Biliary atresia: an overview

Biliary Atresia is the most common cause of persistently direct (conjugated) hyperbilirubinemia in the first three months of life. It is a progressive inflammatory obliteration of the extra- and intrahepatic bile ducts whose pathogenesis remains speculative. This review updates the history, etio-pathological considerations, clinical manifestations, methods of diagnosis, surgical management, and results of this terrible disease in young infants. We will emphasize that persistent jaundice in the newborn period must be managed urgently, a diagnosis should be established early in life, and Kasai portoenterostomy offered to those infants with Biliary Atresia before their eighth week of life. This will allow more than one-third of children to survive. Hepatic transplantation is reserved for those children with failed portoenterostomy, progressive liver failure or late-referral to surgery.