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    Original Investigation
    August 2, 2019

    Referral for Ophthalmology Evaluation and Visual Sequelae in Children With Primary Brain Tumors

    Author Affiliations
    • 1Department of Ophthalmology, Stanford University School of Medicine, Stanford, California
    • 2Department of Pediatrics and Child Neurology, Loma Linda University Children’s Hospital, Loma Linda, California
    • 3Department of Pediatrics and Hematology and Oncology, Loma Linda University Children’s Hospital, Loma Linda, California
    • 4Department of Pediatrics and Hematology and Oncology, Children’s Hospital of Orange County, Orange, California
    • 5Department of Pediatrics and Neonatology, Loma Linda University Children’s Hospital, Loma Linda, California
    • 6Department of Ophthalmology and Neuro-ophthalmology and Pediatric Ophthalmology, Loma Linda University Medical Center, Loma Linda, California
    JAMA Netw Open. 2019;2(8):e198273. doi:10.1001/jamanetworkopen.2019.8273
    Key Points español 中文 (chinese)

    Question  In children with brain tumors, what is the referral rate to ophthalmology and what is the prevalence of visual sequelae and their association with tumor characteristics?

    Findings  In this cohort study of 141 children with primary brain tumors, only 68 (48%) were referred for comprehensive eye examination. While only 15% of those referred had visual symptoms, more than 90% had a variety of visual impairments including both afferent and efferent diseases, with visual field defects being the most common impairment among patients without symptoms.

    Meaning  Ophthalmologic evaluation is recommended for early diagnosis of visual sequelae in children with brain tumors to prevent permanent vision loss.


    Importance  Visual impairment in children with brain tumors has received limited attention, as most pediatric neuro-oncology clinical trials neither require ophthalmologic evaluation on enrollment nor monitor effects of treatment on visual function during and after treatment.

    Objective  To investigate ophthalmology referral patterns for children with primary brain tumors, the prevalence of visual sequelae, and the association between tumor characteristics and vision-related diagnoses.

    Design, Setting, and Participants  This retrospective cohort study included 141 children with primary brain tumors treated at Loma Linda University Children’s Hospital and Eye Institute, a university-based tertiary referral center, between January 2013 and September 2017. Data analysis was completed in March 2019.

    Intervention  Comprehensive ophthalmologic evaluation for children with primary brain tumors.

    Main Outcomes and Measures  Percentage of patients with ophthalmology evaluation, prevalence of abnormal ophthalmic findings, and their association with tumor characteristics.

    Results  A total of 141 children (73 [52%] male; median [range] age, 7 [0-18] years) with primary brain tumors were enrolled in this study. Seventy-three patients (41 [52%] male; median [range] age, 8 [0-17] years) never had formal ophthalmologic evaluation. Sixty-eight patients (32 [48%] male; median [range] age, 7 [0-18] years) were evaluated by 1 of 4 board-certified, fellowship-trained pediatric and/or neuro-ophthalmologists for any visual impairment over a total of 222 visits. Five-year overall survival for patients who had eye examination was not significantly different from those who did not (mean [SD] survival, 78.3% [6.2%] vs 84.9% [4.7%]). Median (range) time from tumor diagnosis to initial ophthalmologic evaluation was 9 (0-94) months. Only 10 of 68 children (15%) presented with visual symptoms at tumor diagnosis, while 61 of 68 (90%) had abnormal findings on examination, including strabismus (41 [60%]), visual acuity impairment (37 [54%]), amblyopia (26 [38%]), papilledema (24 [35%]), visual field defects (13 [19%]), optic atrophy (12 [18%]), and keratopathy (10 [15%]). Strabismus occurred more frequently in patients with posterior fossa tumors (26 of 68 in posterior fossa vs 15 of 68 in other locations; P = .02). The presence of visual field defects in patients with no visual symptoms was 15% (9 of 58). Radiation was significantly associated with amblyopia (odds ratio, 4.5; 95% CI, 1.2-15.7; P = .02).

    Conclusions and Relevance  In this study, more than 50% of children with primary brain tumors were not referred for ophthalmologic evaluation. Although visual symptoms were uncommon, visual impairments occurred more frequently than previously reported. Ophthalmologic evaluation is recommended to identify and manage visual impairment and prevent permanent vision loss in children with brain tumors.