Vogt-小柳-原田综合征葡萄膜炎期荧光素钠及吲哚菁绿同步眼底血管造影特征分析

目的探讨Vogt-小柳-原田综合征(Vogt-Koyanagi-Harada disease,VKH)葡萄膜炎期荧光素钠及吲哚菁绿同步眼底血管造影的影像学特征及其临床意义,为诊断及病程监测提供依据。方法回顾性分析11例22眼VKH葡萄膜炎期患者应用海德堡HRA2共焦激光扫描眼底血管造影系统进行的荧光素钠眼底血管造影(fundus fluorescein angiography,FFA)和吲哚菁绿血管造影(indocyanine green angiography,ICGA)同步检查资料,比较2种检查的影像学特征。结果 11例22眼中,FFA检查8眼(36.36%)后极部多处脉络膜充盈迟缓;9眼(40.91%)视盘渗漏水肿;22眼(100.00%)后极部视网膜色素上皮密集针尖状高荧光渗漏,18眼(81.82%)后极部神经上皮脱离部位FFA晚期形成多囊样或多湖状荧光积存;13眼(59.09%)静脉期散在、均匀脉络膜炎性病灶,呈斑点状低荧光。ICGA检查15眼(68.18%)脉络膜充盈迟缓;14眼(63.64%)脉络膜血管扩张;16眼(72.73%)播散状分布、斑点状脉络膜炎性低荧光病灶;18眼(81.82%)后极部神经上皮脱离区大多呈现持续低荧光,晚期部分囊样病灶呈中强荧光;9眼(40.91%)视盘水肿晚期显示视盘及盘周低荧光。FFA和ICGA同步检查结果:FFA检查100.00%患眼静脉期视盘周及后极部密集点状高荧光渗漏,而在ICGA上无此征象;FFA检查视盘表面毛细血管扩张比ICGA显示更明显;ICGA检查脉络膜充盈迟缓15眼(68.18%)及斑点状炎性病灶16眼(72.73%),均高于FFA检查结果的8眼(36.36%)和13眼(59.09%),而且ICGA早期脉络膜血管扩张显示更清晰;后极部神经上皮脱离病灶在FFA静脉期表现为低荧光,晚期形成荧光积存,而病变区域ICGA检查相应部位多为持续低荧光。结论 VKH葡萄膜炎期FFA清晰显示视网膜色素上皮损伤特征以及炎症累及视盘、视网膜的程度和范围;ICGA能够清晰显示脉络膜循环改变及脉络膜炎症的活动性和范围。FFA和ICGA同步检查对VKH诊断及发病机制有重要的指导意义。     

伏格特小柳原田综合征的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(indocyanine green angiography,ICGA)检查作为伏格特小柳原田(Vogt-Koyanagi-Harada,VKH)综合征临床观察指标的意义。 方法 对26例VKH患者荧光素眼底血管造影(fundus fluorescein angiography,FFA)和ICGA检查资料进行分析。 结果 FFA表现为色素上皮水平多数针尖状渗漏,快速形成多湖状荧光积存。治疗后上述改变很快消失。葡萄膜炎期ICGA表现：①66.7%的患眼为多数灶状弱荧光区域和脉络膜大、中血管荧光影像减少;②70.8%的患眼脉络膜血管扩张;③79.2%的患眼为造影后期显示灶状强荧光区。在病变恢复期,与FFA检查比较,ICGA的异常发现恢复缓慢。 结论 ICGA 可较好提供VKH的脉络膜循环损害的信息,并在评价疗效上有意义。(中华眼底病杂志,2000,16:9-11)     

Vogt-小柳-原田病的临床特点和治疗分析

目的探讨Vogt-小柳-原田病(Vogt-Koyanagi-Harada,VKH)的临床特点、治疗和预后等问题。方法连续收集近两年间在我院就诊的VKH患者45例,对其眼部表现、全身症状、荧光素眼底血管造影(fundus fluo-rescein angiography,FFA)和治疗进行回顾性分析。结果45例患者均为双眼发病,男女比例是1:1.4,平均年龄39.3岁。发病前有前驱症状者37例(82.2%),主要为头痛。就诊时有眼外症状和体征者39例(86.7%),包括中枢神经系统异常、听力障碍、白发脱发和白癜风。发病后2周内就诊者11例,表现为双眼脉络膜炎、视盘及视网膜水肿;发病后2周至3个月内就诊者24例,主要表现为非肉芽肿性前葡萄膜炎、后葡萄膜炎和浆液性视网膜脱离;复发患者(病程3个月以上)10例,主要表现为肉芽肿性前葡萄膜炎、晚霞状眼底改变和Dalen-Fuchs结节。FFA检查表现为斑驳状高荧光、视盘染色和多湖样染料积存。所有患者给予大剂量糖皮质激素治疗,疗程为3～18个月。初发组94.3%患者葡萄膜炎完全控制,视力显著提高。10例复发者中炎症完全控制7例(70%),视力有不同程度提高。结论前驱症状、临床病程、眼外表现及FFA等有助于VKH的诊断,早期给予大剂量糖皮质激素治疗,绝大部分患者可控制炎症,改善视力。     

Behcet病和Vogt- Koyanagi-Harada综合征的吲哚青绿及荧光素眼底血管造影分析

目的 探讨Behcet病和Vogt-Koyanagi-Harada（VKH）综合征的脉络膜视网膜病变特征。方法 对17例（27只眼）具有活动性葡萄膜炎的Behcet病和18例（36只眼）VKH综合征患者行吲哚青绿血管造影术（indocyanine green angiography,ICGA)和荧光素眼底血管造影术（fundus fluorescein angiography,FFA)检查，对其脉络膜视网膜进行观察。结果 Behcet病患者中，FFA检查显示均有视网膜微血管渗漏；ICGA检查发现11只眼（40．7％）有脉络膜血管扩张，通透性增强。VKH综合征患者中，FFA后期检查可见视网膜微血管呈多湖状染料积存、视盘染色或弥散性视网膜色素上皮（retinal pigment epithelium,RPE)损害；ICGA检查，发现36只眼（100．0％）脉络膜血管扩张、通透性增强，其中多灶性弱荧光斑32只眼（88．9％），脉络膜灌注不良28只眼（77．8％）。结论 Behcet病是以视网膜血管炎为主的炎性病变，但脉络膜亦可受累；VKH综合征主要累及脉络膜和RPE，ICGA主要表现为脉络膜血管扩张和多灶性弱荧光。FFA与ICGA结合能全面了解Behcet病和VKH综合征的脉络膜视网膜病变特征和损害形式。     

Vogt-小柳-原田综合征的眼底荧光血管造影分析

目的：探讨Vogt-小柳-原田综合征(Vogt-Koyanagi-Harada syndrome,VKHS)的眼底荧光血管造影(fundus fluorescein angiography,FFA)的表现及其临床意义。

方法：对VKHS患者16例30眼进行眼部详细检查,并行眼底彩色照相和FFA等检查。

结果：Vogt-小柳-原田综合征葡萄膜炎期的眼底表现为3种类型,脉络膜炎性渗出及渗出性视网膜脱离是视力损害的主要原因,而视盘水肿充血对视力损害较轻,且恢复较好。

结论：FFA的特征性表现对该病早期具有重要诊断意义。     

多灶性脉络膜炎的吲哚青绿眼底血管造影观察

目的 探讨利用吲哚青绿眼底血管造影(indocyanine green,ICGA)检查作为多灶性脉络膜炎临床观察指标的意义.方法 分别对29例(45眼)多灶性脉络膜炎患者荧光素眼底血管造影(fundus fluorescein angiography,FFA)和ICGA检查资料进行分析,根据其造影特点分为临床前期、活动期、瘢痕期.结果 临床前期10例(17眼)患者FFA表现正常,而ICGA造影后期出现了大小不一的弱荧光斑;活动期13例(19眼)患者FFA表现造影早期弱荧光斑,造影后期强荧光斑,ICGA造影早期表现为弱荧光斑,其间可夹杂有强荧光斑,其弱荧光斑的数量多于FFA所见;瘢痕期6例(9眼)患者有2眼造影后期出现3～4个新弱荧光斑,其他7眼患者FFA表现强荧光但无渗漏,ICGA各期均表现为弱荧光斑,其病灶范围和个数与FFA相对应.结论 ICGA能较好地提供多灶性脉络膜炎脉络膜循环损害的信息,并可指导治疗及判断视力预后.     

脉络膜炎的吲哚青绿血管造影分析

目的 探讨脉络膜炎在吲哚青绿血管造影(indocyanine green angiography,ICGA)下的表现特征。 方法 对13例(16眼)脉络膜炎患者均作了ICGA和眼底荧光血管造影(funds fluorescein angiography,FFA)检查。 结果 ICGA可显示临床和FFA所发现不了的脉络膜炎性损害灶，其表现为：①脉络膜血管扩张，扩张的血管呈节段样外 观、边缘粗糙；②脉络膜血管通透性增高所致的斑片状强荧光；③脉络膜充盈缺损；④脉络膜炎性水肿所致的异常暗背景荧光；⑤涡静脉扩张。 结论 ICGA不仅能较好的评价脉络膜炎的血液循环受损程度,而且能较全面地观察到脉络膜炎所致的损害病灶。(中华眼底病杂志,1998,14:92-94)     

Vogt-小柳-原田综合征Dalen-Fuchs结节的荧光素和吲哚青绿眼底血管造影分析

目的：探讨Vogt-小柳原田综合征（Vogt-Koyanagi-Harada syndrome,VKHS)Dalen-Fuchs结节的眼底表现及其临床意义。方法：对36例患者作了眼底彩色照相及荧光素眼底血管造影（fundus fluorescein angiography FFA),其中6例同时作了吲哚青绿血管造影（indocyanine green angiography,ICGA)。结果：36例VKHS患者中,存在Dalen-Fuchs结节者16例（44．44％）,类圆形黄色结节位于下方中周部者最多（93％）,急性期的Dalen-Fuchs结节FFA后期结节染色,而ICGA呈边界清楚的弱荧光,恢复期的Dalen-Fuchs结节FFA和ICGA均显著结节下的脉络膜血管萎缩及RPE萎缩,色素增殖改变,ICGA显示的Dalen-Fuchs结节比眼底彩色照相和FFA要清晰。结论：Dalen-Fuchs结节是VKHS的一个重要临床特征表现,ICGA能更好显示这种结节样损害。     

特殊类型葡萄膜炎眼底荧光血管造影分析

目的 探讨特殊类型葡萄膜炎患者的眼底图像特征及荧光素眼底血管造影(fundusfluorescein angiography,FFA)的特征.方法 回顾分析山西医科大学第一医院2005年4月至2008年2月在眼科门诊行FFA检查并确诊为葡萄膜炎的患者56例98只眼的眼底和FFA图像特征及相关临床资料.结果 56例98只眼葡萄膜炎患者中,双眼发病的有42例84只眼,单眼发病的有14只眼.其中以Behcet病(Behcet disease)最多有25例48只眼;Vogt-小柳-原田病(VKH)有13例25只眼;急性视网膜坏死综合征(ARNS)有10例16只眼;系统性红斑狼疮(SLE)伴葡萄膜炎有8例9只眼.多数患者眼底主要表现为:视盘充血,边界不清,黄斑呈囊样水肿,静脉迂曲扩张,动脉变细,后极部网膜弥漫性水肿.FFA主要表现为:视盘荧光着染且在中后期可见荧光渗漏,黄斑囊样荧光积存,网膜血管轻到中度荧光染色伴渗漏,视网膜深层平面片状荧光渗漏.VKH患者的眼底在赤道前网膜下可见Dalen-Fuch结节.结论 不同疾病可引起不同类型的葡萄膜炎.通过FFA检查可确诊特殊类型的葡萄膜炎,对其治疗起到重要的指导意义.     

梅毒性后葡萄膜炎眼底表现及眼底血管造影特征

梅毒性后葡萄膜炎是一组累及脉络膜、视网膜及视网膜血管的炎症性病变.目前,临床上主要通过荧光素眼底血管造影(FFA)检查了解其病变情况,但由于视网膜色素上皮(RPE)的影响,使FFA对脉络膜炎症的观察受到限制[1].为探讨吲哚青绿血管造影(ICGA)检查在梅毒性后葡萄膜炎中的意义,我们对一组梅毒性后葡萄膜炎患者的眼底及FFA和ICGA检查特征进行了总结分析.现将结果报道如下.     

Vogt-小柳-原田综合征漏误诊分析

目的：分析我国Vogt-小柳原田（VKH）综合征临床诊断中存在的问题。方法：对1996年1月－2000年12月于中山眼科中心葡萄膜炎专科就诊的,以往资料完整的123例VKH综合征患者的临床资料。特别是漏诊,误诊,发病至确诊时间和以往诊断等进行分析。结果：123例VKH综合征患者的盲目发生率为28．9％,在后葡萄膜炎期,前葡萄膜受累期和以前葡萄膜炎反复发作为特征的全葡萄膜炎期的漏诊,误诊率分别为96．7％,86．2％和74．0％,眼部发病至确诊时间平均为24．5个月。结论：我国VKH综合征患者的漏诊和误诊十分常见,诊断水平亟待提高。     

Indocyanine green angiography in posterior uveitis

Literature review for indocyanine green angiography and evaluate the role of indocyanine green angiogram (ICGA) in patients with posterior uveitis seen at a tertiary referral eye care centre. Detailed review of the literature on ICGA was performed. Retrospective review of medical records of patients with posterior uveitis and dual fundus and ICGA was done after institutional board approval. Eighteen patients (26 eyes) had serpiginous choroiditis out of which 12 patients had active choroiditis and six patients had healed choroiditis, six patients (12 eyes) had ampiginous choroiditis, six patients (12 eyes) had acute multifocal posterior placoid pigment epitheliopathy, eight patients (10 eyes) had multifocal choroiditis, four patients (eight eyes) had presumed ocular histoplasmosis syndrome, four patients (eight eyes) had presumed tuberculous choroiditis, two patients (four eyes) had multiple evanescent white dot syndrome and two patients (four eyes) had Vogt Koyanagi Harada (VKH) syndrome. The most characteristic feature noted on ICGA was the presence of different patterns of hypofluorescent dark spots, which were present at different stages of the angiogram. ICGA provides the clinician with a powerful adjunctive tool in choroidal inflammatory disorders. It is not meant to replace already proven modalities such as the fluorescein angiography, but it can provide additional information that is useful in establishing a more definitive diagnosis in inflammatory chorioretinal diseases associated with multiple spots. It still needs to be determined if ICGA can prove to be a follow up parameter to evaluate disease progression.     

The Choroidal Vascularity Index Decreases and Choroidal Thickness Increases in Vogt–Koyanagi–Harada Disease Patients During a Recurrent Anterior Uveitis Attack

Purpose: To measure changes in the choroidal vascularity index (CVI) in chronic Vogt–Koyanagi–Harada (VKH) disease during a recurrent anterior uveitis attack.

Methods: Forty VKH patients and 40 normal controls were included in this study. Choroidal images were recorded before and during a recurrent anterior uveitis attack, as well as after appropriate treatment. CVI was measured by the binarization technique using ImageJ software (Bethesda, MD).

Results: The CVI was 0.75 ± 0.09 in quiescent VKH patients, which was significantly higher compared to healthy controls (0.70 ± 0.05, p ＜ 0.0001). The CVI significantly decreased to 0.72 ± 0.09 when granulomatous anterior uveitis appeared in these patients. However, it returned to 0.75 ± 0.08 after uveitis resolved.

Conclusions: A significant decrease of the CVI occurred during recurrent anterior uveitis in chronic VKH. CVI may provide a novel parameter to guide the treatment of VKH disease.     

Indocyanine green angiography findings in patients with long-standing Vogt-koyanagi-Harada disease: a cross-sectional study

ABSTRACT: BACKGROUND: To investigate indocyanine green angiography (ICGA) findings in patients with long-standing Vogt-Koyanagi-Harada (VKH) disease and their correlation with disease activity on clinical examination as well as with systemic corticosteroid therapy. METHODS: Twenty-eight patients (51 eyes) with long-standing ([GREATER-THAN OR EQUAL TO]6 months from disease onset) VKH disease whose treatment was tapered based only in clinical features were prospectively included at a single center in Brazil. All patients underwent standardized clinical evaluation, which included fundus photography, fluorescein angiography and ICGA. Clinical disease activity was determined based in the Standardization in Uveitis Nomenclature Working Group. Fisher exact test and logistic regression models were used for statistical analysis. RESULTS: Disease-related choroidal inflammation on ICGA was observed in 72.5% (31 of 51 eyes). Angiographic findings suggestive of (choroidal and/or retinal) disease activity were not observed on FA. Clinically active disease based on clinical evaluation was observed in 41.2% (21 of 51 eyes). In these 21 eyes, disease-related choroidal inflammation on ICGA was observed in 76.2% (16 of 21 eyes); in the remaining eyes (without clinical active disease) disease-related choroidal inflammation on ICGA was observed in 70.0% (21 of 30 eyes). In respect to systemic corticosteroid therapy, 10 patients (18 of 51 eyes) were under treatment with prednisone. In these 10 (18 of 51 eyes) patients, disease-related choroidal inflammation on ICGA was observed in 83.3% (15 of 18 eyes); in the remaining patients (33 of 51 eyes) disease-related choroidal inflammation on ICGA was observed in 66.7% (22 of 33 eyes). CONCLUSION: ICGA findings suggestive of disease-related choroidal inflammation were observed in a considerable proportion of patients with long-standing VKH disease, independent of the inflammatory status of the disease on clinical examination or current use of systemic corticosteroid. Therefore, the current study reinforces the crucial role of ICGA to assist the management and treatment of patients with long-standing VKH disease.     

多发性后极部色素上皮病变的吲哚青绿和荧光素眼底血管造影

目的 研究多发性后极部色素上皮病变(multifocal posterior pigment epitheliopathy,MPPE)的视网膜和脉络膜循环特点,探讨该病的发病机制及其与中心性浆液性脉络膜视网膜病变(central serouschorioretinopathy,CSC)之间的关系。 方法 对6例MPPE患者的7只眼吲哚青绿血管造影(indocyanine green angiography,ICGA)和荧光素眼底血管造影(fundus fluorescein angiography,FFA)检查结果进行对比分析。 结果 5例5只眼FFA检查为活动期,其中4只眼在ICGA造影早期有脉络膜充盈延迟,占80%。FFA显示有渗漏处,ICGA均有相应的渗漏灶。在渗漏灶的周围及后极部尚有弥漫性相对强荧光。病变恢复期,FFA已无明显渗漏,ICGA仍显示脉络膜荧光渗漏。 结论 MPPE与CSC有着相同的ICGA改变,但前者病变范围更加广泛。MPPE为CSC的较严重型,为原发于脉络膜病变的疾病。(中华眼底病杂志,2000,16：6-8)     

A Rare Case of Unilateral Progressive Vision Loss and Pachymeningitis

We describe a 32-year-old man with presumed Vogt-Koyanagi Harada (VKH) syndrome, whose presenting symptoms were headache and progressive loss of vision in the right eye. Neuro-ophthalmic examination showed anterior and posterior uveitis, and retinal detachment in the right eye. Ocular coherence tomography (OCT) showed extensive submacular fluid in the right eye, while the fundus fluorescein angiogram (FFA) confirmed perifoveal retinal pigment epithelium (RPE) disruption and multifocal fluorescein leakage in the right eye. The brain MRI showed a small crescent of dependent fluid layering in the right posterior globe adjacent to the right optic nerve head, and pachymeningeal enhancement of the skull base dura along the clivus.This case demonstrates the utility of brain MRI and OCT findings in the early diagnosis of VKH syndrome, in the absence of prominent clinical signs of meningitis. Aggressive treatment is critical to preserve vision and prevent development of other systemic complications of the disease.     

Vogt-小柳-原田综合征

Vogt-小柳-原田综合征（VKH综合征）在我国较常见。本文就其诊断及鉴别诊断的相关内容作一介绍。VKH综合征前驱期症状为发热、头痛、耳鸣、听力减退。急性期为双眼视力急性下降,后部视网膜多个盾形浅脱离与视盘充血、水肿。恢复期为晚霞样眼底与Dallen—Fuch结节（D—F结节）。慢性复发期主要表现为反复发作的肉芽肿性前葡萄膜炎。B超、超声生物显微镜、荧光素眼底血管造影、吲哚青绿血管造影与相干光断层扫描可协助诊断并了解治疗效果。