Median Arcuate Ligament Syndrome Clinical Presentation, Pathophysiology, and Management: Description of Four Cases

Ihsan Al Bayati,Brian R Davis,Richard W Mccallum,Jesus R Diaz,Mahesh Gajendran

doi:10.3390/gidisord3010005

Abstract

Median arcuate ligament syndrome (MALS), otherwise called celiac artery compression syndrome (CACS), is an uncommon disorder that results from an anatomical compression of the celiac axis and/or celiac ganglion by the MAL. Patients typically present with abdominal pain of unknown etiology exacerbated by eating along with nausea, vomiting, and weight loss. MALS is a diagnosis of exclusion that should be considered in patients with severe upper abdominal pain, which does not correlate with the objective findings. The cardinal feature which is elicited in the diagnosis of MALS relies on imaging studies of the celiac artery, demonstrating narrowing during expiration. The definitive treatment is the median arcuate ligament’s surgical release to achieve surgical decompression of the celiac plexus by division of the MAL. This article describes our experience with this entity, focusing on symptom presentation, diagnostic challenges, and management, including long-term follow-up in four cases.

Highlights

Median arcuate ligament syndrome (MALS), otherwise called celiac artery compression syndrome (CACS), is an uncommon disorder first described in 1965 by Dunbar et al [1].The median arcuate ligament is a muscular arch that connects the diaphragmatic crura to form the anterior margin of the aortic hiatus
MALS is a diagnosis of exclusion that should be considered in patients with severe upper abdominal pain that is out of proportion and does not correlate with the objective findings
Four patients with MALS were identified from the experience of a gastroenterologist (RMC) at an academic referral center directing a center for GI motility and functional bowel disorders from January 2017–June 2020

Summary

Introduction

Median arcuate ligament syndrome (MALS), otherwise called celiac artery compression syndrome (CACS), is an uncommon disorder first described in 1965 by Dunbar et al [1]. The median arcuate ligament is a muscular arch that connects the diaphragmatic crura to form the anterior margin of the aortic hiatus. In patients with MALS, it causes compression of the celiac artery on expiration and entrapment of the celiac plexus (a dense network of ganglia that lies over the anterolateral aorta at the T12 vertebral level) [2]. The pathophysiology of MALS is related to both ischemic and neuropathic mechanisms due to the anatomic relationship of the MAL to the origin of the arterial celiac plexus and ganglion [3] (Figure 1).

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