Idiopathic infantile arterial calcification—A misnomer?

Abstract

An autopsy case of idiopathic arterial disease in an infant was studied in detail. Pathologic features included intimal fibrous proliferation in elastic and muscular arteries, elastic tissue degeneration and scant calcification, and focal round cell infiltration at the junction of intima and media in arteries with intimal proliferation. These changes were widespread but patchy or segmental in distribution. They were associated with evidence of vascular insufficiency in several organs and with the presence of thrombi in many affected arteries. Intimai proliferation appeared to be quantitatively and physiologically the most critical of the pathologic changes. The case is believed to represent an example of so-called idiopathic arterial calcification of infancy. Review of the literature and consideration of this case suggest 2 possibilities: (1) calcification has been overemphasized as a feature of this condition, since it represents only a secondary (and often insignificant) phenomenon, with the intimal proliferation being the fundamental pathologic alteration; or (2) descriptions of this condition include 2 diseases, 1 characterized principally by vascular calcification, and the other by intimal proliferation. It is suggested that the former hypothesis is more tenable. The designation “idiopathic arterial calcification” appears to be misleading, and the condition should be called “occlusive infantile arteriopathy” until its origin can be definitely established.