Familial Exudative Vitreo-Retinopathy (FEVR)

7 main points:

1. Avascular peripheral retina with subsequent dragging of the vessels, with or without retinal folds, as well as preretinal, intraretinal, or subretinal exudation.

2. Present anytime but asymptomatic most times.

3. Staging Classification:

Stage 1 (avascular retinal periphery)

Stage 2 (pretinal neovascularisation)

Stage 3-5 (3: macular on, 4: macular off; 5: total – retinal detachment)

4. Widefield FFA is an invaluable tool to diagnose the condition – signs to watch out for includes peripheral retinal nonperfusion, vessel pruning, avascularity, neovascularization, straightening of vessels, and peripheral vascular anastomoses

5. Treatment includes:

Stage 1 – observe

Stage 2 – laser PRP at areas of peripheral retinal nonperfusion

Stage 3 to 5 – vitrectomy +/- scleral buckle.

6. Anti- VEGF – useful only to contain progression of disease or reduce vascular activity prior to surgery. Not the main therapy.

7. Other diagnosis to consider are ROP and Coat’s disease

 

Useful References:

http://retinatoday.com/pdfs/0313RT_F2_Recchia.pdf

http://eyewiki.aao.org/Familial_Exudative_Vitreoretinopathy_(FEVR)#Differential_Diagnosis

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