2例Stargardt病临床分析并文献复习  

Stargardt’s Disease:Clinical Analysis of 2 Cases and Literature Review

作　　者：李双莲 邓燕[1] 熊伟伟[1] 付燕梅 封炎 殷小龙[1] LI Shuang-lian;DENG Yan;XIONG Wei-wei;FU Yan-mei;FENG Yan;YIN Xiao-long(Department of Ophthalmology,the Second Affiliated Hospital of Nanchang University,Nanchang 330006,China;Department of Ophthalmology,Jiujiang Maternal and Child Health Hospital,Jiujiang 332001,China)

机构地区：[1]南昌大学第二附属医院眼科中心,南昌330006 [2]九江市妇幼保健院眼科,九江332001

出　　处：《南昌大学学报（医学版）》2021年第6期96-100,共5页Journal of Nanchang University：Medical Sciences

基　　金：江西省教育厅科技项目(GJJ190082)。

摘　　要：目的总结Stargardt病的临床辅助检查和处理经验,以完善其临床诊断及鉴别诊断。方法收集于儿童眼科就诊的2例Stargardt病患者的临床诊疗资料,总结处理经验,并对其发病原因、诊断与鉴别诊断及治疗的相关文献进行复习。结果病例1首次就诊在发病早期,双眼视力刚开始下降而眼底未见明显改变,被误诊为双眼球后视神经炎;第2次就诊时眼底出现异常,完善眼底荧光造影(FFA)后确诊为双眼Stargardt病,病情已至晚期阶段。病例2首次就诊时双眼视力下降且矫正无提高,眼底检查见黄斑区反光消失,黄斑区高分辨率光学相干断层成像(HD-OCT)提示视网膜黄斑区变薄、椭圆体带及外核层缺失,及时完善FFA,明确诊断为双眼Stargardt病。结论Stargardt病患者在发病早期易被误诊,临床若遇到出现双眼不明原因视力缓慢下降,且矫正视力无提高的青少年患者,应详细询问病史,及时完善相关辅助检查,以早期对Stargardt病进行诊断及鉴别诊断。Objective To summarize the experience with clinical auxiliary examination and treatment of Stargardt’s disease(STGD)in order to improve its clinical diagnosis and differential diagnosis Methods Clinical data of 2 cases of STGD admitted to the department of pediatric ophthalmology of the Second Affiliated Hospital of Nanchang University were collected to summarize the experience with treatment.In addition,the literature in terms of the etiology,diagnosis,differential diagnosis and treatment of STGD was reviewed.Results In case 1,STGD was at the early stage at the first visit to hospital,and binocular vision began to decline without visible fundus abnormality.The case was misdiagnosed as bilateral posterior optic neuritis.At the second visit,corresponding changes occurred in the fundus and advanced stage of STGD was confirmed by fundus fluorescein angiography(FFA).In case 2,binocular vision decreased at the first visit without improvement after correction.Fundus examination indicated that reflection disappeared in macular area.High resolution optical coherence tomography(HD-OCT)showed thinning of macular area and loss of ellipsoid zone and outer nuclear layer,and STGD was confirmed by FFA.Conclusion STGD patients are often misdiagnosed as amblyopia or retrobulbar optic neuritis in the early stage of the disease.For adolescent patients with unexplained bilateral vision loss and unimproved corrected visual acuity,detailed medical and family history should be obtained and FFA,HD-OCT and other auxiliary examinations should be timely improved for early diagnosis and differentiation of STGD.

关 键 词：STARGARDT病 眼底荧光造影 光学相干断层成像 鉴别诊断 误诊 

分 类 号：R774.5[医药卫生—眼科]