Sonographic recognition of idiopathic arterial calcification of infancy.

Abstract

Idiopathic arterial calcification of infancy is a rare disease of unknown cause. It is characterized by patchy destruction and disruption of the internal elastic membrane, with deposition of calcium hydroxyapatite in this layer [1 ]. In most cases there is also patchy intimal proliferation and fibrosis, which narrows the lumen of the vessel, leading to ischemia. The process affects both systemic and pulmonary arteries of all sizes, but it is myocardial ischemia due to coronary artery involvement that usually leads to death before 1 year of age. Although most of the approximately 80 reported cases were diagnosed at autopsy, the arterial calcifications could usually be perceived retrospectively on radiographs done during life. Among the 13 reported cases in which the findings were recognized ante mortem, there have been four survivors [1-4]. The radiographic findings can be very subtle [5, 6], but we recently encountered an infant in whom sonography revealed striking echogenicity of the arterial walls, which suggested the diagnosis. Case Report