Celiac artery compression syndrome is also known as celiac axis syndrome, median arcuate ligament syndrome and Dunbar syndrome. It is characterized by upper abdominal angina secondary to compression of the celiac trunk by the diaphragmatic crurae.
The median arcuate ligament is the fibrous arch that unites the diaphragmatic crura on either side of the aortic hiatus. In some people, this ligament has a low insertion point and may distort and compress the celiac trunk. Although commonly asymptomatic, it may cause ischemic-type epigastric pain. The typical age of presentation is 20 to 40 year old.
- mostly asymptomatic
- chronic abdominal pain, especially postprandial
- pain relieved in standing position and aggravated by supine position
- weight loss
Doppler ultrasound can be a useful noninvasive diagnostic tool. In young adults, a peak systolic velocity over the compressed segment of the celiac artery of greater than 200 cm/s in the mid position between inspiration and expiration has a reported sensitivity and specificity of 75% and 89%, respectively, in detecting stenosis of 70% or greater 4,6.
CT angiography / angiography
CT angiography and conventional angiography are considered to be the gold standard imaging modalities. These modalities demonstrate focal stenosis that has a characteristic hooked appearance due to the indentation of the celiac trunk on its superior surface. This characteristic hooked appearance of the stenosis, as well as the younger presenting age of the patient, distinguishes this syndrome from the main differential diagnosis of atherosclerotic disease.
It is important to note that superior indentation of the celiac trunk may be seen in normal people if imaging is acquired in expiration. Therefore imaging for accurate diagnosis should ideally be performed in the end-inspiratory phase 4. Furthermore, imaging findings must also be correlated with the clinical history.
Additional features that may be appreciated include post-stenotic dilatation, prominent collaterals, such as the gastroduodenal and common hepatic arteries, and thickening of the median arcuate ligament. A thickness of the median arcuate ligament of greater than 4 mm is considered abnormal 4.
Treatment and prognosis
Symptomatic patients are treated with surgical decompression. This is usually performed laparoscopically by dividing the median arcuate ligament.
- 1. Horton KM, Talamini MA, Fishman EK. Median arcuate ligament syndrome: evaluation with CT angiography. Radiographics. 25 (5): 1177-82. doi:10.1148/rg.255055001 - Pubmed citation
- 2. Muqeetadnan M, Amer S, Rahman A et-al. Celiac artery compression syndrome. Case Rep Gastrointest Med. 2013;2013: 934052. doi:10.1155/2013/934052 - Free text at pubmed - Pubmed citation
- 3. Karen M. Horton, Mark A. Talamini, Elliot K. Fishman. Median Arcuate Ligament Syndrome: Evaluation with CT Angiography. (2005) RadioGraphics. 25 (5): 1177-82. doi:10.1148/rg.255055001 - Pubmed
- 4. Jeffrey Kah Keng Fong, Angeline Choo Choo Poh, Andrew Gee Seng Tan, Ranu Taneja. Imaging Findings and Clinical Features of Abdominal Vascular Compression Syndromes. (2014) American Journal of Roentgenology. 203 (1): 29-36. doi:10.2214/AJR.13.11598 - Pubmed
- 5. Sempere OrtegaCayetano, Gallego RiveraIgnacio, ShahinMahmoud. Gastric ischaemia as an unusual presentation of median arcuate ligament compression syndrome. (2016) BJR|case reports.
- 6. Scholbach T. Celiac artery compression syndrome in children, adolescents, and young adults: clinical and colour duplex sonographic features in a series of 59 cases. J Ultrasound Med 2006; 25:299–305. (2006) Original article