Median Arcuate Ligament Syndrome as a Rare Cause of Epigastric Pain

Introduction. A 44-year-old woman presented to an internal medicine clinic with postprandial epigastric pain that has been worsening for the past year. She also reported associated symptoms of nausea, vomiting, significant weight loss, and diarrhea. Although these symptoms are common in patients with median arcuate ligament syndrome, the presentation of these symptoms simultaneously does not occur often.

Median arcuate ligament syndrome (MALS) is a relatively rare disease; however, it is still an important consideration in patients who have persistent postprandial epigastric pain despite a largely unremarkable workup. Also known as celiac artery compression syndrome or Dunbar syndrome, MALS has a 4:1 female predominance and a 2 per 100,000 prevalence.¹ 

In the literature, there are generally two main philosophies regarding the pathophysiology of MALS: (1) patients either develop postprandial abdominal pain because of distal ischemia secondary to celiac artery compression by the median arcuate ligament² or (2) the symptoms of MALS are attributed to the compression of the celiac nerve plexus, which is positioned superior to the celiac artery trunk, by the median arcuate ligament. Compression of the celiac artery is relatively common in the general population–seen in 10% to 24% of people on imaging studies; however, less than 1% of those with stenosis are symptomatic.^3,4 Our case highlights an approach to the work up for postprandial abdominal pain, as well as the diagnosis and treatment for MALS. 

Case Description. A 44-year-old woman with a past medical history of spontaneous coronary artery dissection, left ventricular thrombus, hypertension, gastroesophageal reflux disease (GERD), ischemic cardiomyopathy (ejection fraction of 10%), and atrial fibrillation, presented for evaluation of postprandial epigastric pain. The patient described the onset of the pain as gradual, progressively worsening during the past year. The pain was further described as sharp, with radiation to the back, and onset of pain correlated with eating. The pain was associated with nausea, vomiting, diarrhea, unintentional weight loss (40 pounds). The patient noted that her pain was only alleviated by food avoidance and exacerbated by large meals and lying in the supine position. Prior to presentation, the patient reported no imaging or other studies.

The patient was taking the following medications: spironolactone 50 mg BID, metoprolol succinate 125 mg QD, sertraline 25 mg QD, amiodarone 200 mg QD, bumetanide 2 mg 5x/day, pantoprazole 40 mg BID, trazodone 25 mg QHS, dapagliflozin 5 mg QD, and warfarin 7.5 mg QD. Her immunizations were up to date. The patient had no known allergies.

The patient appeared alert and oriented with no apparent distress. She was ambulating with adequate motor strength. During her examination, the patient had normal bowel sounds with no presence of an abdominal bruit. Abdomen was non-tender, non-distended, and soft, with no guarding, rebound, or masses. Oral cavity was clear with moist mucous membranes.

The patient underwent esophagogastroduodenoscopy, which was largely unremarkable with exception of a non-bleeding gastric erosion. A computed tomography angiography (CTA) of the abdomen and pelvis was ordered, which revealed proximal celiac artery narrowing adjacent to the median arcuate ligament. A follow-up mesenteric duplex ultrasound was performed, which showed elevated velocities in the celiac axis on both expiration (peak systolic velocity [PSV]: 269 cm/s and End-diastolic volume [EDV]: 62 cm/s) and inspiration (PSV: 202 cm/s and EDV: 80 cm/s), increasing the clinical suspicion for MALS as an explanation for this patient’s symptoms.

To further support the diagnosis, a celiac plexus nerve block (CPB) was performed. The patient reported immediate, significant improvement in her abdominal pain. Ideally, the next step in treatment for suspected MALS would be referral to a vascular surgeon for median arcuate ligament release; however, given our patient’s significant cardiac comorbidities, she was being worked up for heart transplant, which precluded her candidacy from elective median arcuate ligament release surgery.

Discussion. Postprandial epigastric pain is a common concern encountered by inpatient and outpatient providers ranging in severity from benign to life threatening. The typical symptoms of MALS include postprandial abdominal pain, nausea, vomiting, weight loss (typically greater than 20 pounds), food fear/avoidance, and/or diarrhea.⁵

There was a wide differential for postprandial abdominal pain (Table 1). Given the broad differential for postprandial abdominal pain, MALS is often diagnosed years after presentation, often leading to significant morbidity for patients as well as increased costs to the health care system.⁶ In this case, a diagnosis was made in one year, with the main indicator being the celiac artery narrowing noticeable on the CTA of the abdomen and pelvis.

Once this disease is suspected, an ultrasound with doppler should be performed. If the peak expiratory flow is above 350 cm/s in combination with a celiac artery deflection angle of more than 50°, there is an 83% sensitivity and 100% specificity for MALS.⁷ The CPB can provide some diagnostic clarity and may indicate which patients would benefit from median arcuate ligament release.⁷ The CPB can be performed percutaneously and CT-guided or endoscopically via an endoscopic ultrasound-guided CPB.^{8, 9} The CPB directly inhibits the sympathetic pain pathway in the region of interest via an injection of corticosteroids or long-acting anesthetics near the celiac plexus.¹⁰ Relief from pain can be seen almost immediately post-procedure if sodium channel inhibitors are administered concurrently. Patients benefiting from CPB could be considered appropriate candidates for surgery. In this case, as the patient’s condition improved significantly following the CPB, our diagnosis of MALS in the patient was correct. Since MALS is an often-overlooked consideration when assessing postprandial pain, leading to typically late diagnoses, we recommend that physicians consider this condition more frequently in their differential diagnoses.

Table 1. Postprandial abdominal pain differential diagnoses

Definitive treatment of MALS requires surgical intervention, termed median arcuate ligament release (MAL release). The first MAL release was successfully completed in 1963 by Harjola.¹¹ The surgical procedure is typically performed by the removal of portions of the median arcuate ligament, as well as the nerve and ganglionic tissue, surrounding the celiac trunk, thereby releasing the celiac artery.¹² The percentage of patients experiencing symptom remission post-ligament release has been cited as up to 84.6%.¹³ The MAL is directly adjacent to the celiac artery and plexus, posing significant challenges to surgical exposure. Importantly, MAL release is associated with an increased risk of significant hemorrhage, which does preclude some patients from pursuing operative intervention. 

There are several different approaches to performing a MAL release: open approach, a laparoscopic approach, or via robotic-assisted laparoscopy. The open surgical approach is the most invasive, involving a large laparotomy incision with significant manipulation of the bowel to allow exposure.¹² Alternatively, the laparoscopic approach is minimally invasive, resulting in decreased morbidity, quicker recovery, and decreased postoperative pain in comparison with the open approach.¹⁴ As a result, laparoscopic MAL release has become the standard surgical treatment for MALS.¹³ Because of limited instrument maneuverability and a high conversion to open surgery rate of 9% to 10% with the laparoscopic approach, robotic-assisted MAL release is becoming increasingly favored.^15,16 With the robotic-assisted approach, surgeons gain improved visualization and operator controls, while offering the same benefits of the laparoscopic approach compared with the open approach.¹⁷

Conclusion. Although rare, MALS should be considered as a cause for postprandial abdominal pain. It can be diagnosed using CTA abdomen pelvis in conjunction with a mesenteric doppler ultrasound. It can further be delineated by response to a CT-guided or EUS-guided celiac ganglion block and definitively treated surgically with a MAL release procedure.

Postprandial epigastric pain is a common concern encountered by inpatient and outpatient providers ranging in severity from benign to life threatening. Indeed, MALS can be attributable to an anatomic variant characterized by a low-riding median arcuate ligament and a slightly superiorly located celiac artery resulting in compression of the celiac plexus and/or artery. This compression is thought to lead to postprandial abdominal pain. Our case highlights an approach to the work up for postprandial abdominal pain, as well as the diagnosis and treatment for MALS. 

AUTHORS:
Farah Khan MD FACP^{1, 2, 3} • Daniyaal Syed BA • Daniel Levy MD⁴ • Luke Mollnow DO⁴

AFFILIATIONS:
¹Department of Internal Medicine, Rush University Medical Center, Chicago, Illinois
²Ascension Saint Joseph Hospital, Chicago, Illinois
³Advocate Illinois Masonic Medical Center, Chicago, Illinois
⁴Hospital Corporations of America (HCA) Sky Ridge Medical Center Graduate Medical Education (GME) Internal Medicine Residency Program

CITATION:
Farah K, Daniyaal S, Daniel L, et al. Median arcuate ligament syndrome as a rare cause of epigastric pain. Consultant. Published online May 21, 2025. doi:10.25270/con.2025.04.000001.

Received September 5, 2024 Accepted December 20, 2024

DISCLOSURES:
The authors report no relevant financial relationships.

ACKNOWLEDGEMENTS:
Patient consent was obtained for publication of the case details.

CORREPONDENCE:
Daniyaal Syed, 5011 Persimmon Lane, Castle Rock, CO, 80109 (dsyed36@gmail.com)

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