Automated Segmentation Measurements of the Outer Nuclear Layer in Determining Cases of Hydroxychloroquine Toxicity: A Retrospective Study

Jain, Akshay; Livnat, Itamar; Tsipursky, Michael S.; Almasov, Alexandra; Garwood, Kathleen Campbell; Labriola, Leanne T.

doi:10.1177/2474126419836067

Cited by 4 publications

(1 citation statement)

References 31 publications

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“…Prior studies have sought to use either commercially available or customized software to assess parafoveal loss of outer versus inner retinal layers for hydroxychloroquine retinal toxicity, which has a well-established and discernable predilection for parafoveal OCT changes. [28][29][30][31] Other work has suggested that for hydroxychloroquine toxicity the outer nuclear layer (ONL) thinning may precede parafoveal ellipsoid disruption, or continue after cessation. 32,33 As such, perhaps subtle thinning of the ONL as measured by automated segmentation in CLN2 disease may precede more advanced photoreceptor outer segment degeneration and thus serve as a sensitive biomarker for disease onset, progression, and response to treatment.…”

Section: Introductionmentioning

confidence: 99%

Automated Retinal Layer Segmentation in CLN2-Associated Disease: Commercially Available Software Characterizing a Progressive Maculopathy

Kovacs

Orlin

Sondhi

et al. 2021

Trans. Vis. Sci. Tech.

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CLN2-associated disease is a hereditary, fatal lysosomal storage disorder characterized by progressive brain and retinal deterioration. Here, we characterize the inner and outer retinal degeneration using automated segmentation software in optical coherence tomography scans, providing an objective, quantifiable metric for monitoring subtle changes previously identified with a validated disease classification scale (the Weill Cornell Batten Scale).Methods: This study is a retrospective, single-center cohort review of images from examinations under anesthesia in treatment-naïve patients with CLN2-associated disease. Automated segmentation software was used to delineate retinal nerve fiber, ganglion cell layer (GCL), and outer nuclear layer (ONL) thickness measurements in the fovea, parafovea, and perifovea based on age groups (months): 30 to 38, 39 to 45, 46 to 52, 53 to 59, 60 to 66, and 67 or older.Results: Twenty-seven eyes from 14 patients were included, with 8 serial images yielding 36 interpretable optical coherence tomography scans. There was a significant difference in parafoveal ONL thickness between 39 to 45 and 46 to 52 months of age (P = 0.032) not seen in other regions or retinal layers. Perifoveal ONL demonstrated a difference in thickness between the 60 to 66 and greater than 67 months age cohorts (P = 0.047). There was strong symmetry between eyes, and high segmentation repeatability. Conclusions:Parafoveal ONL thickness represents a sensitive, early age indicator of CLN2-associated degeneration. Outer retinal degeneration is apparent at younger ages than inner retinal changes though in treatment-naïve patients all retinal layers showed significant differences between 60 to 66 and more than 67 months of age.Translational Relevance: This study establishes sensitive, quantitative biomarkers for assessing retinal degeneration in a large cohort natural history study in anticipation of future clinical trials.

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Section: Introductionmentioning

confidence: 99%

Automated Retinal Layer Segmentation in CLN2-Associated Disease: Commercially Available Software Characterizing a Progressive Maculopathy

Kovacs

Orlin

Sondhi

et al. 2021

Trans. Vis. Sci. Tech.

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Hydroxychloroquine treatment alters retinal layers and choroid without apparent toxicity in optical coherence tomography

Polat

Okçu

Yılmaz

2022

Photodiagnosis and Photodynamic Therapy

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Association between hydroxychloroquine intake and damage to the outer nuclear layer in eyes without manifest retinal toxicity

Salameh,

Doumit,

Jalkh

et al. 2024

BMC Ophthalmol

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Background Hydroxychloroquine (HCQ) is widely used to treat various autoimmune diseases but carries a risk of retinal toxicity, particularly with prolonged use. Despite advancements, uncertainty persists regarding optimal screening methods. Recent advances in OCT have enabled early detection of retinal damage, with studies suggesting that thinning of specific retinal layers may be an early indicator of toxicity. However, there is a gap in research on outer nuclear layer (ONL) thinning in HCQ users without apparent retinal toxicity. This information is crucial for improving screening and identifying the ONL as a reliable biomarker for screening. Therefore, this study aimed to investigate the association between HCQ intake and ONL damage in eyes without manifest retinal toxicity. Methods A case‒control study was conducted at the ophthalmology department of Eye and Ear Hospital International from July 2022 to June 2023. The study included 20 individuals on HCQ and 20 age-matched controls. The data were obtained through chart reviews, and participants underwent comprehensive ophthalmic assessments. Results A total of 80 eyes were analyzed. Patients on HCQ exhibited significantly thinner perifoveal, parafoveal, and overall ONL compared to controls (P < .001, P < .012, and P < .004, respectively). Similarly, this association was found in the nasal, inferior, and temporal quadrants of both the inner (region 3: P < .01, region 4: P < .001, and region 5: P < .03) and outer zones (region 7: P < .04, region 8: P < .001, region 9: P < .02), most pronounced in the inferior regions. The cumulative dose was weakly associated with decreased ONL thickness only in the nasal quadrant of the inner zone (region 3: P < .047). Correlation analysis of the initial and most recent OCT scans in the same individuals revealed a weak association with ONL thinning in the central zone (region 1: P < .0048). Conclusion The thickness of the ONL can significantly decrease in patients taking HCQ, even in the absence of of manifest retinal toxicity. This study is the first to evaluate this association in eyes with negative screening and diagnostic tests for HCQ retinopathy. The findings suggest that ONL thickness could serve as an early diagnostic indicator for HCQ retinal toxicity.

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Automated Segmentation Measurements of the Outer Nuclear Layer in Determining Cases of Hydroxychloroquine Toxicity: A Retrospective Study

Cited by 4 publications

References 31 publications

Automated Retinal Layer Segmentation in CLN2-Associated Disease: Commercially Available Software Characterizing a Progressive Maculopathy

Automated Retinal Layer Segmentation in CLN2-Associated Disease: Commercially Available Software Characterizing a Progressive Maculopathy

Hydroxychloroquine treatment alters retinal layers and choroid without apparent toxicity in optical coherence tomography

Association between hydroxychloroquine intake and damage to the outer nuclear layer in eyes without manifest retinal toxicity

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