TY - JOUR
T1 - CHANGES of CONE PHOTORECEPTOR MOSAIC in AUTOSOMAL RECESSIVE BESTROPHINOPATHY
AU - Nakanishi, Ayami
AU - Ueno, Shinji
AU - Hayashi, Takaaki
AU - Katagiri, Satoshi
AU - Ito, Yasuki
AU - Kominami, Taro
AU - Fujinami, Kaoru
AU - Tsunoda, Kazushige
AU - Iwata, Takeshi
AU - Terasaki, Hiroko
N1 - Publisher Copyright:
Copyright © by Ophthalmic Communications Society, Inc. Unauthorized reproduction of this article is prohibited.
PY - 2020/1/1
Y1 - 2020/1/1
N2 - Purpose:To assess the morphological changes of cone photoreceptors in eyes with autosomal recessive bestrophinopathy.Methods:Both eyes of five patients with autosomal recessive bestrophinopathyunderwent spectral domain optical coherence tomography and adaptive optics fundus imaging. The cone photoreceptor densities were measured at intervals of 100 m between 500 m nasal and temporal eccentricities from the foveal center.Results:The median age of the patients was 30 years (range, 23-45 years), and the best-corrected visual acuity ranged from 20/20 to 20/80. Adaptive optics fundus images showed reduced cone photoreceptor densities corresponding to the damages of the photoreceptor layer in the spectral domain optical coherence tomography images in four patients with relatively good best-corrected visual acuity. The cone photoreceptor densities at the center of the fovea were less than one-third of the normal cone densities (range 11,600-30,400 cells/mm2). Cone photoreceptor mosaics were visible over the lesions with serous retinal detachment and retinal edema, although they were partially hyporeflective.Conclusion:There is a significant cone photoreceptor loss in the macular region of patients with autosomal recessive bestrophinopathy, although they had relatively good visual acuity. Monitoring cone photoreceptors by adaptive optics fundus imaging should provide accurate assessments of the disease status and indications for future therapeutic interventions.
AB - Purpose:To assess the morphological changes of cone photoreceptors in eyes with autosomal recessive bestrophinopathy.Methods:Both eyes of five patients with autosomal recessive bestrophinopathyunderwent spectral domain optical coherence tomography and adaptive optics fundus imaging. The cone photoreceptor densities were measured at intervals of 100 m between 500 m nasal and temporal eccentricities from the foveal center.Results:The median age of the patients was 30 years (range, 23-45 years), and the best-corrected visual acuity ranged from 20/20 to 20/80. Adaptive optics fundus images showed reduced cone photoreceptor densities corresponding to the damages of the photoreceptor layer in the spectral domain optical coherence tomography images in four patients with relatively good best-corrected visual acuity. The cone photoreceptor densities at the center of the fovea were less than one-third of the normal cone densities (range 11,600-30,400 cells/mm2). Cone photoreceptor mosaics were visible over the lesions with serous retinal detachment and retinal edema, although they were partially hyporeflective.Conclusion:There is a significant cone photoreceptor loss in the macular region of patients with autosomal recessive bestrophinopathy, although they had relatively good visual acuity. Monitoring cone photoreceptors by adaptive optics fundus imaging should provide accurate assessments of the disease status and indications for future therapeutic interventions.
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U2 - 10.1097/IAE.0000000000002363
DO - 10.1097/IAE.0000000000002363
M3 - Article
C2 - 30308565
AN - SCOPUS:85077025369
SN - 0275-004X
VL - 40
SP - 181
EP - 186
JO - Retina
JF - Retina
IS - 1
ER -