Outer nuclear layer relevance in visual function correlated to quantitative enface OCT parameters in Stargardt disease

@article{Mucciolo2021OuterNL,
  title={Outer nuclear layer relevance in visual function correlated to quantitative enface OCT parameters in Stargardt disease},
  author={Dario Pasquale Mucciolo and Myrta Lippera and Dario Giorgio and Andrea Sodi and Ilaria Passerini and Francesca Cipollini and Gianni Virgili and Fabrizio Giansanti and Vittoria Murro},
  journal={European Journal of Ophthalmology},
  year={2021},
  volume={31},
  pages={3248 - 3258}
}
Purpose: To evaluate the correlation between Best Corrected Visual Acuity (BCVA) and the following parameters in Stargardt Disease (STGD): Central Retinal Thickness (CR-T), Central Outer Nuclear Layer Thickness (C-ONL-T), Areas of macular Photoreceptor loss (PHRa), and Retinal Pigment Epithelium (RPE) loss (RPEa). Methods: A total of 64 eyes of 32 STGD patients were included in the study. All patients received a comprehensive ophthalmological examination, color fundus photographs, fundus auto… 
1 Citations

Figures and Tables from this paper

The role of multimodal imaging and vision function testing in ABCA4-related retinopathies and their relevance to future therapeutic interventions
The aim of this review article is to describe the specific features of Stargardt disease and ABCA4 retinopathies (ABCA4R) using multimodal imaging and functional testing and to highlight their

References

SHOWING 1-10 OF 39 REFERENCES
Correlation of SD-OCT findings and visual function in patients with retinitis pigmentosa
TLDR
Data show that in patients afflicted with RP, ELM and OS/RPE layers are independently associated with BCVA on multivariate regression analysis, and highlight the key-role of external retinal layers in determining the visual function impairment attributable to RP.
Serial Spectral Domain Ocular Coherence Tomography Measurement of Outer Nuclear Layer Thickness in Rhegmatogenous Retinal Detachment Repair
TLDR
The finding that ONLT is reduced in detached retinae carries implications for visual prognosis.
Assessment of central visual function in Stargardt's disease/fundus flavimaculatus with ultrahigh-resolution optical coherence tomography.
TLDR
For the first time, an in vivo visualization and quantification of transverse, central photoreceptor loss and correlation with visual function is possible and UHR-OCT could be of future use in judging patients' prognoses in Stargardt's disease.
Reproducibility of Measurements of Retinal Structural Parameters Using Optical Coherence Tomography in Stargardt Disease
TLDR
STGD1's complex morphology made outer retina segmentation challenging, and ONL and TR thicknesses and the EZ intact area in the ETDRS inner ring hold potential as structural endpoints for STGD1 trials.
En face OCT in Stargardt disease
TLDR
Enface OCT proved to be a clinically useful tool for the management of STGD patients, illustrating in vivo the structural abnormalities of the different retinal layers.
Correlation of outer nuclear layer thickness with cone density values in patients with retinitis pigmentosa and healthy subjects.
TLDR
The ONL thickness and cone density were correlated in normal eyes and eyes with RP, but both were strongly correlated with retinal eccentricity, precluding estimation of cone density from OnL thickness.
ELLIPSOID ZONE MAPPING AND OUTER RETINAL ASSESSMENT IN STARGARDT DISEASE
TLDR
Eyes with Stargardt disease frequently have significant disruption of the ellipsoid zone and outer segments, including atrophy and attenuation, and this degenerative change was successfully quantified with a novel assessment platform and identified correlates with visual function.
Correlation between photoreceptor layer integrity and visual function in patients with Stargardt disease: implications for gene therapy.
TLDR
The results of this study suggest that a comprehensive approach in the examination of Stargardt patients has the potential to improve the understanding of vision loss and may provide a sensitive measure to evaluate the efficacy of future experimental therapies in patients with STGD.
A comparison of fundus autofluorescence and retinal structure in patients with Stargardt disease.
TLDR
In three patients, photoreceptor abnormalities were seen in the fovea on SD-OCT without an equivalent abnormality on FAF, which suggests that in these patients, the structural integrity of the photoreceptors may be affected earlier than changes in the RPE at least as detected by FAF.
...
...