[关键词]
[摘要]
家族性渗出性玻璃体视网膜病变(FEVR)是一种遗传性视网膜血管疾病，最典型的临床特征是周边无血管区，病情进展可导致视网膜脱离，晚期可继发白内障、青光眼、角膜混浊甚至眼球萎缩，是亚洲儿童、青少年视网膜脱离的主要原因之一。FEVR相关视网膜脱离主要通过手术治疗，包括玻璃体切除术、巩膜扣带术，有时联合视网膜光凝术、抗血管内皮生长因子(VEGF)治疗等。手术方式选择有赖于详细的术前、术中评估，包括患眼分型、分期、纤维增殖情况和眼前段并发症等，不同分期患眼解剖及视功能预后大不相同。孔源性视网膜脱离成年患者的手术复位率较为理想，而牵拉性视网膜脱离儿童患者手术复位率相对较低。低龄、晚期患者手术难度大、预后不良，手术治疗策略存在争议。本文就FEVR相关视网膜脱离的手术治疗效果与进展进行综述。
[Key word]
[Abstract]
Familial exudative vitreoretinopathy(FEVR)is a hereditary disorder of retinal angiogenesis, of which the most typical clinical feature is the peripheral avascular area. The progression of the disease can lead to retinal detachment(RD), followed by secondary complications such as cataracts, glaucoma, corneal opacification, and even bulbi phthisis. As one of the main reasons for the RD in Asian children and juvenile, FEVR-associated retinal detachment(FEVR-RD)is mainly treated by surgery, including pars plana vitrectomy, scleral bucking, and combined with retinal photocoagulation or anti-vascular endothelial grouth factor(VEGF)sometimes. The surgery of FEVR-RD requires careful pre/intra-operative evaluation to determine the best surgical procedure and its outcome, including the nature of the affected eye, the stage, the fibrosis of fiber proliferation and the anterior complications, and the anatomy and prognosis of eyes in different stages vary greatly. Adult patients with rhegmatogenous RD have a higher rate of post-operative retina reattachment compared to young patients with tractional RD. In young or advanced patients, surgery is difficult and the prognosis is less optimistic, thus the surgery strategy is controversial. This article reviews the surgical outcomes and progress of FEVR-associated RD.
[中图分类号]
[基金项目]
国家自然科学基金项目(No.82171069); 上海市青年科技英才扬帆计划项目(No.20YF1429700); 上海申康医院发展中心《促进市级医院临床技能与临床创新三年行动计划》关键支撑项目(No.SHDC2020CR5014-002)
