VISTA Clinic Binningen BL, 4201 Binningen, Switzerland.
Department of Ophthalmology, University of Basel, 4056 Basel, Switzerland.
Genes (Basel). 2021 Feb 14;12(2):272. doi: 10.3390/genes12020272.
(1) Background: The aim of our study was to investigate the relationship between retinal metabolic alterations (retinal vessel oximetry, RO) and structural findings (retinal vessel diameter, central retinal thickness and retinal nerve fiber layer thickness, RNFL) in patients with inherited retinal diseases (IRDs). (2) Methods: A total of 181 eyes of 92 subjects were examined: 121 eyes of 62 patients with IRDs were compared to 60 eyes of 30 healthy age-matched controls. The retinal vessel oximetry was performed with the oxygen saturation measurement tool of the Retinal Vessel Analyser (RVA; IMEDOS Systems UG, Jena, Germany). The oxygen saturation in all four major peripapillary retinal arterioles (A-SO; %) and venules (V-SO; %) were measured and their difference (A-V SO; %) was calculated. Additionally, retinal vessel diameters of the corresponding arterioles (D-A; µm) and venules (D-V; µm) were determined. The peripapillary central retinal thickness and the RNFL thickness were measured using spectral domain optical coherence tomography (SD-OCT) (Carl Zeiss Meditec, Dublin, CA, USA). Moreover, we calculated the mean central retinal oxygen exposure (cO-E; %/µm) and the mean peripapillary oxygen exposure (pO-E; %/µm) per micron of central retinal thickness and nerve fiber layer thickness by dividing the mean central retinal thickness (CRT) and the RNFL thickness with the mean A-V SO. (3) Results: Rod-cone dystrophy patients had the highest V-SO and A-SO, the lowest A-V SO, the narrowest D-A and D-V and the thickest RNFL, when compared not only to controls ( ≤ 0.040), but also to patients with other IRDs. Furthermore, in rod-cone dystrophies the cO-E and the pO-E were higher in comparison to controls and to patients with other IRDs ( ≤ 0.005). Cone-rod dystrophy patients had the lowest cO-E compared to controls and patients with other IRDs ( ≤ 0.035). Evaluated in central zones, the cO-E was significantly different when comparing cone-rod dystrophy (CRD) against rod-cone dystrophy (RCD) patients in all zones ( < 0.001), whereas compared with controls and patients with inherited macular dystrophy this was observed only in zones 1 and 2 ( ≤ 0.018). The oxygen exposure was also the highest in the RCD group for both the nasal and the temporal peripapillary area, among all the evaluated groups ( ≤ 0.025). (4) Conclusions: The presented metabolic-structural approach enhances our understanding of inherited photoreceptor degenerations. Clearly demonstrated through the O-E comparisons, the central and the peripapillary retina in rod-cone dystrophy eyes consume less oxygen than the control-eyes and eyes with other IRDs. Rod-cone dystrophy eyes seem to be proportionally more exposed to oxygen, the later presumably leading to more pronounced oxidative damage-related remodeling.
（1）背景：我们研究的目的是调查遗传性视网膜疾病（IRDs）患者的视网膜代谢改变（视网膜血管血氧测量，RO）与结构发现（视网膜血管直径、中央视网膜厚度和神经纤维层厚度，RNFL）之间的关系。（2）方法：共检查了 92 名受试者的 181 只眼睛：121 只眼睛的 62 名 IRD 患者与 30 名年龄匹配的健康对照组的 60 只眼睛进行了比较。使用视网膜血管分析仪（RVA；IMEDOS Systems UG，耶拿，德国）的氧饱和度测量工具进行视网膜血管血氧测量。测量了四个主要视盘周围视网膜动脉（A-SO；%）和小静脉（V-SO；%）的氧饱和度，并计算了它们之间的差异（A-V SO；%）。此外，还确定了相应动脉（D-A；µm）和小静脉（D-V；µm）的视网膜血管直径。使用谱域光学相干断层扫描（SD-OCT）（卡尔蔡司 Meditec，都柏林，CA，美国）测量视盘周围中央视网膜厚度和神经纤维层厚度。此外，我们通过将平均中央视网膜厚度（CRT）和 RNFL 厚度除以平均 A-V SO，计算了每微米中央视网膜厚度和神经纤维层厚度的平均中央视网膜氧暴露（cO-E；%/µm）和平均视盘周围氧暴露（pO-E；%/µm）。（3）结果：与对照组（≤0.040）相比，与其他 IRD 患者相比，杆状和锥状营养不良患者的 V-SO 和 A-SO 最高，A-V SO 最低，D-A 和 D-V 最窄，RNFL 最厚。此外，与对照组和其他 IRD 患者相比，杆状和锥状营养不良患者的 cO-E 和 pO-E 更高（≤0.005）。与对照组和其他 IRD 患者相比，锥状和杆状营养不良患者的 cO-E 最低（≤0.035）。在中央区域评估时，与对照组和其他 IRD 患者相比，锥状和杆状营养不良患者在所有区域（<0.001）的 cO-E 差异均有统计学意义，而与对照组和遗传性黄斑营养不良患者相比，仅在区域 1 和 2 观察到这种情况（≤0.018）。在所有评估组中，RCD 组的鼻侧和颞侧视盘周围区域的氧暴露量也是最高的（≤0.025）。（4）结论：所提出的代谢-结构方法增强了我们对遗传性光感受器退行性疾病的理解。通过 O-E 比较清楚地表明，与对照组和其他 IRD 患者相比，杆状和锥状营养不良患者的视网膜中央和视盘周围区域消耗的氧气更少。杆状和锥状营养不良患者的眼睛似乎受到更多的氧气暴露，后者可能导致更明显的氧化损伤相关重塑。
