Biliary atresia: an evolving perspective

From 1967 to 1984, 50 of our patients with extrahepatic biliary atresia had surgical exploration. Of 40 biliary drainage procedures, bile drained in 21 (52%). Thirty-four patients had portoenterostomy, three had portocholecystostomy, and the most recent six patients had a valved hepatoduodenal conduit. Successful biliary drainage was related to the presence of microscopic ducts at the porta hepatis in 20 of 21 infants. Twenty patients are alive, 12 from two to six years postoperatively (one with a liver transplant). Seven have normal serum bilirubin values. Height and weight exceed the 50th percentile in 5/15 patients studied. Multiple episodes of cholangitis have occurred in 11 patients with portoenterostomy and two with hepatoduodenal conduits. In 12 patients, hemorrhage from the stoma necessitated closure of the stoma before 1 year of age. Five of the six patients with hepatoduodenal conduit are alive two years postoperatively.