Ben Ner Daniel, Sher Ifat, Hamburg Amit, Mhajna Mohamad O, Chibel Ron, Derazne Estela, Sharvit-Ginon Inbal, Pras Eran, Newman Hadas, Levy Jaime, Khateb Samer, Sharon Dror, Rotenstreich Ygal
Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, Israel,
Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel,
Clin Ophthalmol. 2019 Mar 5;13:465-475. doi: 10.2147/OPTH.S191486. eCollection 2019.
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.
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.
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).
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.
使用彩色瞳孔计，确定贝斯特卵黄样黄斑营养不良（BVMD）患者对在16.2°视野（VF）中的76个测试点呈现的聚焦蓝光和红光刺激的瞳孔反应。
对16名参与者进行了一项观察性研究：7名携带杂合子BEST1突变的BVMD患者和9名年龄相仿的对照者。所有参与者均接受了最佳矫正视力、彩色瞳孔测量和汉弗莱视野检查。测定瞳孔收缩百分比（PPC）、最大瞳孔收缩速度（MCV）和MCV潜伏期（LMCV）。
在VF测试点中，分别有47%和43%的点上，BVMD患者对红色刺激的平均PPC和MCV记录值比对照者的平均值低超过2个标准差（SE）。在VF测试点中，分别有36%和24%的点上，患者对蓝色刺激的平均PPC和MCV记录值比对照者的平均值低超过2个SE。患者对红光的平均和中位数MCV记录值与他们的汉弗莱平均偏差评分（分别为=-0.714，=0.071和=-0.821，=0.023）以及视力（分别为=0.709，=0.074和=0.655，=0.111）相关。与对照者相比，分别在54%和93%的VF测试点上，BVMD患者对红光和蓝光的平均LMCV记录值明显更短。对红光反应的LMCV进行的受试者操作特征分析确定了VF中心的一个测试点，具有较高的诊断准确性（曲线下面积为0.94）。
彩色瞳孔测量法可能潜在地用于对BVMD患者视网膜不同位置的视杆和视锥细胞功能进行客观无创评估。
