Congenital hepatic fibrosis: report of two new cases and review of the literature.

Abstract

Two cases of congenital hepatic fibrosis (CHF) are presented and 75 other cases previously reported in the literature are reviewed. The condition is usually noted in the preschool age. Hematemesis or enlarged liver are the presenting symptoms in 75 percent. Portal hypertension occurs in 50 to 70 percent of the patients. In 55 percent the condition is sporadic and in this group renal affection is found in 33 percent. These renal changes are of the “tubular ectasia” type. The remaining 45 percent have affected siblings and here renal involvement occurs in 70 percent. In this group the renal changes resemble the “hereditary cystic disease of the kidney.” Death has occurred in 50 percent of the patients, mainly from uremia or massive variceal hemorrhage. The survivors have no or only minor symptoms, The patients with portal hypertension due to CHF are ideal candidates for portosystemic shunt. The possibility of prophylactic shunt in these patients when they are over 15 years of age is discussed.