Dataset Information

QUANTITATIVE ANALYSIS OF LONGITUDINAL CHANGES IN MULTIMODAL IMAGING OF LATE-ONSET RETINAL DEGENERATION.

ABSTRACT:

Purpose

To quantitatively analyze clinically relevant features on longitudinal multimodal imaging of late-onset retinal degeneration to characterize disease progression.

Methods

Fundus autofluorescence (FAF), infrared reflectance, and optical coherence tomography imaging of 4 patients with late-onset retinal degeneration were acquired over 3 to 15 years (20 visits total). Corresponding regions of interest were analyzed on FAF (reticular pseudodrusen [RPD], "speckled FAF," and chorioretinal atrophy) and infrared reflectance (hyporeflective RPD and target RPD) using quantitative measurements, including contour area, distance to fovea, contour overlap, retinal thickness, and texture features.

Results

Cross-sectional analysis revealed a moderate correlation (RPD FAF ∩ RPD infrared reflectance = 63%) between contour area across modalities. Quantification of retinal thickness and texture analysis of areas contoured on FAF objectively differentiated the contour types. A longitudinal analysis of aligned images demonstrates that the contoured region of atrophy both encroaches toward the fovea and grows monotonically with a rate of 0.531 mm/year to 1.969 mm/year (square root of area, n = 5 eyes). A retrospective analysis of precursor lesions of atrophy reveals quantifiable progression from RPD to speckled FAF to atrophy.

Conclusion

Image analysis of time points before the development of atrophy reveals consistent patterns over time and space in late-onset retinal degeneration that may provide useful outcomes for this and other degenerative retinal diseases.

SUBMITTER: Vanderford EK

PROVIDER: S-EPMC8279727 | biostudies-literature | 2021 Aug

REPOSITORIES: biostudies-literature

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Publications

QUANTITATIVE ANALYSIS OF LONGITUDINAL CHANGES IN MULTIMODAL IMAGING OF LATE-ONSET RETINAL DEGENERATION.

Vanderford Elliott K EK De Silva Tharindu T Noriega Dominique D Arango Mike M Cunningham Denise D Cukras Catherine A CA

Retina (Philadelphia, Pa.) 20210801 8

<h4>Purpose</h4>To quantitatively analyze clinically relevant features on longitudinal multimodal imaging of late-onset retinal degeneration to characterize disease progression.<h4>Methods</h4>Fundus autofluorescence (FAF), infrared reflectance, and optical coherence tomography imaging of 4 patients with late-onset retinal degeneration were acquired over 3 to 15 years (20 visits total). Corresponding regions of interest were analyzed on FAF (reticular pseudodrusen [RPD], "speckled FAF," and chor ...[more]

PMID: 33332808

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Project description:PurposeThe purpose of this study was to present our findings on the natural history of late-onset retinal degeneration (LORD) in patients with molecularly confirmed C1QTNF5 heterozygous pathogenic variants and assess suitability of retinal structure parameters for disease monitoring.MethodsSixteen patients with C1QTNF5-LORD were retrospectively identified from Moorfields Eye Hospital, UK. Fundus autofluorescence (FAF), optical coherence tomography (OCT) scans, and best-corrected visual acuity (BCVA) were collected. Area of atrophy (AA) was manually drawn in FAF images. Ellipsoid zone (EZ) width and foveal retinal thickness of the whole retina and outer retina were extracted from OCT scans. Age-related changes were tested with linear-mixed models.ResultsPatients had median age of 62.3 years (interquartile range [IQR] = 58.8-65.4 years) at baseline, and median follow-up of 5.1 years (IQR = 2.6-7.6 years). AA, EZ width, and retinal thickness parameters remained unchanged until age 50 years, but showed significant change with age thereafter (all P < 0.0001). AA and EZ width progressed rapidly (dynamic range normalized rates = 4.3-4.5%/year) from age 53.9 and 50.8 years (estimated inflection points), respectively. Retinal thickness parameters showed slower progression rates (range = 1.6-2.5%/year) from age 60 to 62.3. BCVA (median = 0.3 LogMAR, IQR = 0.0-1.0 at baseline) showed a rapid decline (3.3%) from age 70 years. Findings from patients with earlier disease showed FAF atrophy manifests in the temporal retina initially, and then progresses nasally.ConclusionsPatients with LORD remained asymptomatic until age 50 years, before suffering rapid outer retinal degeneration. EZ width and AA showed rapid progression and high interocular correlation, representing promising outcome metrics. Clinical measures also capturing the temporal retina may be preferable, enabling earlier detection and better disease monitoring.Translational relevanceArea of atrophy in FAF images and OCT-measured EZ width represent promising outcome metrics for disease monitoring in patients with C1QTNF5-LORD.

Dataset Information

QUANTITATIVE ANALYSIS OF LONGITUDINAL CHANGES IN MULTIMODAL IMAGING OF LATE-ONSET RETINAL DEGENERATION.

Purpose

Methods

Results

Conclusion

Publications

QUANTITATIVE ANALYSIS OF LONGITUDINAL CHANGES IN MULTIMODAL IMAGING OF LATE-ONSET RETINAL DEGENERATION.

Similar Datasets

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