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Neuroendovascular Procedures in Patients with Ehlers-Danlos Type IV: Multicenter Case Series and Systematic Review

Varun Padmanaban1, Patricia P Yee2, Sravanthi Koduri3

  • 1Department of Neurosurgery, Penn State Milton S. Hershey Medical Center, Hershey, Pennsylvania, USA.

World Neurosurgery
|November 19, 2022
PubMed

Insights

Modern neuroendovascular procedures show high success rates for cerebrovascular diseases in vascular Ehlers-Danlos syndrome (vEDS) patients. However, careful management is crucial due to significant complication and mortality risks, particularly vascular injuries.

Area of Science:

  • Neurology
  • Vascular Surgery
  • Genetics

Background:

  • Vascular Ehlers-Danlos syndrome (vEDS) presents with severe vascular fragility due to type III procollagen defects.
  • Cerebrovascular conditions like dissections, aneurysms, and fistulae are prevalent in vEDS patients.
  • Previous endovascular treatments in vEDS were associated with high risks, often using outdated methods.

Approach:

  • A multi-institutional retrospective case series (3 centers) was combined with a systematic literature review.
  • Data from 2000-2021 were analyzed to assess neuroendovascular procedures in vEDS patients.
  • Sixty-six procedures across 59 vEDS patients were evaluated for safety and efficacy.

Key Points:

  • Direct cavernous carotid fistulas (DCCF) were the most common condition treated.
  • Neuroendovascular procedures achieved a 94% success rate.
  • The overall complication rate was 30%, with a 7.5% mortality rate.

Conclusions:

  • Neuroendovascular interventions can be successful for cerebrovascular diseases in vEDS.
  • High complication and mortality rates necessitate meticulous patient care.
  • Vascular access and procedural injuries remain critical challenges in vEDS treatment.
Abstract

Keywords:
AneurysmCavernous carotid fistulaComplicationsEhlers-Danlos type IVNeuroendovascular proceduresOutcomesVascular Ehlers-Danlos

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