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
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