Pathology videos & books

Correct Answer - A Ans. is 'a' i.e., Marfan's syndrome Cystic medial necrosis (CMN) is a disorder of large arteries, characterized by an accumulation of a basophilic ground substance in the media with cyst-like lesions. It is known to occur in certain connective tissue diseases such as Marfan syndrome, Ehlers-Danlos syndrome, and annuloaortic ectasia, which usually result from degenerative changes in the aortic wall. The relationships between CMN and congenital heart defects as well as other disorders have been evidenced. The mechanisms are still controversial, even though many molecular studies have been conducted it is characterized by elastic tissue fragmentation and separation of the elastic and fibromuscular elements of the tunica media by small cleft like spaces where the normal elastic tissue is lost; these areas are filled with the amorphous extracellular matrix of connective tissue and resemble but are not truly cysts.

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