P. Bertheau et al., ADENOMATOUS HYPERPLASIA OF THE LIVER IN A PATIENT WITH CONGENITAL HEPATIC-FIBROSIS, Journal of hepatology, 20(2), 1994, pp. 213-217
We report the case of a 30-year-old man suffering from congenital hepa
tic fibrosis, who was treated at age 12 by splenorenal shunt for porta
l 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 nod
ules. Histological examination of the surgical specimen showed adenoma
tous hyperplastic nodules with a few atypical foci in an otherwise fib
rous but non-cirrhotic liver. Such lesions have not yet been described
in congenital hepatic fibrosis. The respective roles of congenital he
patic fibrosis and splenorenal shunts in the development of these nodu
les are discussed. (C) Journal of Hepatology.