Difference between revisions of "Cystic medial degeneration"
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Cystic_medial_degeneration_-_high_mag.jpg | |||
| Width = | |||
| Caption = Cystic medial degeneration. [[H&E stain]]. | |||
| Synonyms = cystic medial necrosis | |||
| Micro = basophilic ground substance in the media (seen on Movat's [[stain]]), disruption of the elastic lamina (seen on elastic trichrome stain), +/-focal necrosis. | |||
| Subtypes = | |||
| LMDDx = aortic dissection without apparent underlying pathology | |||
| Stains = [[Movat's stain]] (basophilic ground substance present), elastin stain (fragmentation of elastin) | |||
| IHC = | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[blood vessels|large arteries]] | |||
| Assdx = | |||
| Syndromes = [[Marfan's syndrome]], [[Ehlers-Danlos syndrome]], others | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = chest pain, shortness of breath | |||
| Prevalence = uncommon | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = dependent on severity and associated pathology | |||
| Other = | |||
| ClinDDx = [[myocardial infarction]], other causes of chest pain, others | |||
| Tx = surgical repair or conservative management - see [[aortic dissection]] | |||
}} | |||
'''Cystic medial degeneration''' (abbreviated '''CMD'''), also '''cystic medial necrosis''',<ref>URL: [http://emedicine.medscape.com/article/756835-overview http://emedicine.medscape.com/article/756835-overview]. Accessed on: 12 August 2010.</ref> is [[vascular disease|vascular pathology]] of the large [[blood vessel]]s. It is suggestive of an underlying [[connective tissue disorder]]. | '''Cystic medial degeneration''' (abbreviated '''CMD'''), also '''cystic medial necrosis''',<ref>URL: [http://emedicine.medscape.com/article/756835-overview http://emedicine.medscape.com/article/756835-overview]. Accessed on: 12 August 2010.</ref> is [[vascular disease|vascular pathology]] of the large [[blood vessel]]s. It is suggestive of an underlying [[connective tissue disorder]]. | ||
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Note about ''cystic medial necrosis'': | Note about ''cystic medial necrosis'': | ||
*Often ''not'' cystic and ''not'' necrotic. | *Often ''not'' cystic and ''not'' necrotic. | ||
Associations:<ref name=pmid21881719>{{Cite journal | last1 = Yuan | first1 = SM. | last2 = Jing | first2 = H. | title = Cystic medial necrosis: pathological findings and clinical implications. | journal = Rev Bras Cir Cardiovasc | volume = 26 | issue = 1 | pages = 107-15 | month = | year = | doi = | PMID = 21881719 }}</ref> | |||
*[[Marfan's syndrome]]. | |||
*[[Ehlers-Danlos syndrome]]. | |||
*Annuloaortic ectasia. | |||
==Microscopic== | ==Microscopic== | ||
| Line 13: | Line 49: | ||
*Disruption of the elastic lamina (seen on elastic trichrome stain). | *Disruption of the elastic lamina (seen on elastic trichrome stain). | ||
*+/-Focal necrosis. | *+/-Focal necrosis. | ||
DDx: | |||
*[[Aortic dissection]] without apparent underlying pathology. | |||
===Images=== | ===Images=== | ||
| Line 20: | Line 59: | ||
Image:Cystic_medial_degeneration_-_movat_-_low_mag.jpg | Cystic medial degeneration - movat - low mag. (WC/Nephron) | Image:Cystic_medial_degeneration_-_movat_-_low_mag.jpg | Cystic medial degeneration - movat - low mag. (WC/Nephron) | ||
Image:Cystic_medial_degeneration_-_movat_-_intermed_mag.jpg | Cystic medial degeneration - movat - intermed. mag. (WC/Nephron) | Image:Cystic_medial_degeneration_-_movat_-_intermed_mag.jpg | Cystic medial degeneration - movat - intermed. mag. (WC/Nephron) | ||
Image:Cystic_medial_degeneration_-_movat_-_high_mag.jpg | Cystic medial degeneration - movat - high mag. (WC/Nephron) | |||
</gallery> | </gallery> | ||
www: | www: | ||
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*Elastin stains (e.g. [[elastic trichrome stain]]) - disruption of the elastic lamina. | *Elastin stains (e.g. [[elastic trichrome stain]]) - disruption of the elastic lamina. | ||
*[[Movat's stain]] - basophilic ground substance in the media. | *[[Movat's stain]] - basophilic ground substance in the media. | ||
*[[Alcian blue]]-[[PAS]]. | |||
==See also== | ==See also== | ||
*[[Vascular disease]]. | *[[Vascular disease]]. | ||
*[[Aortic dissection]]. | |||
==References== | ==References== | ||
Latest revision as of 18:35, 2 February 2024
| 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]
- 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.
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.