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Abstract

Since the sequential treatment of Kasai operation with or without liver transplantation became available, the overall prognosis of biliary atresia remains unclear. This study examined the prognostic factors from diagnosis. All patients with biliary atresia living in France and born in the years 1986 to 1996 were reviewed. Actuarial survival rates were calculated for survival with native liver, survival after liver transplantation, and overall survival. Potential prognostic factors were analyzed using the logrank test and the Cox model. A total of 472 patients were identified. Ten-year overall survival was 68%. Independent prognostic factors for overall survival were (S = 10-year rates) performance of Kasai operation (performed: S = 69%; not performed: S = 50%), age at Kasai operation (≤45 days: S = 80%; >45 days: S = 66%), anatomical pattern of extrahepatic bile ducts, polysplenia syndrome, experience of the center (≤2 new biliary atresia [BA] patients/year [24 centers]: S = 54%; 3 to 5 [2 centers]: S = 60%; ≥20 [1 center]: S = 78%). Survival with native liver depended on the same independent prognostic factors. In conclusion (1) Kasai operation remains the first line treatment of BA, and (2) early performance of Kasai operation and treatment in an experienced center reduces the need for liver transplantation in infancy and childhood and provides children with the best chance of survival.