Title: Choriocapillaris Flow Signal Impairment in Sorsby Fundus Dystrophy Show Chinese title

Title: 脉络膜毛细血管血流信号损伤在Sorsby视网膜营养不良中的表现

Abstract: Purpose: To quantify choriocapillaris flow alterations in early Sorsby Fundus Dystrophy (SFD) and to investigate the relationship of choriocapillaris flow with the choroidal and outer retinal microstructure. Methods: In this prospective case-control study, 18 eyes of 11 patients with early SFD and 32 eyes of 32 controls without ocular pathology underwent multimodal imaging including spectral-domain optical coherence tomography (OCT)followed by deep-learning-based layer segmentation. OCT-angiography (OCT-A) was performed to quantify choriocapillaris flow signal deficits (FDs). Differences in choriocapillaris flow area percentage between SFD patients and controls were determined and a structure-function correlation with outer retinal layer thicknesses were analyzed based on mixed model analysis. Results: SFD patients exhibited a significantly greater choriocapillaris FDs area percentage than controls (estimate [95% CI] 32.05% [24.31-39.80] vs. 23.36% [20.64-26.09], P<0.001), even when adjusting for age. Choroidal thickness was a structural OCT surrogate of the choriocapillaris FD area percentage (-0.82% per 100 micrometer, P=0.017), whereas retinal-pigment-epithelium-drusen-complex thickness was not informative regarding choriocapillaris FDs (P=0.932). The choriocapillaris FD area percentage was associated with an altered microstructure of the overlying photoreceptors (outer-segments, inner-segments and outer-nuclear-layer thinning of -0.31, -0.12 and -0.47 $\mu$m per %FD, respectively, P<0.001). Conclusions: Patients with early SFD exhibit pronounced abnormalities of choriocapillaris flow signal on OCT-A, which are not limited to areas of sub-RPE deposits seen in OCT imaging. Thus, analysis of the choriocapillaris flow may enable clinical trials at earlier disease stages in SFD and possibly in mimicking diseases with an impaired Bruchs membrane including age-related macular degeneration. Show Chinese abstract

Abstract: 目的：量化早期Sorsby视盘营养不良（SFD）中脉络膜毛细血管血流的变化，并研究脉络膜毛细血管血流与脉络膜和外层视网膜微结构之间的关系。 方法：在这项前瞻性病例对照研究中，11例早期SFD患者的18只眼和32例无眼部病变的对照组的32只眼接受了多模式成像，包括谱域光学相干断层扫描（OCT），随后进行了基于深度学习的层分割。 进行了光学相干断层扫描血管成像（OCT-A）以量化脉络膜毛细血管血流信号缺损（FDs）。 确定了SFD患者和对照组之间脉络膜毛细血管血流面积百分比的差异，并基于混合模型分析，分析了外层视网膜层厚度的结构-功能相关性。 结果：SFD患者表现出显著大于对照组的脉络膜毛细血管FDs面积百分比（估计值[95% CI] 32.05% [24.31-39.80] vs. 23.36% [20.64-26.09]，P<0.001），即使在调整年龄后也是如此。 脉络膜厚度是脉络膜毛细血管FD面积百分比的结构OCT替代指标（每100微米减少0.82%，P=0.017），而视网膜色素上皮玻璃膜疣复合体厚度对于脉络膜毛细血管FDs没有信息（P=0.932）。 脉络膜毛细血管FD面积百分比与覆盖光感受器的微结构改变有关（外段、内段和外核层变薄分别为-0.31、-0.12和-0.47$\mu$m每%FD，分别P<0.001）。 结论：早期SFD患者在OCT-A上表现出明显的脉络膜毛细血管血流信号异常，这些异常不仅限于OCT成像中看到的视网膜色素上皮沉积区域。 因此，对脉络膜毛细血管血流的分析可能使临床试验在SFD的更早期阶段进行，并可能在类似疾病中进行，包括Bruchs膜受损的疾病，如年龄相关性黄斑变性。