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通过光学相干断层扫描血管造影评估强直性肌营养不良中的视网膜血管异常——横断面研究。

Retinal vascular abnormalities in myotonic dystrophy assessed by optical coherence tomography angiography - Cross-sectional study.

作者信息

Vasilijevic Jelena, Peric Stojan, Basta Ivana, Kovacevic Igor, Maric Gorica, Avram Nada, Gunjic Ilija, Bozic Marija

机构信息

Clinic for Eye Diseases, University Clinical Center of Serbia, Belgrade, Serbia.

Faculty of Medicine, University of Belgrade, Belgrade, Serbia.

出版信息

Eur J Ophthalmol. 2025 Jan;35(1):262-268. doi: 10.1177/11206721241247424. Epub 2024 Apr 22.

DOI:10.1177/11206721241247424
PMID:38644803
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).

MATERIAL

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 ( < 0.001 for the DM1 group, and  = 0.001,  = 0.005 and  = 0.026, respectively, for the DM2 group), as well as in the macular zone in the deep capillary plexus for DM1 ( = 0.002) and deep macular and perifoveal zone for DM2 ( = 0.007,  = 0.001, respectively). The foveal avascular zone showed no significant differences between DM1 and DM2 compared to their control groups ( = 0.320 and  = 0.945, respectively).

CONCLUSION

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比较强直性肌营养不良症(DM)患者和非DM患者的光学相干断层扫描血管造影(OCTA)定量参数,检测视网膜和脉络膜血管系统的变化。

材料

横断面研究。纳入了41例连续的DM患者。纳入标准为1型和2型DM的分子诊断。为避免年龄对微血管变化的影响,并便于比较DM1和DM2患者,建立了两个性别和年龄匹配的对照组。

结果

与对照组相比,DM组在浅表毛细血管丛的黄斑、中心凹旁和中心凹周围区域的血管密度显著降低(DM1组P<0.001,DM2组分别为P=0.001、P=0.005和P=0.026),DM1组在深层毛细血管丛的黄斑区域(P=0.002)以及DM2组在深层黄斑和中心凹周围区域(分别为P=0.007、P=0.001)也是如此。与各自的对照组相比,DM1和DM2患者的中心凹无血管区无显著差异(分别为P=0.320和P=0.945)。

结论

我们的结果表明,DM不仅与典型的色素沉着变化有关,还与视网膜浅表和深层微血管异常有关,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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