Case Presentation: A 61-year-old female with past medical history of coronary artery disease, antiphospholipid syndrome, Sjogren’s syndrome, and double stenting of the celiac artery due to focal dissection one month prior to her current hospitalization presented to the hospital with a 6-month history of substernal and mid-epigastric pain which worsened with food intake. Over the past 2-3 weeks prior to hospital admission, the patient was unable to tolerate oral intake due to the significant pain. On physical exam, vitals were stable, she had generalized abdominal tenderness to palpation along with right and left costovertebral angle tenderness. Lab-work did not reveal any acute relevant findings. CT angiogram of the chest along with CT abdomen and pelvis with intravenous contrast revealed moderate to severe stenosis of the celiac trunk likely secondary to compression from the median arcuate ligament. Other etiologies such as autoimmune work-up for vasculitis and gastroenterology work-up with esophagogastroduodenoscopy and colonoscopy did not reveal any acute findings. Decision was made for the patient to undergo a bilateral celiac plexus block. The patient had symptomatic improvement following the procedure, however her symptoms did not completely resolve. Due to her incomplete resolution of her pain, the patient underwent ligation of her median arcuate ligament. The patient had improvement of her symptoms following the ligation and no re-occurrence of her pain.
Discussion: Median arcuate ligament syndrome (MALS) is a rare disease that occurs in 2 out of every 100,000 patients. MALS occurs when there is direct compression of the celiac artery by the median arcuate ligament. This disease also compresses the celiac plexus, which contains the afferent fibers from the upper abdominal viscera as well as sympathetic fibers from the greater and lesser splanchnic nerves. Symptoms of MALS can include post-prandial epigastric pain, nausea, vomiting, decreased appetite, and weight loss. The gold standard for diagnosis is with a CT angiogram. Management typically involves surgical decompression of the median arcuate ligament’s constriction of the celiac artery. Blocking the celiac plexus can also provide symptomatic relief. If symptoms persist despite surgical intervention, revascularization of the celiac artery by either endovascular stenting or bypass can be considered. Patients who are treated non-operatively tend to have worse outcomes. Patients who are not treated for MALS may develop arterial aneurysms in the celiac artery.
Conclusions: MALS is a rare disease that can significantly impact individuals who present with the disease. MALS is a diagnosis of exclusion that requires a high grade of suspicion and radiological findings that are consistent with the disease. Treatment focuses on relieving symptoms through surgical intervention. Further studies need to be complete to evaluate optimal diagnosis measures and further investigate the pathophysiology surrounding the disease.