Microperimetry as an Outcome Measure in Choroideremia Trials: Reproducibility and Beyond

Abstract

Insights

Microperimetry testing in choroideremia (CHM) shows high variability, especially at disease borders. Customized grids improve repeatability, but individual point variation must be considered for monitoring disease progression.

Area of Science:

  • Ophthalmology
  • Visual Electrophysiology
  • Retinal Imaging

Background:

  • Choroideremia (CHM) is a rare X-linked genetic disorder causing progressive vision loss.
  • Accurate assessment of visual function is crucial for monitoring disease progression in CHM patients.
  • Microperimetry (MP) is a key functional test, but its test-retest reliability in CHM needs further investigation.

Purpose of the Study:

  • To evaluate the test-retest repeatability of microperimetry (MP) in choroideremia (CHM) subjects.
  • To compare the performance of standard versus personalized stimulus grids for MP testing in CHM.
  • To assess the impact of stimulus grid customization on the reliability of sensitivity measurements.

Main Methods:

  • Fifteen CHM subjects (28 eyes) underwent repeated MP testing using the Macular Integrity Assessment (MAIA) microperimeter.
  • Both a standard 10° grid and a customized grid, adapted to individual macular pathology, were utilized.
  • Point-wise sensitivity (PWS) and mean sensitivity (MS) were analyzed, with PWS assessed within and outside areas of degeneration. Interpolated volumetric indices were used for customized grids.

Main Results:

  • Standard-grid MP testing showed higher coefficients of variation (CV) in CHM subjects (0.09) compared to controls (0.02).
  • Customized grids, particularly with volumetric indices, improved repeatability (CV of 0.05 for global, 0.02 for regional analysis).
  • Point-wise sensitivity (PWS) variability was significantly higher in CHM, especially at the border of degeneration (10.68 dB vs. 4.74 dB).

Conclusions:

  • Microperimetry in CHM exhibits significant test-retest variability, particularly at the border of degeneration, impacting MS repeatability.
  • Customized stimulus grids and volumetric measures enhance the reliability of both global and regional sensitivity assessments in CHM.
  • The inherent test-retest variation of individual points necessitates careful consideration when evaluating functional decline and disease progression in CHM.

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