关键词: Myotonic dystrophy optical coherence tomography angiography pathogenesis retina retinal dystrophy

来  源:   DOI:10.1177/11206721241247424

Abstract:
BACKGROUND: The aim of the study was to detect the changes in retinal and choroidal vasculature via optical coherence tomography angiography (OCTA) by comparing the quantitative OCTA parameters in patients with and without myotonic dystrophies (DM).
METHODS: The cross-sectional study. Forty-one consecutive patients affected by DMs were enrolled. The inclusion criteria were molecular diagnosis of DM types 1 and 2. To avoid the age effect on microvascular changes and to justify a comparison between DM1 and DM2 patients, two control groups matched for sex and age were established.
RESULTS: The vascular density was found to be significantly decreased in the DM groups compared to the controls in the macular, parafoveal and perifoveal zone of superficial capillary plexus (p < 0.001 for the DM1 group, and p = 0.001, p = 0.005 and p = 0.026, respectively, for the DM2 group), as well as in the macular zone in the deep capillary plexus for DM1 (p = 0.002) and deep macular and perifoveal zone for DM2 (p = 0.007, p = 0.001, respectively). The foveal avascular zone showed no significant differences between DM1 and DM2 compared to their control groups (p = 0.320 and p = 0.945, respectively).
CONCLUSIONS: Our results show that DM is associated not only with the classic pigmentary changes but also with superficial and deep retinal microvasculature abnormalities, suggesting that these changes may be related to local hypoperfusion. Optical coherence tomography angiography is a useful tool for the diagnosis and characterization of retinal changes in DM and should be part of the standard evaluation of these patients.
摘要:
背景:本研究的目的是通过光学相干断层扫描血管造影(OCTA)检测视网膜和脉络膜血管的变化,通过比较有和没有强直性肌营养不良(DM)患者的定量OCTA参数。
方法:横断面研究。纳入了41名受DM影响的连续患者。纳入标准为1型和2型DM的分子诊断。为了避免年龄对微血管变化的影响,并证明DM1和DM2患者之间的比较是合理的,建立了两个性别和年龄相匹配的对照组.
结果:与对照组相比,糖尿病组黄斑区的血管密度明显降低,浅表毛细血管丛的中央凹和中央凹区域(DM1组p<0.001,p=0.001,p=0.005和p=0.026,对于DM2组),以及DM1的深毛细血管丛黄斑区(p=0.002)和DM2的深黄斑和中央凹区域(分别为p=0.007,p=0.001)。与对照组相比,DM1和DM2的中央凹无血管区没有显着差异(分别为p=0.320和p=0.945)。
结论:我们的结果表明,DM不仅与经典的色素性改变有关,而且与浅表和深层视网膜微血管异常有关,提示这些变化可能与局部灌注不足有关。光学相干断层扫描血管造影是诊断和表征DM视网膜变化的有用工具,应成为这些患者标准评估的一部分。
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