Idiopathic portal hypertension presenting with hepatic nodular regenerative hyperplasia after splenectomy: a case...

Idiopathic portal hypertension (IPH) is a rare disease, and its etiology and pathogenesis have not yet been fully clarified. The main clinical manifestations are non-cirrhotic intrahepatic portal hypertension, accompanied by splenomegaly, thrombocytopenia, and recurrent upper gastrointestinal bleeding. The liver histopathologic changes are diverse. Splenectomy is considered an effective treatment for hypersplenism. We report a patient who presented with splenomegaly, then underwent splenectomy to relieve thrombocytopenia based on routine treatment strategies. However, multiple space-occupying lesions were found in the liver about one year later. Thereafter, the lesions were confirmed as nodular regenerative hyperplasia (NRH) by liver biopsy, the patient was finally diagnosed with IPH. We consider that although splenectomy is generally recommended for IPH, under certain circumstances splenectomy may disturb blood flow in the liver, leading to the formation of NRH. Therefore, splenectomy in IPH patients should be chosen carefully.