Biliary atresia and the Kasai operation: continuing care.

Surgical intervention utilizing the Kasai hepatic portoenterostomy has improved the outcome of patients with biliary atresia and provided a population of patients with unique health problems. The clinical course of 21 children followed for three years or longer was reviewed, focusing on their medical management. Ten (47.6%) had successful bile drainage following surgery and experienced a number of specific problems including recurrent cholangitis, nutritional and growth deficiencies, delayed developmental landmarks, portal hypertension, osteomalacia and osteoporosis, and social and psychiatric difficulties. These complications responded to aggressive medical therapy and support. Although the overall three-year survival of this series was 38.1%, in children who were operated upon prior to 2 months of age and in whom the enteric conduit was externalized the three-year survival rate was 66.7%.