Morphological characteristics of eyes with neovascular age-related macular degeneration and good long-term visual outcomes after anti-VEGF therapy.

PURPOSE: To analyse the morphological characteristics of eyes with neovascular age-related macular degeneration (AMD) with good long-term visual acuity after anti-VEGF (vascular endothelial growth factor) therapy. METHODS: Retrospective, observational study of 175 patients with neovascular AMD with >5 years of follow-up after initiating anti-VEGF therapy. Spectral-domain optical coherence tomography images were assessed for thickness of pigment epithelial detachment (PED), subretinal hyper-reflective material (SHRM), subretinal fluid and subfoveal choroidal, as well as the integrity of the outer retinal bands. RESULTS: The final analysis cohort included 203 eyes (175 patients) followed for a mean of 7.84±1.70 years (range: 5-11). The maximum PED thickness in the foveal central subfield (FCS) was significantly lower (p<0.001) in the poor vision group (13.11 µm) compared with the intermediate (86.25 µm) or good (97.92 µm) vision groups, respectively. In contrast, the maximum thickness of SHRM in the FCS was significantly thicker (p<0.001) in eyes with poor vision (149.46 µm) compared with eyes with intermediate vision (64.37 µm) which in turn were significantly thicker (p<0.001) than eyes with good vision (9.35 µm). The good vision group also had better continuity of all outer retinal bands (external limiting membrane, ellipsoid zone, and retinal pigment epithelium) compared with the other two groups (all p<0.001). CONCLUSION: A thicker PED and thinner SHRM were correlated with better vision in eyes with neovascular AMD following long-term anti-VEGF therapy. If replicated in future prospective studies, these findings may have implications for design of optimal anatomic endpoints for neovascular AMD treatment.

Ocular extraintestinal manifestations and treatments in patients with inflammatory bowel disease.

Between 3-47% of patients with inflammatory bowel disease (IBD) have extraintestinal manifestations (EIMs), and between 1.3-86.9% of patients with IBD suffer from ocular EIMs (O-EIMs) making the eye the third most common organ affected. These O-EIMs exist among a spectrum, with a variety of types and amounts of inflammation which can lead to decreased vision, and in some cases, vision loss, without treatment. We performed a literature review concerning O-EIMs in patients who had or were later found to have a diagnosis of IBD in order to identify ocular EIMs that commonly occur with IBD and to assess which patients with IBD may be at higher risk of developing O-EIMs. We were also interested in ascertaining whether O-EIMs were more common in specific populations of people or in specific subtypes of IBD. Lastly, we explored the common treatments of O-EIMs in patients with IBD. Upon review of the literature, we found that the most common O-EIMs are episcleritis and uveitis. Anterior uveitis is more commonly seen, although, inflammation may occur in the posterior segment of the eye as well and may also manifest as retinal vasculitis. While these diagnoses are sometimes known retrospectively, most patients present with nonspecific eye complaints of which decreased vision with or without pain is the most common. Visual symptoms associated with ocular EIMs may be non-specific so physicians should have a low threshold to refer to ophthalmology for visual complaints. It is important to keep in mind that ocular EIMs can cluster with skin and joint EIMs. Screening should be prioritized for female patients with Crohn's disease and concurrent arthritis. Treatments for O-EIMs are outlined and compared in this paper as well.

Progress in the diagnosis of ocular sarcoidosis.

Sarcoidosis is a multisystem granulomatous inflammation that affects multiple organ systems. The spectrum of extraocular and ocular involvement is wide and may precede systemic involvement. The diagnosis of ocular sarcoidosis relies on a combination of clinical findings, laboratory investigations, and radiographic findings. These include but are not limited to serum angiotensin-converting enzyme (ACE), lysozyme, plain-film radiographs of the chest, computed tomography (CT) scans of the chest, pulmonary function testing, bronchoalveolar lavage, and retinal imaging among others. In this review, we highlight current and evolving systemic investigations and approaches to ophthalmic imaging when considering the diagnosis of ocular sarcoidosis.

The impact of COVID-19: variations in volumes and characteristics of retina surgeries.

BACKGROUND: In concordance with medical recommendations in response to COVID-19, Emory Healthcare limited surgical procedures starting March 16, 2020. We investigated the impact of these recommendations on the number, types, and urgency of surgical retina cases. METHODS: We conducted a retrospective review of all surgical patients at the Retina division of the Emory Eye Center from February 17-April 12, 2020 and during the same time period in 2019 and 2018. The demographics of patients and the number, types and urgency of retina surgeries were collected. Descriptive statistics for each variable were reported. Univariate analysis was carried out using the chi-square test or Fisher's exact test for categorical covariates. RESULTS: From February 17-March 15 to March 16-April 12, 2020, total surgeries decreased from 87 to 34. Emergent cases, occurring within 7 days of surgical order placement, decreased from 23 to 18 (p = 0.0056), and urgent cases, occurring within 21 days of surgical order placement, decreased from 26 to 4 (p = 0.0380). From March 16-April 12, 2019 there were 62 surgeries: 21 emergent (34%), 14 urgent (23%). From March 16-April 12, 2018 there were 68 surgeries: 15 emergent (22%), 21 urgent (30%). After March 16, 2020, average patient age decreased from 39.4 to 25.7 years. There were no statistically significant differences in racial make-up or insurance coverage for those having surgery prior to versus after March 16, 2020. CONCLUSION: National recommendations for ophthalmologic surgeries during COVID-19 disproportionately affected older patients and patients with urgent cases at our tertiary care academic medical center. These results may inform the ophthalmologic field of the potential effects of pandemics such as COVID-19 on the surgical retina care of patients.

Health Literacy, Medication Adherence, and Quality of Life in Patients With Uveitis.

PURPOSE: To evaluate health literacy, medication adherence, and quality of life (QOL) in uveitis patients. METHODS: Cross-sectional quality improvement study using questionnaires of health literacy (Short Assessment of Health Literacy), adherence (Beliefs about Medicine Questionnaire), and QOL (12-item Short Form Health Survey). RESULTS: Sixty patients were surveyed: 57% women, 80% identified as Black, and 42% with schooling beyond high school. Forty-three percent of the patients had poor health literacy, with lower scores among those with less schooling (p < .01). Necessity scores were higher for multiple providers (p = .02). Necessity (p = .03) and Concerns (p < .01) scores were higher for patients seeing a rheumatologist. Patients had lower physical (p < .01) and mental QOL (p < .01) than the general US population. CONCLUSIONS: Uveitis patients reported many barriers to care. Despite strong perceptions of treatment necessity, there were significant concerns, especially among patients seeing a rheumatologist. We recommend multidisciplinary care, individualized education, and counseling regarding medication safety and the necessity to improve adherence.

Postacute Sequelae of SARS-CoV-2 Infection and Impact on Quality of Life 1-6 Months After Illness and Association With Initial Symptom Severity.

BACKGROUND: Individuals with coronavirus disease 2019 (COVID-19) may have persistent symptoms following their acute illness. The prevalence and predictors of these symptoms, termed postacute sequelae of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2; PASC), have not been fully described. METHODS: Participants discharged from an outpatient telemedicine program for COVID-19 were emailed a survey (1-6 months after discharge) about ongoing symptoms, acute illness severity, and quality of life. Standardized telemedicine notes from acute illness were used for covariates (comorbidities and provider-assessed symptom severity). Bivariate and multivariable analyses were performed to assess predictors of persistent symptoms. RESULTS: Two hundred ninety patients completed the survey, of whom 115 (39.7%) reported persistent symptoms including fatigue (n = 59, 20.3%), dyspnea on exertion (n = 41, 14.1%), and mental fog (n = 39, 13.5%), among others. The proportion of persistent symptoms did not differ based on duration since illness (<90 days: n = 32, 37.2%; vs >90 days: n = 80, 40.4%; P = .61). Predictors of persistent symptoms included provider-assessed moderate-severe illness (adjusted odds ratio [aOR], 3.24; 95% CI, 1.75-6.02), female sex (aOR, 1.99; 95% CI, 0.98-4.04; >90 days out: aOR, 2.24; 95% CI, 1.01-4.95), and middle age (aOR, 2.08; 95% CI, 1.07-4.03). Common symptoms associated with reports of worse physical health included weakness, fatigue, myalgias, and mental fog. CONCLUSIONS: Symptoms following acute COVID-19 are common and may be predicted by factors during the acute phase of illness. Fatigue and neuropsychiatric symptoms figured prominently. Select symptoms seem to be particularly associated with perceptions of physical health following COVID-19 and warrant specific attention on future studies of PASC.

Use of a Telemedicine Risk Assessment Tool to Predict the Risk of Hospitalization of 496 Outpatients With COVID-19: Retrospective Analysis.

BACKGROUND: Risk assessment of patients with acute COVID-19 in a telemedicine context is not well described. In settings of large numbers of patients, a risk assessment tool may guide resource allocation not only for patient care but also for maximum health care and public health benefit. OBJECTIVE: The goal of this study was to determine whether a COVID-19 telemedicine risk assessment tool accurately predicts hospitalizations. METHODS: We conducted a retrospective study of a COVID-19 telemedicine home monitoring program serving health care workers and the community in Atlanta, Georgia, with enrollment from March 24 to May 26, 2020; the final call range was from March 27 to June 19, 2020. All patients were assessed by medical providers using an institutional COVID-19 risk assessment tool designating patients as Tier 1 (low risk for hospitalization), Tier 2 (intermediate risk for hospitalization), or Tier 3 (high risk for hospitalization). Patients were followed with regular telephone calls to an endpoint of improvement or hospitalization. Using survival analysis by Cox regression with days to hospitalization as the metric, we analyzed the performance of the risk tiers and explored individual patient factors associated with risk of hospitalization. RESULTS: Providers using the risk assessment rubric assigned 496 outpatients to tiers: Tier 1, 237 out of 496 (47.8%); Tier 2, 185 out of 496 (37.3%); and Tier 3, 74 out of 496 (14.9%). Subsequent hospitalizations numbered 3 out of 237 (1.3%) for Tier 1, 15 out of 185 (8.1%) for Tier 2, and 17 out of 74 (23%) for Tier 3. From a Cox regression model with age of 60 years or older, gender, and reported obesity as covariates, the adjusted hazard ratios for hospitalization using Tier 1 as reference were 3.74 (95% CI 1.06-13.27; P=.04) for Tier 2 and 10.87 (95% CI 3.09-38.27; P<.001) for Tier 3. CONCLUSIONS: A telemedicine risk assessment tool prospectively applied to an outpatient population with COVID-19 identified populations with low, intermediate, and high risk of hospitalization.

Discharge characteristics and care transitions of hospitalized patients with COVID-19.

Little is known about the follow-up healthcare needs of patients hospitalized with coronavirus disease 2019 (COVID-19) after hospital discharge. Due to the unique circumstances of providing transitional care in a pandemic, post-discharge providers must adapt to specific needs and limitations identified for the care of COVID-19 patients. In this study, we conducted a retrospective chart review of all hospitalized COVID-19 patients discharged from an Emory Healthcare Hospital in Atlanta, GA from March 26 to April 21, 2020 to characterize their post-discharge care plans. A total of 310 patients were included in the study (median age 58, range: 23-99; 51.0% female; 69.0% African American). The most common presenting comorbidities were hypertension (200, 64.5%), obesity (BMI≥30) (138, 44.5%), and diabetes mellitus (112, 36.1%). The median length of hospitalization was 5 days (range: 0-33). Sixty-seven patients (21.6%) were admitted to the intensive care unit and 42 patients (13.5%) received invasive mechanical ventilation. The most common complications recorded at discharge were electrolyte abnormalities (124, 40.0%), acute kidney injury (86, 27.7%) and sepsis (55, 17.7%). The majority of patients were discharged directly home (281, 90.6%). Seventy-five patients (24.2%) required any home service including home health and home oxygen therapy. The most common follow-up need was an appointment with a primary care provider (258, 83.2%). Twenty-four patients (7.7%) had one or more visit to an ED after discharge and 16 patients (5.2%) were readmitted. To our knowledge, this is the first large study to report on post-discharge medical care for COVID-19 patients.

Retinal vasculitis: A framework and proposal for a classification system.

Retinal vasculitis, a poorly understood process involving inflammation or ischemia of the retinal vessel wall, may occur in association with a systemic process, although it can also be isolated to the retina. Because of the limited ability to perform histopathological studies on retinal vessels, there is no gold standard for diagnosis. Thus, there is utility in creating a classification system for retinal vasculitis and improving diagnostic strategies for this disease. We provide a framework for understanding retinal vasculitis based on size, location, and etiology. We hope that this information can be implemented in the clinical setting to provide some diagnostic strategies for this often confusing entity.

Characteristics and outcomes of COVID-19 positive patients with diabetes managed as outpatients.

We analyzed disease outcomes for patients with diabetes and laboratory-confirmed COVID-19 who were managed outpatient and followed by the Emory COVID-19 Virtual Outpatient Management Clinic (ECVOMC). The rate of hospitalization for patients with diabetes was double the overall rate of hospitalization for patients in the ECVOMC.

Phenotypic Spectrum of Pentosan Polysulfate Sodium-Associated Maculopathy: A Multicenter Study.

IMPORTANCE: A unique pigmentary maculopathy was recently described in 6 patients with long-term exposure to pentosan polysulfate sodium (PPS), a long-standing oral therapy for interstitial cystitis. OBJECTIVE: To characterize the exposure characteristics and clinical manifestations of PPS-associated maculopathy. DESIGN, SETTING, AND PARTICIPANTS: In this multi-institutional case series, medical records of patients who exhibited the characteristic maculopathy in the setting of prior PPS exposure were retrospectively reviewed. Data were collected from August 1, 2012, to October 1, 2018, and data were analyzed from October 2018 to January 2019. MAIN OUTCOMES AND MEASURES: Drug exposure, visual acuity, and retinal imaging characteristics. RESULTS: Of the 35 included patients (70 eyes), 34 (97%) were female, and the median (range) age was 60 (37-79) years. The median (range) duration of PPS intake was 15 (3-22) years, and the median (range) cumulative exposure was 1.61 (0.44-4.31) kg. The leading visual symptoms were metamorphopsia, blurred vision, and prolonged dark adaptation. Median (range) logMAR visual acuity of all eyes was 0.10 (-0.12 to 1.18). Fundus examination often revealed hyperpigmented macular spots (34 of 64 eyes [53%]) with interspersed pale-yellow deposits, although less commonly in eyes that exhibited retinal pigment epithelial atrophy (6 of 26 eyes [23%]; P < .001). Optical coherence tomography showed foci of retinal pigment epithelium elevation or thickening associated with hyperreflectance on near-infrared reflectance imaging. Fundus autofluorescence imaging typically revealed a symmetric, confluent pattern of hyperautofluorescent and hypoautofluorescent spots that involved the fovea in all eyes and extended to the retinal periphery in 24 eyes (36%). Longitudinal evaluation demonstrated dynamic changes in pigmentary abnormalities. CONCLUSIONS AND RELEVANCE: These findings suggest that PPS-associated maculopathy is a vision-threatening condition that can manifest in the setting of long-term exposure to the drug. Multimodal imaging posits a distinctive clinical phenotype, characterized in this cohort by dynamic alterations within the retinal pigment epithelium and at the retinal pigment epithelium-photoreceptor interface. Ongoing work might explore causality and direct screening guidelines.

Extranodal Rosai-Dorfman of the Pancreas Presents with Bilateral Granulomatous Anterior Uveitis.

BACKGROUND/AIMS: To report a case of a 65-year-old female who presented with bilateral anterior granulomatous uveitis, which led to the diagnosis of Rosai-Dorfman Disease (RDD) and the discovery of an associated pancreatic mass. METHODS: This is a retrospective case report of a single patient. RESULTS: A 65-year-old African American Female received a full workup for bilateral anterior granulomatous uveitis and was found to have a pancreatic mass on imaging. Biopsy of this mass was diagnostic for RDD, a rare proliferative histiocytic disorder, which classically presents with painless cervical lymphadenopathy, leukocytosis, and hypergammaglobulinemia. CONCLUSION: Although rare, ocular involvement, including eyelid and orbital masses, and anterior uveitis, can herald the development of other systemic manifestations or RDD and can aid in the diagnosis of this systemic disease.

Decreased severity of age-related macular degeneration in amblyopic eyes.

AIM: To evaluate whether people with age-related macular degeneration (AMD) and a history of amblyopia have equal severity of AMD in both eyes. METHODS: Billing records were used to locate all people with a history of amblyopia and AMD evaluated between 1 January 2003 and 1 June 2015 at a single ophthalmology institute. Two ophthalmic graders blinded to amblyopia status determined the severity of AMD in each eye using fundus photos and a validated grading scale. RESULTS: A total of 14 people were found to have AMD and a documented history of amblyopia. Average patient age was 77.0 years and average best corrected visual acuity was 20/160 in eyes with a history of amblyopia and 20/40 in fellow eyes without amblyopia. Eyes with a history of amblyopia were found to have a lower AMD severity score (mean lower score: -1.38; paired t-test P=0.019). Of the 11 people with asymmetric disease severity, 10 individuals had worse AMD in the non-amblyopic eye while one person had worse AMD in the amblyopic eye (P=0.0067). CONCLUSIONS: Our pilot study suggests that eyes with a history of amblyopia may manifest decreased severity of AMD compared with non-ambylopic eyes in the same patient. Further research is warranted to investigate this clinical observation.

Ultra-Widefield Fundus Autofluorescence Imaging of Patients with Retinitis Pigmentosa: A Standardized Grading System in Different Genotypes.

PURPOSE: To report a genotype-phenotype correlation study of patients with retinitis pigmentosa (RP) based on ultra-widefield (UWF) fundus autofluorescence (FAF) imaging. DESIGN: Case series. PARTICIPANTS: Thirty-four patients with RP. METHODS: This retrospective study included RP patients with confirmed causative genetic variants and UWF FAF imaging data. Qualitative grading criteria including the pattern of macular abnormal autofluorescence, decreased autofluorescence (DAF), and its extent and distribution were applied to evaluate the genotype-phenotype correlation. MAIN OUTCOME MEASURES: The main parameters measured were increased or decreased patterns and extent of autofluorescence. RESULTS: Thirty-four unrelated patients 38±19 years of age (range, 9-82 years) were enrolled. Mutations in 17 different genes were detected in patients, including 7 patients having mutations in USH2A, 4 in DHDDS, 4 in RPGR, 3 in PRPF31, and 3 in RP1. Patients with nummular DAF and widespread DAF were significantly older (59±14 years and 56±19 years, respectively). All 3 patients with PRPF31 mutations showed an abnormal macular ring hyperautofluorescence and a circular pattern of coarse DAF distributed in Early Treatment Diabetic Retinopathy Study fields 1, 2, and 3 with sparing of the far periphery. In other genotypes, no specific DAF or macular abnormal autofluorescence pattern could be discerned. CONCLUSIONS: Specific UWF FAF characteristics in RP patients were correlated strongly with patient age and stage of the disease. Particular UWF FAF characteristics were found to be more prominent in a unique genotype.

Vogt-Koyanagi-Harada disease.

Vogt-Koyanagi-Harada disease, a severe bilateral granulomatous intraocular inflammation associated with serous retinal detachments, disk edema, and vitritis, with eventual development of a sunset glow fundus, is an autoimmune inflammatory condition mediated by T cells that target melanocytes in individuals susceptible to the disease. Vogt-Koyanagi-Harada disease presents clinically in 4 different phases: prodromal, uveitic, convalescent, and recurrent, with extraocular manifestations including headache, meningismus, hearing loss, poliosis, and vitiligo, to varying degrees. There have been considerable advances in imaging modalities resulting in earlier diagnosis and improved understanding of this disease. Ocular coherence tomography has replaced other imaging modalities in the diagnosis of acute and chronic Vogt-Koyanagi-Harada disease by revealing exudative detachments of the retina in the acute stage, along with choroidal thickening and demonstrating choroidal thinning in the chronic stage. Treatment of this disease is initially with corticosteroids, with a transition to immunomodulatory drugs for long-term control. Patients with Vogt-Koyanagi-Harada disease can have good final outcomes if treated promptly and aggressively and thus avoid complications such as sunset glow fundus, cataracts, glaucoma, subretinal fibrosis, and choroidal neovascularization.

A pre-retinal arterial loop causing a branch retinal artery occlusion.

Pre-retinal arterial loops are usually incidental findings during examinations in healthy individuals. They are rarely associated with arterial occlusion, with only nine previously reported cases in the literature. The authors report a case of a pre-retinal arterial loop associated with a branch retinal artery occlusion in a myopic young man. They also report the first documented optical coherence tomography scan through the loop causing the branch retinal artery occlusion.