Department of Neurosurgery, The Walton Centre, Lower Ln, Liverpool, L9 7LJ, United Kingdom.
Department of Neurosurgery, Western General Hospital, Crew Road, Edinburgh, EH4 2XU, United Kingdom.
Clin Neurol Neurosurg. 2020 Jun;193:105775. doi: 10.1016/j.clineuro.2020.105775. Epub 2020 Mar 3.
Vascular Ehlers-Danlos (vEDS) is a rare form of the Ehlers-Danlos Syndrome (EDS) where arterial fragility results from mutations in the gene that encodes type III collagen. The disease can lead to major neurological complications including carotico-cavernous fistulae (CCF), aneurysms of the Circle of Willis and endovascular procedures have an increased risk profile due to the delicate vasculature. Management of intracranial disease in vEDS requires an intricate understanding of the syndrome but is still associated with significant complications that lead to morbidity and mortality. As well as providing an approach to the management of neurovascular complications in vEDS, the relevant literature regarding nosology, aetiology and genetics of the condition is summarised here. Particular emphasis is placed on the two most common intracranial complications, namely carotico-cavernous fistulas and and cerebral aneurysms. Pros and cons of surgical and endovascular interventions are discussed and a technical discussion is concentrated on the surgical aspects of management.
血管型埃勒斯-当洛斯综合征（vEDS）是埃勒斯-当洛斯综合征（EDS）的一种罕见形式，其动脉脆弱性是由于编码 III 型胶原的基因突变引起的。该疾病可导致主要的神经并发症，包括颈动脉海绵窦瘘（CCF）、Willis 环动脉瘤，并且由于血管脆弱，血管内治疗的风险特征增加。vEDS 患者颅内疾病的管理需要对该综合征有深入的了解，但仍与导致发病率和死亡率的严重并发症相关。除了提供 vEDS 神经血管并发症管理方法外，本文还总结了该疾病的分类学、病因学和遗传学的相关文献。特别强调了两种最常见的颅内并发症，即颈动脉海绵窦瘘和脑动脉瘤。讨论了手术和血管内干预的优缺点，并集中讨论了管理的手术方面的技术问题。
