ORTHOTOPIC LIVER-TRANSPLANTATION FOR CONGENITAL BILIARY ATRESIA - AN 11-YEAR, SINGLE-CENTER EXPERIENCE

Objective The authors analyze a single center's 11-year experience wit h 190 orthotopic liver transplants for congenital biliary atresia. Sum mary Background Data Hepatic portoenterostomy generally is the initial treatment for children with congenital biliary atresia. Despite multi ple modifications of the hepatic portoenterostomy, two thirds of treat ed patients still develop recurrent cholestasis portal hypertension, c holangitis, and cirrhosis. Therefore, the only hope of long-term survi val in the majority of children with congenital biliary atresia is def initive correction with orthotopic liver transplantation. Methods The medical records of 190 consecutive patients undergoing orthotopic live r transplantation for congenital biliary atresia from July 1, 1984 to February 29, 1996 were reviewed. Results were analyzed via Cox multiva riate regression analysis to determine the statistical strength of ind ependent associations between pretransplant covariates and patient aci d graft survival. Actuarial patient and graft survival was determined at 1, 2, and 5 years. The type and incidence oi post-transplant compli cations were determined, as was the quality of long-term graft functio n. The median follow-up period was 3.21 years. Results The liver graft s were comprised of 155 whole-organ, 24 reduced-size, and 11 living-do nor organs. Median pretransplant values for recipient age, weight, and total bilirubin were 1.4 years, 12.3 kg, and 13.8 mg/dL, respectively . One hundred sixty-four patients (85%) were United Network for Organ Sharing (UNOS) status for 1 or 2 at the time of liver transplantation. One hundred fifty-nine patients (84%) received a single graft, wherea s 31 patients required 37 retransplants. The 1, 2 and 5 year actuarial patient survival rates were 83%, 80%, and 78% respectively, whereas g raft survival rates were 81%, 77%, and 76%, respectively. Cox multivar iate regression analysis demonstrated that pretransplant total bilirub in, UNOS status, and graft type significantly predicted patient surviv al, whereas recipient age, weight, and previous hepatic portoenterosto my did not. Current median follow-up values of bilirubin and aspartate aminotransferase levels in the 154 surviving patients were 0.5 mg/dL and 34 international units/L, respectively. Conclusion Long-term patie nt survival after orthotopic liver transplantation for congenital bili ary atresia is excellent and is independent of recipient age, weight, or previous hepatic portoenterostomy. Optimal results are obtained in this patient population when liver transplantation is performed before marked hyperbilirubinemia, and when possible, using a living-donor gr aft.