Aleman Tomas S, Sandhu Harpal S, Serrano Leona W, Traband Anastasia, Lau Marisa K, Adamus Grazyna, Avery Robert A
Scheie Eye Institute at the Perelman Center for Advanced Medicine, University of Pennsylvania, Philadelphia2Division of Ophthalmology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania3Department of Ophthalmology, University of Pennsylvania, Philadelphia.
Scheie Eye Institute at the Perelman Center for Advanced Medicine, University of Pennsylvania, Philadelphia.
JAMA Ophthalmol. 2017 May 1;135(5):487-490. doi: 10.1001/jamaophthalmol.2017.0451.
The diagnostic path presented narrows down the cause of acute vision loss to the cone photoreceptor outer segment and will refocus the search for the cause of similar currently idiopathic conditions.
To describe the structural and functional associations found in a patient with acute zonal occult photoreceptor loss.
DESIGN, SETTING, AND PARTICIPANTS: A case report of an adolescent boy with acute visual field loss despite a normal fundus examination performed at a university teaching hospital.
Results of a complete ophthalmic examination, full-field flash electroretinography (ERG) and multifocal ERG, light-adapted achromatic and 2-color dark-adapted perimetry, and microperimetry. Imaging was performed with spectral-domain optical coherence tomography (SD-OCT), near-infrared (NIR) and short-wavelength (SW) fundus autofluorescence (FAF), and NIR reflectance (REF).
The patient was evaluated within a week of the onset of a scotoma in the nasal field of his left eye. Visual acuity was 20/20 OU, and color vision was normal in both eyes. Results of the fundus examination and of SW-FAF and NIR-FAF imaging were normal in both eyes, whereas NIR-REF imaging showed a region of hyporeflectance temporal to the fovea that corresponded with a dense relative scotoma noted on light-adapted static perimetry in the left eye. Loss in the photoreceptor outer segment detected by SD-OCT co-localized with an area of dense cone dysfunction detected on light-adapted perimetry and multifocal ERG but with near-normal rod-mediated vision according to results of 2-color dark-adapted perimetry. Full-field flash ERG findings were normal in both eyes. The outer nuclear layer and inner retinal thicknesses were normal.
Localized, isolated cone dysfunction may represent the earliest photoreceptor abnormality or a distinct entity within the acute zonal occult outer retinopathy complex. Acute zonal occult outer retinopathy should be considered in patients with acute vision loss and abnormalities on NIR-REF imaging, especially if multimodal imaging supports an intact retinal pigment epithelium and inner retina but an abnormal photoreceptor outer segment.
所呈现的诊断路径将急性视力丧失的原因缩小到视锥光感受器外段，并将重新聚焦对目前类似特发性病症病因的探寻。
描述在一名患有急性区域性隐匿性光感受器丧失患者中发现的结构和功能关联。
设计、背景和参与者：一名青少年男性患者的病例报告，该患者在大学教学医院进行眼底检查正常但存在急性视野缺损。
全面眼科检查、全视野闪光视网膜电图（ERG）和多焦ERG、明适应消色差和双色暗适应视野检查以及微视野检查的结果。使用光谱域光学相干断层扫描（SD - OCT）、近红外（NIR）和短波（SW）眼底自发荧光（FAF）以及近红外反射率（REF）进行成像。
患者在左眼鼻侧视野出现暗点后一周内接受评估。双眼视力均为20/20，双眼色觉正常。双眼眼底检查以及SW - FAF和NIR - FAF成像结果均正常，而NIR - REF成像显示在黄斑颞侧有一个低反射区域，与左眼明适应静态视野检查中发现的致密相对性暗点相对应。SD - OCT检测到的光感受器外段损失与明适应视野检查和多焦ERG检测到的致密视锥功能障碍区域共定位，但根据双色暗适应视野检查结果，杆介导的视力接近正常。双眼全视野闪光ERG结果正常。外核层和视网膜内层厚度正常。
局限性、孤立性视锥功能障碍可能代表急性区域性隐匿性外层视网膜病变复合体中最早的光感受器异常或一种独特的病症。对于急性视力丧失且NIR - REF成像异常的患者，应考虑急性区域性隐匿性外层视网膜病变，特别是如果多模态成像显示视网膜色素上皮和视网膜内层完整但光感受器外段异常。
