特发性视网膜血管炎、动脉瘤、视神经、视网膜炎综合征临床观察

目的 观察特发性视网膜血管炎、动脉瘤、视神经视网膜炎综合征（IRVAN综合征）的临床特征。 方法 回顾分析3例经全身系统检查、眼底彩色照相以及荧光素眼底血管造影（FFA）等检查确诊的IRVAN综合征患者的临床资料。 结果 3例患者均有特发性视网膜血管炎，而且是视网膜动脉的炎症，视盘及视网膜血管多发性大动脉瘤以及因视盘水肿、视盘周围渗出引起的视神经视网膜炎，2例患者周边部视网膜血管有闭塞区。 结论 IRVAN综合征的临床特征有特发性视网膜血管炎，视网膜和视盘动脉血管管壁上多发性大动脉瘤，以及由于血管炎症和动脉瘤引起的视网膜、视盘渗出、水肿以及由此产生的视神经视网膜炎。(中华眼底病杂志，2007，23：180-183)     

玻璃体内注射曲安奈德治疗继发于IRVAN的黄斑水肿

目的:报告玻璃体内注射曲安奈德对特发性视网膜炎、血管炎、动脉瘤及视神经视网膜炎(IRVAN)所致的黄斑水肿的治疗效果。方法:病例报告。结果:患者诊断为特发性视网膜炎、血管炎、动脉瘤及视神经视网膜炎导致的伴有视力丧失的继发性黄斑水肿。玻璃体内注射曲安奈德后黄斑水肿减轻,视力显著改善。结论:以前的报告建议采取全视网膜光凝,玻璃体切除术及全身和眼部类固醇治疗。对于继发于IRVAN的黄斑水肿和血管炎,玻璃体内注射曲安奈德是一种安全有效的治疗方法。     

玻璃体内注射曲安奈德治疗继发于IRVAN的黄斑水肿

目的：报告玻璃体内注射曲安奈德对特发性视网膜炎、血管炎、动脉瘤及视神经视网膜炎（IRVAN）所致的黄斑水虢曲治疴效栗,方法：病例报告。结果：患者诊断为特发性视网膜炎、血管炎、动脉瘤及视神经视网膜炎导致的伴有视力丧失的继发性黄斑水肿。玻璃体内注射曲安奈德后黄斑水肿减轻,视力显著改善。结论：以前的报告建议采取全视网膜光凝,玻璃体切除术及全身和眼部类固醇治疗。对于继发于IRVAN的黄斑水肿和血管炎,玻璃体内注射曲安奈德是一种安全有效的治疗方法。     

IRVAN综合征

特发性视网膜血管炎、动脉瘤和视神经视网膜炎综合征是一种病因不明的较为少见的视网膜血管炎性疾病。其炎症来源尚不明确,以典型的眼底表现为特征,患者视力严重受损,且预后不佳。视网膜激光光凝术对部分病例可能有效,但目前临床上尚无确切的治疗方法。本文对国内外各相关报道进行综述,为临床诊治提供一定的参考。     

IRVAN综合征的临床特征和治疗分析

目的 观察IRVAN综合征的临床特征,探讨其治疗时机的选择.方法 回顾分析5例(10只眼)确诊为IRVAN综合征的患者的资料,并根据其临床特征分为五期.对6只眼采用视网膜激光光凝治疗,4只眼联合应用玻璃体切割术与视网膜激光光凝术治疗,以观察疗效.结果 5例患者10只眼中均有特发性视网膜动脉炎、后极部多发动脉瘤、视神经视网膜炎,周边部有视网膜血管闭锁及大面积毛细血管无灌注区.4只眼出现玻璃体积血,2只眼有视盘新生血管,1只眼黄斑区出现毛细血管无灌注区.其中4只眼处于Ⅱ期,6只眼均处于Ⅲ期.经过治疗后,目前视力≥0.6者4只眼,0.1～0.6者1只眼,≤0.1者5只眼.结论 5例10只眼均具有IRVAN综合征的特点,符合其诊断标准.在Ⅱ期即周边部视网膜无灌注区形成新生血管之前或初期进行激光光凝治疗能稳定眼底病变的进展.     

特发性视网膜血管炎、动脉瘤、视神经视网膜炎综合征和Eales病荧光素眼底血管造影对比分析

目的 观察特发性视网膜血管炎、动脉瘤、视神经视网膜（IRVAN）综合征和Eales病荧光素眼底荧光血管造影（FFA）特征的异同。方法 回顾分析IRVAN综合征患者4例8只眼和Eales病患者43例68只眼的FFA检查资料。所有患者均行常规眼科检查,包括视力、眼压、裂隙灯显微镜、间接检眼镜眼底检查。4例Eales病患者单眼玻璃体积血眼底窥不清,行对侧眼检查,其他所有患者双眼常规彩色眼底照相、共焦激光扫描FFA检查。IRVAN综合征4例8只眼,均为双眼发病。男性1例, 女性3例;年龄16～43岁,平均年龄（27.00±12.93）岁。Eales病43例68只眼, 男性32例, 女性11例;年龄6～59岁,平均年龄（30.79±11.46）岁。双眼29例54只眼,单眼14例14只眼。两组患者眼底均可见视网膜血管白鞘或白线状改变、视网膜出血渗出、玻璃体积血。结果 FFA检查结果显示,IRVAN综合征8只眼后极部动、静脉均受累;均可见多发性视网膜大动脉瘤。周边部毛细血管闭塞形成无灌注区7只眼,占87.50%;视盘水肿荧光渗漏5只眼,占62.50%;视神经萎缩2只眼,25.00%;出血遮挡1只眼,占12.50%;视盘新生血管2只眼,占25.00%;视网膜新生血管4只眼,占50.00%;黄斑水肿4只眼,50.00%。Eales病43例68只眼中,所有患眼周边病变区视网膜静脉管壁渗漏;累及后极部静脉32只眼,占47.06%;动脉同时受累5只眼,占7.35%;周边部毛细血管闭塞形成无灌注区38只眼,占55.88%;视盘渗漏29只眼,占42.65%;视盘新生血管4只眼,占5.88%;视网膜新生血管26只眼,占38.24%;黄斑水肿15只眼,占22.06%。IRVAN综合征与Eales病患者在后极部动脉受累眼数、静脉受累眼数、动脉瘤眼数之间比较,差异有统计学意义(P均＜0.05);视盘渗漏、无灌注区、视盘和视网膜新生血管、黄斑水肿眼数之间比较,差异无统计学意义(χ2=0.479,1.449,0.068,1.676;P＞0.05)。结论 IRVAN综合征和Eales病均可发生视网膜动脉和静脉不同程度的炎性改变,并均可导致视盘水肿渗出。IRVAN综合征后极部视网膜动、静脉受累明显高于Eales病,特别是视盘旁及后极部特征性大动脉瘤样改变有助于IRVAN综合征的诊断及IRVAN综合征和Eales病的鉴别诊断。     

IRVAN综合征治疗一例

IRVAN（idiopathic retinal vasculitis,anenrysms,and neuroretinitis,IRVAN）是特发性视网膜血管多，动脉瘤，视神经视网膜炎的英文缩写词，属于罕见的眼部综合征，目前尚无确切的中文病名。     

IRVAN综合征的治疗及其疗效观察

目的：：观察分析不同方法治疗特发性视网膜血管炎、动脉瘤、视神经视网膜炎综合征( idiopathic retinal vasculitis, aneurysms and neuroretinitis,IRVAN)的效果。方法：收集经临床确诊的IRVAN综合征患者7例14眼。其中Ⅱ期4眼,Ⅲ期10眼;视力≥0.6者6眼,0.3者4眼,≤0.1者4眼。4眼行视网膜激光光凝无灌注区;Ⅲ期中4眼行全视网膜光凝( PRP ),2眼行雷珠单抗玻璃体腔注射联合PRP,4眼行玻璃体切割术联合PRP。随访6mo~10a,观察视力及眼底病变情况。结果：治疗后视力提高者6眼,视力维持不变者6眼,视力提高后又下降者2眼;其中≥0.6者9眼,0.3~0.6者4眼,≤0.1者1眼。Ⅱ期视力稳定4眼,1眼于随访期内出现无灌注区扩大,补充完成PRP后病情稳定;Ⅲ期中4眼行PRP后视力维持不变2眼,视力提高2眼;2眼给予雷珠单抗联合PRP治疗后视力提高;4眼伴玻璃体积血者,治疗后视力提高,其中2眼病情稳定,2眼反复出现玻璃体积血,视力下降。眼底见毛细血管无灌注区封闭,视盘或视网膜新生血管萎缩。结论：Ⅱ期病变行PRP治疗长期效果好;Ⅲ期病变可根据情况给予单独PRP或联合玻璃体切除术或抗VEGF治疗等,但可出现病情反复。早期行PRP治疗可有效控制病变发展。     

IRVAN综合征一例

41岁女性以双眼视物疲劳1年就诊。根据眼底表现及荧光素眼底血管造影（FFA）表现诊断为特发性视网膜血管炎、动脉瘤和神经视网膜炎（IRVAN）综合征二期。给予双眼周边视网膜无灌注区激光光凝,随访1年病情稳定。     

双侧视网膜动脉炎伴多发性瘤样动脉扩张激光光凝治疗疗效观察

双侧视网膜动脉炎伴多发性瘤样动脉扩张(BRAMAD),又称IRVAN综合征,是一少见的眼底病,最初被认为有很好的自限性,但后来的文献报道该病可造成严重的视力下降[1].以往文献中以病例报告较为多见[2-6],成组病例的治疗观察报道不多.我们对一组临床确诊的IRVAN综合征患者采取激光光凝为主的治疗,现将结果报道如下.     

Single Bilateral Dexamethasone Implant in Addition to Panretinal Photocoagulation and Oral Azathioprine Treatment in IRVAN Syndrome

The idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a disease characterized by multiple retinal macroaneurysms, neuroretinitis and peripheral capillary nonperfusion. Visual loss may result from either ischemia-related complications or macular involvement. Treatment is not always rewarding. We report a case with stage 2 IRVAN syndrome who was successfully treated with a single bilateral intravitreal dexamethasone implant in addition to panretinal photocoagulation and systemic azathioprine treatment.Key Words: Azathioprine, Dexamethasone implant, IRVAN syndrome, Laser photocoagulation, Ozurdex     

Long-term outcomes in a series of idiopathic retinal vasculitis,aneurysms, and neuroretinitis (IRVAN) syndrome

Objective

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare entity with a potentially poor visual prognosis. Our objective is to review the clinical presentation and long-term outcomes of patients with IRVAN syndrome.

Extensive dynamics in location,shape, and size of aneurysms in a patient with idiopathic retinal vasculitis,aneurysms, and neuroretinitis (IRVAN) syndrome. Idiopathic retinal vasculitis,aneurysms, and neuroretinitis

PURPOSE: To describe an unusual case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome with rapid dynamics in the number and appearance of the aneurysms. DESIGN: Observational case report. METHODS: Clinical and angiographic data of the patient were reviewed. RESULTS: In the course of only 6 months, preexisting retinal aneurysms resolved while new ones appeared. Changes were observed in the shape and size of preexisting lesions. The resolution of lesions in eyes previously untreated by laser is reported for the first time. CONCLUSIONS: Vascular lesions in IRVAN syndrome may show an unusually rapid turnover. The resolution of aneurysms is a part of the natural course of the disease and may occur without previous retinal laser photocoagulation.     

Idiopathic retinal, vasculitis, aneurysms and neuroretinitis (IRVAN): case report

A case of a 14 year-old boy that was admitted complaining of bilateral ocular visual blurring for 2 years is reported. The ophthalmological examination disclosed bilateral mild optic disc hyperemia and swelling, retinal exudation, few retinal hemorrhages, multiple aneurysms, as well as vasculitis. Fluorescein angiography showed extensive peripheral retinal ischemia, dilatations and hyperfluorescence of the vessels walls, and leakage of the optic disc in the late phases in both eyes. This rare case represents an entity characterized by peripheral retinal vascular occlusion, retinal vasculitis, multiple posterior retinal aneurysms, and neuroretinitis (IRVAN). Systemic evaluation and laboratory work-up did not suggest any systemic abnormality. Panretinal laser photocoagulation was performed in both eyes, and the patient was treated with oral prednisone with maintenance of the visual acuity after 1 year of follow-up. Laser treatment should be considered when angiographic evidence of widespread retinal no perfusion is present, and before the development of signs of retinal neovascularization.     

急性视网膜坏死综合征诊治新进展

急性视网膜坏死综合征(acute retinal necrosis syndrome,ARNS)是以急性葡萄膜炎、闭塞性视网膜动脉血管炎、融合性坏死性视网膜炎及后期的视网膜脱离为主要临床表现的一组眼部综合征,部分患者早期可合并有眼压升高.目前病因及发病机制尚不完全明确,多认为可能和病毒感染有关,主要以单纯疱疹病毒(herpes simplex virus,HSV)、水痘-带状疱疹病毒(varicella-zoster virus,VZV)、EB病毒及巨细胞病毒(cytomegalovirus,CMV)等病毒感染为主,诊断主要依靠临床表现、专科检查及病原学检查等.急性视网膜坏死综合征发病急,进展速度快,早期临床表现缺乏特异性,误诊率高,治疗棘手,预后较差,是眼部致盲的重要眼病,一旦确诊,及时给予局部及全身抗病毒、预防性视网膜激光光凝及必要时需行玻璃体切除术联合硅油填充术治疗.研究表明,早期有效的治疗措施,可阻止病情进展,提高患者视力.因此,早期确诊及治疗急性视网膜坏死综合征非常重要,本文将结合文献对急性视网膜坏死综合征的诊断及治疗进行综述.