Biliary system disease

Fan Ying
General Surgery Department
of Shengjing Hospital, CMU
preface

1. Anatomy and Physiology

2. imaging examination
3. congenital choledochal cyst
4. cholelithiasis
5. Biliary system infection
6. primary sclerosing cholangitis , PSC
7. biliary ascariasis
8. common complications of biliary system disease
9. bile duct injury
10. biliary system tumor
 1. Anatomy and Physiology

Extrahepatic Biliary Tract

Left hepatic duct extrahepatic bifurcation;

anterior to portal vein bifurcation;
Right hepatic duct

Common hepatic duct Lays anterolateral to the hepatic artery

and portal vein
Extrahepatic Biliary Tract
Common bile duct Common hepatic duct join Cystic duct
to form; extend to the papilla of Vater;
Empty into the duodenum

Cystic duct
Gallbladder
1. Anatomy and Physiology
1. Anatomy and Physiology

Common bile duct Empty into the duodenum

7cm-9cm in lengh (depending on the junction with cystic duct)

Supraduodenal;
Three segments:
Retroduodenal;
Intrapancreatic;

long common duct

distal common bile duct
short common duct within the duodenal wall
Pancreatic duct
through two distinct ostia
1. Anatomy and Physiology

Gallbaldder In continuity with the common hepatic and common bile

duct through the cystic duct

5 cm-8 cm in lengh
3 cm-5 cm in diameter
Has a capacity of 40~60ml
Fundus
Body
Anatomy divided into Infundibulum
Neck
Valves of heister
Cystic duct
Calot triangle: The triangle bounded by the common hepatic duct
medially,the cystic duct inferiorly and the inferior
surface of the liver superiorly is known as Calot
triangle.
cystic artery ,right hepatic artery, para-right hepatic
duct run within the triangle makes an important area
of dissection during cholecytectomy.
1. Anatomy and Physiology

Bile ducts Modify , store , regulate , the flow of bile

liver
bile 800~1200ml/d
canaliculi
modified by the absorption and secretion ( water and electrolytes)
bile duct
Responsive to neurogenic,humoral, chemical stimuli
increase decrease
Vagal stimulation Splanchnic nerve stimulation

Hydrochloric acid
Gastrointestinal hormone: Increase secretion of
Proteins chloride-rich fluid by bile
secretin ducts and ductules
Fatty acids

in duodenum
1. Anatomy and Physiology

Bile composition:
water

electrolytes Sodium, potassium, calcium, chlorine have the same

concentration in bile as in plasma.
bile salts Cholate, chenodeoxycholate Synthesized in liver by cholesterol
excreted into bile by hepatocyte and aid in the digestion and absorbtion of
fats in the intestines.
proteins

cholesterol
lipids Synthesized in liver
phospholipids

bile pigment the metabolic product from the breakdown of hemoglobin

1. Anatomy and Physiology

Concentrates hepatic bile 500ml 50ml

Gallbladder: Stores hepatic bile
Diliver it into duodenum in response to a meal
Following a meal, the gallbladder contracts in response to
both vagally mediated cephalic phase of activity and the
release of CCK.

Next 60~120min, 50%~70% of gallbladder bile is

steadily emptied into the intestine tract.

The next 60~90min, gallbladder refilling then

occur gradually.
2. imaging examination

(1) Ultrasonography:
Ultrasound of abdomen is an extremely useful method for identifying gallstones and
pathologic changes in the gallbladder. Now, it is the routine evaluation of patient suspected
of having gallstone disease.
High specificity (>98%)
for diagnosis of cholelithiasis
High sensitivity (>95%)
Thickening of the gallbladder wall
Detail signs of cholecystitis Impacted stone in the neck

Pericholecystic fluid

Initial screening test for Dilation of the extrahepatic

extrahepatic biliary obstruction bile duct (>10mm)

Evaluate intrahepatic lesions

Intraoperative ultrasound Assess resectability
Determine involvement of vascular structures
2. imaging examination

(2) Computed Tomography:

the most informative tool for examining intra-abdominal pathology

but for biliary tract disease, For gallstones sensitivity (55%~65%)

less effective in comparison
to ultrasonography Bile and stone appear isodense on CT (unless calcified)

Site
But it is accurate at identifying of extrahepatic biliary obstruction
cause

Powful for evaluating biliary Hepatobiliary or pancreatic neoplasm

tract disease when differential Liver abscess
diagnosis includes a question of
Hepatic parenchymal disease (cirrhosis, organ atrophy)
2. imaging examination

(3) Cholangiography
involves the installation of contrast into biliary tree
is the most accurate and sensitive method to delineate the intrahepatic and extrahepatic biliary tree.
precise location and cause to be ascertained

MRCP ( Magnetic Resonance Cholangiopancreatography, MRCP)

Non-invasive bile/water is phase contrasted

CT Cholangiography IV contrast excreted in the biliary system

ERCP ( Endoscopic Retrograde

invasive Cholangiopancreatography, ERCP)

(2%~5%complication)
PTC (Percutaneous Transhepatic
Cholangiography, PTC)
2. imaging examination

Cholangiography MRCP
2. imaging examination

Cholangiography
Illustrate distal common bile duct or ampullary obstruction

Provides tisse samples

ERCP
Offer the opportunity for a therapeutic intervention
(take out the stones in common bile duct)

Fault No information regarding Tumor size, local invasion,

distant spread.

decompress the biliary system

PTC Placement of transhepatic catheters
Anatomical landmarks

Access for non-operative dilation of stricture

2. imaging examination

Cholangiography ERCP
2. imaging examination

Cholangiography ERCP
2. imaging examination

(4) Radioisotope scanning

Tc-EHIDA
99m

functional evaluation
2. imaging examination

(5) choledochoscope
 3. congenital choledochal cyst

Biliary Cysts Cystic dilation of the biliary ducts

A high common bile duct–
Pathogenesis pancreatic duct junction a long common channel.

reflux of pancreatic mucosal injury,

anomalous junction of chronic inflammation,
fluid into the distal
pancreaticobiliary duct and weakening of the
common hepatic duct
bile duct wall

congenital hypoplasia

hereditary factor
3. congenital choledochal cyst

Biliary Cysts
(Type I-V)

I ： cyst
II ： diverticulum
III ： biliary openning
IV ： intra- and extra-
V ： Caroli disease
 3. congenital choledochal cyst

Biliary Cysts
Clinical Presentation The classic clinical triad associated with choledochal cysts includes
right upper quadrant pain, jaundice, and an abdominal mass .
The incidence of carcinoma in the choledochal cyst ranges from 2.5% to 26%
The diagnosis can be established with ultrasound or CT, MRI .
ERCP and PTC are useful in defining the ductal anatomy.

Management The goals of management are to relieve symptoms and prevent long-term
complications of biliary cysts such as cholangitis, portal hypertension, cirrhosis, and
potential carcinoma.
Cholecystectomy, resection of the choledochal cyst, with Roux-en-Y
hepaticojejunostomy is the appropriate treatment for type I and II choledochal cysts.
Liver transplantation offers a potential cure of Caroli's disease.
4. cholelithiasis (Calculous Biliary Disease)

Epidemiology
Among most common gastrointestinal illness,

Antopsy report: healthy people with a prevalence of 11%~36%

Female sex Hereditary spherocytosis

obesity
Risk factor Gastric surgery
for gallstones pregnancy
Sickle cell disease
Fatty foods

crohn’s disease
Terminal ileal resection
4. cholelithiasis

pathogenesis
Gallstones represent an inability to maintain certain biliary solute in a solubilized state,
primarily cholesterol and calcium salts.

cholesterol stones Pure cholesterol stones are uncommon,

Containing calcium salts in the center.
Classified by
cholesterol content

black
Pigment stones

brown
4. cholelithiasis

Cholesterol Gallstones
Cholesterol supersaturation in bile
Pathogenesis
involves three stages
Crystal nucleation

Stone growth Polyhedron shape

Gallbladder mucosal and motor function plays a key role in gallstone formation.

Micelles (bile salt-phospholipid-cholesterol complex)

The key to maintaining
cholesterol in solution is
Cholesterol –phospholipid vesicles

excess cholesterol production Exceed their capability to Crystal precipitation occur

transport cholesterol
4. cholelithiasis

Pigment Gallstones
Pigment stones contain less than 20% cholesterol and are dark owning to the presence of
calcium bilirubinate.
Black and brown stone have little in common and should be considered as separate entities.

Hereditary spherocytosis
Black pigment stones Associated with hemolytic conditions Sickle cell disease

(blood system disease) Cirrhosis

in hemolytic states, bilirubin load and
concentration of unconjugated bilirubin increase

Brown pigment stones Usually found in bile duct, contain more cholesterol and
calcium palmitate, associated bacterial infection
Bacteria producing slime that cause enzymatic hydrolysis of
soluble conjugated bilirubin glucuronide to produce insoluble free
bilirubin, which then precipitates with calcium.
4. cholelithiasis Calculous Biliary Disease

（ 1 ） cholecystolithiasis / gallstone
2/3 patients with gallstone present with these repeated attacks.
Constant and usually last 1 to 5 hours
pain Subsides by 24hours.
The primary symptom Persisits longer than 1day,suggest
acute cholecystitis
Often referred to Server enough to recall them accurately and
as biliary colic number them
（ typical
Clinical symptom ）
Accompany with other sympotoms:nausea,
vomiting, abdominal distension
presentation
Fever and jaundice Are rare
Asymptomatic In many cases, Treatment is not necessary

Atypical presentation Rule out other causes: peptic ulcer, pneumonia,

renal calculi, liver disease,hernia,reflux.
Physical examination
Completely normal or mild tenderness
and liver fanction tests
4. cholelithiasis

The presence of symptoms

Clinical presentation Treatment is necessary
Typically biliary colic

Diagnosis Ultrasound is the standard diagnostic exam for gallstones

relies on 1. Confirm the presence of gallstone

2. Provide other important information for surgeon

Diagnosis imaging Presence of polyps

Common bile duct diameter

Hepatic abnormalities
3. Occasionally , no evidence of gallstone on ultrasound
or only sludge
Mirizzi’s syndrome Severe jaundice is suggestive of common bile duct stones or
obstruction of the bile ducts by severe pericholecystic inflammation,
(Type I-IV) secondary to impaction of a stone in the infundibulum of the
gallbladder that mechanically obstructs the bile duct.
4. cholelithiasis Calculous Biliary Disease

elective
Laparoscopic cholecystectomy Optimal treatment for
symptomatic cholelithiasis
( LC )
90% Rendered symptom free after LC

Diabetic patients Have a cholecystectomy,

promptly
Management Special patients
symptomatic
Pregnant women Fail dietary modification

Undergo surgery during

the second trimester.
Diet advice Avoid dietary fats and large meal
4. cholelithiasis Calculous Biliary Disease
To be continued

Thank you!

Suyang
General Surgery Department
of Shengjing Hospital, CMU
4. cholelithiasis Calculous Biliary Disease

(2) Choledocholithiasis (extrahepatic bile duct stone)

Common bile duct stones are found in 6% to 12% of patients with stones in the gallbladder .
Primary ~ formed within the biliary tract
Common bile duct stones
Secondary ~ form initially in the gallbladder and
migrate through the cystic duct
retained < 2 years after cholecystectomy
Common bile duct stones

recurrent > 2 years after cholecystectomy

Primary stones with biliary stasis and infection . The causes of biliary stasis
include: biliary stricture, papillary stenosis, tumors, or other
(secondary) stones

Secondary stones

cholangitis abscess
result in
liver injury pancreatitis
4. cholelithiasis Calculous Biliary Disease

Choledocholithiasis

may be silent and are often

discovered incidentally
Asymptomatic
after cholecystectomy , about 1% to 2% of
patients present with a retained stone.

biliary colic, jaundice, lightening of the

presentation suspicious features stools, and darkening of the urine.
fever and chills may be present

Serum bilirubin (>3.0 mg/dL), serum

Lab test aminotransferases, and alkaline phosphatase
are commonly elevated

However, laboratory values may be

normal in 1/3 of patients.
4. cholelithiasis
Choledocholithiasis

accompanying stones in the gallbladder

estimate the diameter of the CBD ( >8mm)
Ultrasound bowel gas
( commonly the first test ) echogenic shadows in only 60-70%
CT
Diagnosis
sensitivity 95%
MRC
specificity 98%

ERCP potentially therapeutic test of choice

complications ： cholangitis and pancreatitis

MRCP+EUS
4. cholelithiasis
Choledocholithiasis
ERCP EST ENBD
provides ductal clearance of the stones

Endoscopic Suitable for : worsening cholangitis, ampullary stone impaction,

Cholangiography biliary pancreatitis, multiple comorbidities,
Fail in : multiplecirrhosis
stones, intrahepatic stones, impacted stones,
difficulty with cannulation, duodenal diverticula, or
biliary stricture

Complete clearance in 71% ~ 75%

Management with a 5% ~ 8% complication rate

Laparoscopic Common Bile Duct Exploration

Open Common Bile Duct Exploration
4. cholelithiasis
Choledocholithiasis
Endoscopic Cholangiography
With the increased use of endoscopic, percutaneous, and laparoscopic
Open techniques, open common bile duct exploration is rarely performed .
Common Bile Duct It is associated with low operative mortality (1%-2%) and operative
Exploration morbidity (8%-16%).
Stones impacted in the ampulla ( Roux-en-Y
choledochojejunostomy)

Management

Laparoscopic Common Bile Duct Exploration

4. cholelithiasis
Choledocholithiasis
Endoscopic Cholangiography
Open Common Bile Duct Exploration

Laparoscopic LCBDE through the cystic duct or common bile duct allows the stones
Common Bile Duct to be retrieved
Management Exploration
T tube is left in place allow for postoperative choledochoscope
4. cholelithiasis Calculous Biliary Disease

(3) hepatolithiasis (intrahepatic bile duct stone)

etiology infection, parasites, bile stasis, malnutrition, anatomic variation
pigment stone
1. obstruction; 2. infection; 3.carceration

pain; fever;
presentation
cirrosis, portal hypertention

management
5. Biliary system infection
(1) Acute Calculous Cholecystitis

Obstruction Gallbladder
biliary colic
of cystic duct distend

Gallstone
Pathophysiology Gallbladder Thickened and
dislodges Inflammation
wall become reddish wall with
subserosal resolve
inflamed most cases
hemorrhage
Severe cases 5%~10%

Necrosis of Abscess
gallbladder wall formation
5. Biliary system infection

pain Right upper quardrant pain

most common Similar in severity but longer in duration than colic
symptoms of AC

Other symptoms Fever, nausea, vomiting,

Clinical Physical exam Tenderness pain, guarding, rigidity Spread to

presentation peritoneum
Palpable gallbladder and Murphy’s sign

Lab tests Mild leukocytosis 12,000~14,000cells/mm3

Mild elevation in serum bilirubin and transaminases

Mirizzi’s syndrome Severe jaundice is suggestive of common bile duct

stones or obstruction of the bile ducts by severe
pericholecystic inflammation, secondary to
impaction of a stone in the infundibulum of the
gallbladder that mechanically obstructs the bile duct.
5. Biliary system infection

Clinical presentation Mentioned above

Diagnosis Ultrasound is the most uesful test

1. For cholecystitis: sensitivity 85%; specificity 95%

2. Identifying gallstone
Diagnosis imaging 3.Thickening fo the gallbladder wall (>4mm)
4.pericholecystic fluid

5.gallbladder distention
6.impacted stone
5. Biliary system infection

IV influids
Antibiotics Should be initialed after diagnosis

Analgesia

Cholecystectomy The definitive treatment for acut cholecystitis

Early operation is prefered over interval or delayed operation
Management
(2~3 day) (6~10 week,
cooling-off period)
Laparoscopic cholecystectomy Perfered approach to patient
( LC ) Conversion to open procedure if inflammation prevent
adequate visualization (4~35%)
Advantages in morbidity rate, hospital stay, time to
return to work.
Convert factor: increased age, male gender, obesity,
thickened gallbladder wall
5. Biliary system infection

(2) Acute Acalculous Cholecystitis （ Acute inflammation without gallstones ）

◆ accounts for 5% to 10% of all patients with acute cholecystitis

more commonly to gangrene, empyema, or perforation
◆
◆ exact etiology is unclear, gallbladder stasis and ischemia are believed as causative factors.
◆ occurs most frequently in elderly and critically ill patients after trauma, burns, long-term
parenteral nutrition, and major operations

﹡signs and symptoms parallel acute calculous cholecystitis

Presentation ﹡ may also present with only unexplained fever, leukocytosis,
﹡ and hyperamylasemia without right upper quadrant tenderness.
﹡ Ultrasonography is the diagnostic test of choice
﹡ Emergency cholecystectomy is the appropriate treatment
﹡ Because of the high incidence of gangrene, perforation, and
Management empyema, open cholecystectomy is often the preferred approach .
﹡ If patients are unfit for surgery, percutaneous, ultrasound-guided,
or CT-guided cholecystostomy is the treatment of choice
5. Biliary system infection

(3) Acute Cholangitis (AOSC/ACST)

Cholangitis is an ascending bacterial infection of the biliary ductal system with obstruction
most due to choledochal stones, benign and malignant strictures, anastomotic strictures,
cholangiocarcinoma, and periampullary cancer.
Fever and chills are the most common presentation
Charcot triad or Reynold's pentad may arise ( including jaundice, fever,
abdominal pain, mental status changes, and
hypotension )
Diagnosis When intrabiliary pressures rise to 18 to 20 cm H2O, organisms rapidly appear in
both the blood and lymph.
Leukocytosis, hyperbilirubinemia, and elevations of alkaline phosphatase and
transaminases all are common in patients with cholangitis .
ultrasound, CT, and MRI may be helpful in identifying the cause of obstruction.

IV antibiotics and aggressive hydration are the initial treatment .

Management Biliary decompression should be performed by ERCP or PTC
Or an emergent operation and decompression of the common bile duct with a
T tube should be performed.
the mortality rate of cholangitis is about 2%, but is much higher in patients with
toxic cholangitis (5%).
6. Primary Sclerosing Cholangitis (psc)

Primary sclerosing cholangitis (PSC) is a cholestatic liver disease characterized by

fibrotic strictures involving the intrahepatic and extrahepatic biliary tree in the absence of a
known cause.
Leads to bile duct loss, cirrhosis, and liver failure.
Genetic and immunologic factors appear to have a role in the pathogenesis of this disease.
Associated with inflammatory bowel disease and primarily ulcerative colitis.
Patients with PSC are at increased risk for developing cholangiocarcinoma.

About 75% of patients are symptomatic with evidence of cholestatic

Clinical Presentation liver disease such as jaundice, pruritus, and fatigue.
Symptoms of bacterial cholangitis are uncommon.
The median survival for patients with PSC is 10 to 12 years
Cholangiography confirms the diagnosis of PSC with evidence of diffuse multifocal
Diagnosis strictures in both intrahepatic and extrahepatic bile ducts . A liver biopsy can
determine the degree of hepatic fibrosis or the presence of cirrhosis .
Best treatment is liver transplantation, with a 5-year patient survival rate of 85%
Management .
the risk for cholangiocarcinoma should be considered in the managment.
Biliary strictures can be dilated or stented through percutaneous or endoscopic route,
but the role of nontransplant surgery is limited .
7. biliary ascariasis

etiology ascariasis
acute pancreatitis; cholangitis; liver abscess; gallbladder perforation;

severe symptoms vs slight sign （ slight tenderness ）

presentation
colic infection and jaundice

management ultrasonography diagnosis

spasmolysis

non-operative treatment expelling parasite

infection control
ERCP
operation
8. common complications of biliary system disease

3%~10% of acute cholecystitis

(1) gallbladder perforation
LC ; OC; cholecystostomy; peritoneal drainage

cause : infection ; injury; tumor; vascular factor

(2) hemobilia cyclic hemorrhage
managment: non-operative treatment
arterial embolism or operation

(3) inflammatory stricture of the bile duct

pathology : stricture + cystic expansion + pigment stone
symptoms : repeated cholangitis
managment: EST; Roux-en-Y ; hepatectomy

(4) bacterial liver abscess

(5) acute gallstone pancreatitis

9. bile duct injury

(1) traumatic bile duct injury

Classification
(2) iatrogenic bile duct injury

Etiology anatomical factor; pathological factor; operative factor; heat injury; ischemic injury

during operation: bile leakage; double opening; intraoperative cholangiography

Diagnosis
after operation: bile peritonitis; bile drainage; obstructive jaundice;
repeated cholangitis; perihepatic seroperitoneum
repair < 3mm
Managment anastomosis < 2cm
Roux-en-Y > 2cm
10. biliary system tumor

(1) Polyploid Lesions of the Gallbladder

cholesterol polyps Most common, multiple, <10mm

adenomyomatosis
adenomas difficult to distinguish from adenocarcinoma

symptomatic should undergo LC.

asymptomatic Lesions ＞ 10 mm ， LC
Lesions < 10 mm, observed with follow-up imaging.
10. biliary system tumor gallbladder cancer

(2) carcinoma of the gallbladder

aggressive malignancy, 5-year survival rates : 5% to 38%. prognosis is poor.
Incidence
the fifth most common gastrointestinal malignancy
two to three times more common in women than
men, in part because of the higher incidence of
gallstones in women.
Etiology

Risk factors: Gallstones, Porcelain gallbladder, Anomalous pancreatobiliary

junction ， Choledochal cysts, Adenomatous gallbladder polyps,
Primary sclerosing cholangitis, Obesity, Salmonella typhi infection
Among these factors, gallstones are the most common one (. in 75% to 90% of cases )
The incidence of gallbladder cancer is about seven times more common in the presence of
cholelithiasis and chronic cholecystitis than in people without gallstones.
the risk is higher in symptomatic gallstones than asymptomatic gallstones.
the pathogenesis is likely related to chronic inflammation.
10. biliary system tumor gallbladder cancer

Pathology and Staging

Ninety percent of cancers of the gallbladder are classified as adenocarcinoma
The current TNM classification of the American Joint Committee on Cancer (AJCC) is
shown in Table below. The appropriate management and overall prognosis are strongly
dependent on tumor stage.
TNM Staging for Gallbladder Cancer
T1 Tumor invades lamina propria (T1a) or muscular (T1b) layer
T2 Tumor invades perimuscular connective tissue, no extension beyond the serosa or into the
liver
T3 Tumor perforates the serosa (visceral peritoneum) and/or directly invades into liver and/or
one other adjacent organ or structure such as the stomach, duodenum, colon, pancreas,
omentum, or extrahepatic bile ducts
T4 Tumor invades main portal vein or hepatic artery or invades multiple extrahepatic organs
and/or structures
N0 No lymph node metastases
N1 Regional lymph node metastases
M0 No distant metastases
M1 Distant metastases
10. biliary system tumor gallbladder cancer

Clinical Presentation

right upper quadrant abdominal pain , often mimicking cholecystitis and cholelithiasis.

40% of patients present with symptoms of chronic cholecystitist,.

Another common presentation is similar to acute cholecystitis, with a short duration of
pain associated with vomiting, fever, and tenderness pain.

Signs and symptoms of malignant biliary obstruction with jaundice, weight loss, and
right upper quadrant pain are also common .

Gallbladder cancer is often misdiagnosed as chronic cholecystitis, pancreatic cancer,

acute cholecystitis, choledocholithiasis, or gallbladder hydrops
10. biliary system tumor gallbladder cancer

Diagnosis

Ultrasonography is often the first diagnostic examination. The sensitivity in the detection
of gallbladder cancer ranges from 70% to 100%.
A mass replacing the gallbladder lumen and an irregular gallbladder wall are common
sonographic features of gallbladder cancer.

CT scan usually demonstrates a mass replacing the gallbladder or extending into adjacent
organs. also demonstrates the adjacent vascular anatomy.

With MRI, gallbladder cancers may be differentiated from the adjacent liver and biliary
obstruction or encasement of the portal vein may also be easily visualized

Cholangiography also may be helpful.

If radiographic studies suggest that the tumor is unresectable (liver or peritoneal
metastases, portal vein encasement, or extensive hepatic invasion), a biopsy of the
tumor is warranted and can be performed under ultrasound or CT guidance.
10. biliary system tumor gallbladder cancer

Management
The appropriate operative procedure for gallbladder cancer is determined by the pathologic stage.
Cholecystectomy is adequate therapy for patients with T1 tumors .
Patients suspected gallbladder cancer should undergo open cholecystectomy to minimize
the chance of bile spillage and tumor dissemination .

Gallbladder cancer of stages II and III, the gallbladder should be managed with an
“extended cholecystectomy.” lymphadenectomy of the cystic duct,
pericholedochal, portal, right celiac, and posterior pancreatoduodenal lymph nodes
In those cases in which the cystic duct stump margin is positive for malignancy, common
duct resection with Roux-en-Y reconstruction is mandatory.
For larger tumors, an anatomic liver resection may be required to achieve a
histologically negative margin. ( >2cm margin)
No randomized data have demonstrated improved survival with either chemotherapy
or radiation.
Survival （ depending on the stages ）
fewer than 15% of all patients with gallbladder cancer are alive after 5 years. The median
survival for stage IV patients at the time of presentation is only 1 to 3 months.
10. biliary system tumor Bile Duct Cancer

(3) carcinoma of the bile duct

Uncommon tumor; Located most commonly at the hepatic duct bifurcation (60%-80% of cases).
Less commonly in the distal common bile duct or in the intrahepatic bile ducts.

Incidence
The reported incidence of cholangiocarcinoma in the United States is 1 or 2 cases per
100,000 population.

Risk Factors primary sclerosing cholangitis, choledochal cysts, and hepatolithiasis

Bile duct cancers in patients with primary sclerosing cholangitis are most often
extrahepatic, commonly occur near the hepatic duct bifurcation
Hepatolithiasis is also a definite risk factor for cholangiocarcinoma, which will
develop in 5% to 10% of patients with intrahepatic stones.
Hepatitis B and C are also now recognized as risk factors of intrahepatic cholangiocarcinoma.

Biliary-enteric anastomosis may also increase the future risk for cholangiocarcinoma. .
10. biliary system tumor Bile Duct Cancer

Staging and Classification

Intrahepatic

classified anatomically Perihilar

Distal

Bismuth classification of I common hepatic duct

perihilar cholangiocarcinoma
II bifurcation without involvement of
secondary intrahepatic ducts.

III right or left hepatic ducts

involve the hepatic ducts on both sides

IV
10. biliary system tumor Bile Duct Cancer

Staging
Cholangiocarcinoma is also staged according to the tumor, lymph node, metastasis
(TNM) classification of the AJCC.

Stage 0 Tis N0 M0
Stage I T1 N0 M0
Stage II T2 N0 M0
Stage III T1 or T2 N1 or N2 M0
Stage IVA T3 Any N M0
Stage IVB Any T Any N M1

Tis, carcinoma in situ; T1, tumor invades the subepithelial connective tissue; T2, tumor invades
peri. bromuscular connective tissue; T3, tumor invades adjacent organs.
N0, no regional lymph node metastases; N1, metastasis to hepatoduodenal ligament lymph
nodes; N2, metastasis to peripancreatic, periduodenal, periportal, celiac, and/or superior
mesenteric artery lymph nodes.
M0, no distant metastasis; M1, distant metastasis.
10. biliary system tumor Bile Duct Cancer

Clinical Presentation

jaundice More than 90% of patients with perihilar or distal tumors present with jaundice

Patients with intrahepatic cholangiocarcinoma are rarely jaundiced until late

in the course of the disease.

Less common presenting pruritus, fever, mild abdominal pain,

clinical features fatigue, anorexia, and weight loss.

Except for jaundice, the physical examination is usually normal in patients

with cholangiocarcinoma.
10. biliary system tumor Bile Duct Cancer

Diagnosis

Lab test total serum bilirubin level greater than 10 mg/dL

elevations in alkaline phosphatase
Serum CA 19-9 may also be elevated in patients

The radiologic evaluation of patients with cholangiocarcinoma should

imaging. delineate the overall extent of the tumor, including involvement of the bile
ducts, liver, hilar vessels, and distant metastases.

The initial radiographic studies consist of either abdominal ultrasound

or CT scanning

MRC offers good resolution of both the intrahepatic and

extrahepatic biliary tree, but could be substituted with PTC or ERCP in
patients that will require preoperative or palliative biliary drainage
10. biliary system tumor Bile Duct Cancer

Surgical exploration should be undertaken in patients without evidence of

metastatic or locally unresectable disease; intraoperatively, some patients may be found
to have either peritoneal or hepatic metastases or, more likely, locally unresectable
disease

Distal cholangiocarcinoma pancreaticoduodenectomy

Intrahepatic cholangiocarcinoma hepatic resection

Perihilar cholangiocarcinoma bloc resection of the

extrahepatic bile ducts and
gallbladder with 5- to 10-mm
bile duct margins, and
regional lymphadenectomy
with Roux-en-Y
hepaticojejunostomy.
Thank you!

Fan Ying
General Surgery Department
of Shengjing Hospital, CMU