Liver pathology findings in infant with Caroli's syndrome

Abstract

Introduction: Caroli's syndrome (CS) is a rare congenital disorder characterized by intrahepatic bile duct dilatation and congenital hepatic fibrosis. The clinical features of this condition include signs of portal hypertension, cholangitis and lithiasis. Liver transplantation is the ultimate treatment in most patients with liver failure. Case Report: A three month old infant treated with the diagnosis of biliary atresia, after two liver biopsies presented with distended abdomen, hepatosplenomegaly and signs of portal hypertension. Liver transplantation was preformed after four months. We found ectatic hilar bile ducts and intrahepatic bile duct dilatation. The pathologic finding of congenital hepatic fibrosis and proliferated dilated bile ducts suggested the