THE OUTCOME OF SURGERY FOR BILIARY ATRESIA AND THE CURRENT STATUS OF LONG-TERM SURVIVORS

Between 1953 and 1995, 300 patients with biliary atresia underwent sur gery at Tohoku University Hospital. The 10-year survival of patients w ho were operated on in or before 1965 was 9%. But the survival rate we nt up ta 61% in patients operated on between 1976 and 1985. Eighty-fiv e patients including 2 who developed liver failure after Kasai operati on and underwent liver transplantation have survived more than 10 year s. Eleven of them (13%) have recurrent or persistent jaundice. Of the 30 patients who have survived more than 20 years (10 males and 20 fema les, age range; 20 to 41 years), 20 underwent hepatic portoenterostomy , 8 underwent hepaticoenterostomy and the remaining 2 underwent hepati c portocholecystostomy. None of these patients has undergone liver tra nsplantation Twenty-two patients have led near-normal lives. The remai ning 8 patients have experienced some troubles due to cholangitis, por tal hypertension intrahepatic gallstones and so on. TWO Of them are co nsidered as candidates for liver transplantation. While the majority o f long-term survivors of biliary atresia have good quality of life, cl ose long-term follow-up is essential even in patients with biliary atr esia aged 20 years or more.