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Abstract
Purpose:
To report the prevalence and the characteristics of outer retinal tubulation (ORT) in Bietti’s crystalline dystrophy in 6 eyes of 3 patients.
Case summary:
Three patients with Bietti’s crystalline dystrophy were examined using color fundus photography, fundus auto-fluorescence picture, spectral domain optical coherence tomography (SD-OCT), fluorescein angiography, electroretinogram and electrooculogram. ORT was detected in 3 of 3 (100%) Bietti’s crystalline dystrophy patients. SD-OCT B-scan revealed hy-perreflective material inside the hyporeflective internal space with hyperreflective border. ORT was observed under the fovea or outside the fovea and on the outer nuclear layer in the retina. ORTs were no greater than 70 x 140 μ m in size when measured us-ing SD-OCT B-scan image.
References
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Figure 1.
(A) Color fundus photography of the right eye of a 63-years-old woman with outer retinal tubulation in Bietti’s crystalline dystrophy. It reveals multiple hyper-reflective granules. (B-1, B-2) Spectral domain OCT (C-scan) shows short or long pattern of outer retinal tabulation (arrow). (C) Infrared reflectance image shows crystal deposits as small spots with hyperfluorescence. (D) Spectral domain OCT (B-scan) shows circular and ovoid structures with hyper-reflective borders and contains hyper-reflective mate-rial within their lumen representing outer retinal tubulation. Arrow indicates outer retinal tubulation. OCT = optical coherence tomography.
Figure 2.
(A) Color fundus photography of the left eye of a 63-years-old woman with outer retinal tubulation in Bietti’s crystalline dystrophy. It reveals multiple hyper-reflective granules. (B-1, B-2) Spectral domain OCT (C-scan) shows 4-5 outer retinal tabulation. Arrows and circles indicate outer retinal tubulation. (C) Infrared reflectance image shows crystal deposits as small spots with hyperfluorescence. (D) Spectral domain OCT (B-scan) shows circular and ovoid structures with hyper-reflective borders and contains hyper-reflective material within their lumen representing outer retinal tabulation (arrows). OCT = optical coherence tomography.
Figure 3.
(A) Color fundus photography of the right eye of a 57-years-old man with outer retinal tubulation in Bietti’s crystalline dystrophy. It reveals multiple hyper-reflective granules. (B-1, B-2) Spectral domain OCT (C-scan) shows multiple outer retinal tubulation. Arrow indicates outer retinal tubulation. (C) Infrared reflectance image showed crystal deposits as small spots with hyperfluorescence. (D) Spectral domain OCT (B-scan) shows circular and ovoid structures with hyper-reflective borders and con-tains hyper-reflective material within their lumen representing outer retinal tabulation (arrow). OCT = optical coherence tomography.
Figure 4.
(A) Color fundus photography of the left eye of a 57-years-old man with outer retinal tubulation in Bietti’s crystalline dystrophy. It reveals multiple hyper-reflective granules. (B-1, B-2) Spectral domain OCT (C-scan) shows multiple outer retinal tab-ulation (arrow). (C) Infrared reflectance image shows crystal deposits as small spots with hyperfluorescence. (D) Spectral domain OCT (B-scan) shows circular and ovoid structures with hyper-reflective borders and contains hype-rreflective material within their lumen representing outer retinal tubulation. Arrow indicates outer retinal tubulation. OCT = optical coherence tomography.
Figure 5.
(A) Color fundus photography of the right eye of a 46-years-old woman with outer retinal tubulation in Bietti’s crystalline dystrophy. It reveals multiple hyper-reflective granules. (B-1, B-2) Spectral domain OCT (C-scan) shows short or long pattern of outer retinal tubulation. Circle indicates outer retinal tubulation (C) Infrared reflectance image shows crystal deposits as small spots with hyperfluorescence. (D) Spectral domain OCT (B-scan) shows circular and ovoid structures with hyper-reflective borders and contains hyper-reflective material within their lumen representing outer retinal tubulation. Arrows indicate outer retinal tubulation. OCT = optical coherence tomography.
Figure 6.
(A) Color fundus photography of the left eye of a 46-years-old woman with outer retinal tubulation in Bietti’s crystalline dystrophy. It reveals multiple hyper-reflective granules. (B-1, B-2) Spectral domain OCT (C-scan) shows short or long pattern of outer retinal tubulation. Circle indicates outer retinal tubulation (C) Infrared reflectance image shows crystal deposits as small spots with hyperfluorescence. (D) Spectral domain OCT (B-scan) shows circular and ovoid structures with hyper-reflective borders and contains hyper-reflective material within their lumen representing outer retinal tubulation. Arrows indicate outer retinal tubulation. OCT = optical coherence tomography.
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