A Review and Case Discussion on a Rare Cause of Non-cirrhotic Portal Hypertension

Abstract

Nodular regenerative hyperplasia (NRH) is a rare cause of non-cirrhotic portal hypertension that should be considered in patients with no risk factors for chronic liver disease or in any unusual presentation of variceal hemorrhage. We present a case of an 82-year-old Chinese female, with a history of previous metastatic sigmoid carcinoma with oxaliplatin use, who presented with melena. A gastroscopy done revealed one column of grade 3 esophageal varix, two columns of grade 2 esophageal varices, and a type 1 gastroesophageal varix with stigmata of recent hemorrhage. Cyanoacrylate glue therapy was performed without any complications. A follow-up computed tomography (CT) imaging of the abdomen did not reveal any significant features of cirrhosis or venous thrombosis, and the decision was made for a transjugular liver biopsy with hepatic venous pressure gradient (HVPG) measurement. The measured HVPG was 6 mmHg, and the liver biopsy showed features consistent with NRH.

A Case of Non-cirrhotic Portal Hypertension With Antiphospholipid Syndrome

“…The lesions found in NCPH are vascular and classified by the site of resistance to portal blood flow (prehepatic, hepatic, and posthepatic), and patients typically present with PHT without evidence of parenchymal dysfunction in the liver [9]. Most cases of NCPH are believed to be caused by endothelial cell lesions, intimal thickening, thrombotic obliterations, and scarring of the hepatic venous circulation along with increased blood flow [10].…”