Cystic medial degeneration
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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.
| Cystic medial degeneration | |
|---|---|
| Diagnosis in short | |
 Cystic medial degeneration. H&E stain. | |
|
| |
| 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 |
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]
- Marfan's syndrome.
- Ehlers-Danlos syndrome.
- Annuloaortic ectasia.
Microscopic
- Basophilic ground substance in the media (seen on Movat's stain).
- Disruption of the elastic lamina (seen on elastic trichrome stain).
- +/-Focal necrosis.
DDx:
- Aortic dissection without apparent underlying pathology.
Images
Cystic medial degeneration - low mag. (WC/Nephron)
Cystic medial degeneration - high mag. (WC/Nephron)
Cystic medial degeneration - movat - low mag. (WC/Nephron)
Cystic medial degeneration - movat - intermed. mag. (WC/Nephron)
Cystic medial degeneration - movat - high mag. (WC/Nephron)
www:
Stains
- Elastin stains (e.g. elastic trichrome stain) - disruption of the elastic lamina.
- Movat's stain - basophilic ground substance in the media.
- Alcian blue-PAS.
Images
See also
References
- ↑ URL: http://emedicine.medscape.com/article/756835-overview. Accessed on: 12 August 2010.
- ↑ Yuan, SM.; Jing, H.. "Cystic medial necrosis: pathological findings and clinical implications.". Rev Bras Cir Cardiovasc 26 (1): 107-15. PMID 21881719.
- ↑ URL: http://emedicine.medscape.com/article/756835-overview. Accessed on: 12 August 2010.
- ↑ 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.