scholarly journals Correlation of Macular Focal Electroretinogram with Ellipsoid Zone Extension in Stargardt Disease

2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Edoardo Abed ◽  
Giorgio Placidi ◽  
Luigi Calandriello ◽  
Marco Piccardi ◽  
Francesca Campagna ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Negative Relationship ◽  
Macular Thickness ◽  
Stargardt Disease ◽  
Macular Function ◽  
Ellipsoid Zone ◽  
Focal Electroretinogram ◽  
Cone Function

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.

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 ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Pigment Epithelium ◽  
Outer Nuclear Layer ◽  
Retinal Pigment ◽  
Ophthalmological Examination ◽  
Stargardt Disease ◽  
Rpe Atrophy ◽  
The Mean ◽  
Fundus Photographs ◽  
Auto Fluorescence

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.

scholarly journals Preservation of the Photoreceptor Inner/Outer Segment Junction in Dry Age-Related Macular Degeneration Treated by Rheohemapheresis

2015 ◽  
Vol 2015 ◽  
pp. 1-7 ◽  
Author(s):  
Eva Rencová ◽  
Milan Bláha ◽  
Jan Studnička ◽  
Vladimír Bláha ◽  
Miriam Lánská ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Macular Degeneration ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Treated Group ◽  
Plasma Viscosity ◽  
Age Related Macular Degeneration ◽  
Control Group ◽  
Macular Function ◽  
Age Related

Aim. To evaluate the long-term effect of rheohemapheresis (RHF) treatment of age-related macular degeneration (AMD) on photoreceptor IS/OS junction status.Methods. In our study, we followed 24 patients with dry AMD and drusenoid retinal pigment epithelium detachment (DPED) for a period of more than 2.5 years. Twelve patients (22 eyes) were treated by RHF and 12 controls (18 eyes) were randomized. The treated group underwent 8 RHF standardized procedures. We evaluated best-corrected visual acuity, IS/OS junction status (SD OCT), and macular function (multifocal electroretinography) at baseline and at 2.5-year follow-up.Results. RHF caused a decrease of whole-blood viscosity/plasma viscosity at about 15/12%. BCVA of treated patients increased insignificantlyP=0.187from median 74.0 letters (56.2 to 81.3 letters) to median 79.0 letters (57.3 to 83.4 letters), but it decreased significantly from 74.0 letters (25.2 to 82.6 letters) to 72.5 letters (23.4 to 83.1 letters) in the control groupP=0.041. The mfERG responses in the region of eccentricity between 1.8° and 7° were significantly higher in treated patientsP=0.04.Conclusions. RHF contributed to sparing of photoreceptor IS/OS junction integrity in the fovea, which is assumed to be a predictive factor for preservation of visual acuity.

scholarly journals Ellipsoid Zone Thickness in Sporadic Adult-Onset Foveomacular Vitelliform Dystrophy

2021 ◽  
Author(s):  
Shayan Yousefi ◽  
Liran Tiosano ◽  
Tareq Jaouni ◽  
Edward Averbukh ◽  
Eyal Banin ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
T Test ◽  
Disease Stage ◽  
Foveal Avascular Zone ◽  
Adult Onset ◽  
Ellipsoid Zone ◽  
Anova Test ◽  
Avascular Zone

Abstract Purpose: To evaluate the thickness of the ellipsoid zone (EZ) layer and its correlation with visual acuity and the disease stage in eyes with Adult-onset foveomacular vitelliform dystrophy (AFVD).Materials and Methods: Ninety-two eyes of 57 patients with AFVD were evaluated. Four consecutive spectral-domain optical coherence tomography (SD-OCT) scans from each study eye were analyzed. Retinal layers were segmented, and the EZ layer thickness was measure in two areas of the macula: at the center of fovea (CF) and at the foveal avascular zone edge (FE).Results: The mean±SD EZ thickness for was 16.5±9.6 microns at the center of fovea (CF) and 17± 9.8 microns at the edge of foveal avascular zone (FE; p=0.006, t-test). Compared to 30 healthy eyes, the EZ was thicker at the vitelliform stage in both CF and FE (p<0.001 in both points, t-test), and during the pseudohypopyon or vitelliruptive stages in CF (p=0.007, t test), but not in edge of the fovea (p=0.15, t test). Visual acuity was better in eyes with intact EZ compared to absent EZ (p=0.001 for both CF and FE, ANOVA test). There was a trend for an association between EZ thickness and the stage of AFVD (p=0.06, ANOVA test). Conclusion: The foveal EZ thickness in AFVD, is thicker comparing with controls. This might suggest that impaired retinal pigment epithelium phagocytosis or excess photoreceptor EZ production play important role in the pathogenesis of AFVD.

scholarly journals Retinal Pigment Epithelial and Outer Retinal Atrophy in Age-Related Macular Degeneration: Correlation with Macular Function

2020 ◽  
Vol 9 (9) ◽  
pp. 2973
Author(s):  
Maria C. Savastano ◽  
Benedetto Falsini ◽  
Grazia M. Cozzupoli ◽  
Alfonso Savastano ◽  
Gloria Gambini ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Retinal Pigment Epithelium ◽  
Macular Degeneration ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Age Related Macular Degeneration ◽  
Outer Retina ◽  
Macular Function ◽  
Age Related ◽  
Combined Use

The purpose of this study was to investigate the relationship between the retinal pigment epithelium (RPE) and outer retina changes, expressed in terms of sub-RPE illumination (SRI) on optical-coherence tomography (OCT), and central retinal function, measured by visual acuity and focal electroretinogram (fERG), in patients with non-exudative age-related macular degeneration (neAMD). In this retrospective study, 29 eyes of 29 patients affected by early (24.14%), intermediate (41.38%), and advanced (34.48%) neAMD were evaluated. All enrolled eyes were studied with OCT to measure the total area of SRI, by using an automated standardized algorithm. Visual acuity and fERG were assessed. The area of SRI was negatively correlated with fERG amplitude (r ≤ −0.4, p ≤ 0.02) and best-corrected visual acuity (BCVA) (r ≤ 0.4, p ≤ 0.04). Our results indicate that the severity of retinal pigment epithelium and outer retina atrophy (RORA), indirectly quantified through the detection of SRI areas by commercial OCT algorithms, is correlated with central retinal dysfunction, as determined by visual acuity and fERG, supporting the combined use of structural exams and functional tests as valid tools to detect the extent of RPE and photoreceptors’ disruption.

scholarly journals Combined Hamartoma of the Retina and Retinal Pigment Epithelium in a Patient with Gorlin Syndrome: Spontaneous Partial Resolution of Traction Caused by Epiretinal Membrane

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 ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Retinal Pigment Epithelium ◽  
Epiretinal Membrane ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Spontaneous Resolution ◽  
Fundus Examination ◽  
Gorlin Syndrome ◽  
Clinical Context

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.

Anatomical and functional changes in neovascular AMD in remission: comparison of fibrocellular and fibrovascular phenotypes

2019 ◽  
Vol 104 (1) ◽  
pp. 47-52 ◽  
Author(s):  
Lea Querques ◽  
Mariacristina Parravano ◽  
Enrico Borrelli ◽  
Adele Chiaravalloti ◽  
Massimiliano Tedeschi ◽  
...  
Keyword(s):  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Age Related Macular Degeneration ◽  
Study Cohort ◽  
Neovascular Amd ◽  
Macular Function ◽  
Functional Changes ◽  
Age Related ◽  
Rpe Atrophy

PurposeTo investigate the anatomical changes and the macular function in neovascular age-related macular degeneration (AMD) eyes, according to the recognition of either fibrocellular or fibrovascular phenotype.MethodsWe enrolled eyes with previously treated neovascular AMD in remission (no subretinal haemorrhage, sign of fluid in or under the retina and no treatment for at least 6 months). Subjects underwent multimodal imaging assessment and were tested for macular sensitivity using microperimetry. The study cohort was divided according to the presence of fibrosis on multicolour (MC) images, yielding two distinct phenotypic subgroups: (1) fibrocellular group and (2) fibrovascular group.ResultsNineteen eyes were classified as fibrocellular on MC images, while 22 eyes as fibrovascular. Mean±SD age was 73.9±11.0 years in the fibrocellular group and 75.9±7.1 years in the fibrovascular group (p=0.221). Best-corrected visual acuity was 0.7±0.5 logarithm of the minimum angle of resolution (LogMAR) in the fibrocellular group and 0.3±0.2 LogMAR in the fibrovascular group (p=0.003). On the optical coherence tomography and fundus autofluorescence evaluation, 17/19 eyes with the fibrocellular phenotype and 8/22 eyes with the fibrovascular phenotype displayed the presence of retinal pigment epithelium (RPE) atrophy (p=0.001). The perfusion density within the neovascular lesion was 28.9%±9.9% in the fibrocellular group and 44.2%±5.9 % in the fibrovascular group (p<0.0001).ConclusionNeovascular AMD eyes in remission and with evidence of fibrocellular scar are characterised by RPE atrophy and reduced perfusion, which are associated with a higher degree of functional impairment. These findings suggest that maturation of vessels in fibrosis might be a better target in neovascular AMD treatments rather than their abolishment.

Diagnosis and treatment of peripheral exudative haemorrhagic chorioretinopathy

2019 ◽  
Vol 104 (6) ◽  
pp. 874-878 ◽  
Author(s):  
Sarah Vandefonteyne ◽  
Jean-Pierre Caujolle ◽  
Laurence Rosier ◽  
John Conrath ◽  
Gabriel Quentel ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Multimodal Imaging ◽  
Chart Review ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Treatment Options ◽  
Treatment Strategies ◽  
Choroidal Melanoma ◽  
Endothelial Growth Factor

PurposePeripheral exudative haemorrhagic chorioretinopathy (PEHCR) is a rare disorder that is often misdiagnosed. The aim of this study was to better characterise PEHCR and to assess treatment options.Material and methodsRetrospective multicentric chart review.ResultsOf 84 eyes (69 patients) with PEHCR referred between 2005 and 2017, the most common referral diagnosis was choroidal melanoma (41.3%). Bilateral involvement was found in 21.7% of cases. Haemorrhagic retinal pigment epithelium detachment was the most common peripheral lesion (53.6%). Maculopathy was associated with peripheral lesions in 65.8% of cases. PEHCR lesions were mostly heterogeneous (58.8%) on B-scan ultrasonography. Choroidal neovascularisation was found in 10 eyes (26.3%) out of 38 eyes that underwent fluorescein angiography. Polyps were observed in 14 eyes (58.3%) out of 24 eyes that underwent indocyanine green angiography. Fifty-one eyes were treated (62.2%). Intravitreal injections (IVTI) of antivascular endothelial growth factor (VEGF) were the most used treatment (36.6%) before laser photocoagulation, photodynamic therapy, vitrectomy and cryotherapy. Only vitrectomy improved visual acuity. Most lesions (65.6%) regressed at the last follow-up visit.ConclusionIn case of PEHCR, multimodal imaging is useful to avoid misdiagnosis, to characterise PEHCR lesions and to guide treatment strategies. Regression of PEHCR lesions was observed in two-thirds of the patients. Vitrectomy improved visual acuity. More than a third of patients underwent anti-VEGF IVTI. Further studies are needed to assess IVTI’s efficacy.

scholarly journals Bis(monoacylglycero)phosphate lipids in the retinal pigment epithelium implicate lysosomal/endosomal dysfunction in a model of Stargardt disease and human retinas

2017 ◽  
Vol 7 (1) ◽  
Author(s):  
David M. G. Anderson ◽  
Zsolt Ablonczy ◽  
Yiannis Koutalos ◽  
Anne M. Hanneken ◽  
Jeffrey M. Spraggins ◽  
...  
Keyword(s):  
Retinal Pigment Epithelium ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Stargardt Disease
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