Biliary atresia is characterized by the absence of the large bile duct outside the liver. Bile and waste products normally excreted in the bile accumulate in the liver and cause progressive injury. The cause of the disorder is not known, but it does not appear to be a genetic or inherited disease. Affected patients usually appear healthy at birth, but persistent jaundice is soon apparent. For patients who do not respond to surgery, liver transplantation offers the only hope of long-term survival.
Biliary atresia is the most common indication for liver transplant in children.
Choledochal cyst This disorder is characterized by a cystic dilation of the bile duct either outside or inside the liver. When the cyst does not empty at all, the patient presents with jaundice and symptoms like those of biliary atresia. Occasionally, the cyst drains intermittently and jaundice and pain occur sporadically. This disorder is treatable by surgery if addressed early, before scarring and infection in the liver are present. Paucity of the intrahepatic bile ducts Paucity of bile ducts is characterized by a decreased number of small bile channels inside the liver.