Biliary atresia: the King's College Hospital experience (1974-1995)

Abstract

The survival experience of 338 infants born with biliary atresia between January 1973 and December 1995 was analyzed. All the infants had their initial surgery at a single UK centre. These infants were divided into three groups based on year of birth; group 1 (1970s, n = 38); group 2 (1980s, n = 182), and group 3 (1990s, n = 118). The data from group 1 were incomplete and selected, and comparisons with the remaining groups were therefore restricted. However, all infants who had been treated since 1980 underwent portoenterostomy or hepaticojejunostomy and were included.

Results: In the whole cohort there were 89 deaths (26%), 79 children (23%) who underwent liver transplantation and 170 children (50%) who were alive at last follow-up. The 5- and 10-year actuarial survival for group 2 was 50% and 41%, respectively and the 5-year actuarial survival for group 3 was 60%. Overall, 57 children have survived to 10 years after surgery for biliary atresia. There has been a progressive fall in the age at surgery from a median of 77 days in group 1, through 69 days in group 2 to 56 days in group 3 (P < .0001). However, there was no significant difference in outcome to 5 years between the age cohorts (< 40 days, 41 to 60 days, 61 to 99 days, and > or = 100 days; P > .1) for the infants treated since 1980 (n = 200).

Conclusions: Portoenterostomy is an effective long-term procedure for biliary atresia in about 40% to 50% of infants. The remaining 50% to 60% will require transplantation mostly within 2 years of age, although there is also a continuing need beyond 5 and 10 years. The age at surgery has limited usefulness as a predictor of survival after portoenterostomy and certainly should not be used to dictate primary treatment.