Department of Neurosurgery, Chang Gung University and Linkou Chang Gung Memorial Hospital, Taoyuan, Taiwan.
Department of Radiology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea.
J Neuroradiol. 2019 Nov;46(6):390-397. doi: 10.1016/j.neurad.2018.10.010. Epub 2018 Nov 16.
Multiply occurring intracranial dural arteriovenous fistulas (dAVFs) have been documented but rarely occur, and neither pathogenesis nor prognosis is clearly understood. This study was conducted to analyze angiographic characteristics of multiple dAVFs and to chronicle our treatment experience.
Between April, 2002 and January, 2018, data prospectively collected from 310 patients with intracranial dAVFs were systematically reviewed, assessing clinical and anatomic outcomes of endovascular treatment in 32 patients with multiple dAVFs (≥ 2 fistulas each). Lesions were categorized as multifocal or diffuse type, depending on presentation, and further characterized as progressive or non-progressive disease.
Overall, 18 patients (56.3%) experienced aggressive presentations, including intracerebral hemorrhage or venous infarction. Cortical venous reflux (CVR) was observed in 26 patients (81.3%), and sinus thrombosis or occlusion was seen in 24 (75.0%). Clinical outcomes in patients with multifocal fistulas (n = 11) were excellent (100%), marked by a moderately high rate of complete occlusion (54.5%). Those with progressive disease (n = 10) regularly displayed certain angiographic findings, namely diffuse configuration (100%), sinus thrombosis (100%), and CVR (100%). Complete anatomic obliteration was achieved in 12 patients (37.5%), and in 26 patients (81.3%), clinical outcomes were favorable.
Multiple dAVFs are typically aggressive at presentation, given strong associations with CVR and sinus thrombosis. In diffuse-type fistulas, the potential to recur or progress is high. Although definitive treatment poses a challenge, outcomes of endovascular therapeutics may be still optimized in this setting through strategic procedural modifications and careful follow-up monitoring.
多发颅内硬脑膜动静脉瘘（dAVF）已有相关记录，但极为罕见，其发病机制和预后均不清楚。本研究旨在分析多发 dAVF 的血管造影特征，并报告我们的治疗经验。
2002 年 4 月至 2018 年 1 月期间，前瞻性收集了 310 例颅内 dAVF 患者的数据，对 32 例（每个瘘管≥2 个）多发 dAVF 患者的血管内治疗的临床和解剖学结果进行了系统回顾。根据病变表现将病变分为局灶性或弥漫性，进一步分为进展性或非进展性疾病。
共有 18 例（56.3%）患者表现为侵袭性，包括颅内出血或静脉梗死。26 例（81.3%）患者存在皮质静脉反流（CVR），24 例（75.0%）患者存在窦血栓或闭塞。11 例局灶性瘘患者（n=11）的临床结局良好（100%），完全闭塞率较高（54.5%）。10 例进展性疾病患者（n=10）经常出现某些血管造影表现，即弥漫性形态（100%）、窦血栓形成（100%）和 CVR（100%）。12 例患者（37.5%）达到完全解剖学闭塞，26 例患者（81.3%）临床结局良好。
多发 dAVF 通常在出现时具有侵袭性，与 CVR 和窦血栓形成密切相关。弥漫性瘘管具有较高的复发或进展风险。尽管确定性治疗具有挑战性，但通过策略性手术修改和仔细的随访监测，这种情况下的血管内治疗效果仍可优化。
