cystic medial necrosis

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cys·tic me·di·al ne·cro·sis

loss of elastic and muscle fibers in the aortic media, with accumulation of mucopolysaccharide, sometimes in cystlike spaces between the fibers; a disease of unknown cause, which may be inherited and predisposes to dissecting aneurysms.


Jakob, Austrian physician, 1874-1937.
Erdheim disease - a disease of unknown cause that may be inherited and that predisposes to dissecting aneurysms. Synonym(s): cystic medial necrosis
Erdheim syndrome - acromegaly causing cervical stiffening and often associated with clavicular hypertrophy. Synonym(s): Scaglietti-Dagnini syndrome
Erdheim tumor - a suprasellar neoplasm, usually cystic, that develops from the nests of epithelium derived from Rathke pouch. Synonym(s): craniopharyngioma
References in periodicals archive ?
Fishbein et al (2), reported chronic arteritis and cystic medial necrosis pattern in the LITA grafts of four patients undergoing CABG.
We present a young patient with cystic medial necrosis who presented with atypical clinical symptoms and ascending thoracic aortic dissection was diagnosed by CT imaging.
These studies detail numerous cases of aortic aneurysm, dissection, or rupture in nonpregnant patients and note that some share histopathologic features, such as cystic medial necrosis, with Marfan syndrome patients (Pediatrics 102[1]:e12, 1998).
Spontaneous dissection of three major coronary arteries subsequent to cystic medial necrosis.