Median Arcuate Ligament Compression Syndrome Management

Median arcuate ligament compression syndrome arises from extrinsic constriction of the proximal celiac artery by the fibrous median arcuate ligament of the diaphragm. Clinically it presents with postprandial abdominal pain, weight loss and, in some cases, nausea or diarrhoea. Diagnosis is established through imaging modalities such as Doppler ultrasound, computed tomography angiography and magnetic resonance angiography, which demonstrate dynamic stenosis of the celiac trunk that typically worsens on expiration. Management necessitates a multidisciplinary approach involving vascular surgeons, radiologists and gastroenterologists. Conservative measures, including dietary modification and analgesia, may be considered in mild cases. Definitive treatment centres on restoring arterial flow and relieving neuropathic pain by division of the median arcuate ligament. Surgical options include open, laparoscopic or robot-assisted release, often combined with intraoperative assessment of blood flow. Endovascular techniques, such as percutaneous transluminal angioplasty with or without stent placement, may serve as adjuncts or stand-alone therapies in select patients. Patient selection is critical, as anatomical variations and co-existing disorders—such as mast cell activation syndrome—can influence outcomes. Long-term follow-up focuses on symptom relief, resumption of body weight and nutritional status, as well as surveillance imaging to confirm vessel patency. Global experience has demonstrated that minimally invasive ligament release achieves durable relief in the majority of well-selected patients, emphasising its role as first-line therapy in specialised centres.

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Median Arcuate Ligament Compression Syndrome Management publication trend

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Technical terms

Median arcuate ligament compression syndrome: Rare vascular disorder caused by focal constriction of the celiac artery by the median arcuate ligament of the diaphragm, leading to ischaemic and neuropathic abdominal symptoms.

Laparoscopic median arcuate ligament release: Minimally invasive surgical division of fibrous ligamentous fibres overlying the celiac trunk to restore arterial flow and decompress the celiac plexus.

Angioplasty: Endovascular procedure in which a balloon is inflated within a stenosed artery to dilate the lumen, frequently accompanied by stent deployment to prevent recoil.

Mast cell activation syndrome: Immunological condition characterised by inappropriate release of mast cell mediators, which may mimic or exacerbate gastrointestinal symptoms of vascular compression syndromes.

References

1. Prognostic factors for the long term outcome after surgical celiac artery decompression in MALS. Orphanet Journal of Rare Diseases (2023).
2. Treatment of median arcuate ligament syndrome: outcome of laparoscopic approach. ABCD Arquivos Brasileiros de Cirurgia Digestiva (São Paulo) (2020).
3. Open vascular treatment of median arcuate ligament syndrome. BMC Surgery (2017).
4. Median arcuate ligament syndrome diagnosis on Computed Tomography: what a radiologist needs to know. Radiology Case Reports (2021).
5. A Severe Case of Median Arcuate Ligament Syndrome with Successful Angioplasty and Stenting. Case Reports in Vascular Medicine (2012).
6. Median Arcuate Ligament Syndrome Clinical Presentation, Pathophysiology, and Management: Description of Four Cases. Gastrointestinal Disorders (2021).