Publications by authors named "Adrienne W Scott"

56 Publications

HIF-1α and HIF-2α redundantly promote retinal neovascularization in patients with ischemic retinal disease.

J Clin Invest 2021 Jun;131(12)

Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.

Therapies targeting VEGF have proven only modestly effective for the treatment of proliferative sickle cell retinopathy (PSR), the leading cause of blindness in patients with sickle cell disease. Here, we shift our attention upstream from the genes that promote retinal neovascularization (NV) to the transcription factors that regulate their expression. We demonstrated increased expression of HIF-1α and HIF-2α in the ischemic inner retina of PSR eyes. Although both HIFs participated in promoting VEGF expression by hypoxic retinal Müller cells, HIF-1 alone was sufficient to promote retinal NV in mice, suggesting that therapies targeting only HIF-2 would not be adequate to prevent PSR. Nonetheless, administration of a HIF-2-specific inhibitor currently in clinical trials (PT2385) inhibited NV in the oxygen-induced retinopathy (OIR) mouse model. To unravel these discordant observations, we examined the expression of HIFs in OIR mice and demonstrated rapid but transient accumulation of HIF-1α but delayed and sustained accumulation of HIF-2α; simultaneous expression of HIF-1α and HIF-2α was not observed. Staggered HIF expression was corroborated in hypoxic adult mouse retinal explants but not in human retinal organoids, suggesting that this phenomenon may be unique to mice. Using pharmacological inhibition or an in vivo nanoparticle-mediated RNAi approach, we demonstrated that inhibiting either HIF was effective for preventing NV in OIR mice. Collectively, these results explain why inhibition of either HIF-1α or HIF-2α is equally effective for preventing retinal NV in mice but suggest that therapies targeting both HIFs will be necessary to prevent NV in patients with PSR.
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http://dx.doi.org/10.1172/JCI139202DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8203455PMC
June 2021

Artificial intelligence for improving sickle cell retinopathy diagnosis and management.

Eye (Lond) 2021 May 6. Epub 2021 May 6.

Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine and Hospital, Baltimore, MD, USA.

Sickle cell retinopathy is often initially asymptomatic even in proliferative stages, but can progress to cause vision loss due to vitreous haemorrhages or tractional retinal detachments. Challenges with access and adherence to screening dilated fundus examinations, particularly in medically underserved areas where the burden of sickle cell disease is highest, highlight the need for novel approaches to screening for patients with vision-threatening sickle cell retinopathy. This article reviews the existing literature on and suggests future research directions for coupling artificial intelligence with multimodal retinal imaging to expand access to automated, accurate, imaging-based screening for sickle cell retinopathy. Given the variability in retinal specialist practice patterns with regards to monitoring and treatment of sickle cell retinopathy, we also discuss recent progress toward development of machine learning models that can quantitatively track disease progression over time. These artificial intelligence-based applications have great potential for informing evidence-based and resource-efficient clinical diagnosis and management of sickle cell retinopathy.
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http://dx.doi.org/10.1038/s41433-021-01556-4DOI Listing
May 2021

Sickle Cell Disease and the Eye-Everything Old Is New Again.

Authors:
Adrienne W Scott

JAMA Ophthalmol 2021 03;139(3):337-338

Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland.

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http://dx.doi.org/10.1001/jamaophthalmol.2020.6526DOI Listing
March 2021

Association of Acute Macular Neuroretinopathy or Paracentral Acute Middle Maculopathy with Sickle Cell Disease.

Ophthalmol Retina 2021 Jan 19. Epub 2021 Jan 19.

Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland. Electronic address:

Purpose: To investigate the structural and microvascular changes in the retina and choriocapillaris in patients with sickle cell disease (SCD) demonstrating acute vision loss and acute macular neuroretinopathy (AMN) or paracentral acute middle maculopathy (PAMM) using multimodal imaging including OCT and OCT angiography (OCTA).

Design: Retrospective case series.

Participants: Four hemoglobin SS (HbSS) or hemoglobin SC (HbSC) patients who demonstrated vision loss attributed to AMN (n = 2) or PAMM (n = 2).

Methods: Clinical characteristics including best-corrected visual acuity and multimodal imaging features from fundus photography, fluorescein angiography, OCT, and OCTA were analyzed.

Main Outcome Measures: Longitudinal changes in the structure and microvasculature of the retina and choriocapillaris were examined.

Results: In 2 patients with AMN, characteristic hyperreflective changes were observed in the outer nuclear layer with involvement of the ellipsoid and interdigitation zones. In 1 patient, the lesion was located in the foveal avascular zone, and only flow deficits in the choriocapillaris were noted. In the second patient, no flow loss was observed in the superficial capillary plexus (SCP) corresponding to the lesion, but flow loss was seen in the deep capillary plexus (DCP) and choriocapillaris. At the respective 1-year and 4-month follow-up examinations, the hyperreflectivities improved with no ensuing retinal atrophy. Deep capillary plexus and choriocapillaris flow also improved. Of the 2 patients with PAMM, 1 had PAMM in association with branch retinal arteriolar occlusions. In both patients, a hyperreflective band involving the inner plexiform, inner nuclear, and outer plexiform layers was seen in or adjacent to areas of prior macular thinning. These areas corresponded to flow deficits in both the SCP and DCP. At the respective 1-month and 1-year follow-up examinations, the hyperreflective band improved with ensuing atrophy of the middle retinal layers. OCT angiography demonstrated worsening of flow deficits in the corresponding SCP and DCP.

Conclusions: To the best of our knowledge, this is the first report of AMN and the largest case series of AMN or PAMM in SCD to date. Paracentral acute middle maculopathy may precede macular thinning commonly observed in SCD. OCT angiography is a useful tool to demonstrate associated microvascular changes in AMN and PAMM in SCD.
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http://dx.doi.org/10.1016/j.oret.2021.01.003DOI Listing
January 2021

Deep Learning Detection of Sea Fan Neovascularization From Ultra-Widefield Color Fundus Photographs of Patients With Sickle Cell Hemoglobinopathy.

JAMA Ophthalmol 2021 02;139(2):206-213

Retina Division, Wilmer Eye Institute, The Johns Hopkins University School of Medicine and Hospital, Baltimore, Maryland.

Importance: Adherence to screening for vision-threatening proliferative sickle cell retinopathy is limited among patients with sickle cell hemoglobinopathy despite guidelines recommending dilated fundus examinations beginning in childhood. An automated algorithm for detecting sea fan neovascularization from ultra-widefield color fundus photographs could expand access to rapid retinal evaluations to identify patients at risk of vision loss from proliferative sickle cell retinopathy.

Objective: To develop a deep learning system for detecting sea fan neovascularization from ultra-widefield color fundus photographs from patients with sickle cell hemoglobinopathy.

Design, Setting, And Participants: In a cross-sectional study conducted at a single-institution, tertiary academic referral center, deidentified, retrospectively collected, ultra-widefield color fundus photographs from 190 adults with sickle cell hemoglobinopathy were independently graded by 2 masked retinal specialists for presence or absence of sea fan neovascularization. A third masked retinal specialist regraded images with discordant or indeterminate grades. Consensus retinal specialist reference standard grades were used to train a convolutional neural network to classify images for presence or absence of sea fan neovascularization. Participants included nondiabetic adults with sickle cell hemoglobinopathy receiving care from a Wilmer Eye Institute retinal specialist; the patients had received no previous laser or surgical treatment for sickle cell retinopathy and underwent imaging with ultra-widefield color fundus photographs between January 1, 2012, and January 30, 2019.

Interventions: Deidentified ultra-widefield color fundus photographs were retrospectively collected.

Main Outcomes And Measures: Sensitivity, specificity, and area under the receiver operating characteristic curve of the convolutional neural network for sea fan detection.

Results: A total of 1182 images from 190 patients were included. Of the 190 patients, 101 were women (53.2%), and the mean (SD) age at baseline was 36.2 (12.3) years; 119 patients (62.6%) had hemoglobin SS disease and 46 (24.2%) had hemoglobin SC disease. One hundred seventy-nine patients (94.2%) were of Black or African descent. Images with sea fan neovascularization were obtained in 57 patients (30.0%). The convolutional neural network had an area under the curve of 0.988 (95% CI, 0.969-0.999), with sensitivity of 97.4% (95% CI, 86.5%-99.9%) and specificity of 97.0% (95% CI, 93.5%-98.9%) for detecting sea fan neovascularization from ultra-widefield color fundus photographs.

Conclusions And Relevance: This study reports an automated system with high sensitivity and specificity for detecting sea fan neovascularization from ultra-widefield color fundus photographs from patients with sickle cell hemoglobinopathy, with potential applications for improving screening for vision-threatening proliferative sickle cell retinopathy.
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http://dx.doi.org/10.1001/jamaophthalmol.2020.5900DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7774049PMC
February 2021

Variable Practice Patterns for Management of Sickle Cell Retinopathy.

Ophthalmol Retina 2021 Jul 7;5(7):715-717. Epub 2020 Dec 7.

Wilmer Eye Institute, Department of Ophthalmology, Johns Hopkins University, Baltimore, Maryland. Electronic address:

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http://dx.doi.org/10.1016/j.oret.2020.11.017DOI Listing
July 2021

Implementation of Novel Protocols in an Ophthalmic Ambulatory Surgical Center to Resume Surgical Procedures During COVID-19.

Qual Manag Health Care 2021 Jan/Mar 01;30(1):69-73

Wilmer Eye Institute Ambulatory Surgical Centers, Johns Hopkins Surgical Center Series, Bel Air, Maryland.

Background And Objectives: With the resumption of elective ophthalmic surgery during COVID-19, revised protocols were mandated to protect both staff and patients from transmission while increasing case numbers. We discuss a widely generalizable and in-depth protocol intended to safely allow the restart of elective procedures in 2 dedicated ophthalmic ambulatory surgical centers (ASCs) using monitored anesthesia care.

Methods: A single-center review of protocols and practices designed to limit COVID-19 transmission.

Results: All patients were tested within 72 hours prior to the procedure with a COVID-19 nasal swab to assess for active disease. A distance of 6 ft between each stretcher and the staff within the ASC was maintained when possible. Preoperative anesthetizing and dilating eye drops were administered from multiuse bottles without contact with surfaces. Surgical cases were restarted at a reduced capacity of a maximum of 7 per day to distance patient arrivals. Removal of waiting room chairs and the creation of new break areas allowed for social distancing.

Conclusion: As recommendations change on the basis of an increased understanding of the COVID-19 virus, ophthalmologists and ASC staff need to tailor protocols and workflows to limit transmission of virus with resumption of ocular surgery.
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http://dx.doi.org/10.1097/QMH.0000000000000285DOI Listing
December 2020

Longitudinal assessment of quantitative ultra-widefield ischaemic and vascular parameters in sickle cell retinopathy.

Br J Ophthalmol 2020 Oct 31. Epub 2020 Oct 31.

The Tony and Leona Campane Center for Excellence in Image-Guided Surgery and Advanced Imaging Research, Cole Eye Institute, Cleveland Clinic, Cleveland, Ohio, USA

Purpose: To evaluate longitudinal quantitative ischaemic and vasculature parameters, including ischaemic index, vessel area, length and geodesic distance in sickle cell retinopathy (SCR) on ultra-widefield fluorescein angiography (UWFA).

Methods: Optimal UWFA images from two longitudinal timepoints of 74 eyes from 45 patients with SCR were aligned and a common region of interest was determined. A deep-learning augmented ischaemia and vascular segmentation platform was used for feature extraction. Geodesic distance maps demonstrating the shortest distance within the vascular masks from the centre of the optic disc were created. Ischaemic index, vessel area, vessel length and geodesic distance were measured. Paired t-test and linear mixed effect model analysis were performed.

Results: Overall, 25 (44 eyes) patients with HbSS, 14 (19 eyes) with HbSC, 6 (11 eyes) with HbSthal and other genotypes were included. Mean age was 40.1±11.0 years. Mean time interval between two UWFA studies was 23.0±15.1 months (range: 3-71.3). Mean panretinal ischaemic index increased from 10.0±7.2% to 10.9±7.3% (p<0.005). Mean rate of change in ischaemic index was 0.5±0.7% per year. Mean vessel area (p=0.020) and geodesic distance (p=0.048) decreased significantly. Multivariate analysis demonstrated baseline ischaemic index and Goldberg stage are correlated with progression.

Conclusion: Longitudinal ischaemic index and retinal vascular parameter measurements demonstrate statistically significant progression in SCR. The clinical significance of these relatively small magnitude changes remains unclear but may provide insights into the progression of retinal ischaemia in SCR.
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http://dx.doi.org/10.1136/bjophthalmol-2020-317241DOI Listing
October 2020

Use of Contact Lenses to Optimize OCT Scans of the Optic Nerve in Glaucoma Suspects or Patients with Glaucoma with High Myopia.

Ophthalmol Glaucoma 2020 May - Jun;3(3):196-201. Epub 2020 Jan 14.

Wilmer Eye Institute, Johns Hopkins University School of Medicine, Bel Air, Maryland; Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland.

Purpose: Patients with myopia are at increased risk for the development of glaucoma. The inability to correct for axial length on spectral-domain (SD) OCT translates into lower signal strength and scan reliability in patients with high axial myopia. We evaluated the effectiveness of a contact lens to increase the signal strength and to assess optic nerve dimensions and nerve fiber layer (NFL) thickness using SD OCT in patients with glaucoma or who are glaucoma suspects with high axial myopia.

Design: Single-center, prospective, interventional study.

Participants: Patients with axial lengths of more than 25.5 mm with a diagnosis of glaucoma or glaucoma suspect.

Methods: The optic nerve cube 200×200 scan using the Cirrus SD OCT 400 (Carl Zeiss Meditec, Inc., Dublin, CA) was carried out first without the use of a contact lens and then repeated with placement of the contact lens to correct for the spherical equivalent of the refractive error.

Main Outcome Measures: The primary outcome measure was the change in the average NFL thickness before and after use of the contact lens. Secondary outcome measures included the changes in cup volume, disc area, and rim area on OCT.

Results: Twelve patients were recruited (20 eyes); the average axial length was 27.06 mm, and the average signal strength interval increased by 1.73 (P = 0.001). With the use of a contact lens, the average NFL thickness was significantly thicker. None of the changes in the secondary outcome measures were significant: rim area, cup volume, or disc area.

Conclusions: Based on our data, the use of a contact lens statistically improved the signal strength and average NFL thickness of the SD OCT scan. The ability to capture the perimeter of the optic disc accurately can be limited in the setting of peripapillary atrophy, which was present in all but 2 participants. Future studies with a larger number of participants and a wider range of axial myopia to discern if contact lens correction has a greater effect on the highest axial lengths are needed.
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http://dx.doi.org/10.1016/j.ogla.2020.01.002DOI Listing
January 2020

Interocular asymmetry of foveal avascular zone morphology and parafoveal capillary density in sickle cell retinopathy.

PLoS One 2020 10;15(6):e0234151. Epub 2020 Jun 10.

Ophthalmology, New York Eye and Ear Infirmary of Mount Sinai, New York, New York, United States of America.

Objectives: To examine interocular asymmetry of foveal avascular zone (FAZ) and parafoveal capillary density metrics in sickle cell retinopathy (SCR) using optical coherence tomography angiography (OCT-A).

Methods: This cross-sectional, retrospective study evaluated SCR patients and unaffected controls who underwent 3x3mm macular OCT-A imaging using a spectral domain-OCT system. FAZ (area, perimeter, and acircularity index) and parafoveal capillary density metrics were computed for both eyes of each participant. In unaffected controls, interocular difference in FAZ and parafoveal capillary density metrics were evaluated using Bland-Altman plots. SCR patients with interocular difference outside the upper 97.5% and lower 2.5% limits of agreement from controls were defined as having interocular asymmetry. Area under receiver operating characteristic curve (AROC) was also performed to determine the ability of the absolute interocular difference to differentiate between subjects with SCR-including non-proliferative SCR (NP-SCR) and proliferative SCR (P-SCR)-and unaffected controls.

Results: Thirty-one patients with SCR (21 NP-SCR and 10 P-SCR) and 14 race-matched and age-matched controls were included for analysis. Interocular asymmetry was seen for all FAZ and parafoveal capillary density metrics in NP-SCR and P-SCR subjects. SCR subjects showed greater disease severity in the left-eye for FAZ and parafoveal capillary density metrics.

Conclusions: NP-SCR and P-SCR patients demonstrated quantifiable interocular asymmetry in FAZ and parafoveal capillary density metrics compared to unaffected subjects, with left-eye predominance in disease severity.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0234151PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7286490PMC
August 2020

Wide-field imaging of sickle retinopathy.

Int J Retina Vitreous 2019 12;5(Suppl 1):27. Epub 2019 Dec 12.

Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, 600 North Wolfe Street, Maumenee 719, Baltimore, MD 21287 USA.

Background: Wide-field imaging is a newer retinal imaging technology, capturing up to 200 degrees of the retina in a single photograph. Individuals with sickle cell retinopathy commonly exhibit peripheral retinal ischemia. Patients with proliferative sickle cell retinopathy develop pathologic retinal neovascularization of the peripheral retina which may progress into sight-threatening sequelae of vitreous hemorrhage and/or retinal detachment. The purpose of this review is to provide an overview of current and future applications of wide-field retinal imaging for sickle cell retinopathy, and recommend indications for best use.

Main Body: There are several advantages to wide-field imaging in the clinical management of sickle cell disease patients. Retrospective and prospective studies support the success of wide-field imaging in detecting more sickle cell induced retinal microvascular abnormalities than traditional non-wide-field imaging. Clinicians can easily capture a greater extent of the retinal periphery in a patient's clinical baseline imaging to follow the changes at an earlier point and determine the rate of progression over time. Wide-field imaging minimizes patient and photographer burden, necessitating less photos and technical skill to capture the peripheral retina. Minimizing the number of necessary images can be especially helpful for pediatric patients with sickle cell retinopathy. Wide-field imaging has already been successful in identifying new biomarkers and risk factors for the development of proliferative sickle cell retinopathy. While these advantages should be considered, clinicians need to perform a careful risk-benefit analysis before ordering this test. Although wide-field fluorescein angiography successfully detects additional pathologic abnormalities compared to traditional imaging, a recent research study suggests that peripheral changes differentially detected by wide-field imaging may not change clinical management for most sickle cell patients.

Conclusions: While wide-field imaging may not carry a clinically significant direct benefit to all patients, it shows future promise in expanding our knowledge of sickle cell retinopathy. Clinicians may monitor peripheral retinal pathology such as retinal ischemia and retinal neovascularization over progressive time points, and use sequential wide-field retinal images to monitor response to treatment. Future applications for wide-field imaging may include providing data to facilitate machine learning, and potential use in tele-ophthalmology screening for proliferative sickle retinopathy.
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http://dx.doi.org/10.1186/s40942-019-0177-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6907105PMC
December 2019

Retinal Thickness and Microvascular Changes in Children With Sickle Cell Disease Evaluated by Optical Coherence Tomography (OCT) and OCT Angiography.

Am J Ophthalmol 2020 01 29;209:88-98. Epub 2019 Aug 29.

Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. Electronic address:

Purpose: To compare the severity of macular vascular changes in children with sickle cell disease (SCD) vs age- and race-matched controls.

Design: Cross-sectional study.

Methods: Children (<18 years old) with HbSS and HbS variant (HbSC and HbS thalassemia) genotypes, and their age- and race-matched controls, were recruited between January 2017 and December 2018. All subjects underwent optical coherence tomography angiography (OCTA) scans centered on the fovea and temporal macula. Retinal thickness, superficial capillary plexus (SCP) and deep capillary plexus (DCP) vessel density (VD), and foveal avascular zone (FAZ) size were measured and compared between HbSS and HbS variant vs controls.

Results: Thirty-four HbSS, 34 HbS variant (Goldberg staging 0-3 for SCD eyes), and 24 control eyes (total 48 children, aged 5-17 years) were included. Total VD (3-mm ETDRS circle) was lower in HbS variant eyes than in controls for both the SCP (42.9% vs 47.7%, P = .02) and DCP (47.4% vs 52.6%, P = .01). In HbSS eyes, VD was lower in the DCP (47.7%, P = .008) but not in the SCP (45.5%, P = .5), compared to controls. A higher proportion of HbSS (n = 18, 55%) than HbS variant eyes (n = 9, 26%) had pathologic areas of retinal thinning associated with SCP and DCP flow loss (P = .03). However, retinal thickness measurements and FAZ size did not differ between either HbSS or HbS variant group vs controls.

Conclusions: Children with SCD have similar retinal thickness but less dense vasculature on OCTA compared to age and race-matched controls, suggesting that microvascular insult may precede structural thinning.
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http://dx.doi.org/10.1016/j.ajo.2019.08.019DOI Listing
January 2020

Foveal avascular zone morphology and parafoveal capillary perfusion in sickle cell retinopathy.

Br J Ophthalmol 2020 04 23;104(4):473-479. Epub 2019 Jul 23.

Ophthalmology, New York Eye and Ear Infirmary of Mount Sinai, New York City, New York, USA

Background/aims: To assess foveal avascular zone (FAZ) morphology and parafoveal capillary perfusion in patients with various stages of sickle cell retinopathy (SCR) using optical coherence tomography angiography (OCT-A).

Methods: This is a multi-institutional retrospective study of patients with various stages of SCR compared with healthy controls. Parafoveal OCT-A images obtained using a commercial spectral domain-OCT system were reviewed. Foveal-centred 3×3 mm full vascular slab OCT-As were used for image processing and data analysis. FAZ area, perimeter, and acircularity index were determined on the OCT-A image after manual delineation of the FAZ border. Quadrant-based parafoveal capillary density and per cent area deviating from normal distribution were also measured.

Results: Fifty-two patients with SCR (33 non-proliferative and 19 proliferative) and 20 age and race-matched healthy controls were included. One randomly selected eye per study participant was analysed. FAZ perimeter and acircularity index were significantly greater in SCR eyes when compared with the controls. While parafoveal capillary density was significantly lower, per cent area deviated from normal distribution was significantly higher in SCR eyes than that of the control. However, no statistically significant difference between the two SCR stages was observed. In quadrant-based analysis, the temporal quadrant showed greater parafoveal capillary dropout due to SCR, with the most profound effect in patients with proliferative SCR.

Conclusions: Abnormal FAZ morphology and altered parafoveal capillary perfusion were found in patients with SCR. Our customised OCT-A image analysis method uniquely highlights significant quantitative alterations in perfusion density mapping in a qualitative display, with minimal obscuration of OCT-A image detail.
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http://dx.doi.org/10.1136/bjophthalmol-2019-314567DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6980907PMC
April 2020

Correlation of Ultra-Widefield Fluorescein Angiography and OCT Angiography in Sickle Cell Retinopathy.

Ophthalmol Retina 2018 06 9;2(6):599-605. Epub 2017 Dec 9.

Retina Division, Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland. Electronic address:

Purpose: To determine whether the degree of peripheral nonperfusion seen on ultra-widefield (UWF) fluorescein angiography (FA) correlates with measures of macular vascular flow as seen on OCT angiography (OCTA) in sickle cell retinopathy.

Design: Prospective, observational study.

Participants: Patients with sickle cell disease undergoing an eye examination at an urban, tertiary medical center.

Methods: All patients underwent dilated fundus examination as well as UWF FA and macular OCTA imaging on the same day. The peripheral nonperfusion seen on UWF FA was measured to calculate an ischemic index (visualized nonperfusion/total visualized retinal area × 100%), and OCTA measurements of macular vessel density were recorded. The degree of peripheral nonperfusion and vessel density were then correlated.

Main Outcome Measures: Correlation between ischemic index as seen on UWF FA and macular vessel density on OCTA.

Results: Thirty-six eyes from 19 patients (10 women, 9 men) with a mean age of 30.8 years were included. Sickle genotypes included 14 patients with SS (73.7%), 4 with SC (21.1%), and 1 with β-thalassemia (5.2%). Average ischemic index was 4.4% for all eyes and was found to be higher in patients with sickle SC (8.0%) than in those with sickle SS (3.2%; P = 0.01). Ischemic index also was higher in those with proliferative sickle cell retinopathy (9.3%) than in those without (2.8%; P < 0.01). Ischemic index on UWF FA showed a statistically significant correlation (P < 0.05) with vessel density on OCTA in the temporal subfield of the superficial capillary plexus and in all subfields of the deep capillary plexus.

Conclusions: Peripheral nonperfusion seen on UWF FA is greater in those with sickle SC disease and proliferative retinopathy and is correlated with macular vessel density on OCTA, especially the deep retinal plexus.
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http://dx.doi.org/10.1016/j.oret.2017.10.011DOI Listing
June 2018

Evolution of Leukemic Retinal Hemorrhages Documented by Spectral-Domain OCT and Color Fundus Photography.

Ophthalmol Retina 2018 05 18;2(5):494-501. Epub 2017 Oct 18.

Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland. Electronic address:

Purpose: Retinal hemorrhages are observed frequently in patients with leukemia. However, little is known about the impact and natural history of these hemorrhages. The purpose of this study was to describe leukemic retinal hemorrhages using multimodal imaging and to monitor their evolution longitudinally.

Design: Retrospective case series.

Participants: A total of 11 eyes of 7 symptomatic leukemic patients with posterior segment hemorrhages.

Methods: Single-center study performed at the Johns Hopkins Hospital. Symptomatic leukemic patients with posterior segment hemorrhages underwent serial dilated fundus examinations. The hemorrhages were documented longitudinally with color fundus photographs and spectral-domain (SD) OCT.

Main Outcomes Measures: Microanatomic locations of leukemic retinal hemorrhages and their impact on vision and evolution over time.

Results: A total of 7 patients (71.4% men; 57.1% white, 28.6% black, and 14.3% Hispanic) were included, with 11 eyes showing posterior segment hemorrhages. The median age at presentation was 49.8 years. All patients had intraretinal hemorrhages; these involved the vitreous and sub-internal limiting membrane (ILM) space in 1 and 3 patients, respectively. The median total follow-up duration was 4.0 months. At the final follow-up visits, 4 of 6 patients showed complete resolution of hemorrhages on examination and color fundus photographs. The final SD-OCT images of all patients did not show any retinal thinning, disruption of the ellipsoid zone, disorganization of the retinal layers, intraretinal fluid, or subretinal fluid.

Conclusions: Symptomatic leukemic retinal hemorrhages are associated with anemia and thrombocytopenia. These hemorrhages, including visually significant central sub-ILM hemorrhages, tend to be self-limiting and resolve within a few months with treatment of the underlying disease.
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http://dx.doi.org/10.1016/j.oret.2017.08.014DOI Listing
May 2018

Progressive Retinal Thinning in Sickle Cell Retinopathy.

Ophthalmol Retina 2018 12 24;2(12):1241-1248.e2. Epub 2018 Aug 24.

Wilmer Eye Institute, Johns Hopkins School of Medicine, Baltimore, Maryland. Electronic address:

Purpose: To determine the rate of retinal thinning on spectral-domain (SD) OCT in patients with sickle cell compared with controls.

Design: Retrospective, longitudinal study.

Participants: Patients with sickle cell and age-similar control patients with SD-OCT macula volume scans at least 10 months apart were reviewed.

Methods: Automated segmentation of the SD-OCT macula volume scans was performed using the Iowa Reference Algorithms to divide the retina into 3 combined retina layers: superficial (retinal nerve fiber layer and ganglion cell layer), middle (inner plexiform layer and inner nuclear layer), and outer (outer plexiform layer to retinal pigment epithelium). The mean retinal thickness in each Early Treatment Diabetic Retinopathy Study (ETDRS) subfield was recorded. The rate of retinal thickness change was calculated using a multilevel mixed-effects model.

Main Outcome Measures: The rate of retinal thickness change in the superficial, middle, and outer retina.

Results: Thirty-eight eyes of 24 patients with sickle cell (age: mean 40 years, range 18-67 years; genotype: 12 hemoglobin SS, 11 hemoglobin SC, 1 not available) and 30 eyes of 30 control patients were followed on average for 25.5 months. The mean initial retinal thickness was thinner in the sickle cell group compared with the control group (superficial: 69.95 vs. 72.22 μm; middle: 65.24 vs. 71.22 μm; outer: 171.37 vs. 175.55 μm). The mean rate of retinal thinning was greater for the sickle cell group than the control group (superficial: -0.89 vs. -0.41 μm/year; middle: -0.45 vs. +0.18 μm/year; outer: -0.50 vs. -0.02 μm/year). The rate of thinning in patients with sickle cell was statistically significant (P < 0.05) for the superficial retina in the center subfield and nasal and temporal inner subfields, as well as the middle retina in the temporal inner subfield. Compared with controls, there was a faster rate of retinal thinning for the middle retina in the temporal inner subfield. Rates of retinal thinning were similar regardless of whether qualitative areas of focal retinal thinning were seen on initial imaging.

Conclusions: Patients with sickle cell exhibit a progressive retinal thinning predominantly affecting the superficial and middle retina that may represent chronic microvascular insult.
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http://dx.doi.org/10.1016/j.oret.2018.07.006DOI Listing
December 2018

Loss of Peak Vision in Retinal Vein Occlusion Patients Treated for Macular Edema.

Am J Ophthalmol 2019 09 4;205:17-26. Epub 2019 Apr 4.

Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, USA. Electronic address:

Purpose: To evaluate long-term visual and anatomic outcomes in patients with retinal vein occlusion (RVO) treated with anti-vascular endothelial growth factor (VEGF) agents.

Design: Prospective, interventional case series.

Participants: Patients with central RVO (CRVO) or branch RVO (BRVO).

Methods: Number of anti-VEGF injections and improvement from baseline best-corrected visual acuity (BCVA) and central subfield thickness (CST) were prospectively recorded in 40 eyes of 39 CRVO patients and 50 eyes of 47 BRVO patients.

Results: Mean follow-up was 58 months for BRVO and 78 months for CRVO. Within 6 months of last follow-up, 58% of BRVO patients and 75% of CRVO patients required anti-VEGF injections to control edema. Analysis of the course of each patient over time showed that for BRVO patients, BCVA letter score increased by a mean of 24, from baseline of 52 (20/100) to peak of 76 (20/32), and subsequently decreased by 13, to 63 (20/50), at final visit; and for CRVO patients, BCVA letter score increased by a mean of 26, from baseline of 48 (20/100) to peak of 74 (20/32), and subsequently decreased by 18, to 56 (20/80), at last follow-up. Loss from peak BCVA occurred primarily owing to persistent/recurrent edema and related foveal damage.

Conclusions: Patients with RVO showed large improvements in BCVA after initiation of anti-VEGF injections, but in many patients some visual gains were lost over time owing to bouts of recurrent edema. Sustained suppression of VEGF may help to provide optimal outcomes in RVO and reduce treatment burden.
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http://dx.doi.org/10.1016/j.ajo.2019.03.029DOI Listing
September 2019

Outcome After Exchange Transfusion for Central Retinal Vein Occlusion Associated With Extensive Capillary and Arteriolar Nonperfusion in a Patient With Hemoglobin SS Disease.

JAMA Ophthalmol 2019 06;137(6):718-720

Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine and Hospital, Baltimore, Maryland.

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http://dx.doi.org/10.1001/jamaophthalmol.2019.0165DOI Listing
June 2019

Detection of Age-Related Macular Degeneration by Portable Optical Coherence Tomography Operated by Nonexpert Personnel: Potential Use for Screenings.

J Vitreoretin Dis 2019 Jan 20;3(1):16-20. Epub 2018 Nov 20.

Retina Division, Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD, USA.

Purpose: The purpose of this study is to determine the sensitivity and specificity of detecting age-related macular degeneration (AMD) using portable optical coherence tomography (OCT) operated by nonexpert photographers on undilated patients.

Methods: In this case-control study, 92 individuals were recruited from the glaucoma and retina clinics at the Wilmer Eye Institute (Johns Hopkins University, Baltimore, MD). Using the portable iVue (Optovue, Inc, Fremont, CA) spectral-domain OCT (SD-OCT), 2 nonexpert photographers acquired retina map scans on undilated eyes of all participants. In total, 33 AMD eyes and 105 control eyes were evaluated and graded by ophthalmologists masked to the diagnoses.

Results: Detection of AMD on the portable OCT by ophthalmologists exhibited sensitivities of 0.91 and 0.94 and specificities of 0.88 and 0.89, for graders 1 and 2, respectively. A strong intergrader agreement was observed (κ = 0.87).

Conclusions: Nonexpert photographers can use a portable OCT on undilated eyes to acquire images for the detection of AMD. These findings present the potential utility of implementing a portable OCT in community screenings for earlier detection and treatment of disease.
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http://dx.doi.org/10.1177/2474126418810146DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8142863PMC
January 2019

Atopic dermatitis is associated with increased prevalence of multiple ocular comorbidities.

J Allergy Clin Immunol Pract 2019 Jan 17;7(1):298-299. Epub 2018 Oct 17.

Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, Md; Johns Hopkins Bloomberg School of Public Health, Baltimore, Md. Electronic address:

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http://dx.doi.org/10.1016/j.jaip.2018.10.009DOI Listing
January 2019

Clinical and Ophthalmic Factors Associated With the Severity of Sickle Cell Retinopathy.

Am J Ophthalmol 2019 01 29;197:105-113. Epub 2018 Sep 29.

Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. Electronic address:

Purpose: To identify associations between severity of sickle cell retinopathy (SCR) and other clinical, laboratory, or treatment factors relevant to sickle cell disease (SCD).

Design: Retrospective cohort study.

Methods: We investigated clinical, laboratory, and demographic associations with the severity of SCR in 296 patients seen at both our SCD specialty clinic and our retina clinic. Multivariate multinomial logistic regression was used to estimate the association between each clinical variable and severity of SCR.

Results: Multivariate analysis showed that in patients with sickle cell anemia (SCA) genotypes, older age (95% confidence interval [CI], 1.04-1.15; P < .001) and male sex (95% CI, 0.13-0.87; P = .02) were associated with proliferative sickle cell retinopathy (PSR). In patients with genotypic variants, visual symptoms (95% CI, 1.36-21.62; P = .02) were associated with PSR. Laser photocoagulation and vitrectomy surgery, the standard interventions for PSR, were associated with older age (95% CI, 1.05-1.13; P < .001), visual symptoms (95% CI, 1.48-7.40; P = .004), higher hemoglobin level (95% CI, 1.14-1.65; P = .001), and no chronic transfusion (95% CI, 0.16-1.09; P = .08) across the whole cohort.

Conclusions: These findings may inform clinicians of the symptoms, systemic findings, and disease-modifying therapies most frequently associated with SCR in SCD patients. Visual symptoms such as blurred vision or floaters were associated with progression of SCR and may be criteria for referral for retinal examination. Chronic transfusion therapy may be protective against the need for retinal laser photocoagulation or vitrectomy. Prospective studies are necessary to further explore risk factors for SCR and to identify which individuals with SCD are at risk for incident or progression of retinopathy.
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http://dx.doi.org/10.1016/j.ajo.2018.09.025DOI Listing
January 2019

Vision Preference Value Scale and Patient Preferences in Choosing Therapy for Symptomatic Vitreomacular Interface Abnormality.

JAMA Ophthalmol 2018 06;136(6):658-664

Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland.

Importance: While symptomatic vitreomacular interface abnormalities (VIAs) are common, assessment of vision preference values and treatment preferences of these may guide treatment recommendations by physicians and influence third-party payers.

Objective: To determine preference values that individuals with VIA assign to their visual state and preferences of potential treatments.

Design, Setting, And Participants: In this cross-sectional one-time questionnaire study conducted between December 2015 and January 2017, 213 patients from tertiary care referral centers in Thailand, the United Kingdom, and the United States were studied. Patients with symptomatic VIA diagnosed within 1 year of data collection, visual acuity less than 20/20 OU, and symptoms ascribed to VIAs were included. Data were analyzed from January 2017 to November 2017.

Main Outcomes And Measures: The primary end points were overall mean preference value that individuals with VIA assigned to their visual state and patients' preferences for potential treatments. Preference values were graded on a scale from 0 to 1, with 0 indicating death and 1 indicating perfect health with perfect vision.

Results: Of the 213 included patients, 139 (65.3%) were women, and the mean (SD) age was 65.6 (7.7) years. Diagnoses included epiretinal membrane (n = 100 [46.9%]), macular hole (n = 99 [46.5%]), and vitreomacular traction (n = 14 [6.6%]). The mean (SD) vision preference value was 0.76 (0.15), without differences identified among the 3 VIA types. More participants were enthusiastic about vitrectomy (150 [71.1%]) compared with intravitreal injection (120 [56.9%]) (difference, 14.2%; 95% CI, 5.16-23.3; P = .002). Adjusted analyses showed enthusiasm for vitrectomy was associated with fellow eye visual acuity (odds ratio, 10.99; 95% CI, 2.01-59.97; P = .006) and better-seeing eye visual acuity (odds ratio, 0.03; 95% CI, 0.001-0.66; P = .03). Overall enthusiasm for treatment was associated with fellow eye visual acuity (odds ratio, 7.22; 95% CI, 1.29-40.40; P = .02). Overall, most participants (171 [81.0%]) were enthusiastic about surgery, injection, or both.

Conclusions And Relevance: Study participants reported similar preference values among 3 types of VIAs. The data suggest that most patients with these conditions would be enthusiastic about undergoing vitrectomy or an injection to treat it, likely because of the condition's effect on visual functioning, although there may be a slight preference for vitrectomy at this time.
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http://dx.doi.org/10.1001/jamaophthalmol.2018.1272DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6145775PMC
June 2018

Multimodal Retinal Imaging in Incontinentia Pigmenti Including Optical Coherence Tomography Angiography: Findings From an Older Cohort With Mild Phenotype.

JAMA Ophthalmol 2018 05;136(5):467-472

Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland.

Importance: Incontinentia pigmenti (IP) is a rare, X-linked dominant disease with potentially severe ocular complications that predominantly affect the peripheral retina. However, little is known about its effects on the macula.

Objective: To describe the structural and vascular abnormalities observed in the maculas of patients with IP and to correlate these findings with peripheral pathologies.

Design, Setting, And Participants: Prospective, cross-sectional study at Wilmer Eye Institute, Johns Hopkins University. Five participants with a clinical diagnosis of IP were included and underwent multimodal imaging with ultra-wide-field fluorescein angiography (FA), spectral-domain optical coherence tomography (OCT), and OCT angiography.

Main Outcomes And Measures: The structural and vascular abnormalities observed on spectral-domain OCT and OCT angiography and their correlation with peripheral pathologies seen on ultra-wide-field FA.

Results: A total of 9 eyes from 5 patients (median age, 20.5 years; range, 8.4-54.2 years) were included. Median Snellen visual acuity was 20/32 (range, 20/16 to 20/63). ultra-wide-field FA-identified retinal vascular abnormalities in all 7 eyes in which FA was obtained. These abnormalities included microaneurysms, areas of nonperfusion, and vascular anastomoses, most of which were peripheral to the standard view of 30° FA with peripheral sweeps. Structural abnormalities were observed in 6 eyes on spectral-domain OCT, including inner retinal thinning and irregularities in the outer plexiform layer. Optical coherence tomography angiography abnormalities were noted in all 9 eyes, including decreased vascular density, abnormal vascular loops, and flow loss in the superficial and deep plexuses, which corresponded to areas of retinal thinning on spectral-domain OCT.

Conclusions And Relevance: Although our study is limited by the small sample size, the findings suggest that multimodal imaging is useful for detecting structural and vascular abnormalities that may not be apparent on ophthalmoscopy in patients with IP. Macular pathologies, especially a decrease in vascular density on OCT angiography, are common. Further studies are needed to characterize further the association between macular and peripheral abnormalities in patients with IP.
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http://dx.doi.org/10.1001/jamaophthalmol.2018.0475DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5876832PMC
May 2018

Shortest Distance From Fovea to Subfoveal Hemorrhage Border Is Important in Patients With Neovascular Age-related Macular Degeneration.

Am J Ophthalmol 2018 05 28;189:86-95. Epub 2018 Feb 28.

The Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland. Electronic address:

Purpose: To identify factors influencing visual outcome in patients with neovascular age-related macular degeneration (NVAMD) and subfoveal hemorrhage (SFH) treated with anti-vascular endothelial growth factor (VEGF) agents.

Design: Retrospective case series.

Methods: Anti-VEGF-treated eyes with SFH > 1 disc area (DA) were identified (n = 16) and changes in visual acuity (VA) and central subfield thickness (CST) from baseline to last follow-up, along with SFH area, thickness, minimum distance from fovea to SFH border, and time to resolution, were determined.

Results: At baseline, mean (± standard error of the mean) size and thickness of SFH were 14.9 ± 2.8 DA and 386.6 ± 46.9 μm, and mean Snellen VA and CST were 20/250 and 591.7 ± 57.0 μm. Median follow-up was 47.6 months. While more than 50% of patients had VA ≤ 20/200 at baseline and all time points through week 48, the percentage of patients with VA ≥ 20/50 increased to 30%-40% at months 6 and 12 and remained stable through month 48. Spearman rank correlation demonstrated 2 independent variables that correlated with good visual outcome, smaller area of SFH at baseline (r = -0.630; P = .009), and high frequency of anti-VEGF injections (r = 0.646; P = .007). In exceptional patients with good visual outcome despite large baseline SFH, shortest distance between the fovea and hemorrhage border significantly correlated with baseline VA (r = -0.503, P = .047) and final VA (r = -0.575, P = .02).

Conclusions: Patients with NVAMD and thick SFH, but short distance between fovea and uninvolved retina, can have good visual outcomes when given frequent anti-VEGF injections.
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http://dx.doi.org/10.1016/j.ajo.2018.02.015DOI Listing
May 2018

UTILITY OF ULTRA-WIDEFIELD RETINAL IMAGING FOR THE STAGING AND MANAGEMENT OF SICKLE CELL RETINOPATHY.

Retina 2019 May;39(5):836-843

Retina Division, Wilmer Eye Institute, Johns Hopkins School of Medicine, Baltimore, Maryland.

Purpose: To determine whether ultra-widefield (UWF) retinal imaging changes the staging or management of sickle cell retinopathy compared with clinical examination.

Methods: Prospective, observational study including patients with sickle cell disease. All patients underwent dilated fundus examination by a fellowship-trained retina specialist, as well as UWF fundus photography (FF) and fluorescein angiography (FA). Sickle retinopathy stage and treatment recommendation per eye were determined after clinical examination, UWF-FF, and UWF-FA, respectively, and differences in retinopathy stage and treatment recommendation were compared.

Results: A total of 70 eyes from 35 patients (17 women, 48.6%), mean age 30.4 years, were included. Sickle genotypes included 26 patients with sickle SS (74.3%), 7 SC (20.0%), and 2 β(+)thalassemia (5.7%). Based on examination, most eyes (42/70; 60.0%) had no visible retinopathy. Based on UWF-FF, about half of the eyes were found to be Goldberg Stage 2 or above (36/70; 51.4%). Based on UWF-FA, nearly all eyes were Goldberg Stage 2 or above (63/70; 90%). However, clinical examination reliably detected neovascularization, and in no case did the addition of UWF imaging change management relative to examination alone.

Conclusion: Ultra-widefield imaging detects a higher stage of sickle cell retinopathy compared with clinical examination alone, but these differences may not be clinically significant.
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http://dx.doi.org/10.1097/IAE.0000000000002057DOI Listing
May 2019

Evaluation of Medical Students' Perception of an Ophthalmology Career.

Ophthalmology 2018 03 28;125(3):461-462. Epub 2017 Nov 28.

Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland. Electronic address:

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http://dx.doi.org/10.1016/j.ophtha.2017.10.032DOI Listing
March 2018

Use of Optical Coherence Tomography by Nonexpert Personnel as a Screening Approach for Glaucoma.

J Glaucoma 2018 01;27(1):64-70

Retina Division, Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD.

Purpose: This pilot study was conducted to assess optical coherence tomography (OCT) as a screening tool for glaucoma when used by nonexpert personnel.

Methods: This prospective case-control study included 54 patients with open-angle glaucoma and 54 age-matched comparison individuals. Optovue iVue SD-OCT imaging was performed by nonprofessional photographers on undilated patients. The sensitivity, specificity, negative predictive value, and positive predictive value of iVue scan parameters for detecting open-angle glaucoma were evaluated.

Results: The iVue cup to disc vertical ratio had a sensitivity of 0.96 [95% confidence interval (CI), 0.90-1.00] at 90% specificity and was strongly correlated with both the Cirrus HD-OCT cup to disc vertical ratio (Pearson coefficient=0.84) and the cup to disc ratio observed on dilated clinical examination by faculty ophthalmologists (Pearson coefficient=0.80). The retinal nerve fiber layer (RNFL) parameters performed robustly, but the ganglion cell complex parameters showed limited diagnostic value. The inferior quadrant thickness was among the best performing RNFL parameters, with a sensitivity of 0.87 (95% CI, 0.78-0.96) and a specificity of 0.88 (95% CI, 0.80-0.97) using the iVue normative database thresholds for abnormality.

Conclusions: OCT imaging may be performed by nonprofessional photographers on undilated patients, and quantitative parameters derived from the resultant images, particularly the vertical cup to disc ratio and the RNFL inferior quadrant thickness, demonstrate sensitivities and specificities that may be adequately robust for glaucoma screening in the community setting.
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http://dx.doi.org/10.1097/IJG.0000000000000822DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5741480PMC
January 2018

Reply.

Am J Ophthalmol 2017 11 20;183:163-164. Epub 2017 Sep 20.

Baltimore, Maryland.

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http://dx.doi.org/10.1016/j.ajo.2017.09.006DOI Listing
November 2017

Increased Frequency of Topical Steroids Provides Benefit in Patients With Recalcitrant Postsurgical Macular Edema.

Am J Ophthalmol 2017 Jun 7;178:163-175. Epub 2017 Apr 7.

The Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland.

Purpose: To compare standard and frequent topical steroids for postsurgical macular edema (ME).

Design: Randomized clinical trial.

Methods: Subjects with postsurgical ME stratified into post-cataract surgery ME (PCSME) and post-other surgery ME (POSME) were randomized to ketorolac 4 times a day (qid) + 1% prednisolone acetate (PA) every hour while awake (q1hWA, Group 1) or qid (Group 2). Mean change from baseline best-corrected visual acuity (BCVA) was determined at week 12, after which group 2 subjects with persistent edema were crossed over to PA q1hWA.

Results: Twenty-two subjects (13 PCSME and 9 POSME) were randomized to Group 1 and 20 (12 PCSME and 8 POSME) to Group 2. At week 12, change from baseline BCVA (ETDRS letters) in Group 1 vs 2 was +11.6 vs +8.5 (P = .32) and for subgroups was +10.6 vs +7.8 (P = .23) for PCSME and +13.1 vs +9.4 (P = .47) for POSME. Mean change from baseline central subfield thickness (CST, μm) at week 12 in Group 1 vs 2 was -100.8 vs -63.9 (P = .30). Mean change from baseline intraocular pressure was +2.6 vs +1.7 mm Hg (P = .52). Eight subjects in Group 2 with residual ME at week 12 were switched to PA q1hWA and at week 24, the mean changes from week 12 BCVA and CST were +7.0 letters (P = .01) and -108.25 μm (P = .04).

Conclusions: Our data suggest that patients with postsurgical ME should initially be treated with ketorolac and PA qid, but if edema does not resolve after 12 weeks, a switch to ketorolac qid and PA q1hWA may provide benefit.
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http://dx.doi.org/10.1016/j.ajo.2017.03.033DOI Listing
June 2017

Visual function quality of life measure changes upon conversion to neovascular age-related macular degeneration in second eyes.

Qual Life Res 2017 08 29;26(8):2139-2151. Epub 2017 Mar 29.

Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD, USA.

Purpose: To determine changes in quality of life measures when choroidal neovascularization (CNV) developed in the second eye of patients with initially unilateral neovascular age-related macular degeneration (AMD).

Methods: We analyzed responses to the 39-item National Eye Institute Visual Function Questionnaire (NEI-VFQ), 36-item Short Form Health Survey (SF-36), and Hospital Anxiety and Depression Scale (HADS) at baseline, and prior to and following second eye CNV diagnosis in 92 participants enrolled in two Submacular Surgery Trials. Paired t-tests for sample sizes over 30 and Wilcoxon signed-rank tests for sample sizes <30 were performed to compare scores.

Results: CNV development resulted in statistically and clinically significant changes in responses to 20 of 39 NEI-VFQ items, indicating visual function decline during a mean interval of 25 months. Little difference was noted between baseline scores and prior to CNV diagnosis, which averaged 8.9 months duration. Subscales demonstrated a statistically significant decline in general vision, near activities, distance activities, social functioning, role difficulties, dependency, and driving. There were minimal changes in the HADS and SF-36 scales.

Conclusion: CNV development in the second eye had a dramatic effect on visual functioning based on patient responses to the NEI-VFQ questionnaire. Our investigation is believed to be the first study using data collected prospectively to demonstrate vision-related quality of life changes that resulted from development of CNV in AMD patients.
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http://dx.doi.org/10.1007/s11136-017-1547-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5511091PMC
August 2017
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