Long-term Outcome of 88 Patients With Biliary Atresia Treated by the Kasai Procedure

Abstract

Purpose : To assess the long-term outcome of biliary atresia patients treated in our institution by the Kasai procedure from 1967 to 1998. Materials and Methods : A total of 88 patients with biliary atresia were treated by the Kasai procedure from 1967 to 1998 at Niigata University. The study period was divided arbitrarily into three periods, 1967 to 1978 (period A, n=31) ; 1979 to 1988 (period B, n=32) ; 1989 to 1998 (period C, n=25). Results : Jaundice disappeared in 47% (n=41) of all patients, and 28% (n=25) survived. In patients treated during period C, jaundice disappeared in 80% (n=20), and 60% (n=15) survived as a result of refinement of operative procedures and improvement in postoperative medical therapy. The overall 10-year cumulative survival rate was 40% and 15-year cumulative survival rate was 35%, improving to 63% and 50% respectively in patients showing disappearance of jaundice at least once after surgery. Poor prognosis was evident in patients who had undergone repeat-Kasai operation. There was no difference in the incidence of postoperative cholangitis between any period or the types of bile drainage procedure performed. On the other hand, mortality due to cholangitis improved during period C. Of the 25 patients who have survived, 6 patients are problem free, and the rest have postoperative complications. Conclusion : Outcome of biliary atresia treated by the Kasai procedure has improved over the past 10 years. Some patients deteriorated despite initial disappearance of jaundice due to postoperative complications in the long-term. As the length of follow-up increases, clinical assessment should be performed regularly and must include comprehensive analysis.