摘要目的 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.

abstractsObjective 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.