Outcomes of chemo versus no therapy for optic pathway glioma in NF1
Reviewed by Fiona Rowe

The aim of this study was to evaluate the visual outcomes of a cohort of children with optic pathway gliomas and neurofibromatosis type 1 (NF1), with comparison of children treated with chemotherapy or not. This was a retrospective study of 58 children; 24 treated with chemotherapy. Age at diagnosis was 4.3 ±3.4 years; 35 females and 23 males. Glioma stage was stage 1 for 27 children, stage 2 for 14 and stage 3 for 17 children. Visual signs were observed by 21 children including visual acuity (VA) loss, strabismus and proptosis. Overall best corrected visual acuity (BCVA) at diagnosis was 0.4 ±0.8 logMAR. In the no treatment group, BCVA improved by 0.08 logMAR (pre VA of 0.2 ±0.3 improving to post VA of 0.1 ±0.5) whilst in the chemo group, BCVA worsened by 0.48 logMAR (pre VA of 0.8 ±1.3 reducing to post VA of 1.3 ±1.3). Overall, 30 eyes showed loss in mean thickness of the peripapillary retinal nerve fibre layer: 17 eyes in the chemo group. Worse outcomes were expected in the chemo group as these children had more progressed disease. However, chemotherapy halted any further progression of visual loss in three quarters of this group. Chemotherapy had a good safety profile in this study.

Visual outcomes of optic pathway glioma treated with chemotherapy in neurofibromatosis type I.
Jose P, Couceiro R, Passos J, Teizeira FJ.
JOURNAL OF PEDIATRIC OPHTHALMOLOGY AND STRABISMUS
2022;59(2):128-35.