Rose Lou Marie C.
Agbay, MD DPSP
Normal gallbladder histology. The undulating mucosal epithelium overlies a delicate
lamina and only one smooth muscle layer. This is different from elsewhere in the
gut, where two muscle layers exist (muscularis mucosa and muscularis propria).
Congenital Anomalies
1. Congenitally absent
2. Gallbladder duplication
conjoined or independent cystic ducts
3. Bilobed gallbladder
presence of longitudinal or transverse septum
4. Aberrant locations of the gallbladder
partial or incomplete embedding on the liver substance
5. Folded fundus
phrygian cap
6. Agenesis
any portion of the hepatic or common bile ducts
7. Hypoplastic narrowing of biliary channels
true “biliary atresia”
Congenital Anomalies
1. Congenitally absent
2. Gallbladder duplication
conjoined or independent cystic ducts
3. Bilobed gallbladder
presence of longitudinal or transverse septum
4. Aberrant locations of the gallbladder
partial or incomplete embedding on the liver substance
5. Folded fundus
phrygian cap
6. Agenesis
all or any portion of the hepatic or common bile ducts
7. Hypoplastic narrowing of biliary channels
true “biliary atresia”
CHOLELITHIASIS (GALLSTONES)
cholesterol stones
- 90% of cases
- crystalline cholesterol monohydrate
pigment stones
- bilirubin calcium salts
Pathogenesis of Cholesterol Stones
Four contributing factors for cholelithiasis:
1. The bile must be supersaturated with
cholesterol
- Cholesterol is rendered soluble in bile by
aggregation with water-soluble bile salts
and water-insoluble lecithins
2. Hypomotility of the gallbladder promotes
nucleation
3. Cholesterol nucleation in the bile is
accelerated
- When cholesterol concentrations exceed
the solubilizing capacity of bile
(supersaturation), cholesterol can no
longer remain dispersed and nucleates
into solid cholesterol monohydrate
crystals
4. Hypersecretion of mucus in the gallbladder
traps the nucleated crystals, leading to their
aggregation into stones
Pathogenesis of Pigment Stones
Pigment gallstones
complex mixtures of abnormal insoluble calcium salts of
unconjugated bilirubin along with inorganic calcium salts
Disorders that are associated with elevated levels of
unconjugated bilirubin in bile
1. Hemolytic syndromes
secretion of conjugated bilirubin into the bile increases
about 1% of bilirubin glucuronides are deconjugated in the biliary
tree, the large amounts of unconjugated bilirubin produced may
exceed its solubility
2. Severe ileal dysfunction (or bypass)
3. Bacterial contamination of the biliary tree
4. Infection of the biliary tract
Escherichia coli, Ascaris lumbricoides, O. sinensis
infection of the biliary tract leads to release of microbial β-
glucuronidases, which hydrolyze bilirubin glucuronides
Cholesterol stones
Pure cholesterol stones are pale yellow, round to ovoid, and have a finely
granular, hard external surface .
Stones composed largely of cholesterol are radiolucent.
Pigment gallstones
“Black” pigment stones
-contain oxidized polymers of the
calcium salts of unconjugated
bilirubin, small amounts of calcium
carbonate, calcium phosphate, and
mucin glycoprotein, and some
cholesterol monohydrate crystals
- sterile gallbladder bile
- < 1.5 cm in diameter; present in
great number
- contours are usually spiculated
and molded
- radiopaque
Pigment gallstone
“Brown” pigment stones
-pure calcium salts of unconjugated
bilirubin, mucin glycoprotein, a
substantial cholesterol fraction, and
calcium salts of palmitate and
stearate
- infected intrahepatic or extrahepatic
ducts
- laminated and soft and may have a
soap-like or greasy consistency
- radiolucent
Cholesterolosis
- cholesterol hypersecretion by the
liver promotes excessive
accumulation of cholesterol esters in
the lamina propria of the gallbladder
- mucosal surface – studded with
minute yellow flecks (strawberry
appearance)
CHOLECYSTITIS
Acute cholecystitis
Chronic cholecystitis
Acute Cholecystitis
Acute calculous cholecystitis
an acute inflammation of the gallbladder
90% of cases caused by obstruction of the neck or cystic duct
primary complication of gallstones
develops in diabetic patients who have symptomatic gallstones
most common reason for emergency cholecystectomy
Acalculous cholecystitis
cholecystitis without gallstones
10% of patients with cholecystitis
Acute calculous cholecystitis
-- chemical irritation and inflammation of the obstructed gallbladder
mucosal
disruption of the mucosal epithelium
phospholipases
normally protective exposed to the direct
hydrolyzes luminal
glycoprotein mucus detergent action of bile
lecithins to toxic
layer salts
lysolecithins
prostaglandins released distention and
within the wall of the increased intraluminal
gallbladder dysmotility
distended gallbladder pressure compromise
develops
contribute to mucosal blood flow to the
and mural inflammation mucosa
-- occur in the absence of bacterial infection
-- only later in the course may bacterial contamination develop
Acute calculous cholecystitis
progressive right upper quadrant or epigastric pain
mild fever, anorexia, tachycardia, sweating, nausea, and
vomiting
(-) jaundice
hyperbilirubinemia
obstruction of the common bile duct
mild to moderate leukocytosis
mild elevation in serum alkaline phosphatase
Acute acalculous cholecystitis
cystic duct
obstruction in the
ischemia
absence stone
formation
Contributing factors
inflammation and edema of the wall compromising blood flow
gallbladder stasis, and accumulation of microcrystals of cholesterol
(biliary sludge), viscous bile, and gallbladder mucus
Risk factors
sepsis with hypotension and multisystem organ failure
immunosuppression
major trauma and burns
diabetes mellitus
infections
Acute acalculous cholecystitis
clinical symptoms tend to be more insidious
symptoms are obscured by the underlying conditions precipitating the
attacks
diagnosis rests on a high index of suspicion
delay in diagnosis or the disease
gangrene and perforation
primary bacterial infection
Salmonella typhi and staphylococci
Acute cholecystitis enlarged and tense
bright red or blotchy, violaceous
to green-black discoloration,
imparted by subserosal
hemorrhages
serosal covering is frequently
layered by fibrin
an obstructing stone is usually
present in the neck of the
gallbladder or the cystic duct
lumen may contain one or more
stones and is filled with a cloudy
or turbid bile that may contain
large amounts of fibrin, pus, and
hemorrhage
Chronic Cholecystitis
repeated episodes of mild to severe acute cholecystitis
>90 % of cases associated with cholelithiasis
supersaturation of bile predisposes to both chronic inflammation
and stone formation
E. coli and enterococci
Clinical features:
recurrent attacks of either steady or colicky epigastric or right
upper quadrant pain
nausea, vomiting, and intolerance for fatty food
Chronic Cholecystitis
Serosa
smooth and glistening
dulled
subserosal fibrosis
dense fibrous adhesion
sequelae of preexistent
acute inflammation
Wall
variably thickened
opaque gray-white
Lumen
fairly clear, green-yellow,
mucoid bile
stones
Chronic Cholecystitis
--scattered lymphocytes, plasma Rokitansky-Aschoff sinuses
cells, and macrophages are found in buried crypts of epithelium within the
the mucosa and in the subserosal gallbladder wall
fibrous tissue --reactive proliferation of the mucosa
--marked subepithelial and and fusion of the mucosal folds
subserosal fibrosis
Hydrops of the gallbladder
Choledocholithiasis
Presence of stones within the bile ducts of the biliary tree
Associated with...
pigmented stones
biliary tract infections
Asymptomatic
Symptomatic
(1) obstruction
(2) pancreatitis
(3) cholangitis
(4) hepatic abscess
(5) secondary biliary cirrhosis
(6) acute calculous cholecystitis
Cholangitis
Bacterial infection of the bile ducts
result of any lesion that creates obstruction to bile flow
choledocholithiasis
biliary strictures
indwelling stents or catheters
tumors
acute pancreatitis
fungi
viruses
parasites
Ascending cholangitis
Infection of intrahepatic biliary radicles
bacteria enter the biliary tract through the sphincter of Oddi
enteric gram-negative aerobes
E. coli, Klebsiella, Enterococcus, or Enterobacter
Clostridium and Bacteroides (mixed infection)
acute inflammation of the wall of the bile ducts with entry of neutrophils
into the luminal space
fever, chills, abdominal pain, and jaundice
suppurative cholangitis
most severe form
purulent bile fills and distends bile ducts
Biliary atresia
a complete or partial obstruction of the lumen of the
extrahepatic biliary tree within the first 3 months of life
progressive inflammation and fibrosis of intrahepatic or extrahepatic
bile ducts
major contributor to neonatal cholestasis
occurs in approximately 1 : 12,000 live births
single most frequent cause of death from liver disease in early
childhood
accounts for 50% to 60% of children referred for liver transplantation
rapidly progressing secondary biliary cirrhosis
Biliary atresia
Two major forms
Fetal form
associated with other anomalies
ineffective establishment of laterality of thoracic and abdominal organ
development
malrotation of abdominal viscera, interrupted inferior vena cava,
polysplenia, and congenital heart disease
presumed cause..
aberrant intrauterine development of the extrahepatic biliary tree
Perinatal form
more common
unknown etiology
presumed cause...
viral infection and autoimmunity
reovirus, rotavirus, and cytomegalovirus
Biliary atresia
Type I
disease is limited to the common duct with patent proximal
branches
Type II
disease is limited to the hepatic bile ducts with patent proximal
branches
Type III
obstruction of bile ducts at or above the porta hepatis
Salient features of biliary atresia
-inflammation and fibrosing stricture of the hepatic or common bile ducts
-periductular inflammation of intrahepatic bile ducts
-progressive destruction of the intrahepatic biliary tree
Liver biopsy
-marked bile ductular proliferation, portal tract edema and fibrosis, and
parenchymal cholestasis
-inflammatory destruction of intrahepatic ducts leads to paucity of bile ducts and
absence of edema or bile ductular proliferation
Biliary atresia
Clinical Features
Female>Male
normal birth weight postnatal weight gain
normal stools acholic stools
serum bilirubin: 6 to 12 mg/dL
moderately elevated aminotransferase
moderately elevated alkaline phosphatase
liver transplantation with accompanying donor bile ducts
death usually occurs within 2 years of birth
without surgical intervention
Choledochal cysts
congenital dilations of the common bile duct
children before age 10
jaundice and/or recurrent abdominal pain (biliary colic)
female-to-male ratio is 3 : 1 to 4 : 1
Consequence...
stone formation
stenosis and stricture
pancreatitis
obstructive biliary complications within the liver
bile duct carcinoma
Different types of choledochal cysts. Normal anatomy is illustrated in the left upper frame.
Type I choledochal cyst is most common and is divided into three categories. Illustrated
here is diffuse or cylindrical dilatation of the common bile duct. Type V choledochal cyst is
an intrahepatic bile duct cyst, which may be single or multiple.
Surgically resected type I choledochal The lining of a choledochal cyst is eroded
cyst in continuity with the gallbladder except for a small cleft-like space centrally.
and cystic duct. The lining of the cyst is The wall is edematous with scant chronic
hyperemic and contained scant bilious inflammation.
material.
Adenomyosis of the gallbladder
hyperplasia of the muscle layer, containing intramural hyperplastic
glands
CARCINOMA OF THE
GALLBLADDER
CARCINOMA OF THE GALLBLADDER
most common malignancy of the extrahepatic biliary tract
slightly more common in women
occurs most frequently in the seventh decade of life
mean 5-year survival rate about 5% to 12% despite surgical
intervention
gallstones (cholelithiasis)
most important risk factor (in 95% of cases)
gallbladders containing stones or infectious
irritative trauma and chronic inflammation
carcinogenic derivatives of bile acids
Infiltrating pattern
-more common
-poorly defined area of diffuse
thickening and induration of the
gallbladder wall
-several square centimeters
or may involve the entire
gallbladder
-scirrhous and have a very firm
consistency
-deep ulceration can cause
direct penetration of the
gallbladder wall or fistula
formation to adjacent viscera
into which the neoplasm has
grown
Exophytic pattern
grows into the lumen as an
irregular, cauliflower mass but
at the same time invades the
underlying wall
luminal portion may be
necrotic, hemorrhagic, and
ulcerated
the most common sites of
involvement are the fundus
and the neck; about 20%
involve the lateral walls
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