Back to Journals » Clinical Ophthalmology » Volume 13

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


Video abstract presented by Ygal Rotenstreich.

Views: 33

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


Creative Commons License This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution - Non Commercial (unported, v3.0) License. By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms.

Download Article [PDF]  View Full Text [HTML][Machine readable]