U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

ERDHEIM CYSTIC MEDIAL NECROSIS OF AORTA

MedGen UID:
97904
•Concept ID:
C0392775
•
Disease or Syndrome
Synonyms: Cystic medial necrosis of aorta; Erdheim Cystic medial necrosis of aorta
SNOMED CT: Medionecrosis of aorta (45894003); Medionecrosis aortae idiopathica cystica (45894003); Erdheim's cystic medial necrosis (45894003)
 
OMIM®: 607086

Definition

An idiopathic disorder characterized by the degeneration of the aortic media tissue and formation of cyst-like spaces. It may lead to aortic dissection. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVERDHEIM CYSTIC MEDIAL NECROSIS OF AORTA

Recent clinical studies

Etiology

Surgical Outcome and Histological Differences between Individuals with TGFBR1 and TGFBR2 Mutations in Loeys-Dietz Syndrome.
Seike Y, Matsuda H, Ishibashi-Ueda H, Morisaki H, Morisaki T, Minatoya K, Ogino H
Ann Thorac Cardiovasc Surg 2021 Feb 20;27(1):56-63. Epub 2021 Jan 6 doi: 10.5761/atcs.oa.20-00223. PMID: 33408307Free PMC Article
Multiple arterial thromboses due to cystic medial degeneration Erdheim-Gsell: A case report.
Jud P, Gary T, Hafner F, Tiesenhausen K, Ott T, Oswald WK, Brodmann M
Medicine (Baltimore) 2017 Nov;96(47):e8782. doi: 10.1097/MD.0000000000008782. PMID: 29381979Free PMC Article
Impact of connective tissue disease on the surgical outcomes of aortic dissection in patients with cystic medial necrosis.
Fujiyoshi T, Minatoya K, Ikeda Y, Ishibashi-Ueda H, Morisaki T, Morisaki H, Ogino H
J Cardiothorac Surg 2017 Nov 23;12(1):97. doi: 10.1186/s13019-017-0663-8. PMID: 29169363Free PMC Article
Clinicopathological study on penetrating atherosclerotic ulcers and aortic dissection: distinct pattern of development of initial event.
Wada H, Sakata N, Tashiro T
Heart Vessels 2016 Nov;31(11):1855-1861. Epub 2016 Feb 18 doi: 10.1007/s00380-016-0813-2. PMID: 26892532

Diagnosis

Histologic differences between the ascending and descending aortas in young adults with fibrillin-1 mutations.
Seike Y, Minatoya K, Matsuda H, Ishibashi-Ueda H, Morisaki H, Morisaki T, Kobayashi J
J Thorac Cardiovasc Surg 2020 Apr;159(4):1214-1220.e1. Epub 2019 Feb 15 doi: 10.1016/j.jtcvs.2019.01.126. PMID: 30905418
Multiple arterial thromboses due to cystic medial degeneration Erdheim-Gsell: A case report.
Jud P, Gary T, Hafner F, Tiesenhausen K, Ott T, Oswald WK, Brodmann M
Medicine (Baltimore) 2017 Nov;96(47):e8782. doi: 10.1097/MD.0000000000008782. PMID: 29381979Free PMC Article
Impact of connective tissue disease on the surgical outcomes of aortic dissection in patients with cystic medial necrosis.
Fujiyoshi T, Minatoya K, Ikeda Y, Ishibashi-Ueda H, Morisaki T, Morisaki H, Ogino H
J Cardiothorac Surg 2017 Nov 23;12(1):97. doi: 10.1186/s13019-017-0663-8. PMID: 29169363Free PMC Article
Kommerell's diverticulum in the current era: a comprehensive review.
Tanaka A, Milner R, Ota T
Gen Thorac Cardiovasc Surg 2015 May;63(5):245-59. Epub 2015 Jan 31 doi: 10.1007/s11748-015-0521-3. PMID: 25636900
Cystic medionecrosis of the carotid bifurcation as an uncommon cause of stroke.
Lesser K, Boltze C, Böttcher J, Lesser TG
Neurologist 2012 Nov;18(6):395-7. doi: 10.1097/NRL.0b013e31826a9974. PMID: 23114674

Therapy

Surgical Outcome and Histological Differences between Individuals with TGFBR1 and TGFBR2 Mutations in Loeys-Dietz Syndrome.
Seike Y, Matsuda H, Ishibashi-Ueda H, Morisaki H, Morisaki T, Minatoya K, Ogino H
Ann Thorac Cardiovasc Surg 2021 Feb 20;27(1):56-63. Epub 2021 Jan 6 doi: 10.5761/atcs.oa.20-00223. PMID: 33408307Free PMC Article
Bilateral axillary artery aneurysms after Bentall procedure in Marfan syndrome.
Haruki T, Ito H, Sakata K, Kobayashi Y
Asian Cardiovasc Thorac Ann 2015 Nov;23(9):1072-4. Epub 2014 Apr 14 doi: 10.1177/0218492314532278. PMID: 24732088

Prognosis

Impact of connective tissue disease on the surgical outcomes of aortic dissection in patients with cystic medial necrosis.
Fujiyoshi T, Minatoya K, Ikeda Y, Ishibashi-Ueda H, Morisaki T, Morisaki H, Ogino H
J Cardiothorac Surg 2017 Nov 23;12(1):97. doi: 10.1186/s13019-017-0663-8. PMID: 29169363Free PMC Article
Bilateral axillary artery aneurysms after Bentall procedure in Marfan syndrome.
Haruki T, Ito H, Sakata K, Kobayashi Y
Asian Cardiovasc Thorac Ann 2015 Nov;23(9):1072-4. Epub 2014 Apr 14 doi: 10.1177/0218492314532278. PMID: 24732088
Acute aortic dissection associated with cystic medial necrosis of unknown etiology.
Akutsu K, Kawamoto M, Sato N, Yamamoto T, Tamura K, Mizuno K, Tanaka K
J Nippon Med Sch 2012;79(2):159-62. doi: 10.1272/jnms.79.159. PMID: 22687361
Rare complications of pregnancy: aortic cystic medionecrosis, gallbladder carcinoma, Hodgkin lymphoma.
Velemínsky M Jr, Fuxová A, Sák P
Neuro Endocrinol Lett 2011;32(3):242-5. PMID: 21712775
Cystic medial necrosis Erdheim Gsell as a rare reason for spontaneous rupture of the ascending aorta.
Gary T, Seinost G, Hafner F, Gorkiewicz G, Brodmann M
Vasa 2011 Mar;40(2):147-9. doi: 10.1024/0301-1526/a000085. PMID: 21500180

Clinical prediction guides

Clinicopathological study on penetrating atherosclerotic ulcers and aortic dissection: distinct pattern of development of initial event.
Wada H, Sakata N, Tashiro T
Heart Vessels 2016 Nov;31(11):1855-1861. Epub 2016 Feb 18 doi: 10.1007/s00380-016-0813-2. PMID: 26892532

Supplemental Content

Table of contents

    Clinical resources

    Consumer resources

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • OMIM
      Related records in OMIM
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed
    • PubMed (OMIM)
      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...