遗传性视网膜变性疾病中的外网状层囊腔。
Outer retinal tubulation in inherited retinal degenerative disease.
机构信息
Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan.
出版信息
Retina. 2013 Jul-Aug;33(7):1462-5. doi: 10.1097/IAE.0b013e31828221ae.
PURPOSE
To investigate the prevalence and characteristics of outer retinal tubulation (ORT) seen in inherited retinal degenerative diseases.
METHODS
A total of 354 eyes of 177 patients were examined with spectral domain optical coherence tomography. One hundred and twelve patients had retinitis pigmentosa, 58 patients had cone dystrophy, and 7 patients had the Bietti crystalline dystrophy. The images obtained by horizontal and vertical scans were analyzed to explore the possible presence of ORT, estimate their prevalence, morphologic character, and their location in the retinal layers.
RESULTS
With spectral domain optical coherence tomography, ORT was identified in 0 of 112 patients with retinitis pigmentosa, unilaterally in 3 of 58 patients with cone dystrophy, and bilaterally in 5 of 7 patients with the Bietti crystalline dystrophy. Outer retinal tubulation was detected under the fovea, and in the outer nuclear layer, ORT was detected in the Bietti crystalline dystrophy with a significantly higher frequency than in cone dystrophy (P < 0.001).
CONCLUSION
There was a higher rate of ORT in the Bietti crystalline dystrophy among inherited retinal degenerative diseases.
目的
研究遗传性视网膜变性疾病中外层视网膜管腔（ORT）的发生率和特征。
方法
对 177 例患者的 354 只眼进行了光谱域光相干断层扫描检查。其中 112 例为色素性视网膜炎，58 例为 Cone 营养不良，7 例为 Bietti 结晶状营养不良。分析水平和垂直扫描获得的图像，以探讨 ORT 的可能存在，估计其发生率、形态特征及其在视网膜各层中的位置。
结果
应用光谱域光相干断层扫描技术，在 112 例色素性视网膜炎患者中，0 例单侧出现 ORT，58 例 Cone 营养不良患者中，3 例单侧出现 ORT，7 例 Bietti 结晶状营养不良患者中，5 例双侧出现 ORT。在黄斑下和外核层中均可检测到 ORT，Bietti 结晶状营养不良中 ORT 的检出率明显高于 Cone 营养不良（P<0.001）。
结论
在遗传性视网膜变性疾病中，Bietti 结晶状营养不良的 ORT 发生率较高。
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