A rare case of Idiopathic Arterial Calcification of Infancy (IACI), inherited as an autosomal recessive disease, is reported

Joshi, PS

Sarmila, N

Vani, R

English

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Idiopathic Infantile arterial calcification –A Very rare case

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Case Report:
Idiopathic Infantile arterial calcification –A Very rare case
Sharmila N, Professor,
Prashant. S. Joshi, Assistant Professor,
Dept. of Obstetrics and Gynecology, Adi Chunchangiri Institute of Medical Sciences, BG Nagara, Mandya, Karnataka, India, 
Vani Ravikumar, Asst Professor Dept. of Pathology,
Bangalore Medical College, Bangalore
Address For Correspondence:
Dr. Prashant. S. Joshi,
Assistant Professor, Dept. of Obstetrics and Gynecology,
Adi Chunchangiri Institute Of Medical Sciences,
B.G. Nagara, Mandya, Karnataka, India.
E-mail: joshpj@rediffmail.com
Citation:  Sarmila N, Joshi PS, Vani R. Idiopathic Infantile arterial calcification – A Very rare case.  Online J Health Allied Scs.  
2010;9(1):11
URL: http://www.ojhas.org/issue33/2010-1-11.htm
Open Access Archives: http://cogprints.org/view/subjects/OJHAS.html and http://openmed.nic.in/view/subjects/ojhas.html
Submitted: Mar 31, 2010; Accepted: Jun 10, 2010; Published: Jul 30, 2010
Abstract:
A rare case of Idiopathic Arterial Calcification of Infancy 
(IACI), inherited as an autosomal recessive disease, is repor-
ted.
Key Words: Arterial calcification, Infancy, Autosomal re-
cessive
 
Introduction:
Calcification of heart and vessels in fetus is a very rare condi-
tion.  It  may  be  dystrophic  or  metastatic.  An  extremly  rare 
form of vascular calcification has been termed Idiopathic Ar-
terial Calcification of Infancy (IACI) which is inherited in an 
autosomal  recessive  pattern. We  report  a  case of  IACI dia-
gnosed in our hospital.
Case Report:
A 26 year old woman, gravida 2, para 1 with no living child, 
with 20 weeks amenorrhea came for antenatal check up to our 
clinic. Her previous pregnancy was full term normal delivery 
and baby died at 11th month of age, reasons not being clear. 
Anomaly scan done at 20 weeks was reported as normal fetus. 
Patient came with preterm labor at 28 weeks of pregnancy and 
delivered a preterm baby weighing 1.2 kg which died 5 min 
after birth. Baby was sent for autopsy, as there was history of 
loss of first child. Autopsy report revealed fetal arterial calci-
fication which involved massive calcification of aortic vessels, 
coronary vessels,  pulmonary vessels,  pancreatic  vessels  and 
vessels  in  the  kidney.  Liver  showed  extramedullary  hema-
topoiesis and there was also hypertrophy of myocardium.
Fig 1: Calcification of descending aorta
Fig 2: Calcification of aorta
1
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Online Journal of Health and Allied Sciences
Peer Reviewed, Open Access, Free Online Journal
Published Quarterly :  Mangalore, South India : ISSN 0972-5997
Volume 9, Issue 1; Jan-Mar 2010
Fig 3: Calcified pulmonary vessels
Fig 4: Calcified renal vessels
Fig 5: Myocardial hypertrophy
Discussion:
Idiopathic arterial calcification of infancy or occlusive infant-
ile arterial calcification1 is a rare congenital disorder which is 
usually fatal in first six months of life2 and very few have sur-
vived more than one year.  This condition is inherited as an 
autosomal recessive pattern3 and diagnosis is usually made at 
autopsy in most of the cases.4 Only about 161 cases have been 
described by literature to date, with antemortem diagnosis be-
ing made on the radiographic or sonographic demonstration of 
arterial calcification in only a few cases. It was first described 
by Bryant  and White  in  1901.  It  is  characterized by wide-
spread and extensive  calcification  and stenosis  of  large  and 
medium sized arteries. This results from the deposition of cal-
cium hydroxyapatite in the arterial internal elastic lamina lay-
er, which leads to rapid progressive ischemic heart failure and 
refractory hypertension either prenatally or most commonly in 
neonatal and early infancy period.5 Biochemical investigations 
are usually normal and there is no identifiable abnormality of 
calcium metabolism.2
References:
1. Saigal  G.  Idiopathic  arterial  calcification  in  in-
fancy: pathology, diagnosis and treatment modalit-
ies. Indian J Pediatr 2002;69:265-267. 
2. Stuart  G.  Idiopathic  arterial  calcification  in  two 
siblings:  failure of  treatment  with  diphosphonate. 
Br Heart J 1990;64:156-9. 
3. Anderson KA, Burbach JA, Fenton LJ, Jaqua RA, 
Barlow  JF.  Idiopathic  arterial  calcification  in  in-
fancy  in newborn siblings with unusual light and 
electron  microscopic  manifestations.  Arch Pathol  
Lab Med 1985;109:838-843. 
4. Nagar AM. Antenatal detection of idiopathic arteri-
al calcification with hydrops fetalis.  J Ultrasound 
Med 2003;22:653-659. 
5. Nasrallah MK.Prenatal diagnosis of idiopathic in-
fantile  arterial  calcification  with  hydrops  fetalis. 
Ultrasound Obstet Gynecol 2009;34; 601-604.
2


Antenatal detection of idiopathic arterial calcification with hydrops fetalis.

Idiopathic arterial calcification in infancy in newborn siblings with unusual light and electron microscopic manifestations. Arch Pathol Lab Med

Idiopathic arterial calcification in infancy: pathology, diagnosis and treatment modalities.

Idiopathic arterial calcification in two siblings: failure of treatment with diphosphonate.

Nasrallah MK.Prenatal diagnosis of idiopathic infantile arterial calcification with hydrops fetalis.

Idiopathic Infantile arterial calcification –A Very rare case

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