Arrigo Alessandro, Aragona Emanuela, Antropoli Alessio, Bianco Lorenzo, Bandello Francesco, Parodi Maurizio Battaglia
Ophthalmology Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy ; and.
Eye Repair Unit, Division of Neuroscience, IRCCS San Raffaele Scientific Institute, Milan, Italy .
Retina. 2025 Oct 1;45(10):1959-1966. doi: 10.1097/IAE.0000000000004560.
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.
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.
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.
CM showed no negative impact on the long-term visual outcome of USH2A -associated RP.
视网膜色素变性（RP）常并发黄斑囊样水肿（CM）。CM对RP长期预后的临床影响仍不明确。我们描述了伴有和不伴有CM的USH2A相关RP眼的5年预后情况。
我们回顾性分析了USH2A相关RP患者。患有CM的眼未接受过治疗。我们收集了最佳矫正视力、椭圆体带（EZ）丢失率以及5年随访期间CM变化的数据。主要观察指标为CM对最佳矫正视力和EZ丢失率的影响。次要观察指标是评估CM持续存在或消退的临床影响。
我们纳入了25只患有CM的RP眼和25只未患CM的眼。未发现年龄和性别分布存在差异。患有和未患CM的眼的视力相似（P<0.05），尽管持续性CM的眼的视力略差。与患有CM的眼相比，未患CM的眼的EZ宽度明显更低。然而，未患CM和患有CM的眼每年的EZ丢失率在统计学上相似。在CM消退之前，EZ丢失率保持相似的进展情况，CM消退后，EZ丢失率显著加速。在此事件之后，EZ丢失率显示出与CM消退之前记录的趋势相似。CM消退和复发对最佳矫正视力无显著影响（P>0.05）。此外，我们发现CM再次出现对EZ丢失率无显著影响。
CM对USH2A相关RP的长期视力预后无负面影响。
