Background Craniocervical junction (CCJ) arteriovenous fistulas (AVFs) are rare. The current treatment strategies for AVFs with different angioarchitecture need to be clarified. The present study aimed to analyse the correlation between angioarchitecture and clinical characteristics, share our experience in treating this disease and identify risk factors associated with subarachnoid haemorrhage (SAH) and poor outcomes. Methods A total of 198 consecutive patients with CCJ AVFs from our neurosurgical centre were retrospectively reviewed. The patients were grouped according to their clinical manifestations, and their baseline clinical characteristics, angioarchitecture, treatment strategies and outcomes were summarised. Results The patients’ median age was 56 years (IQR 47–62 years). The majority of patients were men with 166 (83.8%) patients. The most common clinical manifestation was SAH (52.0%), followed by venous hypertensive myelopathy (VHM) (45.5%). The most common CCJ AVFs type was dural AVF, with 132 (63.5%) fistulas. The most frequent fistula location was C-1 (68.7%) and dural branch of vertebral artery (70.2%) was the most involved arterial feeders for fistulas. The most common direction of venous drainage was descending intradural drainage (40.9%), followed by ascending intradural drainage (36.5%). Microsurgery was the most common treatment strategy applied for 151 (76.3%) patients, 15 (7.6%) patients were treated with interventional embolisation only, and 27 (13.6%) received both interventional embolisation and microsurgical treatment. The learning curve for microsurgery only was analysed by cumulative summation method, and the turning point was the 70th case, and blood loss in post-group was lower than that in pre-group (p=0.034). At the last follow-up, there were 155 (78.3%) patients with favourable outcomes (modified Rankin Scale(mRS)<3). Age≥56 (OR 2.038, 95% CI 1.039 to 3.998, p=0.038), VHM as the clinical manifestation (OR 4.102, 95% CI 2.108 to 7.982, p<0.001) and pretreatment mRS≥3 (OR 3.127, 95% CI 1.617 to 6.047, p<0.001) were significantly associated with poor outcomes. Conclusion The arterial feeders and direction of the venous drainage were important factors in the clinical presentations. The location of fistula and drainage vein was essential for choosing different treatment strategies. Older age, VHM onset and poor pretreatment functional status predicted poor outcomes. Data are available upon reasonable request.

中文翻译：

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颅颈交界处动静脉瘘的临床特征、治疗策略和结局：193例患者的队列研究

背景 颅颈交界处 (CCJ) 动静脉瘘 (AVF) 很少见。目前针对不同血管结构的 AVF 的治疗策略需要明确。本研究旨在分析血管结构与临床特征之间的相关性，分享我们治疗该疾病的经验，并确定与蛛网膜下腔出血（SAH）和不良预后相关的危险因素。方法对我院神经外科中心连续198例CCJ AVF患者进行回顾性分析。根据患者的临床表现对患者进行分组，总结他们的基线临床特征、血管结构、治疗策略和结果。结果 患者的中位年龄为 56 岁（IQR 47-62 岁）。大多数患者为男性，共有 166 名患者（83.8%）。最常见的临床表现是SAH（52.0%），其次是静脉性高血压脊髓病（VHM）（45.5%）。最常见的 CCJ AVF 类型是硬脑膜 AVF，有 132 个（63.5%）瘘管。最常见的瘘管部位是C-1（68.7%），椎动脉硬脑膜分支（70.2%）是瘘管受累最多的动脉供血来源。最常见的静脉引流方向是下行硬膜内引流（40.9%），其次是上行硬膜内引流（36.5%）。显微手术是最常见的治疗策略，151 例（76.3%）患者中，15 例（7.6%）患者仅接受介入栓塞治疗，27 例（13.6%）患者同时接受介入栓塞和显微手术治疗。仅采用累积求和法分析显微手术的学习曲线，转折点为第70例，组后失血量低于组前（p=0.034）。末次随访时，有 155 例（78.3%）患者预后良好（改良 Rankin 量表（mRS）<3）。年龄≥56岁（OR 2.038，95% CI 1.039至3.998，p=0.038），VHM为临床表现（OR 4.102，95% CI 2.108至7.982，p<0.001）且治疗前mRS≥3（OR 3.127，95%） CI 1.617 至 6.047，p<0.001）与不良结果显着相关。结论 动脉供血和静脉引流方向是影响临床表现的重要因素。瘘管和引流静脉的位置对于选择不同的治疗策略至关重要。年龄较大、VHM 发病和治疗前功能状态不佳预示着结果不佳。数据可根据合理要求提供。