Can Structural Grading of Foveal Hypoplasia Predict Future Vision in Infantile Nystagmus?

Abstract

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.Design: Longitudinal cohort study.Participants: Forty-two patients with infantile nystagmus (19 with albinism, 17 with idiopathic infantile nystagmus, and 6 with achromatopsia) were examined.Methods: Spectral-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 Measures: Grade 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.Results: We imaged 81 eyes from 42 patients with infantile nystagmus of mean age 19.8 months (range, 0.9e33.4 months; standard deviation [SD], 9.4 months) at the first handheld OCT scan. Mean follow-up was 44.1 months (range, 18.4e63.2 months; SD, 12.0 months). Structural grading was the strongest predictor of future VA

Long-Term Investigation of Retinal Function in Patients with Achromatopsia

“…15,16,26 Recently, it has been suggested that structural grading of foveal hypoplasia may predict future vision in patients with infantile nystagmus (including ACHM). 44 In our cohort, BCVA was better by six ETDRS letters on average in patients without foveal hypoplasia.…”

Response to spectral-domain optical coherence tomography foveal morphology as a prognostic factor for vision performance in congenital aniridia

“…Our recent prediction study included OCT data for preverbal infants and young children at first examination, then BCVA was recorded when the child was old enough to participate in gold standard LogMAR chart testing. 4 Moreover, in the study by Casas-Llera et al, we wonder whether cataracts could have influenced BCVA? Or perhaps the subcapsular cataracts were not in the visual axis?…”

“…We would like to highlight that handheld OCT is possible in awake infants with nystagmus from birth in our experience, without sedation or dilation, such as in our latest study with infants as young as 28 days old. 4 We accept that a great number of eye departments do not have access to or experience in handheld OCT imaging, therefore it was reasonable for the authors to exclude children too young to cooperate with conventional OCT.…”

“…Their study demonstrated that higher foveal hypoplasia grades correlated with poorer BCVA, consistent with findings from our unit and others. 2,4 It would be highly valuable to assess whether BCVA in the younger children improved further over time. Our recent prediction study included OCT data for preverbal infants and young children at first examination, then BCVA was recorded when the child was old enough to participate in gold standard LogMAR chart testing.…”

Detection of intracranial hypertension in children using optical coherence tomography: a systematic review protocol

“…26 OCT has been used to describe the normal development of the optic nerve 27 and fovea 27 28 in children. OCT has also been used in a wide range of conditions within paediatric ophthalmology, including retinopathy of prematurity, 29 retinoblastoma, 30 nystagmus, 31 albinism, 32 achromatopsia, 33 foveal hypoplasia, 34 optic nerve hypoplasia, 35 primary congenital glaucoma, 36 microcephaly 37 and others. Furthermore, OCT has been successfully used as a diagnostic tool in a variety of conditions associated with ICH, including idiopathic ICH, craniosynostosis and hydrocephalus.…”