Congenital hepatic fibrosis

Abstract

Four cases of hepatic fibrosis and portal hypertension in one family are described. They belong to one sibship-three sisters and one brother. Histological features of the liver supported the diagnosis of congenital hepatic fibrosis. The family was unusual in that the ages of presentation were different. The patients described illustrate' the preservation of hepatic function and the clinical presentation from the effects of portal hypertension suggesting a good result may be anticipated from surgery.