Noncirrhotic portal hypertension in the adult: case report and review of the literature

Abstract

Noncirrhotic portal hypertension results from thrombosis of the extrahepatic portal vein that subsequently is recanalized. Liver function is preserved. In the adult, esophageal variceal hemorrhage is the most common presentation and may occur years after the portal vein thrombosis. We report the case of a 34-year-old man who presented with recurrent esophageal variceal hemorrhage. After ultrasonographic and angiographic evaluation, a diagnosis of idiopathic noncirrhotic portal hypertension was made. Due to recurrent esophageal variceal bleeding, the patient required surgical intervention to control bleeding. The incidence of noncirrhotic portal hypertension is unknown. Multiple etiologies may cause the disorder, although nearly half are idiopathic. The pathogenesis, clinical manifestations, diagnostic evaluation, natural history, prognosis, and management of noncirrhotic portal hypertension are discussed. Endoscopic management of esophageal variceal bleeding is the preferred therapy. However, when endoscopic treatment fails to control variceal hemorrhage, a distal splenorenal shunt is likely to be the most successful operation.