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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


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Dr Scott Fraser

Video abstract presented by Ygal Rotenstreich.

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Daniel Ben Ner,1,2 Ifat Sher,1 Amit Hamburg,1,2 Mohamad O Mhajna,1,2 Ron Chibel,1,2 Estela Derazne,2 Inbal Sharvit-Ginon,3,4 Eran Pras,2,5 Hadas Newman,2,6 Jaime Levy,7 Samer Khateb,7 Dror Sharon,7 Ygal Rotenstreich1,2

1Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, Israel; 2Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; 3Department of Psychology, Bar Ilan University, Ramat Gan, Israel; 4The Joseph Sagol Neuroscience Center, Sheba Medical Center, Ramat Gan, Israel; 5The Matlow’s Ophthalmo-Genetics Laboratory, Department of Ophthalmology, Assaf-Harofeh Medical Center, Zerifin, Israel; 6Ophthalmology Department, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel; 7Department of Ophthalmology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel

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.

Keywords: Best vitelliform macular dystrophy, pupillary light reflex, perimetry, pupilloperimetry, visual field

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