Adenomatous hyperplasia of the liver in a patient with congenital hepatic fibrosis.

We report the case of a 30-year-old man suffering from congenital hepatic fibrosis, who was treated at age 12 by splenorenal shunt for portal hypertension, and who developed numerous, large hepatic nodules. The patient underwent liver transplantation because of recurrent episodes of acute cholangitis and the presence of foci of dysplasia in the nodules. Histological examination of the surgical specimen showed adenomatous hyperplastic nodules with a few atypical foci in an otherwise fibrous but non-cirrhotic liver. Such lesions have not yet been described in congenital hepatic fibrosis. The respective roles of congenital hepatic fibrosis and splenorenal shunts in the development of these nodules are discussed.