Cystic medial necrosis

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Cystic medial necrosis
Cystic medial degeneration - movat - intermed mag.jpg
Micrograph showing cystic medial degeneration, the histologic correlate of familial thoracic aortic aneurysms. The image shows abundant basophilic ground substance in the tunica media (blue at top of image) and disruption of the elastic fibers. The tunica adventitia (yellow at bottom of image) with vaso vasorum is also seen. Movat's stain.
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 and dissection; FTAAD; 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; Erdheim disease, medionecrosis aortae idiopathica cystica, medionecrosis of the aorta, mucoid medial degeneration

Overview

Cystic medial necrosis is an autosomal dominant[1] disorder of large arteries. There is a degenerative breakdown of collagen, elastin, and smooth muscle caused by aging which contributes to the weakening of the wall of the aorta. This weakening of the wall of the aorta can result in the formation of an 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. There is a loss of elastic and muscle fibers in the medial layer of the aorta along with the accumulation of mucopolysaccharides in cystlike spaces between the fibers (hence the term cystic). In the aorta, this can result in the formation of a fusiform aneurysm, and a subsequent risk of aortic dissection.

Genetics

Genetic variants include ACTA2 as well as:

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 the following disorders:

Epidemiology and Demographics

The disease tends to percent after 40 years of age and is twice as common in men as in women.

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. synd/2409 at Who Named It
  3. J. Erdheim. Medionecrosis aortae idiopathica (cystica). Archiv für pathologische Anatomie und Physiologie und für klinische Medizin, 1929, 273: 454-479.
  4. Myxomatous Degeneration and Cystic Medial Necrosis Associated With Acromegaly Sharon M. Ondreyco, MD; H. Daniel Lewis, Jr, MD; Charles R. Hartman, MD Arch Intern Med. 1980;140(4):547-549. doi:10.1001/archinte.1980.00330160107039



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