Su Xin, Song Zihao, Ma Yongjie, Zhang Hongqi, Zhang Peng
Department of Neurosurgery, Xuanwu Hospital, Capital Medical University, No. 45 Changchun Street, Xicheng District, Beijing, 100053, China.
China International Neuroscience Institute (CHINA-INI), Beijing, 100053, China.
Neurosurg Rev. 2025 Jul 1;48(1):535. doi: 10.1007/s10143-025-03696-7.
Intracranial multiple dural arteriovenous fistulas (DAVFs) are rare, with most reports limited to small case series and basic descriptive analyses. To better understand this condition-often linked to lower cure rates and higher progression risk-we conducted a comparative analysis of their angiographic characteristics, clinical presentations, and outcomes.
Data were retrospectively collected from the Dural Arteriovenous Fistula Research and Management in China (DREAM-INI) database. Lesions were classified as multifocal or diffuse based on angiographic features, and as progressive or non-progressive based on follow-up angiographic findings. We compared these subtypes as well as multiple versus single DAVFs, and further investigated the predictors of disease progression in patients with multiple DAVFs.
A total of 90 cases of multiple synchronous DAVFs were included. Compared to the multifocal type, diffuse-type patients were younger (P = 0.002), and more frequently exhibited sinus occlusion/stenosis (P = 0.019), sinus-type DAVF (P < 0.001), pial artery supply (P = 0.009), venous congestion (P = 0.030), and lower complete obliteration rates (P = 0.001). Progressive DAVFs were associated with younger age (P = 0.001), sinus-type DAVF (P = 0.020), higher rates of diffuse-type lesions (P < 0.001), pial artery supply (P = 0.037), deep venous drainage (P = 0.032), and venous congestion (P = 0.005). Among the 14 treatment-related complications, 8 (57.1%) were associated with pial artery embolization. Compared to single DAVFs, patients with multiple DAVFs had a significantly lower rate of good outcomes (P < 0.001) and a higher incidence of fistula-related death (P = 0.001). On multivariate analysis, only younger age remained a significant predictor of progression (OR 2.86; 95% CI 1.02-7.99; P = 0.045).
Diffuse and progressive types often occur in younger patients and are associated with sinus-type fistulas and venous hypertension-related angioarchitectural features. Disease progression may involve enhanced neoangiogenesis. Treatment of refractory DAVFs should prioritize reducing venous hypertension and improving cerebral hemodynamics, rather than solely pursuing complete obliteration.
颅内多发性硬脑膜动静脉瘘(DAVF)较为罕见,大多数报道仅限于小病例系列和基本描述性分析。为了更好地了解这种通常与较低治愈率和较高进展风险相关的疾病,我们对其血管造影特征、临床表现和预后进行了对比分析。
数据来自中国硬脑膜动静脉瘘研究与管理(DREAM - INI)数据库,采用回顾性收集。根据血管造影特征将病变分为多灶性或弥漫性,根据随访血管造影结果分为进行性或非进行性。我们比较了这些亚型以及多发性与单发性DAVF,并进一步研究了多发性DAVF患者疾病进展的预测因素。
共纳入90例多发性同步DAVF患者。与多灶性类型相比,弥漫性类型患者更年轻(P = 0.002),更常出现静脉窦闭塞/狭窄(P = 0.019)、静脉窦型DAVF(P < 0.001)、软脑膜动脉供血(P = 0.009)、静脉淤血(P = 0.030),且完全闭塞率较低(P = 0.001)。进行性DAVF与年轻(P = 0.001)、静脉窦型DAVF(P = 0.020)、弥漫性病变发生率较高(P < 0.001)、软脑膜动脉供血(P = 0.037)、深部静脉引流(P = 0.032)和静脉淤血(P = 0.005)相关。在14例与治疗相关的并发症中,8例(57.1%)与软脑膜动脉栓塞有关。与单发性DAVF相比,多发性DAVF患者的良好预后率显著较低(P < 0.001),瘘相关死亡率较高(P = 0.001)。多因素分析显示,只有年轻仍然是进展的显著预测因素(OR 2.86;95%CI 1.02 - 7.99;P = 0.045)。
弥漫性和进行性类型常发生于年轻患者,与静脉窦型瘘及静脉高压相关的血管构筑特征有关。疾病进展可能涉及新生血管生成增强。难治性DAVF的治疗应优先降低静脉高压和改善脑血流动力学,而非单纯追求完全闭塞。