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.

Live donor liver transplantation for fulminant hepatic failure in children.

The mortality rate among children with fulminant hepatic failure (FHF) on the waiting list for cadaveric donor liver transplantation (CDLT) is high. Results of emergency CDLT in this situation often are unsatisfactory, and a long-term survival rate less than 30% has been reported. Live donor liver transplantation (LDLT) for FHF in children has been advocated, but is reported rarely. We present our experience with LDLT in children with FHF. Between September 1993 and December 2002, primary LDLT was performed for 26 children; 8 of these children had FHF. Patient demographics, clinical and laboratory data, surgical details, complications, and graft and patient survival are reviewed. Four boys and four girls received left-lateral segment (n = 7) and full left-lobe (n = 1) grafts. Mean age was 2.9 +/- 1.2 years (range, 3 months to 11 years). Causes of FHF were drug induced in 2 patients and idiopathic in 6 patients. One child received a blood group-incompatible graft. Two patients died; 1 patient of cytomegalovirus infection at 8.6 months and 1 patient of recurrent hepatitis of unknown cause at 2.8 months after LDLT. The child who received a mismatched graft had refractory rejection and underwent a second LDLT with a blood group-compatible graft 19 days afterward. He eventually died of lymphoproliferative disease. Another patient developed graft failure related to venous outflow obstruction and survived after retransplantation with a cadaveric graft. With a median follow-up of 13.2 months (range, 2.8 to 60.3 months), actuarial graft and patient survival rates were 50% and 62.5%, respectively. Survival results appear inferior compared with those of 18 children who underwent LDLT for elective conditions during the same study period (graft survival, 89%; P =.051; patient survival, 89%; P =.281). Although survival outcomes are inferior to those in elective situations, LDLT is a timely and lifesaving procedure for children with FHF.

Cholangitis after hepatic portoenterostomy for biliary atresia: a multivariate analysis of risk factors.

Cholangitis occurred in 59% of 77 patients who underwent the portoenterostomy procedure for biliary atresia between 1980 and 2000. Good postoperative bile drainage was associated with a lower risk of cholangitis than partial (odds ratio, 5.72; 95% CI, 2.89-11.3) or poor (odds ratio, 3.29; 95% CI, 1.89-5.7) bile drainage. Cholangitis was not an independent risk factor for death or liver transplantation.