Cystic_medial_degeneration

Familial thoracic aortic aneurysm

Familial thoracic aortic aneurysm

Medical condition


Familial thoracic aortic aneurysm is an autosomal dominant[1] disorder of large arteries.

Quick Facts Other names ...

There is an association between familial thoracic aortic aneurysm and Marfan syndrome as well as other hereditary connective tissue disorders.

Signs and symptoms

A degenerative breakdown of collagen, elastin, and smooth muscle caused by aging contributes to weakening of the wall of the artery.[2]

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

Genetics

Types include:

More information Type, OMIM ...

Diagnosis

Treatment

Terminology

It is sometimes called "Erdheim cystic medial necrosis of aorta", after Jakob Erdheim.[3][4]

The term "cystic medial degeneration" is sometimes used instead of "cystic medial necrosis", because necrosis is not always found.[citation needed]


References

  1. Wiesenfarth, John, http://www.emedicine.com/emerg/topic28.htm, October 4, 2005
  2. J. Erdheim. Medionecrosis aortae idiopathica (cystica). Archiv für pathologische Anatomie und Physiologie und für klinische Medizin, 1929, 273: 454-479.

Share this article:

This article uses material from the Wikipedia article Cystic_medial_degeneration, and is written by contributors. Text is available under a CC BY-SA 4.0 International License; additional terms may apply. Images, videos and audio are available under their respective licenses.