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.

Erdheim,

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 ?
Causes of descending TAAs include atherosclerosis, cystic medial necrosis, chronic dissection, and aortitis [3].
Our patient had trileaflet aortic valve, negative family history, and no medial degeneration (previously designated cystic medial necrosis) in the pathology, thus making the congenital process less likely.
Included in the differential diagnosis are inflammatory arteritis, collagen vascular disease, mycotic aneurysms, cystic medial necrosis seen in type IV Ehlers Danlos and Marfan's syndrome, and fibromuscular dysplasia (FMD) which are also important to consider [6, 7].
The aorta possesses histologically evident cystic medial necrosis making it vulnerable to dissection.
Cystic medial necrosis, intimal tear, and angiomatosis are some of the infrequent, associated microscopic findings.
commented that delayed aortic dissection after cardiac surgery was not related to technical operative errors or clamp injury, but to cystic medial necrosis or hypertension.12
Fishbein et al (2), reported chronic arteritis and cystic medial necrosis pattern in the LITA grafts of four patients undergoing CABG.
Cystic medial necrosis has been reported in association with CAD in several studies.
Coexistence of fibromuscular dysplasia and cystic medial necrosis in a patient with Marfan's syndrome and bilateral carotid artery dissections.
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. Chest 1999; 116: 1473-5.