Conventional treatment of biliary atresia: long-term results.

From 1968 to 1983, 271 patients were treated for biliary atresia by a group of surgeons from the same pediatric surgical unit, in Paris, using procedures adapted to the local anatomy and all derived from the Kasai technique. Eighty children have survived more than 10 years since the surgery, without the need for liver transplantation during the 10-year period. However, three children died subsequently from complications of the liver disease. Thirteen others later underwent liver transplantation, which accounted for three additional deaths. Thus, of the 64 patients left for study, 38 had a good result with respect to serum bilirubin level, but 18 of them still have symptoms of portal hypertension. Among another group of 14 patients with serum bilirubin levels between 18 and 36 mumol/L, 11 are leading a near-normal life. The mean follow-up period for this study is 14 years; the oldest patient is aged 24 years. One patient, already the mother of a normal son, is awaiting her second baby; she was treated by portocholecystostomy at 2 months of age. As a rule, liver transplantation should not be considered an alternative to the Kasai operation as initial treatment of biliary atresia. It may be the only form of treatment for survivors without jaundice, if survival becomes compromised by complications owing to portal hypertension or pulmonary shunts.