基于光相干断层扫描血管成像评估Leber遗传性视神经病变患者视网膜微血流特征  

Evaluation of retinal microvascular characteristics in Leber hereditary optic neuropathy based on optical coherence tomography angiography

作　　者：贾楠[1] 王佳伟[1] 朱丽平[1] 孟超[1] Jia Nan;Wang Jiawei;Zhu Liping;Meng Chao(Department of Neurology,Beijing Tongren Hospital,Capital Medical University,Beijing 100730,China)

机构地区：[1]首都医科大学附属北京同仁医院神经内科,北京100730

出　　处：《中华医学杂志》2025年第13期1010-1016,共7页National Medical Journal of China

摘　　要：目的利用光相干断层扫描血管成像(OCTA)评估Leber遗传性视神经病变(LHON)患者视网膜微血流特征。方法回顾性收集2022年8月至2024年8月在首都医科大学附属北京同仁医院神经内科确诊为11778G>A位点突变的LHON患者,同期招募患者家属和医院职工作为健康对照者。LHON患者按病程分为A组(无症状期)、B组(亚急性期)、C组(活动期)和D组(慢性期),采用OCTA图像比较各组黄斑区微血流特征,分析视力损害与血流和结构参数相关性。结果共纳入LHON患者21例42只眼和健康对照组15名30只眼。A组5只眼,B组11只眼,C组10只眼,D组16只眼。LHON患者和健康对照组年龄分别为(24.2±4.0)和(24.3±3.9)岁。B、C、D组患者病程时间分别为(3.1±1.4)、(9.6±1.2)和(16.4±3.4)个月。LHON患者最佳矫正视力(BCVA)最小分辨角对数(LogMAR)值(1.30±0.76)高于健康对照组(0)(P<0.001)。LHON患者黄斑区灌注密度(PD)和血流长度密度(VLD)与健康对照组比较,A组鼻侧PD和VLD(P=0.034和P=0.044),B组PD、VLD平均值、鼻侧、下方PD和VLD及上方VLD(P<0.05),C组和D组黄斑各区PD、VLD和平均值均低于健康对照组(P<0.001)。所有LHON组黄斑各区神经节细胞复合体(GCC)厚度及平均值均低于健康对照组(均P<0.05)。A组颞侧和下方盘周视网膜神经纤维层(pRNFL)厚度及平均值均高于健康对照组(均P<0.05)。B组颞侧pRNFL厚度和平均值均低于健康对照组(均P<0.001)。pRNFL厚度平均值及所有区域厚度在C组和D组中均低于健康对照组(均P<0.001)。BCVA LogMAR值与所有血流参数和结构参数呈负相关(r=-0.744~-0.969,均P<0.001)。血流参数中,BCVA LogMAR值与VLD(r=-0.881~-0.900)的相关性相对较强。结论LHON患者黄斑区微血流损害随疾病进展逐渐加重。视力损害与OCTA血流参数和结构参数均呈相关性,早期出现黄斑区微血流改变提示视力预后差。Objective To evaluate retinal microvascular characteristics in patients with Leber hereditary optic neuropathy(LHON)by optical coherence tomography angiography(OCTA).Methods The LHON patients diagnosed with 11778G>A mutation in the Department of Neurology,Beijing Tongren Hospital,Capital Medical University from August 2022 to August 2024 were retrospectively collected.Family members of the patients and hospital staff were recruited as healthy controls during the same period.LHON patients were divided into group A(asymptomatic stage),group B(subacute stage),group C(dynamic stage)and group D(chronic stage)according to the course of disease.OCTA images were used to compare the macular microvascular characteristics of each group,and the correlation between visual impairment and the blood flow parameters and structural parameters was analyzed.Results A total of 21 LHON patients with 42 eyes and 15 healthy controls with 30 eyes were included.There were 5 eyes in group A,11 eyes in group B,10 eyes in group C,and 16 eyes in group D.The ages of LHON patients and healthy controls were(24.2±4.0)years and(24.3±3.9)years,respectively.The duration of disease in group B,C and D were(3.1±1.4)months,(9.6±1.2)months and(16.4±3.4)months,respectively.The Logarithm of the minimum Angle of Resolution(LogMAR)of best corrected visual acuity(BCVA)in LHON patients(1.30±0.76)was higher than that in the healthy control group(0)(P<0.001).When macular perfusion density(PD)and blood flow length density(VLD)in LHON patients were compared with those in healthy controls,nasal PD and VLD in group A(P=0.034 and P=0.044),the average value,nasal and inferior PD and VLD,and superior VLD in group B(P<0.05),and the average values of PD,VLD and macular areas in groups C and D were lower than those in the healthy control group(P<0.001).The thickness and average value of the general ganglion cell complex(GCC)in all regions of the macular in the LHON group were lower than those in the healthy control group(P<0.05).The temporal and inferior peripapilla

关 键 词：视神经萎缩 遗传性 Leber 光相干断层扫描血管成像 黄斑血流 神经节细胞复合体 视网膜神经纤维层 横断面研究 

分 类 号：R774.1[医药卫生—眼科]