Abstract
BACKGROUND: Idiopathic portal hypertension (IPH) is a subtype of portal hypertension that arises in the absence of cirrhosis. IPH frequently manifests with clinical features typical of portal hypertension, including splenomegaly and esophagogastric fundal varices, along with other associated symptoms. Imaging studies may indicate portal hypertension; however, they typically do not provide evidence of cirrhosis. There are no standardized diagnostic criteria for IPH, and diagnosis is often established by excluding other hepatic diseases. Liver biopsy remains the most reliable approach to verify the diagnosis of IPH. CASE SUMMARY: A patient previously diagnosed with "hepatitis B cirrhosis" at an external hospital presented to our facility with gastrointestinal bleeding. Initial assessment revealed minor liver injury, splenomegaly, esophagogastric varices, and portal hypertension. Imaging studies did not indicate cirrhosis and repeated hepatitis B serology tests yielded negative results. After excluding various causes of cirrhosis and other non-cirrhotic etiologies of portal hypertension, liver biopsy confirmed the diagnosis of IPH. The patient was managed with regular endoscopic therapy and long-term carvedilol administration. CONCLUSION: Currently, there are no standardized diagnostic criteria for IPH, and its diagnosis is generally established by excluding other conditions. Liver biopsy remains the most reliable method for IPH diagnosis.