Cookies help us deliver our services. By using our services, you agree to our use of cookies. Learn more

Median Arcuate Ligament Syndrome

What is Median Arcuate Ligament Syndrome?

The median arcuate ligament syndrome (MALS) is a cause of chronic abdominal pain affecting both children and adults alike. Chronic abdominal pain is a very common condition that can have significant negative, long-term psychosocial consequences, including increased risk for anxiety, school and work absences, poor functional capacity, and a poor quality of life. While the exact cause of the pain is unknown, compression of the celiac artery and/or the celiac plexus nerves by the diaphragm can result in pain that is worsened with eating or sometimes with exercise. Other symptoms include nausea and weight loss. In some patients the symptoms can be devastating and can lead to erroneous diagnoses of an eating disorder, psychiatric conditions, or functional abdominal pain (e.g. irritable bowel syndrome, abdominal migraine). The diagnosis is made based on a combination of the clinical symptoms and radiology imaging. There is a surgical procedure that can be performed that is effective in approximately 60-80% of patients.

 

Synonyms

  • Celiac Artery Compression Syndrome
  • Median Arcuate Ligament Syndrome
  • Dunbar Syndrome
  • Harjola-Marable Syndrome
  • Celiac Axis Syndrome
  • Marable Syndrome
  • Celiac Trunk Compression Syndrome

The median arcuate ligament syndrome (MALS) is a cause of chronic abdominal pain affecting both children and adults alike. Chronic abdominal pain is a very common condition that can have significant negative, long-term psychosocial consequences, including increased risk for anxiety, school and work absences, poor functional capacity, and a poor quality of life. While the exact cause of the pain is unknown, compression of the celiac artery and/or the celiac plexus nerves by the diaphragm can result in pain that is worsened with eating or sometimes with exercise. Other symptoms include nausea and weight loss. In some patients the symptoms can be devastating and can lead to erroneous diagnoses of an eating disorder, psychiatric conditions, or functional abdominal pain (e.g. irritable bowel syndrome, abdominal migraine). The diagnosis is made based on a combination of the clinical symptoms and radiology imaging. There is a surgical procedure that can be performed that is effective in approximately 60-80% of patients.

Acknowledgement of Median Arcuate Ligament Syndrome has not been added yet.

The characteristic MALS patient is more likely to be young adult female, which is consistent with demographic characteristics of other chronic abdominal pain (CAP) patients. However, given the wide distribution of celiac artery compression, the syndrome affects both young and old as well as women and men.

 

Name Abbreviation
Celiac Artery Compression Syndrome CACS
Median Arcuate Ligament Syndrome MALS
Dunbar Syndrome Dunbar Syndrome
Harjola-Marable Syndrome Harjola-Marable Syndrome
Celiac Axis Syndrome Celiac Axis Syndrome
Marable Syndrome Marable Syndrome
Celiac Trunk Compression Syndrome Celiac Trunk Compression Syndrome

The median arcuate ligament is formed by the merging of the right and left attachments of the diaphragm as they cross over the aorta as it enters the abdominal cavity from the chest. The relationship of the ligament to the celiac artery origin determines compression: when the celiac artery comes off the aorta above the diaphragm, this can result in compression; when the celiac artery branches off the aorta below the diaphragm, there is no compression. In a study of 75 autopsies, the median arcuate ligament crossed the celiac artery origin entirely (33%) or partially (48%) in a majority of individuals, resulting in significant celiac artery compression.1

Since 13-50% of healthy asymptomatic patients have a form of celiac artery compression and a much smaller percentage of these patients actually report symptoms consistent with MALS,2 there is significant debate amongst doctors regarding the existence, the causes, and the management of MALS. The cause of the symptoms is thought to be due to either poor blood flow from celiac artery compression, nerve irritation from compression celiac nerve plexus, or a combination of both. Compression of the celiac artery may result in blood flow coming from another abdominal blood vessel (the superior mesenteric artery) and going to the stomach and liver when a patient eats. This is known as a “steal phenomenon” and a possible cause of abdominal pain.3-5 Another theory is that the nerves in the area of the celiac artery (the peri-aortic ganglia and celiac nerve plexus) are also thought to be overstimulated leading to spasm (vasoconstriction) of the arteries going to the stomach and small bowel and this results in symptoms. Another theory is the compression of the nerves causes interference of the brain/stomach (neuro-enteric) pain pathways resulting in hypersensitivity and pain in the stomach. Common surgical teaching maintains that chronic gastrointestinal ischemia occurs when two of the three major intestinal blood vessels have blocked blood flow; however, now many doctors believe that gastrointestinal ischemia may have a number of different causes including a neurologic component.

Compression of the celiac artery by the median arcuate ligament is a poorly understood vascular compression syndrome involving the celiac artery and celiac nerve plexus that results in upper abdominal pain (frequently made worse with eating), weight loss, nausea and vomiting. Sometimes a doctor may hear a soft whooshing sound with a stethoscope (bruit) over the upper abdomen that may mean there is a vascular blockage. Some patients who are athletes may experience recurrent upper abdominal pain that is brought on by moderate to intense cardiovascular work outs. Additional symptoms associated with the diagnosis, but frequently indicative of other medical problems include palpitations (hearing or feeling your own heartbeat), chest pain, diarrhea, constipation, and difficulty sleeping.

Name Description
Weight Loss Weight Loss
Diarrhea Diarrhea
Abdominal pain Abdominal pain
Vertigo or dizziness Vertigo or dizziness
Nausea and vomiting Nausea and vomiting
Bloating Distention of abdomen
Gastroparesis Gastroparesis is a condition that affects the normal spontaneous movement of the muscles (motility) in your stomach. Ordinarily, strong muscular contractions propel food through your digestive tract. But if you have gastroparesis, your stomach's motility is slowed down or doesn't work at all, preventing your stomach from emptying properly.

Because there are many patients with celiac artery compression and no symptoms, and because there are many causes for abdominal pain, it is important that patients are evaluated for all possible common causes of abdominal pain before being diagnosed with MALS. (Table 1)

There has been very little published with specific protocols for diagnosis of MALS. Mak, et al reported the use of a specific diagnostic protocol. Complete medical evaluation should include blood work (complete blood count, chemistry panel, liver function tests, amylase, lipase, inflammation markers (erythrocyte sedimentation rate, C-reactive protein), pre-albumin, thyroid function tests), upper gastrointestinal imaging studies, small bowel follow-through, abdominal ultrasound, upper endoscopy with biopsy, and evaluation for inflammatory bowel disease and celiac disease. Patients are then screened with mesenteric duplex ultrasound. Positive findings demonstrate elevated blood flow velocities (PSV=peak systolic velocity) in the celiac artery greater than 200 cm/sec and an end diastolic velocity (EDV) greater than 55 cm/sec. Further demonstration of a decrease or even normalization of the velocities with deep inspiration is suggestive of celiac artery compression.4 Patients then undergo CT (computerized tomography) scan, MRA (magnetic resonance angiogram) or sometimes an angiogram to confirm the change in the shape of the celiac artery in both inspiratory and expiratory phases.4

Once other common causes of pain have been excluded and celiac artery compression is confirmed, it is crucial that patients are evaluated for proper patient selection for surgical intervention. Patient characteristics reported to be predictive of successful outcomes following surgery include consistent abdominal pain after eating, patients between the ages of 40-60 years, and weight loss of 20 pounds or greater. Surgery tends to not help in patients in which the pain is atypical, there are periods of remission, in patients over the age of 60 years, in patients with a history of alcohol abuse, and weight loss of less than 20 pounds.4, 6-9

Additionally, Mak et al reported incorporating psychiatric and chronic pain service in the pre-operative and post-operative evaluations given the correlation between chronic physical pain and psychological pain. Pre-operatively, all patients are evaluated by a multi-disciplinary team consisting of general and vascular surgery, psychiatry, and pain service. This team then discusses each patient, and surgery is not considered until the patient has been unanimously cleared by the entire team.4 We have found this approach to be extremely helpful to the patients.10-12

Celiac artery compression syndrome is a diagnosis of exclusion. Pertinent workup of this condition may include simple tests to rule out other etiologies causing diagnostic uncertainty. These can include colonoscopy, ultrasound of the liver, pancreas, and gallbladder, upper gastrointestinal endoscopy, complete blood count, hepatic function testing, serum amylase and lipase, C-reactive protein, and testing of certain antibodies such as anti-smooth muscle antibody (ASMA). Several diagnostic modalities can be employed to make a diagnosis of celiac artery compression syndrome. These can be categorized as non-invasive and invasive modalities.

The invasive modalities include conventional visceral angiography while the invasive modalities include Doppler ultrasound (US), magnetic resonance imaging (MRI), and computerized tomography angiography (CTA). On ultrasound, there is a demonstration of elevated celiac artery peak systolic velocities with deep expiration. More specifically, the following two criteria are supportive for the diagnosis of celiac artery compression syndrome on ultrasound: expiratory peak velocity of greater than 200 cm/s and deflection angle greater than 50 degrees. 

Additionally, the following findings may also be encountered: the abnormal origin of the celiac artery, flow reversal in the hepatic artery and lowering of velocity in the celiac artery when the patient stands erect. Conventional visceral angiography shows partial to complete stenosis of the celiac artery secondary to extrinsic compression with possible post-stenotic dilation and retrograde filling of the celiac artery. During visceral angiography, intravascular ultrasound can be used to demonstrate ostial compression of the celiac artery with expiration. CTA shows compression of the celiac axis with focal stenosis and post-stenotic dilation. On these modalities, one can appreciate the difference in perfusion of the celiac artery during inspiration and expiration. Additional studies that may be done to diagnose celiac artery compression syndrome include gastric tonometry and percutaneous celiac ganglion block.

Reference updated April 2021: https://www.ncbi.nlm.nih.gov/books/NBK470601/

Name Description
Open or Laparoscopic Celiac Artery Release Surgery Surgery to release compression of the celiac artery by the median arcuate ligament and/or the celiac nerve plexus. Can be made via open incision or laparoscopy.
Prognosis of Median Arcuate Ligament Syndrome has not been added yet.
Tips or Suggestions of Median Arcuate Ligament Syndrome has not been added yet.
References of Median Arcuate Ligament Syndrome has not been added yet.
2021 Living Rare, Living Stronger NORD Patient and Family Forum June 26-27 Created by PearlyEverlasting
Last updated 21 Jun 2021, 02:02 PM

Posted by PearlyEverlasting
21 Jun 2021, 02:02 PM

NORD, National Organization for Rare Disorders, is having its annual Patient and Family Forum this weekend, June 26-27!

As a Patient or Family Member, you can join this virtual event for free, and/or pay for registration or make a donation.

This is a wonderful opportunity to meet your rare disorder peers and supporters, as well as learn from rare disease experts and professionals.

Be sure to register for this online event: 2021 Living Rare, Living Stronger NORD Patient and Family Forum

Here is the URL for the link: https://livingrare.org/

https://livingrare.org/wp-content/uploads/2021/04/lrls-people.png

Community Resources
Title Description Date Link
Rare Disease Foundation

The Rare Disease Foundation, a Canadian organization, provides information and support for Patients, Families and the Medical communities.

https://rarediseasefoundation.org/

Rare Disease Foundation

07/07/2021
NORD: National Organization for Rare Disorders

NORD is a fantastic American organization for those of us with a rare disorder!

They have resources for Patients and Families, for Researchers, for Advocacy, and for Networking. Check out their webpages!

https://rarediseases.org/

07/06/2021
For Sonographers--A Quiz on MALS

Take this quiz to check your interpretive skills:

Ultrasound findings in Median Arcuate Ligament Syndrome

06/13/2021
Orillia (Canada) woman opens up about rare disorder

"Jane Brennan waited eight years to be diagnosed with median arcuate ligament syndrome; she urges others to 'keep pressing for answers'"

Read Jane's story by clicking on the link.

06/13/2021
Whatʼs Wrong With Me? Readers Digest, Nov 2017

From Readers Digest, a personal story of being diagnosed with MALS:

"In 2014, while working in South Africa, she was contacted by a medical student and patient advocate from California who had heard about her case and wanted to run it by more specialists. Eventually it reached Dr. Christopher Skelly in Chicago, who suspected she had median ar- cuate ligament syndrome."

06/13/2021
Median Arcuate Ligament Syndrome Clinical Presentation, Pathophysiology, and Management: Description of Four Cases–2021

This 2021 article describes the authors' experiences with this entity, focusing on symptom presentation, diagnostic challenges, and management, including long-term follow-up.

06/13/2021
Median arcuate ligament syndrome-2020

This 2020 review article summarizes the literature pertaining to the pathophysiologic mechanism, presentation, diagnosis, and management of MALS. A suggested diagnostic workup and treatment algorithm are presented.

06/13/2021
Median Arcuate Ligament Syndrome (MALS)–2021

Informative description of MALS, including history, symptoms, diagnosis, treatment, prognosis from the Cleveland Clinic. Reviewed for content by professionals in March 2021.

06/13/2021
Median arcuate ligament syndrome: A clinical dilemma–2021

To this day, controversy persists around the pathology of this syndrome, with some groups labeling it a vascular disease, while others consider it a neurogenic disease.

Initially, treatment for MALS was through an open surgical approach, in which the main objective was celiac artery revascularization.

The inevitable consequence of this operation was a complete neurolysis performed during exposure of the vessels.

This neurolysis is now believed to be the main technical aspect that results in symptom improvement, rather than revascularization.

Over the last decade, experience with minimally invasive approaches has grown, and our understanding of the disease has highlighted the role of the celiac plexus nerve fibers as the most relevant anatomic structure related to this syndrome.

 

06/13/2021
Harjola Marable Syndrome: A Rare Case Report–2018

Here is a synopsis of this 2018 Case Report:

We describe a case of 31 years old male patient who presented with complaints of pain in epigastric region and was diagnosed as celiac artery syndrome after extensive workup including OGD scopy, USG and CT angiography. He underwent laparoscopic release of median arcuate ligament which resulted in relief of symptom

06/13/2021
Laparoscopic treatment of Dunbar syndrome: A case report–2017

Key Features of this 2017 paper:

Dunbar syndrome is a rare disorder due to external compression of the celiac trunk by the median arcuate ligament. The symptoms include abdominal pain, nausea and vomiting. Laparoscopic treatment was suggested as a safe procedure. The aim of this study is to present a case of Dunbar syndrome underwent laparoscopic treatment.

A 17-year-old female patient presented at emergency room with upper abdominal pain and dyspepsia, related to food intake. A selective arteriography of the celiac trunk revealed stenosis due to compression of the celiac artery. The decompression of the celiac trunk by the median arcuate ligament was performed. Postoperative course was uneventful and the patient was discharged on the 5th postoperative

Conclusions

Laparoscopic division of the arcuate ligament in patients with Dunbar syndrome is feasible and safe. This procedure can be performed uneventful in order to restore quality of life of the patient.

06/13/2021
Median Arcuate Ligament Syndrome–2019

This 2019 review article summarizes the literature pertaining to the pathophysiologic mechanism, presentation, diagnosis, and management of MALS. A suggested diagnostic workup and treatment algorithm are presented.

06/13/2021
National MALS Foundation

Helpful information about MALS and how to have a treatment plan and team.

06/13/2021
Median Arcuate Ligament Syndrome

National Organization for Rare Disorders–NORD, information pages.

06/13/2021

Clinical Trials


Cords registry

CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access.

Enrolling is easy.

  1. Complete the screening form.
  2. Review the informed consent.
  3. Answer the permission and data sharing questions.

After these steps, the enrollment process is complete. All other questions are voluntary. However, these questions are important to patients and their families to create awareness as well as to researchers to study rare diseases. This is why we ask our participants to update their information annually or anytime changes to their information occur.

Researchers can contact CoRDS to determine if the registry contains participants with the rare disease they are researching. If the researcher determines there is a sufficient number of participants or data on the rare disease of interest within the registry, the researcher can apply for access. Upon approval from the CoRDS Scientific Advisory Board, CoRDS staff will reach out to participants on behalf of the researcher. It is then up to the participant to determine if they would like to join the study.

Visit sanfordresearch.org/CoRDS to enroll.

Community Leaders

PearlyEverlasting

I reside in Toronto, Canada, and live with two rare conditions: MALS (Median Arcuate Ligament Syndrome) and PID (Primary Immune Deficiency).

It took decades of being a super-sleuth to be properly diagnosed with these conditions! My hope is to provide whatever experiences I have gained along the way to assist you with your research for the right people and right answers.

Thankfully, these days there are so many excellent organizations to contact for education, support, and networking, and RareShare is providing a host for us all to network and build a strong and vibrant presence as an important patient and provider population.

Please feel welcome to view the many items here! They are meant for all: patients, family and supporters, caregivers, medical professionals, research proferssionals, and healthcare advocates.

And yes, I can still play the Oboe!

 

Expert Questions

Ask a question

Community User List

I reside in Toronto, Canada, and live with two rare conditions: MALS (Median Arcuate Ligament Syndrome) and PID (Primary Immune Deficiency).

It took decades of being a super-sleuth to be properly...

test


Start a Community


Don't See Your Condition On Rareshare?

Start your own! With a worldwide network of 8,000 users, you won't be the only member of your community for long.

FAQ


Have questions about rareshare?

Visit our Frequently Asked Questions page to find the answers to some of the most commonly asked questions.

Discussion Forum


Communities

Our Communities

Join Rareshare to meet other people that have been touched by rare diseases. Learn, engage, and grow with our communities.

FIND YOUR COMMUNITY
Physicians

Our Resources

Our rare disease resources include e-books and podcasts

VIEW OUR EBOOKS

LISTEN TO OUR PODCASTS

VIEW OUR GUIDES

Leaders

Our Community Leaders

Community leaders are active users that have been touched by the rare disease that they are a part of. Not only are they there to help facilitate conversations and provide new information that is relevant for the group, but they are there for you and to let you know you have a support system on Rareshare.