Idiopathic portal hypertension: a case report

Idiopathic portal hypertension (IPH) is diagnosed in patients with clinical symptoms of portal hypertension, patent portal vein, and the absence of morphological signs of liver cirrhosis, or when no other reason can be found for liver disease. IPH refers to a number of vaguely defined histological entities including idiopathic portal hypertension, incomplete septal cirrhosis, focal regenerative hyperplasia, and partial nodular transformation. These entities most likely belong to the spectrum of one disease, originating from the impairment of intrahepatic circulation.

A young male patient was admitted to hospital due to splenomegaly, thrombocytopenia and leucopenia. One year earlier the patient had undergone transjugular intrahepatic portocaval shunt (TIPS) due to symptoms of portal hypertension, but without effect. Liver biopsy disclosed increased fibrosis of portal areas with the penetration of a few thin streaks of connective tissue into liver parenchyma, and isolation of single nodules just under the liver capsule. There were also discrete vascular changes. Liver cirrhosis was ruled out. The patient underwent splenectomy. The spleen manifested only signs of passive hyperemia. In the follow-up 6 months after surgery there was a marked improvement of the patient's general status and normal thrombocyte level.

Splenomegaly may be the main symptom of IPH without liver cirrhosis. Histopathological assessment of liver biopsy can exclude liver cirrhosis, which has major consequences for treatment. In such patients splenectomy is usually sufficient, without the need for liver transplantation.