Perifoveal Chorioretinal Atrophy after Subretinal Voretigene Neparvovec-rzyl for RPE65-Mediated Leber Congenital Amaurosis

To report an anatomic change following subretinal injection of voretigene neparvovec-rzyl (VN) for RPE65-mediated Leber congenital amaurosis. Multicenter, retrospective chart review. Patients who underwent subretinal VN injection at each of 4 participating institutions. Patients were identified as having perifoveal chorioretinal atrophy if (1) the areas of atrophy were not directly related to the touch-down site of the subretinal cannula; and (2) the area of atrophy progressively enlarged over time. Demographic data, visual acuity, refractive error, fundus photographs, OCT, visual fields, and full-field stimulus threshold (FST) were analyzed. Outcome measures included change in visual acuity, FST, visual fields, and location of atrophy relative to subretinal bleb position. A total of 18 eyes of 10 patients who underwent subretinal injection of VN were identified as having developed perifoveal chorioretinal atrophy. Eight of 10 patients (80%) developed bilateral atrophy. The mean age was 11.6 years (range, 5–20 years), and 6 patients (60%) were male. Baseline mean logarithm of the minimum angle of resolution visual acuity and FST were 0.82 (standard deviation [SD], 0.51) and −1.3 log cd.s/m2 (SD, 0.44), respectively. The mean spherical equivalent was −5.7 diopters (D) (range, −11.50 to +1.75 D). Atrophy was identifiable at an average of 4.7 months (SD, 4.3) after surgery and progressively enlarged in all cases up to a mean follow-up period of 11.3 months (range, 4–18 months). Atrophy developed within and outside the area of the subretinal bleb in 10 eyes (55.5%), exclusively within the area of the bleb in 7 eyes (38.9%), and exclusively outside the bleb in 1 eye (5.5%). There was no significant change in visual acuity (P = 0.45). There was a consistent improvement in FST with a mean improvement of −3.21 log cd.s/m2 (P < 0.0001). Additionally, all 13 eyes with reliable Goldmann visual fields demonstrated improvement, but 3 eyes (23.1%) demonstrated paracentral scotomas related to the atrophy. A subset of patients undergoing subretinal VN injection developed progressive perifoveal chorioretinal atrophy after surgery. Further study is necessary to determine what ocular, surgical delivery, and vector-related factors predispose to this complication

ISCHEMIC INDEX AND NEOVASCULARIZATION IN CENTRAL RETINAL VEIN OCCLUSION

Purpose: To explore the association of angiographic nonperfusion with anterior segment and posterior segment neovascularization in central retinal vein occlusion (CRVO). Methods: An imaging database at one institution was searched for the diagnosis of central retinal vein occlusion. Ultra wide field fluorescein angiograms were graded for image quality, the presence of retinal neovascularization, and the quantity of nonperfusion; an ischemic index (ISI) was calculated. Charts were reviewed to exclude eyes with previous treatment and to determine which eyes had anterior segment or posterior segment neovascularization on the day of the angiogram. Time from onset to presentation could not accurately be ascertained. Results: In a 39-month period, there were 69 eyes that met inclusion criteria. The mean ISI was 25% (SD, 26%; range, 0-100%), and 15 eyes (21%) with neovascularization had a mean ISI of 75% (range, 47-100%) compared with eyes without neovascularization that had an ISI of 6% (range, 0-43%). Ischemic index significantly correlated to neovascularization, and eyes that had evidence of neovascularization had an ISI >45% (P < 0.0001). Conclusion: Ultra wide field fluorescein angiography provides visualization of nonperfusion in eyes with central retinal vein occlusion. Eyes with neovascularization on the day of the angiogram were found to have significantly larger areas of retinal nonperfusion compared with eyes without neovascularization. A prospective study is indicated to know if early treatment of peripheral retinal nonperfusion in CRVO improves outcomes. RETINA 31:105-110, 201