特发性玻璃膜疣在 Best 型卵黄样黄斑营养不良中的表现。

INTRARETINAL HYPERREFLECTIVE FOCI IN BEST VITELLIFORM MACULAR DYSTROPHY.

机构信息

Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele, Milano, Italy.

Department of Ophthalmology, University of Verona, Verona, Italy.

出版信息

Retina. 2018 Dec;38(12):2379-2386. doi: 10.1097/IAE.0000000000001893.

DOI:10.1097/IAE.0000000000001893

PMID:29065010

Abstract

PURPOSE

To report on the presence of hyperreflective foci (HF) on spectral domain optical coherence tomography in patients with Best vitelliform macular dystrophy (BVMD), and to describe the relationship between HF and stages of the disease.

METHODS

Consecutive patients diagnosed with BVMD were enrolled in a prospective cross-sectional study. All patients and control subjects underwent a complete ophthalmologic examination, including best-corrected visual acuity and spectral domain optical coherence tomography.

MAIN OUTCOME MEASURE

identification of HF in BVMD. Secondary outcome: assessment of the HF in each stage and correlation with best-corrected visual acuity.

RESULTS

Overall, 75 eyes of 39 patients were included in the study (Stage 1: 13%, Stage 2: 43%, Stage 3: 15%, Stage 4: 21%, and Stage 5: 8%). On spectral domain optical coherence tomography assessment, intraretinal HF were present in 83% of all eyes, in 91% of eyes affected by clinical BVMD (Stages 2-5) and in 100% of patients in Stages 4 and 5. In 46% of clinically diseased eyes, HF were localized in the fovea and in correspondence with the BVMD lesions at the level of the outer nuclear layer and outer plexiform layer. Hyperreflective foci were present in 16% of control eyes. Mean number of HF in eyes affected by clinical BVMD stood at 7.67 ± 7.35. These were predominantly small HF (6.23 ± 6.14, P < 0.001) localized in the outer nuclear layer (5.19 ± 5.38, P = 0.001) and presented largely in the extrafoveal, rather than the foveal area (5.21 ± 5.57 vs 2.46 ± 2.73, P = 0.001). Analysis of HF distribution revealed that the control group and Stage 1 eyes had the fewest HF; Stage 4 displayed a significant increase in the number of HF compared with Stages 2 and 3; Stage 5 also showed an increased number of HF, although this difference was statistically significant only with Stage 3 eyes. The best-corrected visual acuity was negatively related to the number of HF, with best-corrected visual acuity deteriorating as the number of HF increased in Stages 2 to 5 (P < 0.001).

CONCLUSION

This study describes the presence of HF in BVMD using spectral domain optical coherence tomography. Our data suggest that HF identification is correlated with the progression of the disease and could represent a useful biomarker of BVMD.

摘要

目的

报告患有 Best 型卵黄样黄斑营养不良（BVMD）患者的光谱域光相干断层扫描（SD-OCT）中存在强反射焦点（HF），并描述 HF 与疾病阶段之间的关系。

方法

连续纳入确诊为 BVMD 的患者进行前瞻性横断面研究。所有患者和对照者均接受完整的眼科检查，包括最佳矫正视力和 SD-OCT。

主要观察指标

BVMD 中 HF 的识别。次要观察指标：评估每个阶段的 HF 并与最佳矫正视力相关。

结果

共有 39 例患者的 75 只眼纳入本研究（1 期：13%，2 期：43%，3 期：15%，4 期：21%，5 期：8%）。SD-OCT 评估显示，所有眼中存在视网膜内 HF 者占 83%，临床 BVMD 受累眼中占 91%（2-5 期），4 期和 5 期患者中占 100%。在 46%的临床受累眼中，HF 局限于黄斑区，与外核层和外丛状层的 BVMD 病变相对应。在 16%的对照眼中存在 HF。临床受累眼中 HF 的平均数量为 7.67±7.35 个。这些 HF 主要为小的 HF（6.23±6.14，P＜0.001），局限在外核层（5.19±5.38，P=0.001），主要位于黄斑外区（5.21±5.57 比 2.46±2.73，P=0.001）。HF 分布分析显示，对照组和 1 期患者的 HF 数量最少；与 2 期和 3 期相比，4 期的 HF 数量显著增加；5 期的 HF 数量也增加，但与 3 期相比仅具有统计学意义。最佳矫正视力与 HF 数量呈负相关，在 2 期至 5 期，随着 HF 数量的增加，最佳矫正视力逐渐恶化（P＜0.001）。