Retinal Changes in Pediatric Glaucoma and Nonglaucomatous Optic Atrophy

Purpose To describe the incidence and significance of retinal changes in pediatric eyes with glaucoma and nonglaucomatous optic atrophy by optical coherence tomography (OCT). Design Retrospective observational case series. Methods OCT scans performed on children with either glaucoma or nonglaucomatous optic atrophy were retrospectively reviewed. Excluded were poor-quality scans and diagnoses of ocular hypertension or glaucoma suspects. Results Included were 227 eyes (227 children), 151 with glaucoma and 76 with nonglaucomatous optic atrophy. Outer retinal changes were present in 20 of 151 glaucoma eyes (13.2%) (12 prior intraocular surgery, 5 uveitis, 3 primary retinopathy) and 6 of 76 nonglaucomatous optic atrophy eyes (7.9%) (1 retinal vasculitis, 3 papilledema, 2 infiltrative optic neuropathy). Excluding eyes with outer retinal changes, isolated inner nuclear layer (INL) cysts were found in 6 of 131 eyes with glaucoma (4.6%) (3 uveitis, 1 prior hydrocephalus, 1 uveitis and pars plana vitrectomy, and 1 juvenile open-angle glaucoma) and 21 of 70 eyes with nonglaucomatous optic atrophy (30%) (5 optic neuritis, 11 anterior visual pathway tumors, 2 papilledema, 3 other) (P <.0001). Compared to eyes without INL cysts, those with INL cysts had thinner average retinal nerve fiber layer (RNFL) (78.2 ± 1.8 μm vs 52.0 ± 4.8 μm, P <.0001) and worse vision (logMAR = 0.33 ± 0.04 vs 0.65 ± 0.09, P <.002). Conclusions INL cysts are more frequent in pediatric nonglaucomatous optic atrophy than glaucoma; they are associated with worse vision and thinner RNFL. Outer retinal changes were exclusively seen in pathology that directly affected the retina. In children, INL cysts and/or outer retinal changes without prior history of intraocular surgery or uveitis should prompt further evaluation.