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Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient

机译:小儿Marfan综合征患者的破裂性腹主动脉瘤修复

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摘要

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.
机译:马凡氏综合症是一种众所周知的常染色体显性结缔组织疾病,由于原纤维蛋白基因FBN1的自发突变,具有一系列的解剖特征,包括主动脉变性。尽管在文献中已经充分记录了威胁生命的解剖和上升的动脉瘤破裂,但很少报道腹主动脉瘤和小儿动脉瘤。在此病例报告中,我们描述了腹主动脉瘤破裂的小儿马凡氏综合症患者的表现,成功的开放手术修复和康复。

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