A 59-year-old woman with dissection of the thoracic aorta and a fusiform aneurysm of the abdominal aorta without evidence of Marian's syndrome underwent aneurysmorrhaphy with a bifurcated expanded polytetrafluoroethylene graft. Histological specimens of the aneurysmal wall revealed the presence of idiopathic cystic medial necrosis. As typical findings of idiopathic cystic medial necrosis in the aortic wall are very rare except in cases of Marian's syndrome, the present case is reported and the implications of this condition are discussed.
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