Ophthalmological Evaluation in Children Presenting With a Primary Brain Tumor

Abstract

Background: Children with a brain tumor are prone to develop visual impairment, which to date is often underestimated and unrecognized. Our aim was to assess the prevalence of ophthalmological evaluation and abnormal ophthalmological findings, and investigate whether demographic and tumor-related characteristics are associated with abnormal ophthalmological findings in children presenting with a primary brain tumor. Methods: Medical records of all 90 children diagnosed with a primary brain tumor between June 2018 and May 2019 and treated at the Princess Máxima Center for Pediatric Oncology, a tertiary referral center in the Netherlands, were retrospectively reviewed. Univariate regression analysis was used to investigate associations between demographic, tumor-related and clinical characteristics, and abnormal ophthalmological findings. Results: Sixty children (34 male [56.7%]; median [range] age, 9.3 [0–16.9] years) underwent ophthalmological evaluation within 6 weeks before or after diagnosis, 11 children (5 male [45.5%]; median [range] age, 5.7 [0.1–17.2] years) were seen more than 6 weeks before or after diagnosis, and 19 children (7 male [36.8%]; median [range] age, 7.2 [1.9–16.6] years) did not receive ophthalmological evaluation within at least 6 months from diagnosis. A total of 19 children (21.1%) presented with visual symptoms as first sign leading to the diagnosis of a brain tumor. Children who presented with visual symptoms (odds ratio [OR], 22.52; 95% confidence interval [CI], 4.90–103.60) and/or hydrocephalus (OR, 3.60; 95% CI, 1.38–9.36) at diagnosis were more often seen for ophthalmological evaluation. The most common abnormal ophthalmological findings were eye movement disorders (66.0%), papilledema (44.1%), and visual field defects (58.1%). Eye movement disorders occurred more frequently in patients with an infratentorial tumor (OR, 4.71; 95% CI, 1.03–21.65). The risk of papilledema was associated with older age (OR, 1.19; 95% CI, 1.05–1.34), hydrocephalus (OR, 9.63; 95% CI, 2.68–34.61), and infratentorial (OR, 9.11; 95% CI, 1.77–46.78) and supratentorial (OR, 13.13; 95% CI, 1.92–89.52) tumors. Conclusions: In this study, most children with a primary brain tumor underwent ophthalmological evaluation around diagnosis, 21% of the children were not evaluated. The high prevalence of abnormal ophthalmological findings stresses the importance of early standardized ophthalmological evaluation to detect visual impairment and provide timely treatment to potentially prevent permanent visual loss.

Trends and Clinical Characteristics of Pediatric Patients Presenting to an Ophthalmology Emergency Department with an Initial Diagnosis of Optic Nerve Head Elevation

“…A study by Nuijts et al examining the ophthalmological findings in children presenting with primary brain tumors found that papilledema was present in nearly half (44.1%) of patients. 8 In our study, tumors represented only 1.9% of our overall population and 6.3% of the patients with papilledema; conversely, all 4 patients with intracranial tumors presented with papilledema. Our overall prevalence was also lower than the 15.8% of children with papilledema who were diagnosed with tumors in a study by Hyde et.…”

Ophthalmological Findings in Youths With a Newly Diagnosed Brain Tumor

“…These findings are in line with previous studies, although the exact prevalence numbers of the specific ophthalmological diagnoses slightly differ. In particular, the percentage of papilledema was higher (74%) 34 and lower (11%-44%) 12,13,19,[35][36][37] in previous studies, whereas the percentage of visual field defects was comparable (27%) 13 or higher (50%-58%) 12,34 in previous studies, and the percentage of decreased VA (50%-54%) 13,20 and strabismus (45%-60%) 12,13,19,20 was higher in previous studies. An explanation for these differences in prevalence numbers may be referral and selection bias in previous retrospective studies, as also suggested by some of the authors 12,13,19,20 ; these biases are feasible given the incomplete ophthalmological evaluation in a substantial proportion of the included children in those studies.…”

“…In particular, the percentage of papilledema was higher (74%) 34 and lower (11%-44%) 12,13,19,[35][36][37] in previous studies, whereas the percentage of visual field defects was comparable (27%) 13 or higher (50%-58%) 12,34 in previous studies, and the percentage of decreased VA (50%-54%) 13,20 and strabismus (45%-60%) 12,13,19,20 was higher in previous studies. An explanation for these differences in prevalence numbers may be referral and selection bias in previous retrospective studies, as also suggested by some of the authors 12,13,19,20 ; these biases are feasible given the incomplete ophthalmological evaluation in a substantial proportion of the included children in those studies. One study 20 only reported ophthalmological findings for children who initially presented to the ophthalmologist, which may explain the higher prevalence of abnormal ophthalmological findings in that study.…”

“…11 However, despite the high prevalence of visual impairment in children with a brain tumor, ophthalmological evaluation is not standard of care and only 48% to 67% are referred for ophthalmological evaluation. 12,13 Previous studies have focused particularly on subgroups of brain tumors (ie, optic pathway gliomas, craniopharyngiomas, and pineal region tumors) that are known to cause visual impairment. [14][15][16][17][18] Other studies have included children with all types of brain tumors but were primarily retrospective in nature, making them more prone to selection bias.…”

“…[14][15][16][17][18] Other studies have included children with all types of brain tumors but were primarily retrospective in nature, making them more prone to selection bias. 12,13,19,20 Prospective studies including larger numbers of patients with all types of brain tumors that investigate the visual function with standardized ophthalmological evaluation are lacking. Thus, we conducted a prospective, nationwide study of a cohort of consecutive youths with a newly diagnosed brain tumor in the Netherlands to assess the prevalence and types of abnormal ophthalmological findings.…”