Idiopathic portal hypertension

A 60-yr old Italian woman presented with repeated gastrointestinal hemorrhages due to ruptured esophageal varices. No evidence of liver disease could be demonstrated by laboratory tests or by multiple liver biopsies. Angiography revealed a patent portal trunk and the presence of esophageal varices. A side-to-side portacaval shunt was performed, which caused the disappearance of the esophageal varices. There was no recurrent digestive hemorrhage during a 24-month follow-up. A mild deterioration of liver function was demonstrated by laboratory data 16 months after surgical operation and some mild episodes of hepatic encephalopathy occurred. This case can be considered an example of idiopathic portal hypertension, a rare pathological condition with a higher prevalence in certain geographical areas. Its etiology is not known. One important aspect of this entity is its potential to evaluate the effects of surgical portacaval shunt procedure in the absence of liver damage.