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Observational Study
. 2022 Feb:234:81-90.
doi: 10.1016/j.ajo.2021.07.018. Epub 2021 Jul 22.

Characterization of Retinal Function Using Microperimetry-Derived Metrics in Both Adults and Children With RPGR-Associated Retinopathy

Evgenia Anikina  1 , Michalis Georgiou  2 , James Tee  1 , Andrew R Webster  1 , Richard G Weleber  3 , Michel Michaelides  4
Affiliations
Observational Study

Characterization of Retinal Function Using Microperimetry-Derived Metrics in Both Adults and Children With RPGR-Associated Retinopathy

Evgenia Anikina et al. Am J Ophthalmol. 2022 Feb.

Abstract

Purpose: To investigate microperimetry testing of retinitis pigmentosa GTPase regulator gene (RPGR)-associated retinopathy in a cohort of children and adults.

Design: Prospective observational case series.

Methods: The coefficient of repeatability and intraclass correlation coefficient (ICC) of mean sensitivity (MS) were calculated for mesopic microperimetry. Best-corrected visual acuity (BCVA), contrast sensitivity (CS), MS, total volume (VTOT), and central 3-degree field volume (V3) from volumetric and topographic analyses were acquired.

Results: The study recruited 76 individuals with RPGR (53 adults, 23 children). The mean follow-up period was 2.8 years. The ICC values for MS, VTOT, and V3 were 0.982 dB (95% CI, 0.969-0.989 dB), 0.970 dB-steradian (sr) (95% CI, -0.02658 to 0.03691 dB-sr), and 0.986 dB-sr (95% CI, 0.978-0.991), respectively. The r values for interocular MS, VTOT, and V3 were 0.97 (P < .01), 0.97 (P < .01), and 0.98 (P < .01), respectively, indicating strong interocular correlation. The interocular correlation of progression for MS, VTOT, and V3 was 0.81 (P < .01), 0.64 (P < .01), and 0.81 (P < .01), respectively. There was no statistically significant difference in the interocular progression rates for MS or VTOT. V3 did show a statistically significant difference. Most patients lost retinal sensitivity rapidly during their second and third decades of life.

Conclusions: The high degree of reproducibility of results and the good interocular correlation lends this method to accurately monitoring disease progression, as well as supporting validation of the use of MP in assessing the outcomes of gene therapy clinical treatment trials.

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Figures

FIGURE 1
FIGURE 1
Flow chart of subject recruitment and participation in testing. MP = microperimetry; RPGR = retinitis pigmentosa GTPase regulator.
FIGURE 2
FIGURE 2
Test-retest reliability assessment. Bland-Altmann plots of the test-retest reliability of the (A) mean sensitivity measurements in dB, and the volumetric measurement in dB-steradian (dB-sr) of (B) total retinal sensitivity (VTOT) and (C) of the fovea-centered area of radius 3° (V3).
FIGURE 3
FIGURE 3
Interocular symmetry assessment. Bland-Altmann plots of the (A) interocular symmetry of the mean sensitivity (MS) and (B) the volumetric measurement of total retinal sensitivity (VTOT). dB-sr = in dB-steradian.
FIGURE 4
FIGURE 4
Progression of retinal functional loss. Plots with best-fit lines of all individuals (X-linked retinitis pigmentosa [XLRP], 27; cone dystrophy [COD], 2; cone-rod dystrophy [CORD], 1; and sector retinitis pigmentosa [RP], 1) in the cohort with ≥3 consecutive retinal sensitivity measurements. A. The mean sensitivity. The volumetric measurement of (B) total retinal sensitivity (VTOT) and (C) fovea-centered area of radius 3° (V3) are presented for the analyzed right eyes. dB-sr = dB-steradian.
See this image and copyright information in PMC

References

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