Congenital Hepatic Fibrosis

Congenital hepatic fibrosis & is an autosomal recessive disease, in most cases associated with renal abnormalities typical of autosomal recessive polycistic kidney disease &. The hepatic abnormalities of congenital hepatic fibrosis are seen in a variety of disorders. The precise pathogenesis of the disease is unknown, but is thought to be associated with hepatic fibrosis and/or portal vein abnormalities (duplication of the intrahepatic branches). A ductal plate malformation of interlobular bile ducts has been suggested as the pathogenetic mechanism &. Different clinical types of congenital hepatic fibrosis are recognized. Symptoms may appear early or late &. The liver is usually enlarged and quite firm, with a prominent left lobe. The spleen is also palpable, and occasionally the kidneys. The histopathologic picture is not uniform. In some patients, congenital hepatic fibrosis is characterized by fibrous enlargement of the portal tracts, with abnormally shaped bile ducts. In others, the liver shows bands of connective tissue linking the adjacent portal tracts. The cause of the involution of biliary and renal tubules remains unknown. A defect in the tubular basement membrane has been demonstrated &.
In patients with chronic cholangitis and progressive hepatic dysfunction, liver transplantation is indicated.