Management, follow-up & prognosis


The main objective is the early diagnostic and treatment of the disease. Prevention of the primary manifestations of the disease is not possible at present, but prevention and treatment of manifestations mostly related to the neovascularization process may be achieved by prophylactic cryotherapy or argon laser.

The management of FEVR, however, is a matter of controversy and not all authors defend prophylaxis with photocoagulation or cryocoagulation. According to the majority of researchers, the main indication for prophylactic treatment is peripheral neovascularisation and exudative retinal detachment, although the results of this approach have not been conclusive. Since FEVR has such a variable course, only patients at high risk of progression should receive prophylactic treatment.

Scleral buckling procedures and vitrectomy are used to treat retinal detachment.

Therapies under investigation include angiogenesis inhibitors such as antivascular endothelial growth factor (anti-VEGF) drugs (pegaptanib, bevacizumab).


Children should undergo regular fundus examination to evaluate for development of retinal neovascularization, traction, or exudate.

Family screening (ideally by fundus fluorescein angiography to identify any characteristic lesions) is recommended.

Genetic counseling to young adults who are affected or at risk should be offered.


The clinical course of FEVR is variable, but the most severe forms may be active for life, with variable periods of quiescence. Long-term outcomes for serious disease include severe visual impairment and blindness.