19 - Diseases of The Gallbladder and The Extrahepatic Biliary

Diseases of the Gallbladder and
the Extrahepatic Biliary Tract

Dr Binyam.M (MD)
Out line
• Normal gall bladder
• Congenital anomalies
• Disorders of the Gall bladder
• Disorders of the extrahepatic bile ducts
• Tumors
Normal gallbladder
Congenital Anomalies
1.Absence
2.Aberrant location
3.Bilobed gall bladder
4.Biliary atresia.
5.Duplication
6.Folded fundus (phrygian cap)
Diseases of Gallbladder
Cholelithiasis (Gallstones)
• 10% to 20% of Western adult
• Higher in Latin America(20% to 40%)
• There are two main types of gallstones
1. In the West about 80% are cholesterol
stones, containing crystalline cholesterol
monohydrate.
2. The remainder are composed predominantly
of bilirubin calcium salts and are designated
pigment stones.
Pathogenesis
• Bile is the only significant pathway for
elimination of excess cholesterol from the
body.
• Four events simultaneously occur:
1. Supersaturation of the bile with cholesterol
2. Cholesterol nucleation in bile is accelerated
3. Hypomobility of the gallbladder (stasis),
which promotes nucleation
4. Mucus hypersecretion to trap the crystals,
and enhancing their aggregation into stones
Ctd…
• Pigment gallstones are complex mixtures of
abnormal insoluble calcium salts of unconjugated
bilirubin along with inorganic calcium salts
• The presence of unconjugated bilirubin in the

biliary tree increases the likelihood of pigment
stone formation (infections of the biliary tree)
• The precipitates are primarily insoluble calcium

bilirubin salts
Risk Factors for Gallstones
1.Cholesterol Stones.
1. Advancing age
2. Female sex hormones
3. Female gender
4. Oral contraceptives
5. Pregnancy
6. Obesity
7. Rapid weight reduction
8. Gallbladder stasis
9. Inborn disorders of bile acid metabolism
Ctd…
2.Pigment Stones
1. Chronic hemolytic syndromes
2. Biliary infection
3. Gastrointestinal disorders: ileal disease (e.g.
Crohn disease), ileal resection or bypass, cystic
fibrosis with pancreatic insufficiency
 However, 80% of individuals with gallstones

have no identifying risk factors other than age
and gender
Morphology
• Cholesterol stones arise exclusively in the
gallbladder and consist of 50% to 100%
cholesterol.
• Pure cholesterol stones are pale yellow
 They are ovoid and firm
 They can occur singly but most are multiple
• Most cholesterol stones are radiolucent,
• although as many as 20% may have sufficient
calcium carbonate to render them
radiolucent = penetratable by x-rays
radiopaque
radiopaque = impentratable by x-rays
Ctd…
 Pigment stones may arise anywhere in the
biliary tree
• Classified as
1. black (Sterile)
2. brown (Infected)
• The stones contain calcium salts of unconjugated bilirubin
• Black stones are usually small and present in large quantities.

• Brown stones tend to be single or few in number.
• Because of calcium carbonates and phosphates,

50% to 75% of black stones are radiopaque
Clinical features
• 70% to 80% remain asymptomatic throughout life
• The symptoms are striking: pain tends to be
excruciating(very painfuL),either constant or
"colicky" (spasmodic)
• Inflammation of the gallbladder also generates pain
• Severe complications include
1. empyema
2. perforation
3. fistulae
4. inflammation of the biliary tree
5. obstructive cholestasis or pancreatitis
• Intestinal obstruction - "gallstone ileus”
Intestinal obstruction (gallstone Ileus):
Fistula was formed due

to inflammation of gall
bladder
Then stone go through the

fistula to the iliac “Narrow
site of small intestine” lead
to gallstone ileus”
Cholecystitis
• Inflammation of the gallbladder may be acute,
chronic, or acute superimposed on chronic.
• Almost always occurs in association with

gallstones.
• Its epidemiologic distribution closely parallels

that of gallstones
Acute Cholecystitis
• Precipitated by obstruction of the gallbladder
neck or cystic duct
• Most common major complication of
gallstones and the most common reason for
emergency cholecystectomy
• Acute abdomen and occasionally symptoms
may be mild and resolve without medical
intervention
Pathogenesis
1. Results from chemical irritation and
inflammation of the obstructed gallbladder
2. Lecithin to lysolecithin, which is toxic
3. The protective glycoprotein mucous layer is
disrupted, exposing the mucosal epithelium
4. Prostaglandins released within the wall
5. Compromised blood flow to the mucosa
• These events occur in the absence of bacterial

infection; only later may bacterial
contamination develop
Acute acalculous Cholecystitis
• 5% to 12% of acute cholecystitis
– contain no gallstones
– Thought to result from ischemia
• Most of these cases occur in seriously ill
patients:
1. Diabetes mellitus
2. Immunosuppresion
3. severe burns & severe trauma (e.g. motor vehicle
accidents)
4. sepsis
Morphology
Acute cholecystitis
• Gallbladder is usually enlarged and tense
• Fibrin and, in severe cases, suppurative exudate may
cover the serosa
• The gallbladder lumen is filled with cloudy or turbid
bile that contains
– fibrin
– blood
– frank pus.
• In 90% of cases stones are present, blocking
1. neck of the gallbladder
2. cystic duct
Empyema of the gallbladder
• Gallbladder wall is thickened, edematous, and
hyperemic.
• In severe cases it is transformed into a green-
black necrotic organ.
empyema of gallblader = severe acute cholecystitis
with purulent inflammation of the gallbladder.
Gangrenous cholecystitis
• Histologically, the usual patterns of acute
inflammation are seen(i.e., edema, leukocytic
Chronic Cholecystitis
• May be the sequel to repeated bouts of acute
cholecystitis, but in most instances it develops
without any history of acute attacks
• Like acute cholecystitis it is almost always
associated with gallstones
Ctd…
• The evolution of chronic cholecystitis is obscure
• Gallstones do not seem to have a direct role in
– initiation of inflammation
– development of pain
• Rather, supersaturation of bile predisposes to
both chronic inflammation and, stone formation
• Microorganisms, usually
– E. coli
– enterococci, can be cultured from the bile in only
about one-third of cases
Morphology
• The gallbladder may be contracted, normal
sized, or enlarged
• Mucosal ulcerations are infrequent; the
subserosa is often thickened from fibrosis
• In the absence of superimposed acute
cholecystitis, mural lymphocytes are the only
sentinels of inflammation
Complications
1. Bacterial superinfection with cholangitis or
sepsis
2. Gallbladder perforation and local abscess
formation
3. Gallbladder rupture with diffuse peritonitis
4. Biliary enteric (cholecystenteric) fistula
DISORDERS OF EXTRAHEPATIC BILE DUCTS
Choledocholithiasis
• The presence of stones within the biliary tree
• Asymptomatic stones are found in about 10%
of patients at the time of surgical
cholecystectomy
Ctd…
• Symptoms may develop because of
1. biliary obstruction
2. pancreatitis
3. cholangitis
4. hepatic abscess
5. chronic liver disease with secondary biliary
cirrhosis
6. acute calculous cholecystitis
Cholangitis
• Acute inflammation of the wall of bile ducts
• Almost always caused by bacterial infection of
the normally sterile lumen
• Obstruction is the cause most commonly by
choledocholithiasis
• Ascending cholangitis
- refers to the propensity of bacteria, once
within the biliary tree,
to infect intrahepatic biliary ducts
Ctd…
• The usual pathogens are
1. E. coli
2. Klebsiella
3. Clostridium
4. Bacteroides
5. Enterobacter
6. group D streptococci are also common.
• Usually mixed organisms
• In some world populations, parasitic cholangitis is a
significant problem: Fasciola hepatica,
schistosomiasis, Clonorchis sinensis or Opisthorchis
viverrini, and cryptosporidiosis
Clinical feature
• Bacterial cholangitis usually produces
1. fever
2. chills
3. abdominal pain
4. jaundice
• The most severe form of cholangitis is suppurative
cholangitis,
• in which purulent bile fills and distends bile ducts,
with an attendant risk of liver abscess formation
Biliary Atresia
• Biliary atresia is defined as a complete
obstruction of bile flow caused by destruction
or absence of all or part of the extrahepatic
bile ducts with in the first three months of life.
• A major contributor to neonatal cholestasis,
accounting for one-third of infants with
neonatal cholestasis
• It is the most frequent cause of death from
liver disease in early childhood
Pathogenesis
• Two major forms based on timing of luminal
obliteration
1. Fetal form
2. Perinatal form
Ctd…
• The salient features of biliary atresia include
(1) inflammation and fibrosing stricture of the hepatic
or common bile ducts
(2) inflammation of major intrahepatic bile ducts,
with progressive destruction of the intrahepatic
biliary tree
(3) florid features of biliary obstruction on liver biopsy
(4) periportal fibrosis and cirrhosis within 3 to 6
months of birth
Tumors
Carcinoma of the Gallbladder
Morphology
• Cancers of the gallbladder assume either
exophytic or infiltrating patterns of
growth(more common)
• The infiltrating tumors are scirrhous and very
firm
• The exophytic pattern grows into the lumen as
an irregular, cauliflower mass, but at the same
time it invades the underlying wall
Ctd…
• Most carcinomas of the gallbladder are
adenocarcinomas
• Papillary, Poorly differentiated, or
Undifferentiated infiltrating tumors
• About 5% are squamous cell carcinomas or
have adenosquamous differentiation
• Advanced disease: liver directly, the cystic
duct and adjacent bile ducts and portal
hepatic lymph nodes
Cholangiocarcinoma of
Intrahepatic bile ducts

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19 - Diseases of The Gallbladder and The Extrahepatic Biliary

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Diseases of the Gallbladder and

the Extrahepatic Biliary Tract

• The presence of unconjugated bilirubin in the

• The precipitates are primarily insoluble calcium

 However, 80% of individuals with gallstones

• Black stones are usually small and present in large quantities.

• Because of calcium carbonates and phosphates,

Fistula was formed due

Then stone go through the

• Almost always occurs in association with

• Its epidemiologic distribution closely parallels

• These events occur in the absence of bacterial

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