Chu-Yen Huang1,2, Eugene Yu-Chuan Kang1,2, Lung-Kun Yeh1,2, An-Lun Wu3, Pei-Kang Liu4,5,6,7, I-Wen Huang4, Joseph Ryu4, Laura Liu1,2, Wei-Chi Wu1,2, Chi-Chun Lai1,2,8, Kuan-Jen Chen9,10, Nan-Kai Wang11. 1. Department of Ophthalmology, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, Taiwan. 2. College of Medicine, Chang Gung University, Taoyuan, Taiwan. 3. Department of Ophthalmology, Mackay Memorial Hospital, Hsinchu, Taiwan. 4. Department of Ophthalmology, Edward S. Harkness Eye Institute, Columbia University Irving Medical Center, Columbia University, New York, USA. 5. Department of Ophthalmology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan. 6. Department of Ophthalmology, School of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan. 7. Institute of Biochemical Science, National Sun Yat-Sen University, Kaohsiung, Taiwan. 8. Department of Ophthalmology, Chang Gung Memorial Hospital, Keelung, Taiwan. 9. Department of Ophthalmology, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, Taiwan. cgr999chiayi@yahoo.com.tw. 10. College of Medicine, Chang Gung University, Taoyuan, Taiwan. cgr999chiayi@yahoo.com.tw. 11. Department of Ophthalmology, Edward S. Harkness Eye Institute, Columbia University Irving Medical Center, Columbia University, New York, USA. wang.nankai@gmail.com.
Abstract
BACKGROUND: To analyze multiple imaging modalities in patients with Bietti crystalline dystrophy (BCD) and to investigate which factors from these modalities are associated with best corrected visual acuity (BCVA). METHODS: In this retrospective study, 40 eyes from 22 patients with BCD were included and were separated into group 1 (BCVA ≤20/200) and group 2 (BCVA > 20/200). Data including BCVA and characteristic findings from near-infrared reflectance (NIR) imaging, fundus autofluorescence (FAF), and spectral domain-optic coherence tomography (SD-OCT) were analyzed and compared. The outcome measures of multimodal imaging were evaluated for correlation with BCVA. RESULTS: NIR is a good diagnostic tool for detecting either crystalline or sclerotic vessels in BCD. Patients in group 1 tended to have a thinner choroid (P = 0.047) with ellipsoid zone (EZ) disruption (P = 0.011). Calculation of the area under the curve indicated that EZ disruption detected on SD-OCT could be a good predictor of legal blindness in BCD. CONCLUSION: For the diagnosis of BCD, NIR could be a good diagnostic tool. Of the studied imaging modalities, we found that EZ disruption at the fovea were strongly associated with legal blindness, which could be easily assessed by SD-OCT.
BACKGROUND: To analyze multiple imaging modalities in patients with Bietti crystalline dystrophy (BCD) and to investigate which factors from these modalities are associated with best corrected visual acuity (BCVA). METHODS: In this retrospective study, 40 eyes from 22 patients with BCD were included and were separated into group 1 (BCVA ≤20/200) and group 2 (BCVA > 20/200). Data including BCVA and characteristic findings from near-infrared reflectance (NIR) imaging, fundus autofluorescence (FAF), and spectral domain-optic coherence tomography (SD-OCT) were analyzed and compared. The outcome measures of multimodal imaging were evaluated for correlation with BCVA. RESULTS: NIR is a good diagnostic tool for detecting either crystalline or sclerotic vessels in BCD. Patients in group 1 tended to have a thinner choroid (P = 0.047) with ellipsoid zone (EZ) disruption (P = 0.011). Calculation of the area under the curve indicated that EZ disruption detected on SD-OCT could be a good predictor of legal blindness in BCD. CONCLUSION: For the diagnosis of BCD, NIR could be a good diagnostic tool. Of the studied imaging modalities, we found that EZ disruption at the fovea were strongly associated with legal blindness, which could be easily assessed by SD-OCT.
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