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Embolization of Life-Threatening Arterial Rupture in Patients with Vascular Ehlers–Danlos Syndrome

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Abstract

Purpose

To evaluate the safety and efficacy of transarterial embolization of life-threatening arterial rupture in patients with vascular Ehlers–Danlos syndrome (vEDS) in a single tertiary referral center.

Methods

We retrospectively analyzed transarterial embolization for vEDS performed at our institution from 2000 to 2012. The indication of embolization was spontaneous arterial rupture or pseudoaneurysm with acute bleeding. All interventions used a percutaneous approach through a 5F or less introducer sheath. Embolic agents were microcoils and glue in 3 procedures, glue alone in 2, and microcoils alone in 2.

Results

Five consecutive vEDS patients were treated by 7 embolization procedures (4 women, mean age 29.8 years). All procedures were successfully performed. Two patients required a second procedure for newly arterial lesions at a different site from the first procedure. Four of the five patients were still alive after a mean follow-up of 19.4 (range 1–74.7) months. One patient died of multiple organ failure 2 days after procedure. Minor procedural complications were observed in 3 procedures (43 %), all directly managed during the same session. Remote arterial lesions occurred after 3 procedures (43 %); one underwent a second embolization, and the other 2 were observed conservatively. Puncture site complication was observed in only one procedure (14 %).

Conclusion

Embolization for vEDS is a safe and effective method to manage life-threatening arterial rupture.

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Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Interventional Radiology Department, Assistance Publique des Hôpitaux de Paris, Georges Pompidou European Hospital, 20–40, rue Leblanc, 75908, Paris Cedex 15, France

    Takuya Okada, Olivier Pellerin, Massimiliano Di Primio, Georgios Angelopoulos, Jean-Yves Pagny & Marc Sapoval

  2. Rare Vascular Diseases Reference Center, Assistance Publique des Hôpitaux de Paris, Georges Pompidou European Hospital, 20–40, rue Leblanc, 75908, Paris, France

    Michael Frank & Emmanuel Messas

  3. Anesthesia and Surgical Intensive Care Unit, Assistance Publique des Hôpitaux de Paris, Georges Pompidou European Hospital, 20–40, rue Leblanc, 75908, Paris, France

    Marie-Fazia Boughenou

  4. Faculté de Médicine, Sorbonne Paris Cité, Université Paris Descartes, Paris, France

    Olivier Pellerin, Massimiliano Di Primio, Georgios Angelopoulos & Marc Sapoval

Authors
  1. Takuya Okada
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  2. Michael Frank
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  3. Olivier Pellerin
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  4. Massimiliano Di Primio
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  5. Georgios Angelopoulos
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  6. Marie-Fazia Boughenou
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  7. Jean-Yves Pagny
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  8. Emmanuel Messas
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  9. Marc Sapoval
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Corresponding author

Correspondence to Takuya Okada.

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Okada, T., Frank, M., Pellerin, O. et al. Embolization of Life-Threatening Arterial Rupture in Patients with Vascular Ehlers–Danlos Syndrome. Cardiovasc Intervent Radiol 37, 77–84 (2014). https://doi.org/10.1007/s00270-013-0640-0

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  • DOI: https://doi.org/10.1007/s00270-013-0640-0

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