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Chromatic pupilloperimetry for objective diagnosis of Best vitelliform macular dystrophy

 

Authors Ben Ner D, Sher I, Hamburg A, Mhajna MO, Chibel R, Derazne E, Sharvit-Ginon I, Pras E, Newman H, Levy J, Khateb S, Sharon D, Rotenstreich Y

Received 23 October 2018

Accepted for publication 9 January 2019

Published 5 March 2019 Volume 2019:13 Pages 465—475

DOI https://doi.org/10.2147/OPTH.S191486

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Justinn Cochran

Peer reviewer comments 2

Editor who approved publication: Dr Scott Fraser

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 (=−0.714, =0.071 and =−0.821, =0.023, respectively) and visual acuity (=0.709, =0.074 and =0.655, =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.
Keywords: Best vitelliform macular dystrophy, pupillary light reflex, perimetry, pupilloperimetry, visual field


 

摘要视频链接:BVMD patients