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==
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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==

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]

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

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.