1Department of Neuroradiology, University Hospital of Orléans.
2Department of Neuroradiology, Pitié-Salpêtrière University Hospital, Sorbonne Université, Paris.
Neurosurg Focus. 2024 Mar;56(3):E9. doi: 10.3171/2024.1.FOCUS23748.
The pathogenesis of intracranial dural arteriovenous fistulas (icDAVFs) is controversial. Cerebral vein thrombosis (CVT) and venous hypertension are recognized predisposing factors. This study aimed to evaluate the incidence of association between icDAVF and CVT and describe baseline aggressiveness and clinical outcomes for icDAVFs associated with CVT. The authors also performed a literature review of studies reporting icDAVF associated with CVT.
Two hundred sixty-three consecutive patients in two university hospitals with confirmed icDAVFs were included. A double-blind imaging review was performed to determine the presence or absence of CVT close or distant to the icDAVF. Location, type (using the Cognard classification), aggressiveness of the icDAVF, clinical presentation, treatment modality, and clinical and/or angiographic outcomes at 6 months were also collected. All prior brain imaging was analyzed to determine the natural history of onset of the icDAVF.
Among the 263 included patients, 75 (28.5%) presented with a CVT concomitant to their icDAVF. For 18 (78.3%) of 23 patients with previous brain imaging available, CVT preceding the icDAVF was proven (6.8% of the overall population). Former/active smoking (OR 2.0, 95% CI 1.079-3.682, p = 0.022) and prothrombogenic status (active inflammation or cancer/coagulation trouble) were risk factors for CVT associated with icDAVF (OR 3.135, 95% CI 1.391-7.108, p = 0.003). One hundred eighty-seven patients (71.1%) had a baseline aggressive icDAVF, not linked to the presence of a CVT (p = 0.546). Of the overall population, 11 patients (4.2%) presented with spontaneous occlusion of their icDAVF at follow-up. Seven patients (2.7%) died during the follow-up period. Intracranial DAVF + CVT was not associated with a worse prognosis (modified Rankin Scale score at 3-6 months: 0 [interquartile range {IQR} 0-1] for icDAVF + CVT vs 0 [IQR 0-0] for icDAVF alone; p = 0.055).
This was one of the largest studies focused on the incidence of CVT associated with icDAVF. For 6.8% of the patients, a natural history of CVT leading to icDAVF was proven, corresponding to 78.3% of patients with previous imaging available. This work offers further insights into icDAVF pathophysiology, aiding in identifying high-risk CVT patients for long-term follow-up imaging. Annual imaging follow-up using noninvasive vascular imaging (CT or MR angiography) for a minimum of 3 years after the diagnosis of CVT should be considered in high-risk patients, i.e., smokers and those with prothrombogenic status.
颅内硬脑膜动静脉瘘（icDAVF）的发病机制存在争议。脑静脉血栓形成（CVT）和静脉高压被认为是易患因素。本研究旨在评估 icDAVF 与 CVT 之间关联的发生率，并描述与 CVT 相关的 icDAVF 的基线侵袭性和临床结局。作者还对报告 icDAVF 与 CVT 相关的文献进行了综述。
在两所大学医院纳入了 263 例确诊为 icDAVF 的连续患者。进行了双盲影像学检查，以确定 icDAVF 附近或远处是否存在 CVT。还收集了 icDAVF 的位置、类型（使用 Cognard 分类）、侵袭性、临床表现、治疗方式以及 6 个月时的临床和/或血管造影结局。分析所有既往脑影像学检查以确定 icDAVF 发病的自然史。
在 263 例纳入患者中，75 例（28.5%）并发 icDAVF 出现 CVT。对于 23 例有既往脑影像学检查的患者中的 18 例（78.3%），证实 CVT 先于 icDAVF 发生（占总体人群的 6.8%）。既往/现吸烟（比值比 2.0，95%置信区间 1.079-3.682，p = 0.022）和促血栓形成状态（活跃炎症或癌症/凝血障碍）是与 icDAVF 相关的 CVT 的危险因素（比值比 3.135，95%置信区间 1.391-7.108，p = 0.003）。187 例（71.1%）患者基线时存在侵袭性 icDAVF，但与 CVT 无关（p = 0.546）。在总体人群中，11 例（4.2%）患者在随访时出现 icDAVF 自发性闭塞。7 例（2.7%）患者在随访期间死亡。icDAVF + CVT 并不与预后较差相关（改良 Rankin 量表评分在 3-6 个月：icDAVF + CVT 为 0[四分位距 {IQR} 0-1]，icDAVF 单独为 0[IQR 0-0]；p = 0.055）。
这是研究 icDAVF 与 CVT 发生率的最大研究之一之一。对于 6.8%的患者，证实了 CVT 导致 icDAVF 的自然史，这相当于有既往影像学检查的患者的 78.3%。这项工作为 icDAVF 病理生理学提供了进一步的见解，有助于识别有 CVT 长期随访影像学检查风险的高危患者。对于高风险患者（即吸烟者和具有促血栓形成状态的患者），应考虑在 CVT 诊断后至少 3 年内使用非侵入性血管成像（CT 或 MR 血管造影）进行年度影像学随访。
