Torsion of spleen and portal hypertension: Pathophysiology and clinical implications

Abstract

The displacement of spleen from its normal location to other places is known as wandering spleen (WS) and is a rare disease. The repeated torsion of WS is due to the presence of long pedicle and absence/laxity of anchoring ligaments. A WS is an extremely rare cause of left-sided portal hypertension (PHT) and severe gastric variceal bleeding. Left-sided PHT usually occurs as a result of splenic vein occlusion caused by splenic torsion, extrinsic compression of the splenic pedicle by enlarged spleen, and splenic vein thrombosis. There is a paucity of data on WS-related PHT, and these data are mostly in the form of case reports. In this review, we have analyzed the data of 20 reported cases of WS-related PHT. The mechanisms of pathogenesis, clinico-demographic profile, and clinical implications are described in this article. The majority of patients were diagnosed in the second to third decade of life (mean age: 26 years), with a strong female preponderance (M:F = 1:9). Eleven of the 20 WS patients with left-sided PHT presented with abdominal pain and mass. In 6 of the 11 patients, varices were detected incidentally on preoperative imaging studies or discovered intraoperatively. Therefore, pre-operative search for varices is required in patients with splenic torsion.

Keywords: Gastric variceal bleeding; Left-sided portal hypertension; Splenectomy; Splenic torsion; Wandering spleen.

Figure 1

Schematic diagram of the mechanisms of varices formation in wandering spleen with splenic torsion. SV: Splenic vein; GV: Gastric varices; CV: Collateral vein; GEV: Gastroepiploeic vein; SMV: Superior mesenteric vein; PV: Portal vein. Please note that thick arrow denotes more frequent mechanism and thin arrow denotes less frequent mechanism.