In three patients, two men aged 57 and 53 years, and a 43-year-old woman, idiopathic portal hypertension, also called non-cirrhotic portal hypertension (NCPH), was diagnosed. The first two patients presented with haematemesis. They were treated by endoscopic rubber band ligation of oesophageal varices. In the second patient, placement of a transjugular intrahepatic portosystemic shunt (TIPS) was necessary due to failure of the ligation treatment. The third patient was treated for HIV infection and had a gastroscopy because of nausea and vomiting, which revealed oesophageal varicosis. None of the patients had liver function impairment. Two of the patients had been treated with medication known to be associated with NCPH (azathioprine for Crohn's disease (second patient) and didanosine for HIV infection (third patient)). These medications were discontinued. The histological features of the patients were heterogeneous (nodular regenerative hyperplasia, periportal fibrosis and periportal dilated structures), but consistent with NCPH. Portal hypertension in the Western world is mostly associated with liver cirrhosis. When portal hypertension occurs in association with patent portal and hepatic veins, and in the absence of liver cirrhosis, NCPH must be considered. The prognosis of this disease is much better than that of cirrhosis.
Nederlands Tijdschrift voor Geneeskunde
Erasmus MC: University Medical Center Rotterdam

Schouten, J. N. L., Verheij, J., & Janssen, H. (2010). Non-cirrhotic portal hypertension: rare cause of upper gastrointestinal bleeding. Nederlands Tijdschrift voor Geneeskunde, 154(13). Retrieved from