Long-term visual outcomes of optic pathway gliomas in pediatric patients without neurofibromatosis type 1

Abstract

Sporadic optic pathway gliomas (OPGs) have been reported to cause more vision loss than OPGs associated with neurofibromatosis type-1, but long-term visual outcome data are limited. The purpose of this study was to report the visual outcomes of a cohort of pediatric patients with sporadic OPGs. This was a retrospective, cohort study at a tertiary care pediatric hospital and cancer institute. The study included all patients with sporadic OPGs evaluated from 1990 to 2014. The primary outcome was visual acuity at final follow-up. Secondary outcomes were risk factors for a poor visual outcome and the rate of progression. There were 59 pediatric patients included in the study. Median age at presentation was 2.5 years old and median follow-up was 5.2 years. In the worse eye at final follow-up, 16 patients (27 %) were 20/30 or better, 9 patients (15 %) were between 20/40 and 20/80, and 34 patients (58 %) were 20/100 or worse. In the better eye at final follow-up, 33 patients (56 %) were 20/30 or better, 11 patients (19 %) were between 20/40 and 20/80, and 15 patients (25 %) were 20/100 or worse. Risk factors for a poor visual outcome included younger age at presentation, optic nerve pallor, and tumor extent. Of the 54 patients (92 %) who received treatment, 40 (74 %) experienced disease progression during or after treatment. A majority of pediatric patients with sporadic OPGs had significant long-term visual impairment. In spite of treatment, tumor progression is common. Serial ophthalmic examinations with quantitative vision measurements are essential in the management of sporadic OPGs.

Visual outcomes after chemotherapy for optic pathway glioma in children with and without neurofibromatosis type 1: results of the International Society of Paediatric Oncology (SIOP) Low-Grade Glioma 2004 trial UK cohort

“…In our NF1 cohort, children with Dodge stage 3 tumours were seven times more likely to experience worsening VA and a poor final VA outcome despite treatment. Wan et al found that postchiasm sporadic OPGs were associated with a worse visual outcome for the less severely affected eye but not with the more severely affected eye 12. In our sporadic OPG cohort, posterior tumour extent was not found to be a significant prognostic factor.…”

Outcomes Following Proton Therapy for Pediatric Low-Grade Glioma

“…This finding resembles the endocrine results in the proton series reported by Greenberger et al 4 After irradiation, we observed a 1% rate of visual deterioration and a 2% rate of hearing loss, both of which compare favorably to outcomes reported after photon therapy in smaller series. [20][21][22] Recent studies have drawn attention to the risk of vasculopathy and brainstem necrosis after proton therapy. 9,23,24 In children undergoing photon therapy for LGG, the comparative data are modest.…”

Morning Glory Disc Anomaly Associated with Ipsilateral Optic Nerve and Chiasm Thickening: Three Cases and Review of the Literature

“…15 Sporadic optic gliomas have been reported to cause more serious long-term visual impairment than those associated with neurofibromatosis type 1. 16 Changes in tumor size and extension and visual acuity would prompt tumor-directed therapy to preserve vision in the contralateral eye. 11,15 Any relation between optic glioma and MGDA has yet to be elucidated.…”