Aim: To assess the results of portoenteroanastomosis (PEA) and liver transplantation (OLT) in extrahepatic biliary atresia (EHBA).
Methods: Out of a series of 148 EHBA, 92 cases primarily treated by us were selected. Survival with the native liver (end point = death or OLT) and its relationship with the age at PEA, type of EHBA, ductal size and bile flow restablishment were assessed. Patient survival was compared in those patients who had access to OLT when needed (Group I, n = 69) and those in whom only PEA was available (Group II, n = 23). (OLT program started in january 1986).
Results: At the end of follow-up, 32 children are alive with their native livers, 22 died and 38 had OLT. 40/85 patients who underwent PEA had complete restablishment of bile flow (47%). The no failure rate (survival of the native liver) at 1, 5, 10 and 20 years, was 91%, 49%, 38% and 21%, respectively. Bile flow restablishment was the only predictor significantly associated with good prognosis (survival of native liver at 5, 10 and 20 years of 89%, 86% and 51%, respectively). Differences in survival were significant (p < 0.001) between patients in groups I and II at 1 year (92% vs 74%), 5 years (78% vs 35%), 10 years (76% vs 30%) and 20 years (76% vs 30%).
Conclusions: Bile flow restablishment after PEA can be obtained in experienced centers in about 50% of cases of EHBA. The combined and sequential use of PEA and OLT allows excellent long-term survival in EHBA.