Cystic medial degeneration

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Cystic medial degeneration
Diagnosis in short

Cystic medial degeneration. H&E stain.

Synonyms cystic medial necrosis

LM basophilic ground substance in the media (seen on Movat's stain), disruption of the elastic lamina (seen on elastic trichrome stain), +/-focal necrosis.
LM DDx aortic dissection without apparent underlying pathology
Stains Movat's stain (basophilic ground substance present), elastin stain (fragmentation of elastin)
Site large arteries

Syndromes Marfan's syndrome, Ehlers-Danlos syndrome, others

Symptoms chest pain, shortness of breath
Prevalence uncommon
Prognosis dependent on severity and associated pathology
Clin. DDx myocardial infarction, other causes of chest pain, others
Treatment surgical repair or conservative management - see aortic dissection

Cystic medial degeneration (abbreviated CMD), also cystic medial necrosis,[1] is vascular pathology of the large blood vessels. It is suggestive of an underlying connective tissue disorder.

General

  • Nonspecific finding - may be seen in a number of conditions.
  • "Medial" refers to tunica media the middle (muscle) layer of an artery.

Note about cystic medial necrosis:

  • Often not cystic and not necrotic.

Associations:[2]

Microscopic

Features:[3][4]

  • Basophilic ground substance in the media (seen on Movat's stain).
  • Disruption of the elastic lamina (seen on elastic trichrome stain).
  • +/-Focal necrosis.

DDx:

Images

www:

Stains

Images

See also

References

  1. URL: http://emedicine.medscape.com/article/756835-overview. Accessed on: 12 August 2010.
  2. Yuan, SM.; Jing, H.. "Cystic medial necrosis: pathological findings and clinical implications.". Rev Bras Cir Cardiovasc 26 (1): 107-15. PMID 21881719.
  3. URL: http://emedicine.medscape.com/article/756835-overview. Accessed on: 12 August 2010.
  4. Ha HI, Seo JB, Lee SH, et al. (2007). "Imaging of Marfan syndrome: multisystemic manifestations". Radiographics 27 (4): 989–1004. doi:10.1148/rg.274065171. PMID 17620463. http://radiographics.rsna.org/content/27/4/989.full.