Biliary Atresia

• Complete obstruction of bile flow due to destruction or absence of all or a portion of extrahepatic bile ducts
• Jaundice usually presents with cholestasis or cholangitis at 3-6 weeks of age in an otherwise healthy infant
• 10-15% have other congenital malformations
• Diagnose by excluding other etiologies of neonatal cholestasis (Differential for Cong Hyperbili)
• ultrasound to r/o choledochal cyst as cause of obstruction.
• Liver biopsy to differentiate intrahepatic cholestasis from biliaryobstruction
• May need intraop cholangiography to make dx
• Treat with Kasai procedure (hepatoportoenterostomy operation) to establish biliary drainage using a Roux en-Y loop of intestine as a conduit
• Ideally performed before 2 months of age, which results in resolution of the jaundice in 30% to 50% of patients. In most infants who have biliary atresia, the liver disease progresses, necessitating liver transplantation.