[Idiopathic portal hypertension].

Patients with portal hypertension without a demonstrable cause have been reported in the literature under several different terms, such as tropical splenomegaly, phlebosclerosis, obliterative portal venopathy of the liver, hepatoportal sclerosis, noncirrhotic portal fibrosis and idiopathic portal hypertension (IPH). Such patients have been described worldwide, with a greater frequency in India and Japan. The etiology of IPH is still unknown, although some of the theories that have been proposed are: exposure to toxic substances or drugs, relationship with the hepatitis-B virus, immunologic abnormalities, systemic or intra-abdominal infections and clotting abnormalities. The main histopathologic findings are periportal fibrosis, obliteration of small portal veins and sclerosis of the interhepatic portal system. Although these abnormalities could be secondary to portal hypertension, it has been proposed that the vascular changes are the primary event that leads to portal hypertension. The site of increased resistance in IPH is found at the presinusoidal level with some component at the sinusoidal and postsinusoidal level. The main symptoms and signs in IPH are upper gastrointestinal tract bleeding secondary to esophago-gastric varices, symptoms related to anemia, and splenomegaly. The long-term prognosis for patients with IPH is better than for cirrhotic patients, with a 77% survival at ten years. Variceal bleeding is the main cause of death, and some treatment to prevent bleeding or its recurrence is warranted. Although no comparative trial has been performed in IPH patients, the surgical management could be the first choice for elective treatment in these patient without liver failure, because of the high re-bleeding rates with chronic sclerotherapy. Pharmacologic management could be considered for prophylactic treatment of these patients.