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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.

 

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.

 

Synonyms for Median Arcuate Ligament Syndrome has not been added yet.

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.

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

Diagnostic tests of Median Arcuate Ligament Syndrome has not been added yet
Treatments of Median Arcuate Ligament Syndrome has not been added yet.
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.
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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 live in Toronto, and had an open surgery in 2018 to correct my celiac artery compression in California. My life changed, for the better!

I moved there from Vancouver, where the two surgeons I had seen said the surgery was considered unnecessary, and that I would have to pay for it out of pocket. It was a drastic decision to move so far away to the USA to have the surgery, but I did my homework and found the right surgeon.

My hope is that Canada coninues to improve treatment options for all of us who live with this debilitating condition, and will find more ways to provide the necessary funding to diagnose the condition, train surgeons, and provide medical coverage for Canadian patients.

Please join the conversation, and share your story, your successes and challenges, of living with MALS.

And, most of all, discover that you and your supporters are not alone in seeking treatment for this rare condition!

 

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I live in Toronto, and had an open surgery in 2018 to correct my celiac artery compression in California. My life changed, for the better!

I moved there from Vancouver, where the two surgeons I had...

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