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Characterization of Retinal Function Using Microperimetry-Derived Metrics in Both Adults and Children With
Evgenia Anikina1, Michalis Georgiou2, James Tee1
1Moorfields Eye Hospital (E.A., M.G., J.T., A.R.B., M.M.), London, United Kingdom; From the University College London Institute of Ophthalmology (E.A., M.G., J.T., A.R.B., M.M.), University College London, London, United Kingdom.
American Journal of Ophthalmology
|July 25, 2021
Summary
Microperimetry testing shows high reproducibility and interocular correlation for monitoring retinitis pigmentosa GTPase regulator gene (RPGR)-associated retinopathy progression. This method supports gene therapy trial outcome assessments.
Area of Science:
- Ophthalmology
- Genetics
- Medical Imaging
Background:
- Retinitis pigmentosa GTPase regulator gene (RPGR)-associated retinopathy is a significant cause of inherited vision loss.
- Accurate monitoring of disease progression is crucial for understanding RPGR-associated retinopathy and evaluating potential treatments.
- Microperimetry (MP) offers a potential method for assessing retinal function in these patients.
Purpose of the Study:
- To evaluate the reliability and reproducibility of microperimetry (MP) testing in individuals with RPGR-associated retinopathy.
- To assess the interocular correlation of retinal sensitivity and volumetric measures in this cohort.
- To determine the utility of MP for monitoring disease progression and supporting gene therapy clinical trials.
Main Methods:
- A prospective observational case series involving 76 individuals (53 adults, 23 children) with RPGR-associated retinopathy.
- Mesopic microperimetry was used to measure mean sensitivity (MS).
- Volumetric and topographic analyses were performed to obtain total volume (VTOT) and central 3-degree field volume (V3). Reproducibility and interocular correlation were calculated using ICC and r values.
Main Results:
- High intraclass correlation coefficients (ICC) were observed for MS (0.982), VTOT (0.970), and V3 (0.986), indicating excellent test-retest reliability.
- Strong interocular correlations were found for MS (r=0.97), VTOT (r=0.97), and V3 (r=0.98).
- Significant interocular correlation of progression was noted for MS (0.81) and V3 (0.81), though VTOT progression showed a lower correlation (0.64). Most patients experienced rapid retinal sensitivity loss in their second and third decades.
Conclusions:
- Microperimetry demonstrates high reproducibility and strong interocular correlation in patients with RPGR-associated retinopathy.
- These findings support the use of MP as a reliable tool for accurately monitoring disease progression.
- The validated use of MP can aid in assessing outcomes for gene therapy clinical trials in RPGR-associated retinopathy.

