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。     

病理性近视黄斑出血的眼底特征及光学相干断层扫描分析

目的:探讨病理性近视黄斑出血的眼底改变及其光学相干断层扫描特征。方法:对42例43眼病理性近视(PM)黄斑出血患者进行眼底彩色照相、荧光素眼底血管造影(FFA)和光学相干断层扫描(OCT)检查。结果:病理性近视黄斑出血的表现分为两类:新生血管型:24例24眼,其眼底特征表现为出血灶呈类圆形,不超过1PD,周围少见渗出及水肿,部分可见新生血管膜;其FFA特征表现为典型性CNV17眼(71%),隐匿性CNV7眼(29%);其OCT特征表现为21眼(87.5%)CNV呈纺锤形或类圆形团块状,呈强或中等强度反射,自视网膜色素上皮层向上突出,位于视网膜神经上皮层下,3眼(12.5%)CNV呈不规则增强紊乱的反射信号,位于视网膜色素上皮层平面。单纯型:18例19眼;其眼底特征表现为出血灶扁平,大小不等,边界欠整齐,周围无渗出及水肿,其FFA特征表现为出血遮蔽荧光,无CNV性高荧光征象,部分患眼可见漆裂纹样透见荧光;其OCT特征表现为色素上皮层下隆起低反射区,脉络膜毛细血管层光带连续。结论:病理性近视新生血管型黄斑出血绝大部分由典型性CNV引起,OCT的典型图像为色素上皮层局限的纺锤状或类圆形的强反射光团,边界较清,视网膜向上隆起,下方可有脉络膜遮蔽;单纯型黄斑出血表现为局限性色素上皮层出血性脱离,OCT检查有利于明确病理性近视黄斑出血的病变性质和病因,并有助于判断预后和指导治疗。     

隐性遗传性卵黄样营养不良伴黄斑裂孔性视网膜脱离一例

19岁女性因视力差、内斜视就诊。双眼后极部较大范围、散在分布卵黄样物质、存在视网膜劈裂,左眼黄斑裂孔及局部视网膜脱离。结合病史、眼底表现与FFA、OCT、眼底自发荧光等诊断为隐性遗传性卵黄样营养不良。出现巨大黄斑裂孔及视网膜脱离者罕见。（眼科,2023,32:66-68）     

特发性眼底血管样条纹的临床特征

目的分析特发性眼底血管样条纹的临床特征。方法分析13例（26眼）特发性眼底血管样条纹患者的临床表现、荧光素眼底血管造影（FFA）及黄斑区光学相干断层扫描（OCT）表现。结果11眼视力≤0.3,占42.31%。26眼眼底后极部均可见类似血管样的放射状条纹,5眼为斑驳状外观,占19.23%;17眼条纹通过黄斑,占65.38%;1眼（3.8%）合并眼外伤致多发性脉络膜破裂出血。FFA显示26眼血管样条纹均表现为透见荧光,11眼黄斑区可见脉络膜新生血管（CNV）影,其亮度逐渐增强,后期有明显的荧光素渗漏。黄斑区OCT检查发现11眼有CNV表现。Ⅰ型CNV（生长于RPE光带下）：表现为在隆起的视网膜色素上皮（RPE）和脉络膜毛细血管层光带下有不均匀的中或高反射带;混合型CNV：表现为CNV侵入RPE光带及视网膜神经上皮层下空间,RPE光带中断,呈现边界不清的高反射组织。结论眼底、FFA及OCT典型表现的综合分析有助于特发性眼底血管样条纹的诊断、分期及治疗指导。     

视网膜血管瘤样增生性病变的眼底光学影像学特征观察及意义

目的观察视网膜血管瘤样增生性病变（RAP）在眼底彩色照相、眼底荧光血管造影（FFA）、吲哚青绿血管造影（ICGA）及光相干断层扫描（OCT）的图像特征改变及意义。方法回顾性分析2008年1月至2014年3月在河北省眼科医院就诊的11例14只眼RAP患者的临床资料,重点观察患眼病变部位在眼底彩色照相、FFA、ICGA及OCT图像中的改变特征。结果I期RAP3只眼,眼底表现为黄斑中心凹旁有1个斑点状或结节状黄红色血管瘤样病灶分布,其在FFA及ICGA中均表现为对应的强荧光,且病灶两端有视网膜小动脉和小静脉相连,呈“发夹”状外观。OCT表现为病灶对应处视网膜内有斑点状或囊状强反光物质分布。Ⅱ期RAP6只眼,眼底检查显示黄斑区视网膜水肿增厚,并有较多出血及硬性渗出分布。FFA及ICGA均显示黄斑区有1—2个斑片状或结节状强荧光灶出现,并有多支视网膜小血管与其吻合。OCT检查显示病灶对应部位神经上皮内或神经上皮下有团块状强反光物质分布,伴有神经上皮层囊样水肿增厚、局限性脱离以及严重的PED。BI期RAP5只眼,眼底表现为黄斑区中央视网膜深层有黄红色盘状或片状新生血管样病灶分布。FFA及ICGA检查显示病灶呈强荧光改变,并均能观察到多支视网膜血管与其吻合。OCT显示病灶对应处神经上皮下有团块状强反光物质分布,并与向上隆起的Bruch膜与脉络膜复合体相连。结论各期RAP病变在各种光学影像学检查中有特征性改变,熟练掌握这些改变特征将有助于临床医师对其做出正确的诊断和分期,并指导治疗。     

曲安奈德联合激光光凝治疗视网膜静脉阻塞继发黄斑水肿

目的：观察玻璃体腔内曲安奈德( trialcinolone acetonide, TA)注射联合黄斑格栅样光凝治疗视网膜静脉阻塞引起黄斑水肿的疗效分析。
　　方法：检眼镜、眼底血管造影( FFA )、光学相干断层扫描( OCT)检查证实的由视网膜静脉阻塞引起的黄斑水肿患者35例35眼,玻璃体内注射TA 2lg,1~4wk内黄斑水肿减轻后行黄斑格栅样光凝,随访6lo,观察视力、眼压、晶状体,OCT 观察黄斑厚度改变,FFA 观察眼底毛细血管渗漏情况。
　　结果：视网膜中央静脉阻塞( central retinal vein occlusion, CRVO)中非缺血型10眼,缺血型14眼;视网膜分支静脉阻塞( branch retinal vein occlusion, BRVO)中非缺血型4眼,缺血型7眼。最终视力提高者19眼,不变者11眼,比术前降低者5眼。 OCT检查黄斑中心凹形态恢复正常者9眼,FFA提示黄斑区荧光素渗漏与术前相比消失或明显减轻;黄斑囊样水肿明显改善者21眼,FFA 提示渗漏比术前减轻;无改善者5眼,FFA 提示黄斑区渗漏比术前加重或不变。
　　结论：玻璃体腔内注射TA 2 lg联合黄斑格栅样光凝治疗视网膜静脉阻塞继发黄斑水肿,可以明显减轻由静脉阻塞引起的黄斑水肿,并提高患者视力,是一种有效可行的方法。     

糖尿病视网膜病变黄斑水肿的检查比较

目的运用裂隙灯前置镜、眼底荧光血管造影(FFA)及光学相干断层扫描术(OCT)对正常人及糖尿病视网膜病变黄斑水肿(DME)进行检测,客观定量分析糖尿病性黄斑水肿,以期早期发现糖尿病性黄斑水肿。方法正常对照组20例(40只眼)。回顾性分析我院2003年5月~2005年10月间非增生性糖尿病患者76例(114只眼),分别行裂隙灯前置镜、FFA和OCT检查。裂隙灯前置镜、FFA检查按常规进行,OCT测量以黄斑中心凹为中心的6mm直径区域内视网膜形态及厚度,对各种检查记录进行比较。结果所有眼均行裂隙灯前置镜检查,肯定有DME者75只眼(65%),怀疑有者9只眼(8%),肯定无者30只眼(18%)。FFA确诊有DME者89只眼(78%),未发现DME者25只眼(22%),其中黄斑局限性水肿占18%,弥漫性水肿占32%,弥漫性水肿伴囊样变性占28%。用OCT检查正常对照组黄斑中心凹6mm直径区域内视网膜平均厚度为(157±10)μm,在糖尿病组同样区域内无黄斑区视网膜增厚者10只眼(9%),有黄斑区视网膜增厚者104只眼(90%),局限性水肿组视网膜平均厚度为(257±43)μm,弥漫性水肿伴囊样变性组视网膜平均厚度为(379±122)μm。经比较,OCT检查的敏感性大大高于裂隙灯前置镜检查,FFA检查无DME组中有15只眼经OCT检查与正常对照组间比较,有显著性差异(P<0.05),另外局限视网膜病变荧光渗漏组、弥漫性荧光渗漏伴囊变组间两两比较也均有显著性差异(P<0.05)。结论对于糖尿病视网膜病变黄斑区水肿,OCT检查其敏感性大大高于裂隙灯前置镜检查,并随着病程的延长,黄斑水肿加重,视网膜厚度及形态改变明显。OCT与FFA检查相互配合,才能较准确评估DME的发生及发展。     

荧光素眼底血管造影在眼挫伤诊断中的应用

目的分析眼挫伤后荧光素眼底血管造影（FFA）的临床表现。方法对68例（78眼）眼球挫伤行眼底血管荧光造影检查。结果视网膜震荡43眼中33眼FFA表现为视网膜动脉静脉稍迂曲、黄斑区点状透见荧光。10眼FFA表现为低荧光，无荧光渗漏。视网膜出血8眼FFA表现出血区荧光遮蔽。脉络膜裂伤9眼FFA表现为血管下方弧形弱荧光、造影晚期呈高荧光。黄斑孔6眼：其中板层孔4眼，FFA未见异常荧光；全层孔2眼FFA显示为黄斑区圆形透见荧光。视神经挫伤12眼FFA表现视盘毛细血管扩张及渗漏，晚期呈强荧光；其中1眼视盘灌注时间晚于视网膜中央动脉灌注。结论眼底血管荧光造影能及时准确地了解眼球挫伤后损伤部位和程度，为指导临床诊疗提供依据，应作为眼球挫伤的常规检查。     

视网膜中央静脉阻塞黄斑损害的两种影像检查的观察

目的探讨视网膜中央静脉阻塞(centralretinalveinocclusion,CRVO)所致黄斑损害的光学相干断层成像(opticalcoherencetomography,OCT)的图像特征与眼底荧光血管造影(fundusfluoresceinangiography,FFA)的关系。设计回顾性研究。研究对象33例33眼的视网膜中央静脉阻塞患者。方法对33例33眼的视网膜中央静脉阻塞患者进行OCT和FFA检查。采用OCT对黄斑水肿的高度进行了测量。结果4眼OCT表现为弥漫黄斑水肿,其FFA表现为黄斑拱环毛细血管轻度扩张渗漏;21眼OCT表现为黄斑囊样水肿,其FFA表现为13眼为典型的花瓣样黄斑囊样水肿,另8眼毛细血管扩张,拱环结构破坏,荧光素渗漏显著;3眼OCT表现为神经上皮脱离伴有黄斑水肿,其FFA表现为毛细血管扩张,荧光素渗漏;4眼OCT表现为视网膜内和/或视网膜下大量出血,其FFA检查表现为黄斑拱环破坏,结构不清,有出血遮挡;1眼OCT黄斑中心凹形态大致正常,其FFA检查表现为黄斑区荧光素轻度渗漏。结论OCT对于黄斑组织结构改变的敏感性优于FFA,并且能精确地分析和追踪观察黄斑水肿的病程演变。而FFA在OCT表现正常时,仍能反映出色素上皮屏障的破坏。因此,OCT和FFA两种检查各有优缺点,同时采用可更加全面地了解CRVO所致黄斑损害情况。     

卵黄样黄斑营养不良一例

男性,10岁。偶然发现双眼视力不佳于2013年12月就诊。眼部检查：视力：右眼矫正0.5（+1.00DS+0.75DC×100）;左眼矫正0.6（+0.50DS+1.00DC×80）;双眼前节正常。眼底检查（图1A、B）：右眼黄斑区见一约1DD边界清晰的黄色病灶呈“煎鸡蛋样”改变,中心凹光反射消失,周边眼底未见异常;左眼黄斑区见一约2DD边界清晰的椭圆形黄色囊样病灶呈“鸡蛋黄样”改变,余眼底未见异常。荧光素眼底血管造影检查（图1C、D）：右眼动脉期黄斑区可见斑驳状强荧光,晚期斑驳状强荧光减弱,其他部位造影无异常;左眼静脉期黄斑区可见椭圆形遮蔽荧光,晚期无改变。眼底自发荧光检查（图1E、F）：右眼与病灶相应处可见自发荧光增强,期间可见斑驳状自发荧光减弱;左眼病灶处自发荧光显著增强。相干光视网膜断层扫描（OCT）（图1G、H）：右眼黄斑区可见局限性神经上皮浅脱离,其下可见浆液性色素上皮脱离;左眼黄斑区可见局限性浆液性色素上皮脱离,部分遮挡其下网膜反射。视网膜电流图（ERG）检查：双眼a、b波幅值及峰潜时无异常。眼电图（EOG）检查,双眼光峰电位/暗谷电位（Arden比）降低。诊断为：双眼卵黄样黄斑营养不良（右眼卵黄破碎期,左眼卵黄病变期）;双眼屈光不正。     

Morphological and functional analyses of adult onset vitelliform macular dystrophy

AIM: To evaluate the morphology and visual function of the macula in eyes with adult onset vitelliform macular dystrophy (AVMD). METHODS: 12 eyes of six patients with AVMD were examined by ophthalmoscopy, scanning laser ophthalmoscopy (SLO), optical coherence tomography (OCT), and multifocal electroretinography (mfERGs). The macular lesions were bilateral in all patients and varied from the typical vitelliform (five eyes), faded vitelliform changes with retinal pigment epithelium (RPE) atrophy (five eyes), and a normal fovea associated with small flecks around the macula (two eyes). RESULTS: SLO demonstrated small abnormal bright spots in the deep retina throughout the posterior retina in all cases. OCT showed a highly reflective fusiform thickened layer at the level of the RPE and choriocapillaris in patients with a submacular yellow vitelliform lesion. A well circumscribed, optically clear space was observed beneath the retinal layer in the macular lesions with RPE atrophy. The mfERGs were significantly reduced not only in the macular area but also in the outermost ring (20-30 degrees ) of the mfERGs. CONCLUSIONS: The submacular materials that accumulate within the RPE or subepithelial layers reported in previous histopathological studies of vitelliform lesions can be detected by OCT. In the macular lesions with RPE atrophy, the material may have disappeared leaving a subretinal or subepithelial optical clear space. These SLO and mfERG observations suggest that the morphological and functional abnormalities may not be localised just in the macular area but may be present throughout the posterior pole in eyes with AVMD.     

Optical coherence tomography in diagnosing adult-onset vitelliform macular dystrophy

PURPOSE: To assess usefulness of optical coherence tomography (OCT) in adult-onset vitelliform macular dystrophy (AVMD) diagnosis. To assess retinal pathological changes during all stages of the disease. MATERIAL AND METHODS: Retrospective case-series report. 16 patients (9 men, 7 women, mean age 60.25), with vitelliform macular dystrophy, who underwent ophthalmic examination including optical coherence tomography (OCT Stratus III, Zeiss Meditec, CA, USA) in 2005 and 2006. Retinal Thickness Map and Fast Retinal Thickness Map Acquisition Protocols were used during OCT scanning. Patients were evaluated with ETDRS charts, biomicroscopy, fluorescein angiography and electrophysiological exams. RESULTS: 7 patients (44%) were referred to our outpatient clinic with diagnosis of macular hole, 7 patients (44%) with diagnosis of AMD. In 2 patients Best disease was suspected. Diagnosis of AVMD was possible to establish in all patients including medical history, fundus photography and OCT. CONCLUSIONS: OCT enables retinal morphology assessment and can be treated as the basis of adult-onset vitelliform macular dystrophy early evaluation, diagnosis differentiation and monitoring progression.     

眼底血管样条纹并发脉络膜新生血管的临床特征及联合治疗

目的 分析眼底血管样条纹（AS）并发CNV的临床特征,探讨光动力疗法（PDT）联合玻璃体腔注射雷珠单抗治疗AS合并黄斑病变的临床疗效及安全性。方法 回顾性系列病例研究。分析21例（42眼）AS的临床资料,包括BCVA、眼底表现、FFA、ICGA以及OCT。其中18例（22眼）合并黄斑CNV,先采用PDT治疗,3 d内玻璃体腔注射雷珠单抗,治疗后定期随访,至少随访12个月。随访时如发现视力下降、黄斑区出现新病灶、视网膜下或层间积液、CNV活动性病变,则重复玻璃体腔注射。数据采用独立样本t检验或配对样本t检验进行分析。结果 本组21例患者均双眼发病,仅5例（24%）合并全身病变,男性为主（76%）,其中18例（86%）继发黄斑CNV,BCVA显著低于病变未侵及黄斑者。联合治疗的22眼末次随访时BCVA较治疗前提高10.4个字母;OCT示治疗后黄斑区视网膜厚度从基线的（338.4±55.2）μm降至（212.6±36.2）μm;FFA（ICGA）显示15眼（68%）CNV完全闭合,渗漏消失,5眼呈瘢痕染色。所有患者接受1次PDT,平均玻璃体腔注射次数3.2次。1例PDT后出现黄斑区视网膜下出血,行玻璃体腔注射雷珠单抗后出血吸收,5例发生一过性眼压升高,4例出现结膜下出血,均完全恢复,无其他明显眼部及全身不良反应。结论 眼底AS具有特殊的眼底表现,FFA（ICGA）有助于明确诊断,相当比例的患者可继发黄斑部CNV。PDT联合玻璃体腔注射雷珠单抗能有效控制AS合并黄斑病变的病情进展,显著改善患者视功能,减少CNV渗漏,且不良反应少。     

Adult vitelliform macular degeneration: a clinicopathological study

AIMS/BACKGROUND: The yellow lesions of adult vitelliform macular degeneration (AVMD) slowly fade, progressing to hyperpigmentation or atrophy. This study aims to provide further observations on the location and nature of the vitelliform material. METHODS: This report describes the clinicopathological correlation of four eyes with AVMD. A retrospective histopathological study of a further 526 aged eyes previously graded for the stage of age-related macular degeneration (AMD) found another 10 eyes with similar pathology. RESULTS: The predominant finding was a collection of extracellular material beneath the sensory retina at the fovea. This material was derived internally from photoreceptor outer segments and externally from the retinal pigment epithelium (RPE), the latter first undergoing hypertrophy and then disruption and attenuation. Fallout of foveal cones occurred over these lesions and the inner retina was thinned, which may explain macular hole formation in this condition. All affected eyes showed histopathological evidence of AMD. CONCLUSIONS: This study confirms that the vitelliform lesions of AVMD lie beneath the sensory retina. In contrast to previous reports, however, it is proposed that the lesions comprise mainly extracellular material consisting of photoreceptor debris, possibly the result of faulty phagocytosis by the RPE, mixed with pigment liberated as the RPE undergoes disruption. The vitelliform lesions therefore are a marker for the area of maximal RPE disturbance.     

相干光断层扫描与荧光素眼底血管造影在中心性浆液性脉络膜视网膜病变诊断中的对比观察

目的观察对比相干光断层扫描（OCT）和荧光素眼底血管造影（FFA）在中心性浆液性脉络膜视网膜病变（CSC）诊断中的特点。设计回顾性病例系列。研究对象临床诊断为CSC的58例（62眼）患者。方法对临床诊断为CSC且均行FFA及OCT检查的58例（62眼）患者的临床资料进行对比观察,分析其影像特征。随访中每月行OCT检查,每3个月行FFA检查,对比两种影像学检查观察疾病演变过程的特点。主要指标FFA及OCT影像学表现。结果58例（62眼）患者OCT检查中,60眼（96．8％）有神经上皮脱离,其中55眼（88．7％）FFA表现为荧光渗漏,且均在OCT神经上皮脱离范围内,另5眼未见异常荧光;5眼（8．1％）伴色素上皮脱离,而FFA未见特殊表现;2眼（3．2％）OCT仅见色素上皮脱离,其中1眼FFA见黄斑鼻侧透见荧光,另1眼无异常荧光。治疗后3个月,OCT检查62眼中16眼（24．2％）见神经上皮脱离,16眼中FFA仅5眼（8．1％）有荧光渗漏;2眼伴色素上皮脱离,而FFA未有特殊表现。对FFA检查及随诊中发现渗漏点在中心凹500μm外者（57眼）行激光光凝治疗。结论OCT和FFA在对CSC的诊断上各具优势,OCT可观察到视网膜病变的形态改变,FFA可定位血管渗漏,利于激光治疗。两者结合是CSC最有效的影像诊断技术。     

儿童遗传性视网膜疾病的眼底自身荧光表现

目的 观察儿童遗传性视网膜疾病的眼底自身荧光(FAF)特征.方法 回顾性分析22例临床资料完整、年龄5～14岁之问的遗传性视网膜疾病患儿的FAF检查结果.其中,Best卵黄样病变8例16只眼,Stargardt病3例6只眼,视锥细胞营养不良3例6只眼,原发性视网膜色素变性(RP)5例10只眼,X连锁青少年型视网膜劈裂症3例6只眼.仔细询问现病史及家族史,行视力、裂隙灯显微镜眼前节检查,间接眼底镜检查,彩色眼底像和FAF照相,其中部分患儿接受了荧光素眼底血管造影(FFA)、视网膜电流图、眼电图、光相干断层扫描检查.对上述患儿的FAF结果特征进行归纳总结,并与其眼底照相和/或FFA结果进行比较分析.结果 3例Stargardt病患儿的6只眼及3例视锥细胞营养不良患儿的6只眼FAF检查显示黄斑区可见对称性的圆形、近圆形弱荧光或荧光缺如区,2例视锥细胞营养不良患儿的4只眼及1例Stargardt病患儿的2只眼可见弱荧光或荧光缺如区外缘环以强荧光;Best卵黄样病变患儿黄斑区可见一个强度均匀或不均匀的强FAF病灶;RP患儿后极部视网膜FAF增强,黄斑区周围可见宽度不等的环形强荧光带,拱环区FAF正常;3例X连锁青少年型视网膜劈裂症患儿中5只眼中心凹部位FAF检查可见蜂窝或颗粒状强荧光.结论 Stargardt病及视锥细胞营养不良患儿黄斑区为近圆形弱荧光,部分病变区外缘环以强荧光;Best卵黄样病变患儿黄斑区为强度均匀或不均匀的强FAF病灶;RP患儿后极部视网膜FAF增强,拱环区FAF正常,黄斑区周围可见宽度不等的环形强荧光带;X连锁青少年型视网膜劈裂症患儿中心凹部位为蜂窝或颗粒状强荧光.

Abstract：

Objective To observe the autofluorescence (AF) manifestation in children with hereditary retinal diseases. Methods The clinical data of 22 children (aged from 5 to 14 years) with hereditary retinal diseases were retrospectively analyzed. There were 8 children (16 eyes) with Best vitelliform macular dystrophy, 3 children (6 eyes) with Stargardt macular dystrophy, 3 children (6 eyes) with macular cone dystrophy, 5 children (10 eyes) with primary retinitis pigmentosa, and 3 children (6 eyes) with X-linked juvenile retinoschisis. The routine clinical examinations included present history, family history, visual acuity, silt-lamp microscopy, indirect ophthalmoscopy, color fundus photography and fundus autofluorescence angiography (FAF). Some patients received fundus fluorescein angiography (FFA),electroretinogram (ERG), electrooculogram (EOG), and ocular coherence tomography (OCT). The characteristics of AF in all the children were analyzed, and were compared with the images of color fundus and/or FFA. Results Symmetry round macular fluorescent weak or absent area was found in all Stargardt disease and cone dystrophy. Weak AF area with surrounded circular increased AF was found in 2 children (4 eyes) with cone dystrophy and 1 child (2 eyes) with Stargardt macular dystrophy. A central round area with regular or irregular intense AF was observed in Best vitelliform macular dystrophy. RP children showed increased AF out of the macular region. Cellular or granular strong AF was found in the fovea of 3 children (5 eyes) with X-linked juvenile retinoschisis. Conclusion The children with hereditary retinal diseases had special AF changes.     

放射状视神经切开术治疗缺血型视网膜中央静脉阻塞伴黄斑水肿的疗效评估

目的评估放射状视神经切开术（RON）治疗缺血型视网膜中央静脉阻塞（CRVO）伴黄斑水肿的效果。方法回顾性分析2005～2007年行RON治疗的18例缺血型CRVO伴明显黄斑水肿患者的临床资料,其中15例术前确诊为缺血型CRVO,3例玻璃体积血手术后确诊为缺血型CRVO。手术前后均进行视力、荧光素眼底血管造影（FFA）、光相干断层扫描（OCT）检查。术后随访1周,1、3、6个月,比较患者手术前后的检查结果。所有患者在应用药物或手术前均签署知情同意书。结果除3例病程太长或黄斑变性视力未恢复,其余15例视力在短期内均有不同程度的提高,视力恢复〈3个月组好于〉3个月组（p=0．025）。眼底照相、OCT及FFA检查结果显示黄斑水肿均在1个月内明显消退,眼底出血3个月内吸收。1例术中穿刺视盘时出现鼻侧视网膜下局限性出血。结论RON能够改善缺血型CRVO合并的黄斑水肿。早期手术干预视力预后较好。术中联合应用的黄斑内界膜剥离术所起的作用尚需进一步评估。     

病理性近视黄斑出血的眼底荧光素血管造影、吲哚青绿血管造影及相干光断层扫描的影像学改变分析

目的分析病理性近视（PM）黄斑出血的荧光素眼底血管造影（FFA）、吲哚青绿血管造影（ICGA）及相干光断层扫描（OCT）的影像学特征及临床意义。方法对病理性近视黄斑出血28例（31只眼）进行FFA、ICGA及OCT检查,并对比分析其检查图像。结果单纯型出血15只眼（48.4%）,FFA表现：出血部位遮蔽荧光,其中8只眼（53.5%）可见漆样裂纹透见荧光;ICGA表现：造影期间出血部位为淡的低荧光,晚期均可见漆样裂纹性条状低荧光;OCT表现：9只眼（60%）在神经上皮层下和4只眼（26.7%）在色素上皮（RPE）下可见中高反射光团,周围神经上皮无水肿增厚,其下脉络膜光带连续,其中2只眼（13.3%）OCT无明显改变;新生血管（CNV）型出血16只眼（51.6%）,FFA表现：14只眼（87.5%）为典型性CNV,2只眼（12.5%）为隐匿性CNV;ICGA表现：14只眼（87.5%）为焦点状CNV,无明显染料渗漏,2只眼（12.5%）未见CNV改变;OCT表现：14只眼（87.5%）为RPE光带断裂,RPE上梭形或类圆形中、高反射光团,2只眼（12.5%）为RPE层断裂伴不规则增厚中高反射信号,神经上皮层均增厚水肿。结论病理性近视黄斑出血分为单纯型和新生血管型,新生血管多表现为典型CNV,不同类型的出血其FFA、ICGA、OCT的改变各具特征,合理选择检查方法可提高诊断率,节省费用。     

Exudative age-related macular degeneration or Best vitelliform macular dystrophy?--A case report

PURPOSE: To report a case of Best vitelliform macular dystrophy referred to the Department Ophthalmology in Krakow with a diagnosis of exudative age-related macular degeneration (AMD). MATERIALS AND METHODS: 70-years old man was diagnosed in our clinic because of a two years history of slow, progressive visual acuity worsening in both eyes with the presence of metamorphopsia. The basic ophthalmic examination was performed with additional diagnostic methods including: colour vision test (Panel D-15), Amsler grid test, contrast sensitivity test (Pelli-Robson chart), fluorescein angiography (FA), indocyanine green angiography (ICGA), electroretinogram (ERG), electrooculogram (EOG) and optical coherence tomography (OCT). RESULTS: Visual acuity in the right eye was: 0.16 and in the left: 0.25. Amsler grid test revealed the presence of bilateral mild etamorphopsia with the relative central scotoma. Pelli-Robson test showed decreased contrast sensitivity perception in both eyes; PO > LO. On fundoscopy in macula of both eyes the symmetrical round, elevated lesions of 1.5 dd with the meniscus of subretinal creamy-yellow masses were present. The early frames of FA showed the presence of round lesions with distinct borders, unchanged in size and shape throught the examination, hypofluorescent in the lower and hyperfluorescent in the upper half of the lesions. Late frames of FA revealed the irregular hyperfluorescence also in lower aspects of the lesions. ICGA showed: round hypofluorescent lesions with isofluorescence in the upper part of the lesions. ERG--revealed no pathology, EOG--showed decreased light response and depressed Arden ratio in both eyes. OCT demonstrated hiperreflectivity of the retinal pigment epithelium with elevation of retina and deletion of the foveolar depression in both eyes. CONCLUSIONS: Based on the results of performed tests the diagnosis of the Best vitelliform macular dystrophy was established. In some cases various pathologies involving the macula may mimic the exudative AMD. The basic ophthalmic examination supported by additional diagnostic methods allow to establish the definitive diagnosis in most cases of macular disorders.