The frequency and outcome of biliary atresia in the UK and Ireland

Background Biliary atresia is an obliterative cholangiopathy of infancy tha t is fatal if untreated, Surgical treatment, the Kasai portoenterostomy, ma y restore bile flow and clear jaundice, and, if successful, achieve a 10-ye ar survival of 90% with a native liver. The outcome of a 2-year cohort of c hildren with biliary atresia in the UK and Ireland was assessed to find the current frequency, the factors influencing outcome, and the medium-term ne ed for liver transplantation. Methods Cases diagnosed between March, 1993, and February, 1995, were notif ied by paediatricians to the British Paediatric Surveillance Unit via a mon thly reporting system. Confirmed cases were followed up by postal questionn aires to notifying paediatricians. Findings 93 cases were confirmed, a frequency of 1/16 700 livebirths, Prima ry surgery was done in 91 children in 15 surgical centres with an early suc cess rate for clearing jaundice of 55% overall. Centres were grouped accord ing to caseload; group A had more than 5 cases/year and group B fewer than 5 cases/year. Early success was higher in group-A centres, odds ratio 2.02 (95% CI 0.86-4.73), but this did not reach statistical significance. Of 41 children in whom surgery was unsuccessful in clearing jaundice 9 (22%) died and 30 (73%) underwent liver transplantation. Survival without liver trans plantation and overall survival were both significantly greater in group-A centres, rate ratios 0.48 (95% CI 0.27-0.86) and 0.32 (0.11-0.94). Actuaria l 5-year survival without transplantation was 61.3% in group-A centres and 13.7% in group-B centres. Actuarial 5-year overall survival was 91.2% in gr oup A and 75% in group B, Once centre size was taken into account, no other factor, including age at surgery, was predictive of survival without trans plantation or overall survival. Interpretation The outcome of children with biliary atresia is related to t he caseload of the surgical centre where they have their primary surgery. C hildren with biliary atresia should be managed in surgical centres with a c aseload of more than five cases annually.