Patches of Dysflective Cones in Eyes With No Known Disease

Bensinger, Ethan; Wang, Yiyi; Roorda, Austin

doi:10.1167/iovs.63.1.29

Cited by 8 publications

(5 citation statements)

References 58 publications

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“…These cones were located in their counterpart image, and analyzed for their spectral type using ORG. Such cones with abnormal reflections have been observed in healthy and diseased eyes[39, 40, 42]. They have previously been shown to have normal function also[41, 42].…”

Section: Discussionmentioning

confidence: 94%

“…In the extreme case, some cones have abnormally low reflectivity, dubbed 'dysflective' [38,39]. It has been noted that normal eyes with no known disease have patches of dysflective cones that sometimes regain their reflectivity over time [40]. Also, using retinal tracking and targeted stimulus delivery, it has been shown cones with low reflectivity exhibit normal perceptual sensitivity [41].…”

Section: Dysflective Conesmentioning

confidence: 99%

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Characterizing Cone Spectral Classification by Optoretinography

Pandiyan

Schleufer

Slezak

et al. 2022

Preprint

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Light propagation in photoreceptor outer segments is affected by photopigment absorption and the phototransduction amplification cascade. Photopigment absorption has been studied using retinal densitometry, while recently, optoretinography (ORG) has provided an avenue to probe changes in outer segment optical path length due to phototransduction. With adaptive optics (AO), both densitometry and ORG have been used for cone spectral classification, based on the differential bleaching signatures of the three cone types. Here, we characterize cone classification by ORG, implemented in an AO line-scan OCT and compare it against densitometry. The cone mosaics of five color normal subjects were classified using ORG showing high probability (~0.99), low error (<0.22%), high test-retest reliability (~97%) and short imaging durations (< 1 hour). Of these, the cone spectral assignments in two subjects were compared against AOSLO densitometry. High agreement (mean: 91%) was observed between the two modalities in these 2 subjects, with measurements conducted 6-7 years apart. Overall, ORG benefits from higher sensitivity and dynamic range to probe cone photopigments compared to densitometry, and thus provides greater fidelity for cone spectral classification.

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Section: Discussionmentioning

confidence: 94%

Section: Dysflective Conesmentioning

confidence: 99%

Characterizing Cone Spectral Classification by Optoretinography

Pandiyan

Schleufer

Slezak

et al. 2022

Preprint

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“…), it is important to bear in mind that waveguiding status is not an unequivocal indication of cone health. 38 Normal cone reflectance has been shown to vary over time, 39 as well as in response to light stimulation. 40 Moreover, measurable function has been found within lesions that lack visible cones in confocal AOSLO images, 41 so it may be that the non-waveguiding cells observed in BCM are functional S-cones.…”

Section: Discussionmentioning

confidence: 99%

Foveal Cone Structure in Patients With Blue Cone Monochromacy

Patterson

Kalitzeos

Kane

et al. 2022

Invest. Ophthalmol. Vis. Sci.

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Purpose Blue cone monochromacy (BCM) is a rare inherited cone disorder in which both long- (L-) and middle- (M-) wavelength sensitive cone classes are either impaired or nonfunctional. Assessing genotype-phenotype relationships in BCM can improve our understanding of retinal development in the absence of functional L- and M-cones. Here we examined foveal cone structure in patients with genetically-confirmed BCM, using adaptive optics scanning light ophthalmoscopy (AOSLO). Methods Twenty-three male patients (aged 6–75 years) with genetically-confirmed BCM were recruited for high-resolution imaging. Eight patients had a deletion of the locus control region (LCR), and 15 had a missense mutation—Cys203Arg—affecting the first two genes in the opsin gene array. Foveal cone structure was assessed using confocal and non-confocal split-detection AOSLO across a 300 × 300 µm area, centered on the location of peak cell density. Results Only one of eight patients with LCR deletions and 10 of 15 patients with Cys203Arg mutations had analyzable images. Mean total cone density for Cys203Arg patients was 16,664 ± 11,513 cones/mm 2 (n = 10), which is, on average, around 40% of normal. Waveguiding cone density was 2073 ± 963 cones/mm 2 (n = 9), which was consistent with published histological estimates of S-cone density in the normal eye. The one patient with an LCR deletion had a total cone density of 10,246 cones/mm 2 and waveguiding density of 1535 cones/mm 2 . Conclusions Our results show that BCM patients with LCR deletions and Cys203Arg mutations have a population of non-waveguiding photoreceptors, although the spectral identity and level of function remain unknown.

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“…Short-term and long-term variations in cone reflectivity and dysflective cones have been noted in normal subjects with no known ocular disease [125,126]. It has been proposed that the changes in the reflectivity of cones are either the result of altered molecular and biological processes, such as phototransduction, or changes in outer segment length, which acts as a biological interferometer [127,128].…”

Section: Cone Matching and Follow-up Imagingmentioning

confidence: 99%

Pearls and Pitfalls of Adaptive Optics Ophthalmoscopy in Inherited Retinal Diseases

et al. 2023

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Adaptive optics (AO) retinal imaging enables individual photoreceptors to be visualized in the clinical setting. AO imaging can be a powerful clinical tool for detecting photoreceptor degeneration at a cellular level that might be overlooked through conventional structural assessments, such as spectral-domain optical coherence tomography (SD-OCT). Therefore, AO imaging has gained significant interest in the study of photoreceptor degeneration, one of the most common causes of inherited blindness. Growing evidence supports that AO imaging may be useful for diagnosing early-stage retinal dystrophy before it becomes apparent on fundus examination or conventional retinal imaging. In addition, serial AO imaging may detect structural disease progression in early-stage disease over a shorter period compared to SD-OCT. Although AO imaging is gaining popularity as a structural endpoint in clinical trials, the results should be interpreted with caution due to several pitfalls, including the lack of standardized imaging and image analysis protocols, frequent ocular comorbidities that affect image quality, and significant interindividual variation of normal values. Herein, we summarize the current state-of-the-art AO imaging and review its potential applications, limitations, and pitfalls in patients with inherited retinal diseases.

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Patches of Dysflective Cones in Eyes With No Known Disease

Cited by 8 publications

References 58 publications

Characterizing Cone Spectral Classification by Optoretinography

Characterizing Cone Spectral Classification by Optoretinography

Foveal Cone Structure in Patients With Blue Cone Monochromacy

Pearls and Pitfalls of Adaptive Optics Ophthalmoscopy in Inherited Retinal Diseases

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