Biliary atresia results in a progressive inflammatory process which may lead to cirrhosis of the liver. The infant looks normal at birth but develops jaundice after the age of 2 to 3 weeks with yellowing of the eyes and skin, light-colored stools and dark urine caused by the build up of the pigment called bilirubin in the blood. The abdomen may be swollen with a firm, enlarged liver. Weight loss and irritability develop as the jaundice increases.
The preferred treatment is to attach the small intestine directly to the liver at the spot where bile is found or expected to drain. Bile flow is re-established in approximately 80% of infants who are operated before 3 months of age. Of these, about 50% will have some bile drainage and as many as 30% will have complete bile drainage. About 20% of infants will not be helped by the procedure. In these cases, the only option is a liver transplant.