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In the aorta,this can result in the formation of a fusiform [[aneurysm]].
In the aorta,this can result in the formation of a fusiform [[aneurysm]].
==Pathology ==
==Pathology ==
Cystic media necrosis is a degenerative breakdown of [[collagen]], [[elastin]], and [[smooth muscle]] caused by aging which contributes to weakening of the wall of the artery.  In the aorta, this can result in the formation of a fusiform [[aneurysm]], and a subsequent risk of [[aortic dissection]].
===Genetics===
===Genetics===
Genetic variants include:
Genetic variants include:
Line 55: Line 57:
==Natural History, Complications, and Prognosis ==
==Natural History, Complications, and Prognosis ==
Cystic medial necrosis is associated with an increased risk of [[aortic dissection]].
Cystic medial necrosis is associated with an increased risk of [[aortic dissection]].
==Presentation==
A degenerative breakdown of [[collagen]], [[elastin]], and [[smooth muscle]] caused by aging contributes to weakening of the wall of the artery.<ref>Wiesenfarth, John, http://www.emedicine.com/emerg/topic28.htm, October 4, 2005</ref>
In the aorta, this can result in the formation of a fusiform [[aneurysm]]. There is also increased risk of [[aortic dissection]].


==References==
==References==

Revision as of 19:04, 21 October 2012

Cystic medial necrosis
ICD-9 441.00
OMIM 607086
DiseasesDB 30073

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: cystic medial degeneration; familial thoracic aortic aneurysm; familial aortic dissection; cystic medial necrosis of aorta [1]; it is sometimes called "Erdheim's cystic medial necrosis", after Jakob Erdheim[2][3]; the term cystic medial degeneration is sometimes used instead of cystic medial necrosis, because necrosis is not always found

Overview

Cystic medial necrosis is an autosomal dominant[1] disorder of large arteries. A degenerative breakdown of collagen, elastin, and smooth muscle caused by aging contribute to weakening of the wall of the artery.

In the aorta,this can result in the formation of a fusiform aneurysm.

Pathology

Cystic media necrosis is a degenerative breakdown of collagen, elastin, and smooth muscle caused by aging which contributes to weakening of the wall of the artery. In the aorta, this can result in the formation of a fusiform aneurysm, and a subsequent risk of aortic dissection.

Genetics

Genetic variants include:

Type OMIM Gene Locus
AAT1 607086 11q23.3-q24
AAT4 132900 MYH11 16p
AAT6 611788 ACTA2 10q

Associated Conditions

There is an association between cystic medial necrosis and Marfan syndrome.

Natural History, Complications, and Prognosis

Cystic medial necrosis is associated with an increased risk of aortic dissection.

References

  1. 1.0 1.1 Online Mendelian Inheritance in Man (OMIM) 607086
  2. Template:WhoNamedIt
  3. J. Erdheim. Medionecrosis aortae idiopathica (cystica). Archiv für pathologische Anatomie und Physiologie und für klinische Medizin, 1929, 273: 454-479.

See also

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