Quantifying the Rate of Ellipsoid Zone Loss in Stargardt Disease

Cai, Cindy X.; Light, Jacob G.; Handa, James T.

doi:10.1016/j.ajo.2017.10.032

Cited by 36 publications

(33 citation statements)

References 57 publications

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“…Only one study has assessed the area of EZ loss longitudinally in STGD1 subjects (mean age 33.0 years) and demonstrated a gradual expansion in the area of EZ loss of 0.31 mm 2 /y, with the mean (± standard deviation) initial area of EZ loss being 4.18 ± 1.91 mm 2 25. In contrast, our study identified a higher mean baseline measurement (6.43 ± 4.67 mm 2 ), and notably the mean annual rate of area of EZ loss was more than 3 times faster (1.20 ± 1.29 mm 2 /year).…”

Section: Discussionmentioning

confidence: 99%

“…Spectral-domain optical coherence tomography (SD-OCT) enables in vivo high-resolution cross-sectional retinal images, allowing for quantitative assessments of the EZ, including manual identification of the transverse EZ loss and delineation of the area of EZ loss from an en face image. The EZ has been characterized in STGD1,22–24 yet only one study has tracked progression by the presence or absence of the EZ using en face OCT 25…”

Section: Introductionmentioning

confidence: 99%

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Cross-Sectional and Longitudinal Assessment of the Ellipsoid Zone in Childhood-Onset Stargardt Disease

Tanna

Georgiou

Strauss

et al. 2019

Trans. Vis. Sci. Tech.

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Purpose To evaluate the reliability of ellipsoid zone (EZ) loss width and area measurements from spectral-domain optical coherence tomography (SD-OCT) images and track disease progression in childhood-onset Stargardt disease (STGD1). Methods Children with molecularly confirmed STGD1 ( n = 46, mean age 12.4 years) underwent SD-OCT for the measurement of the transverse (width) loss of the EZ and en face analysis to quantify the area of EZ loss. All scans were analyzed twice by two graders to evaluate reliability. The annual rate of EZ width and area loss were calculated. Results The intra- and intergrader reliability of transverse EZ loss and area of EZ loss measurements at baseline for both graders was 0.99. The mean annual rate of transverse EZ loss (±standard deviation) was 279.5 ± 259.9 μm/y. The mean rate of area of EZ loss (±standard deviation) was 1.20 ± 1.29 mm 2 /y. The percentage transverse EZ loss was 10.2 ± 9.9%/y, which was significantly lower than the area of EZ loss at 19.4 ± 16.3%/y. High degree of interocular symmetry was observed. Conclusions This is a prospective study on the quantification of EZ loss in children with STGD1 and highlights the reliability of SD-OCT in measuring EZ loss. High intra- and intergrader reliability was observed, with good ability to detect changes over time. Translational Relevance Measuring the area of EZ loss was more sensitive compared with transverse EZ width loss measurements and will be valuable for natural history studies and clinical trials requiring sensitive and reliable structural endpoints.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Cross-Sectional and Longitudinal Assessment of the Ellipsoid Zone in Childhood-Onset Stargardt Disease

Tanna

Georgiou

Strauss

et al. 2019

Trans. Vis. Sci. Tech.

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“…The area of the EZ loss was then calculated using the NIR-R fundus image with the region finder tool of the Heyex software. 33 Figure 1 demonstrates an example of how EZA was measured. Heyex caliper measurements assume an axial length of 24 mm.…”

Section: Spectral Domain Octmentioning

confidence: 99%

Retinal Structure inRPE65-Associated Retinal Dystrophy

Kumaran

Georgiou

Bainbridge

et al. 2020

Invest. Ophthalmol. Vis. Sci.

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PURPOSE. RPE65-associated retinal dystrophy (RPE65-RD) is an early onset, progressive, severe retinal dystrophy. We sought to characterize the natural history of retinal degeneration in affected individuals. METHODS. We performed cross-sectional and longitudinal quantitative and qualitative assessments of retinal architecture in RPE65-RD using spectral domain optical coherence tomography (SD-OCT) and fundus autofluorescence (FAF) imaging. Twenty-six subjects (mean age, 14.8 years, range, 5-24 years) with RPE65-RD underwent SD-OCT and FAF imaging, of whom 14 subjects were followed up over time. Foveal thickness (FT), outer nuclear layer thickness (ONLT), ellipsoid zone width (EZW), and ellipsoid zone area (EZA) were calculated where possible. These were correlated with age, best corrected visual acuity (BCVA), and central 30°retinal sensitivity (V 30). Intra-observer agreement, test-retest repeatability, and interocular symmetry were also investigated. RESULTS. We identified structural interocular symmetry, the presence of autofluorescence in 46% (12/26) of subjects, and the presence of foveal hypoplasia (associated with significantly worse BCVA) in 50% of subjects. EZW and EZA were measurable in 67% (35/52) and 37% (19/52) of eyes, respectively, with both demonstrating good agreement on repeated measurement. The annual rate of progression using EZW was −300.63 μm/year, and −1.17 mm 2 /year in EZA. EZW was found to have a statistically significant correlation with BCVA and V 30. CONCLUSIONS. We identified the presence of autofluorescence in half of our subjects, with foveal hypoplasia also noted in half of our cohort. EZW, and to a lesser extent EZA, were robust measures of retinal degeneration and represent valuable metrics to determine the impact of intervention. (ClinicalTrials.gov number NCT02714816.

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“…There is an ongoing lively debate regarding the exact cellular structure that correlates with this hyper-reflective band and the nomenclature used to describe it. Recent publications [ 10 – 16 ], including that from a consensus International Nomenclature group [ 17 ], refer to this band as the ellipsoid zone (EZ) as it is thought to represent the ellipsoid region of the photoreceptor inner segments which have densely-packed mitochondria that are likely hyper-reflective on OCT [ 18 ]. However, other studies, including a recent one that used adaptive optics to characterize this structure [ 9 ], refer to it as the junction between the inner segments and outer segments (IS/OS) of the photoreceptors [ 2 – 4 , 8 , 19 – 22 ].…”

Section: Introductionmentioning

confidence: 99%

Deep longitudinal transfer learning-based automatic segmentation of photoreceptor ellipsoid zone defects on optical coherence tomography images of macular telangiectasia type 2

Loo¹,

Fang²,

Cunefare³

et al. 2018

Biomed. Opt. Express

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Photoreceptor ellipsoid zone (EZ) defects visible on optical coherence tomography (OCT) are important imaging biomarkers for the onset and progression of macular diseases. As such, accurate quantification of EZ defects is paramount to monitor disease progression and treatment efficacy over time. We developed and trained a novel deep learning-based method called Deep OCT Atrophy Detection (DOCTAD) to automatically segment EZ defect areas by classifying 3-dimensional A-scan clusters as normal or defective. Furthermore, we introduce a longitudinal transfer learning paradigm in which the algorithm learns from segmentation errors on images obtained at one time point to segment subsequent images with higher accuracy. We evaluated the performance of this method on 134 eyes of 67 subjects enrolled in a clinical trial of a novel macular telangiectasia type 2 (MacTel2) therapeutic agent. Our method compared favorably to other deep learning-based and non-deep learning-based methods in matching expert manual segmentations. To the best of our knowledge, this is the first automatic segmentation method developed for EZ defects on OCT images of MacTel2.

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Quantifying the Rate of Ellipsoid Zone Loss in Stargardt Disease

Cited by 36 publications

References 57 publications

Cross-Sectional and Longitudinal Assessment of the Ellipsoid Zone in Childhood-Onset Stargardt Disease

Cross-Sectional and Longitudinal Assessment of the Ellipsoid Zone in Childhood-Onset Stargardt Disease

Retinal Structure inRPE65-Associated Retinal Dystrophy

Deep longitudinal transfer learning-based automatic segmentation of photoreceptor ellipsoid zone defects on optical coherence tomography images of macular telangiectasia type 2

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