Inhibition of Neovascularization but Not Fibrosis With the Fluocinolone Acetonide Implant in Autosomal Dominant Neovascular Inflammatory Vitreoretinopathy

Abstract

Objective To review the effect of the fluocinolone acetonide implant (FA) in subjects with Autosomal Dominant Neovascular Inflammatory Vitreoretinopathy (ADNIV), an inherited, autoimmune uveitis. Methods A retrospective case series was assembled from ADNIV patients who underwent fluocinolone acetonide implantation. Visual acuity and features of ADNIV, including inflammatory cells, neovascularization, fibrosis, and cystoid macular edema were reviewed. Results Nine eyes of five related ADNIV patients with uncontrolled inflammation were reviewed. Follow-up ranged 21.7–56.7 months. Vision at implantation ranged from 20/40 to hand motion. Preoperatively, eight eyes demonstrated vitreous cell (an eighth had a diffuse vitreous hemorrhage). Eight eyes demonstrated cystoid macular edema, seven had an epiretinal membrane, and three manifested retinal neovascularization. Following implantation, vitreous cells resolved in all eyes and neovascularization regressed or failed to develop. Central macular thickness improved in four eyes. During the postoperative course, however, visual acuity continued to deteriorate, with vision at the most recent examination ranging from 20/60 to no light perception. There was also progressive intraocular fibrosis and phthisis in one case. Four eyes underwent cataract surgery. Six of the seven eyes without previous glaucoma surgery demonstrated elevated intraocular pressure at some point, and three of these required glaucoma surgery. Conclusions FA implantation may inhibit specific features of ADNIV such as inflammatory cells and neovascularization, but does not stabilize long-term vision, retinal thickening, or fibrosis. All eyes in this series required cataract extraction, and more than half required surgical intervention for glaucoma. Further studies may identify additional therapies and any benefit of earlier implantation.

Secondary glaucoma in <em>CAPN5</em>-associated neovascular inflammatory vitreoretinopathy

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Functional validation of a human CAPN5 exome variant by lentiviral transduction into mouse retina

“…It is possible that the alterations in distinct signaling cascades by mutant CAPN5 could account for the various inflammatory, degenerative, vascular and fibrotic phenotypes observed in ADNIV patients. This hypothesis is supported by our clinical studies, in which intraocular steroids suppress inflammatory cell recruitment and angiogenic pathways, but not fibrotic or retinal degenerative pathways (9,10).…”

Surgical management of fibrotic encapsulation of the fluocinolone acetonide implant in CAPN5-associated proliferative vitreoretinopathy

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