Avery Robert A, Hardy Kristina K
Department of Neurology, Children's National Medical Center, 111 Michigan Ave, NW, Washington, DC, 20010, USA,
J Neurooncol. 2014 Jan;116(2):341-7. doi: 10.1007/s11060-013-1300-6. Epub 2013 Nov 7.
Children with optic pathway gliomas (OPGs) frequently experience vision loss from their tumors. Most pediatric OPG research has focused on radiographic and visual outcomes, yet the impact of vision loss on quality of life (QOL) in children with OPGs has not been studied. The present study prospectively recruited children ≤ 10 years of age with sporadic or neurofibromatosis type 1 (NF1)-related OPGs. Vision specific QOL was assessed by parent proxy using the Children's Visual Function Questionnaire (CVFQ), and scores were analyzed according to magnitude of visual acuity (VA) loss and presence of visual field (VF) loss. Thirty-six subjects completed the study (53 % female) with median age of 4.6 years. Children with mild, moderate and severe vision loss have lower CVFQ subscale scores, indicating a lower vision specific QOL, compared to those with normal vision. Lower Competence scores were noted in participants with more profound vision loss (p < 0.05), reflecting a decreased ability to complete activities of daily living (e.g., feeding, grooming). Children with two visually impaired eyes were rated as having greater difficulty with social interactions and pleasurable activities (Personality subscale, p = 0.039) compared to those with only one impaired eye. In summary, our findings demonstrate that children with vision loss secondary to their OPG have a decreased vision specific QOL compared to those with normal vision. Measuring vision specific QOL may be considered a meaningful secondary outcome measure for pediatric OPG clinical trials.
患有视路胶质瘤（OPG）的儿童经常因肿瘤而出现视力丧失。大多数儿科OPG研究都集中在影像学和视觉结果上，然而，视力丧失对OPG儿童生活质量（QOL）的影响尚未得到研究。本研究前瞻性招募了年龄≤10岁的散发性或1型神经纤维瘤病（NF1）相关OPG儿童。使用儿童视觉功能问卷（CVFQ）通过家长代理评估特定视力的生活质量，并根据视力（VA）丧失程度和视野（VF）丧失情况分析得分。36名受试者完成了研究（53%为女性），中位年龄为4.6岁。与视力正常的儿童相比，轻度、中度和重度视力丧失的儿童CVFQ子量表得分较低，表明特定视力的生活质量较低。视力丧失更严重的参与者的能力得分较低（p<0.05），反映出完成日常生活活动（如进食、梳理）的能力下降。与只有一只眼睛视力受损的儿童相比，双眼视力受损的儿童在社交互动和娱乐活动方面被评为困难更大（个性子量表，p=0.039）。总之，我们的研究结果表明，与视力正常的儿童相比，因OPG继发视力丧失的儿童特定视力的生活质量下降。测量特定视力的生活质量可被视为儿科OPG临床试验中有意义的次要结局指标。
