Symptomatic Disseminated Cystic Medial Necrosis

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Abstract
SPONTANEOUS dissection and rupture of the aorta may occur in a grossly normal vessel, but microscopical examination reveals degeneration of the media, either secondary to arteriosclerosis extending from the intima or as the only lesion.1 The latter change is popularly referred to as idiopathic cystic necrosis even though many of its features such as focal loss of muscle fibers, fragmentation of elastic tissue and avascular replacement fibrosis without inflammation were described2 before Erdheim3 suggested that an overabundant mucoid ground substance was its main characteristic. This pathologic entity is rare outside the aorta, and even in that vessel it is restricted . . .