Key result
An 11-year-old girl with Marfan's syndrome developed a rare intramural aortic hematoma.
Case Report (n=1)
Highlights the rare occurrence of intramural aortic hematoma in a pediatric patient with Marfan's syndrome.
May prompt consideration of intramural hematoma in symptomatic children with Marfan syndrome; hypothesis-generating for pediatric aortic complications.
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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Aliyu et al. (2014) conducted a case report in Marfan's syndrome with intramural aortic hematoma (n=1). An 11-year-old girl with Marfan's syndrome developed a rare intramural aortic hematoma.
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