Elsevier

Annals of Vascular Surgery

Volume 30, January 2016, Pages 22-27
Annals of Vascular Surgery

VESS Clinical Research
Median Arcuate Ligament Syndrome Is Not a Vascular Disease

https://doi.org/10.1016/j.avsg.2015.07.013Get rights and content

Background

Median arcuate ligament syndrome (MALS) is a rare disorder characterized by postprandial abdominal pain, weight loss, and celiac stenosis. Diagnosis can be challenging, leading to a delay in treatment. We report on our continued experience using a laparoscopic approach for this uncommon diagnosis.

Methods

This is an Institutional Review Board-approved, prospectively collected retrospective analysis of patients treated with laparoscopic MAL release at our institution. Data collected included patient demographics, preoperative symptoms, operative approach, and postoperative outcomes. Patients were then contacted to complete a postoperative survey designed to assess the improvement of symptoms and overall patient satisfaction.

Results

A total of 39 patients (33 women and 6 men) underwent laparoscopic MAL release from March 2007 to July 2014. Mean age was 40.6 years (range, 17–77 years). Thirty of 39 patients had a postoperative celiac axis ultrasound. Twenty-three had a patent celiac axis on postoperative duplex. Of the remaining 7, 5 with residual celiac axis stenosis and 1 with occlusion, reported complete resolution of their symptoms. One remaining patient with occlusion remained symptomatic. Thirty-three of 39 (84.6%) reported symptom relief after surgery. Nine of 33 (27.3%) responders had cardiovascular risk factors versus 4 of 6 (67%) nonresponders. Five patients with atypical presentations underwent preoperative diagnostic celiac plexus block using local anesthetic, with 4 reporting symptom reliefs after block. These 4 patients also reported postoperative symptom relief. One patient of 39 received a postoperative celiac stent placement and remained symptomatic. There were 4 conversions to open surgery (10.3%) and no deaths.

Conclusions

Laparoscopic MAL release continues to be a safe and effective means of managing MALS. Our data suggest that the symptoms associated with MALS are not related to vascular compromise, and atherosclerotic risk factors may predict poorer outcomes. Symptomatic relief is seen in the vast majority of patients undergoing this procedure. However, patient selection remains critically important in obtaining optimal results.

Introduction

Anatomic compression of the celiac artery was first described by Lipshutz in 1917.1 However, the first clinical description of median arcuate ligament syndrome (MALS) or celiac axis compression syndrome was described by Harjola in 19632 and is typified by the triad of epigastric abdominal pain that is worsened with eating, weight loss, and evidence of celiac axis compression by the MAL of the diaphragm. It is a diagnosis of exclusion, as more common diseases of the foregut need to be ruled out before MALS is considered.

During embryologic development, the diaphragm stops its downward descent from the neck to the celiac axis, and forms the MAL. In up to 10%3 of people, there is an anatomic compression of the celiac axis that is intermittent and dependent on the phase of respiration. Controversy has existed surrounding MALS related to the supposition that it is a vascular disease associated with foregut ischemia. The subtotal intermittent occlusion of the celiac axis cannot cause mesenteric ischemia because of the highly collateralized flow between the mesenteric vessels.4 Reports of gastric mucosal oxygen tonometry5 and gastric perfusion3 do not consistently predict who would benefit from MAL release. There are no case reports of gastric necrosis due to MALS. Open revascularization on young otherwise healthy people is not without risk, although no deaths have been reported.6 Endovascular stenting of younger patients is also of questionable durability, especially if there is extrinsic compression. Many of the patients face bias from their practitioners, after extensive gastrointestinal work-up is found to be negative. Psychiatric comorbidity is frequently present in these patients.7 Lacking a proven disease mechanism leads to uncertainty and skepticism for physicians and surgeons.

We hypothesize that MALS is a neurogenic disease, and not related to vascular occlusion. The pathoanatomy is the compression of the celiac plexus by the MAL and the celiac axis, resulting in altered sensation and pain from the somatic nerves in the plexus. These symptoms are much like other nerve compression diseases such as carpal tunnel syndrome and neurogenic thoracic outlet syndrome. To evaluate this hypothesis, we reviewed our single-center experience treating this rare disorder Figure 1.

Section snippets

Methods

A retrospective medical records review of a prospectively maintained database of all patients undergoing minimally invasive treatment for MALS from March 2007 to July 2014 was performed. Patients were then contacted via mail to inform them of the study, and that they would be contacted by phone to participate in a follow-up survey. The Cleveland Clinic Institutional Review Board approved this retrospective review. The patient database was created and maintained in the Digestive Disease

Results

A total of 39 patients were treated with minimally invasive MAL release during the study period. Thirty-five of these procedures were conducted using a pure laparoscopic approach and 4 were converted to an open procedure due to intraoperative hemorrhage. Table I summarizes patient characteristics. Mean age of patients was 41 years (range, 17–77 years) and 84.6% were female. Mean preoperative BMI was 25 kg/m2 (range, 18–38). A total of 35 patients (89.7%) had earlier abdominal operations,

Discussion

In the reports predating laparoscopic release, nearly all patients with MALS underwent concomitant open celiac axis revascularization.3 It was never proved that this was a necessary step, when in accomplishing the revascularization, celiac plexus neurolysis inevitably occurred. In a more recent case report, Sumpio et al.11 proposed an algorithm consisting of laparoscopic MAL release with subsequent celiac axis stenting. This patient had a good outcome, but once again the neurolysis of the

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