Journal List > J Korean Ophthalmol Soc > v.56(7) > 1010040

J Korean Ophthalmol Soc. 2015 Jul;56(7):1141-1148. Korean.
Published online July 16, 2015.  https://doi.org/10.3341/jkos.2015.56.7.1141
©2015 The Korean Ophthalmological Society
Three Cases of Outer Retinal Tubulation in Bietti's Crystalline Dystrophy
Yong Il Kim, MD,1 In Taek Kim, MD, PhD,2 and Jin Seon Kim, MD1
1Cheil Eye Hospital, Daegu, Korea.
2Department of Ophthalmology, Kyungpook National University School of Medicine, Daegu, Korea.

Address reprint requests to Jin Seon Kim, MD. Cheil Eye Hospital, #1 Ayang-ro, Dong-gu, Daegu 701-820, Korea. Tel: 82-53-959-1751, Fax: 82-53-959-1758, Email: ppjinsun25@hanmail.net
Received December 13, 2014; Revised January 26, 2015; Accepted May 26, 2015.

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.


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 autofluorescence 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 hyperreflective 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 × 140 µm in size when measured using SD-OCT B-scan image.

Conclusions

ORT was observed frequently in patients with Bietti's crystalline dystrophy. These findings can potentially predict the extensive photoreceptor abnormalities in retinal pigment epithelial atrophy.

Keywords: Bietti's crystalline dystrophy; Outer retinal tubulation; Spectral domain optical coherence tomography

Figures


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 material within their lumen representing outer retinal tubulation. Arrow indicates outer retinal tubulation. OCT = optical coherence tomography.
Click for larger image


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.
Click for larger image


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 contains hyper-reflective material within their lumen representing outer retinal tabulation (arrow). OCT = optical coherence tomography.
Click for larger image


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 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 hype-rreflective material within their lumen representing outer retinal tubulation. Arrow indicates outer retinal tubulation. OCT = optical coherence tomography.
Click for larger image


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.
Click for larger image


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.
Click for larger image

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