Prevalence of Strabismus Among Children With Neurofibromatosis Type 1 Disease With and Without Optic Pathway Glioma.

Abstract

To evaluate the prevalence of strabismus in neurofibromatosis type 1 (NF-1) by comparing children with normal neuroimaging to those with optic pathway glioma. A retrospective data collection of all children with NF-1 with neuroimaging studies examined at a single medical center between 2000 and 2016. Of the 198 children with NF-1 reviewed, 109 (55%) were male, 121 (61%) had normal neuroimaging, and 77 (39%) had an optic pathway glioma. Mean age at presentation was 6.3 ± 4.7 years and mean follow-up was 4.8 ± 3.1 years. Strabismus was present in 29 (15%) children and was significantly more prevalent in children with NF-1 with optic pathway glioma (21 of 77 [27%]) than in those with normal neuroimaging (8 of 121 [7%], P < .001). Sensory strabismus was only found in children with optic pathway glioma, accounting for most cases (12 of 21 [57%]). A strong association between strabismus and optic pathway glioma is demonstrated by an odds ratio of 5.29 (P < .001). Children with NF-1 with optic pathway glioma have a 4.13 times higher relative risk of developing strabismus than children with NF-1 without it (P = .001). The direction of ocular misalignment in children with NF-1 with optic pathway glioma was not significantly different than that observed in children without optic pathway glioma (P = .197, Fisher's exact test). Only 5 (17%) children with NF-1 with strabismus (3 with optic pathway glioma) underwent corrective surgery to align their eyes. Optic pathway glioma in children with NF-1 is associated with an increased risk of strabismus that is often sensory. Although exotropia is the most common ocular misalignment associated with optic pathway glioma, the direction of strabismus cannot be used as an accurate predictor for its presence. [J Pediatr Ophthalmol Strabismus. 2019;56(1):19-22.].