Remote vascular catastrophes after neurovascular interventional therapy for type 4 Ehlers-Danlos Syndrome

AJNR Am J Neuroradiol. 2000 May;21(5):974-6.

Abstract

Type 4 Ehlers-Danlos Syndrome (EDS 4) is the most malignant form of Ehlers-Danlos Syndrome, often accompanied by neurovasacular complications secondary to vessel dissection or aneurysms. The fragile nature of connective tissue in these patients makes exovascular and endovascular treatment hazardous. We have treated four patients with EDS 4 over the last 8 years by using neuroendovascular procedures. Two of these individuals suffered remote vascular injuries around the time of their procedures and ultimately died. The circumstances surrounding their deaths will make up the body of this report.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Aortic Dissection / diagnostic imaging
  • Carotid Artery Injuries / diagnostic imaging
  • Carotid-Cavernous Sinus Fistula / diagnostic imaging
  • Carotid-Cavernous Sinus Fistula / therapy*
  • Ehlers-Danlos Syndrome / diagnostic imaging
  • Ehlers-Danlos Syndrome / therapy*
  • Embolization, Therapeutic / instrumentation*
  • Fatal Outcome
  • Female
  • Humans
  • Iliac Artery / injuries
  • Iliac Artery / surgery
  • Radiography
  • Reoperation
  • Rupture, Spontaneous
  • Splenic Artery / injuries
  • Splenic Artery / surgery