What Is Biliary Atresia?
Biliary atresia is a rare liver disease which destroys the bile ducts necessary to carry bile from the liver to the intestines. This disease involves the bile ducts outside the liver (extrahepatic) and may progress to severely affect the bile ducts inside the liver (intrahepatic). Bile collects within the liver and other tissues where it acts as a slow poison. Without treatment, cirrhosis and eventual liver failure occur. Biliary means pertaining to the bile, bile ducts, and/or gall bladder. Atresia refers to a congenital absence of or closure of a normal body orifice. Children who have biliary atresia do not have a complete functional biliary system.