Extrahepatic Biliary Atresia Associated with Cyanotic Congenital Heart Disease

Abstract

Three patients with extrahepatic biliary atresia associated with cyanotic con genital heart disease are presented and their clinical courses reviewed. In these patients, the coincidence of cyanosis and jaundice caused an atypical green- appearing cutaneous discoloration which masked the early clinical detection of biliary atresia in one infant and cyanotic heart disease in the other two. In all infants, either partial or complete correction of the congenital cardiac lesion was possible. Two infants died from hepatic disease despite attempted surgical revision and one has satisfactory bile drainage one month following portoenterostomy. Awareness of the rare concurrence of cyanotic congenital heart disease and extrahepatic biliary atresia is helpful to the clinician when evaluating infants with atypical cutaneous discoloration. Early oversight of either underlying disease process can be avoided, and early evaluation and management of both entities can be possible.