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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.
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 The occult 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.