Adaptive Optics Fundus Camera Research Articles

Is Primary Open-Angle Glaucoma a Vascular Disease? Assessment of the Relationship between Retinal Arteriolar Morphology and Glaucoma Severity Using Adaptive Optics.

Retinal vascular abnormalities may be associated with glaucomatous damage. Adaptive optics (AO) is a new technology that enables the analysis of retinal vasculature at the cellular level in vivo. The purpose of this study was to evaluate retinal arteriolar parameters using the rtx1 adaptive optics fundus camera (AO-FC) in patients with primary open-angle glaucoma (POAG) at different stages and to investigate the relationship between these parameters and changes in spectral-domain optical coherence tomography (SD-OCT) and perimetry. Parameters of the retinal supratemporal and infratemporal arterioles (wall thickness (WT), lumen diameter (LD), total diameter (TD), wall-to-lumen ratio (WLR), and cross-sectional area of the vascular wall (WCSA)) were analysed with the rtx1 in 111 POAG eyes, which were divided into three groups according to the severity of the disease, and 70 healthy eyes. The associations between RTX1 values and the cup-to-disk ratio, SD-OCT parameters, and visual field parameters were assessed. Compared with the control group, the POAG groups showed significantly smaller TD and LD values (p < 0.05) and significantly higher WLR and WT values (p < 0.05) for the supratemporal and infratemporal arterioles. TD was significantly positively correlated with the retinal nerve fibre layer (RNFL) and ganglion cell complex (GCC) (p < 0.05). LD was significantly positively correlated with the RNFL, GCC, and rim area (p < 0.05). The WLR was significantly negatively correlated with the RNFL, GCC, rim area, and MD (p < 0.05), while it was significantly positively correlated with the cup-to-disc ratio and PSD (p < 0.05). The results suggest that vascular dysfunction is present in POAG, even at a very early stage of glaucoma, and increases with the severity of the disease.

Evaluation of photoreceptor features in retinitis pigmentosa with cystoid macular edema by using an adaptive optics fundus camera.

Cystoid macular edema (CME) in retinitis pigmentosa (RP) is an important complication causing visual dysfunction. We investigated the effect of CME on photoreceptors in RP patients with previous or current CME, using an adaptive optics (AO) fundus camera. We retrospectively observed the CME and ellipsoid zone (EZ) length (average of horizontal and vertical sections) by optical coherence tomography. The density and regularity of the arrangement of photoreceptor cells (Voronoi analysis) were examined at four points around 1.5° from superior to inferior and temporal to nasal. We also performed a multivariate analysis using CME duration, central macular thickness and transversal length of CME. We evaluated 18 patients with previous or current CME (18 eyes; age, 48.7 ± 15.6 years) and 24 patients without previous or current CME (24 eyes; age, 46.0 ± 14.5 years). There were no significant differences in age, logMAR visual acuity, or EZ length. In groups with and without CME, cell density was 11967 ± 3148 and 16239 ± 2935 cells/mm2, and sequence regularity was 85.5 ± 3.4% and 88.5 ± 2.8%, respectively; both parameters were significantly different. The correlation between photoreceptor density and age was more negative in group with CME. The CME group tended toward greater reductions in duration of CME. Complications of CME in RP patients may lead to a decrease in photoreceptor density and regularity. Additionally, a longer duration of CME may result in a greater reduction in photoreceptor density.

Absolute retinal blood flow in healthy eyes and in eyes with retinal vein occlusion

PurposeTo measure non-invasively retinal venous blood flow (RBF) in healthy subjects and patients with retinal venous occlusion (RVO). MethodsThe prototype named AO-LDV (Adaptive Optics Laser Doppler Velocimeter), which combines a new absolute laser Doppler velocimeter with an adaptive optics fundus camera (rtx1, Imagine Eyes®, Orsay, France), was studied for the measurement of absolute RBF as a function of retinal vessel diameters and simultaneous measurement of red blood cell velocity. RBF was measured in healthy subjects (n = 15) and patients with retinal venous occlusion (RVO, n = 6). We also evaluated two softwares for the measurement of retinal vessel diameters: software 1 (automatic vessel detection, profile analysis) and software 2 (based on the use of deep neural networks for semantic segmentation of vessels, using a M2u-Net architecture). ResultsSoftware 2 provided a higher rate of automatic retinal vessel measurement (99.5 % of 12,320 AO images) than software 1 (64.9 %) and wider measurements (75.5 ± 15.7 μm vs 70.9 ± 19.8 μm, p < 0.001). For healthy subjects (n = 15), all the retinal veins in one eye were measured to obtain the total RBF. In healthy subjects, the total RBF was 37.8 ± 6.8 μl/min. There was a significant linear correlation between retinal vessel diameter and maximal velocity (slope = 0.1016; p < 0.001; r2 = 0.8597) and a significant power curve correlation between retinal vessel diameter and blood flow (3.63 × 10−5 × D2.54; p < 0.001; r2 = 0.7287). No significant relationship was found between total RBF and systolic and diastolic blood pressure, ocular perfusion pressure, heart rate, or hematocrit. For RVO patients (n = 6), a significant decrease in RBF was noted in occluded veins (3.51 ± 2.25 μl/min) compared with the contralateral healthy eye (11.07 ± 4.53 μl/min). For occluded vessels, the slope between diameter and velocity was 0.0195 (p < 0.001; r2 = 0.6068) and the relation between diameter and flow was Q = 9.91 × 10−6 × D2.41 (p < 0.01; r2 = 0.2526). ConclusionThis AO-LDV prototype offers new opportunity to study RBF in humans and to evaluate treatment in retinal vein diseases.

Application of Adaptive Optics in Ophthalmology

The eye, the photoreceptive organ used to perceive the external environment, is of great importance to humans. It has been proven that some diseases in humans are accompanied by fundus changes; therefore, the health status of people may be interpreted from retinal images. However, the human eye is not a perfect refractive system for the existence of ocular aberrations. These aberrations not only affect the ability of human visual discrimination and recognition, but restrict the observation of the fine structures of human eye and reduce the possibility of exploring the mechanisms of eye disease. Adaptive optics (AO) is a technique that corrects optical wavefront aberrations. Once integrated into ophthalmoscopes, AO enables retinal imaging at the cellular level. This paper illustrates the principle of AO in correcting wavefront aberrations in human eyes, and then reviews the applications and advances of AO in ophthalmology, including the adaptive optics fundus camera (AO-FC), the adaptive optics scanning laser ophthalmoscope (AO-SLO), the adaptive optics optical coherence tomography (AO-OCT), and their combined multimodal imaging technologies. The future development trend of AO in ophthalmology is also prospected.

Absolute retinal blood flowmeter using a laser Doppler velocimeter combined with adaptive optics.

.Significance: The development of a technique allowing for non-invasive measurement of retinal blood flow (RBF) in humans is needed to understand many retinal vascular diseases (pathophysiology) and evaluate treatment with potential improvement of blood flow.Aim: We developed and validated an absolute laser Doppler velocimeter (LDV) based on an adaptive optical fundus camera that provides simultaneously high-definition images of the fundus vessels and absolute maximal red blood cells (RBCs) velocity to calculate the absolute RBF.Approach: This new absolute LDV is combined with the adaptive optics (AO) fundus camera (rtx1, Imagine Eyes©, Orsay, France) outside its optical wavefront correction path. A 4-s recording includes 40 images, each synchronized with two Doppler shift power spectra. Image analysis provides a vessel diameter close to the probing beam, and the velocity of the RBCs in the vessels are extracted from the Doppler spectral analysis. A combination of these values gives an average of the absolute RBF.Results: An in vitro experiment consisting of latex microspheres flowing in water through a glass capillary to simulate a blood vessel and in vivo measurements on six healthy humans was done to assess the device. In the in vitro experiment, the calculated flow varied between 1.75 and and was highly correlated () with the flow imposed by a syringe pump. In the in vivo experiment, the error between the flow in the parent vessel and the sum of the flow in the daughter vessels was between and 36% (, ). RBF in the main temporal retinal veins of healthy subjects varied between 0.9 and .Conclusions: The AO LDV prototype allows for the real-time measurement of absolute RBF derived from the retinal vessel diameter and the maximum RBCs velocity in that vessel.

Photoreceptor assessment in focal laser-treated central serous chorioretinopathy using adaptive optics and fundus autofluorescence.

This study analyzed cone density, cone mosaic, and fundus autofluorescence (FAF) images in patients with focal laser-treated central serous chorioretinopathy (CSC).Observational case series.Forty-two eyes of 21 patients with unilateral treated CSC and bilateral best-corrected visual acuity of 1.0 (decimal fraction) were included. FAF and cone mosaic images were obtained in all patients with an adaptive optics fundus camera. Densities were recorded at 20 points throughout the macula, and choroidal thicknesses were measured.Mean choroidal thicknesses were 419.95 ± 110.33 μm in normal eyes, 459.09 ± 90.07 μm in eyes with active CSC, and 438.61 ± 107.57 μm in treated eyes. The highest density of cones in healthy eyes was 38146 cones/mm2, with a 5.66-μm intercellular space (IS), at 700 μm temporal to the center. In eyes with treated CSC, the highest density was 32749 cones/mm2, with a 6.13-μm IS, at 500 μm nasal to the center. In all quadrants, median values of maximum cone density were significantly higher in healthy eyes (P = .02, P = .003, P = .0001, and P = .001). Three types of lesions were identified on FAF and were correlated with those on cone mosaic images. Strong correlations were detected between the presence of hypoautofluorescent lesions on the first FAF image and a greater difference between maximum values of photoreceptor density (r 2 = 0.46, P = .03), as well as between the presence of hypoautofluorescent lesions and the duration of pathology (r 2 = 0.68, P < .001).The presence of hypoautofluorescent lesions and the duration of pathology were negative prognostic factors in CSC. Laser treatment could prevent photoreceptor loss.

CHANGES IN VISUAL ACUITY AND PHOTORECEPTOR DENSITY USING ADAPTIVE OPTICS AFTER RETINAL DETACHMENT REPAIR.

To quantify changes in photoreceptor density using adaptive optics fundus camera in patients after retinal detachment (RD) and to correlate them with macular involvement and best-corrected visual acuity. At 1 and 3 months (M1 and M3) after vitrectomy, 194 patients underwent adaptive optics imagery in both eyes, at 5 locations, that we matched between time points using anatomical landmarks. Twenty-two patients (10 fovea-OFF [OFF] and 12 fovea-ON [ON]) had matched and analyzable adaptive optics images. We used analysis of variance for repeated measures. Best-corrected visual acuity (logarithm of the minimum angle of resolution and Snellen equivalent [SE]) was significantly different between OFF and ON RDs at baseline: 2.0 (2.3-0.95) (SE: 20/2000) versus 0 (0.1-0) (SE: 20/20); at M1: 0.35 (0.5-0.1) (SE: 20/40) versus 0.05 (0-0.1) (SE: 20/25); and at M3: 0.25 (0.3-0.1) (SE: 20/32) versus 0 (0-0) (SE: 20/20). We observed that cone density was stable in fellow eyes between M1 and M3 (P = 0.67); decreased in treated eyes than in fellow eyes (P < 0.05); and increased postoperatively in the ON group (P = 0.02) but not in the OFF group (P = 0.97). Visual acuity and RD type were independently correlated with cone density (P = 0.004, P = 0.000). Postoperative cone density was reduced in OFF RD, but also in the ON group, although the drop recovered during the 3-month follow-up. Cone density was significantly correlated with both visual acuity and type of RD at both time points.

The association between cone density and visual function in the macula of patients with retinitis pigmentosa.

We observed cone photoreceptors by using adaptive optics (AO) fundus camera and optical coherence tomography (OCT) in patients with retinitis pigmentosa (RP) and examined the correlations between cone photoreceptors and visual function over 2years. Six patients with RP were studied. All patients underwent measurement using best-corrected decimal visual acuity, OCT, a Humphrey Field Analyzer with the 10-2 test grid pattern, and AO. AO images of the foveal center were obtained using an rtx1™ AO fundus camera, and cone counting was performed at 0.5mm from the foveal center. AO detected a decrease of cone density over 2years in RP patients. However, visual acuity, foveal sensitivity, and photoreceptor thickness were not changed over the 2years. AO images showed a decrease in the number of foveal cone densities over 2years in patients with RP. AO may shorten the period required to predict the RP progression rate.

Long-term changes of retinal pigment epithelium in the eyes with Vogt-Koyanagi-Harada disease observed by adaptive optics imaging.

Purpose: To observe long-term changes of the retinal pigment epithelium (RPE) in the eyes of patients with Vogt-Koyanagi-Harada (VKH) disease using an adaptive optics (AO) fundus camera, and their correlation with the findings of spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (AF) imaging.Patients and methods: Three eyes of two patients with new-onset acute VKH disease were retrospectively studied. After the serous retinal detachment was resolved by high-dose corticosteroid treatment, the patients were examined with SD-OCT, blue-wavelength AF, near-infrared (NIR) AF, and an AO fundus camera. AO images of the macula were obtained using the rtx1TM AO fundus camera. The area around the foveal center of the hyper-reflective lesion in AO imaging was measured manually. The time at which the serous retinal detachment resolved was set as the baseline, and AO and other images were obtained every 3 to 6 months from the baseline.Results: In all three eyes, lesions with elevation or thickening of the RPE layer were observed with OCT imaging in the macula after the serous retinal detachment resolved. These lesions showed hyper-autofluorescence in NIR-AF image and hyper-reflective lesions with clear boundaries in AO image. The area of the hyper-reflective lesions of AO images in each eye showed an approximately 40% decrease at 6 months from baseline. However, the hyper-reflective lesion remained to some extent after 18 months in case 1 and 36 months in case 2.Conclusions: By using OCT, fundus autofluorescence and AO images, it was possible to observe temporal changes of RPE layer in VKH eyes noninvasively. High-resolution AO images also allow us to observe for improvements in the elevation or thickening of the RPE layer quantitatively.

Differential detection of retinal directionality.

An adaptive optics fundus camera has been developed that uses simultaneous capture of multiple images via adjacent pupil sectors to provide directional sensitivity. In the chosen realization, a shallow refractive pyramid prism is used to subdivide backscattered light from the retina into four solid angles. Parafoveal fundus images have been captured for the eyes of three healthy subjects and directional scattering has been determined using horizontal and vertical differentials. The results for the photoreceptor cones, blood vessels, and the optic disc are discussed. In the case of cones, the observations are compared with numerical simulations based on a simplistic light-scattering model. Ultimately, the method may have diagnostic potential for diseases that perturb the microscopic structure of the retina.

Recovery of macular cone photoreceptors in Vogt-Koyanagi-Harada disease.

Our purpose was to study changes in macular cone photoreceptors in Vogt-Koyanagi-Harada (VKH) disease patients after high-dose corticosteroid treatment using an adaptive optics (AO) fundus camera. We retrospectively analyzed 16 eyes of eight patients with new-onset acute VKH disease that were studied retrospectively. After serous retinal detachment (SRD) had resolved, AO images were obtained using the rtx1™ AO fundus camera over a 12-month course. Cone counting was performed in the nasal, temporal, superior and inferior areas of the macula 0.75mm from the foveal center. A control group of 30 eyes of 30 healthy subjects was established for comparison. In the eyes with VKH disease, the mean cone densities 0.75mm from the foveal center were 11,847 at baseline (resolution of SRD), and 15,218, 16,684 and 17,686 cones/mm2, at 3, 6, and 12months later, respectively. The mean cone densities at 3, 6, and 12months were significantly increased compared to those of baseline (p=0.007, p<0.001, and p<0.001, respectively); however, they were significantly lower than that of the healthy control eyes (p<0.001). The mean cone densities at areas with a previous presence of cystoid spaces were significantly lower than those without cystoid spaces at the baseline, and at 3, 6, and 12months (p<0.001, p=0.007, p<0.001, and p=0.008, respectively). Cone densities were gradually increased after the resolution of SRD in the eyes of VKH disease patients. The presence of cystoid spaces might be a marker of severe damage to cone photoreceptors.

High resolution imaging analysis of female carriers and patients of Choroideremia with CHM gene mutation

PurposeTo report the high resolution imaging features of Japanese patient and female carriers of choroideremia using adaptive optics(AO) and other imaging modalities.MethodsThree carriers and one patient underwent comprehensive examinations including AO fundus camera (rtx1™ Imagine eyes, France), spectral domain optical coherence topography (SD‐OCT)and fundus autofluoresence (FAF). Cone density was measured by peak density method and compared with the data of 34 normal controls. The mutation analysis of the CHM gene was performed by Sanger sequencing. The protocol conformed to the tenets of the Declaration of Helsinki and was approved by the IRB of The Jikei University and Nippon Medical School.ResultsThree female carriers 1, 2, 3 (sister 9 y.o, mother 37 y.o and grandmother 65 y.o) and one patient (6 y.o, male). They had same CHM mutation, c.646delA, p.T216LfsX16. All carriers did not complain any symptoms, the BCVA of all were over 20/20. Fundus examination of carrier 2 showed mild generation in only periphery, the other carriers showed diffuse degeneration. AF showed mottled hypo‐autofluoresence in degeneration area. SD‐OCT of the carriers showed nearly normal,although peripheral SD‐OCT and patient showed diffuse disruptions of interdigitation and ellipsoid zones.Cone density was measured at 2 to 8 degree temporal from the fovea in every 1 degree, using 50 x 50 micron images. Cone counting was performed by AO detect (Imagine eyes) with manual correction. Patient showed lower cone density more than 2 SD of normal control. The carriers showed lower cone density only at 7 and 8 degrees.ConclusionsAO cone counting showed reduction of photoreceptors in carriers mainly at peripheral region. To elucidate the mechanism of peripheral degeneration and foveal sparing of female carriers may help preventing the disease progression of choroideremia patients.

Regeneration of Photoreceptor Outer Segments After Scleral Buckling Surgery for Rhegmatogenous Retinal Detachment

To investigate the regeneration of the coneouter segments in eyes after surgery for fovea-off rhegmatogenous retinal detachment with an adaptive optics (AO) fundus camera and to correlate these findingswith the findings of optical coherence tomography (OCT). Retrospective, observational case series. Medical charts of 21 eyes of 21 patients who had undergone surgery for fovea-off rhegmatogenous retinal detachment were retrospectively studied. Cone mosaic images were obtained with an AO fundus camera. Cone packing density at 2 degrees from the fovea within the previously detached area was measured 6 and 12months after surgery. Retinal thicknesses between the interdigitation zone and the retinal pigment epithelium (IZ-RPE) and between the ellipsoid zone and the retinal pigment epithelium (EZ-RPE) were measured in OCT images. Cone density 12months after surgery was significantly increased from that at 6months (P=.001), but was still significantly lower than that of normal fellow eyes (P < .001). IZ-RPE and EZ-RPE thickness significantly increased from 6 to 12months (P= .045, P= .033, respectively), and these values were not significantly different from those of normal fellow eyes. Multivariate analysis showed that cone density at 12months was significantly associated with IZ-RPE thickness (P= .002), and increases in cone packing density were significantly associated with increases in IZ-RPE thickness (P= .001). Recovery of cone packing density measured by AO was associated with structural recovery of the outer retina observed in OCT, suggesting regeneration of the photoreceptor outer segment after surgery.

Correlation between outer retinal layer thickness and cone density in patients with resolved central serous chorioretinopathy.

We aimed to observe photoreceptors and outer retinal layer thickness by using an adaptive optics (AO) fundus camera and spectral domain optical coherence tomography (SD-OCT) in patients with central serous chorioretinopathy (CSC). Twelve eyes of 12 patients with resolved CSC were studied. After subretinal fluid (SRF) had resolved, AO images of the macula were obtained by using the rtx1TM AO fundus camera (Imagine Eyes, France). Cones in the nasal and temporal areas of the macula, 0.5mm from the foveal center, were counted. Outer retinal layer thickness was measured as the distance between the outer border of external limiting membrane (ELM) and the inner border of retinal pigment epithelium (RPE) at 0.5mm from the foveal center. The first measurement was performed after resolution of the subretinal fluid and was defined as the baseline. Then, the second measurement was performed in the same location after 12months. In the eyes with resolved CSC, the mean logMAR values of visual acuity at baseline and 12months were 0.16 and 0.01, respectively (p = 0.0023). The mean cone densities at baseline and 12months were 12,213 and 17,146 cones/mm2, respectively. The mean cone densities at 12months were significantly increased compared to baseline (p = 0.0014). The mean outer retinal layer thickness at baseline (53.5μm) was significantly thinner than that at 12months (60.7μm) (p = 0.0013). The mean cone densities were significantly correlated with outer retinal layer thickness and logMAR visual acuity. Adaptive optics imaging revealed a gradual increase in the number of macular cone densities during 12months in patients with resolved CSC, which was correlated with outer retinal layer thickness and visual acuity in a short term.

Multi-modal adaptive optics system including fundus photography and optical coherence tomography for the clinical setting

We present a new compact multi-modal imaging prototype that combines an adaptive optics (AO) fundus camera with AO-optical coherence tomography (OCT) in a single instrument. The prototype allows acquiring AO fundus images with a field of view of 4°x4° and with a frame rate of 10fps. The exposure time of a single image is 10 ms. The short exposure time results in nearly motion artifact-free high resolution images of the retina. The AO-OCT mode allows acquiring volumetric data of the retina at 200kHz A-scan rate with a transverse resolution of ~4 µm and an axial resolution of ~5 µm. OCT imaging is acquired within a field of view of 2°x2° located at the central part of the AO fundus image. Recording of OCT volume data takes 0.8 seconds. The performance of the new system is tested in healthy volunteers and patients with retinal diseases.

High-Resolution Imaging of Parafoveal Cones in Different Stages of Diabetic Retinopathy Using Adaptive Optics Fundus Camera.

PurposeTo assess cone density as a marker of early signs of retinopathy in patients with type II diabetes mellitus.MethodsAn adaptive optics (AO) retinal camera (rtx1™; Imagine Eyes, Orsay, France) was used to acquire images of parafoveal cones from patients with type II diabetes mellitus with or without retinopathy and from healthy controls with no known systemic or ocular disease. Cone mosaic was captured at 0° and 2°eccentricities along the horizontal and vertical meridians. The density of the parafoveal cones was calculated within 100×100-μm squares located at 500-μm from the foveal center along the orthogonal meridians. Manual corrections of the automated counting were then performed by 2 masked graders. Cone density measurements were evaluated with ANOVA that consisted of one between-subjects factor, stage of retinopathy and the within-subject factors. The ANOVA model included a complex covariance structure to account for correlations between the levels of the within-subject factors.ResultsTen healthy participants (20 eyes) and 25 patients (29 eyes) with type II diabetes mellitus were recruited in the study. The mean (± standard deviation [SD]) age of the healthy participants (Control group), patients with diabetes without retinopathy (No DR group), and patients with diabetic retinopathy (DR group) was 55 ± 8, 53 ± 8, and 52 ± 9 years, respectively. The cone density was significantly lower in the moderate nonproliferative diabetic retinopathy (NPDR) and severe NPDR/proliferative DR groups compared to the Control, No DR, and mild NPDR groups (P < 0.05). No correlation was found between cone density and the level of hemoglobin A1c (HbA1c) or the duration of diabetes.ConclusionsThe extent of photoreceptor loss on AO imaging may correlate positively with severity of DR in patients with type II diabetes mellitus. Photoreceptor loss may be more pronounced among patients with advanced stages of DR due to higher risk of macular edema and its sequelae.

In vivo characterization of lamina cribrosa pore morphology in primary open-angle glaucoma

To characterize the in vivo morphology of human lamina cribrosa pores in healthy and glaucoma eyes. In this cross-sectional, observational study, a flood-illumination adaptive optics fundus (FIAO) camera was used to perform in vivo, high-resolution, noninvasive imaging of the optic disc and lamina cribrosa in 30 patients diagnosed with primary open-angle glaucoma (POAG), in 15 healthy controls and in 14 healthy subjects with at least one direct relative with POAG. Two masked graders measured each visible lamina cribrosa pore along the major and minor axes in order to categorize pores as oval (minor/major axis ratio<0.75) or round. We used these same measurements to calculate pore surface area as a best-fit oval. Lamina cribrosa pores were visible in 95.2% of the subjects. In 52% of controls, the pores were visualized under the neuroretinal rim. In POAG patients, 78% of visible pores had an oval shape versus 19.4% in controls (P<0.01). Average pore surface area was significantly different (1561 px(2) versus 724 px(2); P<0.01). In healthy subjects with at least one direct relative with POAG, 21% had pores with an appearance comparable to that of subjects in the glaucoma group. On average, lamina cribrosa pores are elongated in POAG eyes and also in healthy eyes of POAG relatives. In vivo characterization of lamina cribrosa pore morphology by FIAO imaging may enhance our understanding of glaucoma, and offer new means for its early detection.

The improvement of Spoke‐Wheel pattern foveoschisis in a patient with X‐linked retinoschisis treated with topical dorzolamide observed by high‐resolution adaptive optics camera

PurposeThe purpose of the study is to report the improvement of foveomacular cavities and spoke‐wheel pattern retinoschisis observed by spectral‐domain optical coherence topmography (SD‐OCT) and high‐resolution adaptive optics fundus camera in a patient with XLRS treated with topical dorzolamide.MethodsA 42 y.o. man with XLRS underwent detailed ophthalmic examinations. A mutation of RS1 gene was detected earlier. The ophthalmological examinations included SD‐OCT, fundus autofluorescence imaging, and full‐field and multifocal ERGs. Fundus images with microscopic resolution were obtained using the AO retinal camera (rtx1, Imagine Eyes, France). He was treated with topical dorzolamide three times a day. Transverse foveomacular cavities was observed by SD‐OCT and the en‐face images of spoke‐wheel pattern foveoschisis was observed by AO fundus camera during a follow‐up period.ResultsHis BCVA was 0.15 in the right eye and 0.3 in the left eye. The right eye showed atrophic macular degeneration and left eye showed spoke‐wheel pattern foveoschisis. SD‐OCT showed the thinning of retinal thickness in the right eye and cystoid foveoschisis in the left eye. AO images showed spoke‐wheel pattern retinal fold in the left eye. The spoke‐wheel pattern in AO was sharper compared to the images obtained by fundus photography and autofluorescence imaging. After 14 month of treatment with topical dorzolamid, improvement of foveomacular cavities in SD‐OCT was observed. The spoke‐wheel pattern retinal fold in AO become obscure after treatment, however still detectable even after foveomacular cavities in SD‐OCT was almost disappeared.ConclusionsAO imaging showed detailed microstructure of spoke‐wheel pattern foveoschisis and their improvement during a follow‐up period. AO imaging may be helpful in clarifying the pathology of the foveoschisis in XLRS.

Detailed Morphological Changes of Foveoschisis in Patient with X-Linked Retinoschisis Detected by SD-OCT and Adaptive Optics Fundus Camera.

Purpose. To report the morphological and functional changes associated with a regression of foveoschisis in a patient with X-linked retinoschisis (XLRS). Methods. A 42-year-old man with XLRS underwent genetic analysis and detailed ophthalmic examinations. Functional assessments included best-corrected visual acuity (BCVA), full-field electroretinograms (ERGs), and multifocal ERGs (mfERGs). Morphological assessments included fundus photography, spectral-domain optical coherence tomography (SD-OCT), and adaptive optics (AO) fundus imaging. After the baseline clinical data were obtained, topical dorzolamide was applied to the patient. The patient was followed for 24 months. Results. A reported RS1 gene mutation was found (P203L) in the patient. At the baseline, his decimal BCVA was 0.15 in the right and 0.3 in the left eye. Fundus photographs showed bilateral spoke wheel-appearing maculopathy. SD-OCT confirmed the foveoschisis in the left eye. The AO images of the left eye showed spoke wheel retinal folds, and the folds were thinner than those in fundus photographs. During the follow-up period, the foveal thickness in the SD-OCT images and the number of retinal folds in the AO images were reduced. Conclusions. We have presented the detailed morphological changes of foveoschisis in a patient with XLRS detected by SD-OCT and AO fundus camera. However, the findings do not indicate whether the changes were influenced by topical dorzolamide or the natural history.

Quantitative Analysis of Cone Photoreceptor Distribution and Its Relationship with Axial Length, Age, and Early Age-Related Macular Degeneration

PurposeIt has not been clarified whether early age-related macular degeneration (AMD) is associated with cone photoreceptor distribution. We used adaptive optics fundus camera to examine cone photoreceptors in the macular area of aged patients and quantitatively analyzed its relationship between the presence of early AMD and cone distribution.MethodsSixty cases aged 50 or older were studied. The eyes were examined with funduscopy and spectral-domain optical coherence tomography to exclude the eyes with any abnormalities at two sites of measurement, 2° superior and 5° temporal to the fovea. High-resolution retinal images with cone photoreceptor mosaic were obtained with adaptive optics fundus camera (rtx1, Imagine Eyes, France). After adjusting for axial length, cone packing density was calculated and the relationship with age, axial length, or severity of early AMD based on the age-related eye disease study (AREDS) classification was analyzed.ResultsPatient’s age ranged from 50 to 77, and axial length from 21.7 to 27.5 mm. Mean density in metric units and that in angular units were 24,900 cells/mm2, 2,170 cells/deg2 at 2° superior, and 18,500 cells/mm2, 1,570 cels/deg2 at 5° temporal, respectively. Axial length was significantly correlated with the density calculated in metric units, but not with that in angular units. Age was significantly correlated with the density both in metric and angular units at 2° superior. There was no significant difference in the density in metric and angular units between the eyes with AREDS category one and those with categories two or three.ConclusionAxial length and age were significantly correlated with parafoveal cone photoreceptor distribution. The results do not support that early AMD might influence cone photoreceptor density in the area without drusen or pigment abnormalities.