Idiopathic portal hypertension

Abstract

Idiopathic portal hypertension (IPH) is a disease presenting as non-cirrhotic portal hypertension due to a presinusoidal portal blood flow block and increased portal blood flow with an enlarged spleen, although the etiologic factors have not been identified. IPH is most common in middle-aged females and is predominant in females versus males in the overall population in Japan. The major signs and symptoms are pancytopenia with splenomegaly and gastroesophageal varices. The clinical manifestations are relatively mild and insidious in most of the patients, except in the case of variceal hemorrhage. A high incidence of portal vein thrombosis is also characteristic. Diagnosis is made by the differentiating IPH from the diseases of portal hypertension with identified causes because there are no specific clinical findings for the diagnosis of IPH. Portal and hepatic venography or liver histology is helpful for the diagnosis. The treatment of portal hypertension-related problems is the same as for problems related to liver cirrhosis. The clinical course and overall survival are well maintained in patients for whom gastroesophageal varices are effectively controlled. However, complications such as refractory ascites or intestinal ectopic varices may appear in the late stages of the disease.