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Nieraj Jain - One of the best experts on this subject based on the ideXlab platform.
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expanded clinical spectrum of pentosan polysulfate Maculopathy a macula society collaborative study
Ophthalmology Retina, 2021Co-Authors: Nieraj Jain, Albert Liao, Sunir J Garg, Samir N Patel, Charles C Wykoff, Nikolas J S London, Rahul N Khurana, David N Zacks, Audina M Berrocal, Brandon J LujanAbstract:Purpose Explore the spectrum of clinical manifestations of pentosan polysulfate (PPS) Maculopathy observed across a range of practice settings. Design Multi-institutional retrospective study. Subjects, Participants, and/or Controls Patients exhibiting findings suggestive of PPS Maculopathy identified from April 30, 2019 through December 4, 2020. Methods Members of The Macula Society submitted cases of presumed PPS Maculopathy for consideration in this series. Diagnosis was confirmed by masked review of fundus imaging. Clinical characteristics of confirmed cases were summarized with descriptive statistics. Main Outcome Measures Pentosan polysulfate exposure characteristics and fundus imaging features. Results There were 74 patients with PPS Maculopathy included in the current study. Median (interquartile range) age at diagnosis was 62.0 years (56.0-65.8). The median duration of exposure to PPS was 14.0 years (10.2 to 18.9), with a median cumulative exposure of 1.5 kg (0.9 – 2.4). The most common presenting symptom was decreased or blurry vision (66.2%), followed by prolonged dark adaption or nyctalopia (32.4%). The most common referral diagnosis was age-related macular degeneration (54.1%); 16.2% of patients were referred for suspected PPS Maculopathy. Novel imaging findings emerged, including highly asymmetric disease in 2 patients, and a prominent vitelliform Maculopathy in 2 patients. Conclusions Most patients with PPS Maculopathy exhibit characteristic findings on multimodal fundus imaging in the setting of high cumulative exposure to the oral drug. Some patients in the current study manifested novel imaging findings, expanding our understanding of the phenotypic spectrum of this condition. We recommend considering standardized ophthalmic screening of patients treated with PPS.
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pentosan polysulfate Maculopathy versus age related macular degeneration comparative assessment with multimodal imaging
Canadian Journal of Ophthalmology-journal Canadien D Ophtalmologie, 2021Co-Authors: Joseph S Christiansen, Alexander C Barnes, Duncan E Berry, Nieraj JainAbstract:OBJECTIVE To evaluate whether pentosan polysulfate Maculopathy manifests distinctive imaging features that can be differentiated from those found in age-related macular degeneration (AMD). METHODS Local databases were queried to identify patients with a diagnosis of interstitial cystitis who were seen at the Emory Eye Center between May 2014 and January 2019 and who had fundus imaging available for review. Ninety patients met the eligibility criteria. Masked graders categorized patients based on imaging characteristics as follows: category 1: pentosan polysulfate Maculopathy; category 2: AMD or drusen; category 3: neither; and category 4: unsure. Pentosan polysulfate exposure characteristics were compared among groups. RESULTS Of the 90 subjects evaluated, 79 (88%) were female and the median age was 61.5 years (range, 30-89). Seventeen patients were placed in category 1; 25 in category 2; 47 in category 3, and; 1 in category 4. Among categories 1 to 4, respectively, 17 (100%), 15 (60%), 28 (60%), and 0 patients had exposure to pentosan polysulfate (p = 0.007). Mean cumulative exposure to pentosan polysulfate across the four categories was 2.1, 0.36, 0.34, and 0 kg, respectively (p < 0.00001). Eyes with pentosan polysulfate Maculopathy did not have typical drusen in the macula. CONCLUSION Although pentosan polysulfate Maculopathy resembles some aspects of AMD, the 2 conditions can be differentiated with the use of multimodal fundus imaging.
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potential new onset clinically detectable pentosan polysulfate Maculopathy years after drug cessation
Retinal Cases & Brief Reports, 2020Co-Authors: Joshua M Barnett, Nieraj JainAbstract:Purpose To describe a potential case of pentosan polysulfate Maculopathy that appeared to manifest nearly three years after drug cessation. Methods Complete ophthalmic examination including multimodal fundus imaging, electroretinography, automated perimetry, and molecular testing were performed. Results A 44-year-old female with a 435g cumulative exposure to pentosan polysulfate sodium presented 38-months after drug cessation with six months of worsening metamorphopsia and prolonged dark adaptation. Fundus exam and multimodal fundus imaging demonstrated characteristic features of pentosan polysulfate Maculopathy, and molecular testing was unremarkable. In contrast, color fundus photos of the same patient acquired at an outside facility 25 months prior did not display features consistent with PPS Maculopathy. Conclusion This case suggests that new onset clinically detectable pentosan polysulfate Maculopathy may develop years after drug cessation. If corroborated, this finding has important ramifications for PPS dosing and surveillance guidelines.
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association of macular disease with long term use of pentosan polysulfate sodium findings from a us cohort
British Journal of Ophthalmology, 2020Co-Authors: Nieraj Jain, Brian L VanderbeekAbstract:Background/Aims A series at a single clinical centre recently demonstrated an association between the interstitial cystitis drug pentosan polysulfate sodium (PPS) and a vision-threatening pigmentary Maculopathy. The aim of this study was to determine if an association exists between PPS use and macular disease in a large national cohort. Methods A retrospective, matched cohort study using data from a large US medical claims database from 2002 to 2016 was performed. A total of 3012 and 1604 PPS users were compared with 15 060 and 8017 matched controls at 5 and 7 years, respectively. The primary outcome measures included (1) any new diagnosis of a hereditary or secondary pigmentary Maculopathy (atypical Maculopathy outcome), and (2) any new diagnosis of dry age-related macular degeneration (AMD) or drusen in addition to the aforementioned diagnoses (atypical Maculopathy+AMD outcome). Results At the 5-year and 7-year follow-up, 9 (0.3%) and 10 (0.6%) PPS patients progressed to the atypical Maculopathy outcome compared with 32 (0.2%) and 25 (0.3%) control patients, respectively. 103 (3.4%) and 87 (5.4%) PPS patients developed the atypical Maculopathy+AMD outcome compared with 440 (2.9%) and 328 (4.1%) control patients at 5 and 7 years, respectively. At 5 years, multivariate analysis showed no significant association (p>0.13). At 7 years, PPS users had significantly increased odds of having the atypical Maculopathy+AMD outcome (OR=1.41, 95% CI 1.09 to 1.83, p=0.009). Conclusions PPS exposure was associated with a new diagnosis of macular disease at the 7-year follow-up in a large national cohort.
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pentosan polysulfate Maculopathy versus inherited macular dystrophies comparative assessment with multimodal imaging
Ophthalmology Retina, 2020Co-Authors: Alexander C Barnes, Adam M Hanif, Nieraj JainAbstract:Purpose To evaluate whether pentosan polysulfate (PPS) Maculopathy manifests distinctive characteristics that permit differentiation from hereditary maculopathies with multimodal fundus imaging. Design Retrospective review. Participants Emory Eye Center databases were queried for the following International Classification of Diseases codes from May 20, 2014, through October 22, 2019: 362.70 (unspecified hereditary retinal dystrophy), 362.74 + H35.52 (pigmentary retinal dystrophy), 362.76 + H35.54 (dystrophies primarily involving the retinal pigment epithelium), and H35.50 (unspecified macular degeneration). Methods Fundus images for each patient were evaluated, including color fundus photographs, fundus autofluorescence images, and spectral-domain OCT images. Cases with imaging sufficient for diagnostic classification were analyzed. Masked graders classified patient images as follows: highly suggestive of PPS Maculopathy; some features resembling PPS Maculopathy, but not classic disease; and clearly distinct from PPS Maculopathy. Main Outcome Measures Sensitivity and specificity for identification of PPS Maculopathy by masked reviewers. Results A total of 1394 patients were evaluated, and 1131 had imaging sufficient for classification. Fifteen patients were categorized as having findings highly suggestive of PPS Maculopathy; 25 patients showed some features resembling PPS Maculopathy but not classic disease; and 1091 patients showed evidence of disease clearly distinct from PPS Maculopathy. All 10 patients with PPS Maculopathy in this dataset were correctly categorized as having PPS Maculopathy. Five patients without PPS exposure were categorized incorrectly as having PPS Maculopathy. This represented a 100% sensitivity and 99.6% specificity for identification of PPS Maculopathy by masked review of fundus imaging in this dataset. Conclusions The imaging characteristics of PPS Maculopathy allow for differentiation from hereditary maculopathies even in the absence of known exposure to the drug.
David S Greenfield - One of the best experts on this subject based on the ideXlab platform.
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Three-Dimensional High-Speed Optical Coherence Tomography for Diagnosis of Hypotony Maculopathy Following Glaucoma Filtration Surgery
2015Co-Authors: Margot L Goodkin, Dilraj S Grewal, David S GreenfieldAbstract:Purpose—To describe the clinical findings of hypotony Maculopathy using three dimensional (3D) topography maps reconstructed from spectral domain optical coherence tomography (SD-OCT) imaging, and compare SD-OCT with time domain OCT (TD-OCT) for hypotony Maculopathy diagnosis. Methods—This was an observational non-comparative case series comprising of seven patients with hypotony Maculopathy following trabeculectomy with mitomycin C. All patients underwent consecutive imaging with TD-OCT (Stratus OCT) and SD-OCT using various high-resolution instruments. 3D surface maps obtained using SD-OCT were compared to linear scans obtained using TD-OCT. Results—Two of 7 eyes had minimally detectable folds with TD-OCT imaging in the vertical axis. Five eyes did not show folds on TD-OCT. 3D topographic maps using SD-OCT demonstrated advanced retinal and subretinal folds throughout the macular region in all 7 patients. All eyes (4 cases) with no topographically detectable folds using SD-OCT within the foveal avascular zone had visual acuity of 20/25 or better. Three eyes with obvious contour disruption within the foveal pit had visual acuity ranging from 20/30 to 20/70. Conclusions—SD-OCT using 3D surface topography mapping provides greater sensitivity for hypotony Maculopathy diagnosis and monitoring as compared with TD-OCT. Disruption of the foveal pit as detected using SD-OCT is associated with reduced visual acuity
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three dimensional high speed optical coherence tomography for diagnosis of hypotony Maculopathy after glaucoma filtration surgery
Journal of Glaucoma, 2010Co-Authors: Margot L Goodkin, Dilraj S Grewal, David S GreenfieldAbstract:Purpose To describe the clinical findings of hypotony Maculopathy using 3-dimensional (3D) topography maps reconstructed from spectral domain optical coherence tomography (SD-OCT) imaging, and compare SD-OCT with time domain OCT (TD-OCT) for hypotony Maculopathy diagnosis. Methods This was an observational noncomparative case series comprising 7 patients with hypotony Maculopathy after trabeculectomy with mitomycin-C. All patients underwent consecutive imaging with TD-OCT (Stratus OCT) and SD-OCT using various high-resolution instruments. 3-D surface maps obtained using SD-OCT were compared with linear scans obtained using TD-OCT. Results Two of 7 eyes had minimally detectable folds with TD-OCT imaging in the vertical axis. Five eyes did not show folds on TD-OCT. 3-D topographic maps using SD-OCT demonstrated advanced retinal and subretinal folds throughout the macular region in all 7 patients. All eyes (4 cases) with no topographically detectable folds using SD-OCT within the foveal avascular zone had visual acuity of 20/25 or better. Three eyes with obvious contour disruption within the foveal pit had visual acuity ranging from 20/30 to 20/70. Conclusions SD-OCT using 3-D surface topography mapping provides greater sensitivity for hypotony Maculopathy diagnosis and monitoring as compared with TD-OCT. Disruption of the foveal pit as detected using SD-OCT is associated with reduced visual acuity.
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hypotony Maculopathy after filtering surgery with mitomycin c incidence and treatment
Ophthalmology, 1997Co-Authors: Ivan J Suner, David S Greenfield, Michael P Miller, Marcelo T Nicolela, Paul F PalmbergAbstract:Abstract Purpose: Hypotony Maculopathy after glaucoma filtering surgery with adjunctive mitomycin C has been reported to occur in 3% to 14% of cases. The authors evaluated its incidence when using a corneal safety-valve incision intended to reduce its occurrence. The authors also evaluated a technique for reversing hypotony Maculopathy by reoperation using two sets of stitches in the scleral flap, with one set tied tightly to temporarily raise the intraocular pressure, stretch the sclera, and flatten chorioretinal folds. Methods: The authors reviewed the results of 699 procedures performed between April 1991 and October 1994. All were performed or supervised by one surgeon (PFP). Results: Hypotony Maculopathy developed in 9 (1.3%) of 699 eyes. There was a statistically significant higher incidence in primary filters (4%) as compared to secondary filters or combined procedures. After revision, eight (89%) of nine recovered visual acuity of greater than or equal to 20130 and the mean intraocular pressure was 14.5 ± 4 mmHg at a mean follow-up of 15 months. Conclusion: The incidence of hypotony Maculopathy after glaucoma filtering surgery with mitomycin C using a corneal safety-valve incision is less than that reported in the literature without this incision. There is an increased risk in myopes, young patients, and primary filters. Early intervention with the described scleral flap revision technique usually allows restoration of prefiltration visual acuity without compromise of bleb function.
Francesco Viola - One of the best experts on this subject based on the ideXlab platform.
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sickle cell Maculopathy identification of systemic risk factors and microstructural analysis of individual retinal layers of the macula
PLOS ONE, 2018Co-Authors: Laura Dellarti, Giulio Barteselli, Lorenzo Riva, Elisa Carini, Giovanna Graziadei, Eleonora Benatti, Alessandro Invernizzi, Maria Domenica Cappellini, Francesco ViolaAbstract:PURPOSE: To identify systemic risk factors for sickle cell Maculopathy, and to analyze the microstructure of the macula of Sickle Cell Disease (SCD) patients by using automated segmentation of individual retinal layers. METHODS: Thirty consecutive patients with SCD and 30 matched controls underwent spectral-domain optical coherence tomography (SD-OCT) and automated thickness measurement for each retinal layer; thicknesses for SCD patients were then compared to normal controls. Demographic data, systemic data, and lab results were collected for each SCD patient; multivariate logistic regression analysis was used to identify potential risk factors for sickle cell Maculopathy. RESULTS: Ongoing chelation treatment (p = 0.0187) was the most predictive factor for the presence of sickle cell Maculopathy; the odds were 94.2% lower when chelation was present. HbF level tended to influence sickle cell Maculopathy (p = 0.0775); the odds decreased by 12.9% when HbF increased by 1%. Sickle cell Maculopathy was detected in 43% of SCD patients as patchy areas of retinal thinning on SD-OCT thickness map, mostly located temporally to the macula, especially in eyes with more advanced forms of sickle cell retinopathy (p = 0.003). In comparison to controls, SCD patients had a subtle thinning of the overall macula and temporal retina compared to controls (most p<0.0001), involving inner and outer retinal layers. Thickening of the retinal pigment epithelium was also detected in SCD eyes (p<0.0001). CONCLUSIONS: Chronic chelation therapy and, potentially, high levels of HbF are possible protective factors for the presence of sickle cell Maculopathy, especially for patients with more advanced forms of sickle cell retinopathy. A subtle thinning of the overall macula occurs in SCD patients and involves multiple retinal layers, suggesting that ischemic vasculopathy may happen in both superficial and deep capillary plexi. Thinning of the outer retinal layers suggests that an ischemic insult of the choriocapillaris may also occur in SCD patients.
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Sickle cell Maculopathy : identification of systemic risk factors, and microstructural analysis of individual retinal layers of the macula
'Public Library of Science (PLoS)', 2018Co-Authors: L. Dell&apos, Giulio Barteselli, Lorenzo Riva, Elisa Carini, Giovanna Graziadei, Eleonora Benatti, Alessandro Invernizzi, Maria Domenica Cappellini, Francesco ViolaAbstract:PURPOSE:To identify systemic risk factors for sickle cell Maculopathy, and to analyze the microstructure of the macula of Sickle Cell Disease (SCD) patients by using automated segmentation of individual retinal layers. METHODS: Thirty consecutive patients with SCD and 30 matched controls underwent spectral-domain optical coherence tomography (SD-OCT) and automated thickness measurement for each retinal layer; thicknesses for SCD patients were then compared to normal controls. Demographic data, systemic data, and lab results were collected for each SCD patient; multivariate logistic regression analysis was used to identify potential risk factors for sickle cell Maculopathy. RESULTS: Ongoing chelation treatment (p = 0.0187) was the most predictive factor for the presence of sickle cell Maculopathy; the odds were 94.2% lower when chelation was present. HbF level tended to influence sickle cell Maculopathy (p = 0.0775); the odds decreased by 12.9% when HbF increased by 1%. Sickle cell Maculopathy was detected in 43% of SCD patients as patchy areas of retinal thinning on SD-OCT thickness map, mostly located temporally to the macula, especially in eyes with more advanced forms of sickle cell retinopathy (p = 0.003). In comparison to controls, SCD patients had a subtle thinning of the overall macula and temporal retina compared to controls (most p
Amani A Fawzi - One of the best experts on this subject based on the ideXlab platform.
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AOSLO imaging in poppers Maculopathy shows high resolution loss of central macular cones
'Elsevier BV', 2021Co-Authors: Cole N. Rojas, Amani A Fawzi, Manjot K. GillAbstract:Purpose: To use new adaptive optics scanning laser ophthalmoscopy (AOSLO) technology to better image macular pathology in poppers Maculopathy. Observations: A 40-year-old patient was found to have poppers Maculopathy. Best corrected visual acuity was decreased to 20/40 OD and 20/50 OS, spectral domain optical coherence tomography found outer retinal disruption of the fovea, and AOSLO imaging showed significant decrease in cone density of the fovea of both eyes. Conclusions and importance: Poppers Maculopathy is a rare, but visually significant, complication of popper abuse. AOSLO technology demonstrates significant cone damage in poppers Maculopathy. The striking loss of cones revealed by AOSLO imaging shows how AOSLO imaging can elucidate macular pathology
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spectrum of retinal vascular diseases associated with paracentral acute middle Maculopathy
American Journal of Ophthalmology, 2015Co-Authors: Xuejing Chen, Ehsan Rahimy, Robert C Sergott, Renata Portella Nunes, Eduardo Cunha De Souza, Netan Choudhry, Nathan E Cutler, Samuel K Houston, Marion R Munk, Amani A FawziAbstract:Purpose To evaluate the spectrum of retinal diseases that can demonstrate paracentral acute middle Maculopathy and isolated ischemia of the intermediate and deep capillary plexus. Design Retrospective, multicenter, observational case series. Methods This is a retrospective case series review of 9 patients (10 eyes) from 5 centers with paracentral acute middle Maculopathy lesions and previously unreported retinal vascular etiologies. Case presentations and multimodal imaging, including color photographs, near-infrared reflectance, fluorescein angiography, spectral-domain optical coherence tomography (SD OCT), and orbital color Doppler imaging, are described. Baseline and follow-up findings are correlated with clinical presentation, demographics, and systemic associations. Results Five men and 4 women, aged 27–66 years, were included. Isolated band-like hyperreflective lesions in the middle retinal layers, otherwise known as paracentral acute middle Maculopathy, were observed in all patients at baseline presentation. Follow-up SD OCT analysis of these paracentral acute middle Maculopathy lesions demonstrated subsequent thinning of the inner nuclear layer. Novel retinal vascular associations leading to retinal vasculopathy and paracentral acute middle Maculopathy include eye compression injury causing global ocular ischemia, sickle cell crisis, Purtscher's retinopathy, inflammatory occlusive retinal vasculitis, post-H1N1 vaccine, hypertensive retinopathy, migraine disorder, and post–upper respiratory infection. Conclusion Paracentral acute middle Maculopathy lesions may develop in a wide spectrum of retinal vascular diseases. They are best identified with SD OCT analysis and may represent ischemia of the intermediate and deep capillary plexus. These lesions typically result in permanent thinning of the inner nuclear layer and are critical to identify in order to determine the cause of unexplained vision loss.
David Sarraf - One of the best experts on this subject based on the ideXlab platform.
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self inflicted laser handheld laser induced Maculopathy a novel ocular manifestation of factitious disorder
Retinal Cases & Brief Reports, 2017Co-Authors: Alessandro Rabiolo, Srinivas R Sadda, David Sarraf, Riccardo Sacconi, Chiara Giuffre, Eleonora Corbelli, Adriano Carnevali, Lea Querques, Bailey K Freund, Francesco BandelloAbstract:Purpose To report a case of factitious self-inflicted handheld laser-induced Maculopathy. Methods A 29-year-old man presented to our clinic complaining of a step-wise progressive loss of vision that abruptly began in his left eye but then became bilateral. He underwent comprehensive ocular examination, including visual acuity testing, biomicroscopic, dilated funduscopic examination, structural optical coherence tomography, en face structural optical coherence tomography, optical coherence tomography angiography, fundus autofluorescence, fluorescein angiography, and indocyanine green angiography. Results Visual acuity was 20/200 in both eyes. Although the multimodal imaging was highly consistent with handheld laser-induced Maculopathy, the patient continued to deny this behavior. Conclusion Self-inflicted handheld laser-induced Maculopathy may be a novel manifestation of factitious disorder. Clinical suspicion for this should remain high in the presence of the signature multimodal retinal findings despite denial by the patient. This category of patients could benefit from psychiatric referral, to prevent further ocular or extra-ocular self-injury.
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assessing deep retinal capillary ischemia in paracentral acute middle Maculopathy by optical coherence tomography angiography
American Journal of Ophthalmology, 2016Co-Authors: Julia Nemiroff, Ehsan Rahimy, Srinivas R Sadda, Laura Kuehlewein, Irena Tsui, Rishi R Doshi, A Gaudric, Michael B Gorin, David SarrafAbstract:Purpose To assess microvascular blood flow of the deep retinal capillary plexus in eyes with paracentral acute middle Maculopathy using optical coherence tomography (OCT) angiography. Design Retrospective, multicenter observational case series. Methods Clinical and multimodal imaging findings from 8 patients with paracentral acute middle Maculopathy were reviewed and analyzed. OCT angiography scans were analyzed and processed, and vessel density was calculated. Results Eight patients (7 male, 1 female, aged 9–82 years) were included. OCT angiography was obtained at either the acute (4 cases) or old stage (4 cases). Scans of the deep capillary plexus showed preservation of perfusion in acute lesions and capillary attenuation in old cases. Cases of central retinal artery occlusion showed marked loss of the deep capillary plexus. The mean vessel density of the superficial capillary plexus in normal fellow eyes was 12.8 ± 1.8 mm −1 vs 12.1 ± 1.9 mm −1 in eyes with paracentral acute middle Maculopathy (reduction −6.0%, P = .08). The mean vessel density of the deep capillary plexus in normal fellow eyes was 17.5 ± 1.4 mm −1 vs 14.7 ± 3.5 mm −1 in eyes with paracentral acute middle Maculopathy (reduction −19.4%, P = .04). This significant difference was representative of the eyes with old lesions. Conclusion Paracentral acute middle Maculopathy lesions correspond to preservation of perfusion in focal acute lesions and to pruning of the plexus in old cases. Cases of central retinal artery occlusion demonstrate marked hypoperfusion of the deep capillary plexus. Our study further supports an ischemic pathogenesis of this retinal vasculopathy.
