Department of Ophthalmology, Hopital Intercommunal de Creteil, University of Paris XII, Creteil, France.
Retina. 2011 May;31(5):959-66. doi: 10.1097/IAE.0b013e3181f441c1.
To determine the visual field patterns obtained by the preferential hyperacuity perimetry (PHP) in patients with Best vitelliform macular dystrophy with mutations in the BEST1 gene.
Consecutive patients with Best vitelliform macular dystrophy underwent a complete ophthalmologic examination, including functional assessment by best-corrected visual acuity and Foresee PHP and morphologic assessment by fundus biomicroscopy, fundus autofluorescence, and spectral-domain optical coherence tomography. The functional "PHP visual field defect index" (which is the max peak value of the metamorphopsia [maximal distortion value at the visual field] + the max peak value of the scotoma [maximal scotoma value at the visual field]) and best-corrected visual acuity were analyzed about the disease stage.
Thirty eyes of 15 consecutive patients (8 men and 7 women; mean age 39 ± 24 years) were included for analysis. Based on fundus biomicroscopy, fundus autofluorescence, and spectral-domain optical coherence tomography, the macular lesions could be counted as follows: previtelliform lesions in 5 eyes of 3 patients (Stage 1), vitelliform lesions in 2 eyes of 2 patients (Stage 2), pseudohypopyon lesions in 6 eyes of 5 patients (Stage 3), vitelliruptive lesions in 4 eyes of 3 patients (Stage 4), atrophic lesions in 7 eyes of 5 patients (Stage 5), and fibrotic lesions in 6 eyes of 4 patients (Stage 6). Best-corrected visual acuity and PHP visual field defect index were averaged for each stage. Best-corrected visual acuity showed a good correlation (P = 0.01) with the morphologic severity (stage) of the disease (Pearson correlation = -0.88). Similarly, the PHP visual field defect index showed a good correlation (P = 0.03) with the morphologic severity (stage) of the disease (Pearson correlation = 0.78). Finally, best-corrected visual acuity showed a good correlation (P = 0.02) with the functional PHP visual field defect index (Pearson correlation = -0.83) about the morphologic stage of the disease.
Preferential hyperacuity perimetry could be considered an adjunctive useful tool in the evaluation of functional impairment and disease progression in patients with Best vitelliform macular dystrophy.
确定 BEST1 基因突变的 Best 卵黄样黄斑营养不良患者通过超敏偏好视野计（PHP）获得的视野模式。
连续的 Best 卵黄样黄斑营养不良患者接受全面的眼科检查，包括最佳矫正视力和 Foresee PHP 的功能评估以及眼底生物显微镜、眼底自发荧光和频域光相干断层扫描的形态评估。分析疾病阶段的功能“PHP 视野缺陷指数”（即形觉变形的最大峰值[视野中最大变形值] + 视野中最大暗点的最大峰值[视野中最大暗点值]）和最佳矫正视力。
纳入 15 例连续患者（8 名男性和 7 名女性；平均年龄 39±24 岁）的 30 只眼进行分析。根据眼底生物显微镜、眼底自发荧光和频域光相干断层扫描，黄斑病变可如下计数：3 例患者的 5 只眼有前卵黄病变（1 期），2 例患者的 2 只眼有卵黄病变（2 期），5 例患者的 6 只眼有假性假性病变（3 期），3 例患者的 4 只眼有卵黄破裂病变（4 期），5 例患者的 7 只眼有萎缩病变（5 期），4 例患者的 6 只眼有纤维化病变（6 期）。为每个阶段平均最佳矫正视力和 PHP 视野缺陷指数。最佳矫正视力与疾病形态严重程度（分期）呈良好相关性（P=0.01）（Pearson 相关系数=-0.88）。同样，PHP 视野缺陷指数与疾病形态严重程度（分期）呈良好相关性（P=0.03）（Pearson 相关系数=0.78）。最后，最佳矫正视力与疾病形态阶段的功能 PHP 视野缺陷指数呈良好相关性（P=0.02）（Pearson 相关系数=-0.83）。
超敏偏好视野计可被视为评估 Best 卵黄样黄斑营养不良患者功能损害和疾病进展的有用辅助工具。
