Visual impairment in children with a brain tumor: a prospective nationwide multicenter study using standard visual testing and optical coherence tomography (CCISS study)

BACKGROUND: Children with a brain tumor have a high risk of impaired vision. Up to now, visual acuity measurement, visual field testing and orthoptic testing are the most informative diagnostic investigations for the assessment of visual function. Evaluating vision in children can be challenging given the challenges in cooperation, concentration and age-dependent shifts in visual tests. Since visual loss due to a brain tumor can be progressive and irreversible, we must aim to detect visual impairment as early as possible. Several studies have shown that optical coherence tomography facilitates discovery of nerve fiber damage caused by optic nerve glioma. Consequently, early detection of potential ocular damage will effect treatment decisions and will provide timely referral to visual rehabilitation centers. METHODS/DESIGN: The CCISS study is a prospective, observational, multicenter cohort study in The Netherlands. Patients aged 0–18 years with a newly diagnosed brain tumor are invited for inclusion in this study. Follow-up visits are planned at 6, 12, 18 and 24 months. Primary endpoints are visual acuity, visual field and optical coherence tomography parameters (retinal nerve fiber layer thickness and ganglion cell layer – inner plexiform layer thickness). Secondary endpoints include the course of visual function (measured by visual acuity, visual field and optical coherence tomography at different follow-up visits), course of the disease and types of treatment. DISCUSSION: The CCISS study will heighten the awareness of visual impairment in different types of brain tumors in children. This study will show whether optical coherence tomography leads to earlier detection of visual impairment compared to standard ophthalmological testing (i.e. visual acuity, visual field testing) in children with a brain tumor. Furthermore, the systematic approach of ophthalmological follow-up in this study will give us insight in the longitudinal relation between the course of visual function, course of the disease and types of treatment in children with a brain tumor. TRIAL REGISTRATION: The CCISS study is prospectively registered in the Netherlands Trial Register (NTR) since April 2019. Identifier: NL7697.