Idiopathic infantile arterial calcification with survival to adult life

Moran, in an excellent review, defined idiopathic infantile arterial calcification as a rare disorder of undetermined etiology, characterized by deposition of calcific material along the internal elastic membrane of large, medium, and small arteries. Fibrous proliferation in the intima occurs and the arterial lumen is narrowed. The constellation of affected tissues varies but the coronary arteries are almost always involved whereas the arteries of the central nervous system are spared. Death from myocardial infarction usually results within the first six months of life. To date there have been only three survivors. We present the case history of a fourth survivor, a 22-year-old woman who, at the age of three months, was diagnosed as having idiopathic infantile arterial calcification.