A Rare Case of Idiopathic Noncirrhotic Portal Hypertension in a Young Patient.

The most common cause of portal hypertension (PH) is liver cirrhosis. When symptoms of PH develop in noncirrhotic patients secondary to hepatic or systemic disorders, it is termed as noncirrhotic portal hypertension (NCPH) while idiopathic noncirrhotic portal hypertension (INCPH) is the term for PH present without any identifiable underlying cause. INCPH is a diagnosis of exclusion when all other causes of liver cirrhosis have been ruled out. A liver biopsy is required to diagnose INCPH. Presuming that PH is secondary to cirrhosis when no liver biopsy is performed is not always true. The incidence and prevalence of INCPH is increasing, especially in developed countries. We present a rare case of INCPH and portal vein thrombosis in a young 23-year-old male with no significant past medical history and no underlying hepatic or systemic disease.