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Review |

Median Arcuate Ligament Syndrome—Review of This Rare Disease

Erinn N. Kim, MD1,2; Kathleen Lamb, MD3; Daniel Relles, MD3; Neil Moudgill, MD4; Paul J. DiMuzio, MD3; Joshua A. Eisenberg, MD5
[+] Author Affiliations
1Medical student, Jefferson Medical College, Philadelphia, Pennsylvania
2now with Department of Surgery, University of California, San Francisco (UCSF), Fresno
3Division of Vascular and Endovascular Surgery, Department of Surgery, Thomas Jefferson University, Philadelphia, Pennsylvania
4Division of Vascular Surgery, University of South Florida, Tampa
5Division of Vascular and Endovascular Surgery, Advanced Surgical Associates of New Jersey, Pennington
JAMA Surg. 2016;151(5):471-477. doi:10.1001/jamasurg.2016.0002.
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Published online

Importance  Median arcuate ligament (MAL) syndrome is a rare disease resulting from compression of the celiac axis by fibrous attachments of the diaphragmatic crura, the median arcuate ligament. Diagnostic workup and therapeutic intervention can be challenging.

Objective  To review the literature to define an algorithm for accurate diagnosis and successful treatment for patients with MAL syndrome.

Evidence Review  A search of PubMed (1995-September 28, 2015) was conducted, using the key terms median arcuate ligament syndrome and celiac artery compression syndrome.

Findings  Typically a diagnosis of exclusion, MAL syndrome involves a vague constellation of symptoms including epigastric pain, postprandial pain, nausea, vomiting, and weight loss. Extrinsic compression of the vasculature and surrounding neural ganglion has been implicated as the cause of these symptoms. Multiple imaging techniques can be used to demonstrate celiac artery compression by the MAL including mesenteric duplex ultrasonography, computed tomography angiography, magnetic resonance angiography, gastric tonometry, and mesenteric arteriography. Surgical intervention involves open, laparoscopic, or robotic ligament release; celiac ganglionectomy; and celiac artery revascularization. There remains a limited role for angioplasty because this intervention does not address the underlying extrinsic compression resulting in symptoms, although angioplasty with stenting may be used in recalcitrant cases.

Conclusions and Relevance  Median arcuate ligament syndrome is rare, and as a diagnosis of exclusion, diagnosis and treatment paradigms can be unclear. Based on previously published studies, symptom relief can be achieved with a variety of interventions including celiac ganglionectomy as well as open, laparoscopic, or robotic intervention.

Figures in this Article

Figures

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Figure 1.
Median Arcuate Ligament in Relation to the Celiac Artery

Note the low-riding median arcuate ligament compared with normal anatomy.

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Figure 2.
Algorithm for Diagnosis and Management of Median Arcuate Ligament (MAL) Syndrome

CT indicates computed tomography; HIDA, hepatobiliary iminodiacetic acid; MR, magnetic resonance; and RUQ, right upper quadrant.

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Figure 3.
Diagnostic Techniques for Median Arcuate Ligament (MAL) Syndrome

A, Ultrasonography of the celiac axis revealing elevated peak systolic velocity (PSV), end diastolic velocity (EDV), resistive index (RI), and more than a 200% change in pulse volume amplitude with inspiration. B, Computed tomographic angiography (CTA) of the abdomen demonstrating compression of the celiac axis (arrow) with expiration due to obstruction by MAL. C, Magnetic resonance angiography demonstrating stenosis of the celiac axis with poststenotic dilatation, consistent with MAL syndrome. Arrow indicates area of celiac axis occlusion with expiration and an area of poststenotic dilatation of the celiac artery that occurs with proximal obstruction. D, Aortogram with compression of the celiac axis by the MAL with poststenotic dilatation.

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Figure 4.
Mesenteric Arteriograms

A, Mesenteric arteriogram during expiration; arrow indicates obstruction of celiac axis with expiration. B, Mesenteric arteriogram during inspiration; arrow indicates patency of celiac axis with inspiration.

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