The Improved Outlook for Biliary Atresia with Corticosteroid Therapy

Controversy exists regarding the efficacy of corticosteroids on bile flow after Kasai portoenterostomy in biliary atresia (BA). Fourteen patients who had BA and underwent Kasai portojejunostomy between November 1990 and March 1996 were subject of this study. Corticosteroid therapy ("blast" type) was used for inadequate bile drainage. Corticosteroid support was unnecessary in one patient with good bile drainage, and corticosteroids were aggressively used in the remaining 13 patients. Two patients who had no response to an initial blast subsequently responded and now are doing well. The remaining 11 patients responded to corticosteroids with varying degrees. Three had a limited response, and two ultimately underwent liver transplantation. There was one death caused by subdural hematoma. Three had an excellent initial response. However, one subsequently deteriorated because of intractable cholangitis, requiring liver transplantation. Ten survivors with native liver are anicteric with satisfactory growth and quality of life. Aggressive corticosteroid therapy is an important part of the management after Kasai portoenterostomy. The initial response to steroids does not necessarily reflect the final outcome.