家族性渗出性玻璃体视网膜病变相关的孔源性视网膜脱离临床特征的回顾性观察性研究

Clinical features of familial exudative vitreoretinopathy-associated rhegmatogenous retinal detachment

目的分析研究家族性渗出性玻璃体视网膜病变(FEVR)相关的孔源性视网膜脱离(RRD)患者的临床特征。方法回顾性研究。收集自2018年5月至2020年8月就诊于安徽医科大学第一附属医院眼科的RRD患者共202例(203只眼),观察分析经广角眼底照相及荧光素眼底血管造影(FFA)确诊为FEVR相关的RRD所占的比例及其临床特征。结果FEVR相关的RRD共19例(19只眼),包括男性15例(15只眼),女性4例(4只眼),年龄(23.21±9.37)岁(10~45岁),占同期RRD患者的9.31%(19/202)。在10~30岁的RRD患者中,FEVR相关的占60.71%(17/28)。术前及术后平均视力分别为0.14±0.16、0.14±0.18(P>0.05)。其中,73.68%(14/19)的视网膜脱离部位为颞侧,裂孔多位于颞侧78.95%(15/19),42.11%(8/19)患者具有2个及以上裂孔。所有患者均合并对侧眼的异常,表现为视网膜无血管区、血管异常、视网膜变性及裂孔、视网膜嵴样改变、视网膜皱褶等。结论PEVR是导致青少年人群发生RRD的主要原因之一,早期诊断、及时干预是改善患者视力预后的关键。

Objective To analyze the clinical features of familial exudative vitreoretinopathy(FEVR)-associated rhegmatogenous retinal detachment(RRD).Methods A total of 202 patients(203 eyes)with RRD at the First Affiliated Hospital of Anhui Medical University from May 2018 to August 2020 were enrolled for retrospective analysis.The proportion and clinical characteristics of FEVR-associated RRD diagnosed using ultra-wield-field fundus photography or fundus fluorescein angiography were observed and analyzed.Results FEVR-associated RRD accounted for 9.31%(19/202)of RRD patients in the same period,including 15 males(15 eyes)and 4 females(4 eyes)with an average age of 23.21±9.37 years.Among RRD patients aged 10 to 30 years,FEVR-associated RRD accounted for 60.71%(17/28).The average visual acuity before and after the surgery was 0.14±0.16 and 0.14±0.18,respectively(P>0.05).Among the FEVR-associated RRD patients,73.68%(14/19)of retinal detachment was located in the temporal side,78.95%(15/19)retinal holes were located in the temporal side,and 42.11%(8/19)had more than one retinal holes.All of the fellow eyes in the 19 patients were found abnormal,lesions including retinal avascular area,retinal vascular abnormalities,retinal degeneration or breaks,retinal ridge and retinal folds.Conclusions FEVR is one of the main causes of RRD in adolescents.Early diagnosis and timely intervention are crucial for improving the visual prognosis of patients.