• Characterized by progressive obliteration of the extrahepatic and intrahepatic bile ducts. • Etiology is unknown; incidence 1 in 12,000 live births. • Presently, there is no medical therapy to reverse the obliterative process • Patients who are not offered surgical treatment uniformly develop biliary cirrhosis, portal hypertension, and death by 2 years of age. • MC indication for pediatric liver transplantation Pathology • Bile duct proliferation, severe cholestasis with plugging, and inflammatory cell infiltrate are the pathologic hallmarks of this disease. • Over time, these changes progress to fibrosis with end-stage cirrhosis. • Positive for neural cell adhesion molecule (CD56) staining Variants of biliary atresia • Patency to the level of CBD • Patency to the level of common hepatic duct • Left and right hepatic duct at porta involved, solid porta hepatis (90%) Clinical Features • Infants with biliary atresia present with jaundice at birth or shortly thereafter. • Infants with biliary atresia characteristically have acholic, pale gray stools, secondary to obstructed bile flow. • With passage of time, progressive failure to thrive and, if untreated, develop stigmata of liver failure and portal hypertension (splenomegaly and esophageal varices) • Associated malformations in 25%: Polysplenia, malrotation, preduodenal portal vein, and intrahepatic vena cava. Diagnosis • USG of the liver and GB is important in the evaluation of the infant with cholestasis. • USG: GB is shrunken and CBD is not visible. A triangle cord sign found on ultrasound has a predictive accuracy of 95%, the gallbladder ghost triad in which the gallbladder is short (<1.9 cm) and irregular and lacks an echogenic inner lining also got good sensitivity. • Next diagnostic step: Percutaneous liver biopsy if the hepatic synthetic function is normal (diagnostic accuracy 90%). • Hepatobiliary scintigraphy: In cases in which the USG and biopsy findings are inconclusive (absent excretion into the intestine) Treatment • Exploratory laparotomy: If the needle biopsy or abdominal ultrasound is consistent with BA • Intra-operative cholecystocholangiography: To confirm the diagnosis, demonstration of the fibrotic biliary remnant and definition of absent proximal and distal bile duct patency • Treatment of choice: Kasai hepatoportoenterostomy (Roux-en-Y hepaticojejunostomy) Postoperative Management • Ursodeoxycholic acid (facilitate bile flow) + Methylprednisolone (anti-inflammatory agent) + TMP-SMX (antimicrobial prophylaxis) • Cholangitis is the MC post-operative complication. Prognosis • About 30% of infants undergoing hepatoportoenterostomy before 60 days of age will have a long-term successful outcome and not require liver transplantation. • Liver transplantation in the patients who develop progressive hepatic fibrosis with resultant portal hypertension and progressive cholestasis. • Serum bilirubin at 3 months after surgery seems to be strongly predictive of long-term survival.