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Abstract：
Purpose: To compare peripapillary choroidal vascularity among Leber\'s Hereditary Optic Neuropathy (LHON) patients at different stages of natural course and healthy controls using optical coherence tomography (OCT), and to evaluate peripapillary choroidal vascularity changes in LHON patients before and after gene therapy. Methods: 57 LHON patients and 15 healthy controls were enrolled in this prospective clinical study. LHON patients were divided into three duration groups based on stage of disease progression. Both patients and healthy controls underwent OCT scans focused on the optic disc at baseline with Heidelberg Spectralis, and patients underwent OCT at 1, 3, and 6 months after gene therapy. OCT images were converted and binarized using ImageJ software. Choroidal thickness (CT), total choroidal area (TCA), and choroidal vascularity index (CVI) in each quadrant of OCT images were measured to evaluate peripapillary choroidal vascularity. Results: At baseline, the average CT was not significantly different between LHON patients at different stages and between healthy controls (P = 0.468). Although average TCA and average CVI were slightly higher in LHON patients at different stages than in healthy controls, the difference was not statistically significant (P = 0.282 and 0.812, respectively). After gene therapy, The average TCA at 1 month after gene therapy was significantly higher than that before gene therapy (P = 0.003), while no significant differences were found in the average CT or average CVI in LHON patients before and 1,3 and 6 months after gene therapy using pairwise comparisons (all P > 0.05). Conclusions: No significant difference was found in choroidal vascularity of LHON patients at different stages and healthy controls. Choroidal vascularity seems to stay stable after gene therapy.
摘要：
目的：使用光学相干断层扫描（OCT）比较Leber遗传性视神经病变（LHON）患者在自然病程的不同阶段和健康对照者的脉络膜周围血管分布，并评估LHON患者基因治疗前后脉络膜周围血管的变化。方法：纳入57例LHON患者和15例健康对照者的前瞻性临床研究。根据疾病进展阶段将LHON患者分为三个持续时间组。患者和健康对照均在基线时用HeidelbergSpectralis对视盘进行了OCT扫描，患者在基因治疗后1,3和6个月接受OCT检查.使用ImageJ软件转换和二值化OCT图像。脉络膜厚度(CT),脉络膜总面积(TCA),测量OCT图像各象限中的脉络膜血管指数(CVI)以评估脉络膜周围血管分布。结果：在基线，不同分期的LHON患者和健康对照组的平均CT无显著差异(P=0.468).尽管LHON患者在不同阶段的平均TCA和平均CVI略高于健康对照组，差异无统计学意义(P=0.282和0.812)。基因治疗后,基因治疗后1个月的平均TCA明显高于基因治疗前(P=0.003),而LHON患者在基因治疗前和治疗后1、3、6个月的平均CT和平均CVI比较均无显著性差异(均P>0.05)。结论:不同分期LHON患者脉络膜血管分布与健康对照组无明显差异。基因治疗后脉络膜血管似乎保持稳定。
