoke mediated injury to respiratory tract.

COHb.

******ebook converter DEMO Watermarks*******

 Early diagnosis of bronchopulmonary injury is thus critical for survival.
history of closed- space exposure, facial burns, and carbonaceous debris in mouth, pharynx, or sputum.
standard diagnostic method is bronchoscopy of the upper airway of every burn patient.
Gamelli and others established a grading system of inhalation injury (0 to 4) derived from findings at initial
bronchoscopy and based on Abbreviated Injury Score criteria.
Bronchoscopic criteria that are consistent with inhalation injury included.
airway edema.
inflamation.
mucosal necrosis.
presence of soot and charring in the airway.
tissue sloughing.
carbonaceous material in the airway.
treatment of inhalation injury should start immediately with the administration of 100% oxygen by face mask
or nasal cannula.
Maintenance of the airway is critical.
As mentioned before, if early evidence of upper airway edema is present, early intubation is required because
the upper airway edema normally increases during 9 to 12 hours.
Prophylactic intubation without good indi- cation, however, should not be performed.
Management of inhalation injury consists of ventilatory support, aggressive pulmonary toilet
;bronchoscopicremoval of casts, and nebulization therapy.
Nebulization therapy can consist of heparin, alpha mimetics, or polymyxin B and is applied between two and
six times a day.
Prophylactic antibiotics are not indicated but are imperative with documented lung infections.

Electrical Burns:

Low-voltage injuries cause small, localised, deep burns.

They can cause cardiac arrest through pacing interruption without significant direct myocardial damage.
High-voltage injuries damage by flash (external burn) and conduction (internal burn).
Myocardium may be directly damaged without pacing interruption.
Limbs may need fasciotomies or amputation.
Look for and treat acidosis and myoglobinuria.
3% to 5% of all admitted burned patients are injured from electrical contact.
lowest resistance to current is associated with the nerves, blood vessels, and muscles.
skin has a relatively high resistance to electrical current and is therefore mostly spared.
The muscle is the major tissue through which the current flows, and thus it sustains the most damage.
Injuries are divided into high- and low-voltage injuries.
Low- voltage injury is similar to thermal burns without transmission to the deeper tissues; zones of injury from
the surface extend into the tissue. Most household currents (110 to 220 V) produce this type of injury, which
causes only local damage.
high-voltage injury consists of varying degrees of cutaneous burn at the entry and exit sites combined with
hidden destruction of deep tissue.
Initial evaluation consists of cardiopulmonary resuscitation if ventricular brillation is induced.
Ventricular fibrillation is the MCC of death.
most serious derangements occur in the 1st 24 hours after injury.
If patients with electrical injuries have no cardiac dysrhythmias on initial electro- cardiogram or recent history
of cardiac arrest, no further monitoring is necessary.
The most signicant injury is within the deep tissue, and subsequent edema formation can cause vas-cular
compromise to any area distal to the injury.
Assessment should include circulation to distal vascular beds because immedi- ate escharotomy and fasciotomy
may be required.
Muscle damage results in release of hemochromogens (myoglobin), which are filltered in the glomeruli and
may result in obstructive nephropathy. therefore, vigorous hydration and infusion of intravenous sodium
bicarbonate (5% continuous infusion) and mannitol (25 g every 6 hours for adults) are indicated to solubilize
the hemochromogens and to maintain urine output if signicant amounts are found in the serum.

******ebook converter DEMO Watermarks*******

Chemical Burns:

Cold Injuries:

Cold injuries are principally divided into two types: acute cold injuries from industrial accidents and frostbite.
The tissue is more resistant to cold injury than to heat injury, and the inflammatory reaction is not as marked.
The assessment of depth of injury is more difficult, so it is rare to make the decision for surgery early.
Frostbite injuries affect the peripheries in cold climates.
The initial treatment is with rapid rewarming in a bath at 42°C.
The cold injury produces delayed microvascular damage similar to that of cardiac reperfusion injury. The level
of damage is difficult to assess, and surgery usually does not play a role in its management, which is
conservative, until there is absolute demarcation of the level of injury.

Trauma:
Triage:
Triage is the process of prioritizing patient treatment during mass-casualty events.
Principles of Triage:
Do the Most Good for the Most Patients Using Available Resources
the principles of triage are applied when the number of casualties exceeds the medical capabilities that are
immediately available to provide usual and customary care.
Determine Triage Category Types:

Red implies life-threatening injury that requires immediate intervention and/or operation.
Yellow implies injuries that may become life- or limb-threatening if care is delayed beyond several hours.
Green patients are the walking wounded who have suffered only minor injuries. These patients can sometimes
be used to assist with their own care and the care of others.
Black is frequently used to mark dead patients.
Many systems add another color, such as blue, for “expectant” patients—those who are so severely injured that,
given the current number of casualties requiring care, the decision is made to simply give palliative treatment
while first caring for red (and perhaps some yellow) patients. Patients who are classified as expectant because
of the severity of their injuries would typically be the first priority in situations in which there are only two or

******ebook converter DEMO Watermarks*******

 three casualties requir- ing immediate care. However, the rules, protocols, and standards of care change in the
face of a mass-casualty event. Remember: “Do the most good for the most pa- tients using available resources.”

Primary Survey and Resuscitation:

The initial management of the traumatised patient must first consist of a rapid primary evaluation and
resuscitation of vital functions as soon as abnormalities are detected.
Only when the patient has been stabilised and the team are content with the pri- mary survey is a more detailed
secondary assessment carried out.
The primary survey comprises the fundamental principles of the ATLS system, the ‘ABCDE’ of trauma care.

ABCDE of emergency management:

A = Airway and cervical spine control – Ensure a clear airway. The mouth and upper airway should be
inspected for foreign bodies: these should be removed. In an unconscious patient the initial airway management
may be a simple chin lift or jaw thrust; if this is unsuccessful in maintaining an airway then an oral (Guedel) or
nasopharyngeal airway can be used. If these fail to maintain the airway then intubation will be necessary. In
patients with severe maxillofacial trauma a surgical airway such as jet insufflation (needle cricothyroidotomy)
or surgical cricothvroidotomv may be needed. Emergency tracheostomy has no role as an emergency airway
manoeuvre.
B = Breathing – Check for chest movements, asymmetry of movements, respiratory rate, abrasions or bruising
over the chest, cyanosis, use of accessory muscles, distension of neck veins. Examine the chest for pain,
crepitations (indicating subcutaneous emphysema), auscultation, percussion and palpation of the trachea.
Needle decompression may be needed for tension pneumothorax and a chest drain may be required for
pneumothorax or hacniothorax.
C = Circulation and haemorrhage control – i.v. access should be gained with two large bore cannula (12-14G)
in the antecubital fossa. Two litres (L) of Hurtmann's solution should be rapidly infused. Alternative sites for
vascular access include central veins, i.e. subclavian or femoral (internal jugular can be difficult to use due to
the presence of C-spine collars), cut-down onto the long saphenous vein and intraosseous infusion (children
only). Obvious haemorrhage can be treated with compression dressings. Tourniquets are not indicated.
D = Disability – In the primary survey a rapid assessment of neurological status is made. This includes
assessment of pupillary size and level of consciousness. The level of consciousness can be remembered by the
mnemonic AVPU:
A = Alert.
V = responds to Vocal stimuli.
P = responds to Painful stimuli.
U = Unresponsive.
E = Exposure and environmental control – The patient should be fully undressed and examined from head to
toe (secondary survey). The patient's temperature must be monitored and hypothermia prevented by covering
with warming blankets and the use of Harmed i.v. fluids.

Adjuncts to the primary survey (recent AIIMS question):

Blood tests – full blood count, urea and electrolytes, clotting screen, glucose, toxicology, cross-match.
ECG, pulse oximetry, arterial blood gas (ABG).
Two wide-bore cannulae for intravenous fluids.
Urinary and gastric catheters.
Radiographs of the cervical spine, chest and pelvis.

Secondary survey:

The secondary survey is a head-to-toe evaluation of the trauma patient, i.e. a complete history and physical
examination, including a reassessment of all vital signs.
Each area of the body should be completely examined.
A full neurological examination is carried out including a GCS (Glasgow Coma Score) determination.

******ebook converter DEMO Watermarks*******

A mnemonic to help remember this is to take an AMPLE history:

Allergies.
Medications.
Previous illnesses.
Last meal.
Events surrounding injury.

Trauma scores:

******ebook converter DEMO Watermarks*******

AIS is a anatomy based trauma score.
Thoracic Trauma:

Thoracic injury accounts for 25 per cent of all severe injuries.

In most of these patients, the cause of death is haemorrhage.
About 80 per cent of patients with chest injury can be managed non- operatively.
The key to a good outcome is early physiological resuscitation followed by a correct diagnosis.
Life-threatening injuries can be remembered as the ‘deadly dozen’.
Six are immediately life-threatening and should be sought during the primary survey.
six are potentially life- threatening and should be detected during the secondary survey.

Investigation:

A chest radiograph is the investigation of first choice.

A spiral computed tomography scan provides rapid diagnoses in the chest and abdomen.
Ultrasound can be used to differentiate between contusion and the actual presence of blood.
A chest tube can be a diagnostic procedure, as well as a therapeutic one, and the benefits of insertion often
outweigh the risks.

The pitfalls of investigation are:

failure to auscultate both front and back (an inflated lung will ‘float’ on a haemothorax, so auscultation from
the front may sound normal).
failure to pass a nasogastric tube if rupture of the diaphragm is suspected.
******ebook converter DEMO Watermarks*******
 pursuing radiological investigation (radiography or CT scan) instead of resuscitation in the unstable patient.

Computed tomography scan:

The CT scan has become the principal and most reliable examination for major injury in thoracic trauma.
Scanning with contrast allows for three-dimensional reconstruction of the chest and abdomen, as well as of the
bony skeleton.
In blunt chest trauma, the CT scan will allow the definition of fractures, as well as showing haematomas,
pneumothoraces and pulmonary contusion.
In penetrating trauma, the scan may show the track or presence of the missile and allow the proper planning of
definitive surgery.
CT scanning has replaced angiography as the diagnostic modality of choice for the assessment of the thoracic
aorta.

Management:

Most patients who have suffered penetrating injury to the chest can be managed with appropriate resuscitation
and drainage of haematoma.
If a sucking chest wound (OPEN PNEUMOTHORAX) is present, this should not be fully closed but should be
covered with a piece of plastic, closed on three sides, to form a one-way valve, and thereafter an under- water
chest drain should be inserted remote from the wound.
No attempt should be made to close a sucking chest wound until controlled drainage has been achieved, in case
a stable open pneumothorax is converted into an unstable tension pneumothorax.
In blunt injury, most bleeding occurs from the intercostal or internal mammary vessels and it is relatively rare
for these to require surgery.
If bleeding does not stop spontaneously, the vessels can be tied off or encircled.
In blunt chest compressive injury, there is injury to the ribs and frequently to the underlying structures as well,
with an associated lung contusion.

Life Threatening Injuries

Tension pneumothorax:

Injury results in the formation of a ‘one-way’ valve - air enters the pleural cavity but is unable to escape,
therefore pressure (or tension) in the chest rises, causing collapse of the ipsilateral lung; compression over the
heart, distortion of the vena cava and tracheal deviation to opposite side.

Symptoms and signs:

Tachypnoea.
use of accessory muscles.
cyanosis.
hypotension (due to kinking of vena cava and decreased venous return),(this is a hallmark feature).
deviated trachea (away from the affected side).
distended neck veins (variable sign).
hyper-resonant percussion and absent breath sounds.

Management:

This is a clinical diagnosis.

Needlelethoracostomy.
Immediate management is the placement ofa wide bore cannula in the 2nd intercostal space in the mid-
clavicular line.
Tube Thoracostomy: A chest drain must then replace this as the tension pneumothorax has been converted to a
simple pneumothorax. This is the definite management.

******ebook converter DEMO Watermarks*******

Open pneumothorax:

Also known as Sucking chest wound.

Produced by injuries that cause large defects of the chest wall, i.e. gunshot wound.
The air is sucked in to the lungs via defect. Hence, known as sucking chest wound.
The injury leads to equalization of intrathoracic and atmospheric pressure.

Symptoms and signs:

Tachypnoea, use of accessory muscles, cyanosis, obvious chest wound.
Management:

Immediate management is the placement of a dressing secured on three sides to create a ‘flutter-valve’
(securing on four sides will produce a tension pneumothorax).
Definitive treatment requires closure of the chest wall defect and tube thoracostomy remote from the wound.

Haemothorax and massive haemothorax:

Most often due to penetrating injury to the hilar or systemic vessels.

Can occur with blunt injury with rib fractures and damage to intercostals vessels.
Massive haemothorax is defined as immediate evacuation of 1.5 L at insertion of a chest drain or >200 mL
every hour after drain insertion.
It produces hypoxia by the pressure effect of the additional volume in the thorax compressing the lung but also
by hypovolaemia.
Symptoms and signs.
Signs of shock, tachypnoea, using accessory muscles, cyanosis, dull percussion note and absent breath sounds.

Management:
for Hemothorax : tube thoracostomy.
for Massive Hemothorax: thoracotomy followed by ligation of the bleeding vessels.
However the chest drain should be clamped before proceeding for surgery as it allows rexpansion of the lungs and
also facilitates tamponade effect over the bleeding vessels.
Flail chest:
The diagnosis is made clinically, not by radiography. On inspiration, the loose segment of the chest wall is displaced
inwards and therefore less air moves into the lungs. To confirm the diagnosis, the chest wall can be observed for
paradoxical motion of a chest wall segment dur- ing respiration and during coughing. Voluntary splinting of the
chest wall occurs as a result of pain, so mechanically impaired chest wall movement and the associated lung
contusion all contribute to the hypoxia. There is a high risk of developing a pneumothorax or haemothorax.
Traditionally, mechanical ventilation was used to ‘internally splint’ the chest, but had a price in terms of intensive
care unit resources and ventilation-dependent morbidity.

Occurs when more than two adjacent ribs are fractured in two or more places.
The blunt force required to disrupt the integrity of the thoracic cage typically produces an underlying
pulmonary contusion as well
This results in a segment of the chest moving paradoxically with respiration (in with inspiration and out with
expiration). Contrary to belief, it is not the paradoxical chest movement that causes respiratory problems but
the lung contusion underlying the rib injury.

Symptoms and signs:

Pain, bruising, tachypnoea, paradoxical respiratory movement (often not present acutely due to muscle
splinting).

******ebook converter DEMO Watermarks*******

Management:

Analgesia and oxygen administration is the first line management.

Currently, treatment consists of oxygen administration, adequate analgesia (including opiates) and
physiotherapy.
If a chest tube is in situ, intrapleural local analgesia can be used as well.
Ventilation is reserved for cases developing respiratory failure despite adequate analgesia and oxygen.
Surgery to stabilise the flail chest may be useful in a selected group of patients with isolated or severe chest
injury and pulmonary contusion.

Cardiac Tamponade:
Usually occurs as a result of penetrating trauma, blood within the pericardium compresses the heart leading to
cardiogenic shock.
Symptoms and signs:

Becks triad (distended neck veins, hypotension, muffied heart sounds),

pulsusparadoxus,
Kussmaul’ssign ,
Small complexes on ECG, EMD
Because the pericardium is not acutely distensible, the pressure in the pericardial sac will rise to match that of
the injured chamber.
When this pressure exceeds that of the right atrium, right atrial filling is impaired and right ventricular preload
is reduced. This ultimately leads to decreased right ventricular output.
Additionally, increased intrapericardial pressure impedes myocardial blood flow, which leads to subendocardial
ischemia and a further reduction in cardiac output.

Management:

Diagnosis of hemopericardium is best achieved by bedside ultrasound of the pericardium

In patients with any hemodynamic disturbance, a pericardial drain is placed using ultrasound guidance
Removing as little as 15 to 20 mL of blood will often temporarily stabilize the patient’s hemodynamic status,
and alleviate subendocardial ischemia with associated lethal arrhythmias, and allow safe transport to the OR for
sternotomy.
Emergency management:usg guided Pericardiocentesis is successful in decompressing tamponade in
approximately 80% of cases;

Needle pericardiocentesis:

Surgically prepare the chest and infiltrate local anaesthetic, then insert a 16-18-gauge needle 2 cm below the
xiphisternum and advanced upwards towards the tip of the left scapula.
The procedure should be performed with ECG monitoring.
If the needle is advanced too far then there will be ECG changes such as ST elevation, etc.
Once all the blood has been aspirated then a catheter with a 3-way tap can be left in case of reaccumulation.

Aortic disruption (traumatic rupture of the aorta - TRA):

This is due to deceleration injuries such as in RTAs or a fall from a great height.
The commonest point of deceleration injury in the aorta is in the ascending aorta just proximal to the
innominate artery and at the point of attachment of the ligamentumarteriosum.
Tears in the ascending aorta often have associated cardiac damage and rarely reach hospital.
tears at the ligamentumarteriosum may be contained by adventitia and allow the patient to reach hospital
(typically young males).

Symptoms and signs:

******ebook converter DEMO Watermarks*******
 The patient may have no signs or be moribund with signsof massive haemothorax.
Other signs include upper extremity icreasedBP with diminished pulses in the lower limbs, diminished pulses
in the upper limbs (due to occlusion of vessels along the aortic arch) and neurological compromise from spinal
ischaemia.

Investigations:

CXR(1. signs include widened mediastinum, 2. fractured 1st or 2nd rib, 3. obliterated aortic knuckle, 4. pleural
cap- small amount of blood in the pleural cavity, 5. deviated trachea to the right, 6. elevation of right bronchus
and right deviation of NG tube).

Arch aortogram:
The diagnosis is confirmed by a CT scan of the mediastinum or possibly by transoesophageal echocardiography.
Management :

Initially, management consists of control of the systolic arterial blood pressure (to less than 100 mmHg).

Thereafter, an endovascular intra-aortic stent can be placed or the tear can be operatively repaired by direct repair or
excision and grafting using a Dacron graft.
Surgical repair with resection of damaged segment and replacing with a graft, endovascular repair.
Tracheobronchial injuries:

Severe subcutaneous emphysema with respiratory compromise can suggest tracheobronchial disruption.
A chest drain placed on the affected side will reveal a large air leak and the collapsed lung may fail to re-
expand.
If after insertion of a second drain the lung fails to re-expand, referral to a trauma centre is advised.
Bronchoscopy is diagnostic.
Treatment involves intubation of the unaffected bronchus followed by operative repair.

Diaphragmatic injuries:

Any penetrating injury below the fifth intercostal space should raise suspicion of diaphragmatic penetration
and, therefore, injury to the abdominal contents.
Blunt injury to the diaphragm is usually caused by a compressive force applied to the pelvis and abdomen.
The diaphragmatic rupture is usually large, with herniation of the abdominal contents into the chest.
Diagnosis of blunt diaphragmatic rupture is missed even more often than penetrating injuries in the acute phase.
Most diaphragmatic injuries are silent and the presenting features are those of injury to the surrounding organs.
Diagnostic laparoscopy is the standard of investigation.
All diaphragmatic injuries are to be surgically repairedby ABDOMINAL approach.

******ebook converter DEMO Watermarks*******

Emergency Thoracotomy:
Indications for thoracotomy include the need to perform:

internal cardiac massage.

control of haemorrhage from injury to the heart or lung.
control of intrathoracic haemorrhage from other causes.
control of massive air leak.

Indications for Thoracotmy in Thoracic Injuries:

Indications for Emergency Room Thoracotomy:

******ebook converter DEMO Watermarks*******

Abdominal Trauma
Abdominal Injury:
Patients who have suffered abdominal trauma can generally be classified into the following categories based on their
physiologi-cal condition after initial resuscitation:
1. Haemodynamically ‘normal’: investigation can be completed before treatment is planned.
2. Haemodynamically ‘stable’: investigation is more limited.
It is aimed at establishing whether the patient can be managed non-operatively, whether angioembolisation can be
used, or whether surgery is required.
3. Haemodynamically ‘unstable’: investigations need to be suspended as immediate surgical correction of the
bleeding is required.
Investigation:

Investigations are driven by the cardiovascular status of the patient.

In torso trauma, the best and most sensitive modality is a CT scan with intravenous contrast;
USG/FAST is usually the 1st line investigation for abdominal trauma.

Focused abdominal sonography for trauma:

FAST is IOC for unstable BLUNT Trauma Abdomen:

Focused abdominal sonography for trauma (FAST) is a technique whereby ultrasound (sonar) imaging is used
to assess the torso for the presence of free blood, either in the abdominal cavity or in the pericardium.
The technique therefore focuses on 4 areas:

1. Sub-xiphoid space (to look for cardiac tamponade).

2. Hepato Renal Pouch (to look for collection).

******ebook converter DEMO Watermarks*******

 3. Spleno Renal Pouch (to look forcollection).
4. Pouch of douglas (to look for collection).

There should be no attempt to determine the nature or extent of the specific injury.
FAST is usually a rapid, reproducible, portable and non-invasive bedside test, and can be performed at the
same time as resuscitation.
FAST is accurate at detecting >150 mL of free blood.
however, it is very operator- and experience-dependent and, especially if the patient is very obese or the bowel
is full of gas, it may be unreliable.
Hollow viscus injury is difficult to diagnose, even in experienced hands, and has a low sensitivity (29–35 %)
for organ injury without haemoperitoneum.
FAST is also unreliable for excluding injury in penetrating trauma.
If there is doubt, the FAST examination should be repeated.
sub-xiphoid space is the 1st examined window.

E-FAST: Extensions of the FAST technique to include assessment of the chest for haemothorax and pneumothorax,
as well as assessment of the extremities.

Diagnostic peritoneal lavage:

Diagnostic peritoneal lavage (DPL) is a test used to assess the presence of blood in the abdomen.
A gastric tube is placed to empty the stomach and a urinary catheter is inserted to drain the bladder.
A cannula is inserted below the umbilicus, directed caudally and posteriorly.
The cannula is aspirated for blood (>10 mL is deemed as positive) and, following this, 1000 mL of warmed
Ringer’s lactate solution is allowed to run into the abdomen and is then drained out.
The presence of >100 000 red cells/μL or >500 white cells/μL is deemed positive (this is equivalent to 20 mL
of free blood in the abdominal cavity).
In the absence of laboratory facilities, a urine dipstick may be useful.
Drainage of lavage fluid via a chest drain indicates penetration of the diaphragm.

******ebook converter DEMO Watermarks*******

Although DPL has largely been replaced by FAST , it remains the standard in many institutions where FAST is not
available or is unreliable.
DPL is especially useful in the hypotensive, unstable patient with multiple injuries as a means of excluding intra-
abdominal bleeding.
Computed tomography:

CT has become the ‘gold standard’ for the intra-abdominal diagnosis of injury in the stable patient.
The scan should be performed using intravenous contrast.
CT is sensitive for blood, and individual organ injury, as well as for retroperitoneal injury.
An entirely normal abdominal CT is usually sufficient to exclude injury.

The following points are important when performing CT:

Despite its tremendous value, it remains an inappropriate investigation for unstable patients.
If duodenal injury is suspected from the mechanism of injury, oral contrast may be helpful.
If rectal and distal colonic injury is suspected in the absence of blood on rectal examination, rectal contrast may
be helpful.

Diagnostic laparoscopy:

Diagnostic laparoscopy (DL) or thoracoscopy may be a valuable screening investigation in stable patients with
penetrating trauma, to detect or exclude peritoneal penetration and/or diaphragmatic injury.
In most institutions, evidence of penetration requires a laparotomy to evaluate organ injury, as it is difficult to
exclude all intra-abdominal injuries laparoscopically.
When used in this role DL reduces the non-therapeutic laparotomy rate.
DL is not a substitute for open laparotomy, especially in the presence of haemoperitoneum or contamination.

Individual Organ Injury:

Liver:

The liver is usually compressed between the impacting object and the rib cage or vertebral column.
Liver is the MC organ injury in penetrating trauma(earlier small bowel was considered to be the mc site)
In the stable patient, CT is the investigation of choice. It provides information on the liver injury itself, as well
as on injuries to the adjoining major vascular and biliary structures.

Liver injury can be graded and managed using the American Association for the Surgery of Trauma (AAST) Organ
Injury Scale (OIS)

******ebook converter DEMO Watermarks*******

Management:
The operative management of liver injuries can be summarised as ‘the four Ps’:
1. push.
2. Pringle.
3. plug.
4. pack.

At laparotomy, the liver is reconstituted as best as possible in its normal position and bleeding is controlled by
direct compression (push).
The inflow from the portal triad is controlled by a Pringle’s manoeuvre, with direct compression of the portal
triad, either digitally or using a soft clamp.
This has the effect of reducing arterial and portal venous inflow into the liver, although it does not control the
backflow from the inferior vena cava and hepatic veins.

Any holes due to penetrating injury can be plugged directly and, after controlling any arterial bleeding, the liver
can then be packed.
Bleeding points should be controlled locally when possible and such patients should subsequently undergo
angioembolisation.
It is not usually necessary to suture penetrating injuries of the liver.
If there has been direct damage to the hepatic artery, it can be tied off.
******ebook converter DEMO Watermarks*******
 Damage to the portal vein must be repaired,as tying off the portal vein carries a greater than 50 per cent
mortality rate. If it is not technically feasible to repair the vein at the time of surgery, it should be shunted and
the patient referred to a specialist centre.
Penetrating injuries and deep tracts can be plugged using silicone tubing or a Sengstaken–Blakemore tube.
Placing omentum into cracks in the liver is not recommended.

Spleen:

spleen is the most commonly injured intra-abdominal organ followed by the liver and small bowel in blunt
trauma patients.
Clinically, patients with splenic injury may present with hypotension, left upper quadrant pain, or tenderness to
palpation or diffuse peritonitis from extravasated blood.
Referred pain to the left shoulder on deep inspiration, in face of splenic hematoma, is known as Kehr’s sign.

Management:

Most series indicate that approximately 60–80% of patients presenting with blunt splenic injury can be
managed nonoperatively at level I or II trauma centers.
Patients selected for nonoperative management must have:

1. stable vital signs.

2. be free of peritoneal signs or other concern for hollow viscus injury.
3. have no evidence of free extravasation of IV contrast from the splenic parenchyma.
Indications of failure of nonoperative management:
1. Higher splenic injury grade.
2. age greater than 55 years.
3. moderate to large hemoperitoneum.
4. subcapsular hematoma.
5. portal hypertension.

Presence of a contained contrast blush within the parenchyma of the spleen represents pseudoaneurysm
formation of a branch of the splenic artery.
Angioembolization is now commonly used to selectively occlude the arterial branches containing these injuries.

Splenectomy:

Patients requiring urgent or emergent intervention for splenic hemorrhage may develop hypothermia,
coagulopathy, and visceral edema.
the most expeditious and safest course of action under these conditions is removal of the spleen.

Splenorrhaphy:

Hemodynamically stable patients found to have small to moderate amounts of parenchymal hemorrhage at
laparotomy may be candidates for splenic repair.
Approximately 50% of the spleen is required to preserve adequate phagocytic and immunologic function. If
this cannot be achieved, a splenectomy is probably the best option.

Overwhelming Postsplenectomy Infection:

Increased incidence of infection by capsulated bacterias such as

H. influenza; Pneumococcus; Meningococcus.

Adults following trauma is felt to be lower than the incidence seen after splenectomy for hematological
******ebook converter DEMO Watermarks*******
 disorders such as idiopathic thrombocytopenic purpura (ITP), lymphoma, and thalassemia.
Currently, anyone older than 2 years should receive the 23 valent pneumococcal vaccine and a one-time dose of
the Haemophilusinfluenzae and meningococcal vaccine.
A one-time booster dose of the pneumococcal vaccine is recommended 5 years after the original vaccine.

Pancreas:

Most pancreatic injury occurs as a result of blunt trauma.

The major problem is that of diagnosis, because the pancreas is retroperitoneal organ.
CT remains the mainstay of accurate diagnosis.
Amylase estimation is only relatively sensitive.
Classically, the pancreas should be treated with conservative surgery and closed suction drainage.
Injuries to the tail are treated by closed suction drainage, with distal pancreatectomy if the duct is involved.
Proximal injuries (to the right of the superior mesenteric artery) are treated as conservatively as possible,
although partial pancreatectomy may be necessary.
The pylorus can be temporarily closed (pyloric exclusion) in association with a gastric drainage procedure.
A Whipple’s procedure (pancreaticoduodenectomy) is rarely needed and should not be performed in the
emergency situation because of the very high associated mortality rate.
A damage control procedure with packing and drainage should be performed and the patient referred for
definitive surgery once stabilised.

Stomach:

Most stomach injuries are caused by penetrating trauma.

Blood may be present and is diagnostic if found in the nasogastric tube.
Surgical repair is required but great care must be taken to examine the stomach fully, as an injury to the front of
the stomach can be expected to have an ‘exit’ wound elsewhere on the organ.

Duodenum:

Duodenal injury is frequently associated with injuries to the adjoining pancreas.

The only sign may be gas in the periduodenal tissue seen at CT.
Smaller injuries can be repaired primarily.
Major injuries and those also involving the head of the pancreas should undergo initial damage control surgery
and be referred for definitive care.

Small bowel:

The small bowel is frequently injured as a result of blunt trauma.

The individual loops may be trapped, causing high-pressure rupture of a loop or tearing of the mesentery.
Small bowel injuries need urgent repair.
Haemorrhage control takes priority and these wounds can be temporarily controlled with simple sutures.
The small bowel can also be temporarily occluded until haemorrhage control has been achieved.
In blunt trauma, the mesenteric vessels damage, and the bowel ischaemia which results, may dictate the extent
of a resection.
Resections should be carefully planned to limit the loss of viable small bowel but should be weighed against an
excessive number of repairs or anastomoses.
Haematomas in the small bowel mesenteric border need to be explored to rule out perforation.
With low-energy wounds, primary repair can be performed after debridement of any dead tissue, whereas more
destructive wounds associated with military-type weapons require resection and anastomosis.

Colon:

Injuries to the colon from blunt injury are relatively infrequent, and are a more frequent penetrating injury.
If relatively little contamination is present and the viability is satisfactory, such wounds can be repaired

******ebook converter DEMO Watermarks*******

 primarily.
If, however, there is extensive contamination, the patient is physiologically unstable, or the bowel is of doubtful
viability, then the colon can be closed off and a defunctioning colostomy formed.

Rectum:

Only 5 per cent of colon injuries involve the rectum.

These are generally from a penetrating injury, although occasionally the rectum may be damaged following
fracture of the pelvis.
Digital rectal examination will reveal the presence of blood, which is evidence of colorectal injury.
These injuries are often associated with bladder and proximal urethral injury.
With intraperitoneal injuries, the rectum is managed as for colonic injuries.
Full-thickness extraperitoneal rectal injuries should be managed with either a diverting end-colostomy and
closure of the distal end (Hartmann’s procedure) or a loop colostomy.
Presacral drainage is generally no longer used.

Retroperitoneal hemorrhage:

Injury to the retroperitoneum is often difficult to diagnose, especially in the presence of other injury, when the
signs may be masked.
Intraperitoneal diagnostic tests (ultrasound and diagnostic peritoneal lavage) may be negative.
The best diagnostic modality is the computed tomography (CT) scan, but this requires a physiologically stable
patient.

The retroperitoneum is divided into three zones.

Zone 1 (central): central haematomas should always be explored, once proximal and distal vascular control has
been obtained.
Zone 2 (lateral): lateral haematomas are usually renal in origin and can be managed non-operatively, they may
some- times require angioembolisation.
Zone 3 (pelvic): pelvic haematomas are exceptionally dif- ficult to control and, whenever possible, should not
be opened; they should be controlled with packing (intra- or extraperitonial) and angioembolisation.

Damage control surgery:(important topic for exams):

Following major injury, protracted surgery in the physiologically unstable patient can in itself prove fatal.
Patients with the ‘deadly triad’ of hypothermia, acidosis and coagulopathy are those at highest risk. ‘
Damage control’ or ‘damage limitation surgery’ is a concept that originated from naval strategy, whereby a
******ebook converter DEMO Watermarks*******
 ship which has been damaged may have minimal repairs needed to prevent it from sinking, while definitive
repairs wait until it has reached port.
The minimum surgery needed to stabilise the patient’s condition may be the safest course until the
physiological derangement can be corrected.

Damage control surgery is restricted to only two goals:

1. stopping any active surgical bleeding;
2. controlling any contamination.

Once these goals have been achieved, then the operation is suspended and the abdomen temporarily closed.
The patient’s resuscitation then continues in the intensive care unit.
Once the physiology has been corrected, the patient warmed and the coagulopathy corrected, the patient is
returned to the operating theatre for any definitive surgery.
Abdomen is temporarily closed with BOGOTA BAG.

******ebook converter DEMO Watermarks*******

Shock:
Shock is defined as an abnormality of the circulation that causes inadequate organ perfusion and oxygenation.

Five types of shock may be encountered in surgical practice:

1. hypovolaemic.
2. septic.
3. cardiogenic.
4. neurogenic.
5. anaphylactic.

Hypovolaemic Shock:
This is due to decreased circulating blood volume.
Causes:

haemorrhage, e.g. trauma, haematemesis, ruptured aortic aneurysm.

dehydration, e.g. severe vomiting or diarrhoea, third space loss in inflammatory conditions.
******ebook converter DEMO Watermarks*******
 burns resulting in massive loss of serum.

Septic Shock:
Septic shock is part of the systemic inflammatory response syndrome (SIRS).

Sepsis is defined as SIRS with a confirmed source of infection.

Septic shock is defined as hypotension and hypoperfusion despite adequate fluid resuscitation.
Septic shock is uncommon in trauma unless there has been a delay in presentation.
Pathophysiology:

Septic shock is due to the release of a number of pro-inflammatory mediators such as IL-l, IL-6, TNF-ex, PAF
and the eicosanoids; and as a result of bacterial endotoxins (lipopolysaccharides).
Septic shock is usually due to Gram-negative organisms such as E. coli, Klebsiellaand pseudomonas, although
peptidoglycans and teichoic acids in Gram-positive bacteria can also have similar effects.

The pathophysiology underlying shock in septic patients includes:

peripheral vasodilation.
INCREASED vascular permeability (third space loss).
peripheral arteriovenous shunting.
myocardial depression due to toxic effects on heart.
uncoupling of oxidative phosphorylation and anaerobic respiration leading to severe metabolic acidosis.
There may be an obvious source of infection, together with a predisposing condition. The patient may be
confused and restless; initially the skin is hot and flushed and the pulse characteristically ‘bounding’.
Vasoconstriction and the classic signs of shock may develop later.

******ebook converter DEMO Watermarks*******

Reatment:

This is urgent and involves resuscitation, identification of the source of sepsis, appropriate antibiotic therapy
and any necessary surgery to eradicate the focus of infection.

Complications:

Sepsis and septic shock can progress to MODS (multi-organ dysfunction syndrome) and MOPS (multi-organ
failure syndrome).

Neurogenic Shock:

Neurogenic shock is due to impaired descending sympathetic pathways in the spinal cord.
this results in loss of vasomotor tone and sympathetic innervation to the heart.
This leads to pooling of blood in the lower limbs.
Although neurogenic shock can occur with spinal injury, it is not synonymous with spinal shock.
SPINAL SHOCK refers to the flaccidity and areflexia seen after a spinal injury.
Neurogenic shock can also occurs from certain nervous stimuli, i.e. fright- this leads to a sudden dilation of the
splanchnic vessels and a bradycardia- the transient hypotension may lead to collapse.

Symptoms and signs:

The classic sign of neurogenic shock in the trauma patient include:

Bradycardia - due to loss of sympathetic tone.

Hypotension – there is no narrowed pulse pressure.
No vasoconstriction of peripheries.

Treatment:

In the trauma patient shock should never be assumed to be neurogenic; hypovolaemia is by far the most
common cause of hypotension and patients with spinal injury often have concurrent thoracic or abdominal
injuries.

Management Includes:

Maintain spinal immobilization

Vasopressors may be needed to maintain blood pressure
Atropine if significant bradycardias occur.
In the non-trauma setting neurogenic shock is self-limiting.

Cardiogenic Shock:
Cardiogenic shock or ‘pump failure’ is due to a loss of myocardial contractility.
Causes:
These can be divided into:
1. cardiac compressive.
2. cardiac obstructive.
3. functional.
All lead to problems with myocardial function and an inadequate cardiac output.
Compressive shock:

external forces compress the heart and great vessels leading to impairment of diastolic filling, a decrease in

******ebook converter DEMO Watermarks*******

 stroke volume and consequent hypotension.
Causes include.

1. cardiac tamponade.
2. positive pressure ventilation.
3. tension pneumothorax.
4. abdominal compartment syndrome.
Obstructive shock:

occurs when intravascular obstruction, excessive stiffness of arterial walls and microvascular blockage places
an undue stress on the heart.
It may be right- or left-sided.
Tension pneumothorax is the commonest traumatic cause but other causes include valvular stenosis, PE and
ARDS.

Functional:

The heart itself is not functioning efficiently.

This may be due to arrhythmias or impaired muscle function after contusion or infarction.

Symptoms and signs:

Cardiac causes may present with chest pain and collapse.

There may be a past history of cardiac problems or presence of risk factors, i.e. diabetes.
Patients may be dyspnoeic with signs of pulmonary oedema.
The patient may also display the classic signs of shock, i.e. pale, clammy, tachycardia, hypotension.

Treatment:
1. ABC- high flow oxygen administration and i.v. access.
2. Place patient in most comfortable position, i.e. sitting up with pulmonary oedema.
3. Pain relief, e.g. diamorphine.
4. Drugs- consider aspirin (if MI), furosemide (if pulmonary oedema).
5. inotropic agents.

Correct arrhythmias.
Correct U&Es and acid-base abnormalities.
Cardiac monitoring (preferably on CCU).
CVP monitoring- avoid fluid overload.
Consider angioplasty for MI in the postop setting as thrombolytic therapy is contraindicated.
Surgery for valvular abnormalities.

End points of resuscitation:

Best clinical end point is Urine output.
Best perfusion end point is NIRS (Near Infrared Spectroscopy).
Best arterial end point is MVOS (mixed venous oxygen saturation).

******ebook converter DEMO Watermarks*******

Neurotrauma:
ICP and the Monro Kellie doctrine:

Alexander Monro observed in 1783 that the cranium is a ‘rigid box’ containing a ‘nearly incompressible brain’.
Therefore any expansion in the contents, especially haematoma and brain swelling, may be initially
accommodated by exclusion of fluid components, venous blood and cerebrospinal fluid (CSF).
Further expansion is associated with an exponential rise in intracranial pressure.
The result is hypoperfusion and herniation.

Cerebral Blood Flow:

Normal cerebral blood flow (CBF) is about 55 mL/minute for every 100 g of brain tissue.
Ischaemia results when this rate drops below 20 mL/min,
The flow rate is related to: cerebral perfusion pressure (CPP), the difference between mean arterial pressure
******ebook converter DEMO Watermarks*******
 (MAP) and intracranial pressure (ICP): CPP (75–105 mmHg) = MAP (90– 110 mmHg) − ICP (5–15 mmHg).
In the normal brain, variations in vascular tone maintain a constant CBF across a range of MAP between 50
and 150 mmHg, and a corresponding range of CPP, a process termed ‘cerebral autoregulation’.
The limits of this range are elevated in patients with chronic hypertension.
Neurosurgical emergencies, especially head injury, lead to brain swelling, bleeding and hydrocephalus. The
common pathophysiological pathway is then elevated ICP and reduced CPP and CBF.

Herniation syndromes:

The rapid increase in intracranial pressure which accompanies the exhaustion of compensation mechanisms
ultimately results in herniation of brain tissue.
The uncus of the temporal lobe may herniate over the tentorium resulting in pupil
abnormalities(IPSILATERAL PUPIL DILATATION), usually occurring first on the side of any expanding
haematoma.

Cerebellar tonsillar herniation through the foramen magnum compresses medullary vasomotor and respiratory
centres, classically producing Cushing’s triad of hypertension, bradycardia and irregular respiration
UNCAL HERNIATION is the MC type of herniation.
TONSILLAR HERNIATION is the most lethal of all herniation.
Uncal herniation can compress the third nerve, compromising the parasympathetic supply to the pupil, so that
unopposed sympathetic activity produces an enlarged and sluggish pupil, which then, if the compression
continues, becomes fixed and dilated. This is known as HUTCHINSON’s PUPIL.
However, an abnormal pupil size and response may reflect pathology anywhere in the eye or the reflex loop
made up by the optic nerve, the oculomotor nerve, and the brainstem.
Direct ocular trauma or nerve injury in association with a skull base fracture can cause mydriasis (dilated pupil)
present from the time of injury.

The primary survey in head injury:

Ensure adequate oxygenation and circulation.

Check pupil size and response and Glasgow Coma Score as soon as possible.
Check for focal neurological deficits before intubation if possible.
******ebook converter DEMO Watermarks*******
 Check blood sugar for hypoglycaemia
Calculate the GCS.
Look for the Neurological deficit.

Secondary survey:

A full secondary survey will be required. Particular attention must be paid to the head, neck and spine.
Examination of the head should include inspection and palpation of the scalp for evidence of
subgalealhaematoma and scalp lacerations, which may bleed profusely, and potentially overlie fractures.
Examine the face for evidence of fractures, especially to the orbital rim, zygoma and maxilla.
Clinical evidence of a skull base fracture.

IOC for evaluation of head injury is NCCT

Indications for computed tomography (CT) in head injury:
1. GCS <13 at any point.
2. GCS 13 or 14 at 2 hours.
3. Focal neurological deficit.
4. Suspected open, depressed or basal skull fracture.
5. More than one episode of vomiting.
6. Any patient with a mild head injury over the age of 65 years orwith a coagulopathy, for instance warfarin use,
should be scanned urgently.
7. Dangerous mechanism or injury.
8. antegrade amnesia >30 minutes warrants CT within 8 hours.
Classification of traumatic head injuries:

Scalp: open and closed (beware of air under the dura).

Open or Closed.
Skull site: vault and base of skull.
Skull type: linear, comminuted and depressed.
Intracranial bleeding: extradural, subdural, subarachnoid and intraparenchymal.
Brain tissue causes: diffuse, blunt (direct, coup–contrecoup) and penetrating.

Closed Head Injury:

Closed head injury (CHI) is the most common type of TBI.

There are two important factors that affect the outcome of CHI in general.
The initial impact causes the primary injury, defined as the immediate injury to neurons from transmission of
the force of impact.
Subsequent neuronal damage due to the sequelae of trauma is referred to as secondary injury.
Hypoxia, hypotension, hydrocephalus, intracranial hypertension, thrombosis, and intracranial hemorrhage may
all be mechanisms of secondary injury.
One focus of basic research in TBI, critical care medicine, and neurosurgical intervention is to decrease the
effects of secondary injury.

Types of Closed Head Injury:

Concussion:

Concussion is defined as temporary neuronal dysfunction following nonpenetrating head trauma.

The head CT is normal, and deficits resolve over minutes to hours.
This is most common type of traumatic brain injury

Contusion:
******ebook converter DEMO Watermarks*******
 A contusion is a bruise of the brain, and occurs when the force from trauma is sufficient to cause breakdown of
small vessels and extravasation of blood into the brain.
The contused areas appear bright on CT scan.
The frontal, occipital, and temporal poles are most often involved.
The brain sustains injury as it collides with rough, bony surfaces.
Contusions themselves rarely cause significant mass effect as they represent small amounts of blood in injured
parenchyma rather than coherent blood clots. Edema may develop around a contusion, causing mass effect.
Contusions also may occur in brain tissue opposite the site of impact. This is known as a contre-coup injury.

Diffuse Axonal Injury:

Diffuse axonal injury is caused by damage to axons throughout the brain, due to rotational acceleration and
then deceleration.
Axons may be completely disrupted and then retract, forming axon balls.
Small hemorrhages can be seen in more severe cases, especially on MRI. Hemorrhage is classically seen in the
corpus callosum and the dorsolateral midbrain.

Penetrating Injury:
The two main subtypes are :

missile (e.g., due to bullets or fragmentation devices) and

nonmissile (e.g., due to knives or ice picks).

Intracranial haematoma:
Haemorrhage within the cranium occurs in four main sites:
1. extradural.
2. subdural.
3. subarachnoid.
4. intraparenchymal.
Extradural haematoma:

Extradural haematoma results from rupture of an artery, vein or venous sinus, in association with a skull
fracture.
Typically, it is damage to the middle meningeal artery under the thin temporal bone.
A low energy injury mechanism, perhaps with brief loss of consciousness, is sufficient to start the extradural
bleeding.
The patient may then present in the subsequent lucid interval with headache, but without any neurological
deficit.
At this stage, the increase in the intracranial volume is not yet causing a significant rise in intracranial pressure
because compensation is occurring.
once the limits of compensation have been reached after as long as some hours (see Monro Kellie doctrine)
rapid deterioration follows.
There is contralateral hemiparesis, reduced conscious level and ipsilateral pupillary dilatation, the cardinal
signs of brain compression and herniation.
Although this classical presentation occurs in only one third of cases, it emphasises the potential for rapid
avoidable secondary brain injury in patients with minimal primary injury.
Rarely; patients may present with KERNOHAN’s phenomenon where there is ipsilateral hemiparesis.
On CT:
extradural haematomas appear as a lentiform (lens- shaped or biconvex) hyperdense lesion between skull
and brain, constrained by the adherence of the dura to the skull.
Mass effect may be evident, with compression of surrounding brain and mid- line shift.
A skull fracture will usually be evident

******ebook converter DEMO Watermarks*******

management:

Midline shift more than 1 cm and brainstem compression in CT scan point towards raised ICP and need for
emergent surgical intervention.
urgent decompression by doing a CRANIOTOMY is the standard recommendation.

Acute subdural haematoma:

Acute subdural bleeding arises from rupture of bridging cortical vessels.

In contrast to extradural haematoma (and chronic subdural haematoma), acute subdural haematoma is usually
associated with a high energy injury mechanism and significant primary brain injury.
Conscious level is usually therefore impaired at presentation, but may deteriorate further as the haematoma
expands.
Not associated with LUCID Interval.
Since the dura is not adherent to the brain as it is to the skull, subdural blood is free to expand across the brain
surface giving a diffuse concave appearance
Acute subdural bleeds of significant size or with significant associated midline shift require evacuation, and the
cumulative mortality in this group is about 50 per cent.
Craniotomy is the surgical management of choice.

Chronic subdural haematoma:

2-3 week old SDH is known as Chronic SDH.

The patient is generally elderly, may be taking antiplatelet or anticoagulant medications.
usually a history of a recent fall, or falls.
Cerebral atrophy commonly found in the elderly is believed to stretch bridging veins.
These can then rupture after only minor trauma, bleed, and then tamponade (stop bleeding due to the pressure
which has been produced by the bleed).
Subsequent degradation of the blood clot over days or weeks leads to osmotic expansion. It is this which
produces the mass effect.
Presenting features, just as for any expanding intracranial mass, include pressure symptoms, especially
headache and drowsiness, neurological deficit and seizures.
Recent bleeding may be isodense or hyperdense, and mixed density can indicate an acute-on-chronic subdural
haematoma.
Drainage is performed using burr holes, often under local anaesthetic (especially in elderly patients who present
a substantial anaesthetic risk).
Urgency is dictated by the clinical condition of the patient. If clinically stable, a delay of 7–10 days to allow
platelet function to normalise after withdrawal of aspirin may be considered.
Anticoagulation should be reversed either by administration of vitamin K, or urgently by transfusion of
recombinant clotting factors in patients who have deteriorated acutely.
Occasionally, acute-on-chronic bleeds with residual solid clot or septations require a craniotomy for adequate
clot evacuation.

******ebook converter DEMO Watermarks*******

Traumatic subarachnoid haemorrhage:

Trauma is the most common cause of subarachnoid haemorrhage.

Is managed conservatively.
It is not usually associated with significant vasospasm, which characterises aneurysmal subarachnoid
haemorrhage.
The possibility of spontaneous subarachnoid haemorrhage actually leading to collapse and so causing a head
injury needs to be borne in mind and formal or CT angiography may be required to exclude this.
SAH can also be seen in the cisterns surrounding the brainstem.
Traumatic intraventricular hemorrhage (IVH) results from tearing of subependymal veins or as a direct
extension of parenchymal hematoma.
On CT, acute IVH appears as hyperdensity within the ventricular system .
One should always look for hydrocephalus (enlargement of ventricles) in such cases as blood especially in the
fourth ventricle, can obstruct the ow of CSF.
Base of the skull fracture
Can be in the:.
anterior 1/3:
Raccoon eyes or Panda eyes is peri orbital hematoma.
CSF rhinorrhea
Aaociated with cranial nerve 1 injury middle 1/3:
CSF otorrhoea
******ebook converter DEMO Watermarks*******
 Battle’s sign: discolouration around the mastoid
Associated with cranial nerve7,8 injury
Posterior 1/3:
Associated with cranial nerve 9,10,11 injury.

Raised intracranial pressure:

Acutely raised ICP is a neurosurgical emergency.
The symptoms are:

headache.
nausea and vomiting.
diplopia and blurred vision.
drowsiness then coma.

Hydrocephalus:

Hydrocephalus refers to an increase in CSF volume and ventricular enlargement due to disturbance of
production, flow or reabsorption of CSF.

CSF pathways’:

Cerebrospinal fluid (CSF) is produced by the choroid plexus of the lateral ventricles, and flows through the
foramina of Monro into the midline third ventricle.
then through the cerebral aqueduct to the fourth ventricle, before exiting through the foramina of Magendie and
Luschka to the subarachnoid space around the brain.
Hydrocephalus may result from an excess of CSF production (in the rare condition of choroid plexus
papilloma).
from obstruction to circulation (an obstructive hydrocephalus).
from failure of reabsorption (a communicating hydrocephalus).
Hydrocephalus ex vacuo describes the ventriculomegaly associated with brain atrophy.

Disorders of CSF flow with poorly understood pathogenesis manifest in two syndromes,

normal pressure hydrocephalus

Idiopathic intracranial hypertension (IIH).

Normal pressure hydrocephalus:

This is an important cause of dementia since it is readily reversible.

It typically presents in older patients.
Triad of gait disturbance, incontinence and cognitive decline.
It may occur de novo or on a background of previous brain insults including subarachnoid haemorrhage (SAH),
head injury, meningitis and tumour.
Ventriculomegaly is seen on imaging.
******ebook converter DEMO Watermarks*******
 The CSF pressure at lumbar puncture (LP) is typically normal, but it is believed that intermittent elevations in
pressure may be involved in the aetiology.
Lumbar infusion testing involves insertion of a fine drain at lumbar puncture, followed by measurement of the
CSF pressure changes associated with a fluid challenge administered through this.
This allows evaluation of the likely benefit from definitive treatment by shunt insertion.

Idiopathic intracranial hypertension:

This condition presents with features of raised ICP without an underlying tumour, explaining the old terms for
the condition, pseudotumourcerebri or benign intracranial hypertension.
This description is misleading, since IIH can progress rapidly to blindness.
The patient, typically a young overweight female, describes a headache typical of raised pressure, and visual
deterioration.
Examination may reveal papilloedema, and occasionally cranial nerve palsies.
Imaging is unremarkable, but lumbar puncture demonstrates a raised opening pressure >25 mmHg.
The diagnosis is one of exclusion, and the aetiology is not well understood.
Impaired CSF resorption may reflect raised venous pressure, either as a result of sinus thrombosis, or secondary
to raised intra-abdominal pressure in obese patients.
Weight loss and cessation of certain medications including the oral contraceptive pill is often effective. This is
combined with medical therapy.

Investigation of raised intracranial pressure:

Where raised ICP is suspected, computed tomography (CT) is a first-line investigation to demonstrate
hydrocephalus, underlying pathology and to evaluate the degree of mass effect and the patency of the basal
cisterns, the spaces surrounding the brainstem.
This is key to management since lumbar puncture in the setting of raised intracranial pressure can result in
downward herniation of brain structures to replace the fluid drained.
Lumbar puncture in obstructive hydrocephalus risks hernia-tion of the brainstem and cerebellar tonsils.
For communicating hydrocephalus, lumbar puncture is of diagnostic value, deriving an opening pressure and
assessment of the CSF contents.

Management:

Acute hydrocephalus is an emergency since the condition can progress over minutes or hours to coma and
death.
It may be relieved by addressing the underlying pathology, for instance by excision of a tumour responsible for
an obstructive hydrocephalus.
Most often, however, temporary ventricular drainage is required, either as an emergency in an obtunded or
deteriorating patient, or as a precaution during definitive surgery considering the possibility for postoperative
swelling.

External ventricular drain:

External ventricular drains (EVDs) are an effective temporary measure to relieve hydrocephalus.

Ventriculoperitoneal shunts:

Ventriculoperitoneal (VP) shunting comprises insertion of a ventricular catheter, which may be antibiotic
impregnated, into the frontal or occipital horn of the lateral ventricle, while a distal catheter is tunnelled
subcutaneously to the abdomen.
Ventriculoatrial and ventriculopleural shunting is also possible.

Endoscopic third ventriculostomy:

******ebook converter DEMO Watermarks*******

 This procedure is especially useful in obstructive hydrocephalus due to aqueduct stenosis.

******ebook converter DEMO Watermarks*******

Plastic Surgery:
Wound Healing:
Normal wound healing comprises a combination of regeneration and repair. Three mechanisms are involved:

epithelialization.
wound contraction.
extracellular matrix synthesis.

During repair, a complex chain of events eventually leads to the formation of a scar. The process requires:
phagocytosis, chemotaxis, mitogenesis, and the synthesis of collagen and extracellular matrix components.
In certain circumstances, the cellular processes that contribute to repair become unregulated, leading to excessive
scarring in the form of hypertrophic scars and keloids.
Types of Healing:
There are four general types of wound healing:
1. Primary.
2. Delayed primary.
3. Secondary.
4. Healing that occurs in partial-thickness wounds.
Primary Healing:
when wound is closed within hours of its creation.

The wound edges are reapproximated directly using sutures or by some other mechanical means.
Collagen metabolism provides long-term strength to the wound when normal synthesis, deposition, and cross-
linking of the collagen occur.
Matrix metalloproteinase enzymes regulate collagen and extracellular matrix degradation and allow for
remodeling of the wound, leaving a relatively narrow scar.
Epithelialization provides coverage of the wound surface and acts as a barrier from bacterial invasion.

Delayed Primary Healing:

Contaminated wound is left open to prevent wound infection.

The skin and subcutaneous tissues are left unopposed and closure is performed after the normal host defenses
are allowed to debride the wound.
After 3 to 4 days, local phagocytic cell recruitment into the wound has occurred and angiogenesis has begun.
Inflammatory cells are present that destroy contaminating bacteria.
The wound edges are approximated following a delay of several days.
Collagen metabolism is undisturbed and tensile strength develops as if closure had been immediate.

Secondary Healing:
An open full-thickness wound is allowed to close by both wound contraction and epithelialization.

The wound decreases in size by contraction. (myofibroblast is thought to play a key role). The cells appear in
the wound on approximately the 3rd day after wounding and increase in number to a maximal level between
******ebook converter DEMO Watermarks*******
 10th and 21st day.
They disappear as contraction is completed.

Healing of Partial-Thickness Wounds:

Partial-thickness wounds, which involve the epithelium and the superficial portion of the dermis, heal mainly
by epithelialization.
Epithelial cells within the dermal appendages, hair follicles, and sebaceous glands replicate to cover the
exposed dermis.
There is minimal collagen deposition and an absence of wound contraction.

Overview:
The process of wound healing occurs as a sequential cascade of phagocytosis, chemotaxis, mitogenesis, collagen
synthesis, and the synthesis of other matrix components.
Tissue Injury:

Tissue injury initiates the process of bleeding, coagulation, inflammation, cell replication, angiogenesis,
epithelialization, and matrix synthesis.
Tissue injury is characterized by microvascular injury and therefore extravasation of blood into the wound.
Injured vessels constrict rapidly and the coagulation cascade is activated in order to limit the blood loss.
Vasoactive amines and other mediators are released by inflammatory cells, which contribute to the leak of
plasma and proteins into the wound and allow effector cells to enter.

Coagulation:

Coagulation leads to hemostasis. Platelets trapped in the clot are essential for hemostasis as well as for a
Normal inflammatory response. The alpha granules of the platelets contain growth factors, including platelet-
derived growth factor (PDGF), transforming growth factor-beta (TGF-b), and platelet factor IV. These proteins
initiate the wound-healing cascade by attracting and activating fibroblasts, endothelial cells, and macrophages.
The platelets also contain dense bodies that store vasoactive amines, e.g. serotonin, that increase microvascular
permeability.
The end product of both the intrinsic and extrinsic coagulation pathways is fibrin.
Fibrin is essential to early wound healing because it provides the matrix.

Early Inflammation:

The next phase of healing, inflammation, begins with the activation of complement and the initiation of the
classical molecular cascade, which leads to infiltration of the wound with granulocytes within 24 to 48 hours of
injury.
the granulocytes begin to adhere to the endothelial cells in the adjacent blood vessels by a process called
margination, and begin to actively move through the vessel wall, a process known as diapedesis.
The major function of granulocytes is to remove bacteria and foreign debris from the wound, thereby helping to
prevent infection.

Late Inflammation:

Macrophages are the most important cells present in the healing wound and appear to act as the key regulatory
cells for repair.
Circulating monocytes and tissue macrophages, when depleted, cause severe alterations in wound healing with
poor debridement, delayed fibroblast proliferation, inadequate angiogenesis, and poor fibrosis.
Once the circulating monocyte passes through the blood vessel wall and into the wound, it is considered a
wound macrophage.
Between 48 and 72 hours after wounding, macrophages represent the predominant cell type within the wound.
The macrophage functions as a phagocytic cell as well as being the primary producer of growth factors
******ebook converter DEMO Watermarks*******
 responsible for both the production and proliferation of the extracellular matrix (ECM) by fibroblasts,
proliferation of smooth muscle cells, and proliferation of endothelial cells resulting in angiogenesis.
The lymphocyte is the last cell to enter the wound during the inflammatory phase (>72 hours after wounding).

Fibroblast Migration/Collagen Synthesis:

Successful healing requires the migration of mesenchymal cells into the wound. Stimulated by growth factors,
fibroblasts migrate into the wound through the ECM.
By 7 days, they are the predominant cell type in the wound.
At 5 to 7 days after wounding, the fibroblasts begin synthesizing collagen, which increases in a linear fashion
for 2 to 3 weeks.
Collagens provide strength and integrity for all tissues.
Type I collagen is the major structural component of bones, skin, and tendons.
Type II collagen is found predominantly in cartilage.
Type III collagen is found in association with type I collagen in varying ratios depending on the type of
tissue.
Type IV collagen is found in the basement membrane.
Type V collagen is found in the cornea.

Angiogenesis:

Angiogenesis is the process of forming new blood vessels and is ongoing throughout the previously mentioned
phases of wound healing.
Platelets enter the wound in the earliest phase of repair and secrete, among others things, TGF-b, which
indirectly promotes angiogenesis and attracts macrophages.
The platelets also secrete PDGF, which attracts macrophages and granulocytes and promotes angiogenesis.

Epithelialization:
Mitosis of epithelial cells begins 48 to 72 hours after injury.
The rate of epithelial coverage is increased if the wound does not need debridement, if the basal lamina is intact, and
if the wound is kept moist.
Several growth factors modulate epithelialization. E.g. Epidermal growth factor (EGF), basic FGF and keratinocyte
growth factor (KGF).
Remodeling Phase:
Collagen synthesis and breakdown equilibrate to a steady state approximately 21 days after wounding.
There is ongoing collagen synthesis and collagen breakdown as the ECM is continually remodeled.
Collagen degradation is achieved by specific matrix metalloproteinases
Fibronectins are matrix molecules that are involved in wound contraction, cell-cell and cell-matrix interaction, cell
migration, collagen matrix deposition, and epithelialization. They act as a scaffold for collagen deposition.
Abnormality:
Excessive Wound Healing:

Hypertrophic scars and keloids are forms of excessive healing.

Hypertrophic scars are defined as those that remain within the borders of the original scar, whereas keloids
extend beyond the original scar margins.
Wounds that cross skin tension lines, in thick skin or in susceptible locations such as the earlobe, presternal,
and deltoid regions, are more prone to abnormal healing.
Hypertrophic scars generally begin to develop in the weeks after injury, whereas keloids can develop up to 1
year later.
Histologically, mucinous ground substance is present in large amounts in keloids, but fibroblast density is less
******ebook converter DEMO Watermarks*******
 than in hypertrophic scars.

The nonsurgical management of keloids:

1. Physical: Examples of physical forms of treatment include radiotherapy, ultrasound, cryotherapy, pressure, and
laser.
2. Pharmacologic: intralesional steroids
Cleft Lip and Palate:
Epidemiology:

Cleft lip and palate represents the second most frequently occurring congenital deformity (after clubfoot
deformity).
1/700 overall incidence for facial clefting.
Clefting more common in Asians (1/400) and less common in African American (1/2000).
Clefts can be unilateral or bilateral; Left side more common for unilateral.
Syndromic clefting accounts for 50-60% pts Cleft lip, cleft palate or both affects approximately 1 in 750.

Embryology:

Weeks 5 & 6: Maxillary processes grow medially & fuse with frontonasal process
Failure here > cleft lip +/− primary (anterior) palate.
Weeks 7 & 8: Tongue descent, migration & fusion of palatal shelves.
Failure here > cleft secondary (posterior) palate (Pierre-Robin, & other).

Anatomy:

The palatine processes of the maxilla and horizontal lamina of the palatine bones form the hard palate.
Its blood supply is mainly from the greater palatine artery.
The nerve supply is via the anterior palatine and nasopalatine nerves.
The soft palate is a fibromuscular shelf made up of several muscles attached like a sling to the posterior portion
of the hard palate.

******ebook converter DEMO Watermarks*******

 It closes off the nasopharynx by tensing and elevating, thereby contacting Passavants ridge posteriorly.
The soft palate consists of the tensor velipalatini, the levatorvelipalatini, the musculus uvulae, the
palatoglossus, and palatopharyngeus muscles.
CN V supplies the tensor velipalatini, while CN IX and CN X innervate the others.
The levatorvelipalatini is the primary elevator of the palate.

Etiologies:

Teratogens: ethanol (FAS), anti-convulsants, steroids, chemo, excess Vita A.

Maternal / intra-uterine conditions: infant of diabetic mom, amniotic bands.
Chromosomal abnormalities, monogenic causes (AR, AD, XL).
Unknown.

Genetics:

Nonsyndromic inheritance of facial clefting is multifactorial.

Familial inheritance of both cleft lip and palate occurs with varying frequency.
Chromosome aberrations such as trisomy D and E have increased incidence of clefts.
More than 200 recognized syndromes may include a facial cleft as a manifestation.
Common syndromes with cleft palates include Apert’s, Stickler’s and Treacher Collins. Van der Woude’s
and Waardenberg’s syndromes are associated with cleft lip with or without cleft palate.

Diagnosis – Newborn Physical Exam:

******ebook converter DEMO Watermarks*******

 Inspect lip and oropharynx.
Palpate palate with gloved finger (submucous cleft is not visible, but can be felt in bony palate underlying
mucous membrane. This diagnosis is often missed until later childhood when speech problems arise.

Newborn Feeding:

Isolated cleft lip rarely causes feeding problems.

Cleft lip & palate or palate alone may require special nipples, occluders.

Surgical Treatment:

Cleft lip: “Rule of Tens” -- ten weeks, ten pounds, hemoglobin of 10.
Cleft palate: around 1 year, before speech develops.

a. Subsequent surgical revisions required as child grows.

b. Palatal repair- repaired at approximately 9-12 months.
c. Secondary repair- if needed- repaired at approximately 4-6 years.
d. Alveolar cleft- repaired at 8-10 years.
e. Final repair- if needed repaired at 14-16 years.
Associated Problems in Childhood:

ENT problems: often requires ear tubes

a. Hearing loss (cleft palate): Cleft palate is very often associated with eustachian tube dysfunction due to an
abnormal insertion of the levator and tensor velipalatini muscles into the posterior margin of the hard
palate. In addition to middle ear effusion, the patients also appear to have an increased incidence of
cholesteatoma (7%).
b. Indications for myringotomy and tube insertion include a significant conductive hearing loss or persistent
middle ear effusion, recurrent otitis media, or tympanic membrane retraction. Speech problems, often
speech therapy (cleft palate): It is estimated that 75% of patients have velopharyngeal competence
following primary cleft palate surgery, and this can be increased to 90-95% with directed secondary
procedures.

Dental problems, usually orthodontics.

Multi-disciplinary cranio-facial teams address child’s multiple needs.
Airway problems: may arise in children with cleft palates, especially those with concomitant structural or
functional anomalies. eg, Pierre-Robin sequence is the combination of micrognathia, cleft palate, and
glossoptosis.

Van der Woude Syndrome (VWS):

About three percent of people with a cleft have VWS. VWS is inherited in an autosomal dominant pattern. Features
of VWS include:

mounds or depressions (pits) on the lower lip.

cleft lip, with or without cleft palate.
cleft palate alone.
missing teeth.

******ebook converter DEMO Watermarks*******

 Points to Remember:
Cleft lip is due to failure of fusion between medial fronto-nasal process and maxillary process (Normal
fusion takes place between 5-6 weeks of intra-uterine life).
Cleft palate is due to failure of fusion between The palatine processes of the maxilla and horizontal lamina of
the palatine bones.
Combined cleft lip and palate is commonest (Incidence 1:600), isolated cleft lip incidence is 1: 1000.
Cleft palate 45%
Combined palate and lip 40%
Isolated cleft lip 15%
Cleft lip surgery done at: 3 to 6 months.
Age > 10 weeks.
Weight > 10 pounds.
Hemoglobin> 10 gms
Names of surgeries are:
Millard rotation advancement (most commonly done);
Tennison,
Le Muserier,
Tennison- Rendall, Thompson)
Cleft palate: is done between 6 months to 18 months.
If operated separately then soft palate is operated at 6m-12months soft palate at 6 months) and hard palate is
operated at 12-18 months.
If both are operated together than it is operated at around 1 year.
For bone (Alveolus correction) of receded upper lip, orthoplastic surgery is done at 14-16 years (Le Fort I
fracture and advnacement)
Furlow and Tessier opeartions are done for Palate
Post-operative Veloparyngeal incompetence is corrected by “Hynes Pharyngoplasty”

Principle of Plastic Suregry

Geometric Principle of the Z-Plasty:

The Z-plasty is a principle that can be applied to revise and redirect existing scars or to provide additional
length in the setting of scar contractor.
The Z-plasty involves the transposition of two triangular flaps.
The limbs of the Z must be equal in length to the central limb but can extend at varying angles (from 30–90
degrees) depending on the desired gain in length.
For a basic z-plasty using an angle of 60 degrees, this angle can lengthen a contracted scar by about 75 percent
and reorient the direction of the central wound by 90 degrees.
Angles smaller than 60 degrees are easier to transpose but result in less lengthening and realignment of the scar
to less than 90 degrees.
Angles larger than 60 degrees should be avoided because the force required to transpose the flaps increases
markedly, making closure of the wound difficult.
Although angles between 30 and 90 degrees are possible, the 60-degree Z-plasty is most common.
A 60-degree Z-plasty will yield a 75% increase in scar length and a 90-degree change in scar direction.

******ebook converter DEMO Watermarks*******

 45-degree Z-plastythe results in approximately 60 degrees from the initial position and there is a 50% gain in
scar length.
In the 30-degree Z-plasty, the direction of the change is approximately 45 degrees from the initial position of
the central limb and there is a 25% gain in scar length.
Z-plasties with angles less than 30 degrees will greatly increase the risk of tip necrosis.
90 Degree Z plasty leads to 125% increase in length.

Differences between graft and flap:

Skin Grafting:
Partial thickness graft: Thiersch’s graft:

******ebook converter DEMO Watermarks*******

 Includes epidermis and part of dermis.
Graft is thin with better uptake.
Donor site is vast with easy regeneration of epidermis (no special treatment required for donor site).
Contracts up to 40% so not good useful for cosmetic surgery.
Not good for Pressure sites.
Common donor sites are- Thigh (M/C), Upper arm, flexor aspect of forearm, abdominal wall.
Sweat glands and hair follicles are not transferred.
Common cause of graft failure is formation of hematoma below the graft, infection or shearing movement.
Betahemolytic streptococci is absolute C/I for grafting.
Meshing helps in expansion and egress of would fluid.
Meshing increases the size by 1.5 – 1. Times.

Full thickness graft (Wolfe’s graft):

Includes epidermis and dermis.

Uptakes is poorer than split thickness because of thickness.
Only small size can be taken.
Donors site need primary closure and regular dressing.
Minimal contraction so cosmetically better than partial thickness.
Donor sites are:- Eyelid, Post-auricular, Supraclavicular, Antecubital fossa, Axillary, inguinal, Subgluteal fold,
Inframammary fold.

Graft survival:

Classification of flap based on Vessel:

Muscle and Myocutaneous flaps:
Mathes and Nahai classification

One vascular pedicle (eg, tensor fascia lata)

Dominant pedicle(s) and minor pedicle(s) (eg, gracilis).

One dominant pedicle and secondary segmental pedicles (eg, latissimus dorsi)
Two dominant pedicles (eg, gluteus maximus).

Based on Nerve supply:

According to made of innervation (Taylor):
Type I - Single unbranched nerve enters muscle.

******ebook converter DEMO Watermarks*******

Type II - Single nerve, branches prior to entering.
Type III - Multiple branches from same nerve trunk.
Type IV - Multiple branches from different nerve trunks.

Affects suitability for functioning muscle transer.

Skin Flaps:

Unlike a graft, a flap has its own blood supply.

Flaps are usually needed for covering recipient beds that have poor vascularity; reconstructing the full thickness
of the eyelids, lips, ears, nose, and cheeks; and padding body prominences (i.e., for bulk and contour).
A skin flap consists of skin and subcutaneous tissue that are transferred from one part of the body to another
with a vascular pedicle or attachment to the body being maintained for nourishment.
If the flap is pedicled, it is important that the pattern is cut to include the base of the flap and that it is made a
little longer and wider than needed.
The pattern is then tried again, being certain each time that it is shifted so that the base is held in a fixed
position and not allowed to shift with the flap.
The final pattern must be larger than needed, particularly its length, to avoid undue tension and kinking.
Planning a transposition or rotation flap requires special attention to ensure that the most distal part of the flap
is of sufficient length

******ebook converter DEMO Watermarks*******

Local skin flaps are of two types:
Random and Axial Flaps:

Flaps that rotate about a pivot point (rotation, transposition, interpolation flaps).
Advancement flaps (single-pedicle advancement, V-Y advancement, Y-V advancement, and bipedicle
advancement flaps).

Flaps Rotating About a Pivot Point:

Rotation, transposition, and interpolation flaps have in common a pivot point and an arc through which the flap
is rotated.
The radius of this arc is the line of greatest tension of the flap.
The rotation flap is a semicircular flap of skin and subcutaneous tissue that rotates about a pivot point into the
defect to be closed.
A flap that is too tight along its radius can be released by making a short back-cut from the pivot point along
the base of the flap.
A triangle of skin (Burow’s triangle) can be removed from the area adjacent to the pivot point of the flap to aid
its advancement and rotation.
The transposition flap is a rectangle or square of skin and subcutaneous tissue that also is rotated about a pivot
point into an immediately adjacent defect.
Bilobed flap: The key to a successful bilobed flap is an area of loose skin to permit direct closure of the
secondary flap defect.

******ebook converter DEMO Watermarks*******

******ebook converter DEMO Watermarks*******
******ebook converter DEMO Watermarks*******
 The Limberg flap is another transposition flap. This flap, like the bilobed flap and the Z-plasty, depends on the
looseness of adjacent skin.
A Limberg flap is suitable only for closure of rhomboid defects with angles of 60 and 120 degrees.
With the Limberg flap, the sides are of the same length as the short axis of the rhomboid defect.

Advancement Flaps:

All advancement flaps are moved directly forward into a defect without any rotation or lateral movement.
Modifications are the single-pedicle advancement, the V-Y advancement, and the bipedicle advancement flaps.
The single-pedicle advancement flap is a rectangular or square flap of skin and subcutaneous tissue that is
stretched forward.
Advancement is accomplished by taking advantage of the elasticity of the skin and by excising Burow’s
triangles lateral to the flap.
This V-Y technique can be used to lengthen such structures as the nasal columella, eliminate minor notches of
the lip, and, in certain instances, close the donor site of a skin flap.

Skin Grafting:
Skin grafts are divided into 2 major categories:
******ebook converter DEMO Watermarks*******
full-thickness skin grafts (FTSGs) and split-thickness skin grafts (STSGs).
STSGs may be subdivided into:
thin (0.008- to 0.012-mm), a medium (0.012- to 0.018-mm), and thick (0.018- to 0.030-mm) grafts.
STSGs are most commonly used when:

Cosmesis is not a primary concern or when the defect to be corrected is of a substantial size that precludes the
use of an FTSG.
Coverage of chronic unhealing cutaneous ulcers, temporary coverage to allow observation of possible tumor
recurrence, surgical correction of depigmenting disorders with the use of suction blister grafts to line cavities
such as the orbit, and coverage of burn areas to accelerate wound healing and to reduce fluid loss.

The use of FTSGs is indicated:

In defects in which the adjacent tissues are immobile or scarce.

If that adjacent tissue has premalignant or malignant lesions and precludes the use of a flap.
Specific locations for FTSGs include the nasal tip, helical rim, forehead, eyelids, medial canthus, concha, and
digits.
Other indications for the use of FTSG include punch grafting for hair transplantation and minigrafting (punch
grafting) for the surgical correction of depigmenting conditions.

Contraindications:
Contraindications to the use of STSGs include the need to place the graft in areas where good cosmesis or durability
is essential or where significant wound contraction could compromise function.
The use of FTSGs is contraindicated when the recipient bed, due to lack of reasonable vascular supply, cannot
sustain the graft. Using an FTSG on avascular tissues, such as exposed bone or cartilage, most often leads to graft
necrosis.
Uncontrolled bleeding in the recipient bed is another contraindication to the placement of an FTSG because
hematoma and/or seroma formation under the graft compromises graft survival.
Split-thickness skin grafts:
An appropriate donor sites are anterior, lateral, or medial part of the thigh; the buttock; or the medial aspect of the
arm. For larger defects, a large, flat donor surface is ideal for harvesting an STSG.
Full-thickness skin grafts:
Common donor locations for FTSGs include areas of preauricular and postauricular, conchal bowl, supraclavicular,
upper eyelid, nasolabial fold, axillary, antecubital, and inguinal fold skin.
Wound contracture is more common in STSGs than in FTSGs, and it can lead to cosmetic and functional problems.
Named flaps:
Pectoralis major flap: Most commonly used myocutaneousflaf for head and neck. Pectoral branch of
thoracoacromial artery.
Detopectoral flap:
Full thickness fasciocutaneous flap for neck. Based on 1th through 4thbarmches of internal mammary artery.
Trapezius flap:
Transverse cervical artery.
Latissimus Dorsifalp:
Mainly supplied by thoracodorsal artery, also by segmental perforating branches of intercostal and lumber arteries.
TRAM (Transverse Rectus abdominis muscle flap):
******ebook converter DEMO Watermarks*******
Based on superior epigastric artery (Free flap is based on inferior epigastric artery).
Serratus Flap:
Is used for lower chest wall reconstruction (recentaiims question).
Craniosynostosis:

Craniosynostosis involves premature fusion of the cranial sutures.

This results in restricted growth of the skull bones at the involved suture and compensatory growth at the
adjacent patent sutures, causing disfigurement of the cranial shape.
The most common suture involved is sagittal suture (50% to 60%), followed by coronal suture (30% to
35%),metopic suture (5%), and lambdoid suture (2%).
Lambdoid suture synostosis has to be distinguished from positional plagiocephaly because the latter is common
and does not require surgical intervention.
The clinical picture is recognized by the abnormal skull shape associated with each sutural fusion.
Sagittal: elongated skull, or scaphocephaly.
Coronal: Brachycephaly.
Metopic: Trigonocephaly.
Surgical correction involves a wide suturectomy and placement in a cranial remodeling helmet in children
younger than 4 months and a craniotomy and cranial vault reconstruction in older children.
Simple suturectomies have been performed in children younger than 6 months of age under endoscopic
guidance with a small incision.
Patients with coronal craniosynostosis will usually require advancement of the orbital rim in addition to the
cranial remodelling.

Swellings:
Dermoid Cyst:
Dermoid cysts a solitary, or occasionally multiple, hamartomatous tumor. The tumor is covered by a thick
dermislike wall that contains multiple sebaceous glands and almost all skin adnexa. Hairs and large amounts of fatty
masses cover poorly to fully differentiated structures derived from the ectoderm.
In addition to the skin, dermoid cysts can be intracranial, intraspinal, or perispinal. Intra-abdominal cysts, such as
cystic tumors of the ovary or omentum, occur as well.
Causes:
******ebook converter DEMO Watermarks*******
 Dermoid cysts are true hamartomas.
Dermoid cysts occur when skin and skin structures become trapped during fetal development.
Histogenetically, dermoid cysts are a result of the sequestration of skin along the lines of embryonic closure.

History:

Dermoid cysts that are congenital and localized on the neck, head, or trunk are usually visible at birth.
Intracranial, intraspinal, or intra-abdominal dermoid cysts may be suspected after specific or nonspecific
neurologic or gynecologic symptoms occur.

Treatment:
Surgical excision is the treatment of choice in any localization.
Lipoma:

Lipomas are adipose tumors that are often located in the subcutaneous tissues of the head, neck, shoulders, and
back.
These slow-growing, nearly always benign, tumors usually present as nonpainful, round, mobile masses with a
characteristic soft, doughy feel.
Rarely, lipomas can be associated with syndromes such as hereditary multiple lipomatosis, adiposis dolorosa,
Gardner’s syndrome, and Madelung’s disease.
Hereditary multiple lipomatosis, an autosomal dominant condition is found most frequently in men, is
characterized by widespread symmetric lipomas appearing most often over the extremities and trunk.
Gardner’s syndrome, an autosomal dominant condition involving intestinal polyposis, cysts, and osteomas.
Madelung’s disease, or benign symmetric lipomatosis, refers to lipomatosis of the head, neck, shoulders, and
proximal upper extremities. Persons with Madelung’s disease, often men who consume alcohol, may present
with the characteristic “horse collar” cervical appearance.
Dercum’s disease, or adiposis dolorosa, which is characterized by the presence of irregular painful lipomas
most often found on the trunk, shoulders, arms, forearms, and legs.
Dercum’s disease is five times more common in women, is often found in middle age, and has asthenia and
psychic disturbances as other prominent features.
There are also variants such as angiolipomas, neomorphic lipomas, spindle cell lipomas, and adenolipomas.
Most lipomas are best left alone, but rapidly growing or painful lipomas can be treated with excision of the
tumor.

Hemangioma:

A haemangioma is a benign overgrowth of blood vessels in the skin.

It is due to proliferating endothelial cells.
Ten percent of babies develop one or more haemangiomas.
Over 80% occur on the head and neck area.
They can grow for up to 18 months before they start regressing. This regression is known as involution and can
take as long as 3-10 years.
Haemangioma is compressible because it consists of multiple blood-filled vascular spaces.
Other compressible swellings are lymphangiomas, aneurysms, pharyngeal pouch, saphena varix, varicocoele,
pneumatocoele, laryngeocoele, tracheocoele and hernias.
The commonest site of a haemangioma is head and neck region.
It affects internal organs also like liver and spleen.

Different types of haemangioma are:

1. Capillary Haemangioma : Port wine stain, Strawberry angioma, Salmon patch, Spider naevi.
2. Venous Haemangioma (Cavernous haemangioma).
3. Arterial Haemangioma (Circoid aneurysm).
The commonest complication of a haemangioma is Haemorrhage.
******ebook converter DEMO Watermarks*******
Types of haemangiomas:
There are basically two main types of haemangiomas:
Capillary and Cavernous.

Capillary haemangiomas (superficial angiomatous naevi) affect the blood vessels in uppermost layers of the
skin
Cavernous haemangiomas (subcutaneous angiomatous naevi) are more deeply set in the dermis and subcutis.
In some cases, both types of haemangiomas may occur together (mixed angiomatous naevi).

Capillary haemangioma:

The capillary haemangioma or superficial angiomatous naevus is most commonly known as a strawberry
haemangioma (strawberry birthmark, capillary naevus, haemangioma simplex).
It is more common in premature babies and may appear when the baby is a few days or weeks old and rapidly
grows over a few months.
The eventual size varies from a tiny dot to several centimetres in diameter.
Occasionally haemangiomas bleed or ulcerate, but this is rarely serious.
As most strawberry birthmarks disappear without any treatment by themselves over 5-7 years,treatment is
rarely indicated.
If the birthmark grows over the eye, nose or mouth it could interfere with the breathing or feeding problems.
Possible treatment includes oral steroids or laser therapy.
Interferon is no longer advised because it has been associated with the development of cerebral palsy in a few
infants.

Cavernous haemangioma:

This type of birthmark is caused by overgrown blood vessels deep within the skin, resulting in a bluish swollen-
up appearance. They may also grow and then get smaller, sometimes in conjunction with a strawberry mark.
The Kasabach-Merritt syndrome is also known as haemangioma-thrombocytopaenia syndrome. It is a rare
complication of a rapidly growing cavernous haemangioma in the first few months of life.
A defect of blood clotting (coagulopathy) is marked by anaemia, low platelet count and prolonged bleeding.
******ebook converter DEMO Watermarks*******
 The bleeding is thought to result from trapping and destruction of the platelets and depletion of circulating
clotting factors.
The coagulopathy is treated with special blood transfusions, and generally oral steroids to reduce the size of the
haemangioma. The rapid growth of the haemangioma may also result in heart failure.

Other haemangiomas:
The haemangiomas described below are all very rare conditons.

******ebook converter DEMO Watermarks*******

Haemangiomas arising in adults:

Small capillary spots are called Campbell de Morgan lesions (also known as cherry angiomas), and appear most
often around the midtrunk.
They increase in number from about the age of 40. Their cause is unknown.
They can be simply removed by diathermy or laser, but are usually left alone.

Angiomas are common on the face, particularly around the mouth.

On the lip they are known as Venous Lakes, and are bluish in colour.
No treatment is generally required.

******ebook converter DEMO Watermarks*******

Investigations:
Haemangiomas are usually diagnosed clinically and no investigations are necessary. However, when there is
uncertainty about the diagnosis or whether underlying tissues are affected, an ultrasound scan is often performed.
Characteristically, a haemangioma has a firm lobular structure with vessels separating the lobules.
In more complicated cases it may be necessary to perform Magnetic Resonance Imaging (MRI) or angiography to
help plan treatment.
Treatment of a cavernous haemangioma:
Different lines of treatment are:
1. Injection of a sclerosant material (commonest material used is Ethanolamine oleate).
2. Embolization injection (materials used are Gelfoam, alcohol foam & silicon particles).
3. Surgical excision.
4. Laser radiation.
Cystic Hygroma:

A cystic hygroma is a lymphatic malformation (1 in 12,000).

Most (75%) involve the lymphatic jugular sacs and present in the posterior neck region. (Another 20% occur in
the axilla).
Roughly 50% to 65% of hygromas present at birth, and most become apparent by the second year of life.
Occasionally unilocular cysts occur, but more often there are multiple cysts lined by endothelial cells with a
mosaic appearance.
Classically Cystic hygromas are brilliantly translucent
Hygroma may expand to compress the airway.
A diagnosis of cystic hygroma by prenatal ultrasonography before 30 weeks’ gestation has detected a cause of
hidden mortality as well as a condition with a high incidence of associated anomalies, including abnormal
karyotypes and hydrops fetalis.
Occasionally, very large lesions can cause obstruction of the fetal airway. The airway is usually markedly
******ebook converter DEMO Watermarks*******
 distorted, requiring Orotracheal intubation or emergency tracheostomy while the infant remains attached to the
placenta, the so-called “ex utero intrapartum therapy (EXIT) procedure”.
In addition to accumulating lymph fluid, hygromas are prone to infection and hemorrhage within the mass.
Thus, rapid changes in the size of the hygroma may necessitate more urgent intervention.
Complete surgical excision is the preferred treatment. Careful preoperative magnetic resonance imaging (MRI)
to define the extent of the hygroma is crucial.
Because hygromas are not neoplastic tumors, radical resection with removal of major blood vessels and nerves
is not indicated.
Injection of sclerosing agents such as bleomycin or the derivative of Streptococcus pyogenes OK-432
(picibanil) have also been reported to be effective in the management of microcystic variety of cystic
hygromas.

Carcinoma of the Skin:

Skin cancers are the most common forms of cancer.
Basal cell cancers account for nearly two thirds of skin cancer cases, while squamous cell cancers account for 10%
of skin cancers.
Risk Factors:

UV radiation, specifically 280-320 nm UV-B, is the most important risk factor for the development of skin
cancer.
An increased risk is associated with geographic latitude; individuals who live closest to the equator have an
increased risk for the development of skin cancer.
Less common risk factors include exposure to soot (scrotal SCCA in chimney sweeps noted by Sir Percivall
Pott in 1775), tar, polycyclic aromatic hydrocarbons, arsenic pesticides, and pharmaceuticals.
Certain viral factors are also proposed to increase risk for the development of skin cancer (eg, human papilloma
virus [HPV]).
Skin trauma (eg, burns, chronic ulcers) and ionizing radiation also contribute to skin cancer risk.

Clinical Features:
Presentation:

Erythematous, ulcerated, crusting lesion

Area of persistent ulceration
Hyperkeratotic patch
Opaque nodule with or without ulceration
Actinic keratosis (a premalignant condition that may develop into SCCA)

Pathology and Histologic Variants:

The current pathologic designations for premalignant and malignant skin lesions of squamous epithelial origin
are squamous cell carcinoma-in-situ and SCCA.
Bowen disease of the skin and erythroplasia of Queyrat of the penis are clinical expressions of squamous cell
carcinoma-in-situ.
Full-thickness involvement of the epidermis by cells with atypical and dysplastic features characterizes
squamous cell carcinoma-in-situ.
Features include:
loss of orderly maturation as cells progress from basal to superficial layers.
significant variability in nuclear size, shape, and staining between neighboring cells.
mitoses at higher than expected levels.
multinucleation.
dyskeratosis, hyperkeratosis, and parakeratosis.
Lesions with features that fall short of full-thickness involvement are characterized as actinic (solar) keratosis.

******ebook converter DEMO Watermarks*******

By definition, SCCA is a malignant squamous neoplasm in which the cells have penetrated the epithelial basement
membrane and invaded the dermis for a variable distance.
Variants of SCCA are named according to their architectural features, including:

Spindle cell type/ adenoid type/ and verrucous type.

The spindle cell variant has large vesicular nuclei, indistinct cytoplasmic borders, and a spindled pattern, often
resembling dermal sarcomas.
The adenoid (acantholytic) variant consists of nests of squamous cells with pseudoglandular formations
secondary to central acantholysis.
Verrucous carcinomas are both exophytic and endophytic.
The exophytic component displays papillomatosis, hyperkeratosis, and parakeratosis.
The endophytic component manifests as acanthotic extensions of rete pegs with rounded appearance.

Diagnosis:

Punch biopsy.
incisional biopsy.
Excisional biopsy.

Treatment:
Poor prognostic factors:
1. Size greater than 2 cm.
2. Depth greater than 4 mm.
3. Histology - Poorly differentiated/ Rapid growth.
4. Etiology - Burn, scar, and chronic ulcer/ Immunosuppressed patients.
5. Anatomic site - Scalp, nose, lip, eyelid, and ear (The ear is the primary site for aggressive tumor behavior).
6. Perineural invasion.
7. Recurrent lesions.
1. Surgical excision and primary closure occur under local anesthesia. The standard treatment includes 4- to 6-
mm margins for 95% nonrecurrence rate.
2. Mohs micrographic surgery offers better cure rates for lesions associated with high-risk factors. The surgery is
performed using sequential excisions and histologic examination of the entire surgical margin. Subsequent
excisions are performed only of the areas with persistent disease.
3. Radiation is reserved for unusual cases.
4. Topical chemotherapy with 5-fluorouracil may be useful for certain patients.
Risk factors for metastatic disease to regional lymph nodes:
1. include primary site tumor greater than 2 cm.
2. depth greater than 6 mm.
3. rapid growth.
4. immunocompromised host state, anatomic site (eg, ear, temple, lip).
5. perineural invasion.
General guidelines for regional control include the following:

Scalp, forehead, temple, and auricle may drain to paraparotid or intraparotid lymph nodes and to deep cervical
nodes.
Neck dissection is not usually indicated for patients with N0 necks. Monitor these patients, especially for the
first 2 years. Rarely, prophylactic radiation to the neck is considered.
Metastatic disease to the parotid region requires parotidectomy in conjunction with neck dissection.
If cancer involves the skin or a scar from a previous excision or biopsy, include these areas in the surgical
specimen.
Preserve the facial nerve, unless the nerve is invaded directly by a tumor. If the nerve is resected, make every

******ebook converter DEMO Watermarks*******

 attempt to reconstruct the nerve using primary anastomosis, cable graft, or hypoglossal transfer.
Patients who have distal metastatic disease do poorly. Combination surgery, radiation, and chemotherapy may
benefit selected patients.

Head and Neck Cancer:

Squamous cell carcinoma represents more than 90% of all head and neck cancers.
Pathophysiology:

Squamous cell carcinoma is thought to arise from keratinizing or malpighian epithelial cells.
The hallmark of squamous cell carcinoma is the presence of keratin or “keratin pearls” on histology.
These are well-formed desmosome attachments and intracytoplasmic bundles of keratin tonofilaments.
Morphologically, it is variable and may appear as plaques, nodules, or verrucae.
These in turn may be scaly or ulcerated, white, red, or brown.
Verrucous carcinoma has a more favorable prognosis because of infrequent nodal and distant metastasis.

Relevant Anatomy:

The oral cavity is defined as the area extending from the vermilion border of the lips to a plane between the
junction of the hard and soft palate superiorly and the circumvallate papillae of the tongue inferiorly.
This region includes the buccal mucosa, upper and lower alveolar ridges, floor of the mouth, retromolar
trigone, hard palate, and anterior two thirds of the tongue.
The tongue (lateral border) is the most common site of malignancy in the oral cavity.
In India Buccal cavity is the most common site.
Lips: Squamous cell carcinoma is the most common histologic type, with 98% involving the lower lip.
This predilection to the lower lip has been attributed to sun exposure.

Tumor site and lymphatic drainage:

Anterior tongue to subdigastric, submaxillary, or midjugular nodes.

Floor of mouth to subdigastric, submaxillary, or midjugular nodes.
Gingival to jugulodigastric, submaxillary, or midjugular nodes.
Buccal mucosa to submaxillary, preparotid, or jugular nodes.
Hard palate to submaxillary or jugulodigastric.

Treatment:
Several methods for treatment of cancer of the head and neck are acceptable, including surgery, radiotherapy,
chemotherapy, and combinations of these.
Radiotherapy:

Nearly all patients with advanced disease require adjuvant radiotherapy, preoperatively or postoperatively.
Radiation dosage in excess of 6000 cGy is recommended with a boost to areas of high risk.
Indications for radiotherapy include a bulky tumor with significant risk of recurrence (T3 and T4),
histologically positive margins, and perineural or perivascular invasion of tumor.
For the neck, indications for radiotherapy include elective treatment of the N0 neck not treated surgically where
risk of micrometastasis is high, gross residual tumor in the neck following neck dissection, multiple positive
lymph nodes, and extranodal extension of tumor.

Chemotherapy:
Bleomycin with or without electroporation has been used. Cisplatin is another chemotherapeutic drug of choice for
head and neck cancers.
Surgical therapy:

******ebook converter DEMO Watermarks*******

 WIDE LOCAL EXCISION with 2cm tumor free margin with Neck dissection is preferred.
Surgical resection remains the criterion standard for treatment of head and neck cancer.
Management of all but the earliest confirmed neck metastases is best achieved with surgical removal.

Neck dissection:

Regardless of the site of the primary tumor, the presence of a single lymph node in either the ipsilateral or
contralateral side of the neck reduces the 5-year survival rate by 50%.
Modified neck dissection is designed to preserve the spinal accessory nerve, the great auricular, and the
sternocleidomastoid muscle ,jugular vein and submandibular gland also have been preserved.
Classic radical neck dissection was described by Crile in 1901 and includes removal of all 5 levels of cervical
lymph nodes en bloc down to the deep muscular fascia.
This removal includes the sternocleidomastoid muscle, submandibular gland, jugular vein, and spinal accessory
nerve.
This operation remains the best procedure for definitive control of neck disease.
Radical neck dissection can be combined with resection of the primary cancer and postoperative radiation
therapy.

Basal cell carcinoma:

There are four main clinical types of basal cell carcinoma.
1. nodular.
2. superficial.
3. morpheaform.
4. pigmented.
Nodular basal cell carcinoma, the most common type, is a waxy, semitranslucent papule or nodule.
The border is often pearly and rolled, and telangiectasias course over the surface of the lesion. Eventually, central
ulcerations (rodent ulcers) develop.
Superficial basal cell carcinoma usually occurs as a slightly raised, pink or red, scaly, focally crusted plaque with a
threadlike border.
Morpheaform basal cell carcinoma appears as an ivory plaque with overlying telangiectasias. This lesion may be
more difficult to treat than other basal cell cancers.
Pigmented basal cell carcinoma is similar to the nodular and superficial variants but has brown or black
pigmentation.It may be difficult to differentiate from melanoma.
Malignant Melanoma:
Melanoma is a malignancy of pigment-producing cells (melanocytes) occurring in the skin, eyes, ears, GI tract,
leptomeninges of the central nervous system (CNS), and oral and genital mucous membranes. Melanoma accounts
for only 4% of all skin cancers.
Consider lesions exhibiting these features to be potential melanomas:

Asymmetry.
Border notching.
Color variegation with black, brown, red, or white hue.
Diameter > 6 mm.

Types of Malignant Melanoma:

There are four main types of melanoma.

Superficial spreading melanoma.

Nodular melanoma.
LentigoMaligna melanoma (also sometimes called Hutchinson’s melanotic freckle).

******ebook converter DEMO Watermarks*******

 Acral lentiginous melanoma.

These four main types make up 90% of all diagnosed malignant melanoma.
Superficial spreading melanoma:

This is the most common type of melanoma (65-70%).

They are most common in middle aged people.
To start with, they have a radial growth phase (grows in a horizontal plane, along, just above and below the
dermo-epidermal junction) and is clinically macular or only slightly elevated.
The melanoma will not become dangerously at risk of spreading until it begins to grow downwards into the
deeper layers of skin and beyond.
Most common on the trunk in men and women and on the legs in women.

Nodular melanoma:

About 1 in 4 melanomas (25%) are of this type.

It is also found most often in middle aged men > women.
most often found on the chest or back.
Nodular melanomas are often present as bluish black papules.
The depth of the lesion appears to correlate with the prognosis of the patient, and nodular melanoma is less
often amenable to definitive treatment than is the superficial spreading variety.

Lentigomaligna melanoma:

About 1 in 10 melanomas (10%) are this type.

Lentigo MM is most common in elderly people and in parts of the body only exposed to the sun.
Associated with HUTCHINSON’s Melanoticfreckels.
It appears in areas of skin that get a lot of sun exposure, so is commonest on the face.
This type of melanoma grows very slowly, so it may be gradually getting bigger over several years. This lesion
may grow for years as an in-situ tumor before developing the more aggressive vertical growth phase.
In situ precursor lesion usually large (>3 cm diameter), existing for a minimum of 10-15 years, with dermal
invasion characterized by development of dark brown-to-black macular pigmentation or raised blue-black
nodules.

Acral lentiginous melanoma:

This type is most commonly found on the palms and soles or around the big toenail.
It can also grow under the nails.
It is much more common on the feet than on the hands.

Other types of melanoma:

Melanoma can occur anywhere in the body, including in the internal organs. One area where melanoma does occur
is eye. Rare melanoma variants (<2% of melanomas) include the following:

Desmoplastic/neurotropic melanoma.
Mucosal (lentiginous) melanoma.
Malignant blue nevus.
Melanoma arising in a giant congenital nevus.
Melanoma of soft parts (clear cell sarcoma).

Amelanotic melanoma (<2% of melanomas) characteristics are as follows:

Nonpigmented and appearing clinically as pink or flesh colored.

Most commonly occurs in the setting of melanoma metastasis to the skin, presumably because of the inability
of these poorly differentiated cancer cells to synthesize melanin pigment.
******ebook converter DEMO Watermarks*******
The five year survival for tumours <0.75 mm is 95-99%, 0.76-1.49 mm is 80-90%, 1.5-3.99 mm is 60-75%, >4.0
mm is <50%.

Melanomas in men are most common on the back. In women, the commonest site is the legs.

Two genodermatoses, xerodermapigmentosum and familial atypical mole melanomasyndrome, confer a 500-
fold or greater relative risk of developing melanoma.
arise from preexisting nevi; 1% of all cancers.
30–40% mortality.
metastases: latent period of 2–20 years (most commonly 2–5 years).
lymphadenopathy.
in 23% with level II + IV.
in 75% with level V.
bone (11–17%).
often initial manifestation of recurrence; poor prognosis.
axial skeleton (80%); ribs (38%).
lungs (70% at autopsy).
most common site of relapse; most common cause of death.
liver (58% at autopsy): may be calcified, necrotic.
spleen (1–5%): solid or cystic.
bowel + mesentery (8%): mostly in small bowel.
kidney (35%); adrenals (50%); subcutis

Clark staging:

level I: all tumor cells above basement membrane (in situ).

level II: tumor extends to papillary dermis.
level III: tumor extends to interface between papillary and reticular dermis.
level IV: tumor extends between bundles of collagen of reticular dermis.
level V: tumor invasion of sucutaneous tissue (87% metastases).

Breslow staging:

thin: < 0.75 mm depth of invasion.

intermediate: 0.76 – 3.99 mm depth of invasion.
thick: > 4 mm depth of invasion.

Malignant melanoma: gallium imaging:

>50% sensitivity for primary and metastatic sites:

73% sensitivity if lesion is > 2 cm.

17% sensitivity if < 2 cm.

******ebook converter DEMO Watermarks*******

Surgical Care:
Surgery is the primary mode of therapy for localized cutaneous melanoma.
Surgical margins for primary melanoma:

Surgical margins of 5 mm currently are recommended for melanoma in situ, and margins of 1 cm are
recommended for melanomas up to 1 mm in depth (low-risk primaries).
Randomized prospective studies show that 2-cm margins are appropriate for tumors in the intermediate-risk
group (1-4 mm in Breslow depth), although 1-cm margins have been proposed for tumors of 1- to 2-mm
thickness.
Margins of at least 2 cm are recommended for cutaneous melanomas greater than 4 mm in thickness (high-risk
primaries) to prevent potential local recurrence in or around the scar site.

Elective lymph node dissection:

Prophylactic lymph node dissection for primary cutaneous melanoma of intermediate thickness initially was
believed to confer a survival advantage on patients with tumors 1–4 mm in depth. Subsequent clinical trials
have shown no survival benefit for elective lymphadenectomy for melanomas of varying thicknesses on the
extremities and marginal melanomas.

Sentinel lymph node biopsy/dissection:

Lymphatic mapping and sentinel node biopsy effectively have solved the dilemma of whether to perform
regional lymphadenectomy (in absence of clinically palpable nodes) in patients with thicker melanomas (>1
mm in depth).
The sentinel node is examined for the presence of micrometastasis on both routine histology and with
immunohistochemistry; if present, a therapeutic completion lymph node dissection is performed.
A negative sentinel node biopsy prevents the morbidity associated with an unnecessary lymphadenectomy,
since the histology of the sentinel node is characteristic of the entire nodal basin.

Melanoma Surgery:
Resection margins:

Until recently history rather than controlled trials have dictated practice.
Handley (1907).
Hunterian Lecture based on one case.
Recommended 5 cm margin.
Butterworth and Klaude (1934).
Found microscopic lymphatic invasion to 3 cm.
Recommended 5 cm resection margins
Olson (1966).
Trial of resection 1 cm vs. 3 cm resection margins.
Identical local recurrence rate but still recommended 5 cm margin!
WHO Melanoma Group (1990).
Randomised controlled trial of 1 cm vs. 3 cm resection margins.
Resection margins did not influence survival.
Generally accepted resection margins based on clinical appearance are:
Impalpable lesions – 1 cm margin.
Palpable lesion – 2 cm margin.
Nodular lesion – 3 cm margin.

Regional lymphadenectomy:

20% clinically palpable nodes are histologically negative

******ebook converter DEMO Watermarks*******
 20% palpably normal nodes have occult metastases
Therapeutic lymph node dissection provides regional control and prognostic information
No improvement in survival
For tumours <0.75 mm thick – 90% cured by local excision alone
For tumours > 4.0 mm thick – 70% have distant metastases at presentation
For these two groups lymphadenectomy provides no added survival benefit
Lymphadenectomy for ‘intermediate’ thickness tumours controversial.

Morbidity of lymphadenectomy:

Adjuvant Therapy:

Patients at high risk of recurrence should be considered for systemic adjuvant therapy.
Patients include those with.
Primary tumour > 4 mm thick.
Resectable positive locoregional lymph nodes.
No standard adjuvant therapy exists.
Interferon a2b has shown promising results.
Shown to increase disease-free and overall survival.

Isolated limb perfusion:

Intra-arterial chemotherapy.
Commonly used agents - melphalan +/− TNF-alpha.
Used with hyperoxygenation.
Hyperthermia with a temperature of 41-42 °C.
Perfusion generally last about 1 hour.
Usually combined with lymphadenectomy.

Indications:

Intransit metastases.
Irresectable local recurrence.
Adjuvant therapy for poor prognosis tumours.
Palliation to maintain limb function.

******ebook converter DEMO Watermarks*******

Bariatric Surgery:
Classification of Obesity:

******ebook converter DEMO Watermarks*******

Types of Surgeries:

Lparoscopic Adjustable Gastric Banding (LAGB):

Surgery involves putting an adjustable inflatable silastic band around the upper stomach leaving a small pouch
just below the cardia.
Band is connected to a port which is placed subcutaneously at umbilicus.
The degree of restriction can be controlled by the amount of fluid injected into the subcutaneous port.
The perception that the band is reversible is important to some patients (although in reality it is a disadvantage).
Gastric banding is certainly the least risky procedure (0.1 per cent perioperative mortality) as it does not
involve cutting any stomach or bowel and is a relatively easy operation to perform in most patients who have a
BMI <50 kg/m2.
Most patients can expect to lose around 45–50 per cent of their excess weight, especially if the quality of

******ebook converter DEMO Watermarks*******

 follow up is intensive.

Complications:
1. PROLAPSE:ands can fail due to prolapse of the stomach through the band.
2. BAND SLIPPAGE: the band can slip up or down from its initial position.
3. Bands can also erode into the stomach.
4. Failure to loose weight.
One disadvantage of the gastric band is the need for continual band adjustments in the early postoperative period
and occasional long-term adjustments.

Laparoscopic Sleeve gastrectomy(LSG):

Recent most and most popular technique.

The stomach is divided along the greater curvature over a 32 Fr bougie towards the angle of HIS.
The long staple line can leak despite various manoeuvres to avoid leakage, such as applying reinforcing
material and/or gluing.
Another attraction of this procedure is that as it removes most of the grehlin-secreting area of the stomach it
may have a beneficial effect on reducing appetite.
Around 65 per cent excess weight loss can be expected at two years which is as good as gastric bypass without

******ebook converter DEMO Watermarks*******

 any malabsorption issues.
However, there is a tendency for the sleeve to expand over time.
A resleeving procedure is well described or alternatively it is relatively straightforward to convert the procedure
to a gastric bypass or a BPD with a duodenal switch.

Laparoscopic Roux En Y Gastric Bypass:

This is most prefered surgery worldwide.

It produces 65–75 per cent excess weight loss albeit at a higher risk of around 0.5 per cent perioperative
mortality.
Gastric bypass is a very effective operation for alleviating and even curing type II diabetes; reflux; obstructive
sleep apnea.
There are many variations in the actual gastric bypass technique, e.g. antecolic versus retrocolic Roux limb
placement, varying alimentary and biliary limb lengths, additional banding of the gastrojejunal anastomosis to
prevent dilatation, varying methods of doing the gastrojejunal anastomosis and varying methods of closing
potential hernia spaces.
Although weight loss is generally very good the downside of this procedure is the need for careful
postoperative metabolic complications.
The major feature of the operation is a proximal gastric pouch of small size (often <20 mL) that is totally
separated from the distal stomach.
A Roux limb of proximal jejunum is brought up and anastomosed to the pouch.
The pathway of that limb can be anterior to the colon and stomach, posterior to both, or posterior to the colon
and anterior to the stomach.
The length of the biliopancreatic limb from the ligament of Treitz to the distal enteroenterostomy is 20 to 50
cm, and the length of the Roux limb is 75 to 150 cm.
Longer Roux limb lengths are performed but considered to have more malabsorption than the standard gastric
bypass.

Bilio-Pancreatic Diversion:

******ebook converter DEMO Watermarks*******

 This procedure, produces the most malabsorption of all operations, is the most effective with 75–85 per cent
excess weight loss but at the expense of the highest perioperative mortality of 1–2 per cent.
Additionally as time goes by, if the patient does not adhere to their vitamin and micronutrient supplementation
regime they are at severe risk of many deficiency syndromes.
Hypoproteinemia is the mc nutritional deficiency.
Due to the extreme malabsorption these operations produce there is a need for a high protein intake of around
90 g/day which many patients find difficult. If this is not achieved then protein calorie malnutrition can be a
problem.
In correctly selected patients however, the BPD can be very effective, especially in those patients with a very
high BMI.
A BPD also has the same rapid effect as a gastric bypass for alleviating diabetes independent of weight loss.
The duodenal switch variation of the BPD was designed to reduce the need for taking vitamin B12 and reduce
the incidence of anastomotic strictures at the gastrojejunal anastomosis.
In the standard BPD, approximately two-thirds of the distal stomach is removed while in the duodenal switch
variation there is a vertical sleeve gastrectomy.
In the duodenal switch variation, the anastomosis is made to the first part of the duodenum rather than the
stomach as in the standard BPD.
Terminal 200-250 cm of ileum is anastamosed with small gastric pouch in BPD and biliopancreatic limb is
anastamosed 75cm proximal to ileum in BPD.
Very similar approach is used in duodenal switch, where the anastomosis is done with 1st part of duodenum and
biliopancreatic limb approx 100cm proximal to IC junction.

Complications of bariatric surgery:

******ebook converter DEMO Watermarks*******

******ebook converter DEMO Watermarks*******
Fluid and Electrolytes:
Distribution of body fluids:

Total Body Water (TBW) = 50 70% of body weight (BW).

Depends upon.
Lean body mass.
Age.

New born – 75%.

1 Year – 65% (Constant throughout childhood).
Adult Male – 60%.

Adult Female 55% (more s/c fat & small muscle mass).
(Blood volume= roughly 7–8% body wt).

Fluid Compartments:
Rapidly equilibrating with each other:
1. Intracellular fluid (ICF) - 40% of Body Weight.
2. Extracellular fluid (ECF) - 20% of Body Weight.
a. Interstitial fluid (IF) - 14-15%BW.
b. Intravascular fluid (IVF)-5%-6% BW.
3. Third space fluid & transcellular fluid – fluid outside the first two compartments.

******ebook converter DEMO Watermarks*******

Osmolarity (Osmotic pressure):

Depends upon actual number of osmotically active particles in the solution and not their size.
ECF osmolality (mosm/Kg) = 2 (Na+) mEq/L + Glucose/18 rag/dL + BUN/2.8 mg/dl Normal serum osmolality
is 285-290 mosm/L.

Colloid Osmotic pressure (COP) (oncotic pressure):

It is the osmotic pressure generated by the presence of colloid on one side of a membrane which is impermeable to
them. It is primarily responsible for effective osmotic pressure between the plasma and the interstitial fluid
compartment. It is normally about 25 mmHg and tends to draw fluid into the intravascular compartment.
Composition of GI fluid loss:

******ebook converter DEMO Watermarks*******

Daily electrolyte requirements & commonly use I/V fluids:

Electrolytes Imbalance:
Sodium balance:
Normal range - 135 - 145 mEq/L.
Hyponatremia.

******ebook converter DEMO Watermarks*******

Types:
1. Hypovolemic hyponatremia [TBS i].

Severe isotonic dehydration (ECF volume loss).

2. Hypervolemic hyponatremia [Edematous states TBS t].

Occurs in conditions of

a. Cirrhosis, CHF, nephrotic syndrome - ‘Effective’ volume decreases because of low cardiac output (CHF)
or sequestration of fluid outside the central circulation e.g.-↓ plasma oncotic pressure resulting in reduced
renal perfusion.
b. Acute stress, trauma, hypovolemia - endocrine response to injury.
3. Isovolemic hyponatremia [TBS normal].
a. SIADH is the most prevalent etiology of euvolemic hyponatremia.
i. Specific diagnostic criteria that define SIADH include the following:

Hyponatremia.
Hypotonicity.
Inappropriately concentrated urine.
Elevated urine sodium concentration.
Clinical euvolemia.
Normal renal, adrenal, and thyroid function.
No edema.

ii. Excess ADH may emerge from the pituitary gland or an ectopic source:

CNS disorders: Head trauma. Stroke, Neonatal hypoxia, Brain tumor, Hydrocephalus, Cerebral abscess.
Meningitis, Encephalitis, Subarachnoid hemorrhage e.t.c.
Malignancies: neoplasms with a potential to synthesize, store, and secrete ADH (eg, increased levels of ADH
found in -60% of patients suffering from small cell carcinoma of the lung). Others: Brain, Pancreas, Prostate,
Ovary, Lymphoma, Leukemia, Thymoma.
Pulmonary disease: Pneumonia. Tuberculosis, Empyema, Abscess, Asthma, COPD.
Endocrine disorders: Hypothyroidism / myxedema, deficiency.
Drugs.

Analgesics (eg, narcotics, NSAIDS).

Antidepressants (eg, MAO inhibitors, tricyclic antidepressants, selective serotonin reuptake inhibitors).
Barbiturates.
Carbarnazepine.
Cyclophosphamide Clofibrate.
Diuretics (especially thiazides).
Neuroleptics (eg, phenothiazines).
Oral hypoglycemics (eg, chlorpropamide, tolbutamide).
Treatment: For asymptomatic or mildly symptomatic hyponatremia, water restriction.
Demeclocycline Interferes with action of ADH at renal collecting duct.

C/F of hyponatremia:

Neurological dysfunction.
Intracellular movement of water -brain cell edema ↓→flCT→HT, lethargy, confusion, coma. Later tissue signs
of excessive intracellular water e.g. - “Finger printing sign”.
If Hyponatremia develops rapidly, signs of hyper excitability - irritability, muscular twitches & hyperactive
deep tendon reflexes.

******ebook converter DEMO Watermarks*******

Treatment:
Asymptomatic hyponatremia are managed with free water restriction.
Severe, Symptomatic hyponatremia should be treated with hypertonic saline.
Rapid correction of hyponatremia can pontine myelinolysis.
Once S. Sodium levels reach 130mEq/L, the further correction is carried out more slowly by water restriction.
Hypernatremia:
Causes:
Solely due to water loss:

Extrarenal insensible loss- skin/lungs.

Renal-Diabetes insipidus.

Water loss with sodium loss:

Extrarenal- sweat.
Renal osmotic diuresis-glycosuria, urea.

Due to sodium gain:

Iatrogenic.
Adrenal hyperfunction hyperaldosteronism, Cushing’s.

Principal C/F:
CNS system.
Due to dehydration of brain cells.

neuromuscular excitability, twitching, seizures, stupor & coma.

Tissue signs – “dry sticky mucous membranes” are characteristic of this conditionally.
Treatment:
I/V infusion of free water (5% dextrose).
Correction advised slowly over days.
Rapid correction may lead to cerebral edema.
Potassium balance: hyperkalemia & Hypokalemia.
98% of potassium is intracellular, with the concentration gradient maintained by the sodium- and potassium-
activated adenosine triphosphatase (Na+/ K+−ATPase) pump that is controlled by insulin and p-2 receptors.
Cellular concentration is approx 40 times the ECF.
The normal potassium level is 3.5-5.0 mEq/L.
Potassium balance:
Minute-to-minute levels of potassium are controlled by intracellular to extracellular exchange. A balance of GI
intake and renal potassium excretion achieves long-term potassium balance. All regulation of K+ excretion occurs at
distal nephrons.The excess K+ is excreted prompdy. The filtered K+ is nearly completely reabsorbed in the proximal
segments and the K+ in urine is derived almost entirely from K+ secreted in the distal convoluted tubules. K+
secretion is influenced by:

******ebook converter DEMO Watermarks*******

 Aldosterone.
Distal tubular fluid flow rate.
Increased distal delivery of fluids, e.g. loop diuretics, favors K+ excretion.
Acid base balance.
Alkalosis enhances and acidosis depresses renal potassium secretion, probably by inducing corresponding
changes in tubular cell potassium.

Hyperkalemia:
Defined as a potassium level greater than 5.5 mEq/L.
Causes:

Pseudohyperkalemia.
Sample hemolysis.
Redistribution.
Acidosis.
Insulin deficiency.
Drugs.
Beta-blockers.
Acute digoxin intoxication or overdose.
Succinylcholine (releases K* from muscles by depolarizing cell membranes).
Arginine hydrochloride - used to treat metabolic acidosis (drives K+ out of cells).
Hyperkalemic familial periodic paralysis -1 [K+] is associated with repeated attacks of muscle paralysis.
Mechanism obscure.
Excessive endogenous potassium load.
Trauma.
Burns.
Hemolysis.
Excessive exogenous potassium load.
Diminished renal potassium excretion (principal cause).
Potassium-sparing diuretics (spironolactone, triamterene, amiloride).
Effective circulating volume.
Laboratory error.

History:

Patients may be asymptomatic or report the following:

Generalized fatigue & weakness
Paresthesias
Paralysis
Palpitations

ECG changes:

Peaked (tented) T waves (earliest).

PR interval prolongation.
QRS widening.
Loss of P wave.
Sine wave.
Ventricular fibrillation or cardiac arrest in asystole.

Treatment:
1. Calcium: Calcium chloride or calcium gluconate.
2. Alkalinizine agents: Sodium bicarbonate

******ebook converter DEMO Watermarks*******

3. Beta-agonists: Albuterol.
4. Loop diuretics: Purosemide (Lasix).
5. Binding resins: Sodium polystyrene sulfonate (Kavexalate).
6. Dialysis.
Hypokalemia:
Hypokalemia is defined as a plasma potassium level of less than 3.5 mEq/L.
Causes:

Gasrointestinal losses.
Gastrointestinal losses from vomiting, diarrhea, NG suction e.t.c.
Renal.
Metabolic alkalosis (excess bicarbonate delivery to the distal nephron).
Diuretics (MC cause of |[K*].
Thiazides.
Loop diuretics.
CA inhibitors.
Excessive mineralocorticoid effects.
Renal tubular disease.
Hypokalemia due to shifts into the cells (no depletion).
Hypokalemic periodic paralysis - sudden movement of potassium into the cells.
Insulin effects.
Alkalosis.
Increased β- adrenergic activity (or ↓α - adrenergic activity).
rugs.
P-agonists and a-blockers.
Theophylline.
Verapamil (with overdose).
High-dose penicillin.
Ampicillin.
Carbenicillin.

Signs of ↓K*:

Absent tendon reflexes &paralaytic ileus.

A Complications:

Cardiac arrhythmias and acute respiratory failure from muscle paralysis.

TT sensitivity to digitalis → arrhythmias

Lab Studies:

Serum electrolytes
Unlike hyponatremia, serum potassium may not accurately reflect total body stores.
Blood gas analysis.
Assess acid-base status.
Alkalosis may induce hypokalemia.

Other Tests:

ECG:
T-wave flattening.
Appearance of U waves.

******ebook converter DEMO Watermarks*******

 ST-segment depression.
Prolongation of PR interval.
QT prolongation.
Cardiac arrest in systole.
Symptomatic or severe hypokalemia should be corrected with a solution of intravenous potassium.
Potassium chloride – First choice for IV therapy. 10-40 mEq IV infused over 2-3 h; not to exceed 10 mEq/h (40
mEqA with monitoring).
Calcium regulation; Hypercalcemia & Hypocalcemia.
Approximately 99% of calcium is found in bone, and 1% is found in extracellular fluid. Of this 1 %, 50% is in
the free (active) ionized form (1-1.15 mmol/L), 40% is bound to protein (predominantly albumin), and 10%
circulates bound to anions (phosphate, carbonate, citrate, lactate, sulfate).
Normal S. calcium levels - 8 to 10 mg / dL (2.0 to 2.5 mmol/ L or 4 to 5 mEq/L).
Normal ionized calcium levels - 4 to 5.6 mg / dL (1 to 1.4 mmol / L).
Clinical signs and symptoms are observed only with decreases in ionized calcium concentration.
Alkalemia increases binding, thereby decreasing the ionized calcium. Plasma [Ca2+] is maintained within
the reference range by a complex interplay of 3 major hormones, PTH, 1,25-dihydroxyvitamin D (i.e.
calcitriol), and calcitonin.

Hypercalcemia:

Hypercalcemia is defined as a serum calcium level greater than 10.5 mg/dL.

Causes:

Hyperparathyroidism is overall the most common cause of hypercalcemia. Malignancy is the second most
common cause.
Hyperparathyroidism is the most common cause of hypercalcemia on routine screening.
Malignancy is the commonest cause of hypercalcemia in hospitalized patients.

PTH-mediated hypercalcemia (Primary hyperparathyroidism).

It is overall the commonest cause of hypercalcemia. Usually in 3-5 decades.

The incidence of primary hyperparathyroidism is considerably higher in women.

Non-PTH-mediated hypercalcemia:
Malignancies:

Hypercalcemia is the most common life-threatening metabolic disorder associated with neoplastic diseases.
Bronchogenic carcinoma (MC), followed by CA Breast, RCC, and hematological disorders e.g. multiple
myelomas, leukemias or lymphomas (specially theT cell variant).
******ebook converter DEMO Watermarks*******
 The remaining 10% of cases of hypercalcemia are caused by many different conditions, including vitamin
D-related problems, disorders associated with rapid bone turnover, thiazides or renal failure, and, in rare
cases, familial causes.

Causes related to vitamin D:

Vitamin D toxicity.
Excessive ingestion of vitamin D t intestinal calcium absorption.
Granulomatous disease (especially sarcoidosis).
Abnormal metabolism of vitamin D.
Idiopathic infantile hypercalcemia (Williams syndrome).

Causes related to high bone turnover:

Hyperthyroidism.
Immobilization.
Thiazides.
Vitamin A intoxication.

Causes related to renal failure:

Severe secondary hyperparathyroidism due to progressive renal damage.

Milk-alkali syndrome.
Aluminum intoxication as occurs in patients with chronic dialysis.

Other causes related to particular mechanisms:

Decreased renal calcium excretion (or increase renal calcium reabsorption).

Familial hypocalciuric hypercalcemia.
Thiazide diuretics.
Hypophosphatasia.

Signs and symptoms:

Symptoms relate to CNS, renal, GI, and cardiac. CNS symptoms are the most.
common and the earliest symptom usually is lethargy or feeling tired.
Central nervous system:
Irritability.
Memory loss.
Apathy.
Depression.
Dementia.
Lethargy.
Confusion.
Coma.
Renal effects:
Polyuria.
Nocturia.
Volume contraction.
Thirst.
Gastrointestinal effects.
Anorexia.
Pain.
Nausea.
Vomitings.

******ebook converter DEMO Watermarks*******

 Constipation (due to dehydration) & Fecal impaction.

ECG Changes:

QT interval shortening.
PR interval prolongation.
QRS interval lengthening (at very high levels).
T waves flattening or inversion(at very high levels).
A variable degree of heart block.(at very high levels).
Digoxin effects are amplified.

Treatment:

Volume expansion with NS followed by Loop Diuretics.

Inhibition of bone resorption.
Calcitonin.
Bisphosphonates.
Mithramycin (Plicamycin).
Gallium nitrate.
Mobilization.
Reduction of gastrointestinal calcium absorption.
Reduction of dietary calcium.
Oral phosphate - forms insoluble calcium phosphate in the gut.
Dialysis.

Surgical Care:

Parathyroidectomy is the definitive treatment for hyperparathyroidism.

Hypercalcemia due to malignancy may require surgical resection of the tumor.

Hypocalcemia:

A serum calcium level less than 8.5 mg/dL.

Causes:

PTH deficiency.
Vitamin D deficiency.
Miscellaneous disorders.
Acute pancreatitis.
Toxic shock syndrome.
Hypoalbuminemia.
Infiltrative disease: Sarcoidosis, tuberculosis may infiltrate & dysfunction parathyroids.
Drugs.
Calcitonin and bisphosphonates.
Diuretics - Furosemide.
Estrogen inhibits bone resorption.

Clinical features:

Numbness and tingling sensations in the perioral area (earliest sign).

Muscle cramps; may progress to carpopedal spasm (i.e. tetany).
Neurological symptoms, including irritability, confusion, depression, and personality changes, hallucinations,
dementia, extrapyramidal manifestations, and seizures.
Respiratory - laryngospasm and bronchospasm.
******ebook converter DEMO Watermarks*******
 Subclinical tetany.
Chvostek sign.
Trousseau sign.
ECG changes:
Q T interval-used to monitor serum calcium.

Medical Care:
10 ml of 10% calcium gluconate.
Vit D supplement & Calcium.
PTH if required.
Magnesium.

Magnesium (Mg) is the second-most abundant intracellular cation, overall, the fourth-most abundant cation.
The intracellular concentration is 40 mEq/L, while the normal serum concentration is 1.5-2.0 mEq/L. Serum
levels do not necessarily reflect the status of total body stores.
Approx. 60% of total body magnesium is located in bone, 38% in the soft tissues (intracellular) and only less
than 2% is present in the ECF compartment. Of this serum component, 30% is protein bound, 20 % is
complexed, and the remaining 50% is ionized. Analogous to plasma calcium, the free (ie.ionized) fraction of
magnesium is the active component.
Almost all enzymatic processes using phosphorus as an energy source (eg, adenosine triphosphatase [ATPase])
require magnesium for activation. It is involved in nearly every aspect of biochemical metabolism (eg,
deoxyribonucleic acid [DNA] and protein synthesis, glycolysis, oxidative phosphorylation).
Magnesium is a component of chlorophyll and is present in high concentrations in all green plants. Seed grains,
nuts, peas and beans are rich source. Less than 40% of dietary magnesium is absorbed, predominantly in the
jejunum and ileum, and excreted in stool and urine.
Elimination is predominantly renal. The kidney is the main regulator of magnesium concentrations. Normally,
only 3% of filtered magnesium appears in urine; thus, 97% is reabsorbed by the renal tubules. When serum
levels rise above 2.5 mEq/L, magnesium excretion increases dramatically. Conversely, the magnesium
retention by the kidneys is very efficient i.e. the kidney retains a strong capacity to resorb magnesium, and the
main site for reabsorption is the thick ascending loop of Henle (THAL). Several factors may impair renal
reabsorption, such as volume expansion, ethanol ingestion, hypercalcemia, and diuretic administration (eg,
osmotic, thiazide, loop). Of these 3 types of diuretics, loop diuretics have the greatest effect on renal
magnesium wasting because of their site of action.

Hypermagnesemia:
Hypermagnesemia is a rare electrolyte abnormality because the kidney is very effective in eliminating excess
magnesium by rapidly reducing its tubular reabsorption to almost negligible amounts.
Causes:
MC cause: Renal insufficiency.
II MC Cause: Iatrogenic, especially errors in calculating appropriate infusions.
Additional causes include the following:

Acidosis.
Ingestion of magnesium-containing substances such as vitamins, antacids, or cathartics by patients with chronic
renal failure.
Acute renal failure (oliguric phase).
Neonates bom to eclamptic mothers treated with magnesium, which passes through the placental circulation.
Decreased GI elimination and increased GI absorption of magnesium due to intestinal hypomotility.
Tumor lysis syndrome, by releasing massive amounts of intracellular magnesium. (recent question).
Adrenal insufficiency (secondary hypermagnesemia).
Rhabdomyolysis, like tumor lysis syndrome, by releasing significant amounts of intracellular magnesium.
Milk-alkali syndrome.
******ebook converter DEMO Watermarks*******
 Hypothyroidism.
Hypoparathyroidism.
Neoplasm with skeletal muscle involvement.
Lithium intoxication by supposedly decreasing urinary excretion, although the mechanism for this is not
completely clear.
Extracellular volume contraction, as in diabetic ketoacidosis (DKA).
Familial hypocalciuric hypercalcemia- This autosomal dominant disorder is characterized by very low
excretion of calcium and magnesium, and the increase in magnesium reabsorption appears to occur from an
abnormal sensitivity of the loop of Henle to magnesium ions.

Signs and symptoms:

Hypermagnesemia affects the neuromuscular, CNS and cardiac organ systems.

Symptoms of hypermagnesemia usually are not apparent unless the serum magnesium level is greater
than 2 mmol/L.
Concomitant hypocalcemia, hyperkalemia, or uremia exaggerates the symptoms of hypermagnesemia at any
given level.
Nonspecific symptoms (earliest, at 2-4 mmol/1).
These symptoms include nausea (earliest), vomiting, and cutaneous flushing.

Neuromuscular symptoms:
These are the most common presenting problems.
Hypermagnesemia causes blockage of neuromuscular transmission by preventing presynaptic acetylcholine release.
One of the earliest symptoms is loss of deep-tendon reflex.

Facial paresthesias also may occur at moderate serum levels.

Muscle weakness is a more severe manifestation, occurring at levels greater than 5 mmol/L. This manifestation
can proceed to flaccid paralysis, then to depressed respiration, and, eventually, to apnea.
CNS system.
Lightheadedness.
Depressed levels of consciousness.
Stupor or coma.
Conduction system symptoms.
Hypermagnesemia depresses the conduction system of the heart and sympathetic ganglia.
Hypotension.
Bradycardia.
Intraventricular conduction delay.
Arrhythmia, including atrial fibrillation.
Complete heart block and cardiac arrest may occur at levels greater than 7 mmol/L.
Hypocalcemia.
Discussed already.

Lab Studies:

Electrolytes, including potassium, magnesium, and calcium levels.

Elevation in magnesium level is usually not found as an isolated electrolyte abnormality.
Hyperkalemia and hypercalcemia are often present concurrently.
BUN and creatinine levels.
Arterial blood gases (ABG) may reveal a respiratory acidosis.

Other Tests:

An ECG and cardiac monitor may show prolongation of the PR interval or intraventricular conduction delay,
which are nonspecific findings.
The ECG findings may reflect other electrolyte abnormalities such as hyperkalemia.
******ebook converter DEMO Watermarks*******
Treatment:

In patients with mildly increased levels, simply stop the source of magnesium.
In patients with higher concentrations or severe symptoms, other treatments are necessary as follows:

Intravenous fluids with diuretics:

Intravenous fluids e.g. NS, cause the dilution of the extracellular magnesium. These are used with diuretics to
promote increased excretion of magnesium by the kidney. Furosemide (Lasix) is the diuretic of choice. It acts
at loop of Henle to promote magnesium diuresis.

Calcium gluconate:
Calcium directly antagonizes the neuromuscular and cardiovascular effects effects of magnesium. Reserved for
patients with severe or symptomatic hypermagnesemia. 10% IV solution.
Dialysis:
Best used when levels exceed 8 mEq/L, when life-threatening symptoms are present, or in patients with poor renal
function.
Hypomagnesemia:
Causes: Most causes are related to renal and GI losses.

GI losses or low intake.

Malabsorption.
GI secretions in large amounts, e.g. chronic diarrhea, laxative abuse, inflammatory bowel disease, or neoplasm.
Prolonged TPN.

Renal losses:

Primary renal disorders - by decreased tubular reabsorption of magnesium.

Clinical effects:

Neuromuscular irritability (Earliest, at serum magnesium levels less than 1.0 mEq/L).
Hyperactive deep tendon reflexes.
Muscle cramps.
Trousseau and Chvostek signs.
CNS hyperexcitability.
Irritability.
Psychosis.

Treatment:

Magnesium is administered PO (oxide or gluconate form) for patients with mild depletion.
IV replacement, as a sulfate salt is indicated for severe clinical effects.

Miscellaneous Facts:
Pyloric Stenosis:

K, I CL\ metabolic alkalosis with paradoxical acidosis.

Rx = replace ECF with Isotonic NaCl& K+

Anion gap.

******ebook converter DEMO Watermarks*******

Anion gap = (Na++ K+) – (CI + HC03).
= 10-15 meq/1.
Metabolic acidosis.
↑ Anion Gap.

Commonest = shock.
Diabetic ketoacidosis.
Alcohol intoxication.
Uraemia.
Salicylate toxicity.
Oxaloisis.

Normal Anion Gap.

Diarrhea.
Small bowel fistula.
Uretrosigmoidostomy.
Proximal RTA.
Distal RTA.
Dilutional acidosis.

******ebook converter DEMO Watermarks*******

Total Parenteral Nutrition (TPN):
Assessment of The Patient (Prior To Initiation of TPN):

Induration grade:
0= < 0.5 cm.
1= .5 cm.
2 = 1cm.
#PMI % = 158-1.66 × albumin (gm/l) – (0.78 × triceps skin fold in mm.)- (2 × transferrrin gm/l) – 5.8 × delayed
hypersensitivity index):
Requirements to be Calculated:
After assessing the nutritional status of the patient, requirement is calculated in terms of:
A: Fluid requirement.
B: Energy Requirement.
C: Protein or AA requirement.
D: Mineral & Vitamin.
A: Fluid Requirement:
Normal Daily fluid requirement.
Infants: 120ml/kg body weight. Adults: 40ml/lg body weight.
For each 0C rise of Temp. add 200 ml/day.
Abnormal losses are added to daily requirements.
B: Energy Requirement:
Patients BASAL ENERGY EXPENDITURE (BEE) is calculated using HARRIS BENEDICT EQUATION.
For women: 655.10 + 9.56 (W) + 1.85 (H) – 4.68 (A) Kcal/day.
For men: 665.47 + 13.75 (W) + 5 (H) – 6.76 (A) Kcal/day.
W = wt. In Kg.
******ebook converter DEMO Watermarks*******
H = height in cm.
A = age in years.
To the BEE should be added:

A value of 20% of BEE for a pt. without significant metabolic stress.

50% of BEE for patients with marked stress like sepsis and trauma.
100% of BEE for pt. with severe stress like >40% burn.

Harris Benedict equation is based on the data related to healthy subjects. So it may not correctly assess the caloric
need of a hospitalized malnourished patient. Here assessment of Resting energy expenditure is a better guide.

Men REE: (789 × BSA) + 137.

Women REE: (544 × BSA) + 414.

BSA is Basal surface area.

A factor of 20% above REE estimates the need of most of the hospitalized patients, and 40-100% above REE for
>40% burn.
C: Protein and Amino-Acids: Requirement:
Recommended dietary protein allowance:

In non-stressed patients = 0.8 gm/kg body wt./day.

Catabolic patients require = 1.2-1.7 gm/kg body wt./day.

Protein balance = Protein intake – protein loss.

Protein loss = 24 hrs. Urine urea nitrogen (g) × 6.25.
6.25 Gm of protein = 1 Gm of nitrogen.
Calorie to nitrogen ratio should be 100-150: 1 (To minimize protein catabolism).
D: Minerals & Vitamin Requirement.
The parenteral requirement of some of the vitamins may be higher than the enteral requirements, due to:

The micronutrients are delivered into the systemic rather than portal system thereby by-passing the liver and
rapidly excreted by the kidneys.
Many patients requiring TPN have large GUT losses that results in Na, CL, K, and bicarbonate wasting and
also loss of divalent cations and vitamins.
The tubing and exposure to the oxygen and light can also absorb and destroy vitamins (eg. Vit. A) before it
reaches the patient.

Prescribing Parenteral Nutrition:

Steps are:
Step I: Calculate patient’s expenditure for caloric need & protein need.
Step II: identify appropriate amount of Dextrose/ Fat Calorie and amount of amino acids to supply nitrogen acid.
Step II: Order necessary electrolyte, mineral, vitamins & trace elements.
Step IV: Calculate fluid need in which TPN will be given.
Step I: Already described.
Step II:
Dextrose:

In TPN concentrated Dextrose or Glucose is the most commonly prescribed caloric source.
******ebook converter DEMO Watermarks*******
 Dextrose provide 3.4% Kcal/gm.
Thus 500 ml of 50% Dext. Supplies 850 Kcal.
The basic conc. Of dext. is final solution = 20-25% dextrose.

Fat

Fat is needed to prevent essential fatty acids deficiency and also as a source of non-protein calories.
Fat provides 9 Kcal/gm.
Its available as 10% & 20% emulsion providing 1.1 & 2 Kcal/ml.
Thus 500 ml of 10% fat emulsion = 500 × 1.1 = 550 Kcal.
500 ml of 20% fat emulsion = 500 × 2 = 1000 Kcal.

Crystalline Amino Acids: As protein source.

Proteins are not provided for calories but to provide nitrogen for protein catabolism.
6.25 gm of protein contain 1 gm of nitrogen.
The basic solution of TPN contains final conc. of 3-5% amino acid.
Thus 500 ml of 10% AA = 4.63 gm of N+ or 28.9 gm of proteins.

Electrolytes and mineral are provided for maintenance and to for acute loss, should include: Na+, K+, Ca++, Mg+,
Cl-, Po4—
Trace elements given daily are:

0.8 mg Manganese.
1 mg Copper.
4 mg of Zinc.
10 mg Chromium.

Adequate Vitamin supplementation should be done intravenously. Following vitamins have to be given I.M. as they
are unstable in hyperalimentation solution.
Vit. K = 10 mg IMI / Week.
Folic acid = 5 mg / week
Vit B12 = 1 mg / month
3 In 1 TPN solution: combine glucose fat AA and other additives in One bag for infusion over 24 hours.
Advantages:

Decreased risk of infection Due to less manipulation/ Cost saving/ Time saving.
Using a glucose, and fat calorie source provides a more physiologic solution > reduced co2 production.
In this solution up to 40% kcal may be given as fat.

EXAMPLE: ordering TPN for 70 kg man, 170 cm height:

Step I: Calculate caloric and nitrogen needs.
BSA = 1.8.
REE = 7.89 × BSA + 137 = 789×1.8-137= 1557 Kcal.
20% increment: Final REE = 1867 Kcal.
Calculated caloric requirement = 1867 Kcal.
Nitrogen requirement: 70 × 1.3 gm protein = 91 gm protein.
91 gm / 6.25 = 14.5 gm of nitrogen.
Calculated nitrogen need = 14.65 gm NT.
Step II: Ordering solution for 24 hrs. administration.
******ebook converter DEMO Watermarks*******
 800 ml of 50% dextrose. = 1360 Kcal.
500 ml of 10% fat. = 550 Kcal.
900 ml of 10% AA = 15.1 gm N+

Step II: Add electrolyte mineral vitamins.

The starting infusion rate should be 50-100 ml / hr depending to patients cardiovascular and renal status.
This rate gives 1200-2400 Kcal / day.
The increase should be 25-50 ml / hr. every day to allow kidney and pancreas to adjust to increased osmolality and
glucose level.
Specific Formulations in Specific Disese State:
I: TPN in patients with Renal Failure:

Patients in ARF not requiring dialysis require Concentrated TPN, (eg. Glucose-10%, Fat-20%, AA-10%), to
reduce fluid load yet to provide adequate calories to prevent catabolism.
Nitrogen conc. should be less.
After regular dialysis is established protein content can be liberalized to provide 1-1.5 gm protein / kg / day.

II: In hepatic failure:

Here ureagenesis is impaired with accumulation of toxic nitrogenous compounds eg. Ammonia.
Thus TPN is started with a reduced load of protein (0.7 gm/kg).
Solution should contain more of branched chain AA and less of aromatic AA.
Such solution appears to improve encephalopathy though it may not improve survival dictated by underlying
liver failure.

III: In cardiac or respiratory failure.

Fluid and Na+ restriction is indicated in CCF.

In respiratory failure, a TPN solution may provide benefit, which contains higher percentage of calories as fat.
(Fat has a lower RQ then carbohydrate; .07:1, thus less likely to lead to hypercapnia.)
40% of non-protein calories are given as fat if hypercapnia impairs respiratory functions.

Effects of Tpn on Gut Functions:

Monitoring the Patient on TPN:

A: Clinical Data To Be Checked Daily:
1. Patient’s sense of well being, symptoms suggesting fluid overload, high or low blood glucose.
2. Patient’s strength as judged by graded activity, getting out of bed, walking stairs, climbing and weight
measurement.
3. Vitals;Temp. BP, PR, RR.
4. Fluid balance: input vs. output.
5. Delivery equipment of TPN nutrition.
B: Laboratory Data To Be Monitored:
******ebook converter DEMO Watermarks*******
 Nitrogen balance: N+ intake – (UUN + NUN).
UUN= Urine urea N+, NUN = Non urea and insensible losses.
C: Indirect Calorimetery:
To find out how the body is utilizing the caloric intake. It measures the respiratory quotient (RQ).
RQ > 1 Indirect Lipogenesis
RQ = 1 Carbohydrate Utilization
RQ= 0.74–0.85 Mixed fuel utilization.
RQ= 0.7 Fat utilization
Complications of TPN:
It can be broadly classified into 3 categories.
A: Mechanical.
B: Infectious.
C: Metabolic.
A: Mechanical Complications:
Arise either due to wrong placement of catheter or due to maintenance of venous access.

Development of pneumo, hydro, hemo, or chylothorax.

Injury to subclavian artery or brachial plexus.
Malposition of catheter leading to arrhythmias.
Air embolism or catheter embolism.
Thrombophlebitis or thrombosis of SVC.
Slipping of catheter, or hub detachment.

In order to avoid these complications following steps should be taken:

Catheter position must be confirmed by X-ray before hypertonic solution is infused.

Minimal handling of the catheter
Daily check arm of the patient for edema.

B: INFECTION: Catheter sepsis is confirmed if:

The catheter tip and blood cultures are positive for the same organism.
Fever disappears/ decreases within 24 hrs of catheter removal.
No other source of infection is identified.

One of the earliest sign of systemic sepsis is sudden development of glucose intolerance (with or without temp
increase), in a pt. who previously has been maintained on TPN.
Sepsis is more likely with double or triple lumen tube.
Metabolic Complications in TPN:

******ebook converter DEMO Watermarks*******

Hypophosphatemia:
Develops if phosphorus has not been added in amount adequate to meet the requirements for the metabolism of
infused glucose and amino acids. The result is an extra vascular to intracellular shift of phosphate.
Signs and symptoms:
Paresthesia, confusion, convulsion and death.

Associated with Hypophosphatemia is a reduction in erythrocytic 2, 3-diphosphoglycerate leads to increased

affinity of Hb for oxygen hence, less O2 is released to peripheral tissue.
Early metabolic problem specially in elderly and debilitated patients including fluid overload producing CHF
and glucose overload leading to stimulation of insulin secretion which causes intracellular shift of Phosphorus

******ebook converter DEMO Watermarks*******

 and potassium with resultant depletion of phosphorus and potassium leading to arrhythmias, cardiopulmonary
function and neurological symptoms.

To avoid these complications TPN should be started slowly and monitored carefully.

Late metabolic complications include cholestatic liver disease with bile sludging and gall stones.
The exact cause of liver disease is not understood but appears to be linked to the lack of enteral nutrition, the
disease is less likely if some enteral feeding is continued.
Hyperosmolar non-ketotic hyperglycemia develops either if the hypertonic solutions are administered too
rapidly or if the patient has impaired glucose tolerance.
This is particularly common in latent diabetics and in patients following severe surgical stress or trauma.

Treatment of the condition consists of volume replacement, administration of insulin, electrolyte abnormality to be
corrected.
Complications of TPN (Summary):

******ebook converter DEMO Watermarks*******

Classification of Wounds:

Surgical Site Infection

******ebook converter DEMO Watermarks*******

******ebook converter DEMO Watermarks*******
Extra Edge:
Intravenous Cannula:

Complications of Intravenous Canulation:

1. Accidental damage: Nerve, tendon or artery may be inadvertently punctured causing pain and damage.
2. Phlebitis: Characterized by pain and discomfort resulting from in ammation of the intima of the vein. e three main
types are:
a. Mechanical–damage/irritation by a cannula that is too large for the vein or inadequate securement of the
cannula which allows for movement.
b. Chemical—drugs which cause irritation (pH<5 or >9 or extreme osmolarity or vesicant).Vesicant drugs can
cause blistering and necrosis, if they leak into the surrounding tissues.
c. Bacterial–poor hygiene or aseptic techniques leading to infection.
******ebook converter DEMO Watermarks*******
3. Assessed by VIP score (Visual Infusion Phlebitis Score) (Table 10.2).
4. Hematoma: Hematoma may form if the cannula pierces the front and/ or back wall of a vein. Is can occur during
insertion or removal of the cannula and may render the vein unsuitable for further cannulation. Treated by rm
pressure applied for 3–5 minutes.
5. Extravasation: is is the leakage of vesicant uids or drugs into surrounding tissues which can cause local necrosis.
6. Prolonged bleeding time: is may be due to a medical condition or drug therapy. It increases the risk of bruising or
hematoma formation and worsens the consequences of inadvertent arterial puncture.
7. Blood spillage.
8. Needle or blood phobia.
9. Vasovagal faint/syncope: is is due to enervation of the autonomic nervous system. Ensure that the patient is
sitting/lying in a chair/bed whilst undertaking the procedure. However, if the patient begins to feel faint or appears
pale and sweaty, the procedure should be stopped immediately. e cannula should be resited at the rst signs of in
ammation or discomfort.

Sutures:
Absorbable Sutures:
Natural:

Catgut:
It is made from the intestine of cattle or sheep.
It was originally known as kitgut because it was used as strings for a musical instrument called “kit”. And
later, it became catgut.
It is made of protein and so it is degraded by proteolysis.
It is also from another species and so when used, for the second time in a patient, may cause rejection,
stitch abscesses and sensitivity reactions. But the cost is much lower than synthetic sutures.
Plain catgut: Tensile strength is three days and it gets degraded in 7–10 days. So it is useful only for
subcutaneous tissue.
Chromic catgut: When catgut is immersed in chromic acid, the tensile strength becomes three weeks and it gets
degraded in 6 weeks. So it can be used for subcutaneous tissue, muscle, small intestine, stomach, the biliary
tract and the urogenital tract.
2. Kangaroo tendon: e tail of the kangaroo is also used for manufacturing sutures in Australia, and the tensile
strength and degradation times are similar to chromic catgut.

******ebook converter DEMO Watermarks*******

Synthetic:
All synthetic absorbable sutures are degraded by hydrolysis. So they do not cause any tissue reaction and the chance
of rejection is minimal. They are however costly.
Polyglactin:

is is a combination of glycolide and lactate in the ratio 910:90 and so the Ethicon company who devised it
named it “Vicryl 910”.
tensile strength is 3 weeks and it gets degraded in 6 weeks.
It is braided and hence knot holding property is very good and so surgeons love it.
Polyglycolic acid: is is a braided monomer devised by the United States Surgical Corporation who named it
“Dexon”.
Tensile strength is similar to polyglactin. Both polyglactin and polyglycolic acid are colored violet for
visibility and identification
Polyglycaprone: Poliglecaprone is a monofillament, launced by Ethicon, brown in color and the tensile strength
and degradation are similar to polyglactin. it is degraded in 90-120 days.

Polydioxanone:

This is a monofilament with increased memory and colored violet.

Tensile strength lasts for 6 weeks and it gets degraded by180 days.
Hence, it can be used for the biliary tract and for closure of a subcostal and Pfannenstiel incisions in addition to
its use wherever polyglactin can be used. .

Nonabsorbable Sutures:
Natural:
Silk:

Silk is a protein called Sericin, made from the silk worm cocoon. It is braided and colored black for visibility.
It gets degraded by the 3rd year. It loses 30% strength when wet and should not be reautoclaved.

Cotton:

Cotton is made from the cotton seed pod and is cellulose. Cellulose gains strength when wet. It lasts indenitely
in the body.
Linen: linen is made from the ax plant and is cellulose and lasts indenitely in the body.

Synthetic:

Polypropylene: It is a monofilament.
It has increased memory but lasts indefinitely in the body and hence is the best suture for hernia repair,
esophageal, colonic and rectum suturing and closure of the anterior abdominal wall because these
structures take 1 year to regain their original strength.

Polyamide:

It is also known as nylon.

It loses 10% strength per year.
It is a mono lament and has memory but it is still a cheap substitute for polypropylene and is mainly used for
skin suturing.
Only a mono lament should be used for skin suturing as use of a braided suture will allow skin commensals like
staphylococci to migrate along the suture by “wick effect” and colonize the suture tracts.
Polyester: It is a braided suture coated with polybutylate mainly used in cardiovascular, ophthalmic and
******ebook converter DEMO Watermarks*******
 neurosurgeries. It lasts indefinitely.
Stainless steel: It is available in all sizes and last indenitely. Sizing is done by the Brown and Sharpe gauge
sizes.

******ebook converter DEMO Watermarks*******

Needles:
******ebook converter DEMO Watermarks*******
******ebook converter DEMO Watermarks*******
Suturing Technique:

******ebook converter DEMO Watermarks*******

Knots:

******ebook converter DEMO Watermarks*******

******ebook converter DEMO Watermarks*******
Miscellaneous Topics:
Surgical therapy for TRIGEMINAL NEURALGIA (Also called as- Porosopalgia or Fothergill’ disease): Only
for patients who fail medical treatment.
1. Microvascular decompression (Jannetta procedure) involves a small sub-occipital craniotomy for microsurgical
exploration of the dorsal root entry zone of the trigeminal nerve on the affected side. The “offending vessel,
usually the superior cerebellar artery”, is then dissected off the nerve and a barrier (Teflon or PVA [polyvinyl
alcohol] sponge) is placed between the vessel andnerve to prevent continued pulsatile focal compression.
2. Percutaneous trigeminal rhizotomy techniques generally involve radiofrequency heat lesioning of the trigeminal
ganglion, glycerol injection into the spinal fluid of Meckel’s cave, or mechanical trauma to the nerve or ganglion
by transient inflation of a no. 4 Fogarty catheter balloon.
(Zakrzweska and Thomas described 3 types of procedures:
Percutaneous radiofrequency trigeminal gangliolysis (PRTG): Patients may develop minor, local, residual facial
numbness.
Percutaneous retrogasserian glycerol rhizotomy (PRGR): occasionally lose sensation.
Percutaneous balloon microcompression (PBM): Rarely lose sensation.
In each procedure, the surgeon introduces a trocar or needle lateral to the corner of the mouth and, under
fluoroscopic guidance, into the ipsilateral foramen ovale. The ganglion is lysed at this location.

Surgery for Epilepsy:

Epilepsy surgery is indicated in drug resistant epilepsy (DRE) (not responding to at least two appropriate, adequate
anti-epileptic drugs (AEDs).
1: Curative:
A: Resective surgeries: (Very useful in presence of a “concordant” epileptogenic focus).
i. amygdalohippocampectomy.
******ebook converter DEMO Watermarks*******
 ii. lesionectomy.
iii. multilobar resections.
B: Functional surgeries:
i. hemispherotomy.
2: Palliative: (More useful in “substrate negative”, without delineation of an epileptogenic zone).
i. Multiple subpial transaction.
ii. corpus callosotomy (Indicatd in refractory cases like Lenox Gestaut syndrome).
iii. vagal nerve stimulation.
Division according to surgical interventions:
a Temporal- About two-thirds of the patients become seizures free, excepting simple partial seizures, (aura only)
after anterior temporal lobectomy.
b Extra temporal surgeries- Overall response is 45–70%. Hemisphertomy has higher response (75–85%) while
multiple subpial transactions and corpus callosotomy has lower response (30%). Extratemporal epilepsy:
Provided non-eloquent cortex is involved, resection of seizure foci other than temporal lobe can be performed,
although the likelihood of cure is not as high. “U” fibers can be interrupted in eloquent cortex regions by a
technique called multiple subpial transactions. Also, seizure spread from one hemisphere (where they originate)
can be prevented into another hemisphere (which is what is thought to happen in drop attacks), by a technique
called corpus callosotomy (where the anterior 2/3 of the corpus calosum is cut – the posterior 1/3 is spared, to
avoid a disconnection syndrome which would result in an inability of the left brain to comprehend objects seen on
the right occipital cortex).
Division according to outcome:
a. Definitive: offering complete surgical cure or at least 90% chances of reduction in seizures and
b. Palliative: offering reduction of the seizure load rather than cure.
Types:

Anteromedial temporal lobectomy with amygdalo-hippocampectomy: A surgical procedure where the anterior
and the medial part of the temporal lobe resected along with hippocampus, amydala, uncus and the mesial
structures. This is mostly indicated for epilepsies arising from the medial temporal lobe.(< 5% for
complications such as visual field defect, memory problems, other surgical risks).
Selective amygdalo-hippocampectomy: A more technically demanding surgical procedure where only the
mesial structures, like hippocampus, amydala and uncus, are removed, leaving the lateral temporal lobe intact.
Multiple subpial transection: It is “palliative” procedure with aim is to reduce the seizure burden only rather
than to eliminate them completely. It is usually performed on an eloquent cortex, the gyrus is divided into small
blocks of 1 × 1 cm using a special instrument.
Hemispherotomy: A complex surgical procedure where the entire affected hemisphere (in conditions like
Rasmussen’s syndrome) is disconnected from the opposite hemisphere.
Multifocal epilepsy: Epilepsy from multiple foci or with foci in eloquent areas of the brain can be treated by
vagal nerve stimulation. The left vagus nerve is stimulated by an implanted electrode, and while this treatment
provides reduction in seizure activity, it rarely leads to cure. Right vagal stimulation is never done, since it is
thought to lead to cardiac dysrhythmia.

******ebook converter DEMO Watermarks*******

Technique for Suture less Bowel anastomosis:
Fibrin glue:
Fibrin glue consists of four flacons as fibrinogen, aprotinin, trombone, and calcium. When these are brought
together, fibrin clot is established showing similarities to final stages of coagulation cascade. Fibrin clot, by holding
the tissue, acts both as a homeostasis and as glue. Fibrin clot is considered to have a positive effect on anastomosis
by forming a barrier, by bringing tissues end-to-end and facilitating them to remain that way while speeding up
tissue recovery via the substances in its contents.
Biofragmentable anastomotic ring (BAR):
It is a biofragmentable anastomotic device made of absorbable polyglycolic acid (Daxon) and barium sulfate, which
renders it radiopaque. It is made of 2 identical segments attached together on a central frame. Their scalloped rims
prevent tissue ischemia; the bowel anastomosis is created by tying the purse string suture inserted into each end of
the bowel around each component of the BAR. The device is then snapped shut. Between 11 and 16 days after
surgery the fragmented BAR is passed into the stool. An inverted serosa-to-serosa anastomosis is thus created.

******ebook converter DEMO Watermarks*******

May-Thurner syndrome:
The right common iliac vein ascends almost vertically to the IVC while the left common iliac vein takes a more
transverse course. For this reason, the left common iliac vein may be compressed between the right common iliac
artery and lumbosacral spine, leading to thrombosis of the left iliac vein. a condition known as May-Thurner
syndrome (also called iliocaval compression syndrome, Cockett syndrome or iliac vein compression syndrome).

******ebook converter DEMO Watermarks*******

Nutcracker syndrome (of Urology):
Nutcracker syndrome is caused by compression of the left renal vein between the aorta and the superior mesenteric
artery where it passes in the fork formed at the bifurcation of these arteries.
The phenomenon results in left renal venous hypertension.
The syndrome is manifested by left flank and abdominal pain, with or without unilateral haematuria.
In fameales it may cause ‘pelvic congestion syndrome’ characterized by symptoms of dysmenorrhea, dyspareunia,
post-coital ache, lower abdominal pain, dysuria, pelvic, vulvar, gluteal or thigh varices and emotional disturbances.
In males it may cause left renal-to-gonadal vein reflux resulting in lower limb varices and varicoceles in males.
Diagnosis can be confiemd by Doppler ultrasound measurements of the anterior-posterior (A-P) diameter and peak
velocities of the left renal vein.
CT scan showing may show:

Reduced aorta: SMA angle.

Left renal vein stenosis.
Collateral pathways: main collateral pathway is the left gonadal vein which will display early enhancement
during portal venous phase.
Pressure gradient >3 mm Hg on renal venography is also suggestive of this condition

Surgical treatment option include-

Open surgery procedures like vascular transpositions and renal auto transplantation (with significant
morbidity).
Extra-vascular stenting can be performed through open or laparoscopic surgery and involves placement of a
polytetrafluorethylene (PTFE) graft around the LRV, extending from its junction with the inferior vena cava
(IVC) to the point of entry of the left adrenal and gonadal veins.
Intravascular stenting is least morbid in which a self-expanding metallic stent is deployed (under digital
subtraction angiography (DSA) guidance) in the stenotic region of the LRV, with the medial edge of the stent
lying in the inferior vena cava. As this is a benign condition, the overall prognosis is excellent.

******ebook converter DEMO Watermarks*******

PEComa:

PEComas are Perivascular epithelioid cell tumors, rare mesenchymal tumors.

The natural history of these tumors is highly variable ranging from indolent benign lesions to lesions with an
aggressive clinical course including distant metastases.
In 1996, the term PEComa was used for the first time to designate mesenchymal neoplasms containing
epithelioid cells with a close association to vessel walls and immunophenotypic features of smooth muscle and
melanocytic differentiation.
In 2009, PEComas represent a group of ubiquitous visceral, soft tissue and bone neoplasms sharing
morphological and immunohistochemical distinctive features including, at least conceptually,
angiomyolipomas (AML),
clear-cell ‘sugar’ tumor of the lung, lymphangioleiomyomatosis (LAM).
clear-cell myomelanocytic tumor of the falciform ligament/ligamentumteres
unusual extrapulmonary clear-cell tumors. Some of these conditions such as angiomyolipoma and LAM
represent a subset of PEComas that are strongly associated with tuberous sclerosis complex.
The uterus is the most frequent site of origin for PEComas occurring outside the context of tuberous sclerosis.
Usually benign, but some cases have malignant behavior and simulate high grade sarcoma.
Poor prognostic factors: tumor size > 5-8 cm, infiltrative growth pattern, high nuclear grade, >1 mitotic
figure/50 HPF or atypical mitotic figures, coagulative cell necrosis.
Positive stains: Melanocytic markers (HMB45 and MelanA; microphthalmia transcription factor–50%; also
NKI/C3, tyrosinase, S100–33%), smooth muscle markers (MyoD1, smooth .muscle actin-80%, desmin–40%,
calponin), vimentin (80%).
(Main markers are HMB4.5 and Melan A).
Negative stains: Cytokeratin, CD117/c-kit, CD34.
Treatment: Surgical removal.
Sirolimus, an inhibitor of mTORC1 has been shown to give some response in metastatic tumour.
Temsirolimus has also been used in metastatic uterine Pecoma.

Mesenteric Lipodystrophy (Mesenteric Panniculitis):

It is chronic fat degeneration and fibrosis of unknown cause affecting the root of the mesentery produce diffuse
mesenteric thickening or masses.
Its localized form is called Weber-Christian disease presenting with subcutaneous nodules- (“Idiopathic
Relapsing febrile non-suppurative panniculitis”, common in females of 3-50 years).
******ebook converter DEMO Watermarks*******
 Mesenteric Panniculitis is seen in elderly males with recurrent abdominal pain, weight loss, or symptoms of
partial intestinal obstruction.
A hard irregular abdominal mass may be felt in 50% usually in the left upper quadrant.
CT scanning shows the characteristic features of nonhomogeneous masses of fat and soft tissue density.
MRI may suggest the fibrous nature of the lesion and delineates vascular involvement.
The diagnosis is usually made only by biopsy at laparotomy, but resection is neither feasible nor indicated.
The process subsides spontaneously in most cases. Rarely side-to-side intestinal bypass may be required to
relieve obstruction.
A more serious variant (“Retractile mesenteritis”) associated with obstruction of the mesenteric lymphatics and
veins often proves fatal.
Corticosteroids, cyclophosphamide, and azathioprine should be reserved for such cases and for patients with
clinical deterioration. Lymphoma occurs in 15% of cases on follow-up.

Karnofsky performance status

Karnofsky Performance Scale Index is an attempt to quantify cancer patients’ general well-being and activities of
daily life. It allows patients to be classified as to their functional impairment. This can be used to compare
effectiveness of different therapies and to assess the prognosis in individual patients. The lower the Karnofsky score,
the worse the survival for most serious illnesses.
Mailny used in decision making of Thrapy for defferent cancers based on performance status.

Able to carry on normal activity and to work; no special care needed- 80, 90, 100
Unable to work; able to live at home and care for most personal needs; varying amount of assistance needed-
50, 60, 70.
Unable to care for self; requires equivalent of institutional or hospital care; disease may be progressing rapidly-
0 to 40.

Other scoring system used are ECOG/WHO/Zubrod score and Lansky score (Used more often in children).
Mesothelioma Markers:

Positive for – Calretinin (100%), cytokeratin, mesothelin, WT1 (99%), EMA, HBME-1, thrombomodulin;
CD44S, N-cadherin, vimentin, E-cadherin, for Ber-EP4, MOC-31, BG-8.
Negative for- CEA, B72.3, leu-M1, TTF-1, or CA19-9.
The best combination for mesothelioma is positive for calretinin and cytokeratin 5/6 or WT1 and negative for
CEA and MOC-31, Ber-EP4, BG-8 and B72.3.
High levels of hyaluronic acid levels are suggestive of mesothelioma.
High levels of CEA are associated with adenocarcinoma.
Intracytoplasmic neutral mucins (mucicarmine positive after hyaluronidase predigestion) is relatively specific
but not sensitive for adenocarcinoma.

Collagens provide strength and integrity for all tissues:

Type I collagen is the major structural component of bones, skin, and tendons.
Type II collagen is found predominantly in cartilage.
Type III collagen is found in association with type I collagen in varying ratios depending on the type of tissue.
Type IV collagen is found in the basement membrane.
Type V collagen is found in the cornea.

Sutures:
Important points to remember about Sutures.

Absorbable sutures include:-Polyglycolic Acid sutures (Daxon), Polyglactin 910 (Vicryl), Catgut,
Poliglecaprone 25 (Monocryl) and Polydioxanone (PDS) sutures.
Non-Absorbable sutures include:- Polypropylene sutures (Prolene), Nylon (poylamide), Polyester, PVDF
(polyvinylidenedifluorid), silk and stainless steel sutures.
******ebook converter DEMO Watermarks*******
 Natural sutures include silk and catgut sutures whereas all other sutures are synthetic in nature.

Less important points:

Monofilament sutures include: Polypropylene sutures, Catgut, Nylon, PVDF, Stainless steel, Poliglecaprone
and Polydioxanone sutures.
Multifilament or braided sutures include:- PGA sutures, Polyglactin 910, silk and polyester sutures.
Coated sutures include :- PGA sutures, Catgut Chromic, Polyglactin 910, silk and polyester sutures, braided or
twisted nylon, Poliglecaprone and Polydioxanone sutures.
Un-coated sutures include :- Monofilament Polypropylene sutures, monofilament Nylon, PVDF, Stainless steel.

MEN 4:

Multiple endocrine neoplasia type 4 (MEN4) emerged as a novel form of multiple endocrine neoplasia, also
referred to as MENX.
Caused by mutations in the CDKI gene CDKN1B/p27(Kip1) (CDKN1B gene on chromosome 12p13).
MEN4 is caused by heterozygous mutation in the autosomal dominant inheritance.
Parathyroid involvement and less typically with pituitary adenomas are main features (with possible association
with tumors of the adrenals, kidneys, and reproductive organs).

Oncology Update:
Microscopic clues of tumor origin:
1. Signet ring cells: GIT, pancreas, ovary, breast (lobular)
2. Psammoma bodies: Ovary (papillary serous), thyroid, breast, meningioma
3. Papiilary structures: thyroid, ovary, breast, pancreas, mesothelioma, kidney, lung (occasionally).
4. Single file tumor cells: breast (lobular), small cell carcinomas.
5. Intranuclear inclusions: papillary thyroid, melanoma, meningioma, bronchoalveolar carcinoma, hepatocellular
carcinoma.
6. Cell nests: carcinoid, melanoma, paraganglioma, pancreatic islet cell tumors.
7. Rossettes: neuroblastoma, retinoblastoma, neuroendocrine carcinoma, PNET/ Ewing’s sarcoma, ependymoma.

******ebook converter DEMO Watermarks*******

HIV (Surgical aspects):

Low CDC guide is best guide to early Clinical event or death in near future.
Plasma Viral load is best long term guide to prognosis.
Transmission is HIV is much less than Hep B (1 ml of blood has 50 HIV 1 Particles and 109 Hep- B Particles).
Risk of transmission – Max during seroconversion and late stage.
Maximum Risk- Skin perforation by hallow needle > Solid needle > Splashing over mucus membrane/ skin.
Generally standard precaution is preferred over Universal precautions.
Indication of universal precaution are- Homosexuals/ IV drug abuser/ Haemophilia treated with Factor VIII/
Resident of Sub Sahara Africa/ Partner of 1 or more of the above.

Standard precaution:

Carry out procedure in orderly manner/ Assistance kept to minimum/ Not to move during procedures/ Stop
surgery while assistant position is adjusted/ Surgery to be done in slow and methodical manner/ Sharp
instruments to be passed in dish.

Universal precautions:

Safety spectacles n face mask/ Water proof gown (Anterior trunk and arm protection/ Boots (Not open toed
shoes or slippers)/ Two pairs of gloves (Inner glove is bigger and outer is ½ size smaller)- two pairs of gloves
decrease skin contamination by 5 folds.

Post- contamination:

Clean with running water.

Post exposure prophylaxis to be started 1 hr after exposure even if HIV status is unknown (don’t wait for HIV
antibody Titre).
Zidovudin 250 mg BD.
Lamivudin 150 mg BD.
Indinavir 800 mg BD.
(All for 1 month).

Surgeon should receive Hepatitis prophylaxis also.

Immediate HIV testing should be done/ Repeat after 12 weeks.

Most Common anal Cancer – SCC (MCQ) > Kaposi’s > NHL (Peri-rectal/ perianal)

******ebook converter DEMO Watermarks*******

Indications of splenectomy in HIV are:

HIV related Auto-immune thrombocytopenia.

Genomic incorporation of Kaposi’s Virus (Herpes virus 8) encoding IL-6 -> B cell proliferation (Castleman’s
disease) leading to hypersplenism and thrombocytopenia .

******ebook converter DEMO Watermarks*******

******ebook converter DEMO Watermarks*******
******ebook converter DEMO Watermarks*******
******ebook converter DEMO Watermarks*******