周边部视网膜病变相关区域的自发荧光表现

目的 观察周边部视网膜病变相关区域的自发荧光(AF)表现.方法 42例周边部视网膜病变患者60只眼纳入本研究.所有患者均经全景眼底视网膜照相和荧光素眼底血管造影(FFA)检查确诊.应用共焦激光眼底血管造影仪HRAⅡ行黑色素相关近红外自发荧光(NIA)及脂褐质相关自发荧光(FAF)检查,分别采用波长为795、488 nm的激光激发成像.记录9张/s,由共焦激光眼底血管造影仪HRAⅡ自动合成高分辨影像视野为55°,像素为822×768的最终AF像.将AF像是否可见眼底血管及相关视网膜组织成像的影像判断为有、无价值AF像.病变区域是否出现符合正常血管和视网膜组织的荧光表现判断为正常、异常荧光.通过与图像背景灰度比较,将异常荧光分级为无荧光、弱荧光和强荧光,观察周边部视网膜病变相关区域的AF表现.对比分析NIA和FAF检查AF像均呈异常荧光表现者的异常荧光分级的一致性.结果 60只眼中,获取有价值AF像者53只眼,占88.33%;获取无价值AF像者7只眼,占11.67%.NIA检查显示,获取有价值AF像的53只眼中正常荧光28只眼,占52.83%;呈片状、圆点斑状、条带状异常荧光25只眼,占47.17%.FAF检查显示,获取有价值AF像的53只眼中正常荧光2只眼,占3.77%;呈片状或与色素分布较为一致或沿血管分布的异常荧光51只眼,占96.23%.两种检查均呈异常荧光表现的25只眼中,异常荧光分级一致者18只眼,占72.00%;异常荧光分级不一致者7只眼,占28.00%.结论 周边部视网膜病变相关区域存在不同程度的AF表现.

Abstract：

Objective To observe the autofluorescence (AF) manifestation in related lesions of periphery retinopathy. Methods Sixty eyes of 42 patients with periphery retinopathy underwent the examination of Optomap fundus photograph (200°) and fundus fluorescein angiography (FFA). The HRA Ⅱ melanin-related near-infrared fundus autofluorescence (NIA, excitation 795 nm) and lipofuscin-related fundus autofluorescence (FAF, excitation 488 nm) were measured for all the patients. The AF was recorded with nine images per second, and then a final AF image with 55° view and 822 × 768 pixel was generated by the HRA. AF images can be valuable or valueless if there was or was not visible blood vessels and related retinal tissues on the image. AF from lesion regions can be normal or abnormal fluorescence comparing to the normal vascular and retinal tissue AF. The abnormal fluorescence was divided into no AF, weak AF and strong AF relative to the background grayscale. The grading consistency of abnormal fluorescence based on FAF and NIA examination was comparatively analyzed. Results Valuable AF images were captured in 53/60 eyes (88. 33%)and valueless AF images were captured in 7/60 eyes (11.67%). Among 53 eyes with valuable AF image, NIA showed normal fluorescence in 28 eyes (52. 83%), abnormal fluorescence with sheet-like, dot-shaped or stripped in 25 eyes (47.17%); FAF showed normal fluorescence in two eyes (3.77 % ), abnormal fluorescence with sheet-like, scattered along vessels or pigments in 51 eyes (96.23 % ).Twenty-five eyes with abnormal fluorescence were observed both in two examinations, including same grades in 18 eye (72.00%) and different grades in seven eyes (28.00%). Conclusion The AF manifestation with different levels exists in related lesions of periphery retinopathy.     

眼底血管样条纹临床及荧光造影表现

目的 分析眼底血管样条纹(angioidstreaks,AS)的临床特征与眼底荧光血管造影(fundus fluorescein angiography,FFA)及吲哚青绿血管造影(fundus indocyanine green Angiography,ICG)表现.方法 对7例(14只眼)血管样条纹患者行眼科常规检查,眼底照相.其中6例(12只眼)患者行FFA及ICG检查.结果 眼底表现:14只眼眼底见视盘周围血管样放射状条纹,7只眼黄斑区视网膜下出血,黄白色硬性渗出,大小不一的灰黄色病灶(50.00%),3只眼见黄斑区萎缩瘢痕灶(21.43%);FFA表现:血管样条纹10眼表现为透见荧光(83.33%),2只眼表现为中央低荧光,两侧高荧光(16.67%),7只眼在黄斑区出现脉络膜新生血管(choroidalneovascularization,CNV)的强荧光(58.33%),3只眼黄斑区萎缩瘢痕灶晚期荧光着染(25.00%);ICG表现:12只眼血管样条纹表现为早期不显影,中期呈强荧光,7只眼CNV表现为强荧光,3只眼黄斑区萎缩瘢痕灶中2只眼见边界不清斑状CNV.结论 眼底血管样条纹患者的眼底较典型,FFA及ICG能进一步明确诊断,对疾病治疗有指导意义.

Abstract：

Objective To investigate the clinical manifestations, features of fundus fluorescein angiography(FFA)and fundus indocyanine green angiography(ICG)with angioid streaks. Methods Seven patients(14 eyes)with bilateral angioid streaks were examined by the routine ophthalmological examination, FFA and ICG. Colorfundus photographs were also taken. Results Fundus findings: angioid streaks were visualized in the posterior pole of 14 eyes and the macular areas of 7 eyes,(50.00%)had subretinal hemorrhages with neovascularization. FFA: streaks were hyperfluorescent in 10 eyes(83.33%), hypofluorescentareas between hyperfluorescent edges in 2 of the 12 eyes(16.67%), hyperfluorescent were seen in choroidal neovascularization (CNV)of macular in 7 eyes(58.33%). ICG: the result of streaks in 12 eyes showed hypofluorescence at the early phase and hyperfluorescence at the late phase, hyperfluorescent were seen in CNV of macular in 7 eyes, 2 eyes presented occult CNV. Conclusion In all eyes, angioid streaks are well visualized. FFA and ICG results may contribute to the diagnosis and treatment for patients with angioid streaks.     

家族性渗出性玻璃体视网膜病变的荧光素眼底血管造影特征及其诊断价值

目的 观察家族性渗出性玻璃体视网膜病变(FEVR)的荧光素眼底血管造影(FFA)特征,评价FFA对FEVR的诊断价值.方法 经临床检查诊断为FEVR的患儿34例68只眼及其父母64名128只眼纳入本研究.所有受检者均采用裂隙灯显微镜检查眼前节、间接检眼镜检查眼底.患儿同时应用RetcamⅡ视网膜成像系统检查眼底,患儿父母同时行最佳矫正视力检查.根据以上检查表现,对患儿及其父母行FEVR分期.采用RetcamⅡ视网膜成像系统在全身麻醉状态下对患儿行FFA检查;用海德堡HR2眼底血管造影设备对患儿父母行常规FFA检查.观察患儿及其父母不同分期FEVR的FFA特征.结果 患儿68只眼中,正常者3只眼,占4.41%;1期4只眼,占5.88%;2期7只眼,占10.29%;3期2只眼,占2.94%;4期8只眼,占11.76%;5期44只眼,占64.71%.患儿父母128只眼中,正常者74只眼,占57.81%;1期51只眼,占39.84%;2期1只眼,占0.78%;A;5期2只眼,占1.56%.患儿FFA检查发现,1期主要表现为视网膜血管发育不完全,未发育至周边即终止,视网膜周边无灌注.2期在1期FFA表现的基础上,在视网膜异常吻合处有新生血管形成和(或)视网膜渗出异常.3期在2期FFA表现的基础上,存在玻璃体牵引诱发的周边视网膜脱离,但未累及黄斑.4期主要表现为累及黄斑的视网膜脱离.5期主要表现为全视网膜脱离.患儿父母FFA检查发现,1期主要表现为视网膜血管近赤道部突然中止,出现周边无灌注区.2期在1期FFA表现的基础上,在视网膜无灌注区附近有动静脉短路和新生血管形成和(或)视网膜下渗漏.5期主要表现为眼球萎缩.结论 不同分期的FEVR存在不同程度的FFA特征表现;FFA检查可以发现FEVR患者的早期眼底改变,具有重要的诊断价值.

Abstract：

Objective To investigate the characteristics and diagnostic value of fundus fluorescein angiography(FFA)for familial exudative vitreoretinopathy(FEVR).Methods 34 children(68 eyes)with FEVR and 64 parents(1 28 eyes)were included.All the clients were received examinations of slit-lamp biomicroscopy and indirect ophthalmoscopy.Meanwhile the children were examined by Retcam Ⅱ,the best corrected visual acuity of parents were recorded.The children and their parents were classified according to the ocular findings.Among 68 eyes of children,3 eyes(4.41% )were normal,4 eyes(5.88% )were in stage 1,7 eyes(10.29% )were in stage 2,2 eyes(2.94% )were in stage 3,8 eyes(11.76% )were in stage 4 and 44 eyes(64.71% )were in stage 5.Among 128 eyes of parents,74 eyes(57.81% )were normal,51eyes(39.84% )were in stage 1,1 eyes(O.78% )were in stage 2 and 2 eyes(1.56% )were in stage 5.FFA was performed on the children with RetcamⅡunder anesthesia and on the parents with HR2 in order to observe the FFA characteristics in different stage.Results FFA characte ristics in children included uncompleted vascularization of the periphery,peripheral avascular zone(stage 1);neovascularization and/or peripheral subretinal and intraretinal exudation(stage 2);subtotal retinal detachment with attached foyea (stage 3);subtotal retinal detachment with detached foyea(stage 4)and total retinal detachment(stage 5).FFA characteristics in parents included abrupt cessation of the peripheral retinal capillary network and a peripheral avascular zone(stage 1); abnormal peripheral arteriovenous shunts, neovascularization or exudation(stage 2)and atrophia bulbi(stage 5).Conclusions FEVR in different stage has different FFA characteristics.FFA plays an important role in early diagnosis of FEVR.     

中心性浆液性脉络膜视网膜病变荧光素渗漏点的自身荧光改变

目的 观察中心性浆液性脉络膜视网膜病变(CSC)患者荧光渗漏点区域的眼底自身荧光(FAF)改变特点.方法 采用海德堡视网膜血管造影仪对CSC患者67例67只眼行荧光素眼底血管造影(FFA)检查.其中,年龄≤45岁者47只眼,＞45岁者20只眼;急性CSC患者25只眼,慢性或复发性CSC 患者42只眼.使用488 nm波长激光采集FAF图像,观察荧光渗漏点区域的FAF改变特点.结果 67只眼中,FFA荧光渗漏点位置FAF无异常者35只眼,占52.2%;FAF呈点状弱荧光者16只眼,占23.9%;FAF呈小片状弱荧光或斑驳荧光者10只眼,占14.9%;FAF呈强荧光者6只眼,占9.0%.年龄≤45岁的47只眼中,FFA荧光渗漏点区域FAF无异常者26只眼,占55.3%;FAF呈点状弱荧光者11只眼,占23.4%;FAF呈小片状弱荧光或斑驳荧光者7只眼,占14.9%.FAF呈稍强荧光者3只眼,占6.3%.＞45岁的20只眼中,FFA荧光渗漏点位置FAF无异常者9只眼,占45.0%;FAF呈点状弱荧光者5只眼,占25.0%;FAF 呈片状弱荧光或斑驳荧光者3只眼,占15.0%,FAF呈强荧光者3只眼,占15.0%.急性CSC 25只眼中,FFA荧光渗漏点位置FAF无异常改变者20只眼,占80.0%;FAF呈点状弱荧光者4只眼,占16.O%FAF 呈小片状弱荧光或斑驳荧光者1只眼,占4.0%.慢性或复发性CSC 42只眼中,FFA荧光渗漏点区域无异常改变者15只眼,占35.7%;FAF呈点状弱荧光者12只眼,占28.6%;FAF呈小片状弱荧光或斑驳荧光者9只眼,占21.4%;FAF呈强荧光者6只眼,占14.3%.结论 不同年龄和病程的CSC患者FFA荧光渗漏点位置具有特征性的FAF改变.

Abstract：

Objective To investigate the characteristics of fundus autofluorescence (AF) in the leakage site of central serous chorioretinopathy (CSC). Methods Sixty-seven CSC patients (67 eyes)underwent fundus fluorescein angiography (FFA) examination with a confocal scanning angiography (HRA2). Autofluorescence was elicited by the wavelength of 488 nm. The patterns of autofluorescence corresponding to the leakage site on FFA were observed. All the enrolled patients were grouped by age (age≤45 in 47 eyes and age ＞45 in 20 eyes) and courses (acute CSC in 25 eyes and chronic or recurrent CSC in 42 eyes). the patterns of autofluorescence were analyzed respectively. Results There are 4 patterns of AF in the leakage site on FFA of CSC patients: no AF changes, punctuate hypo-AF, expanded hypo-AF or speckled AF, hyper-AF. The percentages of those patterns in all 67 eyes are 52. 2%, 23. 9% , 14. 9% and 9.0% respectively. The percentages of those patterns in the group of age ≤45 (n=47) are 55.3%,23. 4% , 14. 9% and 6. 3% respectively. The percentages of those patterns in the group of age ＞45 (n=20)are 45. 0% , 25. 0% , 15. 0% and 15. 0% respectively. The percentages of those patterns in acute CSC (n=20) are 80.0%, 16.0%, 4.0% and 0% respectively. The percentages of those patterns in chronic or recurrent CSC (n=42) are 35.7%, 28.6%, 21.4% and 14.3% respectively. Conclusion There are different patterns of fundus autofluorescence in different age and courses of CSC patients.     

眼底荧光血管造影在1期糖尿病视网膜病变诊断中应用价值

目的 探讨糖尿病视网膜病变(DR)国际临床分类法1期病变患者眼底荧光血管造影(FFA)表现,评价FFA对早期DR的诊断价值.方法 对76例(152只眼)诊断为糖尿病视网膜病变国际临床分级标准1期病变患者进行眼底及眼底荧光血管造影检查,分析其造影表现.结果 检眼镜下152只眼眼底全部未见异常表现,FFA正常48只眼(31.58%).FFA异常104只眼(68.42%),异常表现中单纯微动脉瘤(MA)强荧光56只眼(53.84%),MA合并毛细血管扩张15只眼(14.41%),MA合并黄斑水肿5只眼(4.81%),黄斑拱环破坏3只眼(2.87%),小片状毛细血管无灌注2只眼(1.92%),窗样缺损23只眼(22.15%).结论 被诊断为DR国际临床分类法1期的患者,大部分已经出现了不同程度的FFA异常表现.因此,FFA是DR早期诊断的较好方法,能提高对DR早期诊断的准确度.

Abstract：

Objective To investigate the fundus fluorescence angiography (FFA) performance of diabetic retinopathy (DR) according to the international clinical classification of period l's patients. To evaluate the diagnostic value of FFA in early DR. Methods Fundus and fundus fluorescence angiography examination were performed and analyzed in 76 of period 1 patients (152 eyes) for the diagnosis of diabetic retinopathy according to the international clinical classification standard of. Results All 152 eyes fundus had no abnormal performance under ophthalmoscope, FFA normal 48 eyes (31.58%); FFA abnormal 104 eyes (68.42%), abnormal performance among a simple micro-aneurysms (MA), high fluorescence 56 eyes (53.84%), MA merge in telangiectasia 15 eyes (14.41%), MA merge in macular edema 5 eyes (4.81%), macular arch ring had damaged 3 eyes (2.87%), small flake with capillary nonperfusion 2 eyes (1.92%), window defect 23 eyes (22.15%).Conclusions DR is diagnosed with period 1 patients according to the international clinical classification, most of the emergence of difference degrees abnormal performance of the FFA. Therefore, FFA is the better method in early diagnosis of DR. It can increase the accurate of diagnosis for DR.     

中心性浆液性脉络膜视网膜病变眼底影像检查特征对比观察

目的 观察探讨中心性浆液性脉络膜视网膜病变(CSC)影像检查特征及其意义.方法 18例临床确诊的CSC患者的21只眼纳入观察.其中,男性12例14只眼,女性6例7只眼.年龄26～47岁,平均年龄(39.1±5.4)岁.急性CSC 9例11只眼,慢性CSC 7例7只眼,复发性CSC 2例3只眼.采集患眼的眼底彩色像以及眼底红外(IR)、自身荧光(FAF)、红外自身荧光(NIR-AF)、荧光素眼底血管造影(FFA)联合吲哚青绿血管造影(ICGA)的图像,对比观察其影像特征及各种影像特征的相互关系.结果 21只患眼的彩色眼底像均可见呈圆形的黄斑区浆液性视网膜神经上皮脱离.在IR像中,21只眼的浆液性视网膜神经上皮脱离区均呈弱反光区.其中,10只眼的弱反光区内夹杂斑驳状强反光斑点,与FFA检查所示渗漏部位一致.在FAF像中,15只眼的视网膜神经上皮脱离区表现为弱荧光;6只眼的视网膜神经上皮脱离区表现为较强荧光.其中,14只眼的FFA检查所示渗漏灶对应处或周边结构可见弱或强荧光,7只眼的FFA检查所示渗漏灶未见异常荧光.3只急性CSC患眼的FAF像上可见视网膜神经上皮脱离区下方大片散在的点状强荧光,这些点状强荧光在ICGA检查期间始终表现为弱荧光.在NIR-AF像中,15只眼的浆液性视网膜神经上皮脱离区呈弱荧光;6只眼的浆液性视网膜神经上皮脱离区呈相对强荧光.其中,14只眼的FFA检查所示渗漏灶对应处呈强或弱或斑驳状荧光,7只眼的FFA检查所示渗漏灶未见异常荧光.在FFA检查时,21只眼均可见荧光渗漏.在药物注射1～5 min的ICGA像中,8只眼表现为区域性脉络膜充盈迟缓;13只眼表现为区域性脉络膜静脉扩张.FFA联合ICGA像中,6只眼在ICGA像中显示的病灶数比在FFA像中多;3只眼FFA检查未显示异常的部位在ICGA晚期像中可见明显的斑片状弱荧光.结论 CSC在IR、FAF及NIR-AF上有其特征性的眼底影像表现.FFA为CSC的主要检查方法,ICGA可以更好的揭示其脉络膜的损害;IR、FAF及NIR-AF对于渗漏灶的检出不如FFA及ICGA.     

中心性浆液性脉络膜视网膜病变的两种眼底自发荧光特征观察

背景 眼底自发荧光(AF)成像是一种新的非侵人性眼底荧光检测技术,可利用共焦激光扫描检眼镜获得两种眼底AF,包括脂褐素相关的AF(FAF)和黑色素相关的近红外AF(NIA).目的 探讨中心性浆液性脉络膜视网膜病变(CSC)患者眼底FAF和NIA两种AF特征.方法 对CSC患者23例28眼进行FAF、NIA和荧光素眼底血管造影(FFA)检查,其中急性期CSC 15例17眼,慢性迁延性CSC 8例11眼.结果 急性期CSC患者FFA荧光素渗漏点AF改变有3种特征:(1)AF增强,包括FAF增强2眼,占11.76％;NIA增强4眼,占23.53％.(2)无AF,包括FAF 10眼,占58.82％;NIA 13眼,占76.47％.(3)AF正常,包括FAF 5眼,占29.42％;NIA 0眼.视网膜浆液性脱离区AF改变有2种特征:(1)AF减弱,包括FAF减弱12眼,占70.59％;NIA减弱10眼,占58.82％.(2)AF增强,包括FAF增强5眼,占29.41％;NIA增强7眼,占41.18％.慢性迁延性CSC患者AF像中,FFA检查视网膜色素上皮(RPE)渗漏点位置表现为无AF,部分无AF点在相应位置的FFA像未见RPE渗漏点,而N1A像中所见的无AF点常常多于FAF.慢性CSC视网膜浆液性脱离区常表现为颗粒样无AF、AF增强及AF减弱等多种AF改变并存的复合病灶,并且AF像显示的异常荧光范围常常大于对应的FFA显示的异常荧光区.结论 AF技术为研究CSC提供了一种活体观察RPE细胞代谢和功能改变的手段.     

65例Stargardt病及眼底黄色斑点症临床和眼底荧光素血管造影分析

目的 观察Stargardt病及眼底黄色斑点症(fundus flavimaculatus，FF)的临床表现及荧光素眼底血管造影(fundus fluoreseein angiography，FFA)特征。方法回顾分析65例Stargarclt病及FF患者130只眼的眼底和FFA检查资料。结果 Stargardt病及FF患者59．0％的患眼视力在0．1以下；90．8％的患眼检眼镜检查可见黄斑区呈“金箔样”反光，其中67．8％的患眼黄斑区视网膜色素上皮呈椭圆型萎缩，萎缩灶范围在1～2个视盘直径(disc diameter，DD)之间，FFA检查黄斑区均表现为横椭圆形强透见荧光，其中52．3％的患眼合并有暗脉络膜背景荧光；23．1％的患眼同时有黄斑及后极部的黄色斑点，FFA检查表现为斑驳状荧光。结论 stargardt病及FIF的临床特征为低视力、黄斑“金箔样”反光，FFA表现为黄斑区横椭圆形透见荧光及斑驳状荧光。     

原田病药物治疗后吲哚青绿眼底血管造影观察

目的 探讨利用吲哚青绿血管造影检查作为原田(Harada)病临床观察指标的意义.方法 对经药物治疗后19例(38只眼)原田病患者同时行荧光素眼底血管造影(fundus fluorescein angiography,FFA)及吲哚青绿血管造影(indocyanine green angiography,ICGA)检查资料进行分析.结果 FFA检查发现有4例(8只眼)视盘轻度染色,视网膜未发现异常改变;ICGA早期及中期无异常表现,但后期8只眼(包括FFA中视盘染色的4只眼)在中周及后极部出现了点状或斑片状弱荧光;3例6只眼FFA及眼底正常,在ICGA后期后极部出现了斑点状弱荧光;24只眼脉络膜大、中血管影像减少,其中12只眼dalen-fuchs结节着色.结论 ICGA可较好提供原田病的脉络膜循环损害的信息,并在评价疗效上有意义.

Abstract：

Objective To investigate the clinical value of indocyanine green angiography(ICGA)in patients with Harada disease.Methods Fundus fluorescein angiography(FFA)and indocyanine green angiography(ICGA)were used for comparative analyses in 26 cases(52 eyes)of Harada disease after treatment.Results Mild dyeing in disk was found only in 4 cases(8 eyes),and the others were not found any abnormal in FFA.There were no any abnormal found in early period and middle period of ICGA,but of 8 eyes(include 4 eyes of dyeing in disk in FFA)multifocal lower fluorescence were found in the mid-periphery and posterior pole of the fundus in the late phase.Of 3 cases(6 eyes)were normal in FFA,however multifocal lower fluorescence were found in the posterior pole of the fundus in the late phase of ICGA.Of 24 eyes were found decreased fluorescence in large and middle choroidal vessel,and 12 eyes of Dalen-Fuchs were colored.Conclusions ICGA may assist in providing valuable information on choroidal circulation of Harada disease and be useful in evaluating the curative effects.     

息肉状脉络膜血管病变在眼底FFA 与ICGA中特征分析

目的 探讨息肉状脉络膜血管病变(PCV)眼底荧光素血管造影(FFA)和吲哚青绿血管造影(ICGA)同步检查的影像学特征及其临床意义.方法 选取62例患者(75只眼)进行眼底彩色照相、FFA 和ICGA同步检查,对黄斑部视网膜下出血病灶其形态及特征做以对比分析.结果 FFA图像显示:PCV75只眼中斑块样视网膜下出血48只眼,占64.0%;伴浆液性视网膜色素上皮(RPED)脱离12只眼,占16.0%;出血性RPED8只眼,占10.7%;脉络膜血管网及息肉样结节7只眼,占9.4%,早期即出现荧光素渗漏并逐渐增强,CNV末端膨大处出现点状高荧光.ICGA图像显示:75只患眼中72只眼呈"蘑菇"状或"树枝"状异常扩张,其血管网末端多个息肉样膨大与彩色照相所见的结节样病变部位吻合.结论 FFA和ICGA同步检查,不但对PCV患者病灶其形态及特征作出对比分析,ICGA检查还能提高PCV视网膜下脉络膜新生血管膜(CNV)的检出率,同时提高PCV患者激光光凝的可能性,而FFA对评估PCV 视网膜下出血及出血性或浆液性视网膜色素上皮脱离、脉络膜血管网及息肉状结节则均有较好的效果.

Abstract：

Objective To observe on fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) in polypoidal choroidal vasculopathy (PCV), and sync examine of its image features and clinical meaning. Methods Sixty-two patients (75 eyes) applied fundus camera, FFA and ICGA synchronous examination, do comparison analysis in macular sub-retinal hemorrhage shape and area. Results FFA image in 75 PCV patients showed 48 eyes (64.0%) with plaque sub-retinal hemorrhage, 12 eyes (16.0%) accompanied with retinal pigment epithelial detachments (RPED), 8 eyes (10.7%) with bleeding RPED, 7 eyes (9.4%) with choroid blood vessel net and polypoidal node of early stage flouresence leakage. ICGA showed that 75 eyes had abnormal choroid blood vessel net extension, 72 with "mushroom" shape or "tree-branch" shape with more polypoidal enlarge in the end which was the same as seen under ophthalmoscope. Conclusions It proves that FFA and ICGA synchronous examination, not only can be used for PCV patients' tissue shape and area comparison analysis, but also improves the finding rate of the choroidal neovascularization (CNV) in PCV, increases the feasibility of photocoagulation to PCV. FFA gives good result to evaluate PCV sub-retinal hemorrhage.     

Fundus autofluorescence in children and teenagers with hereditary retinal diseases

Introduction In adults, evaluation of fundus autofluorescence (AF) plays an important role in the differential diagnosis of retinal diseases. The aim of this study was to evaluate the feasibility of recording AF in children and teenagers and to define typical AF findings of various hereditary retinal diseases during childhood. Methods Fifty patients aged 2 to 16 years with hereditary retinal diseases were analysed using the HRA (Heidelberg Retina Angiograph). To enhance the AF signal, a mean of up to 16 single images was calculated. Twenty healthy children (aged 4–16 years) served as controls. Results In many children as young as 5 years of age and even in one 2-year-old child good AF images could be obtained. To achieve high quality images, larger image series (about 50 single images) were taken and appropriate single images were chosen manually to calculate the mean. Characteristically, Stargardt disease shows a central oval area of reduced AF, often surrounded by more irregular AF. In patients with Best disease, a central round structure with regular or irregular intense AF is visualised. Some patients with X-linked retinoschisis show central radial structures. In many patients with rod-cone dystrophies, a central oval ring-shaped area of increased AF is present. In early-onset severe retinal dystrophy (EOSRD) with RPE65 mutations AF is completely absent, whereas in other forms of Leber congenital amaurosis, AF is normal. Discussion Fundus autofluorescence may visualise disease-specific distributions of lipofuscin in the retinal pigment epithelium, often not (yet) visible on ophthalmoscopy. AF images can be used in children to differentiate hereditary retinal diseases and to facilitate follow-up controls. In many cases, four single images are sufficient to analyse the AF pattern. Presented in part at the 102nd Meeting of the German Ophthalmological Society (DOG)     

Macular dystrophies

Macular dystrophies are a group of hereditary disorders of the macula occurring in children or young adults. The most frequent in France will be presented in detail: Best disease, Stargardt macular dystrophy, cone dystrophy, X-linked retinoschisis, pattern dystrophy, and malattia leventinese. Molecular biology studies have now mapped and identified the genes involved in these macular dystrophies. Analysis of the features of fundus examination will lead to further examinations such as fluorescein angiography, indocyanine green angiography, optical coherent tomography, electroretinography, or electrooculography, in order to confirm the diagnosis. We will also present the differential diagnosis of each of these macular dystrophies.     

Molecular genetics of macular degeneration

Macular degeneration is a leading cause of blindness that affects the aged population. The complexity of the molecular basis of macular disease is now beginning to be elucidated with the identification of disease-causing genes. For example, mutations in the ABCR gene, (recently identified in cones as well) which codes for retinal rod-specific ABCR protein is responsible for Stargardt macular dystrophy/fundus flavimaculatus, an autosomal recessive macular dystrophy with juvenile onset, which accounts for 7% of human retinal degenerative diseases. The gene mutant in X-linked juvenile retinoschisis, XLRS1, is the first macular dystrophy gene to be isolated by positional cloning. Mutations in the peripherin/RDS gene have been shown to be associated with a variety of distinct forms of macular degenerations. The tissue inhibitor of metalloproteinase 3 (TIMP3) is implicated in autosomal dominant Sorsby fundus dystrophy. Best vitelliform macular dystrophy was mapped to 11q12–q13. The cloned gene product is the protein bestrophin, which is a retinal specific gene expressed in the RPE and possibly involved in the metabolism and transport of polyunsaturated fatty acids. The cloning of genes for rare heritable forms of macular degeneration will increase our understanding of the basic pathogenesis of the disease process. In the future this should also allow us to test the hypothesis that the coincidence of subclinical mutations in a number of genes involved in the formation and function of the macula can be responsible for cases of age-related macula-degeneration which is by far the most common form of these macular disorders.     

成年人型卵黄样黄斑营养不良的临床特征

目的研究成年人型卵黄样黄斑营养不良的临床和影像学特征。设计回顾性病例系列。研究对象北京同仁医院9例（13眼）成年人型卵黄样黄斑营养不良患者。方法分析患者的眼底表现、荧光素眼底血管造影（FFA）、相干光断层扫描（OCT）和自体荧光检查结果。主要指标FFA及OCT特征。结果所有患者均无家族史。视力在0．3及以上者8／13眼（61．5％）。所有患者均表现为黄斑区圆形卵黄样微隆起、边界清楚的、不超过1PD的视网膜下病变。FFA显示病变处呈遮蔽荧光,其旁无或显现荧光,在吸收过程中,荧光相应增加。OCT显示在视网膜色素上皮（RPE）光带前见一梭形均匀的高反射区,在吸收过程中,高反射区出现不均匀,或有小的无光反射暗区。结论黄斑区圆形卵黄样不超过1PD的视网膜下病变,无明显视网膜脱离及无病变破裂分层是本病的特点。FFA和OCT检查相结合有助于成年人型卵黄样黄斑营养不良的诊断。     

Fundus autofluorescence imaging in patients with Stargardt dystrophy and fundus flavimaculatus

PURPOSE: To identify patterns of fundus autofluorescence (AF) in patients with Stargardt dystrophy and fundus flavimaculatus. MATERIAL AND METHODS: 20 patients in age 7 to 47 years with Stargardt dystrophy and fundus flavimaculatus, were examined. Ophthalmic evaluation included nonstandarized Snellen visual acuity, complete ophthalmic examination, fluorescein angiography and color fundus photographs. The autofluorescence images were obtained using a confocal scanning laser ophthalmoscope HRA2. RESULTS: In 15 patients lack or decreased AF signal in a foveal region with punctate diffuse spots with increased and decreased AF signal extending far from the macular region were observed. In 4 patients apart from lack of the AF signal in the central area punctate spots were restricted to the macular region. In 1 patient AF image was copletely normal. CONCLUSIONS: Autofluorescence imaging allows for evaluation of the area with changes on the RPE level typical for this disease and is helpful, noninvasive examination for diagnostic process in such patients. A wide variation in clinical phenotype can occur in patients with Stargardt disease and fundus flavimaculatus. Obtained different phenotypes in autofluorescence imaging may correlate with different clinical types of this disease, described in the literature.     

卵黄样黄斑营养不良的光学相干断层扫描影像学特征

目的探讨卵黄样黄斑营养不良各病变阶段光学相干断层扫描(optical coherence tomography,OCT)的影像学特征。方法回顾性分析6例(8眼)卵黄样黄斑营养不良患者的OCT图像特征,并与眼底特征、眼底荧光血管造影进行对照分析。结果卵黄样病变期患者眼底检查可见黄斑区卵圆形隆起病灶,OCT可见视网膜色素上皮层和光感受器层之间有一中等密度反射区域,随病变进展,沉积物厚度增高。萎缩期患者黄斑区见萎缩灶,OCT表现为视网膜色素上皮层脉络膜复合体弥漫性增厚,神经感觉层变薄,合并脉络膜新生血管形成时可见高反射的新生血管膜。结论详细的病史资料、眼底检查、眼底荧光血管造影以及OCT的联合应用,有助于更科学的分析卵黄样黄斑营养不良的临床特征和病理改变。     

Atypical vitelliform macular dystrophy misdiagnosed as chronic central serous chorioretinopathy: case reports

ABSTRACT: BACKGROUND: To report two cases of atypical vitelliform macular dystrophy misdiagnosed as chronic central serous chorioretinopathy. CASE PRESENTATION: Two patients with incidentally discovered abnormalities of the retina without specific symptoms were referred to our hospital for consultation. Bilateral macula atrophic lesions were observed and optical coherence tomography revealed serous retinal detachment in the macula. Fluorescein angiography showed multiple leakages around the central hypofluorescent area and indocyanine green angiography showed partially dilated choroidal vessels. Fundus autofluorescence (FAF) showed a decreasing pattern of autofluorescence in the subretinal fluid area, and increasing autofluorescence at the border of the serous retinal detachment. Both patients were diagnosed with chronic central serous chorioretinopathy. Photodynamic therapy and intravitreal bevacizumab injection were administered for engorged choroidal vessels during follow-up, but neither patient showed improvement in symptoms or ophthalmologic findings. Based on re-evaluation by fundus photography, optical coherence tomography, fluorescein angiography, and comparison of the results of FAF with the first visit, vitelliform macular dystrophy was suspected and a definite diagnosis was made by electrooculography and genetic testing. CONCLUSION: In patients with continuous serous retinal detachment without response to photodynamic therapy or intravitreal bevacizumab injection, careful fundus exam and FAF can be used to diagnose atypical vitelliform macular dystrophy.     

Fundus autofluorescence imaging of retinal dystrophies

Fundus autofluorescence (FAF) is a non-invasive imaging technique that enables the visualization of lipofuscin changes in the retinal pigment epithelium. This study aims to illustrate the spectrum of FAF changes in a variety of retinal dystrophies. For this purpose, we examined patients with retinal dystrophies such as Stargardt disease, Best vitelliform macular dystrophy, and retinal dystrophies associated with mutations in the peripherin/RDS gene. All retinal dystrophies were confirmed by molecular genetic analysis. A broad range of characteristic FAF patterns was observed. Our results indicate that FAF imaging constitutes a useful additive tool in the diagnosis and follow-up of various retinal dystrophies.     

Diagnosis and classification of macular degenerations: an approach based on retinal function testing

The results from literature concerning some aspects of retinal function in macular degenerations (MDs) were reviewed in order to evaluate whether (a) specific patterns of retinal dysfunction may be linked to different clinical phenotypes, and (b) distinct functional profiles may help in orienting molecular diagnosis of diseases. Examined clinical phenotypes included: Stargardt disease/fundus flavimaculatus (St/FF), age-related maculopathy (ARM) and macular degeneration (AMD), pattern dystrophies (PD), Best vitelliform dystrophy (BVD), Sorsby's fundus dystrophy (SFD), autosomal cone-rod dystrophies (CRD). The following functional tests were evaluated: (1) electroretinogram (ERG) (scotopic and photopic according to ISCEV standards, rod and cone photoresponses, rod and cone b-wave intensity-response function, focal ERGs); (2) dark adaptometry (pre-bleach sensitivity and post-bleach recovery kinetics); (3) fundus reflectometry (pigment density and regeneration kinetics). Specific patterns of retinal dysfunction were identified for St/FF, ARM/AMD, SFD and BVD, whereas partially overlapping profiles were found for PD and CRD. Specific functional patterns were associated with different peripherin/RDS gene mutations, as well as with CRX mutations. Combined analysis of different retinal function tests may help to identify different phenotypes of MD, and to orient molecular diagnosis for selected genotypes.     

Vitelliform maculopathy: Diverse etiologies originating from one common pathway

Vitelliform lesions (VLs) are associated with a wide array of macular disorders but are the result of one common pathway: retinal pigment epithelium (RPE) impairment and phagocytic dysfunction. VLs are defined by the accumulation of yellowish subretinal material. In the era of multimodal advanced retinal imaging, VLs can be further characterized by subretinal hyperreflectivity with optical coherence tomography and hyperautofluorescence with fundus autofluorescence. VLs can be the result of genetic or acquired retinal diseases. In younger patients, VLs usually occur in the setting of Best disease. Additional genetic causes of VL include pattern dystrophy or adult-onset vitelliform macular dystrophy. In older patients, acquired VLs can be associated with a broad spectrum of etiologies, including tractional, paraneoplastic, toxic, and degenerative disorders. The main cause of visual morbidity in eyes with VLs is the onset of macular atrophy and macular neovascularization. Histopathological studies have provided new insights into the location, nature, and lifecycle of the vitelliform material comprised of melanosomes, lipofuscin, melanolipofuscin, and outer segment debris located between the RPE and photoreceptor layer. Impaired phagocytosis by the RPE cells is the unifying pathway leading to VL development. We discuss and summarize the nature, pathogenesis, multimodal imaging characteristics, etiologies, and natural course of vitelliform maculopathies.