Median arcuate ligament syndrome Richard Goodall, BSc, MBChB, FHEA, a,b Benjamin Langridge, MA, MBBS, FHEA, a,b Sarah Onida, BSc, MBBS, MRCS, PhD, a,b Mary Ellis, BSc, a,b Tristan Lane, BSc, MBBS, PhD, FRCS, a,b and Alun Huw Davies, MA, DM, DSc, FRCS, FHEA, FEBVS, FACPh, a,b London, United Kingdom ABSTRACT Background: Median arcuate ligament syndrome (MALS) describes the clinical presentation associated with direct compression of the celiac artery by the median arcuate ligament. The poorly understood pathophysiologic mechanism, variable symptom severity, and unpredictable response to treatment make MALS a controversial diagnosis. Methods: This review summarizes the literature pertaining to the pathophysiologic mechanism, presentation, diagnosis, and management of MALS. A suggested diagnostic workup and treatment algorithm are presented. Results: Individuals with MALS present with signs and symptoms of foregut ischemia, including exercise-induced or postprandial epigastric pain, nausea, vomiting, and weight loss. Consideration of MALS in patients’ diagnostic workup is typically delayed. Currently, no group consensus agreement as to the diagnostic criteria for MALS exists; duplex ultra- sound, angiography, and gastric exercise tonometry are used in different combinations and with varying diagnostic values throughout the literature. Surgical management involves decompression of the median arcuate ligament’s constriction of the celiac artery; robotic, laparoscopic, endoscopic retroperitoneal, and open surgical intervention can provide effective symptom relief, but long-term follow-up data (>5 years) are lacking. Patients treated nonoperatively appear to have worse outcomes. Conclusions: MALS is an important clinical entity with significant impact on affected individuals. Presenting symptoms, patient demographics, and radiologic signs are generally consistent, as is the short-to medium-term (<5 years) response to surgical intervention. Future prospective studies should directly compare long-term symptomatic and quality of life outcomes after nonoperative management with outcomes after open, laparoscopic, endoscopic retroperitoneal, and robotic celiac artery decompression to enable the development of evidence-based guidelines for the management of MALS. (J Vasc Surg 2020;71:2170-6.) Keywords: Median arcuate ligament syndrome; Celiac artery compression; Mesenteric ischemia Median arcuate ligament syndrome (MALS) describes a constellation of clinical signs and symptoms associated with celiac artery compression by the median arcuate ligament (MAL). The true prevalence of MALS is unknown. However, individuals with this phenomenon present with symptoms of foregut ischemia including postpran- dial or exercise-induced abdominal pain, nausea, and vomiting, with subsequent food aversion and weight loss. In the early 1960s, Harjola 1 and Dunbar et al 2 demon- strated some relief of symptoms after surgical decom- pression of the celiac artery in a case report and case series, respectively. However, MALS has since been a topic of contention. Controversy about its existence as a syndrome arises from a poorly understood pathophysio- logic mechanism, variable presentations (and severity thereof), and unpredictable response to treatment. Accepted universal diagnostic criteria are lacking. Indeed, diagnosis remains one of relative exclusion and is typically the result of extensive (often invasive and radiation-intense) investigations to exclude more com- mon, alternative causes of abdominal pain. Despite this, the European Society for Vascular Surgery (ESVS) clinical practice guidelines for the management of diseases of mesenteric arteries and veins 3 recognizes MALS as the most common cause of single-vessel arterial stenosis, with the potential for significant morbidity. This review aimed to outline the anatomy and patho- physiology of MALS. Evidence pertaining to its clinical presentation, diagnosis, and management is reported. A practical algorithm for the management of suspected MALS is presented. ANATOMY The MAL is a tough fibrous arch connecting the right and left crura of the diaphragm at the level of the aortic hiatus (T12-L1). It traverses anterior to the aorta and is usu- ally cranial to the celiac artery. The position of the MAL and the origin of the celiac artery vary between individ- uals. A relatively cranial origin of the celiac artery or From the Academic Section of Vascular Surgery, Department of Surgery and Cancer, Imperial College London a ; and the Department of Vascular Surgery, Imperial College Healthcare NHS Trust. b Infrastructure support for this work was supported by the NIHR Imperial Biomedical Research Centre Author conflict of interest: none. Correspondence: Richard Goodall, BSc, MBChB, FHEA, Department of Vascular Surgery, Charing Cross Hospital, 4th Fl, East Wing, Fulham Palace Rd, London W6 8RF, United Kingdom (e-mail: [email protected]). The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 0741-5214 Copyright Ó 2019 by the Society for Vascular Surgery. Published by Elsevier Inc. https://doi.org/10.1016/j.jvs.2019.11.012 2170
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