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

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• Previous endovascular treatments in vEDS were associated with high risks, often using outdated methods.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.