Outer nuclear layer relevance in visual function correlated to quantitative enface OCT parameters in Stargardt disease

2021 ◽  
pp. 112067212199057
Author(s):  
Dario Pasquale Mucciolo ◽  
Myrta Lippera ◽  
Dario Giorgio ◽  
Andrea Sodi ◽  
Ilaria Passerini ◽  
...  

Purpose: To evaluate the correlation between Best Corrected Visual Acuity (BCVA) and the following parameters in Stargardt Disease (STGD): Central Retinal Thickness (CR-T), Central Outer Nuclear Layer Thickness (C-ONL-T), Areas of macular Photoreceptor loss (PHRa), and Retinal Pigment Epithelium (RPE) loss (RPEa). Methods: A total of 64 eyes of 32 STGD patients were included in the study. All patients received a comprehensive ophthalmological examination, color fundus photographs, fundus auto-fluorescence imaging, and Optical Coherence Tomography (OCT). The CR-T and C-ONL-T were evaluated from standard SD-OCT scans. The PHRa and RPEa were calculated from enface OCT scans (sub RPE slab and photoreceptor slab). The collected OCT parameters were evaluated for possible association with BCVA. Results: The mean macular PHRa and RPEa was 16.16 ± 13.36 and 12.05 ± 12.57 mm2 respectively. The mean CR-T measured 120.78 ± 41.49 μm while the mean C-ONL-T was assessed at 4.60 ± 13.73 μm. BCVA showed the highest correlation with the C-ONL-T ( r = −0.72; p < 0.001) while there was no correlation with the CR-T ( r = −0.17; p = 1.00). Conclusions: Enface OCT permits a rapid and precise quantitative evaluation of the macular PHR and RPE atrophy area in STGD. Nonetheless, the OCT parameter that showed the highest correlation with visual acuity in STGD was the ONL thickness.

2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Edoardo Abed ◽  
Giorgio Placidi ◽  
Luigi Calandriello ◽  
Marco Piccardi ◽  
Francesca Campagna ◽  
...  

Stargardt disease (STGD1) is the most common cause of inherited juvenile macular degeneration. This disease is characterized by a progressive accumulation of lipofuscin in the outer retina and subsequent loss of photoreceptors and retinal pigment epithelium. The aim of this study was to evaluate the relationship between cone photoreceptor function and structure in STGD1. Macular function was assessed by visual acuity measurement and focal electroretinogram (FERG) recording while spectral domain optical coherence tomography (SD-OCT) imaging was performed to evaluate the integrity of photoreceptors. FERG amplitude was significantly reduced in patients with Stargardt disease (p<0.0001). The amplitude of FERG showed a negative relationship with interruption of ellipsoid zone (EZ) (R2=0.54, p<0.0001) and a positive correlation with average macular thickness (AMT). Conversely, visual acuity was only weakly correlated with central macular thickness (CMT) (R2=0.12, p=0.04). In conclusion, this study demonstrates that FERG amplitude is a reliable indicator of macular cone function while visual acuity reflects the activity of the foveal region. A precise assessment of macular cone function by FERG recording may be useful to monitor the progression of STGD1 and to select the optimal candidates to include in future clinical trials to treat this disease.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 711.1-711
Author(s):  
D. Monova ◽  
S. Monov ◽  
D. Dimova ◽  
V. Tzankov ◽  
M. Sotirova

Background:Systemic sclerosis (SSc) is a connective tissue disease with heterogeneous manifestations. It affects different organs and therefore requires interdisciplinary diagnostic and therapeutic management.Objectives:The aim of this study is to evaluate the frequency and characteristics of ocular manifestation in patients with systemic sclerosis.Methods:The study involved 31 patients with SSc. All the study subjects underwent complete ophthalmological examination involving visual acuity assessment, examination of anterior and posterior eye segments, Schirmer I test, diameter and mobility of pupils, as well as eyeball mobility assessment of intraocular pressure and ultrasound assessment of vitreous body. Data regarding age, gender, SSc subtype, disease duration, age at diagnosis, nailfold capillaroscopic pattern, systemic corticosteroid or chloroquine use, blood pressure, ocular symptoms and detailed ophthalmic history were recorded.Results:31 patients (3 male, 28 female, mean age 42,7 ± 14,3 years; mean disease duration 10,3±8,1 years) were enrolled in this study. 7 (22,58 %) of them had no ocular symptoms. Among the patients with ocular symptoms, 20 (64.52%) complained of decreased vision, 13 (41,93 %) - of itching, 14 (45,16%) - of burning, 8 (25,81%) - of eye fatigue,4 (12,90 %) - of ocular pain, 4 (12,90%) - of foreign body sensation, 16 (51,61 %) - of dry eye, 5 (52,03 %) -photophobia, 2 (6,45 %) - of floaters, 10 (32,26 %) - of redness. Hardening and thickening of palpebral skin was noted in 27 (87,10 %) patients. Ophthalmological examination revealed higher incidence of the following abnormalities in the study group: myopic astigmatism - in 20 (32,26 %) eyes, vascular abnormalities within fundus - in 24 (38,71%) eyes, increased intraocular pressure (> 21 mm Hg) - in 13 (20,97%) eyes. Mean IOP values were 18,21 ±4,2 mm Hg.Eyelid telangiectasias was noted in 9 (29,03 %) patients, chronic blepharitis - in 13(41,94 %). Lens opacity was found in 16 (51,61 %) patients (27 eyes), mostly in the form of posterior subcapsular cataract (in 20 eyes), nuclear cataract (in 6 eyes) and cortical cataract appearing as focal cystic opacities (in 1 eye). The mean age of patients with cataracts was 49,2 ± 12,3 years (11,4 years older than patients without cataracts). Additionally, 14 of the patients with cataracts were either currently taking or had previously taken systemic corticosteroids. Superficial conjunctival hyperaemia was noted in 20 (64,52 %), and varicose dilatation of subconjunctival and episcleral blood vessels in 9 (29,03 %) and 7 (22,58 %) patients, respectively. In 9 eyes (14,52 %) foci of pigment epithelium were found peripherally on the optic disc, In 6 (9,68 %) eyes - thinning of choroidal capillaries and retinal pigment epithelium, in 8 (12,90 %) - ischemic areas surrounded by microaneurysms and intraretinal extravasation, dilatation of the vessel-free fovea. сonjunctival fibrosis - in 8 (25,81 %) patients. In 25 eyes Schirmer I test results were below 10 mm, and in 7 eyes - below 5 mm. Eyelid stiffness was associated with difficulty in lid eversion and a woody texture upon palpation. Comparing patients with and without eyelid stiffness, we found that the mean age and the age at diagnosis were significantly lower in the former group (p < 0.01 and p < 0.05, respectively). The diffuse subtype was more prevalent among pts with eyelid skin changes (p < 0,05).Conclusion:In patients with SSc numerous abnormalities within the vision of organ may be found. Ocular symptoms are relatively common complications of SSc, and may result in serious, irreversible changes in the organ of vision. Regular ophthalmological examinations are essential among the patients with SSc.Disclosure of Interests:None declared


2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
LakshmiPriya Rangaraju ◽  
Xuejuan Jiang ◽  
J. Jason McAnany ◽  
Michael R. Tan ◽  
Justin Wanek ◽  
...  

Purpose. Diabetes is known to cause alterations in retinal microvasculature and tissue that progressively lead to visual impairment. Optical coherence tomography (OCT) is useful for assessment of total retinal thickening due to diabetic macular edema (DME). In the current study, we determined associations between visual acuity (VA) and retinal layer thickness, reflectance, and interface disruption derived from enface OCT images in subjects with and without DME. Materials and Methods. Best corrected VA was measured and high-density OCT volume scans were acquired in 149 diabetic subjects. A previously established image segmentation method identified retinal layer interfaces and locations of visually indiscernible (disrupted) interfaces. Enface thickness maps and reflectance images of the nerve fiber layer (NFL), combined ganglion cell and inner plexiform layer (GCLIPL), inner nuclear layer (INL), outer plexiform layer (OPL), outer nuclear layer (ONL), photoreceptor outer segment layer (OSL), and retinal pigment epithelium (RPE) were generated in the central macular subfield. The associations among VA and retinal layer metrics were determined by multivariate linear regressions after adjusting for covariates (age, sex, race, HbA1c, diabetes type, and duration) and correcting for multiple comparisons. Results. In DME subjects, increased GCLIPL and OPL thickness and decreased OSL thickness were associated with reduced VA. Furthermore, increased NFL reflectance and decreased OSL reflectance were associated with reduced VA. Additionally, increased areas of INL and ONL interface disruptions were associated with reduced VA. In subjects without DME, increased INL thickness was associated with reduced VA, whereas in subjects without DME but with previous antivascular endothelium growth factor treatment, thickening of OPL was associated with reduced VA. Conclusions. Alterations in retinal layer thickness and reflectance metrics derived from enface OCT images were associated with reduced VA with and without presence of DME, suggestive of their potential for monitoring development, progression, and treatment of DME.


Author(s):  
Thomas J. van Rijssen ◽  
Elon H. C. van Dijk ◽  
Paula Scholz ◽  
Robert E. MacLaren ◽  
Sascha Fauser ◽  
...  

Abstract Purpose To evaluate the clinical outcomes after half-dose photodynamic therapy (PDT) in chronic central serous chorioretinopathy (cCSC) patients with pre-existent fovea-involving atrophy. Methods In this retrospective study, cCSC patients who had a window defect of the retinal pigment epithelium (RPE) on fluorescein angiography (FA), compatible with RPE atrophy, prior to half-dose PDT were included. Results Thirty-four cCSC eyes with typical findings of cCSC on multimodal imaging, and fovea-involving RPE atrophy on FA, were included. At the first visit after PDT (at a median of 1.8 months after half-dose PDT), 20 eyes (59%) had a complete resolution of SRF (p < 0.001), while this was the case in 19 eyes (56%) at final visit (median of 11.3 months after half-dose PDT; p < 0.001). The mean BCVA in Early Treatment of Diabetic Retinopathy Study letters was 71. 2 ± 15.9 at last visit before PDT, which increased to 74.1 ± 14.1 at first visit after PDT (p = 0.093, compared with baseline), and changed to 73.0 ± 19.1 at final visit (p = 0.392, compared with baseline). Both at first visit after PDT and at final visit, a significant decrease in subfoveal choroidal thickness was observed (p = 0.032 and p = 0.004, respectively). Conclusions Half-dose PDT in cCSC patients with pre-existing fovea-involving atrophy may lead to anatomical changes, but not to functional improvements. Ideally, cCSC should be treated with half-dose PDT before the occurrence of such atrophy.


2020 ◽  
Vol 10 (1) ◽  
Author(s):  
S. Scott Whitmore ◽  
Christopher R. Fortenbach ◽  
Justine L. Cheng ◽  
Adam P. DeLuca ◽  
D. Brice Critser ◽  
...  

Abstract Stargardt disease, the most common inherited macular dystrophy, is characterized by vision loss due to central retinal atrophy. Although clinical trials for Stargardt are currently underway, the disease is typically slowly progressive, and objective, imaging-based biomarkers are critically needed. In this retrospective, observational study, we characterize the thicknesses of individual retinal sublayers by macular optical coherence tomography (OCT) in a large cohort of patients with molecularly-confirmed, ABCA4-associated Stargardt disease (STGD1) relative to normal controls. Automated segmentation of retinal sublayers was performed with manual correction as needed, and thicknesses in various macular regions were compared using mixed effects models. Relative to controls (42 eyes, 40 patients), STGD1 patients (107 eyes, 63 patients) had slight thickening of the nerve fiber layer and retinal pigment epithelium-Bruch’s membrane, with thinning in other sublayers, especially the outer nuclear layer (ONL) (p < 0.0015). When comparing the rate of retinal sublayer thickness change over time (mean follow-up 3.9 years for STGD1, 2.5 years for controls), STGD1 retinas thinned faster than controls in the outer retina (ONL to photoreceptor outer segments). OCT-based retinal sublayer thickness measurements are feasible in STGD1 patients and may provide objective measures of disease progression or treatment response.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Ílen Ferreira Costa ◽  
Livia Pimenta Bonifácio ◽  
Fernando Bellissimo-Rodrigues ◽  
Eduardo Melani Rocha ◽  
Rodrigo Jorge ◽  
...  

AbstractTo describe the medium-term ophthalmological findings in patients recovering from COVID-19. Patients recovered from the acute phase of COVID-19 underwent a complete ophthalmological evaluation, including presenting and best-corrected visual acuity (BCVA), refractometry, biomicroscopy, tonometry, break-up time and Schirmer tests, indirect ophthalmoscopy, color fundus picture, and retinal architecture evaluation using optical coherence tomography. Socio-demographic data and personal medical history were also collected. According to the severity of systemic manifestations, patients were classified into mild-to-moderate, severe, and critical. Sixty-four patients (128 eyes) were evaluated 82 ± 36.4 days after the onset of COVID’s symptoms. The mean ± SD duration of hospitalization was 15.0 ± 10.7 days. Seven patients (10.9%) had mild-to-moderate, 33 (51.5%) severe, and 24 (37.5%) critical disease. Median [interquartile ranges (IQR)] presenting visual acuity was 0.1 (0–0.2) and BCVA 0 (0–0.1). Anterior segment biomicroscopy was unremarkable, except for dry eye disease, verified in 10.9% of them. The mean ± SD intraocular pressure (IOP) in critical group (14.16 ± 1.88 mmHg) was significantly higher than in severe group (12.51 ± 2.40 mmHg), both in the right (p 0.02) and left eyes (p 0.038). Among all, 15.6% had diabetic retinopathy, and two patients presented with discrete white-yellowish dots in the posterior pole, leading to hyporreflective changes at retinal pigment epithelium level, outer segment, and ellipsoid layers. The present study identified higher IOP among critical cases, when compared to severe cases, and discrete outer retina changes 80 days after COVID-19 infection. No sign of uveitis was found.


2021 ◽  
pp. 1-3
Author(s):  
Katja Göbel

To describe the medium-term ophthalmological findings in patients recovering from COVID-19. Patients recovered from the acute phase of COVID-19 underwent a complete ophthalmological evaluation, including presenting and best-corrected visual acuity (BCVA), refractometry, biomicroscopy, tonometry, break-up time and Schirmer tests, indirect ophthalmoscopy, color fundus picture, and retinal architecture evaluation using optical coherence tomography. Socio-demographic data and personal medical history were also collected. According to the severity of systemic manifestations, patients were classified into mild-to-moderate, severe, and critical. Sixty-four patients (128 eyes) were evaluated 82 ± 36.4 days after the onset of COVID’s symptoms. The mean ± SD duration of hospitalization was 15.0 ± 10.7 days. Seven patients (10.9%) had mild-to-moderate, 33 (51.5%) severe, and 24 (37.5%) critical disease. Median [interquartile ranges (IQR)] presenting visual acuity was 0.1 (0–0.2) and BCVA 0 (0–0.1). Anterior segment biomicroscopy was unremarkable, except for dry eye disease, verified in 10.9% of them. The mean ± SD intraocular pressure (IOP) in critical group (14.16 ± 1.88 mmHg) was significantly higher than in severe group (12.51 ± 2.40 mmHg), both in the right (p 0.02) and left eyes (p 0.038). Among all, 15.6% had diabetic retinopathy, and two patients presented with discrete white-yellowish dots in the posterior pole, leading to hyporreflective changes at retinal pigment epithelium level, outer segment, and ellipsoid layers. The present study identified higher IOP among critical cases, when compared to severe cases, and discrete outer retina changes 80 days after COVID-19 infection. No sign of uveitis was found.


2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
José L. Sánchez-Vicente ◽  
Miguel Contreras-Díaz ◽  
Trinidad Rueda ◽  
Enrique Rodríguez de la Rúa-Franch ◽  
Fredy E. Molina-Socola ◽  
...  

Purpose. To describe the case of spontaneous resolution of epiretinal membrane in a patient with Combined Hamartoma of the Retina and Retinal Pigment Epithelium (CHR-RPE), in the clinical context of Gorlin Syndrome (GS).Methods. Observational case report of a 12-year-old female patient is presented. The diagnosis of CHRRPE was made by OCT and fundus examination, which showed a mound of disorganized tissue originating from retina and retinal pigment epithelium. Epiretinal membrane (EM) was also detected. Genetic study was performed to confirm the diagnosis of GS.Results. The patient was observed for 39 months, showing spontaneous resolution of the traction caused by the EM and improvement in visual acuity (VA), which was 20/80 at initial presentation, rising to 20/40 after follow-up period.Conclusions. The presence of EM in CHR-REP is a cause of reduction of visual acuity. Management of this condition is controversial; however, we would like to highlight that spontaneous resolution of the traction caused by EM is possible, resulting in recovery of VA.


2013 ◽  
Vol 5 (1) ◽  
pp. 38-44 ◽  
Author(s):  
K Sapkota ◽  
A Pirouzian ◽  
NS Matta

Introduction: Refractive error is a common cause of amblyopia. Objective: To determine prevalence of amblyopia and the pattern and the types of refractive error in children with amblyopia in a tertiary eye hospital of Nepal. Materials and methods: A retrospective chart review of children diagnosed with amblyopia in the Nepal Eye Hospital (NEH) from July 2006 to June 2011 was conducted. Children of age 13+ or who had any ocular pathology were excluded. Cycloplegic refraction and an ophthalmological examination was performed for all children. The pattern of refractive error and the association between types of refractive error and types of amblyopia were determined. Results: Amblyopia was found in 0.7 % (440) of 62,633 children examined in NEH during this period. All the amblyopic eyes of the subjects had refractive error. Fifty-six percent (248) of the patients were male and the mean age was 7.74 ± 2.97 years. Anisometropia was the most common cause of amblyopia (p < 0.001). One third (29 %) of the subjects had bilateral amblyopia due to high ametropia. Forty percent of eyes had severe amblyopia with visual acuity of 20/120 or worse. About twothirds (59.2 %) of the eyes had astigmatism. Conclusion: The prevalence of amblyopia in the Nepal Eye Hospital is 0.7%. Anisometropia is the most common cause of amblyopia. Astigmatism is the most common types of refractive error in amblyopic eyes. Nepal J Ophthalmol 2013; 5(9):38-44 DOI: http://dx.doi.org/10.3126/nepjoph.v5i1.7820


2021 ◽  
Vol 12 ◽  
Author(s):  
Fukashi Ishibashi ◽  
Aiko Kosaka ◽  
Mitra Tavakoli

AimsTo establish the sequential changes by glycemic control in the mean thickness, volume and reflectance of the macular photoreceptor layers (MPRLs) and retinal pigment epithelium in patients with type 2 diabetes without diabetic retinopathy.MethodsThirty-one poorly controlled (HbA1c &gt; 8.0%) patients with type 2 diabetes without diabetic retinopathy undergoing glycemic control and 39 control subjects with normal HbA1c levels (&lt; 5.9%) underwent periodical full medical, neurological and ophthalmological examinations over 2 years. Glycemic variability was evaluated by standard deviation and coefficient of variation of monthly measured HbA1c levels and casual plasma glucose. 3D swept source-optical coherence tomography (OCT) and OCT-Explorer-generated enface thickness, volume and reflectance images for 9 subfields defined by Early Treatment Diabetic Retinopathy Study of 4 MPRLs {outer nuclear layer, ellipsoid zone, photoreceptor outer segment (PROS) and interdigitation zone} and retinal pigment epithelium were acquired every 3 months.ResultsGlycemic control sequentially restored the thickness and volume at 6, 4 and 5 subfields of outer nuclear layer, ellipsoid zone and PROS, respectively. The thickness and volume of outer nuclear layer were restored related to the decrease in HbA1c and casual plasma glucose levels, but not related to glycemic variability and neurological tests. The reflectance of MPRLs and retinal pigment epithelium in patients was marginally weaker than controls, and further decreased at 6 or 15 months during glycemic control. The reduction at 6 months coincided with high HbA1c levels.ConclusionGlycemic control sequentially restored the some MPRL thickness, especially of outer nuclear layer. In contrast, high glucose during glycemic control decreased reflectance and may lead to the development of diabetic retinopathy induced by glycemic control. The repeated OCT examinations can clarify the benefit and hazard of glycemic control to the diabetic retinopathy.


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