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World J Hepatol. Jul 27, 2021; 13(7): 774-780
Published online Jul 27, 2021. doi: 10.4254/wjh.v13.i7.774
Torsion of spleen and portal hypertension: Pathophysiology and clinical implications
Ashish Kumar Jha, Sameer Bhagwat, Vishwa Mohan Dayal, Arya Suchismita
Ashish Kumar Jha, Sameer Bhagwat, Vishwa Mohan Dayal, Department of Gastroenterology, Indira Gandhi Institute of Medical Sciences, Patna 800014, India
Arya Suchismita, Department of Paediatric Hepatology, Institute of Liver and Biliary Sciences, Delhi 110070, India
Author contributions: Jha AK and Bhagwat S were involved in designing and writing the manuscript; Dayal VM and Suchismita A assisted in review of literature; all authors read and approved the final manuscript.
Conflict-of-interest statement: There is no conflict of interest associated with any of the senior author or other coauthors contributed their efforts in this manuscript.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Ashish Kumar Jha, MD, DM, Associate Professor, Department of Gastroenterology, Indira Gandhi Institute of Medical Sciences, Bailey Road, Patna 800014, India. ashishjhabn@yahoo.co.in
Received: February 7, 2021
Peer-review started: February 7, 2021
First decision: May 13, 2021
Revised: May 28, 2021
Accepted: July 2, 2021
Article in press: July 2, 2021
Published online: July 27, 2021
Processing time: 165 Days and 13.5 Hours
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: Wandering spleen; Splenic torsion; Left-sided portal hypertension; Gastric variceal bleeding; Splenectomy

Core Tip: Wandering spleen (WS) is a rare disease. The repeated torsion of WS is due to the presence of long pedicle and absence/laxity of anchoring ligaments.WS is an extremely rare cause of left-sided portal hypertension and severe gastric variceal bleeding. This review comprehensively describes the pathophysiological mechanisms, clinico-demographic profile, and clinical implications of torsion of the spleen. In patients with splenic torsion, varices can be detected incidentally on preoperative imaging studies or intraoperatively. Therefore, pre-operative search for varices is required in patients with splenic torsion.