U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Superior mesenteric artery syndrome

MedGen UID:
21404
•Concept ID:
C0038828
•
Disease or Syndrome
Synonyms: Arteriomesenteric duodenal compression syndrome; Cast syndrome; Vascular compression of the duodenum; Wilkie syndrome
SNOMED CT: SMAS - Superior mesenteric artery syndrome (197006009); Superior mesenteric artery syndrome (197006009)
 
Monarch Initiative: MONDO:0002687
Orphanet: ORPHA622099

Definition

A very rare syndrome characterized by compression of the third portion of the duodenum against the aorta. The compression is caused by the superior mesenteric artery. It results in complete or partial duodenal obstruction. Signs and symptoms include nausea, vomiting, abdominal pain and distention, failure to gain weight, and weight loss. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSuperior mesenteric artery syndrome

Professional guidelines

PubMed

Successful treatment of superior mesenteric artery syndrome by endoscopic ultrasound-guided gastrojejunostomy.
Bronswijk M, Fransen L, Vanella G, Hiele M, van der Merwe S
Endoscopy 2021 Feb;53(2):204-205. Epub 2020 Jun 19 doi: 10.1055/a-1190-3228. PMID: 32559775
SMA syndrome: management perspective with laparoscopic duodenojejunostomy and long-term results.
Jain N, Chopde A, Soni B, Sharma B, Saini S, Mishra S, Mishra S, Gupta R, Bhojwani R
Surg Endosc 2021 May;35(5):2029-2038. Epub 2020 Apr 27 doi: 10.1007/s00464-020-07598-1. PMID: 32342220
Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines.
Ananthan K, Onida S, Davies AH
Eur J Vasc Endovasc Surg 2017 Jun;53(6):886-894. Epub 2017 Mar 27 doi: 10.1016/j.ejvs.2017.02.015. PMID: 28356209

Recent clinical studies

Etiology

The Superior Mesenteric Artery: From Syndrome in the Young to Vascular Atherosclerosis in the Old.
Beita AKV, Whayne TF
Cardiovasc Hematol Agents Med Chem 2019;17(2):74-81. doi: 10.2174/1871525717666190920100518. PMID: 31538906
Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines.
Ananthan K, Onida S, Davies AH
Eur J Vasc Endovasc Surg 2017 Jun;53(6):886-894. Epub 2017 Mar 27 doi: 10.1016/j.ejvs.2017.02.015. PMID: 28356209
Aortoduodenal syndrome.
Takagi H, Watanabe T, Umemoto T; ALICE(All-Literature Investigation of Cardiovascular Evidence) GROUP
Int Angiol 2015 Oct;34(5):454-8. Epub 2014 Sep 12 PMID: 25216355
Superior mesenteric artery syndrome: diagnosis and treatment strategies.
Merrett ND, Wilson RB, Cosman P, Biankin AV
J Gastrointest Surg 2009 Feb;13(2):287-92. Epub 2008 Sep 23 doi: 10.1007/s11605-008-0695-4. PMID: 18810558
Superior mesenteric artery syndrome.
Ahmed AR, Taylor I
Postgrad Med J 1997 Dec;73(866):776-8. doi: 10.1136/pgmj.73.866.776. PMID: 9497945Free PMC Article

Diagnosis

Wilkie's syndrome.
Farina R, Foti PV, Cocuzza G, Costanzo V, Costanzo G, Conti A, Torcitto A, Pennisi M
J Ultrasound 2017 Dec;20(4):339-342. Epub 2017 Aug 3 doi: 10.1007/s40477-017-0257-2. PMID: 29204239Free PMC Article
Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines.
Ananthan K, Onida S, Davies AH
Eur J Vasc Endovasc Surg 2017 Jun;53(6):886-894. Epub 2017 Mar 27 doi: 10.1016/j.ejvs.2017.02.015. PMID: 28356209
Aortoduodenal syndrome.
Takagi H, Watanabe T, Umemoto T; ALICE(All-Literature Investigation of Cardiovascular Evidence) GROUP
Int Angiol 2015 Oct;34(5):454-8. Epub 2014 Sep 12 PMID: 25216355
Entrapment syndromes.
Noorani A, Walsh SR, Cooper DG, Varty K
Eur J Vasc Endovasc Surg 2009 Feb;37(2):213-20. Epub 2008 Dec 1 doi: 10.1016/j.ejvs.2008.10.019. PMID: 19046647
Superior mesenteric artery syndrome: diagnosis and treatment strategies.
Merrett ND, Wilson RB, Cosman P, Biankin AV
J Gastrointest Surg 2009 Feb;13(2):287-92. Epub 2008 Sep 23 doi: 10.1007/s11605-008-0695-4. PMID: 18810558

Therapy

Laparoscopic Surgery for Superior Mesenteric Artery Syndrome.
Lu SB, Guo YQ, Chen RY, Zhang YF
J Invest Surg 2024 Dec;37(1):2387524. Epub 2024 Aug 27 doi: 10.1080/08941939.2024.2387524. PMID: 39191411
Superior mesenteric artery syndrome and disseminated tuberculosis: "Double troubleˮ.
Bhatkar D, Mishra G, Gour S, Munje R
Indian J Tuberc 2022 Oct;69(4):702-705. Epub 2021 Aug 19 doi: 10.1016/j.ijtb.2021.08.019. PMID: 36460412
Superior Mesenteric Artery Syndrome Following Scoliosis Surgery: A Systematic Review of Case Reports.
Fan Y, Cai M, Wang J, Xia L
Ann Vasc Surg 2021 Oct;76:514-535. Epub 2021 Apr 24 doi: 10.1016/j.avsg.2021.03.032. PMID: 33905850
Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines.
Ananthan K, Onida S, Davies AH
Eur J Vasc Endovasc Surg 2017 Jun;53(6):886-894. Epub 2017 Mar 27 doi: 10.1016/j.ejvs.2017.02.015. PMID: 28356209
Scoliosis, superior mesenteric artery syndrome, and adolescents.
Schwartz A
Orthop Nurs 2007 Jan-Feb;26(1):19-24; quiz 25-6. doi: 10.1097/00006416-200701000-00007. PMID: 17273103

Prognosis

Adolescent with Superior Mesenteric Artery Syndrome.
Ren PLJ, Gupta A
J Radiol Case Rep 2020 Mar;14(3):14-23. Epub 2020 Mar 31 doi: 10.3941/jrcr.v14i3.3830. PMID: 33082917Free PMC Article
Wilkie's syndrome.
Farina R, Foti PV, Cocuzza G, Costanzo V, Costanzo G, Conti A, Torcitto A, Pennisi M
J Ultrasound 2017 Dec;20(4):339-342. Epub 2017 Aug 3 doi: 10.1007/s40477-017-0257-2. PMID: 29204239Free PMC Article
Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines.
Ananthan K, Onida S, Davies AH
Eur J Vasc Endovasc Surg 2017 Jun;53(6):886-894. Epub 2017 Mar 27 doi: 10.1016/j.ejvs.2017.02.015. PMID: 28356209
Laparoscopic duodenojejunostomy for superior mesenteric artery syndrome: intermediate follow-up results and a review of the literature.
Chang J, Boules M, Rodriguez J, Walsh M, Rosenthal R, Kroh M
Surg Endosc 2017 Mar;31(3):1180-1185. Epub 2016 Jul 12 doi: 10.1007/s00464-016-5088-2. PMID: 27405482
Familial superior mesenteric artery syndrome.
Martins AR, Cunha JF, Patrício J, Caravana J
BMJ Case Rep 2016 Apr 28;2016 doi: 10.1136/bcr-2016-214784. PMID: 27126095Free PMC Article

Clinical prediction guides

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
Superior mesenteric artery syndrome: a radiographic review.
Warncke ES, Gursahaney DL, Mascolo M, Dee E
Abdom Radiol (NY) 2019 Sep;44(9):3188-3194. doi: 10.1007/s00261-019-02066-4. PMID: 31127323
Superior Mesenteric Artery Syndrome May Be Overlooked in Women with Functional Dyspepsia.
Kawanishi K, Shojima K, Nishimoto M, Abe H, Kakimoto T, Yasuda Y, Hara T, Kato J
Intern Med 2017 Oct 1;56(19):2549-2554. Epub 2017 Sep 6 doi: 10.2169/internalmedicine.8647-16. PMID: 28883239Free PMC Article
Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines.
Ananthan K, Onida S, Davies AH
Eur J Vasc Endovasc Surg 2017 Jun;53(6):886-894. Epub 2017 Mar 27 doi: 10.1016/j.ejvs.2017.02.015. PMID: 28356209
Wilkie's syndrome.
Nana AM, Closset J, Muls V, Kouame J, Jeanmart J, van Gossum A, Gelin M, El Nakadi I
Surg Endosc 2003 Apr;17(4):659. Epub 2003 Feb 10 doi: 10.1007/s00464-002-4252-z. PMID: 12574933

Recent systematic reviews

Superior mesenteric artery syndrome following spine surgery in idiopathic adolescent scoliosis: a systematic review.
Pappalardo G, Pola E, Bertini FA, Nasto LA, Eschweiler J, Schäfer L, Migliorini F
Eur J Med Res 2024 Aug 8;29(1):410. doi: 10.1186/s40001-024-02002-3. PMID: 39118170Free PMC Article
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
Superior Mesenteric Artery Syndrome Following Scoliosis Surgery: A Systematic Review of Case Reports.
Fan Y, Cai M, Wang J, Xia L
Ann Vasc Surg 2021 Oct;76:514-535. Epub 2021 Apr 24 doi: 10.1016/j.avsg.2021.03.032. PMID: 33905850
Gastrointestinal complications associated with anorexia nervosa: A systematic review.
Norris ML, Harrison ME, Isserlin L, Robinson A, Feder S, Sampson M
Int J Eat Disord 2016 Mar;49(3):216-37. Epub 2015 Sep 26 doi: 10.1002/eat.22462. PMID: 26407541
Laparoscopic duodenojejunostomy for superior mesenteric artery syndrome.
Munene G, Knab M, Parag B
Am Surg 2010 Mar;76(3):321-4. PMID: 20349665

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...