Etiologies of Splenic Venous Hypertension: A Review

Abstract

Splenic venous hypertension or left-sided portal hypertension is a rare condition caused by an obstruction of the splenic vein. Usually, it presents with upper gastrointestinal bleeding in the absence of liver disease. Etiologies can be classified based on the mechanism of development of splenic vein hypertension: compression, stenosis, inflammation, thrombosis, and surgically decreased splenic venous flow. Diagnosis is established by various imaging modalities and should be suspected in patients with gastric varices in the absence of esophageal varices, splenomegaly, or cirrhosis. The management and prognosis vary depending on the underlying etiology but generally involve reducing splenic venous pressure. The aim of this review was to summarize the etiologies of splenic venous hypertension according to the mechanism of development.

Keywords: Gastric varices; Hematemesis; Left-sided portal hypertension; Sinistral portal hypertension; Splenic vein thrombosis; Splenic venous hypertension; Upper gastrointestinal bleeding.

Fig. 1. Mechanisms of development of splenic vein hypertension.

(A) Compression. Pancreatic cysts compress the splenic vein, increasing venous flow in the gastric veins and forming varices. Other causes of compression include pancreatic NET, lymphoma, SAA, Bochdalek hernia, enlarged lymph nodes, and RF. (B) Stenosis. Idiopathic splenic vein stenosis increases the venous blood flow in the gastric veins, forming varices. (C) Inflammation. Pancreatitis can lead to fibrosis with subsequent occlusion of the splenic vein, leading to increased flow to the gastric veins and the formation of varices. (D) Thrombosis. ET and PMF can lead to splenic vein thrombosis, increasing blood flow in the gastric veins with varix formation. (E) Surgically decreased splenic venous flow. PV-SMV resection performed in the setting of a pancreatic tumor can lead to increased congestion of the splenic vein with subsequent formation of gastric varices. ET, essential thrombocythemia; NET, neuroendocrine tumor; PMF, primary myelofibrosis; PV-SMV, portal vein-superior mesenteric vein; PV, portal vein; SAA, splenic artery aneurysm; SMV, superior mesenteric vein; SV, splenic vein; SVH, splenic venous hypertension.

Fig. 2. An algorithm for evaluation and management of SVH.

APC, activated protein C; CTA, computed tomography angiography; EGD, esophagogastroduodenoscopy; GI, gastrointestinal; IR, interventional radiology; MRI, magnetic resonance imaging; SVH, splenic venous hypertension; SVT, splenic vein thrombosis; US, ultrasound; w, with; wo, without.