This can result in Cystic medial necrosis or a picture similar to Marfan syndrome.

Aortic root dilatation is thought to be due to a mesenchymal defect as pathological evidence of cystic medial necrosis has been found by several studies.

He is credited with the characterization (or partial characterization) of Erdheim-Chester disease and cystic medial necrosis.

It is sometimes called "Erdheim cystic medial necrosis of aorta", after Jakob Erdheim.

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

This is known as cystic medial necrosis and is most commonly associated with Marfan syndrome and is also associated with Ehlers-Danlos syndrome.

Other causes: Other causes of the development of AAA include: infection, trauma, arteritis, cystic medial necrosis (m. Erdheim).

Familial thoracic aortic aneurysm (also referred to as familial aortic dissection or cystic medial necrosis of aorta) is an autosomal dominant disorder of large arteries.

It is sometimes associated with Marfan syndrome or Loeys-Dietz syndrome, but may also result from Ehlers-Danlos syndrome, atherosclerosis, syphilis, cystic medial necrosis, chest injury, or infective endocarditis.