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Case Reports
. 2005 Jul;12(7):1112-5.
doi: 10.1016/j.arcped.2005.03.043.

[Lethal aortic dissection in a 13-year-old boy with a vascular Ehlers-Danlos syndrome]

[Article in French]
E Laporte-Turpin  1 , M O Marcoux, G Machado, Y Dulac, I Claudet, E Grouteau, C Puget
Affiliations
Case Reports

[Lethal aortic dissection in a 13-year-old boy with a vascular Ehlers-Danlos syndrome]

[Article in French]
E Laporte-Turpin et al. Arch Pediatr. 2005 Jul.

Abstract

Vascular Ehlers-Danlos syndrome(EDS) is at high risk of death by arterial rupture.

Case report: A 13-year-old boy with vascular EDS and aortic dissection was admitted in pediatric emergency care unit. The children died after surgery by massive hemorrhage and prosthetic rupture.

Discussion: The precocity of this vascular accident EDS is uncommon. It occurred despite clinical and echocardiographic follow-up. The death of the children confirmed the difficulties in surgery of vascular manifestation, even if appropriate and rapid management was already done.

Conclusion: Patients with vascular EDS must be identified because of its lethal complications. Friability of the vessels makes surgical treatment difficult. A trial using beta blockers therapy is ongoing.

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