Cystic medial necrosis responsible for aoic dilatation and rupture is seen in -

Ans. is 'd' Marfan syndromeo Cystic medial necrosis is the degenerative disease of media of the aoa.o It characterized by degeneration of collagen and elastic fibers in tunica media of aoa, and also the loss of medial cells that are replaced by multiple cleft of mucoid material.It characteristically affects the proximal aoer, results in circumferential weakness and dilatation which results in the development offusiform aneurysm involving the ascending aoa and sinus of vulsalva.o Cystic medial necrosis is a pathological substrate for aoic dissection.Diseases causing cystic medial necrosis o Marfan syndromeo Ehler-Danlus syndrome (type IV)o Congenital bicuspid aoic valveo Familial thoracic aoic aneurysm syndrome o Pregnancy