原发性视网膜色素变性继发黄斑囊样水肿的治疗进展

Treatment advances of cystoid macular edema secondary to retinitis pigmentosa

原发性视网膜色素变性(RP)是一种以进行性光感受器细胞萎缩及色素上皮细胞萎缩为共同表现的遗传性视网膜变性疾病。其常见的并发症包括以后囊膜混浊为特征的白内障、黄斑囊样水肿(CME)及玻璃体腔内轻度炎症反应等。其中CME可进一步引起患者的视力下降,发病率高达10%~20%。目前RP继发CME的发病机制尚未明确,其主要发病机制可能与视网膜色素上皮层的功能破坏有关。目前治疗方法主要是应用碳酸酐酶抑制剂口服减轻CME的症状,并局部应用糖皮质激素;或行玻璃体切除术以缓解玻璃体视网膜的牵拉。近年来有研究者发现抗血管内皮生长因子治疗RP继发的CME可有效减轻患者的水肿,这为RP继发CME的治疗提供了新的途径。笔者仅就对RP继发CME的发病机制及治疗方法进行综述。

Retinitis pigmentosa ( RP) is an inherited retinal degenerative disease,characterized as the progressive atrophy of photoreceptors and retinal pigment epithelium.Posterior central subcapsular cataract,cystoid macular edema (CME) and mild inflammatory reactions in the vitreous are the most frequent complications of RP.The incidence rate of CME is as high as 10%-20%,which would cause the impairment vision acuity.Currently, the pathogenesis of CME in RP is poorly understood, the dysfunction of retinal pigment epithelial is thought to be the major one.At present, the main treatment method is carbonic anhydraseinhibitor for oral administration which can alleviate the symptoms of CME.Local application of glucocorticoids such as TA or vitrectomy also has certain effect for vitreoretinal traction.Latest researches show that anti-vascular endothelial growth factor therapy can release CME,which provide a new strategy for the treatment of CME in RP.Its pathogenesis and treatment methods of RP secondary to CME were reviewed in this paper.