The efficacy of hepatoportoenterostomy in biliary atresia.

This report describes the treatment and outcome of 66 infants with biliary atresia. Mean age was 79.8 +/- 33.2 days. Diagnosis was achieved by 99mTc DISIDA scanning. Hepatoportoenterostomy (HPE) was performed in 48 cases and hepatoportocholecystostomy in four, with microscopic ducts at the porta hepatis. Fourteen infants without microscopic ducts did not undergo HPE. Patients were staged according to the postoperative result. HPE was successful in 25% of patients (group A), resulted in improvement in 19% (group B), failed in 43% (group C), and was short-term in 13% (group D). In patients less than 90 days of age, the HPE success rate was 31%; 23% improved, and 33% showed no improvement. Age (less than 90 days) and bile clearance were prognostic determinants of success. Reoperation was useful only in patients with a previously successful HPE. Ten of 20 patients referred for liver transplantation survived (50%) (7/11) survived after liver transplantation and 3/9 on the waiting list). Fourteen of 15 patients in group A remain anicteric and well without liver transplantation. Patients in group B have had extended survival (greater than 3 years) but eventually required transplantation. Patients in group C and children more than 90 days old at diagnosis require early liver transplantation. HPE is a useful procedure when performed in infants less than 90 days of age who have biliary atresia.