应用多模影像技术分析多发性一过性白点综合征

Clinical characteristics of multiple evanescent white dot syndrome with multimodal imaging

目的应用多模影像技术分析多发性一过性白点综合征(MEWDS)的临床特点,并据此进一步探讨其发病机制。方法回顾性病例研究。临床检查确诊的MEWDS患者7例(7眼)纳入研究。其中男性2例(2眼)、女性5例(5眼);年龄20~49岁,平均年龄33.8岁。所有患者均行最佳矫正视力(BCVA)、裂隙灯显微镜、间接检眼镜、眼底彩色照相、眼底自发荧光(FAF)、荧光素眼底血管造影(FFA)、吲哚菁绿血管造影(ICGA)、频域光学相干层析成像(SD-OCT)、OCT血管成像(OCTA)、多焦视网膜电图(mf-ERG)检查。平均随访3个月。观察病灶的彩色眼底像、FAF、FFA、ICGA、SD-OCT、OCTA、mf-ERG特征。结果患眼眼底像可见黄斑区橘红色颗粒样改变;FAF表现为后极部大小不一的点片状强荧光及不均匀弥漫性相互融合的强荧光病灶;FFA表现为早期隐约可见散在点状强荧光,中晚期可见花环状排列边界不清的强荧光;ICGA表现为晚期多发散在点状弱荧光病灶,病灶大小不等,部分病灶融合成小片状,并且ICGA弱荧光病灶与FAF、FFA强荧光病灶的大小、形态、部位相一致;SD-OCT表现为黄斑区外层视网膜结构紊乱或中断及外核层可见形态大小不等的强反射物质存积;OCTA:浅层视网膜、深层视网膜、外层视网膜以及脉络膜血管血流未见异常;mf-ERG:黄斑峰值降低。结论多模影像技术对MEWDS的诊断及发病机制的探讨具有重要临床意义。

Objective To observe clinical characteristics of multiple evanescent white dot syndrome(MEWDS)using multimodal imaging,and further to explore its pathogenesis.Methods This was a retrospective case study.Seven patients(7 eyes)diagnosed with MEWDS were enrolled.There were 5 females and 2 males with mean age of 33.8 years(range 20 to 49 years).All patients underwent such tests as best corrected visual acuity(BCVA),slit-lamp biomicroscope,indirect ophthalmoscope,fundus color photography,fundus autofluorescence(FAF),fundus fluorescein angiography(FFA),indocyanine green angiography(ICGA),spectral domain optical coherence tomography(SD-OCT),optical coherence tomography angiography(OCTA)and multifocal electroretinograms(mf-ERG).The mean follow-up duration was 3 months.The color fundus images,FAF,FFA,ICGA,SD-OCT,OCTA and mf-ERG characteristics of the lesions were observed.Results Color fundus imaging showed a granular fovea change.FAF imaging showed irregular stippled hyperautofluorescence in posterior pole.FFA found faintly visible scattered punctate strong fluorescence in early stage and demonstrated punctuate hyperfluorescence in late stage,which were corresponded to the hyperautofluorescence dots in FAF.In ICGA imaging,there was no distinct abnormal finding in early phase and showed some hypofluorescent dots and spots at the posterior pole in late phase.Further,the hypofluorescence seen on ICGA imaging corresponded strongly with the FAF and FFA.SD-OCT imaging showed a disrupted or irregular outer retina and punctate hyper-reflective lesions and hyper-reflective dots were observed in the outer nuclear layer(ONL).OCTA examination revealed a normal vascular pattern,including superfacial layer,deep layer,outer retina and choriocapillaris.mf-ERG showed the decreased peak of the macula.Conclusions Multimodal imaging analysis of the patients with multiple evanescent white dot syndrome shows important clinical significance that may be helpful in the diagnosis of the disease and in further exploring its pathogenesis.