Mitsuhashi Takashi, Miyajima Masakazu, Saitoh Rikizou, Nakao Yasuaki, Hishii Makoto, Arai Hajime
Department of Neurosurgery, Juntendo University School of Medicine, Tokyo, Japan.
Neurol Med Chir (Tokyo). 2004 Oct;44(10):548-53. doi: 10.2176/nmc.44.548.
A 30-year-old female complained of sudden onset of severe proptosis, chemosis, diplopia, and bruit. Right carotid angiography showed a high-flow direct carotid-cavernous fistula (CCF) draining into the engorged superior ophthalmic vein, inferior petrosal sinus, and pterygoid plexus. The patient experienced retroperitoneal bleeding from a ruptured right renal artery after undergoing cerebral angiography. We suspected Ehlers-Danlos syndrome (EDS) type IV, which was confirmed by showing cultured fibroblasts failed to secrete procollagen type III. Endovascular surgery cannot be considered the treatment method of choice in view of the fragility of the arteries and veins in patients with EDS type IV. We treated our patient with extracranial internal carotid artery ligation. Currently, there is no ideal treatment for CCF in patients with EDS type IV. Since CCF is rarely life-threatening, the investigative approach and course of treatment must consider the associated vascular fragility.
一名30岁女性主诉突然出现严重眼球突出、球结膜水肿、复视和杂音。右侧颈动脉血管造影显示存在高流量直接颈动脉海绵窦瘘（CCF），血液引流至扩张的眼上静脉、岩下窦和翼状静脉丛。该患者在接受脑血管造影后出现右肾动脉破裂导致的腹膜后出血。我们怀疑为IV型埃勒斯-当洛综合征（EDS），培养的成纤维细胞未能分泌III型前胶原证实了这一诊断。鉴于IV型EDS患者动静脉的脆弱性，血管内手术不能被视为首选治疗方法。我们对该患者进行了颅外颈内动脉结扎术。目前，对于IV型EDS患者的CCF尚无理想的治疗方法。由于CCF很少危及生命，研究方法和治疗过程必须考虑相关的血管脆弱性。
