Our recent experiences in transferring patients with biliary atresia (BA) to an overseas transplantation center which had required our own clinical judgment with regard to the indications for orthotopic liver transplantation (OLT) prompted us to revise our paper previously written on this same issue. Sixty-four patients who had undergone the Kasai procedure for BA at the Kobe Children's Hospital during the period from 1971 through 1987 were subjected to this study. These 64 patients were classified into 3 groups according to their degree of bile drainage. Group I (n = 31) : bile fully excreted and their serum bilirubin was below 1.0 mg/dl. Group II (n = 21) : bile inadequately excreted, with serum bilirubin over 1.0 mg/dl. Group III (n = 12) : no bile flow achieved. Group I : 3 pts died due to rupture of esophageal varices despite the normal bilirubin level. Liver transplantation was indicated for these pts. Group II : 17 pts died due to progressive cholestatic liver disease. Group III : all died of rapidly progressive liver disease. All these pts required OLT, eventually in Group II and urgently in group III. Unfavorable factors for successful liver transplantation, such as portal vein thrombosis, intrapulmonary shunt or malnutrition should be carefully considered, when trying to decide the timing of OLT regardless ol the serum bilirubin level.
