title: Embolization of life-threatening arterial rupture in patients with vascular Ehlers-Danlos syndrome.
abstract: OBJECTIVE
  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 %).
  
  
  CONCLUSIONS
  Embolization for vEDS is a safe and effective method to manage life-threatening arterial rupture.
authors: Interventional Radiology Department, Assistance Publique des Hôpitaux de Paris, Georges Pompidou European Hospital, 20-40, rue Leblanc, 75908, Paris Cedex 15, France, okabone@gmail.com.
authors: Takuya
authors: T
authors: Okada
authors: Michael
authors: M
authors: Frank
authors: Olivier
authors: O
authors: Pellerin
authors: Massimiliano Di
authors: MD
authors: Primio
authors: Georgios
authors: G
authors: Angelopoulos
authors: Marie-Fazia
authors: MF
authors: Boughenou
authors: Jean-Yves
authors: JY
authors: Pagny
authors: Emmanuel
authors: E
authors: Messas
authors: Marc
authors: M
authors: Sapoval
mesh_terms: D000328:Adult; D001158:Arteries; D003287:Contrast Media; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D004621:Embolization, Therapeutic / Q000379:methods*; D005260:Female; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D012422:Rupture, Spontaneous / Q000628:therapy; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014652:Vascular Diseases / Q000000981:diagnostic imaging / Q000209:etiology / Q000628:therapy*
chemical_list: D003287:Contrast Media