Peripapillary Sparing With Near Infrared Autofluorescence Correlates With Electroretinographic Findings in Patients With Stargardt Disease

Abstract

Citation: Nassisi M, Mohand-Saïd S, Andrieu C, et al. Peripapillary sparing with near infrared autofluorescence correlates with electroretinographic findings in patients with Stargardt disease. Invest Ophthalmol Vis Sci. 2019;60:4951-4957. https://doi.org/ 10.1167/iovs.19-27100 PURPOSE.To evaluate the correlation between the quantification of peripapillary sparing and electroretinogram (ERG) outcomes in autosomal recessive Stargardt disease (STGD1). METHODS.Near infrared fundus autofluorescence (NIR-FAF) images of 101 eyes of 101 patients were retrospectively reviewed. Peripapillary sparing was assessed both qualitatively and quantitatively. The area of spared tissue (AST) was calculated in a 1-mm-wide ring around the optic disc after binarization of the 558 NIR-FAF. These measurements were correlated with the presence of normal ERG (group I), abnormal photopic responses (group II), or abnormal photopic and scotopic responses (group III). RESULTS.AST showed significant correlations with ERG groups (R ¼ À0.802, P < 0.001). While qualitative assessment of peripapillary sparing (i.e., present or not) also showed a significant correlation with ERG groups (R ¼ À0.435, P < 0.001), it was weaker than by AST quantification. The ordinal regression analysis showed that the increase in AST was associated with a decrease in the odds of belonging to ERG groups II and III, with an odds ratio of 0.82 (95% confidence interval [CI] 0.78-0.87), P < 0.001.CONCLUSIONS. The AST around the optic disc in eyes with STGD1 correlates with the impairment of photoreceptors as shown in the ERG. If replicated in future longitudinal studies, the quantification of peripapillary sparing may prove to be a useful parameter for evaluating the visual prognosis of these eyes.

Multimodal in-vivo maps as a tool to characterize retinal structural biomarkers for progression in adult-onset Stargardt disease

“…Retinal layer thicknesses were normal at the more peripheral regions, except for the 3.5 mm region. Notably, thinning of the ORL was observed in this region temporal to the optic disc, which is inside the area reported as the spared region in STGD1 ( 48 ).…”

Retrospective Natural History Study of RPGR-Related Cone- and Cone-Rod Dystrophies While Expanding the Mutation Spectrum of the Disease

“…Indeed, three imaging parameters demonstrated a good association with BCVA and two of them (i.e., peripapillary sparing and CRT) reached an overall correlation coefficient of 0.666. Peripapillary sparing is a feature that can be present in different inherited retinal dystrophies and its prognostic value has already been demonstrated in Stargardt disease [49][50][51][52]. It is not clear whether this specific sign is due to a more favorable photoreceptor/retinal pigment epithelium ratio in the peripapillary area, and/or to the presence of a thicker retinal nerve fiber layer that better shields this area from oxidative light damage [53].…”

Prediction of Function in ABCA4-Related Retinopathy Using Ensemble Machine Learning

“…However, as group 3 eyes (that also showed most impaired BCVA) revealed consistently reduced RPE ( Supplementary Table S2), this specific retinal layer, nevertheless, was attributed distinct feature importance (Supplementary Figure S5). A parameter that was not considered in the study was the area of peripapillary sparing, which can be assessed quantitatively and has been shown to correlate with electroretinographic findings in this disease entity [45,46]. Future models for 'inferred ERG' might include this parameter to further improve the predictions.…”