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Liver Disorders

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Congenital Hepatic Fibrosis (CHF)

A genetic disorder, congenital hepatic fibrosis (CHF) primarily affects children and young adults, but can present at any age. It is attributed to structural abnormalities in the liver; bands of collagen fibers often form around key structures as a result of hepatocellular injury and disrupt function. The etiology remains unknown. Ductal plate malformation, intrahepatic dilation of the bile duct (Caroli's disease), cholangitis and renal disease are associated with CHF. Older patients typically experience portal hypertension.

Clinical symptoms include liver, kidney and spleen enlargement, and gastrointestinal bleeding. Liver biopsy will reveal histiologic evidence of fibrous tissue while ultrasound is generally used to evaluate renal involvement. Treatment may include beta-blockers, sclerotherapy and portosystemic shunting. Mild CHF usually doesn't warrant treatment.