Bile lakes in congenital biliary atresia.

During an 18 year period intrahepatic bile "lakes" were identified at postmortem examination in the hepatic parenchyma of six infants with intrahepatic biliary atresia and four infants with type C extrahepatic atresia. The bile lakes were located in the central portion of the liver and were not identified in any patients under 6 months of age. The lakes did not communicate with identifiable intrahepatic ducts in any of the six patients with intrahepatic atresia. Each of the four patients with type C extrahepatic atresia had a few ductal communications with the peripheral hepatic parenchyma. Bile lakes most likely represent dilated ductal remnants that contain inspissated secretions and cellular debris. They do not characterize potentially curable patients if the condition is associated with intrahepatic atresia. Bile lakes occur late in the course of the disease, beyond the age of 3 months, when irreversible hepatic damage occurs. Nonetheless all infants with biliary atresia should have a hepatic portoenterostomy or biliary-enteric anastomosis regarding the long-term prognosis after such operations.