TOWARD AN IMAGING-CENTRIC DEFINITION OF NONPARANEOPLASTIC AUTOIMMUNE RETINOPATHY.

PURPOSE: To explore characteristic imaging features of nonparaneoplastic autoimmune retinopathy (npAIR) to augment diagnostic criteria. METHODS: This is a retrospective cohort study of patients with npAIR evaluated at the Emory Eye Center between 2013 and 2019. Multimodal fundus images were evaluated to characterize the evolution of the disease. RESULTS: Twenty-one eyes of 12 patients were classified as having npAIR. Five patients (42%) were female, with median (range) age of 59 years (45-85 years). Median baseline visual acuity was 20/30 (20/20 to hand motions). Disease was asymmetric in 11 patients (92%). Common imaging findings included absence of bone spicules (86% of affected eyes), presence of attenuated vessels (86%), and speckled hypoautofluorescence in perimacular and perivenular regions. Three eyes were noted to present early with subtle splotchy fundus autofluorescence abnormality, ultimately developing characteristic speckled perimacular hypoautofluorescence. On optical coherence tomography, 18 eyes (86%) had loss of outer retinal bands with relative foveal sparing and a tapered transition zone. CONCLUSION: Many eyes with npAIR exhibit a subacute, asymmetric, generalized photoreceptor degeneration featuring outer retinal atrophy with relative foveal sparing, retinal vascular attenuation, absence of bone spicules, and speckled hypoautofluorescence often in a perimacular and perivenular distribution. Findings of this study augment diagnostic criteria to improve specificity and accessibility of testing for npAIR.

Anxiety and depression in patients with uveitis: a comprehensive review of observational studies.

PURPOSE OF THE REVIEW: The purpose of this review is to provide a comprehensive summary of observational studies evaluating anxiety and depression in patients with uveitis. RECENT FINDINGS: A higher prevalence of depression was reported in patients with uveitis compared to healthy controls in most observational studies. Symptoms of anxiety were often, but not always, significantly worse in patients with uveitis compared to controls. Most studies found that patients with uveitis had reduced vision-related quality of life that was associated with anxiety and depression symptoms. SUMMARY: Patients with uveitis have a higher risk of experiencing symptoms of depression and could benefit from screening and treatment. Preliminary screening for vision-related quality of life could also help determine if the patient requires assistance in mental health or other aspects of daily living.

Refractive Outcomes of Combined Cataract Surgery and Vitrectomy Compared to Cataract Surgery Alone.

PURPOSE: The aim of the study was to evaluate the refractive outcomes of combined cataract surgery and vitrectomy compared to cataract surgery alone. METHODS: This retrospective chart review study included two groups: (1) combined surgery in 103 eyes (101 patients) who underwent cataract surgery with posterior chamber intraocular lens (PCIOL) placement by a single cataract surgeon and vitrectomy by a single vitreoretinal surgeon at the same surgical setting; (2) cataract surgery alone by the same surgeon in 107 eyes (84 patients). Refractive outcomes and complications between the combined and cataract surgery alone group were compared. The predicted refractive error was compared to postoperative refractive outcomes in both groups, surgically induced astigmatism (SIA), intraoperative or postoperative complications of either cataract surgery or vitrectomy, and cystoid macular edema. RESULTS: There was no statistically significant difference between predicted and actual postoperative refractive outcomes between the combined and cataract surgery alone groups (within ±0.5 diopters [D], p = 0.099; within ±1.0 D, p = 0.721). There was no difference in SIA refractive outcomes between the two groups (p = 0.509). The use of intraoperative gas for retina tamponade did not significantly affect postoperative refractive outcomes. Both cataract surgery and vitrectomy were successfully performed without unexpected complications from either procedure affecting the other. DISCUSSION/CONCLUSION: Combined cataract surgery and vitrectomy allows excellent refractive outcomes equal to cataract surgery alone, allowing each procedure to be performed independently by separate anterior and posterior segment surgeons. Combined procedures can be performed in eyes with a variety of retinal indications and can include fluid-gas exchange with minimal risk of PCIOL malposition or change in targeted refraction.

Approach to Cataract Surgery in an Ebola Virus Disease Survivor with Prior Ocular Viral Persistence.

A 46-year-old patient with previously documented Ebola virus persistence in his ocular fluid, associated with severe panuveitis, developed a visually significant cataract. A multidisciplinary approach was taken to prevent and control infection. Ebola virus persistence was assessed before and during the operation to provide safe, vision-restorative phacoemulsification surgery.

Ebola, COVID-19, and emerging infectious disease: lessons learned and future preparedness.

PURPOSE OF REVIEW: To highlight the lessons learned from the Ebola outbreak that may inform our approach to the COVID-19 pandemic, particularly related to the widespread disruption of healthcare, ophthalmic disease manifestations, and vision health systems strengthening for future outbreaks. RECENT FINDINGS: Coronavirus disease 2019 (COVID-19), first detected in China in December 2019, has become a worldwide health emergency, with significant disruption of all aspects of society, including travel, business, and medical care. Although this pandemic has had unprecedented effects on healthcare delivery in the United States, experiences from recent Ebola virus disease (EVD) outbreaks in Africa provide insight and inform our approach to COVID-19 and outbreak preparedness. Like COVID-19, the rapid emergence of Ebola required new clinical and surgical approaches to understand its associated spectrum of ophthalmic complications and the potential for Ebola viral persistence within the eye and in tear film. Recent reports of ophthalmic findings associated with COVID-19 include conjunctivitis, retinopathy, and molecular evidence of virus within the tear film in a minority of cases. Yet, more rigorous approaches to understand ophthalmic disease and transmission risk associated with COVID-19 are needed. Gaps also remain in our understanding of eye disease associated with other high priority emerging infectious diseases including Nipah, Lassa fever, Marburg virus, and others. SUMMARY: Thoroughly understanding the ophthalmic findings and transmission risk associated with COVID-19 is paramount during this pandemic, providing additional measures of safety while resuming ophthalmic care for all patients. Vision health systems preparedness measures developed during recent EVD outbreaks and the current pandemic provide models for ophthalmic clinical practice, research, and education, as we continue to address COVID-19 and future emerging infectious disease threats.

Persistence of Ebola Virus in Ocular Fluid during Convalescence.

Among the survivors of Ebola virus disease (EVD), complications that include uveitis can develop during convalescence, although the incidence and pathogenesis of EVD-associated uveitis are unknown. We describe a patient who recovered from EVD and was subsequently found to have severe unilateral uveitis during convalescence. Viable Zaire ebolavirus (EBOV) was detected in aqueous humor 14 weeks after the onset of EVD and 9 weeks after the clearance of viremia.

Ocular manifestations of bartonellosis.

PURPOSE OF REVIEW: To review the systemic and ocular complications of Bartonella spp. infections specifically cat scratch disease, encompassing epidemiology, laboratory diagnostics, ophthalmic imagining, and treatment. RECENT FINDINGS: Recent studies have shown that ocular manifestations occur in approximately 4.4% of cat scratch disease patients. The annual prevalence is lower than previously reported to be approximately 12 500 cases annually. Mainstay treatment continues to be oral antibiotics with and without corticosteroids and is dependent on associated systemic manifestations, age, and patient immune status. More recently anti-VEGF agents have been used for complications such as cystoid macular edema and choroidal neovascularization. SUMMARY: Bartonella spp. infections continue to be a common cause uveitis with ophthalmic manifestations ranging from neuroretinits, vascular occlusions, to choroidal granulomas. Review of associated risk factors including contact with feline reservoirs will aid in recognition and diagnosis of this disease entity. Laboratory diagnostics continue to improve to help with the diagnosis of this entity.

Spectral-Domain Optical Coherence Tomography Angiography for the Diagnosis and Evaluation of Polypoidal Choroidal Vasculopathy.

PURPOSE: To compare the diagnostic ability of optical coherence tomography angiography (OCTA) with indocyanine green angiography (ICGA) in polypoidal choroidal vasculopathy (PCV). METHODS: Retrospective review of 47 eyes with PCV imaged with ICGA and OCTA. For each eye, it was determined which imaging modality better delineated the PCV complex. The presence of a branching vascular network (BVN) and polyp(s) were noted. RESULTS: PCV was better visualized with ICGA in 21 eyes (44.7%) and with OCTA in 9 eyes (19.2%). The results were comparable in 17 eyes (36.2%). Of the 44 eyes with BVN on ICGA, 41 eyes (93.2%) also showed BVN on OCTA. Of the 28 eyes with polyp(s) on ICGA, 22 eyes (78.6%) also showed polyp(s) on OCTA. Polyps were high-flow lesions or faint low-flow dilations on OCTA. CONCLUSION: OCTA readily detects BVNs and can detect most polyps, but in many cases ICGA is better able to detect the PCV complex.

Ophthalmic Manifestations and Causes of Vision Impairment in Ebola Virus Disease Survivors in Monrovia, Liberia.

PURPOSE: To describe the ocular findings, visual impairment, and association of structural complications of uveitis with visual impairment in a cohort of survivors of Ebola virus disease (EVD) in Monrovia, Liberia. DESIGN: Retrospective, uncontrolled, cross-sectional study. PARTICIPANTS: Survivors of EVD who were evaluated in an ophthalmology clinic at Eternal Love Winning Africa (ELWA) Hospital in Monrovia, Liberia. METHODS: A cohort of EVD survivors who underwent baseline ophthalmic evaluation at ELWA Hospital were retrospectively reviewed for demographic information, length of Ebola treatment unit (ETU) stay, visual acuity (VA), and ophthalmic examination findings. For patients with uveitis, disease activity (active vs. inactive) and grade of inflammation were recorded according to Standardization of Uveitis Nomenclature criteria. The level of VA impairment was categorized according to World Health Organization classification for VA impairment as follows: normal/mild, VA 20/70 or better; moderate, VA 20/70-20/200; severe, VA 20/200-20/400; blindness, VA <20/400. Visual acuity, length of ETU stay, and structural complications were compared between EVD survivors with and without uveitis. Structural complications associated with moderate VA impairment or poorer were analyzed. MAIN OUTCOME MEASURES: Frequency of ocular complications including uveitis and optic neuropathy in EVD survivors, level of VA impairment in EVD survivors with uveitis, and structural complications associated with VA impairment in EVD survivors. RESULTS: A total of 96 survivors of EVD were examined. A total of 21 patients developed an EVD-associated uveitis, and 3 patients developed an EVD-associated optic neuropathy. Visual acuity was blind (VA >20/400) in 38.5% of eyes with uveitis. Anatomic subtypes of uveitis included anterior, posterior, and panuveitis in 2, 13, and 6 patients, respectively. Examination findings associated with at least moderate visual impairment by World Health Organization criteria (VA <20/70) included keratic precipitates (P < 0.002), posterior synechiae (P < 0.002), vitritis (P < 0.005), and chorioretinal scars (P < 0.02). CONCLUSIONS: Survivors of EVD are at risk for uveitis, which may lead to secondary structural complications, visual impairment, and blindness. Eye care resources should be mobilized for EVD survivors in West Africa because of the frequency of this spectrum of disease complication and its potential for severe VA impairment and blindness.

An update on ocular complications of Ebola virus disease.

PURPOSE OF REVIEW: This review provides a summary of our current understanding of the ophthalmic manifestations of Ebola virus disease (EVD), pathogenesis, treatment options and directions for future study. The individual, public health and global health implications of eye disease in EVD survivors are discussed. RECENT FINDINGS: The West Africa EVD outbreak was of unprecedented magnitude, leading to the largest survivor cohort since the first documented EVD outbreak in 1976. Because of the magnitude of the recent outbreak, thousands of survivors are at-risk of systemic and ophthalmic sequelae termed the 'post Ebola virus disease syndrome'. Uveitis is the most common finding during EVD convalescence and may lead to severe vision impairment or blindness in 40% of affected individuals. Ocular complications leading to vision loss include cataract, retinal scarring, optic neuropathy, hypotony and phthisis bulbi. The pathogenesis of eye disease in EVD survivors likely involves Ebola virus persistence, severe inflammation and tissue edema, which present as acute, rapidly progressive disease or chronic, smoldering disease. Further studies into disease pathogenesis including mechanisms of viral persistence may provide guidance into therapies for uveitis secondary to EVD. SUMMARY: Uveitis is the most common ophthalmic finding in EVD survivors and can lead to vision loss. Further studies into the clinical manifestations and mechanisms of disease are needed to improve therapies for EVD survivors who often have limited access to ophthalmic medical and surgical care.

Ebola virus disease and the eye.

PURPOSE OF REVIEW: The world has witnessed the largest Ebola virus disease (EVD) epidemic in West Africa with an unprecedented number of infected individuals and survivors. Many systemic and ocular complications have been reported in survivors. Herein, we review the ophthalmic complications associated with Ebolavirus. RECENT FINDINGS: EVD causes ocular disease during acute infection and during convalescence. Acute patients manifest with conjunctivitis, subconjunctival hemorrhages, and acute vision loss of unclear cause. The most common complication during recovery is uveitis with live virus implicated in the pathogenesis in one case. SUMMARY: This epidemic has brought to surface the spectrum of medical complications in EVD survivors with ophthalmic disease being one of the most critical because of its impact on individual functional status and its long-term economic implications. Future studies and programs are needed to appropriately evaluate the spectrum of ophthalmic disease in this at-risk population.

Prospective clinical trial of Intravitreal aflibercept treatment for PolypoIdal choroidal vasculopathy with hemorrhage or exudation (EPIC study): 6 month results.

BACKGROUND: Polypoidal choroidal vasculopathy is a variant of choroidal neovascularization and neovascular age related macular degeneration presenting with hemorrhagic and exudative changes within the macula and/or peripapillary region leading to vision loss. In contrast to neovascular age related macular degeneration, polypoidal choroidal vasculopathy has differing clinical manifestations and treatment strategies. Historically, polypoidal choroidal vasculopathy complexes are less responsive to anti-vascular endothelial growth factor therapy with no prospective clinical trials evaluating aflibercept in management of polypoidal choroidal vasculopathy. Herein we prospectively evaluate the efficacy and safety of intravitreal aflibercept in polypoidal choroidal vasculopathy. METHODS: A prospective, open-label, investigator-sponsored trial of intravitreal aflibercept for polypoidal choroidal vasculopathy in 21 eyes was conducted. Injections were administered monthly for 3 initial treatments, then every other month with monthly evaluations. The primary outcome measures were the mean change in best corrected visual acuity and adverse events. Secondary outcome measures included stabilization of vision, presence of subretinal hemorrhage, serous detachment, retinal pigment epithelial detachment, and regression of polypoidal complexes on indocyanine green angiography. RESULTS: At 6 months, the median visual acuity was 20/40 (range 20/25-20/200) with a mean Early Treatment Diabetic Retinopathy Study vision of 68.4 letters. There was a gain of 2.76 Early Treatment Diabetic Retinopathy Study letters at 6 months (p = 0.15). No patient developed severe vision loss (≤15 letters) and vision was stable or improved in 19/21 eyes (91 %). Subretinal fluid resolved in 13/18 eyes (72 %), and subretinal hemorrhage resolved in 6/8 eyes (75 %) respectively. The polyps regressed in 14/21 eyes (67 %) and the branching vascular network decreased in 1 eye and was stable in all other eyes. The retinal pigment epithelial detachment improved in 13/15 eyes (87 %). Bimonthly treatment occurred in 15/21 patients (71 %). There were no adverse events. CONCLUSIONS: Intravitreal aflibercept results in stabilization of vision, resolution of exudative and hemorrhagic complications with regression of polyps in polypoidal choroidal vasculopathy. Eyes with polypoidal choroidal vasculopathy previously treated with ranibizumab and bevacizumab can show marked improvement in the retinal pigment epithelial detachments and persistent polyps with aflibercept therapy. TRIAL REGISTRATION: Clinical trials.gov NCT01871376 , June 4(th) 2013.

Acute neuroretinitis as a delayed manifestation of tubulointerstitial nephritis and uveitis syndrome.

Purpose: Tubulointerstitial nephritis syndrome with uveitis (TINU) is a rare, acquired syndrome characterized by interstitial nephritis with bilateral uveitis. We report a case of TINU with typical bilateral anterior uveitis complicated by an atypical, delayed-onset neuroretinitis in a 12-year old patient. Observation: A 12-year-old female with a 21-month history of TINU featuring chronic bilateral anterior uveitis presented with one week of blurred vision in her left eye. On exam she was found to have new-onset disc edema in the right eye and neuroretinitis in the left eye. After a negative infectious disease workup, the patient was treated with a course of intravenous (IV) solumedrol with prednisone taper and advancement of her systemic immunosuppression. In follow up she demonstrated resolution of her disc edema and neuroretinitis with improved visual acuity and clinical exam. Conclusion: This case stresses the importance of monitoring for additional ocular manifestations including neuroretinitis years after the onset of anterior uveitis in TINU. In comparison to the two published cases of TINU with neuroretinitis, this case shares features of uveitis progression, and thus highlights the value of further description of TINU-associated neuroretinitis.

Variations in Electronic Health Record-Based Definitions of Diabetic Retinopathy Cohorts: A Literature Review and Quantitative Analysis.

Purpose: Use of the electronic health record (EHR) has motivated the need for data standardization. A gap in knowledge exists regarding variations in existing terminologies for defining diabetic retinopathy (DR) cohorts. This study aimed to review the literature and analyze variations regarding codified definitions of DR. Design: Literature review and quantitative analysis. Subjects: Published manuscripts. Methods: Four graders reviewed PubMed and Google Scholar for peer-reviewed studies. Studies were included if they used codified definitions of DR (e.g., billing codes). Data elements such as author names, publication year, purpose, data set type, and DR definitions were manually extracted. Each study was reviewed by ≥ 2 authors to validate inclusion eligibility. Quantitative analyses of the codified definitions were then performed to characterize the variation between DR cohort definitions. Main Outcome Measures: Number of studies included and numeric counts of billing codes used to define codified cohorts. Results: In total, 43 studies met the inclusion criteria. Half of the included studies used datasets based on structured EHR data (i.e., data registries, institutional EHR review), and half used claims data. All but 1 of the studies used billing codes such as the International Classification of Diseases 9th or 10th edition (ICD-9 or ICD-10), either alone or in addition to another terminology for defining disease. Of the 27 included studies that used ICD-9 and the 20 studies that used ICD-10 codes, the most common codes used pertained to the full spectrum of DR severity. Diabetic retinopathy complications (e.g., vitreous hemorrhage) were also used to define some DR cohorts. Conclusions: Substantial variations exist among codified definitions for DR cohorts within retrospective studies. Variable definitions may limit generalizability and reproducibility of retrospective studies. More work is needed to standardize disease cohorts. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Childhood vision impairment and blindness in West Africa: public health measures and implications for systemic health.

Childhood blindness is an issue of global health impact, affecting approximately 2 million children worldwide. Vision 2020 and the United Nations Sustainable Development Goals previously identified childhood blindness as a key issue in the twentieth century, and while public health measures are underway, the precise etiologies and management require ongoing investigation and care, particularly within resource-limited settings such as sub-Saharan Africa. We systematically reviewed the literature on childhood blindness in West Africa to identify the anatomic classification and etiologies, particularly those causes of childhood blindness with systemic health implications. Treatable causes included cataract, refractive error, and corneal disease. Systemic etiologies identified included measles, rubella, vitamin A deficiency, and Ebola virus disease. While prior public health measures including vitamin A supplementation and vaccination programs have been deployed in most countries with reported data, multiple studies reported preventable or reversible etiologies of blindness and vision impairment. Ongoing research is necessary to standardize reporting for anatomies and/or etiologies of childhood blindness to determine the necessity of further development and implementation of public health measures that would ameliorate childhood blindness and vision impairment.

Outcomes in Patients with Vogt-Koyanagi-Harada Disease from the First-line Antimetabolites for Steroid-sparing Treatment Uveitis Trial.

PURPOSE: To compare the effectiveness of methotrexate (MTX) and mycophenolate mofetil (MMF) in achieving corticosteroid-sparing control of uveitis in patients with Vogt-Koyanagi-Harada (VKH) disease. METHODS: A subanalysis of patients with VKH from the First-line Antimetabolites as Steroid-sparing Treatment (FAST) Uveitis Trial, a randomized, observer-masked, comparative effectiveness trial, with comparisons by treatment (MTX versus MMF) and disease stage (acute versus chronic). Individuals with noninfectious uveitis were placed on a standardized corticosteroid taper and block randomized 1:1 to either 25mg weekly oral MTX or 1.5g twice daily oral MMF. The primary outcome was treatment success defined by corticosteroid-sparing control of uveitis at 6 months. Additional outcomes included change in best spectacle-corrected visual acuity (BSCVA), retinal central subfield thickness (CST), and resolution of serous retinal detachment (SRD). RESULTS: Ninety-three out of 216 enrolled patients had VKH; 49 patients were randomized to MTX and 44 to MMF, of which 85 patients (46 on MTX, 39 on MMF) contributed to the primary outcome. There was no significant difference in treatment success by antimetabolite (80.4% for MTX compared to 64.1% for MMF; P=.12) or in BSCVA improvement (P=.78). Methotrexate was superior to MMF in reducing CST (P=.003) and resolving SRD (P=.02). There was no significant difference in treatment success by disease stage (P=.25), but patients with acute VKH had greater improvement in BSCVA (P<.001) and reduction of CST (P=.02) than chronic VKH patients. CONCLUSIONS: MTX and MMF have comparable outcomes as corticosteroid-sparing immunosuppressive therapies for VKH. Visual acuity improvement was greater in acute vs chronic VKH. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT00182929.

American Society of Ophthalmic Plastic and Reconstructive Surgery fellowship survey: fellows selection criteria for training programs.

PURPOSE: To investigate the characteristics that American Society of Ophthalmic Plastic and Reconstructive Surgery fellows seek in fellowship training programs. METHODS: A 14-question anonymous SurveyMonkey survey was created for the 2011 to 2014 American Society of Ophthalmic Plastic and Reconstructive Surgery graduates. The survey evaluated fellow demographics, the interview process, and qualities fellows seek in fellowship training programs. A Likert scale was used to rate different qualities (1: not important, 9: very important), and summary statistics are reported as overall means and standard deviations. Analysis of variance comparisons were made between the different Ophthalmic Plastic and Reconstructive Surgery fellow graduate years, gender, and future practice goals. RESULTS: In total, 87 surveys were e-mailed with 67 responses, a 77% response rate. The qualities with the highest mean were variety of surgical procedures (mean ± standard deviation: 8.6 ± 0.7), volume of procedures/surgeries (8.6 ± 0.7), personality of the program director (8.2 ± 1.3), and interview (7.7 ± 1.4). The characteristics that ranked the lowest in descending order were presence of a county hospital (5.1 ± 2.2), proximity to family (4.8 ± 2.7), didactics (4.7 ± 1.9), and benefits (4.4 ± 2.0). There was no statistically significant difference when comparing the groups according to gender and year of graduation. Fellows who want to pursue a career in academic medicine ranked academic fellowships higher in importance (8.0 ± 1.0) than those who wanted a career in private practice (5.3 ± 2.2). CONCLUSIONS: American Society of Ophthalmic Plastic and Reconstructive Surgery fellows place an emphasis on surgical experience, the program director's personality, and the interview process when ranking fellowship training programs. This information is valuable for program directors to better recruit fellows.