Chromatic pupilloperimetry for objective diagnosis of best vitelliform macular dystrophy

Daniel Ben Ner, Ifat Sher, Amit Hamburg, Mohamad O. Mhajna, Ron Chibel, Estela Derazne, Inbal Sharvit-Ginon, Eran Pras, Hadas Newman, Jaime Levy, Samer Khateb, Dror Sharon, Ygal Rotenstreich*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Purpose: To determine the pupil response of Best vitelliform macular dystrophy (BVMD) patients for focal blue and red light stimuli presented at 76 test points in a 16.2° visual field (VF) using a chromatic pupilloperimeter. Methods: An observational study was conducted in 16 participants: 7 BVMD patients with a heterozygous BEST1 mutation and 9 similar-aged controls. All participants were tested for best-corrected visual acuity, chromatic pupilloperimetry and Humphrey perimetry. Percentage of pupil contraction (PPC), maximal pupil contraction velocity (MCV) and latency of MCV (LMCV) were determined. Results: The mean PPC and MCV recorded in BVMD patients in response to red stimuli were lower by.2 standard errors (SEs) from the mean of controls in 47% and 43% of VF test points, respectively. The mean PPC and MCV recorded in the patients in response to blue stimuli were lower by.2 SEs from the mean of controls in 36% and 24% of VF test points, respectively. The patients’ mean and median MCV recorded in response to red light correlated with their Humphrey mean deviation score (r=−0.714, P=0.071 and r=−0.821, P=0.023, respectively) and visual acuity (r=0.709, P=0.074 and r=0.655, P=0.111, respectively). A substantially shorter mean LMCV was recorded in BVMD patients compared to controls in 54% and 93% of VF test points in response to red and blue light, respectively. Receiver operating characteristic analysis for LMCV in response to red light identified a test point at the center of the VF with high diagnostic accuracy (area under the curve of 0.94). Conclusion: Chromatic pupilloperimetry may potentially be used for objective noninvasive assessment of rod and cone cell function in different locations of the retina in BVMD patients.

Original languageEnglish
Pages (from-to)465-475
Number of pages11
JournalClinical Ophthalmology
Volume13
DOIs
StatePublished - 2019

Funding

FundersFunder number
Accutome, Inc.

    Keywords

    • Best vitelliform macular dystrophy
    • Perimetry
    • Pupillary light reflex
    • Pupilloperimetry
    • Visual field

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