All issues > Volume 35(4); 1992

Case Report

J Korean Pediatr Soc. 1992;35(4):569-574. Published online April 15, 1992.

Two Cases of Congenital Hepatic Fibrosis

Cue Jung CJ Hwang¹, Young Hun YH Kim¹, Dae Kyun DK Koh¹, Byung Churl BC Lee¹

¹Department of Pediatrics, Catholic University Medical College, Seoul, Korea

Abstract

The congenital hepatic fibrosis is a rare condition defined pathologically by the presence within the line of bands of fibrous tissue which after contain linear or circular spaces lined by bile duct cells, It is commonly associated with intrahepatic portal hypertension but hepatocellular function is almost always preserved. The prinicipal clinical features of this disease are abdominal distension, firm hepatomegaly, splenomegaly, hypersplenism and hematemesis or melena due to alimentary bleeding secondary to portal hypertension. We experienced two cases of congenital hepatic fibrosis confirmed by liver biopsy and present with the brief review of related literature.

Keywords :Congenital hepatic fibrosis