Huckfeldt Rachel M, Comander Jason
a Department of Ophthalmology , Massachusetts Eye and Ear , Boston , MA , USA.
Semin Ophthalmol. 2017;32(1):43-51. doi: 10.1080/08820538.2016.1228404. Epub 2016 Oct 17.
Retinitis pigmentosa is a genetically heterogeneous disorder with an estimated prevalence of one in 4,000 that is classically characterized by the progressive constriction of peripheral vision and a later deterioration of visual acuity. Central vision can be compromised earlier in disease, however, in the approximately 25% of patients that have cystoid macular edema. This poorly understood problem can thus significantly impair patient quality of life, particularly as available treatments have limited efficacy. We will review clinical features of retinitis pigmentosa-associated cystoid macular edema, potential causative mechanisms, and finally, evidence supporting currently employed therapies with emphasis upon which management strategies require further evidence-based evaluation.
视网膜色素变性是一种具有遗传异质性的疾病，估计患病率为四千分之一，其典型特征是周边视野逐渐缩小，随后视力下降。然而，在约25%患有黄斑囊样水肿的患者中，疾病早期中心视力就可能受到损害。这个尚未完全了解的问题会严重损害患者的生活质量，尤其是现有治疗方法疗效有限。我们将回顾视网膜色素变性相关黄斑囊样水肿的临床特征、潜在致病机制，最后，阐述支持当前所用治疗方法的证据，重点关注哪些管理策略需要进一步基于证据的评估。
