SONOWORLD : Idiopathic infantile arterial calcification
 
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Obstetrics » Obstetrics 2nd And 3rd Trimester
Idiopathic infantile arterial calcification
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Presentation This is a 35 year old G4P2 patient whose previous child was stillborn due to non-immune hydrops. During the current pregnancy, she had an ultrasound at 20 weeks, showing a normal fetal survey. Subsequently, an ultrasound done at 23 weeks showed subtle but definite intravascular calcifications, particularly in the abdominal aorta. By 28 weeks, there were severe calcifications of the large arteries of the fetal chest and abdomen, including the aorta, iliac and pulmonary arteries. Ultrasound done at 28 and 32 weeks showed complete linear calcifications of the wall of the aorta and iliac arteries, as well as the coronary arteries. After 32 weeks, the fetus developed hydrops and a very large pericardial effusion, with signs of heart failure. The fetus was delivered at 36 weeks and died within the first 24 hours of life, from severe heart failure.

 
 
 
Caption: Longitudinal fetal trunk at 28 weeks.
Description: Severe calcification of the fetal abdominal aorta.
 
 
 
Caption: Transverse fetal trunk at 28 weeks.
Description: Note severe calcification of aorta with distinct shadowing.
 
 
 
Caption: Longitudinal fetal abdomen and pelvis at 32 weeks.
Description: Severe calcification of the lower abdominal aorta, extending through the bifurcation into the iliac arteries.
 
 
 
Caption: Longitudinal fetal abdomen and pelvis at 32 weeks.
Description: Severe calcification of the mid abdominal aorta. Extension into one of the iliac arteries is seen.
 
 
 
Caption: Transverse fetal abdomen at 32 weeks.
Description: Note distinct calcification of abdominal aorta in cross section.
 
 
Caption: Fetal Heart and Great Vessels
Description: This 1.5 Meg AVI file is a transverse view of the fetal heart and mediatinum, showing a large pericardial effusion along with calcification of the great vessels.
 
 
Caption: Fetal Mediastinum
Description: View of the upper fetal mediastinum, showing the intense calcification of the thoracic aorta and pulmonary arteries. (1.1 meg avi video clip)
 
 
Caption: Fetal Heart with Pericardial Effusion
Description: AVI video clip (1.2 meg) of the fetal heart showing a huge pericardial effusion.
 
 
Caption: Flash Video clip of Fetal Heart
Description: View of the upper fetal mediastinum, showing the intense calcification of the thoracic aorta and pulmonary arteries.
 
Differential Diagnosis Progeria syndrome: senile like appearance with alopecia, periarticular fibrosis, skeletal degenerative changes, and early atherosclerosis. never been detected prenatally.
Cockayne syndrome: senile like changes beginning in infancy- similar to progeria. not usually evident until age 2-4 years. never been detected prenatally.
Idiopathic infantile arterial calcification: premature vascular calcification leading to congestive heart failure and death in infancy.
 
Final Diagnosis The pathology report of the first stillborn infant showed calcium hydroxyapatite deposition in the internal elastic lamina and focal initimal proliferation with thickening of vessel walls. The diagnosis was idiopathic infantile arterial calcification. The second pregnancy  had the same diagnosis and outcome.
 
Discussion The etiology of idiopathic infantile arterial calcification is unknown. It is characterized by wide spread arterial calcifications. These calcifications occur in the internal elastic lamina. Calcium incrustation of the lamina leads to rupture followed by occlusive changes. Ihe initial disease may be a primary defect of the elastic fiber.

There are approximately 100 reported cases, with several sets of siblings, similar to this case. The heredity is postulated to be autosomal recessive. The disease is so rare that it is often not recognized until autopsy.

The ultrasound findings include: normal early scans, with abnormalities developing only in the 3rd trimester, polyhydramnios, hydrops, poor cardiac contractility, a visibly calcified aorta and coronary artery origins.

Over a brief time period, there is a decline of cardiac output and fetal activity resulting in stillborns or neonatal deaths. Infants who survive for a short time suffer from poor feeding, cyanosis, heart failure, respiratory distress, and hypertension. Eighty-five percent (85%) of infants die in the first 6 months of life from myocardial infarction, and occlusive vascular disease. The diagnosis of idiopathic infantile arterial calcification should be suspected in infants presenting with evidence of heart failure and myocardial ischemia.

The pathologic characteristics of this disease include cardiac hypertrophy and tortuous coronaries with thick walls and narrow lumens, leading to myocardial infarction. Microscopically, there are calcium deposits along the internal elastic membrane of the arteries, as well as intimal fibrous thickening leading to occlusion of vessels. The most commonly affected arteries include the coronary arteries, and other medium size vessels. The central nervous system vessels are typically spared.

 
Case References Bird, T. Idiopathic arterial calcification in infancy. Arch. Dis. Child. 49: 82-89, 1974.
Hunt et al.  Generalized arterial calcification in infancy. Brit. Med. J. 1: 385-386, 1957.
Meradji, M et al.  Idiopathic infantile arterial calcification in siblings: radiologic diagnosis and successful treatment. J. Pediat. 92: 401-405, 1978.
Moran, JJ et al. Idiopathic arterial calcification of infancy: report of 2 cases occurring in siblings, and review of the literature. Am. J. Clin. Path. 31: 517-529, 1959.
Raphael, SS et al .Arterial medial calcification of infancy in brothers. Canad. Med. Assoc. J. 103: 290-293, 1970.
Stuart, G et al .Idiopathic infantile arterial calcification in two siblings: failure of treatment with diphosphonate. Brit. Heart J. 64: 156-159, 1990.
Spear R et al.  Idiopathis arterial calcification: in utero diagnosis. J Ultrasound Med 9:473-476,1990.
 
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