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Abstract：
BACKGROUND: Pediatric non-galenic pial arteriovenous fistulas (pAVFs) are rare vascular malformations that are characterized by a pial arterial-venous connection without an intervening capillary bed. Outcomes and treatment strategies for pAVFs are highly individualized, owing to the rarity of the disease and lack of large-scale data guiding optimal treatment approaches.
METHODS: We performed a systematic review of pediatric patients (< 18 years at diagnosis) diagnosed with a pAVF by digital subtraction angiogram (DSA). The demographics, treatment modalities, and outcomes were documented for each patient and clinical outcome data was collected. Descriptive information stratified by outcome scores were classified as follows: 1 = excellent (no deficit and full premorbid activity), 2 = good (mild deficit and full premorbid activity), 3 = fair (moderate deficit and impaired activity), 4 = poor (severe deficit and dependent on others), 5 = death.
RESULTS: A total of 87 studies involving 231 patients were identified. Median age at diagnosis was 3 years (neonates to 18 years). There was slight male preponderance (55.4%), and 150 subjects (81.1%*) experienced excellent outcomes after treatment. Of the 189 patients treated using endovascular approaches, 80.3% experienced excellent outcomes and of the 15 patients surgically treated subjects 75% had an excellent outcome. The highest rate of excellent outcomes was achieved in patients treated with Onyx (95.2%) and other forms of EvOH (100%). High output heart failure and comorbid vascular lesions tended to result in worse outcomes, with only 54.2% and 68% of subjects experiencing an excellent outcome, respectively. *Outcomes were reported in only 185 patients.
CONCLUSIONS: pAVFs are rare lesions, necessitating aggregation of patient data to inform natural history and optimal treatment strategies. This review summarizes the current literature on pAVF in children, where children presenting with heart failure as a result of high flow through the lesion were less likely to experience an excellent outcome. Prospective, large-scale studies would further characterize pediatric pAVFs and enable quantitative analysis of outcomes to inform best treatment practices.
METHODS: We performed a systematic review of pediatric patients (< 18 years at diagnosis) diagnosed with a pAVF by digital subtraction angiogram (DSA). The demographics, treatment modalities, and outcomes were documented for each patient and clinical outcome data was collected. Descriptive information stratified by outcome scores were classified as follows: 1 = excellent (no deficit and full premorbid activity), 2 = good (mild deficit and full premorbid activity), 3 = fair (moderate deficit and impaired activity), 4 = poor (severe deficit and dependent on others), 5 = death.
RESULTS: A total of 87 studies involving 231 patients were identified. Median age at diagnosis was 3 years (neonates to 18 years). There was slight male preponderance (55.4%), and 150 subjects (81.1%*) experienced excellent outcomes after treatment. Of the 189 patients treated using endovascular approaches, 80.3% experienced excellent outcomes and of the 15 patients surgically treated subjects 75% had an excellent outcome. The highest rate of excellent outcomes was achieved in patients treated with Onyx (95.2%) and other forms of EvOH (100%). High output heart failure and comorbid vascular lesions tended to result in worse outcomes, with only 54.2% and 68% of subjects experiencing an excellent outcome, respectively. *Outcomes were reported in only 185 patients.
CONCLUSIONS: pAVFs are rare lesions, necessitating aggregation of patient data to inform natural history and optimal treatment strategies. This review summarizes the current literature on pAVF in children, where children presenting with heart failure as a result of high flow through the lesion were less likely to experience an excellent outcome. Prospective, large-scale studies would further characterize pediatric pAVFs and enable quantitative analysis of outcomes to inform best treatment practices.
摘要：
背景：小儿非盖仑性静脉动静脉瘘（pAVFs）是罕见的血管畸形，其特征是无介入毛细血管床的静脉-静脉连接。pAVFs的结果和治疗策略是高度个性化的，由于该疾病的罕见性和缺乏指导最佳治疗方法的大规模数据。
方法：我们对通过数字减影血管造影（DSA）诊断为pAVF的儿科患者（诊断时<18岁）进行了系统评价。人口统计，治疗方式，记录每位患者的结局,并收集临床结局数据.按结果评分分层的描述性信息分类如下：1=优秀（无缺陷和完全病前活动），2=良好（轻度赤字和完全病前活动），3=一般(适度赤字和活动受损)，4=穷人(严重赤字和依赖他人)，5=死亡。
结果：共87项研究，涉及231例患者。诊断时的中位年龄为3岁（新生儿至18岁）。有轻微的男性优势（55.4％），150名受试者（81.1％*）在治疗后表现优异。在使用血管内方法治疗的189名患者中，80.3％的患者获得了出色的结果，在15例接受手术治疗的受试者中，75％的患者获得了出色的结果。在接受Onyx（95.2％）和其他形式的EvOH（100％）治疗的患者中，取得了最高的良好效果。高输出量心力衰竭和并发血管病变往往会导致更差的结果，只有54.2%和68%的受试者经历了优异的结果，分别。*结果仅在185例患者中报告。
结论：pAVFs是罕见的病变，需要汇总患者数据以告知自然史和最佳治疗策略。这篇综述总结了目前关于儿童pAVF的文献，由于高流量通过病变而出现心力衰竭的儿童不太可能获得出色的结果。前瞻性,大规模研究将进一步描述儿科pAVFs的特征,并能够对结局进行定量分析,从而为最佳治疗实践提供依据.
方法：我们对通过数字减影血管造影（DSA）诊断为pAVF的儿科患者（诊断时<18岁）进行了系统评价。人口统计，治疗方式，记录每位患者的结局,并收集临床结局数据.按结果评分分层的描述性信息分类如下：1=优秀（无缺陷和完全病前活动），2=良好（轻度赤字和完全病前活动），3=一般(适度赤字和活动受损)，4=穷人(严重赤字和依赖他人)，5=死亡。
结果：共87项研究，涉及231例患者。诊断时的中位年龄为3岁（新生儿至18岁）。有轻微的男性优势（55.4％），150名受试者（81.1％*）在治疗后表现优异。在使用血管内方法治疗的189名患者中，80.3％的患者获得了出色的结果，在15例接受手术治疗的受试者中，75％的患者获得了出色的结果。在接受Onyx（95.2％）和其他形式的EvOH（100％）治疗的患者中，取得了最高的良好效果。高输出量心力衰竭和并发血管病变往往会导致更差的结果，只有54.2%和68%的受试者经历了优异的结果，分别。*结果仅在185例患者中报告。
结论：pAVFs是罕见的病变，需要汇总患者数据以告知自然史和最佳治疗策略。这篇综述总结了目前关于儿童pAVF的文献，由于高流量通过病变而出现心力衰竭的儿童不太可能获得出色的结果。前瞻性,大规模研究将进一步描述儿科pAVFs的特征,并能够对结局进行定量分析,从而为最佳治疗实践提供依据.
