[Biliary tract atresia. Hepatoportoenteric anastomosis and/or liver transplantation]

Abstract

Long-term survival of biliary atresia patients after Kasai hepatoportoenterostomy is being increasingly reported. Favorable prognostic factors are: 1) Young age at operation (max. 60 days). 2) Few incidents of postoperative cholangitis. Jaundice-free long-term survival is achieved in one-fourth to one-third of patients undergoing Kasai hepatoportoenterostomy. Without treatment the average life span is 19 months. Twenty infants with biliary atresia were treated with the Kasai operation between 1976 and 1992. Nine children are alive 11 months to 16 years postoperatively, two after a liver replacement, one at ten months and one at two and a half years old. A tenth child had a well functioning anastomosis but died of heart failure. Eight children with a well functioning anastomosis were all younger than 60 days at the operation. Only one child older than 60 days survived without a liver transplantation. Our results confirm that early diagnosis and treatment of biliary atresia, before 60 days of age, is necessary.