OJHAS: 2010-1-11. Sarmila N, Joshi PS, Vani R. Idiopathic Infantile arterial calcification

ISSN 0972-5997

Published Quarterly

Mangalore, India

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OJHAS Vol. 9, Issue 1: (2010 Jan-Mar)


		Case Report

Idiopathic Infantile arterial calcification –A Very rare case


		Authors

Sharmila N,
Professor,
Prashant. S. Joshi
Assistant Professor,
Dept. of Obstetrics and Gynecology, Adi Chunchangiri Institute Of Medical Sciences, B.G. 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

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http://openmed.nic.in/view/subjects/ojhas.html

This work is licensed under a Creative Commons Attribution-No Derivative Works 2.5 India License

This Article

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 reported.
Key Words: Arterial calcification, Infancy, Autosomal recessive



		Introduction

Calcification of heart and vessels in fetus is a very rare condition. It may be dystrophic or metastatic. An extremly rare form of vascular calcification has been termed Idiopathic Arterial Calcification of Infancy (IACI) which is inherited in an autosomal recessive pattern. We report a case of IACI diagnosed 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 11^th 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 calcification which involved massive calcification of aortic vessels, coronary vessels, pulmonary vessels, pancreatic vessels and vessels in the kidney. Liver showed extramedullary hematopoiesis and there was also hypertrophy of myocardium.


Fig 1: Calcification of descending aorta		Fig 2: Calcification of aorta

Fig 3: Calcified pulmonary vessels		Fig 4: Calcified renal vessels
		Fig 5: Myocardial hypertrophy



		Discussion

Idiopathic arterial calcification of infancy or occlusive infantile arterial calcification¹ is a rare congenital disorder which is usually fatal in first six months of life² and very few have survived 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.⁴Only about 161 cases have been described by literature to date, with antemortem diagnosis being 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 widespread and extensive calcification and stenosis of large and medium sized arteries. This results from the deposition of calcium hydroxyapatite in the arterial internal elastic lamina layer, which leads to rapid progressive ischemic heart failure and refractory hypertension either prenatally or most commonly in neonatal and early infancy period.⁵Biochemical investigations are usually normal and there is no identifiable abnormality of calcium metabolism.²



		References

Saigal G. Idiopathic arterial calcification in infancy: pathology, diagnosis and treatment modalities. Indian J Pediatr 2002;69:265-267.
Stuart G. Idiopathic arterial calcification in two siblings: failure of treatment with diphosphonate. Br Heart J 1990;64:156-9.
Anderson KA, Burbach JA, Fenton LJ, Jaqua RA, Barlow JF. Idiopathic arterial calcification in infancy in newborn siblings with unusual light and electron microscopic manifestations. Arch Pathol Lab Med 1985;109:838-843.
Nagar AM. Antenatal detection of idiopathic arterial calcification with hydrops fetalis. J Ultrasound Med 2003;22:653-659.
Nasrallah MK.Prenatal diagnosis of idiopathic infantile arterial calcification with hydrops fetalis. Ultrasound Obstet Gynecol 2009;34; 601-604.