The Diagnosis and Treatment of Congenital Hepatic Fibrosis

Three cases of congenital hepatic fibrosis underwent splenectomy and cardiofundotransectomy are reported.The clinical manifestations included hepatosplenomegaly,portal hypertension and upper gastrointestinal hemorrhage.The diagnosis was confirmed by pathologic examination.They were followed-up for 1 to 3 years with normal physical development,hepatic functions,and without GI bleeding.The authors state that patients with hepatosplenomegaly,portal hypertension,multiple dilation of intrahepatic bile ducts but without abnormal liver functions,congenital hepatic fibrosis should be considered.The histologic characteristics include fibrous proliferation of interlobular portal areas,and normal structure of hepatic lobules.Early diagnosis and treatment should be stressed to prevent postoperative complications. Key words: Liver disease; Bile suct; intrahepatic; Hypertension; portal