Prognosis of uncorrected biliary atresia: an update

Abstract

In order to provide a baseline for comparison with the results of the Kasai hepatic portoenterostomy, follow-up information was obtained for 89 infants who underwent surgical exploration alone for biliary atresia from 1956 through 1975. Of these patients, one is apparently cured, one is alive with liver failure, three are lost to follow-up and 84 are dead. The rate of apparent cure among patients with follow-up was 1.1%. The average age at death was 12 mo, the mean 10 mo. During this same period, nine patients were operated upon for neonatal biliary obstructions other than biliary atresia, and five are apparently cured. Reported apparent cure rates after the Kasai procedure or one of its variations, while not good, still compare favorably with the cure rate of unoperated patients.