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,所有息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(X2=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.
目的 觀察特髮性視網膜血管炎、動脈瘤、視神經視網膜(IRVAN)綜閤徵和Eales病熒光素眼底熒光血管造影(FFA)特徵的異同. 方法迴顧分析IRVAN綜閤徵患者4例8隻眼和Eales病患者43例68隻眼的FFA檢查資料.所有患者均行常規眼科檢查,包括視力、眼壓、裂隙燈顯微鏡、間接檢眼鏡眼底檢查.4例Eales病患者單眼玻璃體積血眼底窺不清,行對側眼檢查,其他所有患者雙眼常規綵色眼底照相、共焦激光掃描FFA檢查.IRVAN綜閤徵4例8隻眼,均為雙眼髮病.男性1例,女性3例;年齡16～43歲,平均年齡(27.00±12.93)歲.Eales病43例68隻眼,男性32例,女性11例;年齡6～59歲,平均年齡(30.79±11.46)歲.雙眼29例54隻眼,單眼14例14隻眼.兩組患者眼底均可見視網膜血管白鞘或白線狀改變、視網膜齣血滲齣、玻璃體積血. 結果 FFA檢查結果顯示,IRVAN綜閤徵8隻眼後極部動、靜脈均受纍;均可見多髮性視網膜大動脈瘤.週邊部毛細血管閉塞形成無灌註區7隻眼,佔87.50%;視盤水腫熒光滲漏5隻眼,佔62.50%;視神經萎縮2隻眼,25.00%;齣血遮擋1隻眼,佔12.50%;視盤新生血管2隻眼,佔25.00%;視網膜新生血管4隻眼,佔50.00%;黃斑水腫4隻眼,50.00%.Eales病43例68隻眼中,所有息眼週邊病變區視網膜靜脈管壁滲漏;纍及後極部靜脈32隻眼,佔47.06%;動脈同時受纍5隻眼,佔7.35%;週邊部毛細血管閉塞形成無灌註區38隻眼,佔55.88%;視盤滲漏29隻眼,佔42.65%;視盤新生血管4隻眼,佔5.88%;視網膜新生血管26隻眼,佔38.24%;黃斑水腫15隻眼,佔22.06%.IRVAN綜閤徵與Eales病患者在後極部動脈受纍眼數、靜脈受纍眼數、動脈瘤眼數之間比較,差異有統計學意義(P均<0.05);視盤滲漏、無灌註區、視盤和視網膜新生血管、黃斑水腫眼數之間比較,差異無統計學意義(X2=0.479,1.449,0.068,1.676;P>0.05). 結論 IRVAN綜閤徵和Eales病均可髮生視網膜動脈和靜脈不同程度的炎性改變,併均可導緻視盤水腫滲齣.IRVAN綜閤徵後極部視網膜動、靜脈受纍明顯高于Eales病,特彆是視盤徬及後極部特徵性大動脈瘤樣改變有助于IRVAN綜閤徵的診斷及IRVAN綜閤徵和Eales病的鑒彆診斷.
목적 관찰특발성시망막혈관염、동맥류、시신경시망막(IRVAN)종합정화Eales병형광소안저형광혈관조영(FFA)특정적이동. 방법회고분석IRVAN종합정환자4례8지안화Eales병환자43례68지안적FFA검사자료.소유환자균행상규안과검사,포괄시력、안압、렬극등현미경、간접검안경안저검사.4례Eales병환자단안파리체적혈안저규불청,행대측안검사,기타소유환자쌍안상규채색안저조상、공초격광소묘FFA검사.IRVAN종합정4례8지안,균위쌍안발병.남성1례,녀성3례;년령16～43세,평균년령(27.00±12.93)세.Eales병43례68지안,남성32례,녀성11례;년령6～59세,평균년령(30.79±11.46)세.쌍안29례54지안,단안14례14지안.량조환자안저균가견시망막혈관백초혹백선상개변、시망막출혈삼출、파리체적혈. 결과 FFA검사결과현시,IRVAN종합정8지안후겁부동、정맥균수루;균가견다발성시망막대동맥류.주변부모세혈관폐새형성무관주구7지안,점87.50%;시반수종형광삼루5지안,점62.50%;시신경위축2지안,25.00%;출혈차당1지안,점12.50%;시반신생혈관2지안,점25.00%;시망막신생혈관4지안,점50.00%;황반수종4지안,50.00%.Eales병43례68지안중,소유식안주변병변구시망막정맥관벽삼루;루급후겁부정맥32지안,점47.06%;동맥동시수루5지안,점7.35%;주변부모세혈관폐새형성무관주구38지안,점55.88%;시반삼루29지안,점42.65%;시반신생혈관4지안,점5.88%;시망막신생혈관26지안,점38.24%;황반수종15지안,점22.06%.IRVAN종합정여Eales병환자재후겁부동맥수루안수、정맥수루안수、동맥류안수지간비교,차이유통계학의의(P균<0.05);시반삼루、무관주구、시반화시망막신생혈관、황반수종안수지간비교,차이무통계학의의(X2=0.479,1.449,0.068,1.676;P>0.05). 결론 IRVAN종합정화Eales병균가발생시망막동맥화정맥불동정도적염성개변,병균가도치시반수종삼출.IRVAN종합정후겁부시망막동、정맥수루명현고우Eales병,특별시시반방급후겁부특정성대동맥류양개변유조우IRVAN종합정적진단급IRVAN종합정화Eales병적감별진단.
Objective To investigate the characteristics of fundus photography and fundus fluorescein angiography (FFA) of IRVAN (idiopathic retinal vasculitis, aneurysms, and neuroretinitis) syndrome and Eales disease. Methods The fundus photography and FFA data of 4 cases (8 eyes) with IRVAN syndrome and 43 cases (68 eyes) with Eales disease were retrospectively analyzed. All patients received ophthalmic routine examinations, including visual acuity, intraocular pressure, slit-lamp microscope and indirect ophthalmoscope. All patients had taken fundus photography and FFA for both eyes, except 4 patients of Eales disease who had vitreous hemorrhage in one eye. All 4 cases (1 male/3 female )with IRVAN syndrome were bilateral and aged 16-43 years old(mean age 27.00±12.93 years old). 43 cases (32 male/11 female) of Eales disease aged 6-59 years old( mean 30.79±11.46 years old), 29 cases were bilateral and 14 cases were unilateral. Both diseases had retinal vascular white-sheath or white thread-like changes, exudative retinal hemorrhage and vitreous hemorrhage. Results Both arteries and veins of posterior pole of all eyes with IRVAN syndrome were involved and shown multiple retinal macroaneurysms. Other signs of IRVAN syndrome included capillary occlusion and non-perfusion (7/8 eyes, 87.5%), fluorescein leakage and edema of optic disc (5/8 eyes, 62.5%), optic atrophy (2/8 eyes, 25 %), vitreous hemorrhage(1/8 eyes, 12.5 %), neovascularization of optic disc(2/8 eyes, 250%), retinal neovascularization (4/8 eyes, 50%) and macular edema(4/8 eyes, 50%). The signs of Eales disease included fluorescein leakage of peripheral retinal vein (68/68 eyes, 100%), fluorescein leakage of posterior retinal vein (32/68 eyes, 47.06%), artery involvement (5/68 eyes, 7.35%), peripheral capillary occlusion and non-perfusion (38/68 eyes, 55.88 %), fluorescein leakage of optic disc(29/68 eyes, 42.65%), neovascularization of optic disc (4/68 eyes, 5.88%), retinal neovascularization(26/68 eyes, 38.2%) and macular edema(15/68 eyes, 22. 06%). Compared IRVAN syndrome with Eales disease, the difference of artery inflammation, vein inflammation, retinal macroaneurysms in posterior area had statistics significance(all P=0.00, P<0.05), and that of edema of optic disc, retinal vascular nonperfusion area, neovascularization of optic disc, neovascularization elsewhere, and macular edema had no statistics significance(x2=0. 479, P>0.05;P=0.131 ,P>0.05;X2=1.449, P>0.05;X2=0.068, P>0.05;X2=1.676, P>0.05). Conclusions Both IRVAN syndrome and Eales disease may have vein and artery inflammation in posterior pole of the eye, and may result in neuroretinitis. IRVAN syndrome has much more vein and artery inflammation in posterior pole than Eales disease. Posterior retinal macroaneurysms is the most important sign for the diagnosis and differential diagnosis of IRVAN syndrome and Eales disease.
