Idiopathic infantile arterial calcification: imaging evaluation and the usefulness of MR angiography

Idiopathic infantile arterial calcification (IIAC) is a rare condition characterized by extensive calcification and stenosis of large and medium-size arteries. The etiology of the disease is unknown. However, the inheritance pattern has been shown to be autosomal recessive. The clinical presentation is variable, including cardiac failure (most common clinical finding), hypertension, and respiratory failure. Plain radiography, sonography and MRI can aid in the diagnosis. We present a case in which contrast-enhanced MR angiography with breath-hold and cardiac gating techniques allowed complete evaluation of the extent of this disease.