Celiac Artery Compression Syndrome: A Unique Presentation

Celiac artery compression syndrome (CACS), also known as median arcuate ligament syndrome, celiac axis syndrome, and Dunbar Syndrome, is a rare disorder that results from compression of the celiac artery by the median arcuate ligament. The following is a case that depicts an interesting presentation of a patient diagnosed with this rare condition. A 44-year-old male with a history of mutism was brought in by his family for weight loss of 100 lbs with intermittent abdominal pain, weakness and lethargy over a period of five years. His family reported that he had poor nutritional intake, and could only eat a small amount before he seemed to be in pain, and eventually refused to eat. He had no other prior medical history except for mutism, no family history of malignancy, no history of trauma, surgeries, smoking or substance use, and did not take any medications. Physical exam was largely unremarkable. Mesenteric vascular duplex demonstrated severe grade stenosis of the celiac trunk with post-stenotic velocity of 520 cm/sec. Contrast enhanced computed tomography angiography revealed acute angle J-configuration of the takeoff of the celiac axis, with stenosis at its origin and focal post-stenotic dilatation, confirming the diagnosis of CACS. CACS is an elusive diagnosis that should be considered in patients where other causes of abdominal pain and weight loss have been ruled out. The disease can present with the classic triad of post-prandial abdominal pain, weight loss, and an abdominal bruit. Imaging modalities including mesenteric vascular duplex, computed tomography abdominal angiography, magnetic resonance angiography and celiac artery angiography can help make the diagnosis. Treatment involves surgical decompression via division of the median arcuate ligament, with most patients experiencing significant and long-lasting relief from their symptoms.