Very rare metabolic disorder characterized by
deposition of calcium salts in the intima of the aorta, coronary, iliac,
and carotid arteries. Coronary artery disease occurs in early
infancy with heart failure and myocardial infarction. Death usually occurs
before 6 months of age.
Idiopathic Infantile Arterial Calcification; Idiopathic
Arterial Calcification in Infancy; Infantile Arterial Calcifications;
Infantile Occlusive Arteriopathy; Medial Coronary Sclerosis of Infancy;
Occlusive Infantile Arteriopathy.
Calcification of the internal elastic lamina of
arteries leading to rupture of the lamina and occlusive changes in the
affected artery. Altered iron metabolism may play a role in the pathogenesis.
Deficient extracellular inorganic pyrophosphate and a deficiency of plasma
cell membrane glycoprotein-1 (PC-1) nucleoside triphosphate
pyrophosphohydrolase activity can be associated with or is responsible for
ECG changes of occlusive coronary artery disease,
calcification in peripheral arteries demonstrable by ultrasound or CT
scan. Periarticular calcification may be present. Prenatal ultrasound diagnosis is
Respiratory difficulties and coronary artery
disease occur in early infancy with heart failure and myocardial infarction.
Vomiting, abdominal distension, ileus, joint swelling, and hypertension may
coexist. Death usually occurs before the age of 6 months. Treatment with
diphosphonate resolved the calcification in one reported case.
Full assessment of cardiovascular
system with ECG, echocardiography, and angiography. Treatment of
hypertension and cardiac failure should be optimized. Correct anemia or any
preoperative electrolyte disturbances.
Very poor long-term outlook. Anesthetic
technique should be chosen to ensure cardiovascular stability; prevent
hypertension/hypotension and maintain diastolic pressure to ensure coronary
perfusion. Maintain heart rate; prevent excessive tachycardias. Prevent
hypoxia and acidosis. Ensure adequate analgesia. Postoperative oxygen
therapy and monitoring in an intensive care unit may be required.
Avoid drugs that cause tachycardias
or hypotension to ensure diastolic coronary perfusion is maintained.
Levine JC, Campbell J, Nadel A: Prenatal diagnosis of idiopathic
infantile arterial calcification. Circulation
Rutsch F, Vaingankar S, Johnson K, et al: PC-1 nucleoside triphosphate
pyrophosphohydrolase deficiency in idiopathic infantile arterial
calcification. Am J Pathol