chapter  Chapter 27
22 Pages

Biliary Atresia

WithDavid H. Perlmutter

Extrahepatic biliary atresia is a disorder of infancy in which there is obliteration of the hepatic or common bile ducts resulting in irreversible end-stage obstructive liver disease. / Hepatic portoenterostomy (“Kasai procedure”) has permitted long-term survival in approximately 20% of patients and transient improvement in many other affected infants. Liver transplantation is required for treatment of approximately 80% of the affected children. Recent studies have suggested that reovirus infection is involved in some of the cases and abnormalities in morphogenesis are involved in 15–25% of patients who also have anomalies of visceral laterality.