Abstract：
Ehlers-Danlos type IV or vascular Ehlers-Danlos syndrome (vEDS) is a rare inherited disorder characterized by profound vascular fragility resulting from defective production of type III procollagen. Cerebrovascular diseases including spontaneous dissections, cerebral aneurysms, and cavernous carotid fistulae are common. Endovascular therapies in this patient population are known to be higher risk, although many studies (before 2000) involved older techniques and equipment. The purpose of this study is to investigate the safety and efficacy of modern neuroendovascular techniques in the treatment of cerebrovascular diseases in patients with vEDS.
We combined a multi-institutional retrospective case series at 3 quaternary-care centers with a systematic literature review of individual case reports and case series spanning 2000-2021 to evaluate the safety and efficacy of neuroendovascular procedure in patients with vEDS with cerebrovascular diseases.
Fifty-nine patients who underwent 66 neuroendovascular procedures were evaluated. Most of the patients had direct cavernous carotid fistulas (DCCF). Neuroendovascular procedures had a 94% success rate, with a complication rate of 30% and a mortality of 7.5%.
Neuroendovascular procedures can be performed with a high rate of success in the treatment of cerebrovascular diseases in patients with vEDS, although special care is required because complication rates and mortality are high. Access site and procedure-related vascular injuries remain a significant hurdle in treating vEDS with cerebrovascular diseases, even with modern techniques.
We combined a multi-institutional retrospective case series at 3 quaternary-care centers with a systematic literature review of individual case reports and case series spanning 2000-2021 to evaluate the safety and efficacy of neuroendovascular procedure in patients with vEDS with cerebrovascular diseases.
Fifty-nine patients who underwent 66 neuroendovascular procedures were evaluated. Most of the patients had direct cavernous carotid fistulas (DCCF). Neuroendovascular procedures had a 94% success rate, with a complication rate of 30% and a mortality of 7.5%.
Neuroendovascular procedures can be performed with a high rate of success in the treatment of cerebrovascular diseases in patients with vEDS, although special care is required because complication rates and mortality are high. Access site and procedure-related vascular injuries remain a significant hurdle in treating vEDS with cerebrovascular diseases, even with modern techniques.
摘要：
背景：Ehlers-DanlosIV型或血管性Ehlers-Danlos综合征（vEDS）是一种罕见的遗传性疾病，其特征是由III型前胶原产生缺陷引起的严重血管脆性。脑血管疾病，包括自发性夹层，脑动脉瘤,海绵状颈动脉瘘很常见。已知该患者人群的血管内治疗风险较高，尽管许多研究（2000年之前）涉及较旧的技术和设备。目的探讨现代神经血管内技术治疗vEDS患者脑血管疾病的安全性和有效性。
方法：我们将3个四级护理中心的多机构回顾性病例系列与个体病例报告和2000-2021年病例系列的系统文献综述相结合，以评估vEDS合并脑血管疾病患者神经血管内手术的安全性和有效性。
结果：对59例接受了66例神经血管内手术的患者进行了评估。大多数患者患有直接海绵状颈动脉瘘（DCCF）。神经血管内手术成功率为94%,并发症发生率为30％，死亡率为7.5％。
结论：神经血管内手术在治疗vEDS患者的脑血管疾病中可以获得很高的成功率，尽管由于并发症发生率和死亡率很高，因此需要特别护理。介入部位和手术相关的血管损伤仍然是治疗vEDS伴脑血管疾病的重要障碍。即使是现代技术。
方法：我们将3个四级护理中心的多机构回顾性病例系列与个体病例报告和2000-2021年病例系列的系统文献综述相结合，以评估vEDS合并脑血管疾病患者神经血管内手术的安全性和有效性。
结果：对59例接受了66例神经血管内手术的患者进行了评估。大多数患者患有直接海绵状颈动脉瘘（DCCF）。神经血管内手术成功率为94%,并发症发生率为30％，死亡率为7.5％。
结论：神经血管内手术在治疗vEDS患者的脑血管疾病中可以获得很高的成功率，尽管由于并发症发生率和死亡率很高，因此需要特别护理。介入部位和手术相关的血管损伤仍然是治疗vEDS伴脑血管疾病的重要障碍。即使是现代技术。
