Outer retinal circular structures in patients with Bietti crystalline retinopathy
Outer retinal circular structures in patients with Bietti crystalline retinopathy
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DOI：
10.1136/bjo.2010.199356
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发表时间：
2012-03-01
影响因子：
4.1
通讯作者：
Yoshimura, Nagahisa
中科院分区：
文献类型：
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作者：
Kojima, Hiroshi;Otani, Atsushi;Yoshimura, Nagahisa
Bietti结晶性视网膜病变（BCR）是一种独特的视网膜退行性疾病，其特征是视网膜变性，主要位于后极区有许多黄白色晶体。采用光谱域光学相干断层扫描（SD-OCT）研究视网膜的结构变化。方法选取诊断为BCR （n = 12）、视网膜色素变性（RP, n = 292）和视锥细胞营养不良（n = 16）的患者进行研究。作者主要检查了这些患者的眼底照片和SD-OCT、红外和眼底自身荧光图像。结果视网膜色素上皮缺乏斑片状变性病变的部分可见结晶沉积。SD-OCT显示，大多数晶体沉积位于视网膜色素上皮层内侧附近。最常观察到的变化是外层核层的圆形超折射结构。虽然这些结构被认为是先前报道的“管状形成”或“管状变性”，但我们确定许多这些圆形结构是球形结构的切片，并且通常在怀疑正在进行的活动性变性的区域中被注意到。结论BCR外核层具有特征性结构。虽然这种结构的发生率各不相同，但它可能是视网膜变性的特征，可以在许多视网膜退行性疾病中发现。
Background Bietti crystalline retinopathy (BCR) is a distinct retinal degenerative disease characterised by retinal degeneration with many yellow-white crystals located mainly at the posterior pole area. Using spectral domain-optical coherence tomography (SD-OCT), the structural change in retina was investigated.Methods Patients diagnosed with BCR (n = 12), retinitis pigmentosa (RP, n = 292) and cone dystrophy (n = 16) were included in this study. The authors mainly examined fundus photographs and SD-OCT, infrared and fundus autofluorescence images of these patients.Results Crystalline deposits were detected in portions of the retinal pigment epithelium that lacked patchy degenerated lesions. SD-OCT revealed that most of the observed crystalline deposits were located adjacent to the inner side of retinal pigment epithelium layer. The change most frequently observed was circular hyper-refractive structures in the outer nuclear layer. Although the structures were considered to be previously reported "tubular formation" or "tubular degeneration", we determined that many of these circular structures were slices of spherical structures and were typically noted in areas suspected of ongoing active degeneration.Conclusion BCR has characteristic structures in the outer nuclear layer. Although the incidence of the structure varies, it may be characteristic of retinal degeneration and can be found in many retinal degenerative diseases.