Long-term outcome of surgery for biliary atresia

Between 1953 and 1995, 300 patients with biliary atresia underwent surgery at Tohoku University Hospital. Among them, 31 patients survived more than 20 years, while one of these patients died of hepatic failure at the age of 28 years. Of the 30 surviving patients (10 males and 20 females, age range; 20 to 41 years), 20 underwent hepatic portoenterostomy, 8 underwent hepaticoenterostomy and the remaining 2 underwent hepatic portocholecystostomy. None of these patients has undergone liver transplantation. Twenty-two patients have led near normal lives. This includes three married women, one of whom gave birth to 2 healthy babies. The remaining 8 patients have had experienced some troubles due to cholangitis, portal hypertension and intrahepatic gallstones. Two of them who have progressive liver dysfunction are being considered as candidates for liver transplantation. The quality of life of one patient has been severely affected by an unrelated (Turner's syndrome). While the majority of long-term survivors of biliary atresia have good quality of life, close long-term postoperative follow-up is required.