Baek Jisun, Lee Ho-Kyung, Kim Ungsoo Samuel
Department of Ophthalmology, Kim's Eye Hospital, Konyang University College of Medicine, Youngdeungpo 4th 156, Youngdeungpo-gu, Seoul 150-034, Korea.
Doc Ophthalmol. 2013 Jun;126(3):247-51. doi: 10.1007/s10633-013-9377-y. Epub 2013 Mar 2.
To report spectral domain optical coherence (OCT) tomography findings in a case of bilateral peripheral cone dystrophy.
A 21-year-old man complained of blurred visual fields on the lateral sides of both eyes. The patient's best-corrected visual acuity was 20/20 in both eyes. Fundus examination revealed mild temporal pallor, while fluorescein angiography did not show any abnormalities. Humphrey's visual field revealed a C-shaped scotoma. Photopic electroretinogram (ERG) and 30-Hz flicker revealed decreased cone function; however, scotopic ERG noted a normal response. Multifocal ERG revealed a relatively well-preserved macular area but with reduced amplitude in the peripheral areas of both eyes. On OCT, the photoreceptor inner and outer segment junction and retinal pigment epithelium (RPE) layers were well preserved in both eyes, except for a slight decrease in outer layer thickness. Moreover, on the macular thickness map obtained from Cirrus HD-OCT, a reduction in internal limiting membrane-RPE thickness that correlated well with visual field defects was revealed in both eyes. On the retinal nerve fiber layer (RNFL) thickness map obtained from Cirrus HD-OCT, the superior quadrant showed decreased RNFL thickness; however, thickness in the temporal quadrant corresponded with thickness in the macular area and was normal in both eyes.
The OCT of a patient with peripheral cone dystrophy revealed decreased thickness of the macula with well-preserved retinal structures, which may be one of the distinctive features of this condition.
报告一例双侧周边视锥细胞营养不良的光谱域光学相干断层扫描(OCT)结果。
一名21岁男性主诉双眼外侧视野模糊。患者双眼最佳矫正视力均为20/20。眼底检查显示颞侧轻度苍白,而荧光素血管造影未显示任何异常。Humphrey视野检查显示为C形暗点。明视视网膜电图(ERG)和30Hz闪烁检查显示视锥细胞功能下降;然而,暗视ERG显示反应正常。多焦ERG显示黄斑区相对保存良好,但双眼周边区域振幅降低。在OCT上,双眼光感受器内、外节连接及视网膜色素上皮(RPE)层保存良好,只是外层厚度略有降低。此外,在Cirrus HD-OCT获得的黄斑厚度图上,双眼均显示与视野缺损密切相关的内界膜-RPE厚度降低。在Cirrus HD-OCT获得的视网膜神经纤维层(RNFL)厚度图上,上象限显示RNFL厚度降低;然而,颞侧象限厚度与黄斑区厚度相当,双眼均正常。
周边视锥细胞营养不良患者的OCT显示黄斑厚度降低,视网膜结构保存良好,这可能是该疾病的独特特征之一。