急性区域性隐匿性外层视网膜病变的研究进展

Current research of acute zonal occult outer retinopathy

急性区域性隐匿性外层视网膜病变（AZOOR）是一种少见的、视网膜外层功能障碍性疾病.AZOOR好发于年轻女性,多表现为急性起病,早期眼底通常未见特异性改变,多出现1个或多个区域视野缺损和闪光感,多数患者随后出现视网膜色素上皮的萎缩及色素沉着,且经常出现视网膜电图（ERG）异常和视野的缺损,光学相干断层扫描（OCT）可显示内段（IS）/外段（OS）区域性异常改变与视野缺损区位置相对应,同时视野缺损的形态及位置与多焦视网膜电图（mfERG）所示视网膜振幅密度降低区域相吻合.部分患者视功能可以发生永久性损害.眼底检查、mfERG、眼底血管造影（FA）、吲哚青绿血管造影（ICGA）、眼底自发荧光（AF）以及频域OCT等多种影像方法综合分析和研究对于AZOOR的确诊有重要意义.以往对AZOOR认识不足,经常被误诊或漏诊.为了提高眼科医师对AZOOR的认识,就AZOOR的定义、病因、流行病学特征、临床表现、分类、诊断、鉴别诊断、治疗及预后方面的研究进展进行综述.

Acute zonal occult outer retinopathy （AZOOR） is a relatively rare ocular disease that is characterized by the rapid loss of one or more zones of outer retinal function.AZOOR usually occurs in young women.AZOOR patients typically present with the sudden onset of photopsia and acute scotomas related to loss of sectors of outer retinal function.Early in the course of AZOOR,funduscopic appearance was often normal;however,most patients developed zones of retinal pigment epithelial atrophy or pigment clumping over time.Changes in electroretinogram （ERG） and persistent visual field defects were usually observed.Optical coherence tomography （OCT） showed that inner segment （IS）/outer segment （OS） regional abnormal changes corresponding to the visual field defect location area,at the same time the morphology and position of the visual field defect and multifocal electroretinogram （mfERG） shown in retinal amplitude density decreased regions coincide.The visual function of some patients can permanently damage.Fundus examination,mfERG and fluorescein angiography （FA）,indocyanine green angiography （ICGA）,autofluorescence （AF） and frequency domain OCT imaging techniques has an important significance for the diagnosis of AZOOR.Ever since the lack of understanding,AZOOR is often missed or misdiagnosed.In order to improve the understanding of the disease,this paper reviewed the definition,etiology,the epidemiological characteristics,clinical manifestation,classification,diagnosis,differential diagnosis,treatment and prognosis of AZOOR.