黄斑中心凹旁渗出性血管异常复合体临床特征及多模式影像特征观察

Clinical characteristics and multimodal imaging of perifoveal exudative vascular anomalous complex

目的观察黄斑中心凹旁渗出性血管异常复合体(PEVAC)患眼的临床特征和多模式影像特征。方法回顾性病例研究。2014年2月至2020年11月于北京大学人民医院眼科检查确诊的PEVAC患者7例7只眼纳入研究。其中,男性6例,女性1例;年龄(60.1±9.1)岁。均为单眼。主诉视物变形、视力下降分别为3、1例。患者均行最佳矫正视力(BCVA)、眼底彩色照相、光相干断层扫描(OCT)、荧光素眼底血管造影(FFA)检查。BCVA检查采用标准对数视力表进行,统计时换算为最小分辨角对数(logMAR)视力。行OCT血管成像(OCTA)、吲哚青绿血管造影(ICGA)检查分别为4、2只眼。行玻璃体腔注射抗血管内皮生长因子(VEGF)药物联合病灶局部激光光凝治疗3只眼;单纯激光光凝治疗2只眼。随访时间(16.7±19.1)个月。随访时采用首诊时相同设备和方法行相关检查。观察患眼多模式影像特征及治疗反应。结果患眼基线logMAR BCVA为0.33±0.19(0.20~0.80)。所有患眼均可见黄斑中心凹旁孤立血管瘤样病变,伴视网膜硬性渗出,其中可见2个相邻的孤立血管瘤样病变1只眼。FFA、ICGA检查,所有患眼黄斑旁血管瘤样病变早期呈边界清晰的强荧光;未见其他视网膜或脉络膜血管异常。OCT检查,所有患眼黄斑区均可见中心凹旁带有强反射壁的类圆形管腔样结构,伴视网膜内囊腔。黄斑中心凹视网膜厚度(CMT)为(326±125)(207~479)μm。行OCTA检查的4只眼,中心凹旁带有强反射壁的类圆形管腔样结构内均可见血流信号。其中,视网膜浅层毛细血管层(SCP)、深层毛细血管层(DCP)均可见血流信号3只眼;仅SCP可见血流信号1只眼。行玻璃体腔注射抗VEGF药物治疗的4只眼,治疗后视网膜内囊腔无明显改善;视网膜下液吸收、视网膜囊样水肿持续存在、硬性渗出减少1只眼。联合或单纯激光光凝治疗的5只眼治疗后CMT降低,BCVA提高。末次随访时,所有患眼logMAR BCVA 0.16±0.06(0.10~0.20),CMT为(212±34)(154~252)μm;与基线时比较,BCVA差异有统计学意义(t=2.661,P=0.037)。结论PEVAC患者多单眼患病,其眼底表现为黄斑中心凹旁孤立或多发的孤立血管瘤样病变;OCT可见带有强反射壁的类圆形管腔样结构,伴或不伴视网膜内囊腔、硬性渗出和视网膜下液,其内可见血流信号。

Objective:To investigate the clinical features and multimodal imaging features of eyes with perifoveal exudative vascular anomalous complex(PEVAC).Methods:A retrospective case study.From February 2014 to November 2020,7 eyes of 7 patients with PEVAC diagnosed by ophthalmology examination in Department of Ophthalmologyof Peking University People's Hospital were included in this study.There were 6 males and 1 female.The age was 60.1±9.1 years.All were monocular.The chief complaints of visual deformation and vision loss were 3 and 1 cases,respectively.All patients underwent best corrected visual acuity(BCVA),fundus color photography,optical coherence tomography(OCT),fundus fluorescein angiography(FFA).BCVA examination was performed using the standard logarithmic visual acuity chart,which was converted to logarithm of the minimum angle of resolution(logMAR)visual acuity.OCT angiography(OCTA)and indocyanine green angiography(ICGA)were performed in 4 and 2 eyes,respectively.Three eyes were treated with intravitreal injection of anti-vascular endothelial growth factor(VEGF)combined with local laser photocoagulation.Two eyes were treated with laser photocoagulation alone.The follow-up time was 16.7±19.1 months.During follow-up,relevant examinations were performed with the same equipment and methods as at the initial diagnosis.The multimodal imaging characteristics and treatment response of the affected eyes were observed.Results:The baseline logMAR BCVA was 0.33±0.19(0.20-0.80).All eyes showed isolated hemangiomatous lesions in the macular fovea with rigid retinal exudation,and 2 adjacent isolated hemangiomatous lesions were observed in 1 eye.FFA and ICGA examination showed that all eyes with macular hemangiomatous lesions showed clear boundary and strong fluorescence in the early stage.No other retinal or choroidal vascular abnormalities were observed.On OCT examination,circular lumen-like structures with strong reflective wall near the fovea were observed in the macular region of all eyes,accompanied by intraretinal cystic lumen.The macular central retinal thickness(CMT)was 326±125(207-479)μm.In the four eyes examined by OCTA,blood flow signals were observed in the circular lumenoid structures with strong reflective walls adjacent to the fovea.Blood flow signals were observed in the superficial capillary layer(SCP)and deep capillary layer(DCP)of the retina in 3 eyes.SCP showed blood flow signal in 1 eye.In 4 eyes treated with intravitreal injection of anti-VEGF drugs,there was no significant improvement in the intraretinal capsule space after treatment.Subretinal fluid absorption,retinal cystoid edema persisted,and rigid exudation decreased in 1 eye.CMT decreased and BCVA increased in 5 eyes treated with laser photocoagulation or laser photocoagulation alone.At last follow-up,logMAR BCVA was 0.16±0.06(0.10-0.20)and CMT was 212±34(154-252)μm.Compared with baseline,the difference of BCVA was statistically significant(t=2.661,P=0.037).Conclusions:The fundus of PEVAC patients is characterized by solitary or multiple solitary hemangiomatous lesions in the macular fovea.Round lumenoid structures with strong reflective walls,with or without intraretinal cystic lumen,rigid exudate,and subretinal fluid,in which blood flow signals can be seen in OCT.