Ito Yoshiro, Satow Tetsu, Matsubara Hirofumi, Kataoka Hiroharu, Takahashi Jun C
Department of Neurosurgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan; Department of Neurosurgery, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan.
Department of Neurosurgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
World Neurosurg. 2019 Feb;122:123-128. doi: 10.1016/j.wneu.2018.10.158. Epub 2018 Nov 2.
Vascular Ehlers-Danlos syndrome (vEDS) is a vascular disease associated with a genetic collagen abnormality. It is characterized by vessel fragility, vessel rupture, and massive hemorrhage. Carotid-cavernous fistula (CCF) is the most frequent neurovascular complication of vEDS. However, CCF treatment using conventional diagnostic angiography and neuroendovascular therapy can result in a high rate of major complications.
We report a case of a right CCF in a 48-year-old man with vEDS. The carotid artery and jugular vein were exposed by direct neck dissection. To avoid systemic vascular complications, multiple catheters were inserted into the shunt segment via the carotid artery and jugular vein. This transarterial and transvenous multidevice technique enabled compact placement of coils in the shunt segment. The CCF was eliminated via selective shunt occlusion. Postoperative magnetic resonance imaging revealed occlusion of the right CCF.
Selective shunt occlusion via a transarterial and transvenous multidevice technique is a useful and safe approach for treating vEDS-associated CCF.
血管型埃勒斯-当洛综合征（vEDS）是一种与遗传性胶原蛋白异常相关的血管疾病。其特征为血管脆弱、血管破裂和大量出血。颈内动脉海绵窦瘘（CCF）是vEDS最常见的神经血管并发症。然而，使用传统诊断性血管造影和神经血管内治疗的CCF治疗可能导致高比例的严重并发症。
我们报告一例48岁患有vEDS的男性患者发生右侧CCF的病例。通过直接颈部解剖暴露颈动脉和颈静脉。为避免全身血管并发症，经颈动脉和颈静脉在分流段插入多根导管。这种经动脉和经静脉的多装置技术能够在分流段紧密放置弹簧圈。通过选择性分流闭塞消除了CCF。术后磁共振成像显示右侧CCF闭塞。
经动脉和经静脉多装置技术进行选择性分流闭塞是治疗vEDS相关CCF的一种有用且安全的方法。
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