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

TL;DR

A 23-year-old man with no known health issues was diagnosed with a rare liver condition called idiopathic noncirrhotic portal hypertension.

Contribution

This case highlights the importance of liver biopsy in diagnosing rare forms of portal hypertension in young patients.

Findings

A young male with no medical history was diagnosed with idiopathic noncirrhotic portal hypertension.

Portal vein thrombosis was also identified in the patient.

The case emphasizes the need to rule out cirrhosis before diagnosing portal hypertension.

Abstract

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.

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