Median arcuate ligament syndrome - Current state of management

Symptomatic celiac artery compression syndrome (CACS) or median arcuate ligament syndrome (MALS) is a controversial diagnosis that should be considered in patients with chronic abdominal pain of unknown etiology despite an extensive medical evaluation. Once suspected, patients should undergo mesenteric duplex ultrasound. Diagnosis is confirmed with elevated celiac artery velocities which normalize with deep inspiration followed by CT angiogram showing the typical "J-hook" conformation of the celiac artery. Patients should then undergo evaluation by a multi-disciplinary team to appropriately select and prepare patients for potential surgical treatment. Surgical options include release of the median arcuate ligament, with or without neurolysis of the celiac nerve plexus, and with or without concomitant revascularization procedures. Approaches can be open, laparoscopic, or robotic. Surgical treatment has an overall success rate of 70-80% with patients reporting improved abdominal pain and quality of life. Post-operatively, patients can have persistent or recurrent abdominal pain and should undergo re-evaluation for possible need for a revascularization procedure for stenosis of the celiac artery or celiac plexus block if the celiac artery flow is normalized. Additionally, some patients will have persistent pain consistent with functional gastrointestinal disorder (FGID) that will then require medical management. Psychiatric comorbidities have been identified as a predisposing factor that may predict poorer outcomes, and there are preliminary findings suggesting that patients with dysautonomia diagnoses may have worse outcomes as well.