Progressive, Symmetrical, Painless Visual Loss

2021 ◽  
pp. 77-79
Author(s):  
Eric R. Eggenberger ◽  
Marie D. Acierno ◽  
M. Tariq Bhatti ◽  
John J. Chen
Keyword(s):  
Optical Coherence Tomography ◽  
Vision Loss ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Light Sensitivity ◽  
Normal Serum ◽  
Breast Adenocarcinoma ◽  
Optical Coherence ◽  
Autoimmune Retinopathy ◽  
Cancer Associated Retinopathy

A 75-year-old woman with a medical history of mixed connective tissue disease and breast adenocarcinoma sought care for subacute visual “haze” in both eyes characterized by light sensitivity, particularly with commercial fluorescent lighting, progressing over weeks. Visual acuity was 20/40 in each eye. The pupils were equal in size with no relative afferent pupillary defect but were sluggishly reactive to light. Automated perimetry documented peripheral constriction in both eyes. Ocular motility was normal. Ophthalmoscopy showed mild retinal pigment epithelial changes in both maculae with normal optic nerves. Optical coherence tomography showed macular thinning in both eyes. Findings of fundus autofluorescence were normal. Serum testing documented the presence of 3 retinal antibodies, against 30-, 36-, and 46-kDa proteins. A paraneoplastic panel was negative except for low-level ganglionic (alpha 3) acetylcholine receptor autoantibody positivity, which was interpreted as nonspecific autoimmunity. Electroretinography indicated severely decreased scotopic and photopic a and b waves. A diagnosis of paraneoplastic or nonparaneoplastic autoimmune retinopathy was made, consistent with the clinical presentation, optical coherence tomography and electroretinography findings, and the presence of retinal antibodies. There are no established evidence-based guidelines to assist treatment decisions in autoimmune retinopathy, although several lines of therapy have been advocated. No specific immunosuppressive therapy was undertaken in this case. However, if her vision had continued to rapidly worsen over time, empiric immunotherapy would have been instituted. Autoimmune retinopathy includes paraneoplastic and nonparaneoplastic forms. The best-characterized autoimmune retinopathy phenotype is cancer-associated retinopathy. Cancer-associated retinopathy typically presents with subacute, painless, bilateral (although asymmetry has been described) vision loss that is progressive over weeks to months, reflecting both rod and cone dysfunction in most patients. Visual symptoms precede recognition of an underlying cancer in approximately 50% of cases.

scholarly journals Early Detection of Incipient Retinal Pigment Epithelium Atrophy Overlying Drusen with Fundus Autofluorescence vs. Spectral Domain Optical Coherence Tomography

2020 ◽  
Vol 2020 ◽  
pp. 1-8 ◽  
Author(s):  
Anabel Rodríguez ◽  
Marc Biarnés ◽  
Rosa M. Coco-Martin ◽  
Anna Sala-Puigdollers ◽  
Jordi Monés
Keyword(s):  
Optical Coherence Tomography ◽  
Retinal Pigment Epithelium ◽  
Median Time ◽  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Geographic Atrophy ◽  
Optical Coherence ◽  
Rpe Atrophy ◽  
Sd Oct

Purpose. This study aims to find out which tool, fundus autofluorescence (FAF) or spectral domain optical coherence tomography (SD-OCT), is more sensitive in detecting retinal pigment epithelium (RPE) demise overlying drusen and can, therefore, help predict geographic atrophy (GA) appearance in Age-Related Macular Degeneration (AMD). Methods. A single-site, retrospective, observational, longitudinal study was conducted. Patients with intermediate AMD (iAMD) (large (>125 μm) or intermediate (63–125 μm) drusen with hyper/hypopigmentation) with a minimum follow-up of 18 months were included. Drusen with overlying incipient RPE atrophy were identified on SD-OCT defined as choroidal hypertransmission or nascent geographic atrophy (nGA). These selected drusen were, then, traced backwards in time to determine if incipient RPE atrophy overlying drusen was observed on FAF (well-demarcated region of absence of autofluorescence) before, simultaneously, or after having detected the first signs of incipient RPE atrophy on SD-OCT. The number of drusen in which signs of incipient RPE atrophy was detected earlier using FAF or SD-OCT was compared. The time elapsed from the identification with the more sensitive method to the other was recorded and analyzed. Results. One hundred and thirty-three drusen in 22 eyes of 22 patients were included. Of these, 112 (84.2%) drusen showed choroidal hypertransmission and 21(15.8%) nGA. Early signs of atrophy overlying drusen were found simultaneously on SD-OCT and FAF in 52 cases (39.1%, 95% CI 30.8–47.9%), earliest on FAF in 51 (38.3%, 95% CI 30.0–47.2%) and first on SD-OCT in 30 (22.6%, 95% CI 15.8–30.6%; p<0.05). Statistically significant differences were found between both techniques (p=0.005), with FAF detecting it earlier than SD-OCT. When RPE atrophy was found first on FAF, the median time to diagnosis with SD-OCT was 6.6 months (95% CI 5.5 to 8.6), while if detection occurred earlier on SD-OCT, the median time until identification with FAF was 12.6 months (95% CI 6.0 to 23.4; p=0.0003). Conclusions. In iAMD cases in which early atrophy overlying drusen is not detected simultaneously in FAF and SD-OCT, FAF was significantly more sensitive. Nevertheless, a multimodal approach is recommended and required to evaluate these patients.

Torpedo Maculopathy Presenting With Fovea Plana in a 21-Year-Old Female

2017 ◽  
Vol 1 (6) ◽  
pp. 424-427 ◽  
Author(s):  
Douglas S. M. Iaboni ◽  
Mark E. Seamone ◽  
Netan Choudhry ◽  
R. Rishi Gupta
Keyword(s):  
Optical Coherence Tomography ◽  
Fluorescein Angiography ◽  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Spectral Domain ◽  
Optical Coherence ◽  
En Face Oct ◽  
En Face ◽  
Torpedo Maculopathy

Purpose: To report a case of torpedo maculopathy presenting with fovea plana in a 21-year-old female patient. Methods: Multimodal imaging including fluorescein angiography, fundus autofluorescence, and spectral domain optical coherence tomography (OCT) was used to characterize pathology. Results: A well-circumscribed ellipsoidal hypopigmented lesion was observed inferotemporal to the fovea OS. Fluorescein angiography and fundus autofluorescence revealed hyperfluorescence and focal hypoautofluorescence, respectively, corresponding to the hypopigmented lesion. Spectral domain optical coherence tomography imaging of the lesion revealed attenuation of the ellipsoid zone and retinal pigment epithelium with a hyporeflective subretinal cleft. En face OCT imaging demonstrated an area of subretinal hyporeflectivity at the subretinal cleft. Spectral domain optical coherence tomography revealed the absence of a foveal pit. Conclusion: We have described a novel case presentation of fovea plana alongside torpedo maculopathy. The significance of this association remains unclear. Further study into these conditions is necessary to help better determine factors responsible for visual symptoms or lack thereof and circumstances that promote their development.

scholarly journals Fully-automated atrophy segmentation in dry age-related macular degeneration in optical coherence tomography

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yasmine Derradji ◽  
Agata Mosinska ◽  
Stefanos Apostolopoulos ◽  
Carlos Ciller ◽  
Sandro De Zanet ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Macular Degeneration ◽  
Vision Loss ◽  
Retinal Pigment ◽  
Retinal Disease ◽  
Age Related Macular Degeneration ◽  
Retinal Layer ◽  
Optical Coherence ◽  
Age Related ◽  
Layer Segmentation

AbstractAge-related macular degeneration (AMD) is a progressive retinal disease, causing vision loss. A more detailed characterization of its atrophic form became possible thanks to the introduction of Optical Coherence Tomography (OCT). However, manual atrophy quantification in 3D retinal scans is a tedious task and prevents taking full advantage of the accurate retina depiction. In this study we developed a fully automated algorithm segmenting Retinal Pigment Epithelial and Outer Retinal Atrophy (RORA) in dry AMD on macular OCT. 62 SD-OCT scans from eyes with atrophic AMD (57 patients) were collected and split into train and test sets. The training set was used to develop a Convolutional Neural Network (CNN). The performance of the algorithm was established by cross validation and comparison to the test set with ground-truth annotated by two graders. Additionally, the effect of using retinal layer segmentation during training was investigated. The algorithm achieved mean Dice scores of 0.881 and 0.844, sensitivity of 0.850 and 0.915 and precision of 0.928 and 0.799 in comparison with Expert 1 and Expert 2, respectively. Using retinal layer segmentation improved the model performance. The proposed model identified RORA with performance matching human experts. It has a potential to rapidly identify atrophy with high consistency.

scholarly journals Optical Coherence Tomography Angiography (OCTA) findings in Serpiginous Choroiditis

2020 ◽  
Author(s):  
Sergio Macedo ◽  
Dominika Pohlmann ◽  
Matthias Lenglinger ◽  
Uwe Pleyer ◽  
Antonia M. Joussen ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Optical Coherence Tomography Angiography ◽  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Vascular Lesions ◽  
Enhanced Depth Imaging ◽  
Optical Coherence ◽  
Serpiginous Choroiditis ◽  
Diagnostic Modalities

Abstract Background. To describe changes in the retina/choroid in patients with Serpiginous Choroiditis (SC) by Optical Coherence Tomography Angiography (OCTA) in a multimodal imaging approach. Methods. Prospective, monocentric study of 24 eyes of 12 consenting patients diagnosed with SC, who underwent OCTA, which was analyzed and compared to other methods such as enhanced depth imaging-OCT, fluorescein angiography, indocyanine green angiography, and fundus autofluorescence. Results. The study group consisted of 9 patients with peripapillary SC, 1 macular SC, and 2 atypical cases. All eyes presented an inactive SC confirmed by standard imaging. OCTA demonstrated the lesions tridimensionally in great detail. There was no difference in the angioarchitecture among the 3 forms of SC. A loss of the choriocapillaris/retinal pigment epithelium left a “window-defect”, where the vessels of larger caliber of the choroid became recognizable and their appearance inverted (“white-on-black”). A relationship between the presence of segmentation errors (SE) in the slabs and low visual acuity was established with a one-way ANOVA. Conclusions. OCTA was able to non-invasively assess vascular lesions of the choroid/retina in patients with SC with a high degree of correlation to other diagnostic modalities. Consequent long-term assessments could lead to a better understanding of disease progression.

scholarly journals Optical Coherence Tomography Angiography (OCTA) findings in Serpiginous Choroiditis

2020 ◽  
Author(s):  
Sergio Macedo ◽  
Dominika Pohlmann ◽  
Matthias Lenglinger ◽  
Uwe Pleyer ◽  
Antonia M. Joussen ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Optical Coherence Tomography Angiography ◽  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Vascular Lesions ◽  
Enhanced Depth Imaging ◽  
Optical Coherence ◽  
Serpiginous Choroiditis ◽  
Diagnostic Modalities

Abstract Background. To describe changes in the retina/choroid in patients with Serpiginous Choroiditis (SC) by Optical Coherence Tomography Angiography (OCTA) in a multimodal imaging approach.Methods. Prospective, monocentric study of 24 eyes of 12 consenting patients diagnosed with SC, who underwent OCTA, which was analyzed and compared to other methods such as enhanced depth imaging-OCT, fluorescein angiography, indocyanine green angiography, and fundus autofluorescence. Results. The study group consisted of 9 patients with peripapillary SC, 1 macular SC, and 2 atypical cases. All eyes presented an inactive SC confirmed by standard imaging. OCTA demonstrated the lesions tridimensionally in great detail. There was no difference in the angioarchitecture among the 3 forms of SC. A loss of the choriocapillaris/retinal pigment epithelium left a “window-defect”, where the vessels of larger caliber of the choroid became recognizable and their appearance inverted (“white-on-black”). A relationship between the presence of segmentation errors (SE) in the slabs and low visual acuity was established with a one-way ANOVA.Conclusions. OCTA was able to non-invasively assess vascular lesions of the choroid/retina in patients with SC with a high degree of correlation to other diagnostic modalities. Consequent long-term assessments could lead to a better understanding of disease progression.

scholarly journals Association of Fundus Autofluorescence Findings and Outer Retinal Lesions on Optical Coherence Tomography With Visual Acuity in Birdshot Chorioretinopathy

2019 ◽  
Vol 3 (4) ◽  
pp. 235-241
Author(s):  
Laura J. Kopplin ◽  
Marion Munk ◽  
Justin Baynham ◽  
James T. Rosenbaum ◽  
Eric B. Suhler ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Visual Acuity ◽  
Macular Edema ◽  
Vision Loss ◽  
Fundus Autofluorescence ◽  
Demographic Data ◽  
Optical Coherence ◽  
Autofluorescence Imaging ◽  
Birdshot Chorioretinopathy ◽  
Retinal Lesions

Purpose: This article investigates the optical coherence tomography (OCT) and fundus autofluorescence imaging findings in birdshot chorioretinopathy (BSCR) and their association with visual acuity (VA). Methods: In a retrospective, cross-sectional study, we evaluated OCT images for changes in retinal structure including cystoid macular edema (CME), epiretinal membrane, and outer retinal lesions. We assessed autofluorescence images for hypoautofluorescent and hyperautofluorescent changes and noted the distribution of the lesions. Demographic data and VA at the time of imaging were also collected. Associations between OCT and autofluorescence findings and logarithm of the minimum angle of resolution VA were tested using linear regression. Results: We conducted a chart review of 80 eyes from 40 patients with BSCR. Outer retinal lesions were found on OCT in 28 of 80 eyes (35%) and disruption of the outer segment ellipsoid zone (EZ) occurred in 23 eyes (28.7%). Macular hypoautofluorescent lesions were more common than hyperautofluorescent lesions, present in 58.8% and 13% of eyes, respectively. The presence of outer retinal lesions on OCT was significantly associated with reduced VA ( P = .006) as was EZ disruption ( P = .003). These associations remained significant after accounting for the presence of macular edema. There was a trend toward association of macular hypoautofluorescent lesions with decreased vision, although it was not statistically significant ( P = .17). Conclusions: The association of outer retinal lesions with decreased VA suggests a mechanism of central vision loss that is distinct from CME and may provide an additional objective finding to monitor disease activity in BSCR patients.

scholarly journals Acute Retinal Pigment Epitheliitis: Spectral Domain Optical Coherence Tomography, Fluorescein Angiography, and Autofluorescence Findings

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Tuğba Aydoğan ◽  
Esra Güney ◽  
Betül İlkay Sezgin Akçay ◽  
Tahir Kansu Bozkurt ◽  
Cihan Ünlü ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Fluorescein Angiography ◽  
Pigment Epithelium ◽  
Outer Nuclear Layer ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Spectral Domain ◽  
Fundus Examination ◽  
Optical Coherence ◽  
Sd Oct

A 17-year-old presented with central and paracentral scotomas in his right eye for one week. There was no remarkable medical or ocular history. Blood analyses were within normal range. At presentation both eyes’ best-corrected visual acuities were 20/20. Slit-lamp examination result was normal. Fundus examination revealed yellow-white hypopigmented areas in the macula. Fluorescein angiography (FA) showed hypofluorescence surrounded by ring of hyperfluorescence. Fundus autofluorescence (FAF) was slightly increased. Spectral domain optical coherence tomography (SD-OCT) showed disruption of IS/OS junction with expansion of abnormal hyperreflectivity from retinal pigment epithelium to the outer nuclear layer (ONL). One month later fundus examination showed disappearance of the lesions. FA revealed transmission hyperfluorescence. FAF showed increased autofluorescence and pigment clumping. Hyperreflective band in SD-OCT disappeared. Loss of photoreceptor segment layers was observed in some of the macular lesions. The diagnosis of acute retinal pigment epitheliitis can be challenging after disappearance of fundus findings. FA, FAF, and SD-OCT are important tests for diagnosis after resolution of the disease.

scholarly journals Optical Coherence Tomography Angiography (OCTA) findings in Serpiginous Choroiditis

2020 ◽  
Author(s):  
Sergio Macedo ◽  
Dominika Pohlmann ◽  
Matthias Lenglinger ◽  
Uwe Pleyer ◽  
Antonia M. Joussen ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Optical Coherence Tomography Angiography ◽  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Vascular Lesions ◽  
Enhanced Depth Imaging ◽  
Optical Coherence ◽  
Serpiginous Choroiditis ◽  
Diagnostic Modalities

Abstract Background. To describe changes in the retina/choroid in patients with Serpiginous Choroiditis (SC) by Optical Coherence Tomography Angiography (OCTA) in a multimodal imaging approach. Methods. Prospective, monocentric study of 24 eyes of 12 consenting patients diagnosed with SC, who underwent OCTA, which was analyzed and compared to other methods such as enhanced depth imaging-OCT, fluorescein angiography, indocyanine green angiography, and fundus autofluorescence. Results. The study group consisted of 9 patients with peripapillary SC, 1 macular SC, and 2 atypical cases. All eyes presented an inactive SC confirmed by standard imaging. OCTA demonstrated the lesions tridimensionally in great detail. There was no difference in the angioarchitecture among the 3 forms of SC. A loss of the choriocapillaris/retinal pigment epithelium left a “window-defect”, where the vessels of larger caliber of the choroid became recognizable and their appearance inverted (“white-on-black”). A relationship between the presence of segmentation errors (SE) in the slabs and low visual acuity was established with a one-way ANOVA. Conclusions. OCTA was able to non-invasively assess vascular lesions of the choroid/retina in patients with SC with a high degree of correlation to other diagnostic modalities. Consequent long-term assessments could lead to a better understanding of disease progression.

scholarly journals Visible-light optical coherence tomography-based multimodal system for quantitative fundus autofluorescence imaging

2018 ◽  
Vol 243 (17-18) ◽  
pp. 1265-1274 ◽  
Author(s):  
Zahra Nafar ◽  
Rong Wen ◽  
Shuliang Jiao
Keyword(s):  
Optical Coherence Tomography ◽  
Visible Light ◽  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Research Progress ◽  
Retinal Diseases ◽  
Optical Coherence ◽  
Autofluorescence Imaging ◽  
Signal Attenuation

Fundus autofluorescence (FAF) imaging is commonly used in ophthalmic clinics for diagnosis and monitoring of retinal diseases. Lipofuscin in the retinal pigment epithelium (RPE), with A2E as its most abundant component and a visual cycle by-product, is the major fluorophore of FAF. Lipofuscin accumulates with age and is implicated in degenerative retinal diseases. The amount of lipofuscin in RPE can be assessed by quantitative measurement of FAF. However, the currently available FAF imaging technologies are not capable of quantifying the absolute intensity of FAF, which is essential for comparing images from different individuals, and from the same individual over time. One major technical difficulty is to compensate the signal attenuation by ocular media anterior to the RPE (pre-RPE media). FAF intensity is also influenced by fluctuations in imaging conditions such as illumination power and detector sensitivity, all of which need to be compensated. In this review, we present the concept and research progress of using visible-light optical coherence tomography-based simultaneous multimodal retinal imaging to compensate signal attenuation by pre-RPE media and the influence of parameters of the acquisition system for accurate measurement of FAF intensities. Impact statement Quantitative fundus autofluorescence imaging with simultaneous visible-light optical coherence tomography-based multimodal technology has potential significant impact on the diagnosis and monitoring the progression of retinal diseases.

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