Department of Neurosurgery, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo New York, USA; Department of Neurosurgery, Gates Vascular Institute at Kaleida Health, Buffalo, New York, USA.
Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, USA.
World Neurosurg. 2023 Feb;170:226-235.e3. doi: 10.1016/j.wneu.2022.09.007. Epub 2022 Sep 7.
Non-galenic pial arteriovenous fistulas (NGPAVFs) are rare cerebrovascular pathologies accounting for only 1.6%-4.8% of all brain vascular malformations. We performed a comprehensive review of NGPAVF cases reported in the literature to further characterize their clinical patterns of presentation, angiographic features, management, clinical outcomes, and complications.
We searched PubMed, Google Scholar, and Embase from each database's earliest records to April 2022 for all relevant English language articles. A total of 3280 articles were screened to identify those that met prespecified inclusion criteria. Differences in clinical outcomes between children (≤18 years old) and adults (>18 years old) and those articles in which NGPAVFs were associated with the presence of a varix or a hemorrhage were statistically examined.
A total of 242 patients in 86 articles were included. The mean patient age was 18.51 ± 18.80 years. The male-to-female ratio was 1.44:1. Headache was the most common initial presentation (42.6%) in the study cohort. Hemorrhage occurred at a significantly higher frequency in adults (P = 0.004), whereas more children presented with congestive heart failure (P < 0.001). Surgical, endovascular, and combination therapy led to comparable rates of complete NGPAVF obliteration (86.8%, 85.2%, and 88.5%, respectively). Fifty-nine patients (24.4%) experienced a complication, ranging from minor neurological deficit to severe hemorrhage. The mortality rate for the overall cohort was 3.3%, and all deceased patients had a varix associated with their fistulas.
To our knowledge, we report the largest literature review describing the clinical course and characteristics of NGPAVFs. All treatment approaches resulted in favorable obliteration rates and overall patient outcomes.
非 Galenic 脑动静脉瘘（NGPAVF）是一种罕见的脑血管病变，仅占所有脑血管畸形的 1.6%-4.8%。我们对文献中报道的 NGPAVF 病例进行了全面回顾，以进一步描述其临床表现、血管造影特征、治疗、临床结局和并发症。
我们从每个数据库的最早记录开始，在 PubMed、Google Scholar 和 Embase 上搜索了所有相关的英文文献，以确定符合预定纳入标准的文献。对儿童（≤18 岁）和成人（>18 岁）之间的临床结局差异以及 NGPAVF 与静脉瘤或出血并存的文献进行了统计学分析。
共有 86 篇文献中的 242 例患者被纳入研究。患者的平均年龄为 18.51±18.80 岁。男女比例为 1.44:1。头痛是研究队列中最常见的首发症状（42.6%）。成人出血发生率明显较高（P=0.004），而更多的儿童出现充血性心力衰竭（P<0.001）。手术、血管内和联合治疗导致完全闭塞 NGPAVF 的比例相当（分别为 86.8%、85.2%和 88.5%）。59 例（24.4%）患者发生并发症，从轻微的神经功能缺损到严重出血不等。总体队列的死亡率为 3.3%，所有死亡患者的瘘管均与静脉瘤相关。
据我们所知，这是报告 NGPAVF 临床过程和特征的最大文献综述。所有治疗方法均导致了良好的闭塞率和总体患者结局。
