[Hepatoportal sclerosis: apropos of a familial case]

Hepatoportal sclerosis is uncommon in European countries and its diagnosis is difficult. The etiology remains unknown; its familial origin is probably exceptional.

An 18-month-old girl born to a mother with hepatoportal sclerosis had hepatomegaly. She also had a moderate splenomegaly and mild increase transaminase and gamma GT activities. Ultrasound examination failed to show portal hypertension. Histological study of liver showed changes quite similar to those seen in her mother. Two members of the mother's family had portal hypertension.

Familial forms of portal hypertension have been reported: a familial form of incomplete septal cirrhosis and a familial occurrence of cavernous transformation of the portal vein. Obstruction of the portal vessels was not found in our patient, the youngest in whom hepatoportal sclerosis is documented.