Ibironke Josephine O, Gurwood Andrew S
The Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA.
Optometry. 2010 Jan;81(1):22-7. doi: 10.1016/j.optm.2009.07.013.
Acute zonal occult outer retinopathy (AZOOR) is a disorder of unknown etiology with a predilection for young women. AZOOR is characterized by an acute loss of one or more zones of outer retinal function with a corresponding loss of visual field in one or both eyes. Patients present with photopsia, variable funduscopic changes, and abnormal electroretinogram (ERG) findings. There are no proven treatments. Diagnosed cases have a reasonable prognosis because central vision is often spared.
A 24-year-old white woman presented with acute photopsia with clouded temporal vision in the right eye (O.D.). Initial evaluation found a slightly swollen nerve fiber layer with no other outstanding lesions O.D. Over a 7-week follow-up period, there was significant progression into the deep chorioretinal tissues O.D. with an anterior chamber response. At 3 months, an afferent pupillary defect (APD) developed with significant retinal pigment epithelium (RPE) changes. After extensive testing, the consulting retinal specialist identified AZOOR as the etiology. At 21 and 31 months, the process remained in remission. Although primary symptoms subsided, the dense inferior-temporal field defect remained O.D.
AZOOR is a rare condition with subtle and often vague signs and symptoms making diagnosis difficult. The course of AZOOR, its clinical presentations, hypothesis of etiology, differential diagnosis, workup, management, and prognosis are reviewed.
急性区域性隐匿性外层视网膜病变（AZOOR）是一种病因不明的疾病，好发于年轻女性。AZOOR的特征是外视网膜功能的一个或多个区域急性丧失，伴有一只或两只眼睛相应的视野缺损。患者表现为闪光感、眼底改变多样以及视网膜电图（ERG）检查结果异常。目前尚无经证实的治疗方法。已确诊的病例预后较好，因为中心视力通常不受影响。
一名24岁白人女性因右眼（OD）急性闪光感伴颞侧视力模糊就诊。初始评估发现右眼神经纤维层轻度肿胀，无其他明显病变。在7周的随访期内，右眼病变显著进展至脉络膜视网膜深层组织，并伴有前房反应。3个月时，出现传入性瞳孔障碍（APD），视网膜色素上皮（RPE）有明显变化。经过广泛检查，会诊的视网膜专家确定病因是AZOOR。在21个月和31个月时，病情仍处于缓解期。虽然主要症状消退，但右眼致密的颞下视野缺损仍然存在。
AZOOR是一种罕见疾病，其体征和症状细微且常常模糊，难以诊断。本文对AZOOR的病程、临床表现、病因假说、鉴别诊断、检查、治疗及预后进行了综述。
