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Celiac artery stenosis from compression by median arcuate ligament of diaphragm

MedGen UID:
349361
•Concept ID:
C1861783
•
Disease or Syndrome
Synonym: Celiac artery compression
SNOMED CT: CACS - Celiac axis compression syndrome (9250002); Celiac axis compression syndrome (9250002); Celiac artery compression syndrome (9250002); Celiac axis syndrome (9250002); Marable's syndrome (9250002); Median arcuate ligament syndrome (9250002)
 
HPO: HP:0012327
Monarch Initiative: MONDO:0004914
OMIM®: 116870

Definition

Compression of the celiac artery. [from HPO]

Clinical features

From HPO
Abdominal pain
MedGen UID:
7803
•Concept ID:
C0000737
•
Sign or Symptom
An unpleasant sensation characterized by physical discomfort (such as pricking, throbbing, or aching) and perceived to originate in the abdomen.
See: Feature record | Search on this feature
Celiac artery stenosis from compression by median arcuate ligament of diaphragm
MedGen UID:
349361
•Concept ID:
C1861783
•
Disease or Syndrome
Compression of the celiac artery.
See: Feature record | Search on this feature
Show allHide all

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCeliac artery stenosis from compression by median arcuate ligament of diaphragm

Conditions with this feature

Celiac artery stenosis from compression by median arcuate ligament of diaphragm
MedGen UID:
349361
•Concept ID:
C1861783
•
Disease or Syndrome
Compression of the celiac artery.
See: Condition Record
Celiac artery stenosis from compression by median arcuate ligament of diaphragm

Professional guidelines

PubMed

Clinical management of chronic mesenteric ischemia.
van Dijk LJ, van Noord D, de Vries AC, Kolkman JJ, Geelkerken RH, Verhagen HJ, Moelker A, Bruno MJ
United European Gastroenterol J 2019 Mar;7(2):179-188. Epub 2018 Dec 4 doi: 10.1177/2050640618817698. PMID: 31080602Free PMC Article
ACR Appropriateness Criteria(®) Radiologic Management of Mesenteric Ischemia.
Expert Panel on Interventional Radiology:, Fidelman N, AbuRahma AF, Cash BD, Kapoor BS, Knuttinen MG, Minocha J, Rochon PJ, Shaw CM, Ray CE Jr, Lorenz JM
J Am Coll Radiol 2017 May;14(5S):S266-S271. doi: 10.1016/j.jacr.2017.02.014. PMID: 28473083
The presentation and management of aneurysms of the pancreaticoduodenal arcade.
Corey MR, Ergul EA, Cambria RP, Patel VI, Lancaster RT, Kwolek CJ, Conrad MF
J Vasc Surg 2016 Dec;64(6):1734-1740. doi: 10.1016/j.jvs.2016.05.067. PMID: 27871496

Recent clinical studies

Etiology

Median Arcuate Ligament Syndrome: Comparing the Safety of Open and Laparoscopic Management in a Large Cohort.
Alnahhal KI, Tedesco A, Khan ZZ, Irshad A, Salehi P
Ann Vasc Surg 2023 Oct;96:215-222. Epub 2023 Apr 28 doi: 10.1016/j.avsg.2023.04.021. PMID: 37120073
Median arcuate ligament syndrome.
Goodall R, Langridge B, Onida S, Ellis M, Lane T, Davies AH
J Vasc Surg 2020 Jun;71(6):2170-2176. Epub 2019 Dec 25 doi: 10.1016/j.jvs.2019.11.012. PMID: 31882314
Nonatherosclerotic Abdominal Vasculopathies.
Skeik N, Hyde JR, Olson SL, Thaler CM, Abuatiyeh W, Ahmed AK, Lyon DR, Witt DR, Garberich R, Sullivan T
Ann Vasc Surg 2019 Oct;60:128-146. Epub 2019 Jun 12 doi: 10.1016/j.avsg.2019.04.004. PMID: 31200053
Clinical management of chronic mesenteric ischemia.
van Dijk LJ, van Noord D, de Vries AC, Kolkman JJ, Geelkerken RH, Verhagen HJ, Moelker A, Bruno MJ
United European Gastroenterol J 2019 Mar;7(2):179-188. Epub 2018 Dec 4 doi: 10.1177/2050640618817698. PMID: 31080602Free PMC Article
Vascular compression syndromes.
Czihal M, Banafsche R, Hoffmann U, Koeppel T
Vasa 2015 Nov;44(6):419-34. doi: 10.1024/0301-1526/a000465. PMID: 26515219

Diagnosis

Median arcuate ligament syndrome.
Goodall R, Langridge B, Onida S, Ellis M, Lane T, Davies AH
J Vasc Surg 2020 Jun;71(6):2170-2176. Epub 2019 Dec 25 doi: 10.1016/j.jvs.2019.11.012. PMID: 31882314
Clinical management of chronic mesenteric ischemia.
van Dijk LJ, van Noord D, de Vries AC, Kolkman JJ, Geelkerken RH, Verhagen HJ, Moelker A, Bruno MJ
United European Gastroenterol J 2019 Mar;7(2):179-188. Epub 2018 Dec 4 doi: 10.1177/2050640618817698. PMID: 31080602Free PMC Article
Median Arcuate Ligament Syndrome-Review of This Rare Disease.
Kim EN, Lamb K, Relles D, Moudgill N, DiMuzio PJ, Eisenberg JA
JAMA Surg 2016 May 1;151(5):471-7. doi: 10.1001/jamasurg.2016.0002. PMID: 26934394
Vascular compression syndromes.
Czihal M, Banafsche R, Hoffmann U, Koeppel T
Vasa 2015 Nov;44(6):419-34. doi: 10.1024/0301-1526/a000465. PMID: 26515219
Mesenteric ischemia.
Bobadilla JL
Surg Clin North Am 2013 Aug;93(4):925-40, ix. Epub 2013 May 16 doi: 10.1016/j.suc.2013.04.002. PMID: 23885938

Therapy

Median Arcuate Ligament Syndrome: Comparing the Safety of Open and Laparoscopic Management in a Large Cohort.
Alnahhal KI, Tedesco A, Khan ZZ, Irshad A, Salehi P
Ann Vasc Surg 2023 Oct;96:215-222. Epub 2023 Apr 28 doi: 10.1016/j.avsg.2023.04.021. PMID: 37120073
Median arcuate ligament syndrome.
Goodall R, Langridge B, Onida S, Ellis M, Lane T, Davies AH
J Vasc Surg 2020 Jun;71(6):2170-2176. Epub 2019 Dec 25 doi: 10.1016/j.jvs.2019.11.012. PMID: 31882314
Nonatherosclerotic Abdominal Vasculopathies.
Skeik N, Hyde JR, Olson SL, Thaler CM, Abuatiyeh W, Ahmed AK, Lyon DR, Witt DR, Garberich R, Sullivan T
Ann Vasc Surg 2019 Oct;60:128-146. Epub 2019 Jun 12 doi: 10.1016/j.avsg.2019.04.004. PMID: 31200053
Clinical management of chronic mesenteric ischemia.
van Dijk LJ, van Noord D, de Vries AC, Kolkman JJ, Geelkerken RH, Verhagen HJ, Moelker A, Bruno MJ
United European Gastroenterol J 2019 Mar;7(2):179-188. Epub 2018 Dec 4 doi: 10.1177/2050640618817698. PMID: 31080602Free PMC Article
Median arcuate ligament compression of the mesenteric vasculature.
Tracci MC
Tech Vasc Interv Radiol 2015 Mar;18(1):43-50. Epub 2014 Dec 29 doi: 10.1053/j.tvir.2014.12.007. PMID: 25814203

Prognosis

Median arcuate ligament syndrome - Current state of management.
Skelly CL, Mak GZ
Semin Pediatr Surg 2021 Dec;30(6):151129. Epub 2021 Nov 16 doi: 10.1016/j.sempedsurg.2021.151129. PMID: 34930594
Nonatherosclerotic Abdominal Vasculopathies.
Skeik N, Hyde JR, Olson SL, Thaler CM, Abuatiyeh W, Ahmed AK, Lyon DR, Witt DR, Garberich R, Sullivan T
Ann Vasc Surg 2019 Oct;60:128-146. Epub 2019 Jun 12 doi: 10.1016/j.avsg.2019.04.004. PMID: 31200053
Vascular compression syndromes.
Czihal M, Banafsche R, Hoffmann U, Koeppel T
Vasa 2015 Nov;44(6):419-34. doi: 10.1024/0301-1526/a000465. PMID: 26515219
Treatment of celiac artery compression syndrome: does it really exist?
Gloviczki P, Duncan AA
Perspect Vasc Surg Endovasc Ther 2007 Sep;19(3):259-63. doi: 10.1177/1531003507305263. PMID: 17911551
Celiac compression syndrome.
Ghosn PB, Rabbat AG, Trudel J, D'Amico P, Lecours R, Trudel J
Can J Surg 1982 Jul;25(4):377-9. PMID: 7093835

Clinical prediction guides

Median Arcuate Ligament Syndrome is Effectively Relieved with Minimally Invasive Surgery.
Birkhold M, Khalifeh A, Nagarsheth K, Kavic SM
JSLS 2022 Oct-Dec;26(4) doi: 10.4293/JSLS.2022.00067. PMID: 36721735Free PMC Article
Median arcuate ligament syndrome - Current state of management.
Skelly CL, Mak GZ
Semin Pediatr Surg 2021 Dec;30(6):151129. Epub 2021 Nov 16 doi: 10.1016/j.sempedsurg.2021.151129. PMID: 34930594
Pediatric Chronic Abdominal Pain and Median Arcuate Ligament Syndrome: A Review and Psychosocial Comparison.
Mak GZ, Lucchetti AR, Drossos T, Fitzsimmons-Craft EE, Accurso EC, Stiles-Shields C, Newman EA, Skelly CL
Pediatr Ann 2016 Jul 1;45(7):e257-64. doi: 10.3928/00904481-20160613-01. PMID: 27403674
Vascular compression syndromes.
Czihal M, Banafsche R, Hoffmann U, Koeppel T
Vasa 2015 Nov;44(6):419-34. doi: 10.1024/0301-1526/a000465. PMID: 26515219
Treatment of celiac artery compression syndrome: does it really exist?
Gloviczki P, Duncan AA
Perspect Vasc Surg Endovasc Ther 2007 Sep;19(3):259-63. doi: 10.1177/1531003507305263. PMID: 17911551

Recent systematic reviews

Surgical therapy of celiac axis and superior mesenteric artery syndrome.
Jonas JP, Rössler F, Ghafoor S, Kobe A, Pfammatter T, Schlag C, Gutschow CA, Petrowsky H, Müller PC, Oberkofler CE
Langenbecks Arch Surg 2023 Jan 24;408(1):59. doi: 10.1007/s00423-023-02803-w. PMID: 36690823Free PMC Article
Laparoscopic treatment of median arcuate ligament syndrome: a systematic review.
San Norberto EM, Romero A, Fidalgo-Domingos LA, García-Saiz I, Taylor J, Vaquero C
Int Angiol 2019 Dec;38(6):474-483. Epub 2019 Sep 30 doi: 10.23736/S0392-9590.19.04161-0. PMID: 31580040

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