Median Arcuate Ligament Syndrome

What is Median Arcuate Ligament Syndrome?

Median Arcuate Ligament Syndrome is a resultant of the celiac axis compression by the diaphragmatic crura. Anatomically, diaphragmatic crura are attached with fibrous arch named as a median arcuate ligament. The incidence of this disease is rare and often misdiagnosed due to unawareness. The symptomatic presentation is almost similar with other gastric conditions like peptic ulcer, irritable bowel syndrome, appendicitis, gallbladder disease etc. Therefore, the affected patients have to go for differential diagnosis to eliminate the probability of a similar type of other ailments.

Alternatively, Median Arcuate Ligament Syndrome is also termed as celiac axis syndrome, celiac artery compression syndrome or Dunbar syndrome^1,2.

Predominance

Young adults age between 20 to 40 years people is usually affected with Median Arcuate Ligament Syndrome. By analyzing different case reports, thin females are more prevail to get the syndrome¹.

Symptoms

All the symptoms of Median Arcuate Ligament Syndrome arise due to compression of celiac axis and that causes compromised blood circulation.  Symptoms of Median Arcuate Ligament Syndrome include^2,3

• Nausea
• Vomiting
• Abdominal pain, specifically post-prandial abdominal pain leading to an aversion to food
• Weight loss
• Epigastric bruit

Etiology

The etiology of Median Arcuate Ligament Syndrome is yet now not understood fully. But multifactorial causes provides compression of the celiac artery and adjacent neurogenic structures. Case report analyzing provides a possibility of congenital origin and pathophysiology may include the neuropathic or vascular base.

The detailed study of clinical presentation of Median Arcuate Ligament Syndrome showed that only vascular compression of the celiac artery is not solely responsible, as some patients have asymptomatic compression and also the collateral circulation can be maintained by adequate blood supply coming through the superior mesenteric artery. Therefore, the main symptomatic trouble i.e. postprandial abdominal pain cannot arise only due to compression of celiac artery alone^2,3.

Radiology

Median Arcuate Ligament Syndrome can be diagnosed through involving radiology. The use tools are as follows:

Conventional Angiography

Conventional angiography was the gold standard tool to detect the median arcuate ligament syndrome. Catheter angiography is a typical method to conduct the test. The compression of the celiac axis is less prominent during inspiration, but compression increases during expiration. Even the compression pressure during expiration is not same for every affected patient. in a rare instance, the compression arises during expiration can remain to continue during inspiration^2,3.

CT Angiography

CT angiography is an advanced radiological tool to diagnose the median arcuate ligament syndrome. Novel thin-section multidetector CT scanners combining with 3D software is improved technology has the ability to produce high-resolution images of the aorta and adjoined arteries. The visualization of mesenteric vessels is very clear. These benefits of, CT angiographic examination averts conventional angiography^2,3.

Ultrasound

Mesenteric duplex ultrasonography is another radiologic imaging tool utilizes for demonstrating celiac artery compression. Though hepatobiliary ultrasound unable to reveal any apparent defects. Doppler ultrasound is also useful to detect the abnormalities associated with median arcuate ligament syndrome^2,3.

Surgery

There is a mixed approach for surgical management for treating median arcuate ligament syndrome. Many patients complain different abnormalities after operation and complications are sometimes more than the disease-related symptoms. It has been observed that symptomatic relieve after surgery can successfully achieve in patients with 40–60 years of age. The benefits of the successive symptomatic reduction include postprandial pain, weight loss more than 20-lb, post stenotic dilatation, and collateral vessels.

Laparoscopic surgery is one of the advanced options for surgical division of ligament. Laparoscopic surgery is less invasive than the traditional method. This surgical method is also applied for vascular reconstruction, as celiac axis construction is one of the primary cause of median arcuate ligament syndrome.

Invasive process in the celiac artery has a great risk of bleeding, therefore surgical team should be prepared for proper intervention. During surgery, the teamwork of surgical staff is very important to deal with potential vascular complications. The advantage of Laparoscopic surgery also includes faster recovery and less pain after surgery.

The Recent development also looks forward to robot-assisted laparoscopic approach, though much research based data yet not available. But clinician expects the improved three-dimensional acuity of robotics presents better dexterity and management to divide the ligament and fix around the celiac artery than laparoscopic procedures. Although surgeon skill is primary for handle all the surgical procedure^2,3,4.

References

1. Karen M. Horton, Mark A. Talamini, Elliot K. Fishman, Median Arcuate Ligament Syndrome: Evaluation with CT Angiography; DOI: http://dx.doi.org/10.1148/rg.255055001; http://jamanetwork.com/journals/jamasurgery/article-abstract/2498851; online available at http://pubs.rsna.org/doi/full/10.1148/rg.255055001
2. Balaban DH, Chen J, Lin Z, Tribble CG, McCallum RW.; Median arcuate ligament syndrome: a possible cause of idiopathic gastroparesis; Am J Gastroenterol. 1997 Mar;92(3):519-23.; online available at https://www.ncbi.nlm.nih.gov/pubmed/9068484
3. Jae S You, Matthew Cooper, Steven Nishida, Elna Matsuda, Daniel Murariu; Treatment of Median Arcuate Ligament Syndrome Via Traditional and Robotic Techniques; Hawaii J Med Public Health. 2013 Aug; 72(8): 279–281; online available at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3848179/
4. Erinn N. Kim, Kathleen Lamb, Daniel Relles, et al.; Median Arcuate Ligament Syndrome—Review of This Rare Disease; JAMA Surg. 2016;151(5):471-477. doi:10.1001/jamasurg.2016.0002; online available at http://www.vasculardiseasemanagement.com/content/a-case-median-arcuate-ligament-syndrome-successful-angioplasty-and-stenting