Outcome of 21 patients with biliary atresia living more than 10 years

The outcome of 21 survivors (8 males, 13 females; age range, 11 to 29 years) followed for > 10 years after surgery for biliary atresia are discussed. Of the 21 patients, 18 with type III disease had hepatic portoenterostomy (Kasai operation), and 3 with type I disease had hepaticoenterostomy. Twenty patients are alive leading almost normal lives; however, 13 (61.9%) patients did have a history of complications, including hemorrhage from esophageal varices in 10, from a gastric ulcer or erosion in 3, and from a duodenal ulcer in 2; biliary reobstruction in 3; and multiple pulmonary arteriovenous fistulae in 2. Of those with complications, 7 required surgery. One died suddenly at the age of 19 years of a bleeding gastric ulcer. Liver function is normal in 9 (45.0%) of the 20 alive patients, 2 (10.0%) have slight hepatic dysfunction, and 9 (45.0%) have mild-to-moderate hepatic dysfunction. Liver function is almost normal in 3 patients who had a history of variceal hemorrhage.