Recent experience with a modified Sawaguchi procedure for biliary atresia

Abstract

The treatment of biliary atresia by variations of the original Kasai hepatoportoenterostomy has shown early success with good bile flow and the elimination of jaundice in 50% to 70% of cases in many series. Long-term follow-up in many of these patients shows continued problems with ascending cholangitis and progressive liver disease leading to death. Our recent experience with a modified Sawaguchi hepatoportoenterostomy is encouraging. Twelve patients were operated on before two months of age. All but one became jaundice free within 2 to 4 months and had biliary intestinal continuity reestablished within 3 to 6 months. These 11 patients have remained jaundice free with normal growth and development 1 to 8 years postoperatively. Two patients had one and two episodes of cholangitis, respectively. All have continued mild elevations of hepatocellular enzymes but no patient has obvious signs of liver failure. Serial liver biopsies have shown clearing of bile stasis and continued periportal fibrosis. Size and number of ductules in the excised biliary remnant did not correlate with clinical outcome. One patient remained jaundiced after hepatoportoenterostomy and reoperation, and eventually expired. In contrast, two patients operated at 4 and 9 months of age never drained bile and eventually died of bleeding varices and hepatic failure, respectively. The atypical success and relative lack of cholangitis in this series is not readily explained, but may be related to specific technical modifications of the original Sawaguchi procedure.