The Gilbert Family Neurofibromatosis Institute, Children's National Medical Center, Washington, DC2Department of Neurology, Children's National Medical Center, Washington, DC3Department of Ophthalmology, Children's National Medical Center, Washington, DC7.
Department of Oncology, Children's National Medical Center, Washington, DC7The Brain Tumor Institute, Children's National Medical Center, Washington, DC.
JAMA Ophthalmol. 2014 Mar;132(3):265-71. doi: 10.1001/jamaophthalmol.2013.7649.
Monitoring young children with optic pathway gliomas (OPGs) for visual deterioration can be difficult owing to age-related noncompliance. Optical coherence tomography (OCT) measures of retinal nerve fiber layer (RNFL) thickness have been proposed as a surrogate marker of vision but this technique is also limited by patient cooperation.
To determine whether measures of circumpapillary RNFL thickness, acquired with handheld OCT (HH-OCT) during sedation, can differentiate between young children with and without vision loss from OPGs.
DESIGN, SETTING, AND PARTICIPANTS: This cross-sectional analysis of a prospective observational study was conducted at a tertiary-care children's hospital. Children with an OPG (sporadic or secondary to neurofibromatosis type 1) who were cooperative for visual acuity testing, but required sedation to complete magnetic resonance imaging, underwent HH-OCT imaging of the circumpapillary RNFL while sedated.
Area under the curve of the receiver operating characteristic, sensitivity, specificity, positive predictive value, and negative predictive value of the average and quadrant-specific RNFL thicknesses.
Thirty-three children (64 eyes) met inclusion criteria (median age, 4.8 years; range, 1.8-12.6 years). In children with vision loss (abnormal visual acuity and/or visual field), RNFL thickness was decreased in all quadrants compared with the normal-vision group (P < .001 for all comparisons). Using abnormal criteria of less than 5% and less than 1%, the area under the curve was highest for the average RNFL thickness (0.96 and 0.97, respectively) compared with specific anatomic quadrants. The highest discrimination and predictive values were demonstrated for participants with 2 or more quadrants meeting less than 5% (sensitivity = 93.3; specificity = 97.9; positive predictive value = 93.3; and negative predictive value = 97.9) and less than 1% (sensitivity = 93.3; specificity = 100; positive predictive value = 100; and negative predictive value = 98.0) criteria.
Measures of RNFL thickness acquired with HH-OCT during sedation can differentiate between young children with and without vision loss from OPGs. For young children who do not cooperate with vision testing, HH-OCT measures may be a surrogate marker of vision. Longitudinal studies are needed to delineate the temporal relationship between RNFL decline and vision loss.
由于年龄相关的不配合，监测患有视神经胶质瘤（OPG）的幼儿视力恶化可能具有挑战性。视网膜神经纤维层（RNFL）厚度的光学相干断层扫描（OCT）测量已被提出作为视力的替代标志物，但该技术也受到患者合作的限制。
确定在镇静状态下使用手持 OCT（HH-OCT）获得的环周 RNFL 厚度测量值是否可以区分患有 OPG 的视力丧失和无视力丧失的幼儿。
设计、地点和参与者：这是一项在三级儿童医院进行的前瞻性观察性研究的横断面分析。患有 OPG（散发性或继发于神经纤维瘤病 1 型）的儿童，在进行视力测试时合作，但需要镇静才能完成磁共振成像，在镇静状态下接受环周 RNFL 的 HH-OCT 成像。
接受者操作特征曲线下面积、平均和象限特异性 RNFL 厚度的敏感性、特异性、阳性预测值和阴性预测值。
33 名儿童（64 只眼）符合纳入标准（中位年龄 4.8 岁；范围 1.8-12.6 岁）。在视力丧失的儿童（异常视力和/或视野）中，与正常视力组相比，所有象限的 RNFL 厚度均降低（所有比较均<0.001）。使用小于 5%和小于 1%的异常标准，平均 RNFL 厚度的曲线下面积最高（分别为 0.96 和 0.97），与特定解剖象限相比。对于符合 2 个或更多象限小于 5%（敏感性=93.3；特异性=97.9；阳性预测值=93.3；阴性预测值=97.9）和小于 1%（敏感性=93.3；特异性=100；阳性预测值=100；阴性预测值=98.0）标准的参与者，显示出最高的鉴别力和预测值。
镇静状态下使用 HH-OCT 获得的 RNFL 厚度测量值可区分患有 OPG 的视力丧失和无视力丧失的幼儿。对于不配合视力测试的幼儿，HH-OCT 测量值可能是视力的替代标志物。需要进行纵向研究来描绘 RNFL 下降与视力丧失之间的时间关系。
