Portoenterostomy for biliary atresia: Long-term survival and prognosis after esophageal variceal bleeding

Background/purpose

Portoenterostomy is the procedure of choice in patients with biliary atresia. The authors analyzed the long-term survival rate and complications of the procedure in children operated on at The University of Hong Kong Medical Centre between 1979 and 2000.

Methods

Seventy-seven consecutive patients were analyzed by retrospective chart review. The patients were divided into groups according to postoperative bile flow, decade of operation, and age at operation. The data were analyzed statistically.

Results

The overall actuarial transplant-free survival rate was 68% at 10 years after operation. For patients with poor, partial, and good postoperative bile flow, transplant-free survival rate was 0%, 22%, and 96%, respectively (P < .001). Age and decade of operation were not significant risk factors. Esophageal variceal bleeding occurred in 13 patients and was a prognostic indicator for end-stage liver failure (P = .044); the poor prognosis of patients with variceal bleeding, however, was related to poor or partial initial bile drainage.

Conclusions

The postoperative serum bilirubin level is the most important predictor of long-term survival in patients with portoenterostomy for biliary atresia. Esophageal hemorrhage is not an absolute indication for urgent liver transplantation in patients with good bile drainage.