Biliary atresia--lessons from the Dublin experience.

A retrospective study analysing the outcome of 26 infants with extrahepatic biliary atresia (EHBA) is reported. Following surgery (mean age 54 days) 13 patients (50%) achieved 'total' biliary drainage, five (19%) 'partial' drainage and eight (31%) were 'failures'. Patients who developed hepatic decompensation (1985 onwards) were evaluated for liver transplantation (LTX). Eight patients (31%) underwent LTX six of whom were > two years (mean age 3.5 yrs). Overall 12 patients are alive (46%)--seven (27%) have had the Kasai operation, one (4%) hepaticojejunostomy. The other four patients are alive following LTX (survival 50%). Seven infants (27%) were referred beyond the optimal age for portoenterostomy, four (15%) of these infants are now dead. The importance of early referral of infants with EHBA is highlighted in this study. Furthermore portoenterostomy and LTX were shown to be complimentary rather than competitive options for these infants. The proposal that LTX is the primary treatment for EHBA has not been supported by this study.