Dataset Information

Predicting visual acuity in Bietti crystalline dystrophy: evaluation of image parameters.

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.

SUBMITTER: Huang CY

PROVIDER: S-EPMC7860191 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Predicting visual acuity in Bietti crystalline dystrophy: evaluation of image parameters.

Huang Chu-Yen CY Kang Eugene Yu-Chuan EY Yeh Lung-Kun LK Wu An-Lun AL Liu Pei-Kang PK Huang I-Wen IW Ryu Joseph J Liu Laura L Wu Wei-Chi WC Lai Chi-Chun CC Chen Kuan-Jen KJ Wang Nan-Kai NK

BMC ophthalmology 20210204 1

<h4>Background</h4>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).<h4>Methods</h4>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 autofluor ...[more]

PMID: 33541298

Similar Datasets

Project description:BACKGROUND:Bietti crystalline dystrophy (BCD) is a distinct entity of retinitis pigmentosa with a wide range of genotypic and phenotypic variabilities. The goal of the present study was to investigate the morphological, functional and genetic features of BCD. METHODS:A full series of multimodal imaging was performed in four Chinese patients with BCD, including fundus photography, fundus autofluorescence, fundus fluorescein angiography (FFA), indocyanine green (ICG) angiography, optical coherence tomography (OCT) and microperimetry. Electrophysiological tests including full-field electroretinography (ERG) and multifocal ERG were employed. CYP4V2 gene sequencing was performed. RESULTS:Intraretinal crystalline deposits were observed in fundus photographs in all patients. The crystals were better appreciated in infrared images. Autofluorescence imaging demonstrated multifocal patchy hypofluorescence, suggesting massive RPE atrophy. FFA and ICG angiography further confirmed atrophy of the RPE and the underlying choroidal vessels. OCT revealed disruption of the photoreceptors, RPE and the choroid. Outer retinal tubulations (ORTs) confining to the outer nuclear layer were detected in three out of four patients. Full-field ERG showed markedly diminished responses. Multifocal ERG displayed reduced central and peripheral responses in a patient with normal vision. Gene sequencing identified two deletion mutations in CYP4V2, c.802_807del and c.810delT. BCD complicated by choroidal neovascularization (CNV) was diagnosed in one patient, and intravitreal anti-vascular endothelial growth factor (VEGF) injection was given with favorable response. CONCLUSIONS:Multimodal imaging features and electrophysiological findings of BCD patients were comprehensively discussed. A novel deletion mutation, c.802_807del, in the CYP4V2 gene was reported. ORTs are important changes in the outer retina of BCD patients, further investigation of this structure may provide insights into pathology of BCD. Intravitreal anti-VEGF therapy was effective for treatment of BCD complicated by CNV.

Dataset Information

Predicting visual acuity in Bietti crystalline dystrophy: evaluation of image parameters.

Background

Methods

Results

Conclusion

Publications

Predicting visual acuity in Bietti crystalline dystrophy: evaluation of image parameters.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets