Median arcuate ligament syndrome. An unfrequent cause of abdominal pain.
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1 Median arcuate ligament syndrome. An unfrequent cause of abdominal pain. Poster No.: C-2093 Congress: ECR 2015 Type: Educational Exhibit Authors: L. Caminero, M. L. Rozas, M. E. Banegas Illescas, J. A. Villanueva, C. López Menéndez, A. Pinar; Ciudad Real/ES Keywords: Ischemia / Infarction, Computer Applications-3D, CT-Angiography, Vascular, Abdomen DOI: /ecr2015/C-2093 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 10
2 Learning objectives To review the medium arcuate ligament syndrome as an infrequent cause of abdominal pain by showing some of the cases seen in our department. Background Anatomy Medium arcuate ligament is a fibrous arch that bonds the diaphragmatic crura at each side of the aortic hiatus. On the right side diaphragmatic crura origins from the anterior surface of the vertebral bodies from L1 to L4 and on the left side from the first two of three lumbar bodies. The crura also have insertions in the intervertebral disks and in the anterior longitudinal ligament. The medium arcuate ligament in normal conditions goes over the aorta at the height of the first to lumbar vertebral bodies, superior to the celiac axis origin. In 10 to 24% of the population it can have a more caudal way and crosses immediately over the proximal portion of the celiac axis (Fig. 1 on page 3). In a small percentage of the patients, this variant can cause significant hemodynamic compromise. Medium arcuate ligament syndrome (MALS) Also named extrinsic compression syndrome or Dunbar syndrome, it has a 2/ incidence in patients with recurring superior abdominal pain. The definition of this syndrome agglutinates also a clinical and radiological diagnosis. It usually happens in young patients from 20 to 40 years of age and is more frequent in women and has a clinical presentation of epigastric pain (it can be postprandial) and weight loss. In physical examination we can sometimes listen a murmur that changes with breathing in the medium epigastric region. These symptoms are because of a compromised flow caused by the location of the previously mentioned ligament. Treatment Surgical treatment is controversial because many authors doubt that the symptoms are caused by the isolated narrowing of the celiac axis because there is an important Page 2 of 10
3 percentage of patients that have the anomaly but do not have the symptoms. Treatment consists of the laparoscopic division of the medium arcuate ligament, liberating the celiac axis and performing a revascularization procedure if needed. It is more probable that this treatment will successful in patients from 40 to 60 years of age with postprandial pain, poststenotic dilatation and collateral vessels. Images for this section: Fig. 1: On the left side image, sagittal view, we can see the typical anatomy of the medium arcuate ligament following an anterior path to the aorta and over the celiac axis. On the right side image we show the anatomic variant in which the ligament crosses the proximal portion of the celiac axis, that in some patients cause the syndrome. Page 3 of 10
4 Findings and procedure details Diagnosis can be made by Doppler ultrasound ( Fig. 2 on page 4 ) with measurement of the blood speed in the celiac axis at the end of inspiration and exhalation. If we see a peak speed higher than 200 cm/s the patient has a significant flow compromise. The release of multidetector CT scan allow us to perform angiographic studies that will give us valuable information about local anatomy, hemodynamic information, and the presence of concomitant calcifications. The sagittal plane ( Fig. 3 on page 5 ) is the optimum to see the proximal portion of the celiac axis. We must take into account that the CT study is performed in maximum inspiration that is when there is a the smallest compression of the ligament over the celiac axis so we should consider to perform the study with maximum exhalation. The criteria to distinguish this entity from arteriosclerotic stenosis of the celiac axis are the observation of the hooked appearance owing to the fold ( Fig. 4 on page 6, Fig. 5 on page 7 and Fig. 6 on page 8 ) (specially in the proximal portion of the celiac axis) and to the indentation of the adjacent aortic edge. AngioMRI can be useful in children and teenagers to avoid the exposition to ionizing radiation if doppler ultrasound is inconclusive. Images for this section: Page 4 of 10
5 Fig. 2 Page 5 of 10
6 Fig. 3: Abdominal angioct. Sagittal view: in this case the image corresponds with an incidental finding of the insertion variant of the previously mentioned ligament that do not cause symptoms in the patient. Page 6 of 10
7 Fig. 4: Abdominal angioct Sagittal view: in this case the image corresponds with another incidental finding of the variant previously mentioned in a patient with abdominal aorta aneurysm with an excentric thrombus. Page 7 of 10
8 Fig. 5: Abdominal angioct Sagittal view. In this case the patient is a 45 years old woman with a two years long postprandial pain without a clear cause. We can see the typical indentation that causes the low course of the medium arcuate ligament. Page 8 of 10
9 Fig. 6: Abdominal angioct Sagittal view: 70 years old man that came to the hospital because of two years long intestinal angina without a clear cause. We can see the indentation caused by the medium arcuate ligament crossing the proximal part of the celiac axis. It also associates a poststenotic dilatation and celiac axis dissection preserving its branches. Page 9 of 10
10 Conclusion Though there is no consensus over if the MALS is a real entity, the improvement of many patients after adequate treatment makes the radiologist to take this diagnosis into account when confronted to cases of abdominal pain without a clear cause. Personal information References -Karen M. Horton, MD, Mark A. Talamini, MD, Elliot K. Fishman, MD. Median Arcuate Ligament Syndrome: Evaluation with CT Angiography. RadioGraphics 2005; 25: Ramit Lamba, Dawn T. Tanner, Simran Sekhon, John P. McGahan, Michael T. Corwin, Chandana G. Lall. Multidetector CT of Vascular Compression Syndromes in the Abdomen and Pelvis. RadioGraphics 2014; 34: Patten RM, Coldwell DM, Ben-Menachem Y.Ligamentous compression of the celiac axis: CT findings in five patients. AJR Am J Roentgenol 1991;156: Roayaie S, Jossart G, Gitlitz D, Lamparello P,Hollier L, Gagner M. Laparoscopic release of celiac artery compression syndrome facilitated by laparoscopic ultrasound scanning to confirm restoration of flow. J Vasc Surg 2000;32: Soman S, Sudhakar SV, Keshava SN. Celiac axis compression by median arcuate ligament on computed tomography among asymptomatic persons. Indian J Gastroenterol 2010;29(3): Erden A, Yurdakul M, Cumhur T. Marked increase in flow velocities during deep expiration: a duplex Doppler sign of celiac artery compressionsyndrome. Cardiovasc Intervent Radiol 1999;22(4): Page 10 of 10
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