Biliary Atresia

Abstract

A newborn with greater than 2 weeks of jaundice or a primarily conjugated hyperbilirubinemia should be evaluated thoroughly for a cause. Typical symptoms of biliary atresia are elevated conjugated bilirubin, jaundice, acholic stools, and possibly hepatomegaly. There is no definitive test to diagnose biliary atresia; the workup consists of ruling out other diseases and, typically, a HIDA scan and right upper quadrant ultrasound. An intraoperative cholangiogram can be used to demonstrate the biliary anatomy and rule out biliary atresia. A liver biopsy is also performed. The operation of choice for biliary atresia is the Roux-en-Y hepatic portoenterostomy, Kasai procedure. Outcomes include successful long-term biliary drainage (33%), temporary drainage but will be older and more able to withstand a liver transplant (33%), and no relief with progression to liver failure requiring transplantation (33%).