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Median Arcuate Ligament Syndrome: A Case Report and Literature Review on the Challenges of Diagnosis

P-026 - (Poster #26) Median Arcuate Ligament Syndrome: A Case Report and Literature Review on the Challenges of Diagnosis

Thursday, September 4, 2025
4:00 PM - 5:30 PM PST
Location:  Exhibit Hall (Level 4)
Background: Median Arcuate Ligament Syndrome (MALS) is caused by the compression of the celiac artery in addition to the celiac ganglion. The involvement of the celiac ganglion suggests neuropathy as a primary component to MALS pathology. This compression manifests as abdominal pain especially after eating or exercise along with fatigue, weight loss, and nausea. MALS also has associated comorbidities that could potentially lead to a faster diagnosis including, but not limited to postural orthostatic tachycardia syndrome, Ehlers-Danlos syndrome, and mast cell activation syndrome. A study from 2021 was performed on the association of hypermobility disorders and development of compression syndromes and it was found that 72% of patients with at least one compression syndrome had a co-existing hypermobility disorder. The patient in this case report had both hypermobile Ehlers-Danlos Syndrome as well as postural orthostatic tachycardia syndrome at the time of diagnosis along with the classic symptoms of epigastric abdominal pain exacerbated with exercise and eating, weight loss, and extreme fatigue.  

Purpose/Objectives: The goal of this case report is to highlight the challenges of diagnosing median arcuate ligament syndrome. While median arcuate ligament syndrome is rare, it also presents with symptoms that can be vague and not guide a physician to a clear diagnosis. Median arcuate ligament syndrome is diagnosed after all other differentials have been ruled out as there is no specific blood test that can confirm a MALS diagnosis. However, there are associations such as age, gender, and comorbidities that if known by a wider range of specialists, can lead to MALS being included in the differential and ultimately being diagnosed sooner with the use of CT and ultrasound examining celiac artery compression.  

Methods: This patient’s original symptom was abdominal pain that began in 2019. Throughout 2020 multiple PCPs could not determine the cause of the symptoms and the patient was referred to a GI specialist. A full gastrointestinal workup was completed including multiple imaging studies in 2021. No structural abnormalities were noted, and the gastroenterologist made a diagnosis of IBS. By the end of 2021 the patient began having orthostatic symptoms including dizziness, tachycardia, and shortness of breath, ultimately leading to a postural orthostatic tachycardia syndrome (POTS) diagnosis. A year later the patient went to a dysautonomia specialist where a full POTS workup was completed, and a diagnosis of hypermobile Ehlers Danlos Syndrome was made. This additional diagnosis along with POTS lead the physicians into checking for a compression syndrome. Abdominal pain was still the main symptom that no other treatment had worked for thus far. A diagnosis of MALS was made in 2023 using CT imaging and ultrasound to visualize celiac artery compression in association with patient presentation. To determine if the patient was a candidate for surgery, a CT guided celiac plexus block was performed.  

Results: The patient experienced a successful celiac plexus block that resulted in alleviation of pain and nausea for roughly 48 hours. The alleviation of pain from the block demonstrated the patient was a candidate for surgery and then opted to undergo open decompression surgery. Open MALS surgery was performed in January of 2024 to remove the median arcuate ligament and accompanying overlying crus to 4cm above the celiac artery. Celiac plexus nerves were resected back to the celiac ganglion and a partial ganglion resection was performed. The p</span>atient stayed in the hospital for 4 days with the main focus on pain management and adequate food intake. Once discharged the patient focused on mobility and medication tapering. Repeat labs were run one month post op to evaluate adrenal function and all came back within normal limits. The patient had a full recovery back to a normal quality of life within six months which consisted of no epigastric pain or nausea, resolution of shortness of breath, and substantially reduced fatigue. Full weight gain back to original weight prior to symptom onset took 8 months. The patient still has postural orthostatic tachycardia syndrome that is easier to manage post-surgery with oral antihypotensive medication.  

Conclusions/Implications for future research and/or clinical care: Once the patient developed POTS she got to a specialist who knew to look for compression syndromes. However, the patient always had symptoms of hypermobility from the time she was an adolescent. If the hEDS diagnosis could have been made earlier other physicians could have known the association between Ehlers Danlos and compression syndromes. In addition, gastroenterologists should also be aware of compression syndromes and specify when doing radiologic workups to assess celiac artery blood flow. This patient could have been diagnosed with MALS in 2021 if her GI specialist or radiologist knew to examine the celiac artery on her original abdominal CT scan in 2021. The patient’s gastroenterologist, when told the diagnosis of MALS, did not know about any compression syndromes or that MALS was a condition to check for in a gastrointestinal workup. With the main symptoms being gastrointestinally related, it is imperative that gastroenterologists know what MALS is to adequately diagnose and recommend treatment to their patients. For this patient, if MALS was diagnosed after the first CT scan, the patient may never have developed dysautonomia, as it took two years from onset of abdominal pain to the development of postural orthostatic tachycardia syndrome.