目的：观察家族性渗出性玻璃体视网膜病变（familial exudative vitreoretinopathy，FEVR）在荧光素眼底血管造影（fluorescein fundus angiography，FFA）中的特征性表现，并探讨 FEVR患者 FFA的诊断标准，以提高该类疾病的早期临床诊断率。方法将临床检查确诊为 FEVR的患者13例、26只眼纳入研究，经过眼前节及眼底的详细检查，并依据Pendergast制定的FEVR分期标准，对13例26只眼进行FEVR分期，应用海德堡眼底血管造影成像系统进行检查，记录并分析其眼底特征。结果13例26只眼中，1期15只眼，表现为周边视网膜出现无血管区，多位于颞侧，视网膜血管变直、分支增多、异常吻合；2期7只眼，表现为视网膜新生血管形成、荧光素渗漏；3期2只眼，表现为周边视网膜浅脱离，脱离范围较局限；4期2只眼，表现为黄斑区视网膜脱离；5期0只眼。结论FFA是诊断 FEVR的特异检查指标，对 FEVR的分期起到至关重要的作用，有助于指导临床早期诊断及选择最佳治疗方案。%Objective To observe the characteristics of familial exudative vitreoretinopathy (FEVR)on fluorescein fundus angiography (FFA),to discuss the FFA diagnostic criteria in patients who suffered from FEVR,and to improve the clinical diagnostic rate of this disease. Methods Thirteen patients(26 eyes)were diagnosed of FEVR by clinical examination in the Second Hospital of Hebei Medical University.After detailed check in anterior segment and fundus,we classified the eyes according to the criteria of Pendergast for familial exudative vitreoretinopathy.Heidelberg fundus angiography imaging system was applied to inspect 13 patients,and then we recorded and analyzed the characteristics.Results In 1 3 patients(2 6 eyes), 1 5 eyes were at stage 1 with uncompleted vascularization of the periphery,especially in the temporal quadrant,very straight retinal vessels,and more branches of peripheral vessels on FFA;seven eyes were at stage 2 with neovascularization and fluorescein leakage on FFA;two eyes were at stage 3 with limited retinal detachement;two eyes with retinal detachement involving macula were classified as stage 4;no total retinal detachement (stage 5)were observed.Conclusion FFA, as a the specific diagnostic measurement of FEVR,plays an important role in the stage classification of FEVR,and FFA can guide the early diagnosis of FEVR and be of great help in choosing the appropriate treatment.
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