Rare Immunology News

Disease Profile

Median arcuate ligament syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

MALS; Celiac artery compression syndrome; Celiac axis syndrome;

Summary

Median arcuate ligament syndrome (MALS) is a rare disorder characterized by chronic, recurrent abdominal pain related to compression of the celiac artery (which supplies blood to the upper abdominal organs) by the median arcuate ligament (a muscular fibrous band of the diaphragm). It usually presents with symptoms of abdominal pain, weight loss, and an abdominal bruit (abnormal sound of a blood vessel when blocked or narrowed).[1] The cause is not fully understood; however, it is suspected that there could be a combination of vascular (blood supply) and neurogenic (neurological) components involved. Diagnosis is usually confirmed with imaging such as CT angiography, MRI, ultrasound, and arteriography.[2] Surgery is currently the only treatment option and involves releasing the ligament.[3]

Symptoms

Classically, individuals with median arcuate ligament syndrome (MALS) present with a triad of abdominal pain after eating, weight loss (usually >20 pounds), and abdominal bruit (abnormal sound of a blood vessel when blocked or narrowed).[1] 

One review found that abdominal pain is the most common symptom, found to be present in approximately 80% of individuals, while weight loss was found in approximately 48% and abdominal bruit was appreciated in approximately 35%.[4]

Other symptoms include: nausea, diarrhea, vomiting, and delayed gastric emptying.[1][2]

Cause

The cause of median arcuate ligament syndrome (MALS) is disputed. While it was initially thought to be caused by a restriction of blood supply secondary to compression of the celiac artery (supplies blood to the upper abdominal organs) by the median arcuate ligament (a muscular fibrous band of the diaphragm), other factors have been proposed. It has been suggested that nerve dysfunction might additionally be involved, which could explain some of the associated symptoms such as pain and delayed gastric emptying.[1]

Diagnosis

A diagnosis of median arcuate ligament syndrome (MALS) might be suspected in middle aged (40-60) female patients with a triad of symptoms including abdominal pain after eating, weight loss, and abdominal bruit (abnormal sound of a blood vessel when blocked or narrowed). Abdominal imaging is used to confirm the diagnosis and rule out other similarly presenting disorders. Imaging methodologies might include: CT angiographyMRIultrasound, and arteriography.[1][2]

Treatment

Surgery is currently the only treatment option for median arcuate ligament syndrome (MALS). Surgery typically involves decompression of the celiac artery by dividing the fibers of the median arcuate ligament and celiac plexus (network of nerves in the abdomen). Surgical decompression might additionally be combined with stent placement, angioplasty, or vascular reconstruction of the celiac artery.[1]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Social Networking Websites

    • MALS Pals is a closed Facebook group for Median arcuate ligament syndrome
    • Visit the MALS Awareness group on Facebook.

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

      • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
      • The The Cleveland Clinic Web site has an information page on Median arcuate ligament syndrome. Click on the Cleveland Clinic link to view this page.

        In-Depth Information

        • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Median arcuate ligament syndrome. Click on the link to view a sample search on this topic.

          References

          1. Scovell S and Hamdan A. Celiac artery compression syndrome. UpToDate. June 1 2016; https://www.uptodate.com/contents/celiac-artery-compression-syndrome.
          2. Allison J. Porter, Robert B. Yates, Joshua M. Mourot, Brant K. Oelschlager. A Compressed Celiac Artery May Lead to High-Risk Surgery. Medscape. September 11, 2015; https://www.medscape.com/viewarticle/847153.
          3. Celiac Artery Compression Syndrome. Baylor College of Medicine. https://www.bcm.edu/healthcare/care-centers/vascular-surgery/conditions/celiac-artery-compression-syndrome.
          4. Juan Carlos Jimenez, Michael Harlander-Locke, Erik P. Dutson, MD. Open and laparoscopic treatment of median arcuate ligament syndrome. Journal of Vascular Surgery. September 2012; 56(3):869-873. https://www.ncbi.nlm.nih.gov/pubmed/22743019.

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