Cystic medial necrosis: Difference between revisions

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	Cystic medial necrosis;
ICD-9	441.00
OMIM	607086
DiseasesDB	30073

VisualWikitext

Revision as of 19:02, 21 October 2012

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

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.

Presentation

A degenerative breakdown of collagen, elastin, and smooth muscle caused by aging contributes to weakening of the wall of the artery.^[4]

In the aorta, this can result in the formation of a fusiform aneurysm. There is also increased risk of aortic dissection.

References

↑ ^1.0 ^1.1 Online Mendelian Inheritance in Man (OMIM) 607086
↑ Template:WhoNamedIt
↑ J. Erdheim. Medionecrosis aortae idiopathica (cystica). Archiv für pathologische Anatomie und Physiologie und für klinische Medizin, 1929, 273: 454-479.
↑ Wiesenfarth, John, http://www.emedicine.com/emerg/topic28.htm, October 4, 2005

@@ Line 24: / Line 24: @@
 In the aorta,this can result in the formation of a fusiform [[aneurysm]].
 ==Pathology ==
-=== Associated Conditions===
+===Genetics===
-There is an association between cystic medial necrosis and [[Marfan syndrome]].
+Genetic variants include:
-==Natural History, Complications, and Prognosis ==
-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]].
-==Genetics==
-Types include:
 {| class="wikitable"
@@ Line 60: / Line 49: @@
 | 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]].
+==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==