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 prothetic rupture. Discussion. -The precocity of this vascular accident EDS is uncommon. It occured 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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