Nitta Keisuke, Mukai Ryo, Akiyama Hideo
Department of Ophthalmology, Gunma University Graduate School of Medicine, 3-39-15 Showa-machi, Maebashi, Gunma, Japan.
Am J Ophthalmol Case Rep. 2022 Jan 20;25:101277. doi: 10.1016/j.ajoc.2022.101277. eCollection 2022 Mar.
To report an early stage of gyrate atrophy (GA) of the retina and choroid for which spectral-domain optical coherence tomography (SD-OCT) images revealed an obscured interdigitation zone (IZ).
A 13-year-old boy was referred to our department due to blurred vision in his left eye. Best corrected visual acuity was 20/20 and 20/25 in the right and left eye, respectively. Fundus examination revealed scalloped atrophic peripheral chorioretinal lesions in both eyes. Concentrations of plasma and urine ornithine were 1192 nmol/mL and 1930 μmol/g·cre, respectively. Consequently, he was diagnosed with GA. Although Goldmann perimetry found no abnormalities, electroretinogram (ERG) revealed loss of the rod responses and significant attenuation of the cone responses in both eyes. Detailed analysis of the posterior part of the fundus using multifocal electroretinogram showed poor responses, specifically in the nasal macular area of the left eye. SD-OCT showed an obscured IZ corresponding to the attenuated cone response determined by ERG. There was preservation of the retinal pigment epithelium, ellipsoid zone and external limiting membrane.
Obscured IZ during early stage GA was confirmed in a teenage patient with a chief complaint of blurred vision. OCT is useful in the detection of minute morphological changes that occur earlier in GA.
报告视网膜和脉络膜回旋状萎缩（GA）的早期阶段，其光谱域光学相干断层扫描（SD - OCT）图像显示指状交叉区（IZ）模糊。
一名13岁男孩因左眼视力模糊转诊至我科。右眼和左眼的最佳矫正视力分别为20/20和20/25。眼底检查发现双眼周边脉络膜视网膜呈扇形萎缩性病变。血浆和尿液鸟氨酸浓度分别为1192 nmol/mL和1930 μmol/g·cre。因此，他被诊断为GA。尽管Goldmann视野检查未发现异常，但视网膜电图（ERG）显示双眼杆体反应丧失，锥体反应明显减弱。使用多焦视网膜电图对眼底后部进行详细分析显示反应不佳，特别是在左眼鼻侧黄斑区。SD - OCT显示与ERG确定的锥体反应减弱相对应的IZ模糊。视网膜色素上皮、椭圆体带和外界膜保存完好。
一名以视力模糊为主诉的青少年患者在GA早期阶段出现IZ模糊得到证实。OCT有助于检测GA中更早出现的微小形态变化。
