Fibrose hepatique congenitale a propos de 2 cas

ABSTRACT

Congenital hepatic fibrosis is a hepatorenal affection that often expresses itself by portal hypertension, sometimes by angiocholitis and rarely by both associated. Renal lesion is most often latent, but can express itself by renal hypertrophy or characteristic tubular ectasy and rarely by cysts. We report here two cases of congenital hepatic fibrosis with portal hypertension seen in child. Diagnosis is made in the first case on urographic aspects of precalicial tubular ectasy and in the second case on the association of portal hypertension, renal hypertrophy and fibrosis pressing intrahepatic biliary ducts. We remember here the nosologic problem and clinical, evolutive and prognostic aspects of the affection