LARGE REGENERATIVE NODULE IN ALAGILLE SYNDROME – A CASE REPORT

Abstract

Alagille Syndrome is a rare complex multisystem genetic disorder with a wide spectrum of clinical findings. Hepatic involvement in Alagille is characterized by paucity of intrahepatic bile ducts, which often presents with cholestasis and can rarely lead to cirrhosis and hepatic failure requiring liver transplantation. Liver nodules in Alagille can range from benign regenerative to dysplastic nodules and rarely hepatocellular carcinoma. The typical imaging findings of a regenerative nodule on cross sectional imaging can avoid unnecessary biopsy. Here, we present the case of a large regenerative hepatic nodule in Alagille syndrome with typical imaging findings who underwent hepatic transplantation at 9 years due to hepatic failure and histopathology was confirmed on explant liver.