Schievink W I, Piepgras D G, Earnest F, Gordon H
Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota.
J Neurosurg. 1991 Jun;74(6):991-8. doi: 10.3171/jns.1991.74.6.0991.
Spontaneous bilateral carotid-cavernous fistulae and cervical artery dissection is reported in a 20-year-old woman with Ehlers-Danlos syndrome Type IV. The clinical features of 16 previously published cases of spontaneous carotid-cavernous fistulae associated with Ehlers-Danlos syndrome Type IV are reviewed, for a total of 17 cases. The mean age of the 14 women and three men was 31.6 years. Only direct fistulae were encountered. Diagnostic neuroangiography carried morbidity and mortality rates of 36% and 12%, respectively; neuroradiological treatment resulted in death in one of six patients. The possible value of desmopressin in the management of these patients is discussed. In view of the risks of arterial puncture and surgery, the authors emphasize the importance of early recognition of Ehlers-Danlos syndrome.
一名患有IV型埃勒斯-当洛综合征的20岁女性被报道患有自发性双侧颈内动脉海绵窦瘘和颈动脉瘤夹层。回顾了16例先前发表的与IV型埃勒斯-当洛综合征相关的自发性颈内动脉海绵窦瘘病例的临床特征，共计17例。14名女性和3名男性的平均年龄为31.6岁。仅发现直接瘘。诊断性神经血管造影的发病率和死亡率分别为36%和12%；神经放射治疗导致6名患者中的1人死亡。讨论了去氨加压素在这些患者管理中的可能价值。鉴于动脉穿刺和手术的风险，作者强调早期识别埃勒斯-当洛综合征的重要性。
