SPR unknown #35 -- Final

Santhi Kollengode, MD

Paula Dickson, MD

Kiery Braithwaite

Emory University Hospital

Keywords

SPR unknown 35, Idiopathic infantile arterial calcification, artery, radiograph, ultrasound, CT


Publication Date: 2010-08-06

History

6 week old with vomiting, tachypnea and tachycardia

Findings

Chest x-ray (images 1&2): Frontal and lateral chest radiographs demonstrate cardiomegaly, pulmonary congestion and small right pleural effusion.

Ultrasound of the abdomen, including color doppler (images 3-6): Hyperechoic wall of the abdominal aorta representing calcifications. Calcifications of the left renal artery and its branches.

Noncontrast CT of the abdomen (images 7-9): Reformatted noncontrast CT images of the abdomen performed on a portable CT scanner demonstrate linear calcifications of the aorta, celiac axis, superior mesenteric, iliac and renal arteries.

Diagnosis

Idiopathic infantile arterial calcification

DDx

The differential diagnoses based on the patient’s clinical and chest radiograph findings included cardiomyopathy and myocarditis.

Discussion

Echocardiogram demonstrated poor ventricular function, severe mitral regurgitation and questionable calcifications of the aortic wall. The patient was then referred for ultrasound and noncontrast CT studies of the abdomen.

Idiopathic infantile arterial calcification is a rare condition of unknown etiology which affects large and medium sized arteries. It is characterized by generalized arterial calcification within the internal elastic lamina and subsequent fibroblastic intimal proliferation resulting in stenosis of arterial lumens.

The disease is almost invariably fatal with most cases diagnosed at autopsy. It should be suspected in children presenting with cardiac and respiratory symptoms and features of arterial calcification. Normal renal function and calcium-phosphate metabolism is seen in these children, with no metabolic cause for the calcifications.

Calcification has been reported within abdominal viscera, thyroid, visceral arteries and in periarticular soft tissues. Cerebral involvement is rare. Cardiac failure is the most common clinical presentation. Ultrasound and CT are sensitive modalities to detect arterial and visceral calcifications.

Biphosphonates, which are inhibitors of soft-tissue calcifications, have been used as specific therapy. Early death usually results within the first year from involvement of the coronary arteries and myocardial ischemia.

References

  1. Saigal G, Azouz EM (2004) The spectrum of radiologic findings in idiopathic calcification in infancy: pictorial essay. Can Assoc Radiol J 55: 102-107
  2. Whitehall J, Smith M, Altamarino L (2003) Idiopathic infantile arterial calcification: Sonographic findings. J Clin Ultrasound 31: 497-501
  3. Patel M, Andronikou S, Solomon R, Sinclair P, McCulloch M (2004) Idiopathic arterial calcification in childhood. Pediatr Radiol 34(8): 652-5.

9 images