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

Figure 1. Autosomal dominant neovascular inflammatory vitreoretinopathy pedigrees. Two related families with clinical features of autosomal dominant neovascular inflammatory vitreoretinopathy exhibited a dominant pattern of inheritance. Black symbols represent clinically affected subjects. Numbers correspond to patients who underwent fluocinolone acetonide device implantation. Open symbols represent unaffected subjects.

Figure 2. Autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV) case report. A, An electroretinogram showing a diminished b-wave helped diagnose patient 1 with ADNIV at age 17 years. B and C, Composite fundus drawings show vitreoretinal pathology overlaid with treatment that took place over several years beginning at age 21 years when the patient's visual acuity (VA) was 20/100 OU. He developed aggressive ADNIV with cystoid macular edema (CME), an epiretinal membrane, and small punctuate areas of peripheral neovascularization and vitreous hemorrhage in the right eye. There was a central vitreous hemorrhage secondary to neovascularization of the disc with a VA of counting fingers OS. Traction was noted in the periphery in the left eye. Both eyes showed characteristic peripheral pigmentary changes. Oral steroids and panretinal scatter photocoagulation (PRP) initially stabilized the patient to 20/30 OD and 20/50 OS. He subsequently lost vision because of continuous inflammation, CME, and recurrent vitreous hemorrhage while taking steroid-sparing agents over the next 2 years, including methotrexate and infliximab, and moderate doses of prednisone. At age 24 years, fluocinolone acetonide implants were placed in each eye. NVD indicates neovascularization of the disc; NVE, neovascularization elsewhere; OD, right eye; and OS, left eye. D, Preoperative fundus image of the right eye shows an epiretinal membrane and CME (VA 20/100). E, Cystoid macular edema was confirmed by fluorescein angiography. F, Postoperative fundus image shows worsening membrane in the right eye. G and H, Preoperative and postoperative optical coherence tomography (OCT) shows CME that resolved soon after device implantation. Within 1 month after surgery, a mild central retinal vein occlusion developed, and the patient was treated with oral steroids. A small area of retinal neovascularization was noted at that time, precipitating a dose of intravitreal bevacizumab and further laser photocoagulation. All vascular changes regressed in 6 months. Cataract surgery and trabeculectomy were performed in the right eye 1 and 2 years after implant placement, respectively. The right eye also required a laser peripheral iridotomy for pupillary block, but inflammation was controlled nearly 4 years after device implantation without need for replacement. I, Preoperative fundus image of the left eye shows regressed NVD, preretinal fibrosis, and an epiretinal membrane. J, Findings consistent with recurrent CME are apparent on preoperative fluorescein angiography. K, Postoperative fundus image shows progressive fibrosis in the left eye. L, The immediate preoperative OCT shows early tractional membranes superiorly (right side of OCT), without traction on the fovea or significant CME. M, Postoperative OCT shows increased tractional membranes. Implantation in the left eye was followed nearly immediately by elevated intraocular pressure that was controlled initially with topical glaucoma medication. Initially, the VA stabilized to 20/60 OS without recurrent CME, neovascularization, or hemorrhage. One year later, cataract surgery was performed, and 2 years later the implant was replaced and a trabeculectomy was performed. The only systemic immunomodulatory medications this patient received at any time after initial implant surgeries were short courses of prednisone for a mild central retinal vein occlusion in the right eye and any subsequent intraocular surgeries. Despite control of neovascularization and vitreous cells, there was worsening of preretinal fibrosis and tractional membranes and deterioration of VA to 20/80 OD and 20/200 OS.