Department of Ophthalmology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.
School of Medicine, Vita-Salute San Raffaele University, Milan, Italy.
Transl Vis Sci Technol. 2022 Mar 2;11(3):23. doi: 10.1167/tvst.11.3.23.
To analyze the characteristics of the choriocapillaris and the choroid in patients with Alport syndrome (AS) and investigate their clinical and demographic associations.
Multicenter, cross-sectional study. Forty-two eyes with AS were consecutively enrolled. A cohort of 33 healthy eyes was included as controls. Demographics and medical history were collected for each participant. Each eye underwent 3 × 3 swept-source optical coherence tomography angiography (PLEX Elite 9000 2.0; Carl Zeiss Meditec, Dublin, CA, USA) and spectral-domain OCT (Spectralis HRA2; Heidelberg Engineering, Heidelberg, Germany). Choriocapillaris flow deficit (FD) number, mean FD size, total FD area, FD density, subfoveal choroidal thickness (CT), total CT, and choroidal vascularity index (CVI) were compared between AS and control eyes. Factors associated with the FD density and the CVI in AS were explored with multivariable linear mixed models.
There was high intragroup variability in choriocapillaris and choroidal measurements in patients with AS. Choriocapillaris FD in patients with AS were more numerous compared to controls (P = 0.02). FD density in eyes with AS increased with older age (estimate = 0.31% for each year; 95% confidence interval [CI], 0.06-0.57; P = 0.02) and was higher in patients with a history of kidney transplant (estimate = 9.66% in case of positive history; 95% CI, 3.52-15.8; P = 0.006). The CVI was lower in eyes with dot maculopathy (estimate = -3.30% if present; 95% CI, -6.38 to -0.21; P = 0.04) and anterior lenticonus (estimate = -6.50% if present; 95% CI, -10.99 to -2.00; P = 0.006).
Patients with AS with kidney involvement requiring transplant may present with more severe choriocapillaris impairment. Lower choroidal vascularity was found in the presence of other ocular structural abnormalities.
An increased load of choriocapillaris flow deficits on optical coherence tomography angiography was found in patients with Alport syndrome who also had severe kidney disease requiring transplant.
分析 Alport 综合征（AS）患者脉络膜毛细血管和脉络膜的特征，并探讨其临床和人口统计学相关性。
多中心、横断面研究。连续纳入 42 只患有 AS 的眼作为病例组，同时纳入 33 只健康眼作为对照组。收集每位参与者的人口统计学和病史信息。所有眼均接受 3×3 扫频源光学相干断层扫描血管造影（PLEX Elite 9000 2.0；卡尔蔡司医疗技术公司，都柏林，加利福尼亚州，美国）和光谱域 OCT（Spectralis HRA2；海德堡工程公司，海德堡，德国）检查。比较 AS 眼和对照组眼中脉络膜毛细血管血流不足（FD）数量、平均 FD 大小、总 FD 面积、FD 密度、中心凹下脉络膜厚度（CT）、总 CT 和脉络膜血管指数（CVI）。使用多变量线性混合模型探讨与 AS 中 FD 密度和 CVI 相关的因素。
AS 患者脉络膜毛细血管和脉络膜的测量值存在较高的组内变异性。与对照组相比，AS 患者脉络膜毛细血管 FD 更多（P = 0.02）。AS 眼的 FD 密度随年龄增长而增加（估计值为每年增加 0.31%；95%置信区间，0.06-0.57；P = 0.02），并且在有肾移植史的患者中更高（如果有病史，则估计值为 9.66%；95%置信区间，3.52-15.8；P = 0.006）。如果存在点状黄斑病变（如果存在，则估计值为-3.30%；95%置信区间，-6.38 至-0.21；P = 0.04）或前晶状体混浊（如果存在，则估计值为-6.50%；95%置信区间，-10.99 至-2.00；P = 0.006），则 CVI 较低。
有肾脏受累需要移植的 AS 患者可能表现出更严重的脉络膜毛细血管损伤。在存在其他眼部结构异常的情况下，脉络膜血管密度降低。
丁慧敏
