[Surgical and long-term problems in biliary atresia]

The outcome of biliary atresia (BA) has improved after developments in hepatic portojejunostomy since the 1950s. The key to successful hepatic portojejunostomy is an optimal dissection line of the hepatic hilar remnant and anastomotic technique without microductule injury on the dissected surface. Most recent articles have reported that remnant hilar tissue should be dissected and the liver capsule preserved. The long-term problems in BA patients, such as cholangitis, became apparent after increased long-term native-liver survival. When hepatic function is preserved and the cause of cholangitis is localized, both surgical intervention and liver transplantation are viable options. The most appropriate treatment option is chosen based on efficacy, invasiveness, and ability to restore hepatic function. Digestive varices and hypersplenism due to portal hypertension are often controllable via symptomatic treatment. Long-term survivors of BA need to be closely monitored for secondary pulmonary perfusion abnormality; the condition necessitates liver transplantation and affects the outcome of the procedure. Some female survivors of BA conceive, which increases the risk of cholangitis and gastrointestinal bleeding during and after pregnancy. Such patients therefore must be monitored closely. Understanding the etiology and pathophysiology of BA is necessary for a good clinical course with native-liver survival.