斑点状视网膜脉络膜病变的多模式影像学分析

Multimodal Imaging Analysis of Punctate Inner Choroidopathy

目的分析斑点状视网膜脉络膜病变患者的多模式影像学特征。方法纳入2017年4月—2022年11月于西安交通大学第一附属医院眼科门诊经多模式影像诊断为斑点状视网膜脉络膜病变的患者5例(9只眼),分别为多灶性一过性白点综合征(MEWDS)、点状内层脉络膜病变(PIC)、急性后极部多灶性鳞状色素上皮病变(APMPPE)、顽固性进行性鳞状脉络膜视网膜炎(RPC)、多灶性脉络膜炎伴全葡萄膜炎(MCP)各1例。所有患者分别于治疗前、后进行视力、眼压、裂隙灯、眼底彩照及光学相干断层扫描(OCT)检查,MEWDS患者行眼底自发荧光检查,PIC、APMPPE、RPC患者行荧光素眼底血管造影(FFA)检查,APMPPE、RPC患者行吲哚菁绿血管造影(ICGA)检查,MCP患者行光学相干断层扫描血管成像(OCTA)检查。结果斑点状视网膜脉络膜病变中各类疾病的影像学表现既类似又有区别。(1)PIC:活动性病灶呈高自发荧光,在FFA早期呈强荧光,晚期荧光素渗漏,病变晚期可出现视网膜脉络膜萎缩的荧光缺损。ICGA表现为后极部多发弱荧光斑。OCT见病灶部位视网膜色素上皮(RPE)层向上隆起呈局灶性高反射,对应处光感受器内外节破坏,病变晚期开始出现萎缩,在RPE表现为不规则的高反射信号。(2)MCP:萎缩性病灶为低自发荧光,活动性病灶为轻度高自发荧光。FFA表现为进行性斑点状强荧光,病灶萎缩亦呈窗样荧光缺损,ICGA亦体现弱荧光斑点。(3)MEWDS:病灶表现为高自发荧光。FFA表现为“花环状”排列的强荧光点,ICGA表现为弱荧光斑点。OCT表现为椭圆体带紊乱,少数病人RPE上圆顶状高反射或椭圆体带和外核层垂直线性高反射。(4)APMPPE:病变急性期表现为低自发荧光,后期伴高自发荧光。FFA早期低荧光,晚期高荧光,ICGA提示比FFA范围更大的脉络膜低灌注改变的低荧光。OCT表现为椭圆体带破坏伴有局部高反射点。(5)RPC:位于后极部低自发荧光的斑点状改变,FFA早期病灶低荧光,晚期高荧光,ICGA全期呈低荧光样改变。当病变累及黄斑中心凹,OCT示视网膜下液及伴有视网膜内外层高反射性物质的RPE层分离。结论多模式影像学检查技术有助于深入认识疾病的本质,明确疾病的诊断及制定合理治疗随访方案。

OBJECTIVE To analyze the multimodal imaging characteristics of patients with punctate inner choroidopathy.METHODS A total of five patients(nine eyes)diagnosed with punctate inner choroidopathy at the Department of Ophthalmology,First Affiliated Hospital of Xi'an Jiaotong University,from April 2017 to November 2022 were included.The cases included one instance each of multiple evanescent white dot syndrome(MEWDS),punctate inner choroidopathy(PIC),acute posterior multifocal placoid pigment epitheliopathy(APMPPE),relentless placoid chorioretinitis(RPC),and multifocal choroiditis with panuveitis(MCP).Visual acuity,intraocular pressure,slit-lamp examination,fundus photography,and optical coherence tomography(OCT)were performed before and after treatment.Patients with multiple evanescent white dot syndrome(MEWDS)underwent fundus autofluorescence examination,while patients with punctate inner choroidopathy(PIC),acute posterior multifocal placoid pigment epitheliopathy(APMPPE),relentless placoid chorioretinitis(RPC),underwent fluorescein angiography(FFA).Indocyanine green angiography(ICGA)was performed for APMPPE and RPC patients,and optical coherence tomography angiography(OCTA)was conducted for patients with multifocal choroiditis and panuveitis(MCP).RESULTS The imaging manifestations of various diseases in punctate inner choroidopathy are both similar and different.(1)PIC:Active lesions showed high autofluorescence and early hyperfluorescence on FFA,with late leakage and eventual retinal choroidal atrophy.Lesions appeared as multiple weakly fluorescent spots on ICGA.OCT revealed focal hyperreflectivity of the retinal pigment epithelium(RPE)layer with corresponding disruption of the outer retina and RPE atrophy.(2)MCP:Atrophic lesions exhibited low autofluorescence,while active lesions showed mild hyperfluorescence.FFA revealed progressive punctate hyperfluorescence with areas of window defect.ICGA showed weakly fluorescent spots.(3)MEWDS:Lesions exhibited high autofluorescence.FFA showed a"wreath-like"pattern of hyperfluorescent dots,and ICGA revealed weakly fluorescent spots.OCT showed disrupted ellipsoid zone and occasionally hyperreflective round lesions on the RPE and/or vertical linear hyperreflectivity in the outer nuclear layer.(4)APMPPE:Lesions exhibited low autofluorescence in the acute phase and later high autofluorescence.FFA showed early hypofluorescence,late high fluorescence,and ICGA demonstrated larger areas of hypoperfusion than FFA.OCT revealed disrupted ellipsoid zone with localized hyperreflective points.(5)RPC:Spotty changes with low autofluorescence in the posterior pole.Early hypofluorescence on FFA,late high fluorescence,and ICGA showed persistent hypofluorescence.When lesions involved the fovea,OCT revealed subretinal fluid and detachment of the retinal pigment epithelium with hyperreflective material in the retina.CONCLUSIONS Multimodal imaging techniques contribute to a better understanding of the nature of the disease,enabling precise diagnosis and formulation of rational treatment and follow-up plans.