Congenital Hepatic Fibrosis (3 Cases Report)

Abstract

Congenital hepatic fibrosis is a relatively rare disease of children and young adults, that is characterized by hard hepatomegaly, portal hypertension with relative preservation of liver function and underlying architecture, and frequent renal involvement. We report 3 cases of congenital hepatic fibrosis with quite different presentations.The first, aged 2 2/12, presented with hepatomegaly followed by splenomegaly with suspicious renal involvement. The second, aged 2 8/12, had unique feature that congential hepatic fibrosis was associated with situs inversus, nephronophthisis and positive family history. The third, aged 9 9/12, had splenomegaly without hepatomegaly. All cases had splenomegaly.but there were no varices on esophagogram or history of hematemesis or melena.