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Median Arcuate Ligament Syndrome

What is Median Arcuate Ligament Syndrome?

The diaphragm is a structure made of muscle and fibrous tissue that separates the chest cavity from the abdominal cavity and aids with breathing. There are a few openings or hiatus in the diaphragm that allow structures such as the esophagus or the aorta (the largest blood vessel in the body) to pass from the chest cavity to the lower aspects of the body. The median arcuate ligament (MAL) is a fibrous structure that surrounds a part of the opening that allows for the passage of the aorta. The aorta is the largest blood vessel in the body. It directly comes off the heart, moves down the chest cavity, passes through the diaphragm into the abdominal cavity to supply the rest of the body. In most people, the aorta gives rise to a branch called the celiac artery right below the diaphragm. The celiac artery will then branch into more arteries that supply major digestive organs including the stomach, the first part of the small intestine (duodenum), the liver, gallbladder, pancreas, as well as spleen. Median arcuate ligament syndrome (MALS) occurs when the celiac artery is compressed by the MAL, causing numerous symptoms such as abdominal pain after eating, nausea, vomiting, and weight loss. 

 

 

Synonyms

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

The diaphragm is a structure made of muscle and fibrous tissue that separates the chest cavity from the abdominal cavity and aids with breathing. There are a few openings or hiatus in the diaphragm that allow structures such as the esophagus or the aorta (the largest blood vessel in the body) to pass from the chest cavity to the lower aspects of the body. The median arcuate ligament (MAL) is a fibrous structure that surrounds a part of the opening that allows for the passage of the aorta. The aorta is the largest blood vessel in the body. It directly comes off the heart, moves down the chest cavity, passes through the diaphragm into the abdominal cavity to supply the rest of the body. In most people, the aorta gives rise to a branch called the celiac artery right below the diaphragm. The celiac artery will then branch into more arteries that supply major digestive organs including the stomach, the first part of the small intestine (duodenum), the liver, gallbladder, pancreas, as well as spleen. Median arcuate ligament syndrome (MALS) occurs when the celiac artery is compressed by the MAL, causing numerous symptoms such as abdominal pain after eating, nausea, vomiting, and weight loss. 

 

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

While it seems that in up to a quarter of individuals, the celiac artery is compressed by the MAL, it usually doesn’t cause any symptoms. The exact prevalence of symptomatic MAL compression is not known. MALS may occur to anyone, adult or child, but it is most common in women aged 30-50 with less body fat.

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

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.

The first and most common symptom in MALS is pain in the upper and middle abdominal region. This may occur at rest, either constantly or intermittently, and may worsen with eating or exercise, and may improve with leaning forward. Typically, unintentional weight loss follows, partially due to fear of pain with eating. Other symptoms include nausea, vomiting, diarrhea, and bloating. Additionally, there is evidence that MALS is associated with mental health issues such as anxiety which is likely due to chronic pain. MALS is also associated with an increased risk of visceral artery aneurysms which in some individuals may rupture, causing internal bleeding. Signs of a ruptured visceral artery include sudden, severe abdominal pain, and lightheadedness.

 

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.

MALS commonly is associated with abdominal pain, weight loss, and epigastric bruit. An epigastric bruit is a soft whooshing sound heard by a stethoscope in the middle-upper abdominal region which indicates the compression of the celiac artery. The bruit may become louder and quieter as the individual breathes because the diaphragm, and therefore, the MAL move up and down with respiration, relieving and worsening the compression. However, these symptoms are not specific to MALS and can be observed in many other medical conditions. Thus, MALS is considered a diagnosis of exclusion, and comprehensive testing is necessary to rule out all other causes of abdominal pain before a MALS diagnosis is established. These tests include different imaging and functional studies to visualize the celiac artery or blood flow in this vessel.

An abdominal duplex ultrasound may be the first imaging test performed for the diagnosis of MALS. This test uses high-frequency sound waves to visualize the velocity of blood flow through blood vessels and can show the compression of the celiac artery. Another test that is very helpful in diagnosing MALS is a computed tomographic angiography (CTA). This is a test that combines a CT scan with the injection of a special dye into the blood vessels that looks bright white on CT. This can visualize blood flow in the vessel and highlight any vascular abnormalities such as a narrowing. Both ultrasound and CTA can show changes in blood flow with respiration that is associated with MALS. Another test called gastric tonometry measures the partial pressure of carbon dioxide in the GI tract to determine the extent of tissue death due to inadequate oxygen supply (gastric ischemia). This test is done by passing a small balloon attached to a long, narrow tube from the nose into the stomach. 

 

In addition to diagnosis, CTA can also demonstrate the degree of the formation of collateral circulation which is one of the factors that can predict response to treatment. Less collateralization is associated with a better response. In addition, celiac ganglion (a collection of nerve cell bodies) nerve block can also predict response to surgical treatment. This is done by injecting a local anesthetic into the celiac ganglion which temporarily blocks the function of those nerves and the transmission of pain signals. Those who experience short-term pain relief from a nerve block are more likely to respond to surgical treatment. 

 

The goal of treatment is to address the two main mechanisms believed to be involved in causing MALS symptoms: reduced blood flow to digestive organs due to compression and excessive stimulation of celiac nerve bundles. The only treatment option for MALS is a surgical procedure called MAL release or decompression. In most cases, surgery is done laparoscopically meaning that a few small incisions are made on the skin in the area through which the surgical tools attached to a long slender tube are inserted into the abdominal cavity without making an open incision. These surgical instruments are used to separate the MAL from the celiac artery and ganglion and sometimes the celiac ganglion might be completely resected. This allows blood to flow through the celiac artery without obstruction and reduces nerve-related pain. While most people only need a laparoscopic procedure, a subset of individuals may require open surgery to restore blood flow (revascularization) or repair the celiac artery depending on the extent of damage to the vessel. Laparoscopic surgery has a quicker recovery time, while the open procedure requires more time for recovery. Both procedures are usually associated with immediate pain relief. Some individuals may experience recurrent abdominal pain which may require a second operation. 

 

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.

MALS is associated with mental health issues and lower quality of life due to chronic pain, however, there is no evidence that it reduces life expectancy. Around one-third of the individuals undergoing a MAL decompression procedure respond well to this treatment, however, a subset of individuals may experience recurrent pain.

 

Tips or Suggestions of Median Arcuate Ligament Syndrome has not been added yet.

Camacho N, Alves G, Bastos Gonçalves F, et al. Median Arcuate Ligament Syndrome - Literature Review and Case Report. Rev Port Cir Cardiotorac Vasc. 2017;24(3-4):111. DOI: 10.1002/ccr3.4579

 

Goodall R, Langridge B, Onida S, et al. Median arcuate ligament syndrome. Journal of Vascular Surgery. 2019;71(6):2170-2176. doi:https://doi-org.proxy1.lib.uwo.ca/10.1016/j.jvs.2019.11.012

 

Kim EN, Lamb K, Relles D, Moudgill N, DiMuzio PJ, Eisenberg JA. Median Arcuate Ligament Syndrome—Review of This Rare Disease. JAMA Surg. 2016;151(5):471–477. doi:10.1001/jamasurg.2016.0002

 

Manogna D, Gupta A, Seetharaman M. A Ligamentous Agony: Median Arcuate Ligament Syndrome as an Under-Recognized Cause of Abdominal Pain. Cureus. 2020;12(6):e8865. doi: 10.7759/cureus.8865. PMID: 32617247; PMCID: PMC7325387.

 

Nasr LA, Faraj WG, Al-Kutoubi A, et al. Median Arcuate Ligament Syndrome: A Single-Center Experience with 23 Patients. Cardiovascular Interventional Radiology. 2017;40:664–670. https://doi-org.proxy1.lib.uwo.ca/10.1007/s00270-016-1560-6

 

You JS, Cooper M, Nishida S, Matsuda E, Murariu D. Treatment of median arcuate ligament syndrome via traditional and robotic techniques. Hawaii J Med Public Health. 2013;72(8):279-81. PMID: 24349891; PMCID: PMC3848179.

 

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
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
For Sonographers--A Quiz on MALS

Take this quiz to check your interpretive skills:

Ultrasound findings in Median Arcuate Ligament 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

National Organization for Rare Disorders–NORD, information pages.

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–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
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
National MALS Foundation

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

06/13/2021
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
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
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
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

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!

 

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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...

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