Idiopathic arterial calcification of infancy: a clinicopathologic study

Abstract

The clinical and pathologic features of a group of 62 infants dying of idiopathic calcific arterial disease were reviewed. The disease most commonly occurs in infants less than 6 months of age. Pathologically, it is characterized by calcific deposits along the internal elastic membrane of arteries accompanied by fibrous thickening of the intima which causes luminal narrowing. The arterial lesions are widespread but the resultant luminal narrowing invariably promotes myocardial ischemia, causing the infants' deaths. A definite tendency of the disease to occur in siblings has been noted, but additional patterns of inheritance are not yet apparent. Clinical diagnosis is feasible with radiologic study of arteries of the head, neck, and extremities. There is a similarity of idiopathic calcific arterial disease of infancy to the arterial lesions of metastatic calcification in severe renal disease, calcific arterial lesions noted in conjunction with certain cardiovascular anomalies, and hypervitaminosis D. Certain experimental situations and toxic states can also produce calcific and proliferative vascular lesions.