Extrahepatic biliary atresia is the most common indication for liver transplantation in children. Destruction of extrahepatic bile ducts begins in utero or shortly after birth, and may be segmental or complete. The cause is unknown, but it is not thought to be genetic. One in four children have anomalies in other organs.
Patients present early with jaundice, and without treatment develop secondary biliary cirrhosis and die by 1 or 2 years. Most eventually need a liver transplant.