Mrejen Sarah, Khan Samira, Gallego-Pinazo Roberto, Jampol Lee M, Yannuzzi Lawrence A
LuEsther T. Mertz Retinal Research Center, New York, New York2Vitreous Retina Macula Consultants of New York, New York, New York3Department of Ophthalmology, New York University School of Medicine, New York, New York.
Department of Ophthalmology, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
JAMA Ophthalmol. 2014 Sep;132(9):1089-98. doi: 10.1001/jamaophthalmol.2014.1683.
We describe the multimodal imaging in a group of patients showing a distinct clinical entity that best represents acute zonal occult outer retinopathy (AZOOR).
To propose a classification of AZOOR based on clinical fundus and multimodal imaging.
DESIGN, SETTING AND PARTICIPANTS: A retrospective review of patients diagnosed as having AZOOR at 2 centers. After reviewing more than 400 cases diagnosed or referred to us as AZOOR or AZOOR complex, we assembled 30 cases that fit our current definition; (48 eyes) with a median age at diagnosis of 47 years (age range, 17-86 years) and a mean follow-up period of 39 months. Twenty patients were female. Eighteen patients had initially been seen with bilateral lesions, mostly asymmetric (4 cases were symmetric). Most patients had no remarkable medical or ocular history. The median visual acuity at the time of presentation was 20/25 (range, 20/20 to 20/400).
Multimodal imaging, including fundus photography, fluorescein and indocyanine green angiography, fundus autofluorescence imaging, and corresponding eye-tracked spectral-domain coherence tomography imaging.
Each patient was initially seen with visual symptoms of photopsia and scotoma, and most had a detectable lesion in the fundus evident clinically or detected on multimodal imaging. The clinical appearance of the AZOOR lesions varied depending on their duration and location, but some features were characteristic, including a demarcating line of the progression at the level of the outer retina and a trizonal pattern of sequential involvement of the outer retina, retinal pigment epithelium, and choroid, as well as frequent zonal progression. Advanced cases of AZOOR demonstrated disruption of the inner and outer retina and severe damage or loss of the retinal pigment epithelium and the choroid.
A specific definition of AZOOR based on multimodal imaging is proposed to help physicians distinguish it from other diseases of the posterior fundus, including white spot syndromes and autoimmune, hereditary, paraneoplastic, toxic, and other inflammatory retinopathies.
我们描述了一组患者的多模态成像，这些患者表现出一种独特的临床实体，最能代表急性区域性隐匿性外层视网膜病变（AZOOR）。
基于临床眼底和多模态成像提出AZOOR的分类。
设计、地点和参与者：对2个中心诊断为AZOOR的患者进行回顾性研究。在回顾了400多例诊断为或转诊至我们这里的AZOOR或AZOOR综合征病例后，我们选取了30例符合我们当前定义的病例；（48只眼）诊断时的中位年龄为47岁（年龄范围17 - 86岁），平均随访期为39个月。20例为女性。18例患者最初表现为双侧病变，大多不对称（4例对称）。大多数患者无明显的内科或眼科病史。就诊时的中位视力为20/25（范围20/20至20/400）。
多模态成像，包括眼底照相、荧光素和吲哚菁绿血管造影、眼底自发荧光成像以及相应的眼跟踪光谱域相干断层扫描成像。
每位患者最初均有闪光感和暗点等视觉症状，大多数患者在眼底有可检测到的病变，临床上明显或在多模态成像中检测到。AZOOR病变的临床表现因其持续时间和位置而异，但一些特征具有特异性，包括外层视网膜水平的进展分界线、外层视网膜、视网膜色素上皮和脉络膜依次受累的三区模式，以及频繁的区域进展。AZOOR的晚期病例显示内外层视网膜破坏，视网膜色素上皮和脉络膜严重受损或丧失。
基于多模态成像提出了AZOOR的具体定义，以帮助医生将其与眼底后部的其他疾病区分开来，包括白点综合征以及自身免疫性、遗传性、副肿瘤性、中毒性和其他炎症性视网膜病变。
