1Department of Neurosurgery, University of Southern California, Los Angeles, California; and.
2Department of Neurosurgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
J Neurosurg Pediatr. 2023 Feb 24;31(5):469-475. doi: 10.3171/2023.1.PEDS22310. Print 2023 May 1.
The assessment of hemorrhagic risk is of central importance in the management of pediatric brain arteriovenous malformations (AVMs). A recently published scoring system, the R2eD AVM (race, exclusive deep location or not, AVM size < 3 or ≥ 3, venous drainage exclusively deep or not, and monoarterial feeding or >1 arterial feeders) considers patient race and AVM location, size, venous drainage, and arterial supply and was successfully externally validated for use in adults to predict the risk of AVM hemorrhagic presentation. In this study the authors sought to externally validate the R2eD AVM score for prediction of hemorrhagic risk in the pediatric AVM population.
A pediatric database at Boston Children's Hospital was retrospectively reviewed for all patients with a diagnosis of intracranial AVM. Exclusion criteria included age > 21 years, multiple AVMs, and incomplete clinical/radiographic data. Demographic data and R2eD AVM score calculations were collected for all patients. Univariate binary logistic regression and multivariate stepwise backward elimination models were used to determine factors associated with hemorrhagic presentation.
A total of 212 AVMs were identified in 212 patients with vascular anomalies from 1995 to 2021, and 144 patients met the inclusion criteria (74 [51.4%] male, 111 [77.1%] White), of whom 87 (60.4%) patients presented with rupture and 122 (84.7%) underwent resection. The mean age at surgery was 12 years (range 3 months to 20 years). The R2eD AVM scoring system was applied to each patient. The score components of size < 3 cm and exclusive deep venous drainage were significantly associated with rupture risk (p < 0.05). The complete model for the R2eD AVM score had an area under the curve of 0.671 (95% CI 0.586-0.757).
This study demonstrated poor external validity of the R2eD AVM score in predicting pediatric AVM rupture risk. The results suggest that future studies are warranted to determine a better scoring method to capture pediatric rupture risk, given the significant differences in clinical presentation in pediatric compared with adult AVM patients.
在小儿脑动静脉畸形（AVM）的治疗中，出血风险的评估至关重要。最近发表的 R2eD AVM 评分系统（种族、是否为深部单一病灶、AVM 大小＜3cm 或≥3cm、静脉引流是否仅限于深部、单支或多支动脉供血）考虑了患者的种族和 AVM 位置、大小、静脉引流和动脉供血，并成功在成人中进行了外部验证，用于预测 AVM 出血表现的风险。本研究旨在对 R2eD AVM 评分在预测儿科 AVM 人群出血风险方面的有效性进行外部验证。
对波士顿儿童医院的儿科数据库进行回顾性分析，纳入所有颅内 AVM 诊断患者。排除标准包括年龄＞21 岁、多发 AVM 和临床/影像学资料不完整。收集所有患者的人口统计学数据和 R2eD AVM 评分计算结果。采用单因素二项逻辑回归和多因素逐步向后消除模型确定与出血表现相关的因素。
共纳入 1995 年至 2021 年间血管畸形患者的 212 个 AVM，共 212 例患者，其中 144 例符合纳入标准（男性 74 例[51.4%]，白人 111 例[77.1%]），其中 87 例（60.4%）患者破裂出血，122 例（84.7%）患者接受了手术切除。手术时的平均年龄为 12 岁（3 个月至 20 岁）。每位患者均应用 R2eD AVM 评分系统进行评分。AVM 体积＜3cm 和静脉引流仅限于深部是与破裂风险相关的显著评分组成部分（p＜0.05）。R2eD AVM 评分的完整模型曲线下面积为 0.671（95%置信区间 0.586-0.757）。
本研究表明，R2eD AVM 评分系统在预测小儿 AVM 破裂风险方面的外部有效性较差。鉴于小儿 AVM 患者与成人 AVM 患者临床表现存在显著差异，需要进一步研究以确定更好的评分方法来捕捉小儿破裂风险。
