Idiopathic Non-Cirrhotic Portal Hypertension: A case report

Abstract Idiopathic non-cirrhotic portal hypertension is a rare diagnosis with an unknown aetiology. It is characterised by increased intrahepatic portal pressures in the absence of underlying liver disease. We present a unique case of a 32-year-old male patient who presented with a right hydrocele and an incidental finding of pancytopenia, massive splenomegaly, and features of portal hypertension. After known causes of portal hypertension were excluded, a liver biopsy was performed. Based on the liver biopsy findings, which demonstrated fibrotic portal tracts in the absence of cirrhosis and exclusion of other known causes of portal hypertension, our patient was diagnosed with idiopathic non-cirrhotic portal hypertension. Due to the persistent presence of hypersplenism and recurrent episodes of oesophageal variceal bleeding, our patient underwent a splenectomy, after which his features of portal hypertension resolved.