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Ismaël Chehaibou, Ana Clément, Florence Metge, Catherine Edelson, Aude Affortit, Pascal Dureau, Georges Caputo; Clinical presentation and management of Familial Exudative Vitreoretinopathy. Invest. Ophthalmol. Vis. Sci. 2016;57(12):158.
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© ARVO (1962-2015); The Authors (2016-present)
To report the clinical characteristics and surgical outcomes of patients with familial exudative vitreoretinopathy (FEVR).
Retrospective interventional case series of patients with FEVR. Data were collected from patient charts including demographic features, clinical and angiographic findings, initial and final best visual acuity. Depending on the severity of disease, patients were treated either with peripheral laser photocoagulation, cryotherapy or surgery (vitrectomy and/or scleral buckling). Inclusion criteria included clinical diagnosis of FEVR. Primaries outcomes were anatomic features: status of the macula and extent of retinal detachment. The secondary outcome was the visual acuity.
We included 85 eyes of 44 patients. Patients were male predominant (68%) and the average age at presentation was 6,96 +/- 7,88 years (range 1month – 42 years). Stage 1 FEVR was identified in 13 eyes (15%), stage 2 in 17 eyes (20%), stage 3 in 5 eyes (6%), stage 4 in 34 eyes (40%) and stage 5 in 16 eyes (19%). Eighteen eyes didn’t require any treatment. Thirty three eyes were treated only by peripheral laser coagulation or cryotherapy. In this group the macula was attached in 25 eyes (75%) without requiring further intervention. Thirty-four eyes underwent surgery: 15 vitrectomies, 18 vitrectomies and scleral buckling and 1 scleral buckling alone. In this group, at the last examination, the macula was completely attached in 16 eyes (47%). Visual acuity was assessable for 30 eyes and it increased in 11 eyes (37%), remained stable in 15 eyes (50%) and decreased in 4 eyes (13%). Overall, among the 65 eyes with evaluable visual acuities, 25 eyes (38%) achieved Snellen acuities of at least 20/100 and in two eyes acuities dropped to no light perception.
Early diagnosis, based on wide-field fundus examination with fluorescein angiography and prompt structured management are beneficial in patients with FEVR. Peripheral laser photocoagulation prevents from poor evolution in early stages and contributes to reduce extraretinal vascularization and subretinal exudate before surgery in selected cases of retinal detachment. In advanced stages, surgical management improves the retinal reattachment rate and allows preservation of functional visual acuity.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.
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