Biliary atresia

Abstract Biliary atresia is a disease characterized by obliteration of both intra- and extrahepatic bile ducts, and presents as conjugated jaundice and pale stools in the first weeks of life. Its aetiology is poorly understood, and many mechanisms have been invoked to explain its pathology. It is diagnosed by exclusion of other medical, metabolic, and infective causes of conjugated jaundice. Treatment is entirely surgical, being an attempt to restore bile flow from the native liver in the first instance (Kasai portoenterostomy). Liver transplantation is reserved for those in whom this approach has been unsuccessful. Clearance of jaundice is expected in over 60% of infants, with a native liver survival at 5 and 10 years of 43–45%.