Torsion of spleen and portal hypertension: Pathophysiology and clinical implications

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 WSrelated 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.