“Mysterious” left-sided portal hypertension with splenic artery aneurysm
CLINICAL CASE
Abstract
Left-sided (non-cirrhotic) portal hypertension (LSPH), also known as segmental, regional, localized, compartmental, linear, splenoportal or left-sided hypertension, is a rare but life-threatening cause of bleeding from the upper gastrointestinal tract. It usually results from isolated splenic vein obstruction. The incidence of LSPH has increased over the past three decades due to increased awareness of the disease and advances in diagnostic approaches. Since most patients are asymptomatic and have no complications, the exact incidence is unknown. However, it represents less than 5% of all patients with portal hypertension. The prevalence of LSPH varies depending on the geographic region and the population studied. In studies conducted in Asia, LSPH is more common than in Western countries. A recent study confirmed previous presumed knowledge that the most common pathologies leading to splenic vein thrombosis or obstruction and leading to LSPH are chronic pancreatitis, pancreatic pseudocysts, and pancreatic neoplasms. In particular, the presence of a pseudocyst in patients with chronic pancreatitis may be associated with a significantly higher incidence of splenic vein thrombosis. They account for about 18% of LSPH cases and include benign neoplasms, adenocarcinoma, and functioning and nonfunctioning neuroendocrine tumors. The first description of LSPH dates back to 1939. Since then, only retrospective clinical series have been published. Diagnostic criteria for noncirrhotic portal fibrosis include the presence of splenomegaly, normal or mildly altered liver function tests, esophageal and gastric varices, passable portal and hepatic veins, normal or mildly elevated hepatic vein occlusion pressure, and absence of cirrhosis on liver biopsy. Ultrasound Doppler study reveals the presence of dilated portal vein, splenomegaly and the presence of splenorenal shunts and portocaval anastomoses of other localizations. Increased pressure in the portal vein can be measured by direct catheterization of the portal vein using percutaneous hepatic access. Indirectly, it can be assessed by puncturing the spleen and measuring the pressure in the splenic pulp. Liver biopsy confirms the absence of cirrhosis, while revealing a histologic pattern characteristic of idiopathic portal hypertension.
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