Long-term results and clinical problems after portoenterostomy in patients with biliary atresia.

From January 1962 through December 1986, 71 cases of biliary atresia were operated on. Nine of these patients underwent only an exploratory laparotomy. A hepatic portoenterostomy (Kasai's operation) was performed on 62 infants with biliary atresia. Of these 62 children, 18 survived more than 10 years. However, 4 of the 18 died shortly thereafter because of persistent or recurrent jaundice and hepatic failure. Fourteen patients, however, are still alive more than ten years after portoenterostomy, today the actual 10-year survival rate is thus 22.5%. Six of them have no severe complications and their serum total bilirubin is less than 1.0 mg/dl. However, 8 patients, whose serum total bilirubin is more than 1.0 mg/dl, demonstrated numerous clinical problems such as esophageal varices, hypersplenism and recurrent jaundice after cholangitis. One of the patients in the latter group eventually underwent a liver transplantation because of the recurrent jaundice and hepatic insufficiency. In the treatment of biliary atresia, a close and careful follow-up is therefore essential especially for patients whose serum total bilirubin is more than 1.0 mg/dl.