[A rare etiology of portal hypertension in an adult: congenital hepatic fibrosis. Review of current literature in connection with an observation (author's transl)].

On the basis of a personal observation the authors review the current literature on the hypertensive forms of congenital hepatic fibrosis found in an adult subject during G.I. haemorrhage through the rupture of gastro-oesophageal varices. These observations seem to be exceptional and 17 cases were recorded between 1956 and 1976. A complete hemodynamic portal study was carried out only in six cases. It shows a presinusoïdal portal hypertension in three cases, post-sinusoïdal or sinusoïdal portal hypertension in two cases, mixed hypertension in one case. Our observation is outstanding because of the existence of a rise of the wedged hepatic venous pressure varying from one hepatic vein to the other. The prognosis seems to be relatively bad for the short-term as the mortality rate of 2 years reaches at least 29%.