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Biliary Atresia, Extrahepatic
Condition Overview: Extrahepatic biliary atresia is a rare gastrointestinal disorder characterized by destruction or absence of all or a portion of the bile duct that lies outside the liver (extrahepatic bile duct). The bile duct is a tube that allows the passage of bile from the liver into the gall bladder and, eventually, the small intestine. Bile is a liquid secreted by the liver that plays an essential role in carrying waste products from the liver and breaking down fats in the small intestine. In extrahepatic biliary atresia, absence or destruction of the bile ducts results in the abnormal accumulation of bile in the liver. Affected infants may have yellowing of the skin and whites of the eyes (jaundice) and scarring of the liver (cirrhosis). In some cases, additional abnormalities may be present, including heart defects and kidney and spleen malformations. The exact cause of extrahepatic biliary atresia is unknown.. |
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