Abstract
PurposeTo evaluate structural grading and quantitative segmentation of foveal hypoplasia using handheld OCT, versus preferential looking (PL), as predictors of future vision in preverbal children with infantile nystagmus.DesignLongitudinal cohort study.ParticipantsForty-two patients with infantile nystagmus (19 with albinism, 17 with idiopathic infantile nystagmus, and 6 with achromatopsia) were examined.MethodsSpectral-domain handheld OCT was performed in preverbal children up to 36 months of age. Foveal tomograms were graded using our 6-point grading system for foveal hypoplasia and were segmented for quantitative analysis: photoreceptor length, outer segment (OS) length, and foveal developmental index (FDI; a ratio of inner layers versus total foveal thickness). Patients were followed up until they could perform chart visual acuity (VA) testing. Data were analyzed using linear mixed regression models. Visual acuity predicted by foveal grading was compared with prediction by PL, the current gold standard for visual assessment in infants and young children.Main Outcome MeasuresGrade of foveal hypoplasia, quantitative parameters (photoreceptor length, OS length, FDI), and PL VA were obtained in preverbal children for comparison with future chart VA outcomes.ResultsWe imaged 81 eyes from 42 patients with infantile nystagmus of mean age 19.8 months (range, 0.9–33.4 months; standard deviation [SD], 9.4 months) at the first handheld OCT scan. Mean follow-up was 44.1 months (range, 18.4–63.2 months; SD, 12.0 months). Structural grading was the strongest predictor of future VA (grading: r = 0.80, F = 67.49, P < 0.0001) compared with quantitative measures (FDI: r = 0.74, F = 28.81, P < 0.001; OS length: r = 0.65; F = 7.94, P < 0.008; photoreceptor length: r = 0.65; F = 7.94, P < 0.008). Preferential looking was inferior to VA prediction by foveal grading (PL: r = 0.42, F = 3.12, P < 0.03).ConclusionsHandheld OCT can predict future VA in infantile nystagmus. Structural grading is a better predictor of future VA than quantitative segmentation and PL testing. Predicting future vision may avert parental anxiety and may optimize childhood development.
Highlights
To evaluate structural grading and quantitative segmentation of foveal hypoplasia using handheld OCT, versus preferential looking (PL), as predictors of future vision in preverbal children with infantile nystagmus
Thomas et al[10] proposed a structural grading scheme for foveal hypoplasia based on OCT data from adults and older children that has become a widely used scheme, with grades 1 to 4 representing the most to least developed fovea, whereas the atypical grade represents disruption of the photoreceptors seen in achromatopsia
Secondary aims were (1) to assess whether structural grading is comparable with quantitative segmentation in predicting future vision, (2) to compare the predictive power of the grading system with PL visual acuity (VA) results obtained on first examination, and (3) to determine whether the grade remains stable over time
Summary
Purpose: To evaluate structural grading and quantitative segmentation of foveal hypoplasia using handheld OCT, versus preferential looking (PL), as predictors of future vision in preverbal children with infantile nystagmus. Thomas et al[10] proposed a structural grading scheme for foveal hypoplasia based on OCT data from adults and older children that has become a widely used scheme, with grades 1 to 4 representing the most to least developed fovea, whereas the atypical grade represents disruption of the photoreceptors seen in achromatopsia. Visual evoked potentials may represent another option for estimating VA in infantile nystagmus,[18] but this is time consuming and difficult to perform, requiring specialist diagnostic services In this longitudinal cohort study, we harnessed the potential of handheld OCT for the diagnosis and prognosis of foveal hypoplasia in young children. Secondary aims were (1) to assess whether structural grading is comparable with quantitative segmentation in predicting future vision, (2) to compare the predictive power of the grading system with PL VA results obtained on first examination, and (3) to determine whether the grade remains stable over time
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