Surgery management and progress of familial exudative vitreoretinopathy associated retinal detachment

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.

Keywords

• familial exudative vitreoretinopathy
• retinal detachment
• surgery
• retinal reattachment
• pediatric vitreoretinal surgery
• scleral buckling
• pars plana vitrectomy