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Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient

Marfan syndrome is a well-described autosomal dominant connective tissue disorder with a constellation of anatomic characteristics including aortic degeneration as a result of the spontaneous mutation of the fibrillin gene, FBN1. Whereas life-threatening dissection and ascending aneurysmal rupture h...

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Detalles Bibliográficos
Autores principales: Lu, Joyce J., Slaikeu, Jason D., Wong, Peter Y.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5849780/
https://www.ncbi.nlm.nih.gov/pubmed/29541693
http://dx.doi.org/10.1016/j.jvscit.2017.11.008
Descripción
Sumario:Marfan syndrome is a well-described autosomal dominant connective tissue disorder with a constellation of anatomic characteristics including aortic degeneration as a result of the spontaneous mutation of the fibrillin gene, FBN1. Whereas life-threatening dissection and ascending aneurysmal rupture have been thoroughly documented in the literature, aneurysms of the abdominal aorta and those present in the pediatric population have only rarely been reported. In this case report, we describe presentation, successful open surgical repair, and recovery of a pediatric Marfan syndrome patient with a ruptured abdominal aortic aneurysm.