Analysis of patients with biliary atresia coming to liver transplantation.

Seventy-two patients with end-stage liver disease underwent liver transplantation between March 1981 and March 1984; 35 (49%) with biliary atresia, the remainder with other disorders. This provided us with a unique opportunity to analyze factors leading to liver failure in patients who had undergone biliary drainage procedures for "uncorrectable" biliary atresia. Four patients in the biliary atresia group were excluded (no corrective procedure done, 3; "correctable" biliary atresia, 1), leaving 31 patients for study. Transplantation survival was 84% for the study group and 73% in children with other primary liver disorders. Most patients were less than 3 months old at the time of initial surgery, had minimal liver disease, and had accepted corrective operations by experienced surgeons. Despite these "favorable" factors, bile drainage was rarely achieved. All patients with continued bile drainage at the time of transplantation had repeated episodes of cholangitis, and cholangitis was associated with cessation of bile drainage in half of those with transient function. Findings at hepatectomy suggested that in four cases where bile drainage was never achieved, reexploration may have been successful. Complications included those associated with hepatic failure and portal hypertension. Of note were a high incidence of bone disease and a 43% incidence of stomal hemorrhage in patients with stomas. The short-term survival after transplantation was comparable in the biliary atresia group and the children with other disorders. This suggests that while the presence of a previous biliary drainage procedure may increase the technical difficulty of transplantation, it does not decrease survival.