Long-term results in the treatment of extrahepatic bile duct atresia

Abstract

This article is intended as a contribution towards the question of long-term results to be expected when using hepato-portodigestive anastomoses in children. The paper is based on analyses of the course of the disease in 683 children, as placed at the author's disposal by paediatricians and paediatric surgeons in Europe, the United States of America, and Japan, as well as on an evaluation of 56 child patients observed by the author himself. This assessment resulted in the formulation of five conditions determining the prognosis. One can expect a 5-year survival rate of 66 per cent with good clinical, laboratory, sonographic and scintiscan data as well as histological findings, provided surgery is possible before 6 weeks after onset of cholestasis (this usually means surgery before the 8th week of life), provided also that cirrhosis has not set in at the time of surgery and that there are no additional numerical and morphological changes of the interlobular bile ducts; furthermore, if the average total diameter of the bile duct structures in the hepatic porta is at least 450 mu, and if no cholangitides occur postoperatively. If these conditions are not fulfilled, prognosis must be considered as poor. Such patients become candidates for a liver transplant, which has a 4-year survival rate of 58 per cent since introduction of the cyclosporin A-low-dose-steroid treatment. The article also discusses questions of organisation and of ethics connected with liver transplantation.