scholarly journals Reduced foveal cone density in early idiopathic macular telangiectasia

2021 ◽  
Vol 6 (1) ◽  
pp. e000603
Author(s):  
Hongxin Song ◽  
Ethan A Rossi ◽  
David R Williams
Keyword(s):  
Adaptive Optics ◽  
Cellular Level ◽  
Cone Photoreceptor ◽  
Cone Photoreceptors ◽  
Clinically Normal ◽  
Macular Telangiectasia ◽  
Idiopathic Macular Telangiectasia ◽  
Retinal Capillaries ◽  
Manual Correction ◽  
Cone Density

ObjectiveIdiopathic macular telangiectasia (MacTel) is considered primarily a vascular disease affecting juxtafoveal retinal capillaries. However, recent evidence suggests that neuronal changes may occur early in disease development. We used high-resolution adaptive optics retinal imaging to elucidate the foveal cone photoreceptor changes at a cellular level in patients with MacTel.Methods and analysisWe used adaptive optics scanning light ophthalmoscopy (AOSLO) to evaluate the foveal cone photoreceptors in the less-affected eye of patients with asymmetric MacTel. AOSLO images of cone photoreceptors were obtained in a 4°×4° area centred on the foveola. Individual cone positions were identified within a 2°×2° area centred on the fovea, using semiautomatic cone marking software with manual correction, permitting calculation of a map of cone density.ResultsIn all participants, one eye was affected with MacTel, the fellow eye was clinically normal or near normal, with visual acuity of 20/25 or better and subtle angiographic leakage. The foveal cone mosaics were continuous with tight packing and cones exhibited normal reflectivity. However, cone density was significantly lower for all participants (mean=80 733 cones/mm2) within 0.5° than the cone density previously reported for normal eyes.ConclusionsFoveal cone density is lower than normal in the clinically less-affected eyes of patients with asymmetric MacTel. This suggests that cone photoreceptor loss may precede classic obvious vascular changes in idiopathic MacTel.

scholarly journals Human foveal cone photoreceptor topography and its dependence on eye length

eLife ◽  
2019 ◽  
Vol 8 ◽  
Author(s):  
Yiyi Wang ◽  
Nicolas Bensaid ◽  
Pavan Tiruveedhula ◽  
Jianqiang Ma ◽  
Sowmya Ravikumar ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Adaptive Optics ◽  
Axial Length ◽  
Visual Angle ◽  
Cone Photoreceptor ◽  
Foveal Vision ◽  
Cone Mosaic ◽  
New Generation ◽  
Myopic Progression ◽  
Cone Density

We provide the first measures of foveal cone density as a function of axial length in living eyes and discuss the physical and visual implications of our findings. We used a new generation Adaptive Optics Scanning Laser Ophthalmoscope to image cones at and near the fovea in 28 eyes of 16 subjects. Cone density and other metrics were computed in units of visual angle and linear retinal units. The foveal cone mosaic in longer eyes is expanded at the fovea, but not in proportion to eye length. Despite retinal stretching (decrease in cones/mm2), myopes generally have a higher angular sampling density (increase in cones/deg2) in and around the fovea compared to emmetropes, offering the potential for better visual acuity. Reports of deficits in best-corrected foveal vision in myopes compared to emmetropes cannot be explained by increased spacing between photoreceptors caused by retinal stretching during myopic progression.

scholarly journals Computer-assisted photoreceptor assessment on Heidelberg Engineering Spectralis™ High Magnification Module™ images

2021 ◽  
Author(s):  
Timo W. F. Mulders ◽  
B. Jeroen Klevering ◽  
Carel B. Hoyng ◽  
Thomas Theelen
Keyword(s):  
Adaptive Optics ◽  
Nearest Neighbor ◽  
Retinal Disease ◽  
Computer Assisted ◽  
High Magnification ◽  
Cone Photoreceptor ◽  
Friedman Test ◽  
The Third ◽  
Progression Marker ◽  
Cone Density

Abstract Purpose To evaluate reliability and repeatability of computer-assisted measurements of cone photoreceptor metrics on Heidelberg Engineering Spectralis™ High Magnification Module (HMM™) Automatic Real-time Tracking (ART™) images. Methods We analyzed HMM™ images in three separate study arms. Computer-assisted cone identification software was validated using an open-access adaptive optics (AO) dataset. We compared results of the first arm to data from AO and histology. We evaluated intersession repeatability of our computer-assisted cone analysis in the second arm. We assessed the capability of HMM™ to visualize cones in the presence of pathology in the third arm. Results We included 10 healthy subjects in the first arm of our study, 5 additional healthy participants in the second arm and 5 patients in the third arm. In total, we analyzed 225 regions of interest on HMM™ images. We were able to automatically identify cone photoreceptors and assess corresponding metrics at all eccentricities between 2 and 9° from the fovea. Cone density significantly declined with increasing eccentricity (p = 4.890E-26, Friedman test). With increasing eccentricity, we found a significant increase in intercell distance (p = 2.196E-25, Friedman test) and nearest neighbor distance (p = 1.997E-25, Friedman test). Cone hexagonality ranged between 71 and 85%. We found excellent automated intersession repeatability of cone density counts and spacing measurements. In pathology, we were also able to repeatedly visualize photoreceptors. Conclusion Computer-assisted cone photoreceptor analysis on Spectralis™ HMM™ images is feasible, and most cone metrics show excellent repeatability. HMM™ imaging may be useful for photoreceptor analysis as progression marker in outer retinal disease.

Comparison of Cone Pathologic Changes in Idiopathic Macular Telangiectasia Types 1 and 2 Using Adaptive Optics Scanning Laser Ophthalmoscopy

2013 ◽  
Vol 155 (6) ◽  
pp. 1045-1057.e4 ◽  
Author(s):  
Sotaro Ooto ◽  
Masanori Hangai ◽  
Kohei Takayama ◽  
Naoko Ueda-Arakawa ◽  
Akitaka Tsujikawa ◽  
...  
Keyword(s):  
Adaptive Optics ◽  
Scanning Laser ◽  
Scanning Laser Ophthalmoscopy ◽  
Macular Telangiectasia ◽  
Idiopathic Macular Telangiectasia

scholarly journals The Endoplasmic Reticulum Proteostasis Regulator ATF6 is Essential for Human Cone Photoreceptor Development

2020 ◽  
Author(s):  
Heike Kroeger ◽  
Julia M. D. Grandjean ◽  
Wei-Chieh Jerry Chiang ◽  
Daphne Bindels ◽  
Rebecca Mastey ◽  
...  
Keyword(s):  
Endoplasmic Reticulum ◽  
Adaptive Optics ◽  
Vision Loss ◽  
Photoreceptor Cells ◽  
Cone Photoreceptor ◽  
Cone Photoreceptors ◽  
Unfolded Protein ◽  
Activating Transcription Factor ◽  
Protein Response ◽  
Rod Structures

AbstractDysregulation of the endoplasmic reticulum (ER) Unfolded Protein Response (UPR) is implicated in the pathology of many human diseases associated with ER stress. Inactivating genetic variants in the UPR regulator Activating Transcription Factor 6 (ATF6) cause severe congenital heritable vision loss in patients by an unknown pathomechanism. To investigate this, we generated retinal organoids from patient iPSCs carrying ATF6 disease-causing variants and ATF6 null hESCs generated by CRISPR. Interestingly, we found that cone photoreceptor cells in ATF6 mutant retinal organoids lacked inner and outer segments concomitant with absence of cone phototransduction gene expression; while rod photoreceptors developed normally. Adaptive optics retinal imaging of patients with disease-causing variants in ATF6 also showed absence of cone inner/outer segment structures but preserved rod structures, mirroring the phenotypes observed in our retinal organoids. These results reveal that ATF6 is essential for the formation of human cone photoreceptors, and associated absence of cone phototransduction components explains the severe visual impairment in patients with ATF6 -associated retinopathy. Moreover, we show that a selective small molecule ATF6 activator compound restores the transcriptional activity of ATF6 disease-causing variants and stimulates the growth of cone photoreceptors in patient retinal organoids, demonstrating that pharmacologic targeting of ATF6 signaling is a therapeutic strategy that needs to be further explored for blinding retinal diseases.

In vivo evaluation of photoreceptor mosaic in type 2 idiopathic macular telangiectasia using adaptive optics

2010 ◽  
Vol 89 (7) ◽  
pp. e601-e603 ◽  
Author(s):  
Nathalie Massamba ◽  
Giuseppe Querques ◽  
Barbara Lamory ◽  
Lea Querques ◽  
Eric Souied ◽  
...  
Keyword(s):  
Adaptive Optics ◽  
In Vivo Evaluation ◽  
Macular Telangiectasia ◽  
Idiopathic Macular Telangiectasia ◽  
Photoreceptor Mosaic

scholarly journals Three-dimensional composition of the photoreceptor cone layers in healthy eyes using adaptive-optics optical coherence tomography (AO-OCT)

PLoS ONE ◽  
2021 ◽  
Vol 16 (1) ◽  
pp. e0245293
Author(s):  
Adrian Reumueller ◽  
Lorenz Wassermann ◽  
Matthias Salas ◽  
Markus Schranz ◽  
Valentin Hacker ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Adaptive Optics ◽  
Outer Segment ◽  
Three Dimensional ◽  
Optical Coherence ◽  
Cone Photoreceptors ◽  
Cone Mosaic ◽  
Automated Technique ◽  
Healthy Eyes ◽  
Cone Density

Purpose To assess the signal composition of cone photoreceptors three-dimensionally in healthy retinas using adaptive optics optical coherence tomography (AO-OCT). Methods Study population. Twenty healthy eyes of ten subjects (age 23 to 67). Procedures. After routine ophthalmological assessments, eyes were examined using AO-OCT. Three-dimensional volumes were acquired at 2.5° and 6.5° foveal eccentricity in four main meridians (superior, nasal, inferior, temporal). Cone densities and signal compositions were investigated in four different planes: the cone inner segment outer segment junction (IS/OS), the cone outer segment combined with the IS/OS (ISOS+), the cone outer segment tips (COST) and full en-face plane (FEF) combining signals from all mentioned cone layers. Additionally, reliability of a simple semi-automated approach for assessment of cone density was tested. Main outcome measures. Cone density of IS/OS, IS/OS+, COST and FEF. Qualitative depiction and composition of each cone layer. Inter-rater agreement of cone density measurements. Results Mean overall cone density at all eccentricities was highest at the FEF plane (21.160/mm2), followed by COST (20.450/mm2), IS/OS+ (19.920/mm2) and IS/OS (19.530/mm2). The different meridians and eccentricities had a significant impact on cone density, with lower eccentricity resulting in higher cone densities (p≤.001), which were highest at the nasal, then temporal, then inferior and then superior meridian. Depiction of the cone mosaic differed between all 4 layers regarding signal size and packing density. Therefore, different cone layers showed evident but not complete signal overlap. Using the semi-automated technique for counting of cone signals achieved high inter-rater reliability (ICC > .99). Conclusions In healthy individuals qualitative and quantitative changes in cone signals are found not only in different eccentricities and meridians, but also within different photoreceptor layers. The variation between cone planes has to be considered when assessing the integrity of cone photoreceptors in healthy and diseased eyes using adaptive optics technology.

CONE DENSITY LOSS ON ADAPTIVE OPTICS IN EARLY MACULAR TELANGIECTASIA TYPE 2

Retina ◽  
2016 ◽  
Vol 36 (3) ◽  
pp. 545-551 ◽  
Author(s):  
Julie Jacob ◽  
Valérie Krivosic ◽  
Michel Paques ◽  
Ramin Tadayoni ◽  
Alain Gaudric
Keyword(s):  
Adaptive Optics ◽  
Macular Telangiectasia ◽  
Macular Telangiectasia Type 2 ◽  
Cone Density

scholarly journals Assessment of fundus autofluorescence and adaptive optics high-resolution images in macular disorders

2021 ◽  
Author(s):  
Radu Ochinciuc ◽  
Uliana Ochinciuc ◽  
George Balta ◽  
Adrian Teodoru ◽  
Leila Al Barri ◽  
...  
Keyword(s):  
High Resolution ◽  
Adaptive Optics ◽  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Subretinal Fluid ◽  
Cellular Level ◽  
Cone Mosaic ◽  
High Resolution Images ◽  
Cone Density

Abstract Purpose This study analyzed and compared the results of adaptive optics (AO) and fundus autofluorescence (FAF) in various maculopathies. Methods The study included four different types of maculopathy: central serous chorioretinopathy (CSC), retinitis pigmentosa (RP), Stargardt disease (STGD) and phototoxic retinopathy. In all four cases cone mosaic and cone density were obtained using AO fundus camera. Further the high resolution images were compared with the FAF and optical coherence tomography (OCT) results. Results In CSC, FAF and AO were able to show changes in the macula even two years after the subretinal fluid resorption, as opposed to a normal OCT. The improvement of FAF and cone mosaic appearance was concomitant with the visual acuity growth. Several cone mosaic phenotypes were observed in RP and STGD. In RP the cone density was 24.240cones/mm2 in the center, and decreased to 8.163cones/mm2 in the parafoveolar area. In STGD the cone density was lower in the center, 9.219cones/mm2, and higher at the periphery, 12.594cones/mm2. In the case of phototoxic retinopathy, AO and OCT were more effective than FAF in highlighting the photoreceptor and retinal pigment epithelium lesions. Conclusions FAF and AO are very useful tools in macular pathologies examination. FAF can give us a true picture about metabolic changes in the macula while AO allows us to view changes up to the cellular level.

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