Shen Liangbo Linus, Zhang Yi, Wong Jessica, Foote Katharina G, Porco Travis C, Wang Ruikang K, Roorda Austin, Duncan Jacque L
Department of Ophthalmology, University of California San Francisco, San Francisco, California, United States.
Department of Ophthalmology, University of Washington, Seattle, Washington, United States.
Invest Ophthalmol Vis Sci. 2025 Aug 1;66(11):40. doi: 10.1167/iovs.66.11.40.
To investigate longitudinal changes in cone structure, choriocapillaris, and retinal sensitivity in choroideremia.
We assessed cone spacing z scores using adaptive optics scanning light ophthalmoscopy, analyzed choriocapillaris flow deficits (CCFDs) on swept-source optical coherence tomography angiography, and measured retinal sensitivity via microperimetry. Linear mixed-effects models estimated annual change rates in these parameters.
We analyzed 225 regions of interest from 14 eyes of 7 participants with choroideremia, including 6 participants (12 eyes) with follow-up imaging over a median of 3.3 years. The cone spacing z score increased by 0.57 ± 0.18 per year (P = 0.02), independent of distance to the fundus autofluorescence (FAF) border (P = 0.92). CCFD increased by 3.43 ± 0.52% per year at 1° inside (P < 0.001) and 2.03 ± 0.73% per year at 1° outside the FAF border (P = 0.01), but did not change significantly in other regions. Retinal sensitivity changed by -1.50 ± 0.34 dB/year at 1° inside (P < 0.001) and -1.94 ± 0.52 dB/year at 1° outside (P = 0.001) the FAF border, but did not change significantly in other areas. Greater retinal sensitivity loss correlated with closer proximity to the FAF border, greater baseline sensitivity, greater cone spacing z score, and larger CCFDs (P < 0.001 for each).
Cone spacing z score increased uniformly within the residual macular area, and CCFD and retinal sensitivity changed more rapidly within 1° of the FAF border. Regions in this zone are projected to lose 7 dB of sensitivity in approximately 3.5 years, shedding light into future clinical trial design.
研究脉络膜缺损患者视锥细胞结构、脉络膜毛细血管和视网膜敏感性的纵向变化。
我们使用自适应光学扫描激光检眼镜评估视锥细胞间距z分数，通过扫频光学相干断层扫描血管造影分析脉络膜毛细血管血流缺损（CCFDs），并通过微视野计测量视网膜敏感性。线性混合效应模型估计这些参数的年变化率。
我们分析了7名脉络膜缺损患者14只眼中的225个感兴趣区域，其中6名患者（12只眼）进行了中位时间为3.3年的随访成像。视锥细胞间距z分数每年增加0.57±0.18（P = 0.02），与距眼底自发荧光（FAF）边界的距离无关（P = 0.92）。在FAF边界内1°处，CCFD每年增加3.43±0.52%（P < 0.001），在FAF边界外1°处每年增加2.03±0.73%（P = 0.01），但在其他区域无显著变化。在FAF边界内1°处，视网膜敏感性每年变化-1.50±0.34 dB（P < 0.001），在FAF边界外1°处每年变化-1.94±0.52 dB（P = 0.001），但在其他区域无显著变化。更大的视网膜敏感性损失与更接近FAF边界、更高的基线敏感性、更高的视锥细胞间距z分数和更大的CCFDs相关（每项P < 0.001）。
在残留黄斑区内，视锥细胞间距z分数均匀增加，CCFD和视网膜敏感性在FAF边界1°范围内变化更快。该区域的区域预计在约3.5年内丧失7 dB的敏感性，为未来的临床试验设计提供了线索。
