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

Kim, Yong Il; Kim, In Taek; Kim, Jin Seon

doi:2015.56.7.1141

J Korean Ophthalmol Soc > Volume 56(7); 2015 > Article

Case Report

Journal of the Korean Ophthalmological Society 2015;56(7):1141-1148.
DOI: https://doi.org/10.3341/jkos.2015.56.7.1141 Published online July 15, 2015.

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

Yong Il Kim, In Taek Kim, Jin Seon Kim

¹Cheil Eye Hospital, Daegu, Korea. ppjinsun25@hanmail.net
²Department of Ophthalmology, Kyungpook National University School of Medicine, Daegu, Korea.

비에띠결정색소망막이영양증에서 나타난 바깥 망막 관상화 3예

김용일¹⋅김인택²⋅김진선¹

제일안과병원¹, 경북대학교 의학전문대학원 안과학교실²

Received: 13 December 2014 • Revised: 26 January 2015 • Accepted: 26 May 2015

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.

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