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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.

Background: Neurofibromatosis 1 (NF1) has a significant impact on quality of life (QoL)., Objectives: To evaluate QoL in NF1 according to phenotype from the viewpoint of children and proxy., Methods: One hundred and forty families with a child aged between 8 and 16 years, seen consecutively at the National Academic Paediatric Referral Centre for NF1 for a phenotype evaluation, were contacted by mail. Families agreeing to participate were sent two questionnaires, the DISABKIDS for children and proxy and the cartoon version of the Children's Dermatology Life Quality Index (CDLQI). QoL scores were compared with those in other major diseases and were analysed according to age, gender and phenotype., Results: Eighty families agreed to participate, and 79 returned the questionnaires. Using DISABKIDS, NF1 had a higher impact on health-related QoL than asthma (mean+/-SD 75.18+/-18.22 vs. 79.78+/-13.41; P=0.005). The total score was more altered when assessed by proxy than by children (71.20+/-17.94 vs. 75.18+/-18.22; P=0.002). Orthopaedic manifestations, learning disabilities and presence of at least two plexiform neurofibromas were independently associated with a higher impact (P<0.01). The CDLQI score was slightly altered (11.3%). Dermatological signs, such as café-au-lait spots and freckling, did not have a significant impact., Conclusions: Orthopaedic manifestations, learning disabilities and plexiform neurofibromas are the main complications impacting on QoL during childhood NF1. QoL could be considered as an endpoint for intervention studies in this context.

Standard treatment of optic pathways gliomas consists of radiotherapy and surgery when feasible. Owing to the toxicity of irradiation, chemotherapy has emerged as an interesting therapeutic option, especially in young children. This study describes the neuropsychological profile of 27 children (aged between 1.5 and 15.7 years) with optic pathways gliomas treated with chemotherapy as first-line treatment. Eight of them also received radiotherapy as salvage treatment. Eight had neurofibromatosis type 1 (NF1). Intellectual outcome was preserved in children treated with chemotherapy only (mean=107+/-17) compared to children also receiving radiotherapy (mean IQ=88+/-24) or children having NF1 and treated with chemotherapy (mean IQ=80+/-13). Scores for abstract reasoning, mental arithmetic, chessboard/coding, perception, judgement of line orientation were lower in children irradiated than in those treated only by chemotherapy. Children with Nf1 showed subnormal IQ scores with marked impairment of short- and long-term memory. With respect to long-term neuropsychological outcome, our study shows that a chemotherapy-first strategy can preserve the intellectual outcome of these patients either by avoiding the need of radiotherapy or by delaying its use as much as possible.

Objective: To describe the neuropsychological functioning of children treated with surgery only for localized brain tumors in Dana-Farber Cancer Institute Protocol 92-077. Subsequent reports will describe the neuropsychological functioning of children treated with surgery and stereotactic radiation therapy on Dana-Farber Cancer Institute 92-077., Methods: The intellectual functioning of 106 patients was evaluated within 3 months after surgery. An in-depth assessment of the neuropsychological functioning, including an impairment index, was conducted for a subset of 77 school-age children (6-16 yr old) across six functional domains. Descriptive statistics were generated; binomial distribution analyses were performed to assess whether the proportion of individuals with impaired performance on each measure exceeded normative expectations. The impairment index assessed whether poor performance was attributable to a few children or reflected the performance of the cohort as a whole., Results: Although the Full Scale IQ was within normative expectations, the Verbal IQ was higher than the Performance IQ with 45% of individuals showing a significant discrepancy (P < 0.01) between these scales. There was an increased prevalence of poor performance for measures of motor output, verbal memory, and visuospatial organization. The distribution of the impairment index indicated moderate impairment across the school-age cohort rather than severe impairment in a few patients., Conclusion: The results document a moderate level of neuropsychological morbidity among children with brain tumors before stereotactic radiation therapy, presumably referable to the tumor itself and the surgery. The extent to which stereotactic radiation therapy may increase this burden will be assessed in follow-up studies evaluating the longitudinal neuropsychological data.