Idiopathic Arterial Calcification of Infancy: Report of 2 Cases Occurring in Siblings, and Review of the Literature

Idiopathic Arterial Calcification in Infancy

“…The coronary arteries were affected in 42 of the 44 cases reviewed by Moran and Becker (1959) who also noted the presence of widespread lesions in 28 cases where many arteries had been examined. Although examination of our cases was incomplete it is probable that both were genuine examples of the idiopathic generalized variety of infantile arterial calcification, as in neither case was there a history of excessive ingestion of vitamin D or evidence of congenital anomaly of the heart or great vessels.…”

“…The possible aetiology of infantile arterial calcification has been discussed at length by Field (1946), Stryker (1946), Moran and Becker (1959) and Moran and Steiner (1962). Field suggested that the primary defect was in the intercellular substance of the media, but other writers have favoured the elastica as the primary site of calcification.…”

“…Field described focal swelling of the intercellular substance of the media of the common carotid artery as the earliest change in her case, with deposition of calcium in the media adjacent to the internal elastic lamina as a slightly later change. Stryker found the earliest change to be basophilic granulation, presumably calcification, of the elastica, and several other writers (Moran and Becker, 1959;Sladden, 1952) have pointed out that the first calcium deposits are laid down in relation to the internal elastic lamina-sometimes on either side of it.…”

“…In many instances arterial lesions have been associated with renal disease, hypervitaminosis-D or congenital anomalies of the heart and great vessels, but more rarely there has been no demonstrable primary disease. About 50 cases of this idiopathic type of arterial calcification in infancy have now been described and the published material has been comprehensively reviewed on several occasions (Field, 1946;Stryker, 1946;Moran and Becker, 1959). Nevertheless, our knowledge of the condition has not progressed beyond the descriptive stage, and hypotheses of its aetiology show no advance on those put forward by Stryker in 1946.…”

Idiopathic infantile arterial calcification. In utero diagnosis.

“…These arterial lesions may be distinguished from atherosclerosis by the absence of lipid deposition and from Monckeberg's sclerosis by the presence of intimal calcification and subintimal fibrous proliferation. 3 The calcification aggregates are not simple calcium collections, but are more complex compounds. Meradji croscopic patterns were encountered in the current two cases.…”