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Year : 2014  |  Volume : 11  |  Issue : 2  |  Page : 139-141

Intramural aortic hematoma in an 11-years-old girl with Marfan's syndrome

Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria

Correspondence Address:
Ibrahim Aliyu
Department of Paediatrics, Aminu Kano Teaching Hospital, Kano
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0189-7969.142123

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Marfan's syndrome is a rare congenital connective tissue disorder. It occurs worldwide with no sex predilection. Mutation in the fibrillin genes result in production of abnormal elastic tissue, which predisposes the aorta to cystic medial degeneration and may result in dissection, rupture, or aneurysm formation. Other vascular structures like the heart valves may also be involved causing aortic regurgitation and mitral valve prolapse and regurgitation. Aortic dissection is rare in children even in those with Marfan's syndrome. We report a case of an 11-years-old girl with Marfan's syndrome who had an intramural aortic hematoma.

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