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.

Visual Morbidity and Outcomes of Scleritis Associated with Intraocular Inflammation Compared to Isolated Scleritis.

PURPOSE: To compare visual outcomes, ocular complications and therapies for patients with scleritis-associated intraocular inflammation (SAI) and patients with isolated scleritis (IS). RESULTS: A total of 52 patients (36 with SAI and 16 with IS) were reviewed. Mean age (standard deviation) at presentation was 48.4 years old (± 15.4) in the SAI group and 53 years old (± 17.1) in the IS group (p = .37). Visual acuity was worse at presentation and last visit for patients with SAI compared to IS (p = .04). Patients in the SAI group developed greater posterior segment complications than in the IS group (p = .002). CONCLUSIONS: Scleritis with intraocular inflammation was associated with a higher rate of visual morbidity compared to isolated scleritis. More aggressive management strategies may be needed for patients who present with scleritis associated with inflammation.

Risk Factors for Retinal Detachment in Acute Retinal Necrosis.

PURPOSE: Retinal detachment (RD) is associated with poor visual outcomes in patients with acute retinal necrosis (ARN). This research was undertaken to assess the risk factors for RD in ARN. DESIGN: Retrospective cohort study. SUBJECTS: Patients diagnosed with ARN at a tertiary referral center from 2010 to 2020. METHODS: A chart review was performed for all clinical and surgical encounters. Univariate and multivariate logistic analyses of demographic and clinical variables associated with RD were performed. Survival analyses with Kaplan-Meier estimates were performed to compare the time to RD in herpes simplex virus (HSV)- and varicella zoster virus (VZV)-associated ARN. MAIN OUTCOME MEASURES: Demographic information, clinical information (including visual acuity [VA]), intraocular pressure (IOP), intraocular inflammation level, the extent of retinitis, incidence and timing of retinal detachment, date of diagnosis, and treatments performed (including intravitreal injections of antiviral medications). RESULTS: Fifty-four eyes of 47 patients who were diagnosed with ARN were included, with equal proportions of eyes (n = 27; 50%) with VZV-ARN and HSV-ARN. Patients with VZV-ARN were, on average, older, more likely to be men, and more likely to be immunosuppressed compared with patients with HSV-ARN. The clinical characteristics, including the initial VA, initial IOP, anterior segment inflammation, clock hours, and posterior extent of retinitis, were similar between eyes with VZV- and HSV-ARN. In the univariate analysis of clinical and demographic variables associated with the development of RD, initial VA (P = 0.0083) and greater clock hours of retinitis (P = 0.009) were significantly associated with RD. These 2 variables remained significant in the multivariate logistic regression; worse VA at presentation had an odds ratio of 2.34 (95% confidence interval [CI], 1.01-5.44; P = 0.042), and greater clock hours of retinitis had an odds ratio of 1.23 (95% CI, 1.02-1.47; P = 0.025). A Kaplan-Meier survival analysis demonstrated no statistical difference in RD-free survival between HSV- and VZV-ARN. CONCLUSIONS: Patients with VZV-ARN were more likely to be older, male, and immunosuppressed compared with those with HSV-ARN, although no clear difference was observed in RD by viral etiology. Poor initial VA and clock hours of retinitis were significantly associated with RD development and may be relevant for patient counseling and prognosis.

Outcomes of Infectious Endophthalmitis in Patients with Systemic Antibiotic Allergies to Penicillins, Cephalosporins, or Vancomycin.

PURPOSE: The mainstay empiric treatments of bacterial endophthalmitis are intravitreal vancomycin and ceftazidime. In the United States, up to 10% of the general population has a reported penicillin (PCN) allergy. Despite low cross-reactivity between PCN and later-generation cephalosporins, some providers alter the intravitreal antibiotic choice for endophthalmitis because of concern for allergic reactions. We evaluated the management strategies of infectious endophthalmitis in the setting of self-reported systemic antibiotic allergies and the association with adverse reactions after standard intravitreal antibiotic administration. DESIGN: Single-center, retrospective cohort study. PARTICIPANTS: All patients with endophthalmitis between 2005 and 2019 and documented PCN, PCN-analog, cephalosporin, or vancomycin allergy who received intravitreal antibiotics on the basis of International Classification of Diseases 9th and 10th Revisions, and Current Procedural Terminology codes. METHODS: Retrospective chart review. MAIN OUTCOME MEASURES: Any allergic reaction after intravitreal injection, additional surgical interventions required for treatment, and visual function at last recorded visit. RESULTS: Of the 65 patients included in this cohort, the most common causes of endophthalmitis were postcataract extraction surgery (n = 23, 35.4%) and postintravitreal injection (n = 11, 16.9%). All patients (65/65) received intravitreal vancomycin, and 81.5% (53/65) received intravitreal ceftazidime. Of the 53 patients who received intravitreal ceftazidime, 46 (86.8%) had allergies to PCNs alone, 5 (9.4%) had a cephalosporin allergy alone, and 2 (3.8%) had reported allergies to both PCN and cephalosporin antibiotics. Two patients (3.1%) with a documented vancomycin allergy received intravitreal vancomycin without complication. No patients exhibited any systemic or local allergic reactions or complications after intravitreal injection. CONCLUSIONS: There were no documented allergic reactions in this cohort of patients with systemic antibiotic allergies who were treated for infectious endophthalmitis. Our findings are consistent with previous reports of a low allergic cross-reactivity between PCN and later-generation cephalosporins. Ophthalmologists should use evidence-based practices and a careful informed consent process when choosing intravitreal antibiotics for patients with specific antibiotic allergies. In the routine patient with suspected bacterial endophthalmitis, PCN allergy may not be an absolute contraindication to intravitreal cephalosporin use.

Discovery of tear biomarkers in children with chronic non-infectious anterior uveitis: a pilot study.

BACKGROUND: Biomarkers in easily obtained specimens that accurately predict uveitis in children with juvenile idiopathic arthritis (JIA) are needed. Aqueous humor has been studied for biomarkers, but is not routinely available. We evaluated tears from children with chronic anterior uveitis (CAU) for biomarkers reported in aqueous humor. In this pilot study, we used Schirmer strips to collect tears from seven children (nine eyes); three children had JIA- associated uveitis (JIA-U) and four had idiopathic disease (I-CAU). Liquid chromatography-tandem mass spectrometry was used to identify and quantify tear proteins. The Mann-Whitney U test identified differential tear protein expression between children with JIA-U and those with I-CAU. RESULTS: S100A9, LAP3, TTR, MIF, sCD14, S100A8, and SAA1 were detected in tears of all children; the same cytokines have been reported in aqueous humor of children with JIA-U. Tears from children with JIA-U had higher expression of proteins associated with inflammatory arthritis (SEMA3G, TIMP1, HEXB, ERN1, and SAA1) than tears from those with I-CAU. In addition, we found higher expression of sCD14, S100A8, and SAA1, but lower expression of S100A9, LAP3, TTR, and MIF, in tears from children with JIA-U compared to tears from those with I-CAU. CONCLUSIONS: Tears contain similar cytokine profiles to aqueous humor in children with CAU and may be a clinically useful source of disease biomarkers. Tears from children with JIA-U also contain cytokines associated with inflammatory arthritis; furthermore, differential expression of other tear proteins as well may provide clues to intrinsic differences between JIA-U and I-CAU, despite their similar clinical phenotypes.

Choroidal Neovascularization Associated With Birdshot Chorioretinopathy.

BACKGROUND AND OBJECTIVE: Patients with birdshot chorioretinopathy (BCR) may develop visual compromise due to choroidal neovascularization (CNV), and few series address management strategies in the anti-vascular endothelial growth factor (VEGF) era. The purpose of this study was to describe the clinical outcomes of combination anti-VEGF and immunosuppressive therapy for CNV associated with BCR. PATIENTS AND METHODS: Retrospective, interventional case series. Patients with BCR from two tertiary uveitis and retina practices were reviewed. Patients with CNV in association with BCR were identified and reviewed in detail. Clinical features, treatments utilized (ie, anti-VEGF injections, immunosuppressive therapy), and functional and structural outcomes over long-term follow-up were recorded. Outcomes measured included Snellen visual acuity, spectral-domain optical coherence tomography macular thickness during treatment, number and type of anti-VEGF injections, the need for initiation or escalation of immunosuppression, and incidence of CNV in macula-involved versus macula-sparing BCR. RESULTS: Four of 36 BCR patients were diagnosed with choroidal neovascularization (11%). Identification of CNV in all patients prompted treatment with intravitreal anti-VEGF injections and an increase or initiation of local or systemic immunosuppression. Mean Snellen visual acuity improved from 20/60 to 20/30 at final follow-up (P = .02). Mean central subfield thickness improved from 443 µ to 254 µ (P = .04). CNV in association with BCR occurred at a rate of 0.11 events per patient-year (95% CI, 0.02-0.31) in macula-involved BCR versus zero events/patient-year in macula-spared BCR (95% CI, 0-0.058; P = .009). CONCLUSION: Anti-VEGF therapy was effective for the treatment of CNV in BCR patients. A combination of systemic or local immunosuppression and anti-VEGF therapy may be useful in the management of CNV associated with BCR. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:450-457.].

A novel tele-eye protocol for ocular disease detection and access to eye care services.

BACKGROUND: Telemedicine can improve access to care, especially for rural patients, and ophthalmology is a field that lends itself readily to telemedicine because interpretation of photographs is a routine part of diagnosing eye disease and patient care. We developed a novel tele-eye protocol based on diabetic teleretinal screening. We performed a feasibility study to see if our tele-eye program was comparable to the gold standard face-to-face eye exam. MATERIALS AND METHODS: Fifty-two subjects underwent the tele-eye protocol and then received a face-to-face exam. A masked reader reviewed the tele-eye data remotely and developed an impression and plan for the patient. The provider assessments from the face-to-face exams and the tele-eye exams were compared. Sensitivity, specificity, and percentage agreement were calculated for the tele-eye protocol, focusing on the most common age-related eye diseases: cataract, macular degeneration, and glaucoma. The difference between the autorefraction and manifest eyeglass prescription was calculated. RESULTS: The pilot study showed excellent percentage agreement between the screening protocol and the face-to-face exam. The percentage agreement for cataract was 100%, that for macular degeneration was 96%, and that for glaucoma suspect was 87%. The difference between the autorefraction's eyeglass prescription and the final manifest refraction was within American National Standards Institute for lens manufacturing guidelines. CONCLUSIONS: The initial data suggest that the tele-eye program is feasible to execute and appears fairly accurate when compared with the gold standard face-to-face eye exam. However, the study is significantly limited by the small sample size. This pilot provides justification of a much larger study of a similar design.

Spectrum of pattern dystrophy in pseudoxanthoma elasticum.

OBJECTIVE: To study the prevalence, type, and features of pattern dystrophy in patients with pseudoxanthoma elasticum (PXE). METHODS: A search of the photographic records at the Vanderbilt Eye Institute using the keywords "angioid streaks and pseudoxanthoma elasticum" yielded 28 names. Of the 23 subjects meeting the patient selection criteria, 22 were confirmed to have a positive diagnosis for PXE after reviewing the medical history information. The diagnosis was confirmed by the constellation of fundus findings in all 22 subjects, by a clinical examination of the skin in 9, and by a skin biopsy specimen in 1. RESULTS: Pattern dystrophy was present in 16 patients (27 eyes) of those with PXE. Fourteen patients (23 eyes) had fundus pulverulentus, 3 patients (5 eyes) had butterfly-shaped dystrophy, and 1 patient (2 eyes) each had fundus flavimaculatus and reticular dystrophy. One eye of one patient developed solitary vitelliform pattern dystrophy during follow-up. Two patients showed progression from one pattern into another during follow-up. Another patient, who at first showed no evidence of pattern dystrophy in either eye, developed fundus pulverulentus in both eyes 5 years later. One patient had simultaneous evidence of 2 types: butterfly and fundus flavimaculatus pattern in each eye. Angioid streaks were seen in each eye of all patients. Peau d'orange was noted in 18 patients, optic nerve drusen in 5, and retinal crystalline bodies in 9. Choroidal neovascular membrane was present in 15 patients. CONCLUSIONS: All 5 varieties of pattern dystrophy, 2 of which were not previously associated with PXE, were seen in patients with PXE. Fluorescein angiogram was useful in delineating the type and extent of pattern dystrophy.