An active man in his early 80s presented to the hospital with a 6-month history of cramping epigastric abdominal pain that occurred 30 minutes after eating solid food and was associated with nausea and vomiting. His medical history was significant for atrial fibrillation and a history of an open abdominal aortic aneurysm repair. Review of systems revealed a 9-kg weight loss. A recent esophagogastroduodenoscopy was unremarkable, but prior colonoscopy showed focal colitis that was treated with antibiotics. Physical examination revealed he was in sinus rhythm. He had no abdominal tenderness but had an abdominal bruit. Laboratory examination results were unremarkable. Mesenteric duplex ultrasonography showed the following: celiac artery velocity of 394 cm/s on inspiration (Figure, A) and 608 cm/s on expiration (Figure, B), superior mesenteric artery (SMA) velocity of 380 cm/s, and an occluded inferior mesenteric artery. Subsequent angiography corroborated findings.
Mesenteric duplex ultrasonography showing celiac artery velocities with inspiration (A) and expiration (B).
Acute mesenteric ischemia
Median arcuate ligament syndrome
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D. Median arcuate ligament syndrome
The patient was an elderly, but independent man who had postprandial abdominal pain leading to weight loss. He had a history of an open abdominal aortic aneurysm repair and was dependent on his celiac artery and SMA for mesenteric perfusion. Imaging revealed atherosclerotic disease of the SMA with near occlusion of the celiac artery on full expiration. These findings are suggestive of median arcuate ligament (MAL) syndrome.
Median arcuate ligament syndrome, also known as celiac artery compression syndrome or Dunbar syndrome, is a clinical entity caused by extrinsic compression of the celiac axis by the fibers of the diaphragmatic crura, leading to postprandial abdominal pain, nausea and/or vomiting, and weight loss.1 It has a female-to-male ratio of approximately 4:1,2 and its diagnosis is often one of exclusion and controversy.
The anatomic pathogenesis is thought to arise from an abnormally low insertion of the diaphragm and/or high origin of the celiac axis.1,3 Although the degree of compression varies by individual, inspiration causes inferior displacement of the aorta and anterior displacement of the MAL, causing release of the celiac axis and thereby perfusion into the celiac artery.4 Histologically, repetitive compression of the celiac artery by the MAL causes fibrotic changes of all layers of the vessel wall leading to neointimal hyperplasia and medial and adventitial hyperplastic fibrosis.5
The diagnosis of MAL syndrome is often one of exclusion, although the relief of symptoms after treatment has been argued to validate its true entity. When MAL syndrome is suspected, mesenteric duplex with measurements on inspiration and expiration is the preferred initial imaging modality. Findings typically include elevated velocities of the celiac artery on expiration, which normalize on inspiration. Other findings include abnormal origins of the celiac artery, reversal of flow of the hepatic artery, and normalization of the celiac artery when standing erect.6 Findings are often confirmed with computed tomography angiography/magnetic resonance angiography and/or digital subtraction angiography, which can show dynamic variability of celiac artery flow according to breathing cycle.
The treatment for MAL syndrome is ligament release through either a laparoscopic or open approach. A minority of patients require celiac ganglionectomy and/or concomitant celiac artery revascularization, but overall 85% of patients report immediate postoperative relief.7 Some reports demonstrate that patients who undergo ligament release with concomitant revascularization have lower rates of recurrence than with decompression alone.8 In general, endovascular interventions alone have not demonstrated long-term efficacy.9,10
Because of the concomitant atherosclerotic nature of the SMA, the patient underwent an angioplasty with improvement in his postprandial abdominal pain. His age, comorbidities, and previous abdominal surgery made him a poor candidate for laparoscopic MAL release. The patient chose to have close follow-up and modify his diet to multiple small meals per day instead of open surgery.
Corresponding Author: Melina R. Kibbe, MD, Division of Vascular Surgery, 676 N St Clair, Ste 650, Chicago, IL 60611 (firstname.lastname@example.org).
Published Online: January 20, 2016. doi:10.1001/jamasurg.2015.4715.
Conflict of Interest Disclosures: None reported.
Disclaimer: Dr Kibbe is the Editor of JAMA Surgery but was not involved in the editorial review or the decision to accept the manuscript for publication.
Yoon DY, Nemcek AA, Kibbe MR. Postprandial Abdominal Pain. JAMA Surg. 2016;151(3):287-288. doi:10.1001/jamasurg.2015.4715