家族性渗出性玻璃体视网膜病变患儿双亲的眼底血管造影分析

Fundus Angiography Analysis of Parents in Children With Familial Exudative Vitreoretinopathy

目的分析家族性渗出性玻璃体视网膜病变(FEVR)患儿双亲的眼底血管特征,探究对患儿双亲进行眼底筛查对FEVR的诊断价值。方法选取2017年6月至2019年4月华中科技大学同济医学院附属同济医院拟诊断FEVR的患儿的父母共14对父母28人(共56眼),进行眼底血管造影(FFA)筛查,分析其影像学特征,并对阳性率和阳性指征进行统计分析。结果有9人不同程度眼底血管异常,阳性率32.1%;在阳性病例中父亲6人,占66.7%,母亲3人,占33.3%;所有阳性病例中,7人为双眼异常,2人单眼异常;其中阳性指征主要包括血管走形异常、血管分支较多、周边血管末端提前终止、周边小血管扩张并伴有少量荧光渗漏。另有2例发现周边大范围无灌注区、继发新生血管、造影晚期周边血管荧光异常渗漏。未见黄斑牵拉移位、视网膜皱襞、脱离的严重病例。结论眼底荧光血管造影筛查患儿双亲眼底对FEVR的诊断具有重要意义;对父母眼底血管无异常的病例也不能轻易否定FEVR的诊断。

Objective To analyze the fundus vascular characteristics of parents with familial exudative vitreoretinal disease(FEVR)and to explore the diagnostic value of fundus screening for FEVR children’parents.Methods From June 2017 to April 2019,parents of children with FEVR were selected from Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology for fundus angiography(FFA)screening.The imaging characteristics were analyzed,and the positive rate and positive indications were analyzed.A total of 14 parents,28,56 eyes.Results There were 9 patients with abnormal fundus vessels in varying degrees,the positive rate was 32.1%,and 6 fathers(66.7%)were positive.Among all the positive cases,7 cases had binocular abnormalities and 2 cases had monocular abnormalities,among which the positive indications mainly included abnormal appearance of blood vessels,more branches of blood vessels,early termination of the end of peripheral blood vessels,dilatation of peripheral small vessels and a small amount of fluorescence leakage.In other two cases,there was no perfusion area in the surrounding area,secondary neovascularization,and abnormal fluorescence leakage of the peripheral blood vessels in the late stage of angiography.There were no severe cases of macular traction displacement,retinal folds and detachment.Conclusion Fundus fluorescein angiography is of great significance in the diagnosis of FEVR in children with fundus fluorescein angiography.The diagnosis of FEVR can not be easily negated in the cases where there is no abnormality in the fundus blood vessels of parents.