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

E Laporte-Turpin; M O Marcoux; G Machado; Y Dulac; I Claudet; E Grouteau; C Puget

doi:10.1016/j.arcped.2005.03.043

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

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

[Article in French]

Authors

E Laporte-Turpin¹, M O Marcoux, G Machado, Y Dulac, I Claudet, E Grouteau, C Puget

Affiliation

¹ POSU pédiatrique, hôpital des-Enfants, Toulouse, France. laporte-turpin.e@chu-toulouse.fr

PMID: 15964526
DOI: 10.1016/j.arcped.2005.03.043

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.

Publication types

Case Reports
English Abstract

MeSH terms

Adolescent
Aortic Aneurysm, Thoracic / diagnosis
Aortic Aneurysm, Thoracic / etiology*
Aortic Aneurysm, Thoracic / surgery
Aortic Dissection / diagnosis
Aortic Dissection / etiology*
Aortic Dissection / surgery
Blood Vessel Prosthesis
Ehlers-Danlos Syndrome / complications*
Ehlers-Danlos Syndrome / diagnosis
Ehlers-Danlos Syndrome / surgery
Fatal Outcome
Humans
Male