Intravitreal Anti-VEGF Therapy for Choroidal Neovascularization-Associated with Best Vitelliform Macular Dystrophy

Abstract

Purpose : To report on the effect of intravitreal anti-VEGF therapy for choroidal neovascularization (CNV) due to Best vitelliform macular dystrophy (BVMD) at a tertiary referral center.

Methods : This is a retrospective chart review. All electronic medical records with a previous diagnosis of BVMD and CNV/retinal hemorrhage/retinal edema at Casey Eye Institute underwent review of the chart with attention to the effect of anti-VEGF therapy. Adult-onset vitelliform dystrophy was excluded. Patients with inactive CNV that did not require treatment were excluded. ANOVA with Holm-Sidak for multiple comparisons were used for statistical analysis.

Results : Six patients with BVMD required anti-VEGF injection with age at time of treatment ranging from 5 to 57 years (mean: 26 ±23 yrs). Bilateral treatment was necessary in 3 patients (50%), of which 2 were the youngest patients of the cohort at 5 and 8 years of age. Of the 9 eyes that required treatment, the best-corrected visual acuity (BCVA) at presentation ranged from 20/30 to 20/80 (mean: 0.47 ±0.05 logMAR or 20/60). Regardless of the treatment strategy, there was a trend towards better BCVA at 1 month (0.37 ±0.05 logMAR or 20/50, p=0.32) and 3 months (0.32 ±0.07 logMAR or 20/40, p=0.13) with significant improvement by 1 year ranging from 20/25 to 20/30 (0.15 ±0.01 logMAR or 20/30, p=0.0002). Treatment strategy varied from 2 injections to continual injections at various intervals. Two eyes with the longest follow-up time had stable improvement of BCVA of 20/30 at 20 months and 20/25 at 36 months after cessation of treatment.

Conclusions :
While CNV occurs rarely in patients with BVMD at all ages, bilateral cases tended to affect younger patients in our small cohort. Regardless of treatment strategy, BCVA improves significantly with prompt intravitreal anti-VEGF therapy. Additional follow-up in larger cohorts will be required to compare treatment strategies with long-term visual prognosis and rate of active CNV recurrence in BVMD.

This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.