Celiac artery compression: Dunbar syndrome

Abstract

Celiac artery compression syndrome, also referred to as median arcuate ligament syndrome, celiac axis syndrome or Dunbar syndrome is a rare disorder consequent to extrinsic compression of the celiac trunk by the median arcuate ligament. Doppler ultrasound, multi-slice computed tomography angiography, magnetic resonance angiography, or invasive selective angiography can identify stenosis of the initial segment of the celiac artery and confirm diagnosis. Treatment options include open surgical or videolaparoscopic section of the median arcuate ligament and the fibers of the celiac plexus, or percutaneous transluminal angioplasty via an endovascular approach. We report herein an interesting case of a 38-year-old woman diagnosed with this rare condition and successfully treated with the surgical strategy.

An Unusual Presentation of Median Arcuate Ligament Syndrome

“…Demographic reports vary but the condition typically affects women aged 20-60 years of age with thin body habitus. The female to male ratio of MALS is reported to be 3:1 [1]. The reason for this predominance in women remains unclear but may be due to a predilection for the celiac artery to emerge more cephalad in women as compared to men [8].…”

“…Due to its benign nature, nonspecific symptomatology, and significant overlap with other conditions, patients will typically present with a history of an extensive workup to rule out other conditions. MALS is a diagnosis of exclusion, and oftentimes multiple investigations are required to rule out other pathologies such as esophagitis, cholelithiasis, pancreatitis, etc [1].…”

“…The pathophysiology of this condition remains to be elucidated. The cause is most likely multifactorial, combining the effects of celiac artery compression and subsequent foregut ischemia, with celiac plexus and/or celiac ganglion irritation and subsequent splanchnic vasoconstriction to cause the presenting symptoms [ 1 , 6 , 7 , 9 , 10 ]. This multifactorial etiology appears to be supported by a study demonstrating that correction of celiac artery compression alone results in 53% of patients being asymptomatic on long-term follow-up, whereas combined release and revascularization results in 79% of patients being asymptomatic on long-term follow-up [ 11 ].…”

“…The median arcuate ligament (MAL) is located at the T12-L1 level and is formed by the bridging fibers of the left and right crura of the diaphragm. These fibers border the aortic hiatus anteriorly, with the aorta, azygos vein, and thoracic duct passing through the aperture [ 1 - 3 ]. Median arcuate ligament syndrome (MALS) is a condition that occurs when fibers of the MAL forming the aortic hiatus compress the celiac trunk, its branches, or other neurogenic structures [ 4 , 5 ].…”

Atypical Presentation of Median Arcuate Ligament Syndrome in the Emergency Department

“…Treatment historically consists of surgical techniques, although due to the rarity of the syndrome there is no single accepted treatment algorithm and options should be considered in the context of an individual patient’s age and severity of symptoms. General surgeons may perform minimally invasive techniques such as laparoscopic or robotic median arcuate ligament release, while vascular surgeons may perform percutaneous transluminal angioplasty, primary reanastomosis, or celiac artery bypass grafting in refractory cases 6,13–17. IR may also perform angioplasty with stenting 14,18.…”