Macular neovascularization in familial exudative vitreoretinopathy

To describe an unusual patient with autosomal dominant familial exudative vitreoretinopathy (FEVR) who also presented with preretinal fibrosis overlying the macula, extensive macular telangiectasis, and multiple foci of macular neovascularization.

A clinical case is presented including fundus images and fluorescein angiography studies of the patient's left eye containing the unusual manifestations.

To our knowledge, this is the first report of the formation of multifocal, preretinal macular neovascularization in FEVR. The sites of neovascularization corresponded to the attachment points of the vitreous stalk overlying the macula. Two small areas of intraretinal neovascularization were also noted.

The concordance of the vitreoretinal adhesions with the sites of neovascularization suggests that the focal scaffolding may have played a direct mechanical role in encouraging the growth of the macular neovascular tissue in this patient.