CAJ | 학술논문

目的观察视网膜和视网膜色素上皮联合错构瘤(CHRRPE)患眼的眼底影像特征.方法临床确诊为CHRRPE的8例患者8只眼纳入研究.其中,男性5例,女性3例;年龄6～21岁,平均年龄14.13岁.均为单眼发病.就诊时最佳矫正视力0.02～0.2.眼压、眼前节正常.所有患眼均行眼底彩色照相、眼底自身荧光(AF)、荧光素眼底血管造影(FFA)及频域光相干断层扫描(SD-OCT)检查.观察患眼的眼底影像检查特征.结果所有患眼的CHRRPE病灶均位于后极部,呈黄白色隆起,其周围色素沉着,病变部位视网膜血管纡曲以及前膜形成.8只眼中,病变累及黄斑和视盘3只眼,占37.5％;仅累及黄斑5只眼,占62.5％.病灶隆起处及其周围色素沉着区域AF亮度降低7只眼,占87.5％;AF亮度增强部位与病灶表面致密的前膜相对应1只眼,占12.5％.FFA检查发现,所有患眼病变部位及其周围脉络膜背景荧光减弱,视网膜血管因纤维膜收缩、牵引而呈螺旋样纡曲;病灶处视网膜毛细血管荧光素渗漏,病变部位荧光亮度逐渐增强.均未发生视网膜出血、渗出、毛细血管无灌注区、视网膜新生血管及脉络膜新生血管等异常.SD-OCT检查发现,所有患眼病变部位视网膜内层结构不清、增厚,呈强弱不均匀反射信号;视网膜色素上皮(RPE)及光感受器内外节连接(IS/OS)等外层视网膜组织反射信号不均匀减弱.8只眼中,因病灶表面纤维膜致密,导致其下方病灶内反射信号被屏蔽形成空洞样2只眼,占25.0％;病灶内存在与RPE相连的膜样强反射信号1只眼,占12.5％.结论 CHRRPE病灶及其周围视网膜色素沉着区域AF亮度降低;FFA检查早期可见病变部位脉络膜背景荧光减弱,造影过程中视网膜毛细血管荧光素渗漏;SD-OCT检查可见病变部位视网膜增厚、RPE及IS/OS反射信号减弱.
목적 관찰시망막화시망막색소상피연합착구류(CHRRPE)환안적안저영상특정.방법 림상학진위CHRRPE적8례환자8지안납입연구.기중,남성5례,녀성3례;년령6～21세,평균년령14.13세.균위단안발병.취진시최가교정시력0.02～0.2.안압、안전절정상.소유환안균행안저채색조상、안저자신형광(AF)、형광소안저혈관조영(FFA)급빈역광상간단층소묘(SD-OCT)검사.관찰환안적안저영상검사특정.결과 소유환안적CHRRPE병조균위우후겁부,정황백색륭기,기주위색소침착,병변부위시망막혈관우곡이급전막형성.8지안중,병변루급황반화시반3지안,점37.5％;부루급황반5지안,점62.5％.병조륭기처급기주위색소침착구역AF량도강저7지안,점87.5％;AF량도증강부위여병조표면치밀적전막상대응1지안,점12.5％.FFA검사발현,소유환안병변부위급기주위맥락막배경형광감약,시망막혈관인섬유막수축、견인이정라선양우곡;병조처시망막모세혈관형광소삼루,병변부위형광량도축점증강.균미발생시망막출혈、삼출、모세혈관무관주구、시망막신생혈관급맥락막신생혈관등이상.SD-OCT검사발현,소유환안병변부위시망막내층결구불청、증후,정강약불균균반사신호;시망막색소상피(RPE)급광감수기내외절련접(IS/OS)등외층시망막조직반사신호불균균감약.8지안중,인병조표면섬유막치밀,도치기하방병조내반사신호피병폐형성공동양2지안,점25.0％;병조내존재여RPE상련적막양강반사신호1지안,점12.5％.결론 CHRRPE병조급기주위시망막색소침착구역AF량도강저;FFA검사조기가견병변부위맥락막배경형광감약,조영과정중시망막모세혈관형광소삼루;SD-OCT검사가견병변부위시망막증후、RPE급IS/OS반사신호감약.
Objective To investigate the characteristics of fundus image in patients with combined hamartoma of the retina and retinal pigment epithelium (CHRRPE).Methods Eight eyes of 8 cases with CHRRPE were included in this study.There were 5 males and 3 females,ranging from 6 to 21 years old (mean 14.13 years).In all cases,unilateral eye was affected.The best-corrected visual acuity (BCVA) was 0.02 to 0.2.The intraocular pressure in all eyes was normal.The ocular anterior segment in all cases was alright.For all cases,fundus photography,fundus autofluorescence (AF),fundus fluorescein angiography (FFA) and spectral-domain optical coherence tomography (SD-OCT) were taken.The characteristics of fundus image were analyzed.Results All the CHRRPE lesions located at posterior pole,presenting as yellow-white elevations,surrounded by hyperpigmentation.Retinal vascular tortuosity and proliferation of epiretinal membrane were found in all CHRRPE lesions.In 8 affected eyes,lesions were involved in macula and optic disc in 3 eyes (37.5％),lesions were located in macula in 5 eyes (62.5％).AF images showed lesion bulges and the surrounding hyperpigmentation corresponding to the local hypo-AF in 7 eyes (87.5％),dense epiretinal membrane corresponding to the local hyper-AF in 1 eye (12.5％).FFA showed local hypofluorescence of choroidal background corresponding to lesions and the surrounding hyperpigmentation,tortuous retinal vascular due to traction of shrinkage membranes.Retinal capillary leakage and the fluorescence brightness gradually increased in all lesions.No retinal hemorrhage,exudation,area of capillary non-perfusion,retinal and choroidal neovascularization could be found in 8 CHRRPE eyes.SD-OCT showed disorganized and elevated lesion with uneven reflectivity in inner retina,mild attenuation of the retinal pigment epithelium (RPE) and photoreceptor inner segment/outer segment junction in all CHRRPE eyes.Of the 8 CHRRPE eyes,cavernous hypo-reflective shadowing due to the shielded optical signal acquisition below dense epiretinal membranes in 2 eyes (25.0％),membrane-like hyper-reflective signals connecting with RPE in 1 eyes (12.5％).Conclusions Decreased AF appear in the location of CHRRPE lesion and the surrounding hyperpigmentation.Hypofluorescence of choroidal background can be found in the early phase of FFA,the fluorescence brightness of CHRRPE lesion gradually increases in FFA process.The retinal elevation,attenuated signal reflection of the RPE and photoreceptor inner segment/ outer segment junction in CHRRPE lesion can be verified by OCT.