Long-term survival following operations for extrahepatic biliary atresia is not well documented in the literature. This report constitutes a 37-year history of a patient with extrahepatic biliary atresia upon whom William E. Ladd, MD, performed a choledochoduodenotomy when the patient was 9 weeks old. This patient experienced episodes of fever and jaundice over a 12-year period for which an operation—removal of calculus, excision of stenosed segment of hepatic duct, and reconstitution of the hepaticoduodenostomy—was performed at the age of 21 years. Fever and jaundice recurred 11 years later. A third operation, a revision of the hepaticoduodenostomy, was then done. A liver biopsy taken at that time revealed early biliary cirrhosis. Results of liver function tests taken at the age of 35 years were essentially normal. At present he works regularly, has a general diet, and lives a normal life.
Lou MA, Schmutzer KJ, Regan JF. Congenital Extrahepatic Biliary Atresia. Arch Surg. 1972;105(5):771-773. doi:10.1001/archsurg.1972.04180110088022