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Published on: May 31, 2016
Idiopathic infantile arterial calcification: the spectrum of clinical presentations
Curtis R Chong1, Grover M Hutchins
1Department of Pharmacology and Medical Scientist Training Program, The John Hopkins University School of Medicine, Baltimore, MD 21287, USA. crchong@post.harvard.edu
Insights
Idiopathic infantile arterial calcification (IIAC) is a rare condition causing artery calcification. Early presentation and diphosphonate treatment are key for infant survival in IIAC.
Area of Science:
- Pediatric Pathology
- Cardiovascular Medicine
- Rare Diseases
Background:
- Idiopathic infantile arterial calcification (IIAC) is a rare genetic disorder.
- It involves extensive calcification of medium and large arteries, leading to severe complications.
Observation:
- A case report details a 32-week-old infant with hydrops fetalis and heart failure, who died at 4 days.
- Autopsy revealed cardiomegaly, myocardial infarctions, and multifocal arterial calcifications in multiple organs.
- Histopathology showed calcification extending into the intima and media, with a giant-cell reaction and smooth muscle proliferation.
Findings:
- A literature review identified 161 IIAC cases.
- 48% presented prenatally or at birth with hydrops fetalis, heart failure, or respiratory distress.
- 52% presented later (median 3 months) with fever, vomiting, or irritability.
- Crucially, 19 of 22 survivors presented within the first two weeks of life.
- Fifteen survivors received diphosphonate treatment.
Implications:
- Early detection of IIAC, particularly in neonates presenting with hydrops fetalis or heart failure, is critical.
- Prompt treatment with diphosphonates may improve outcomes for IIAC survivors.
- Further research into the pathogenesis and optimal management of IIAC is warranted.
Abstract:
Idiopathic infantile arterial calcification (IIAC) is a rare disorder characterized by extensive calcification of medium and large arteries. We report the case of a 32-week-old infant with hydrops fetalis and heart failure who died at 4 days of age. At autopsy the infant was found to have cardiomegaly, myocardial infarctions and multifocal calcifications of the aorta and arteries in the lungs, heart, thyroid, spleen, and testis. Calcification extended from the internal elastic lamina into the intima and media and was associated with a giant-cell reaction and smooth muscle proliferation. A search of the English language medical literature identified 161 IIAC case reports. Of these, 48% of cases presented in utero or at birth with hydrops fetalis, maternal hydramnios, heart failure, or respiratory distress and 52% present later, at a median age of 3 months, with sudden onset of fever, vomiting, irritability, or respiratory distress in a previously healthy infant. Significantly, 19 of 22 IIAC survivors presented at less than 2 weeks of age, and 15 survivors were treated with diphosphonates.
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