Gall Bladder & Cystic

Duct
Development and congenital anomalies
Congenital abnormalities of GB and Bile
duct
1. Absence of GB
2. Double GB
3. Septum in GB – Phrygian cap
4. Diverticulum of GB
5. Floating GB – GB hang on mesentery liable to undergo torsion
6. Low insertion of cystic duct – Cystic duct open to CBD near ampulla
7. Accessory cholecystohepatic duct – passing directly to GB from liver
Extrahepatic biliary atresia

 Present in 1/12,000 live births

 Male and female – equal incidence
 Extrahepatic bile ducts are progressively destroyed around the time of birth
 Eventually results in biliary cirrhosis and portal hypertension
 Untreated – liver failure occurs before age of 3
 3 main types
 Type 1 – atresia restricted to CBD
 Type 2 – atresia of CHD
 Type 3 – atresia of R and L hepatic ducts
 Assosciated anomalies in 20% cases – cardiac lesions, polysplenia, situs
inversus, Absent venecava and preduodenal portal vein
Clinical features

 1/3rd pts are jaundiced at birth

 LFT shows obstructive pattern
 Prolonged steatorrhea – leads to osteomalacia
 Severe pruritis
 Clubbing and skin xanthomas
D/D

 Neonatal jaundice due to alpha-1 antitrypsin deficiency, choledochal cyst,

inspissated bile syndrome
 Liver biopsy and radionucleotide excretion scans are essential
Rx

 Roux-en-y hepaticojejunostomy
 Kasai procedure – radical excision of all bile duct tissue extending upto liver
capsule. And Roux-en-y jejunal anastomosis to the exposed area of liver.
Better results if done before the age of 8wks. Complication – bacterial
cholangitis, hepatic fibrosis, portal hypertension.
 Liver transplantation if the above procedure is unsuccessful
Caroli’s disease – congenital dilatation
of intrahepatic ducts
 Rare congenital condition characterised by multiple irregular saccular
dilatations of the intrahepatic ducts, separated by segments of normal or
stenotic ducts, with a normal extrahepatic biliary system
 Associated with congenital hepatic fibrosis.
 Presents with – abdominal pain, cholangitis or end stage liver disease
 Majority presents before 30 yrs of age
 Male female – equal distribution
Management

 Multidisciplinary
 Cholangitis / jaundice – treated with appropriate antibiotic therapy and
endoscopic or interventional stenting
 Malignancy is a complication of longstanding disease
 Hepatic resection in pts with limited disease
 Pt with diffuse disease and concomitant hepatic fibrosis – liver
transplantation.
 Recurrence is common, particularly after resection, and long term
surveillance is required.
Choledochal cyst

 Congenital dilatation of intra and/or extrahepatic biliary system.

 Pathogenesis – unclear
 Anomalous junctions of biliary pancreatic junctions observed
 Todani and collegues proposed a classification of cystic disease of biliary tract.
 Type 1 – most common, accounts for 75% of pts
 Type 1a and type 1b – diffuse cystic. Extension into pancreas in 1b
 Type 2 – diverticulum of common bile duct
 Type 3 – diverticulum within pancreas
 Type 4 – extension into liver
 Type 5 – cystic dilatation only of intrahepatic ducts
Clinical features

 May present at any age with jaundice, fever, abdominal pain and rt upper
quadrant mass on examination.
 60% cases are diagnosed before the age of 10yrs
 Adults – pancreatitis
 Increased risk of cholangiocarcinoma with the risk varying directly with age at
dx
Investigation

 USG – confirm presence of abnormal cyst

 MRI/MRCP – reveal anatomy , esp relationship btw the lower end of bile duct
and pancreatic duct.
 CT – delineating the extent of intra- or extrahepatic dilatation
Treatment

 Radical excision of the cyst with reconstruction of biliary tract with Roux-en-y
loop of jejunum is Treatment of choice – reduced incidence of stricture and
recurrent cholangitis
 Complete resection of cyst is important because of association with
development of cholangiocarcinoma
Gall stones

 Most common Biliary pathology

 Types- cholesterol, pigment or mixed
 Asia 80% are pigment stones.
 Mixed stones contain 51–99% cholesterol & calcium salts, bile acids , bile
pigments & phospholipids .
 Obesity ,high calorie diet & certain medications can increase secretion of
cholesterol & supersaturate bile , increasing the lithogenicity of bile.
 Abnormal emptying of GB may promote aggregation of nucleated cholesterol
chrystals ,hence removal gall stones with out removal of gall bladder leads to
gall stone recurrence

 Pigment stones– contain less than 30% cholesterol
 Black & brown
 Black – composed of insoluble bilirubin pigment polymer mixed with calcium
phosphate & calcium carbonate.20 – 30% are black stones. Incidence increase
with age . Accompany hemolysis , usually hereditary spherocytosis or sickle cell
disease .
 Patients with cirrhosis have a higher incidence of pigmented stones.
 Brown pigment stone contain calcium bilirubinate , calcium palmitate , calcium
stearate & cholesterol .They form in the bile duct & related to bile stasis &
infected bile.
 Also associated with foreign bodies with in bile ducts as endoprosthesis, or
parasites as clonorchis sinensis & ascaris lumbicoides.
Clinical presentation

 Right upper quadrant pain radiating to back.

 Dyspepsia
 Flatulence
 Food intolerance especially fatty food,
 Alteration in bowel frequency.
 Biliary colick- severe right upper quadrant pain that ebbs & flows with
vomiting.- several episodes.
 Murphy’s sign- rt upper quadrant tenderness during deep inspiration
 Mass may be palpable,
Empyema GB

 Wall become necrotic & may perforate

 Localised peritonitis
 Courvesiers law----in an obstructive juandice patient palpable nontender GB
is due to distal CBD obstruction secondary to peri pancreatic malignancy not
due to stone because previous inflammation may have made GB wall
thickened & non distentable.
 Mucocele of GB- complete obstruction of cystic duct with re absorption of
intra luminal bile salts & uninfected mucosa secreted by GB epithelium-
palpable GB
 Double impaction-stones simultaneously obstructing cystic duct & distal CBD.
Management of GB stones

 Asymptomatic- observe
 Prophylactic cholecystectomy-DM pts, congenital haemolytic anemia, who
undergo bariatric surgery for morbid obesity.
 With biliary colic or cholecystitis- cholecystectomy.


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