摘要目的 观察分析家族性渗出性玻璃体视网膜病变(FEVR)相关性孔源性视网膜脱离的临床特征.方法 回顾性研究.经对侧眼荧光素眼底血管造影(FFA)检查确诊为FEVR的32例患者纳入研究.其中,男性26例,女性6例,男女发病比例为4.3:1.平均发病年龄为(19.35±8.83)岁.所有患眼均行视力、验光、前置镜、眼底彩色照相及FFA检查.记录患眼视力,屈光状态,视网膜脱离形态,裂孔位置、大小和形态,增生性玻璃体视网膜病变(PVR)的分级及视网膜下增生情况.观察对侧眼的FFA表现特征.结果 患眼屈光度为+2 D～-13 D,视力为光感～0.7.所有患眼均可见颞侧周边部圆形萎缩孔伴或不伴有格子样变性.其中,伴马蹄形裂孔6只眼,占18.18％;伴黄斑裂孔 2只眼,占6.06％.伴有PVR C2级及以上者10只眼,占30.30％.合并视网膜下增生23只眼,占69.70％.对侧眼FFA检查发现,颞侧周边部无血管区伴有动静脉吻合支或静脉分支增多变直,周边部血管通透性增加.结论 FEVR相关性RRD的裂孔多见于颞侧周边部,呈小圆形萎缩孔;多伴有视网膜下增生.

abstractsObjective To investigate the clinical characteristics and surgical management of the familial exudative vitreoretinopathy (FEVR)-associated rhegmatogenous retinal detachment (RRD).Methods Retrospective noncomparative case series.Thirty-three eye of 32 patients were diagnosed FEVR-associated RRD by Fluorescein fundus angiography.There were 26 males and 6 females.The male to female ratio is 4.3 ∶ 1 with an average age of 19.35 ± 8.83 years.The detection of best corrected visual acuity (BCVA),refraction status,fundus photograph and fluorescein fundus angiography(FFA)were underwent in all patients.FEVR was confirmedby FFA and positive family history.The BCVA,refraction status,morphology of retinal detachment,location,size and shape of retinal hole,presence and grade of proliferative vitreoretinopathy (PVR),and subretinal proliferation were recorded.Results As for the refractive status,the scope of refraction was +2.0 D to-13.0 D andthe BCVA were range from light perception to 0.7.Atrophy holes which located at the temporal half were responsible for retinal detachment in all cases.Besides,horseshoe tears were noted in 6 eyes (18.18％),while macular tears were noted in 2 eyes of RRD (6.06％).The PVR greater than stage C2 was noticed in 10 eyes (30.30％),while subretinal proliferation was presented in 23 eyes (69.70％).Conclusions Male predominance,juvenile onset and associated with moderate to high myopia are the main characteristics in FEVR-associated RRD.Atrophy holes at the temporal half and the subretinal proliferations were most commonly in FEVR-associated RRD.Detailed fuduns and FFA examination of the fellow eye should be undergone to avoid misdiagnosis.