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Difference between revisions of "Cystic medial degeneration"
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Cystic medial degeneration (view source)
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'''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 pathology of the large [[blood vessel]]s suggestive of an underlying [[connective tissue disorder]]. | {{ 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]]. | |||
==General== | ==General== | ||
*Nonspecific finding - may be seen in a number of conditions. | *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'': | 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 12: | 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 19: | 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: | ||
| Line 26: | Line 67: | ||
*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== | ||