Cystic medial necrosis
| Cystic medial necrosis | |
| ICD-9 | 441.00 |
|---|---|
| OMIM | 607086 |
| DiseasesDB | 30073 |
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 a 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.[4]
In the aorta,this can result in the formation of a fusiform aneurysm. There is also increased risk of aortic dissection.
There is an association between cystic medial necrosis and Marfan syndrome.
References
- ↑ 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