J Korean Ophthalmol Soc.  2015 Jul;56(7):1141-1148. 10.3341/jkos.2015.56.7.1141.

Three Cases of Outer Retinal Tubulation in Bietti's Crystalline Dystrophy

• Kim YI
• Kim IT
• Kim JS

• KMID: 2148818
• DOI: http://doi.org/10.3341/jkos.2015.56.7.1141

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 x 140 microm 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.

Keyword

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

MeSH Terms

Atrophy
Crystallins*
Electrooculography
Fluorescein Angiography
Humans
Photography
Prevalence
Retina
Retinaldehyde*
Tomography, Optical Coherence
Crystallins
Retinaldehyde

Figure

• 

  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.

Reference

References

1. Kojima H, Otani A, Ogino K, et al. Outer retinal circular structures in patients with Bietti crystalline retinopathy. Br J Ophthalmol. 2012; 96:390–3.

2. Zweifel SA, Engelbert M, Laud K, et al. Outer retinal tubulation: a novel optical coherence tomography finding. Arch Ophthalmol. 2009; 127:1596–602.

3. Iriyama A, Aihara Y, Yanagi Y. Outer retinal tubulation in inherited retinal degenerative disease. Retina. 2013; 33:1462–5.

4. Goldberg NR, Greenberg JP, Laud K, et al. Outer retinal tubulation in degenerative retinal disorders. Retina. 2013; 33:1871–6.

5. Gallego-Pinazo R, Marsiglia M, Mrejen S, Yannuzzi LA. Outer ret-inal tubulations in chronic central serous chorioretinopathy. Graefes Arch Clin Exp Ophthalmol. 2013; 251:1655–6.

6. Dolz-Marco R, Gallego-Pinazo R, Pinazo-Duran MD, et al. Outer retinal tubulation analysis in cases of macular dystrophy. Arch Soc Esp Oftalmol. 2013; 88:161–2.

7. Wolff B, Maftouhi MQ, Mateo-Montoya A, et al. Outer retinal cysts in age-related macular degeneration. Acta Ophthalmol. 2011; 89:e496–9.

8. Yannuzzi LA. The Retinal Atlas. 1st. New York: Elsevier;2010. p. 158–60.

9. Ellabban AA, Hangai M, Yamashiro K, et al. Tomographic fundus features in pseudoxanthoma elasticum: comparison with neo-vascular age-related macular degeneration in Japanese patients. Eye (Lond). 2012; 26:1086–94.

10. Sergouniotis PI, Davidson AE, Lenassi E, et al. Retinal structure, function, and molecular pathologic features in gyrate atrophy. Ophthalmology. 2012; 119:596–605.

11. Lima LH, Burke T, Greenstein VC, et al. Progressive constriction of the hyperautofluorescent ring in retinitis pigmentosa. Am J Ophthalmol. 2012; 153:718–27.

12. Jung JJ, Freund KB. Long-term follow-up of outer retinal tubula-tion documented by eye-tracked and en face spectral-domain opti-cal coherence tomography. Arch Ophthalmol. 2012; 130:1618–9.