THE IMPACT OF CYSTOID MACULOPATHY IN USH2A RETINITIS PIGMENTOSA

Purpose: – Retinitis pigmentosa (RP) is often complicated by cystoid maculopathy (CM). The clinical impact of CM in the RP long-term outcome is still unclear. We described the 5-year outcome of USH2A-associated RP eyes with and without CM.Methods: – We retrospectively analyzed patients with USH2A-associated RP. Eyes with CM were treatment naïve. We collected data about best-corrected visual acuity, ellipsoid zone (EZ) loss rate, and CM behavior over 5 years of follow-up. The main outcome measures were the impact of CM on best-corrected visual acuity and EZ loss rate. Secondary outcome was the assessment of the clinical impact of CM persistence or regression.Results: – We included 25 RP eyes with CM and 25 eyes without CM. No difference in age and gender distributions have been found. Visual acuity was similar between eyes with and without CM (P < 0.05), although it resulted slightly worse in eyes with persistent CM. Eyes without CM had significantly lower EZ width compared with eyes with CM. However, the rate of EZ loss/year was statistically similar between eyes without and with CM. The EZ loss rate maintains similar profiles of progression, up the CM regression, associated with a significant acceleration of EZ loss. After this episode, EZ loss rate showed similar trends than those registered before the CM regression. CM regression and recurrence showed no significant effect on best-corrected visual acuity (P > 0.05). Moreover, we found no significant impact of CM reappearance on the EZ loss rate. Conclusion: – CM showed no negative impact on the long-term visual outcome of USH2A-associated RP.