Retinal Phenotype in Mucopolysaccharidosis Type III.

PurposeThis study was performed to investigate the interocular symmetry of peripapillary retinal nerve fibre layer (RNFL) thickness, as measured by Cirrus high‐definition spectral‐domain optical coherence tomography (OCT), in healthy eyes. A wide range of subject ages and refractive errors was examined.MethodsThe retinal nerve fibre layer thickness was measured in 1,234 healthy eyes from 617 subjects using OCT. Interocular differences (right eye minus left eye) in global area and quadrant nerve fibre layer thicknesses were measured. The effect of age and refractive error on interocular nerve fibre layer thickness difference was also examined.ResultsMeans (and standard deviations) of subjects' ages and average subject refractive errors were 36.4 ± 19.8 years (range: five to 80 years) and ‐2.67 ± 2.91 (range: ‐14.13 to +5.75 D), respectively. Cutoff limits for normal interocular nerve fibre layer thickness differences (2.5th and 97.5th percentiles of normative data) in the global area and in the superior, nasal, inferior and temporal quadrants were 9.5, 23.0, 15.6, 20.0 and 22.6 μm, respectively. Mean interocular retinal nerve fibre layer thickness differences in global area and in superior, nasal, inferior and temporal quadrants were 0.7 (p < 0.001), ‐3.9 (p < 0.001), 2.6 (p < 0.001), 1.1 (p = 0.007) and 3.0 (p < 0.001) μm, respectively. Interocular nerve fibre layer thickness differences were not significantly correlated with age or refractive error (average of right and left eyes, both p > 0.05).ConclusionsSignificant interocular differences in peripapillary retinal nerve fibre layer thickness exist in healthy eyes, particularly in the superior quadrant. This finding should be considered when comparing retinal nerve fibre layer thickness between right and left eyes.

Objetivo: Avaliar o efeito da cirrose na camada de fibras nervosas da retina e na espessura da coroide através da tomografia de coerência óptica com imagem de profundidade aprimorada. Métodos: Este estudo transversal, de único centro, foi realizado no departamento de Oftalmologia da Universidade Bulent Ecevit com a participação do departamento de medicina interna em gtastroenterologia. Os pacientes que foram tratados com o diagnóstico de cirrose (n = 75) foram examinados na clínica da oftalmologia. Foram incluídos pacientes correspondentes em [...]

This study evaluated the effects of intravitreal injection of fasudil (IVF), a Rho-kinase inhibitor, in cases of recent-onset nonarteritic anterior ischemic optic neuropathy (NAION). In this interventional case series, 13 eyes of 13 patients diagnosed with NAION within 14 days of onset were included. The affected eyes received a 0.025 mg/0.05 mL IVF. Functional and structural outcomes were assessed 1 and 3 months following treatment. Best corrected visual acuity (BCVA) was the main outcome measured, with mean deviation (MD) index of the VF test and peripapillary retinal nerve fiber layer thickness as secondary measures. There was a statistically significant improvement in the patients' BCVA 1 and 3 months following IVF; BCVA improved from 1.69 ± 0.55 logMAR at baseline to 0.98 ± 0.47 and 0.93 ± 0.51 logMAR at 1 and 3 months, respectively (P = .004). The change in BCVA was not significant between month 1 and month 3 (P = .22). Peripapillary retinal nerve fiber layer thickness decreased from 173.5 ± 29.28 µm in the baseline evaluation to 85.8 ± 8.8 µm at 1 month, and 62.9± 5.97µm at 3 months (P = .003). MD values changed from 24.60 ± 3.80 to 21.0 ± 6.10 and 20.5 ± 6.50 at 1 and 3 months, respectively (P = .007 and .005, respectively). This pilot study suggests that IVF may be an effective treatment for patients with recent-onset NAION. Larger studies are required to establish the therapeutic role of fasudil for NAION.

Background: Electroconvulsive therapy (ECT) is a method commonly used in the treatment of psychiatric disorders. As ocular side effects from OCT are less studied and discussed, the aim of this study was to evaluate the profile of the peripapillary retinal nerve fiber layer thickness in patients receiving ECT. Method: This study was performed on 30 patients who had indication for ECT. After recording demographic data, retinal nerve fiber layer thickness was measured by using spectral domain optical coherence tomography (Heidelberg Engineering, Heidelberg, Germany) of optic nerve head and peripapillary retinal nerve fiber layer. Results: Our results showed that for most of the studied variables, the normal range was observed in less than 85% of patients receiving ECT. Variables which were outside the normal range were: cup disk (C/D) ratio (right and left eyes were 50% and 46.7%, respectively), vertical C/D ratio (right and left eyes were 53.3% and 46.7%, respectively), and cup volume (right and left eyes were 53.3% and 33.3%, respectively). Conclusion: This study showed that a high percentage of patients receiving ECT had changes in the optic nerve head while intra-ocular pressure in these patients was normal. It is warranted for examiners to pay attention to diagnosis of normal tension glaucoma in patients receiving ECT.

In this retrospective cross-sectional study, we quantitatively analyzed the tomographic features in the neural tissues around the optic disc in patients with diabetic retinopathy with and without panretinal photocoagulation. We analyzed 206 eyes, comprising 33 normal eyes in subjects without diabetes (group I), 30 eyes without diabetic retinopathy (group II), 66 eyes with non-proliferative diabetic retinopathy (group III), 45 eyes with panretinal photocoagulation (group IV), and 32 eyes with normal tension glaucoma (group V). Sequential images acquired using swept-source optical coherence tomography in three-dimensional mode were used to measure peripapillary retinal nerve fiber layer thickness, neuro-retinal rim thickness, anterior lamina cribrosa depth, prelaminar thickness, and thickness of the lamina cribrosa. The peripapillary retinal nerve fiber layer thickness and lamina cribrosa thickness were significantly thinner in group IV than in group III (p = 0.019 and p < 0.001). However, there was no significant difference in rim thickness, anterior lamina cribrosa depth, or prelaminar thickness between groups III and IV (p = 0.307, p = 0.877, and p = 0.212). Multivariate analysis revealed that time since panretinal photocoagulation and thickness of the lamina cribrosa had a significant effect on peripapillary retinal nerve fiber layer thickness (p < 0.001 and p = 0.014). In group IV, there was a negative correlation between time elapsed since panretinal photocoagulation and peripapillary retinal nerve fiber layer thickness, rim thickness, and thickness of the lamina cribrosa (r = -0.765, r = -0.490, and r = -0.419), but no correlation with prelaminar thickness or anterior lamina cribrosa depth (r = 0.104 and r = -0.171). Panretinal photocoagulation may be related to thinning of the peripapillary retinal nerve fiber layer, rim thickness, and lamina cribrosa, but not prelaminar thickness or anterior lamina cribrosa depth. These features are different from the peripapillary features of eyes with typical normal tension glaucoma.

Optic neuropathy is a near ubiquitous feature of Friedreich's ataxia (FRDA). Previous studies have examined varying aspects of the anterior and posterior visual pathways but none so far have comprehensively evaluated the heterogeneity of degeneration across different areas of the retina, changes to the macula layers and combined these with volumetric MRI studies of the visual cortex and frataxin level. We investigated 62 genetically confirmed FRDA patients using an integrated approach as part of an observational cohort study. We included measurement of frataxin protein levels, clinical evaluation of visual and neurological function, optical coherence tomography to determine retinal nerve fibre layer thickness and macular layer volume and volumetric brain MRI. We demonstrate that frataxin level correlates with peripapillary retinal nerve fibre layer thickness and that retinal sectors differ in their degree of degeneration. We also shown that retinal nerve fibre layer is thinner in FRDA patients than controls and that this thinning is influenced by the AAO and GAA1. Furthermore we show that the ganglion cell and inner plexiform layers are affected in FRDA. Our MRI data indicate that there are borderline correlations between retinal layers and areas of the cortex involved in visual processing. Our study demonstrates the uneven distribution of the axonopathy in the retinal nerve fibre layer and highlight the relative sparing of the papillomacular bundle and temporal sectors. We show that thinning of the retinal nerve fibre layer is associated with frataxin levels, supporting the use the two biomarkers in future clinical trials design. © 2022 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

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An investigation of retinal layer thicknesses in unaffected first-degree relatives of schizophrenia patients

To comparatively evaluate the subfoveal choroidal thickness and the peripapillary retinal nerve fiber layer thickness in patients with chronic heart failure relative to control subjects. A total of 72 chronic heart failure patients and 40 healthy control subjects were enrolled in this study. The patients were categorized into 2 groups: group 1: patients with 30-50% left ventricle ejection fraction and group 2: patients with the corresponding fraction value of <30%. The subfoveal choroidal thickness and the peripapillary retinal nerve fiber layer thickness were measured by spectral domain-optical coherence tomography. The mean subfoveal choroidal thickness was 250.24 ± 68.34 µm in group 1 and 216.72 ± 71.24 µm in group 2, while it was 273.64 ± 77.68 µm in the control group. The differences among the 3 groups were statistically significant. The average peripapillary retinal nerve fiber layer thicknesses were 100.34 ± 8.24, 95.44 ± 6.67, and 102.34 ± 8.24 µm, respectively. No significant differences were noted in the peripapillary retinal nerve fiber layer thicknesses between group 1 and control group, but it was significantly lower in group 2. Our study thus revealed that the subfoveal choroidal thickness was lower in patients belonging to both the chronic heart failure groups in comparison to those in the control group. However, the alteration in the peripapillary retinal nerve fiber layer thickness was noted in only patients with <30% left ventricle ejection fraction. In the clinical practice, reductions in these values are correlated with decreased left ventricle ejection fraction, which may be important for the follow-up of chorioretinal diseases and the evaluation of glaucoma risks in patients with chronic heart failures.

To investigate the association between macular thickness and peripapillary retinal nerve fiber layer (RNFL) thickness in Chinese children.This cross-sectional study recruited consecutive cases of healthy pediatric subjects aged 4 to 18 from Caritas Medical Centre in Hong Kong Special Administrative Region, China, from 2013 to 2014. Subjects with only eye, ocular tumors, congenital glaucoma, congenital cataract, congenital nystagmus, microphthalmos, optic nerve or retinal disease, active ocular infections, corneal scars, and severe visual impairment of any cause were excluded. Peripapillary RNFL thickness and macular thickness at 1-mm-diameter fovea center (C1), 3-mm-diameter temporal quadrant (T3), and 3-mm- diameter nasal quadrant (N3) were measured with optical coherence tomography. Best-corrected visual acuity, axial length, and cycloplegic refraction were also recorded. Spearman correlation was used to analyze the association between T3, C1, and N3 with each of the following: average and quadrant RNFL thickness, axial length, and spherical equivalent.In 179 subjects, the mean age was 7.9 ± 3.6 years. There were 90 male and 89 female subjects, all of Chinese ethnicity. The mean spherical equivalent was −0.1 ± 3.1 D and mean axial length was 22.9 ± 1.4 mm. There were significant and positive correlations of the average (T3: r = 0.20, P = 0.04; N3: r = 0.2, P = 0.005), superior (T3: r = 0.20, P = 0.03; N3: r = 0.2, P = 0.03), and inferior (T3: r = 0.20, P = 0.02; N3: r = 0.2, P = 0.01) peripapillary RNFL thicknesses with the T3 and N3 macular thicknesses but not C1. The nasal peripapillary RNFL thickness was also positively correlated with T3 (r = 0.20, P = 0.01). There were no significant associations between the macular thickness (T3, C1, N3) with neither the spherical equivalent (P > 0.2) nor the axial length (P > 0.3).The macular thickness was positive correlated with the peripapillary RNFL thickness in a population of healthy Chinese children.

To assess the peripapillary retinal nerve fiber layer (RNFL) thickness, optic nerve head (ONH) morphologic parameters, and macular thickness and volume in patients affected by obstructive sleep apnea-hypopnea syndrome (OSAHS). This prospective, observational case-control study consisted of 96 eyes of 50 OSAHS patients (mean age of 50.9 ± 12.4 years, best-corrected visual acuity ≥ 20/20, refractive error less than 3 spherocylindrical diopters, and intraocular pressure <21 mmHg) who were enrolled and compared with 64 eyes of 33 age-matched controls. Peripapillary RNFL thickness, ONH parameters, macular thickness and volume were measured by optical coherence tomography (OCT). OSAHS patients showed a significant reduction of the nasal quadrant RNFL thickness (74.7 ± 15.8 μm) compared with those values observed in control patients (81.1 ± 16.6 μm, p=0.047, Student's t-test). No differences in peripapillary RNFL thickness were observed when dividing the OSAHS group in accordance with disease severity. Vertical integrated rim area (VIRA) (0.67 ± 0.41 mm(3) in OSAHS vs 0.55 ± 0.29 mm(3) in controls; p=0.043, Student's t-test), horizontal integrated rim width (HIRW) (1.87 ± 0.31 mm(2) in OSAHS vs 1.8 ± 0.25 mm(2) in controls; p=0.039, Student's t-test) and disc area (2.74 ± 0.62 mm(2) in OSAHS vs 2.48 ± 0.42 mm(2) in controls; p=0.002, Student's t-test) showed significant differences, all of them being higher in the OSAHS group. Severe OSAHS had significant higher disc area (2.8 ± 0.7 mm(2)) than controls (2.5 ± 0.4 mm(2); p=0.016, ANOVA test). Temporal inner macular thickness was significantly higher in mild-moderate OSAHS patients (270 ± 12 μm) than in severe OSAHS patients (260 ± 19 μm; p=0.021, ANOVA test). OSAHS patients showed decreased peripapillary nasal RNFL thickness, and increased ONH area and volume parameters when they were evaluated by OCT. These findings suggest that neuronal degeneration might be present in the retina of OSAHS patients, as previously observed in some neurodegenerative disorders.

To find optical coherence tomographic parameters related with visual acuity, and, thus, which might be used as objective signs to predict visual acuity after future treatment, for example retinal prosthesis implantation, 86 eyes of 45 consecutive patients with retinitis pigmentosa, who showed no macular diseases, underwent optical coherence tomography to measure macular retinal thickness and peripapillary retinal nerve fiber layer (RNFL) thickness, and to obtain horizontal cross-sectional images at the fovea for observation of the inner segment/outer segment (IS/OS) junction line. Best-corrected visual acuity was significantly better either in the right eyes or in the left eyes with greater retinal thickness of all four quadrants of the macular area in the inner ring, encompassing 1-3mm from the foveal center (P<0.05, Spearman rank correlation test), and also with the presence of the IS/OS line at the fovea (P<0.0001, Wilcoxon-Kruskal-Wallis rank sum test). Retinal average thickness in the posterior pole covering the 6×6mm square area was positively correlated with peripapillary RNFL average thickness either in the right eyes or in the left eyes (P<0.05). The average thickness of the peripapillary RNFL became significantly less with age (P<0.05), but was not related with visual acuity. Macular retinal thickness and the presence of the IS/OS line, but not peripapillary RNFL thickness, could serve as objective signs for better visual acuity in retinitis pigmentosa. The macular retinal thickness might be used as an objective predictor to choose patients with retinitis pigmentosa who would be expected to gain vision after retinal prosthesis implantation.

Background/Aim. One of the most reliable methods for structural measurements of glaucomatous damage is spectral domain optical coherence tomography (SD-OCT). The aim of this study was to measure peripapillary retinal nerve fiber layer (RNFL) thickness with SD-OCT in eyes with different stages of glaucoma, as well as to determine which sector in the peripapillary circle is the most vulnerable to glaucomatous damage. Methods. The study included 153 eyes of 93 patients with confirmed primary open angle glaucoma (POAG). All the patients underwent a complete ophthalmic examination, including visual field testing and peripapillary RNFL thickness measured by SD-OCT. They were divided into three subgroups: early, moderate and severe stage of glaucoma based on the mean deviation (MD) index of visual field. The results were presented as mean RNFL thickness: total, in the four quadrants and 12 clock-hour RNFL thickness. Results. The overall mean peripapillary RNFL was 74.95 ? 14.51 ?m. The lower quadrant had the thickest RNFL (92.78 ? 25.84 ?m), followed by upper (88.82 ? 22.04 ?m), nasal (64.31 ? 11.67 ?m) and temporal ones (54.02 ? 12.76 ?m), showing a significant difference (?2 = 273.36, DF = 3, p &lt; 0.001). Comparison between RNFL thickness in early glaucoma and moderate and severe stages revealed that the most sensitive sectors were inferior and superior ones, as well as sectors at 5?7 clock hour position. The greatest decrease in RNFL thickness was observed in the 9 o'clock hour sector in all three glaucoma subgroups (46.99 ? 13.28 ?m), while the RNFL was the thickest in the 6 o'clock hour sector (102.63 ? 34.12 ?m). Conclusion. Peripapillary RNFL thickness is inversely proportional to the degree of glaucomatous damage: the greater the damage, the thinner peripapillary RNFL.

Context: The retina of diabetic patients undergoes neurodegenerative changes, in addition to the vascular changes. Aims: The aim of this study is to evaluate the peripapillary retinal nerve fiber layer (RNFL) thickness in type 2 diabetes mellitus (DM) patients compared to healthy controls, using optical coherence tomography (OCT) and to correlate it with glycemic control. Settings and Design: Prospective study. Subjects and Methods: A total of 90 patients were enrolled in the study. The study group included 120 eyes of 60 type 2 DM patients. Sixty eyes of 30 healthy participants served as the control group. All patients underwent an OCT scan for peripapillary RNFL thickness measurement. Metabolic control of DM was assessed using glycosylated hemoglobin (HbA1c). Statistical Analysis Used: Independent sample t-test was used for normally distributed variables. Pearson's correlation analysis was used to assess the correlation between HbA1c and RNFL thickness. Results: Right eye RNFL thickness was thinner in diabetic patients than controls, with a statistically significant P value (P = 0.002) in the superior quadrant and inferior quadrant (P = 0.019). The average, superior, and inferior RNFL thickness in the left eye was significantly thinner in diabetic patients as compared to controls (P &lt; 0.001, P = 0.001, and P &lt; 0.001 respectively). No significant correlation was found between HbA1c and RNFL thickness. Conclusions: The peripapillary RNFL is thinner in diabetic patients as compared to controls. This is the first study of its kind, among diabetic patients in Goa, which makes this study unique.

BackgroundThough a newly developed spectral domain optical coherence tomography (OCT) is at the center of interests for many ophthalmologic researchers and clinicians, its own characteristics are not fully evaluated yet. The main purpose of this study was to establish the adjusted color probability codes for peripapillary retinal nerve fiber layer (RNFL) thickness in healthy Koreans and to compare them with original color codes provided by spectral domain OCT.MethodsTwo hundred ninety-five healthy Korean eyes were enrolled and their peripapillary RNFL thickness was measured by Cirrus OCT. For each decade of age, the normal thickness reference was determined on the basis of z-scores and the adjusted color probability codes were established. Then the agreements between adjusted and original color codes were calculated using weighted Kappa (Kw) coefficient.ResultsOn the basis of Kw coefficient, the overall agreement between the adjusted and original probability color codes was not excellent (Kw range of 0.500 to 0.806). If the adjusted probability codes were assumed as a standard of comparison, the original color codes showed the false-negative in 11% of eyes and the false-positive in 0.3% of eyes for average RNFL thickness.ConclusionsAdjusted color probability codes judged by the Korean normative data showed a discrepancy with original codes. It implies that normal reference and adjusted probability codes for each ethnicity might be needed to determine whether a certain RNFL thickness is within normal range or not.