ARE THERE TWO FORMS OF MULTIPLE EVANESCENT WHITE DOT SYNDROME?

PURPOSE: To analyze the nature of multiple evanescent white dot syndrome (MEWDS) and differentiate an idiopathic or primary form of MEWDS from a secondary form that is seen in association with other clinical conditions affecting the posterior segment of the eye. METHODS: Clinical and multimodal imaging findings including color fundus photography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography of patients with secondary MEWDS are presented. RESULTS: Twenty consecutive patients with secondary MEWDS were evaluated. Fifteen patients were female. Most were young adults aged between 20 to 40 years with myopia (less than -6 diopters). Pathologic conditions associated with the secondary MEWDS reaction were high myopia (greater than -6 diopters) in two eyes, previous vitreoretinal surgery for rhegmatogenous retinal detachment in 2 eyes, and manifestations of multifocal choroiditis in 18 eyes. In all eyes, the MEWDS lesions followed a course of progression and resolution independent from the underlying condition. CONCLUSION: Secondary MEWDS seems to be an epiphenomenon ("EpiMEWDS") that may be seen in association with clinical manifestations disruptive to the choriocapillaris-Bruch membrane-retinal pigment epithelium complex.

Bilateral Multifocal Choroiditis following COVID-19 Vaccination.

Purpose: To report a case of bilateral choroiditis following COVID-19 vaccination.Study Design: Case report.Results: A 34-year old male presented with visual loss one week after the second dose of COVID-19 vaccine. Examination showed large serous detachment of the macula in the right eye and severe choroidal thickening noted on ultrasonography in both eyes. The patient's condition improved rapidly with oral corticosteroids with significant resolution of the serous detachments within two weeks of initiating treatment and complete visual recovery subsequently.Conclusions: The onset of ocular symptoms starting within one week following vaccination suggests an inflammatory or autoimmune response to the vaccine. Ophthalmologists should consider the option of autoimmune and other inflammatory ocular problems, which may manifest as uveitis, following COVID-19 vaccination. Timely diagnosis and treatment with corticosteroids can result in good visual and structural outcome.

Classification Criteria for Multifocal Choroiditis With Panuveitis.

PURPOSE: To determine classification criteria for multifocal choroiditis with panuveitis (MFCPU). DESIGN: Machine learning of cases with MFCPU and 8 other posterior uveitides. METHODS: Cases of posterior uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the posterior uveitides. The resulting criteria were evaluated on the validation set. RESULTS: One thousand sixty-eight cases of posterior uveitides, including 138 cases of MFCPU, were evaluated by machine learning. Key criteria for MFCPU included (1) multifocal choroiditis with the predominant lesions size >125 µm in diameter; (2) lesions outside the posterior pole (with or without posterior involvement); and either (3) punched-out atrophic chorioretinal scars or (4) more than minimal mild anterior chamber and/or vitreous inflammation. Overall accuracy for posterior uveitides was 93.9% in the training set and 98.0% (95% confidence interval 94.3, 99.3) in the validation set. The misclassification rates for MFCPU were 15% in the training set and 0% in the validation set. CONCLUSIONS: The criteria for MFCPU had a reasonably low misclassification rate and seemed to perform sufficiently well for use in clinical and translational research.

Long-term Outcome of Zonal Outer Retinopathy in Punctate Inner Choroidopathy or Multifocal Choroiditis.

Purpose: To report the long-term prognosis of punctate inner choroidopathy (PIC) or multifocal choroiditis (MFC) and associated zonal outer retinopathy (ZOR).Method: Retrospective study in patients with PIC/MFC and ZOR with a clinical follow-up of 4 years or longer.Results: There were 14 patients in this study (M: F = 11:3). All patients received systemic steroid therapy. The initial and final median logarithm of minimal angle of resolution of BCVA were 1.00 and 0.22 (p = .002). Ellipsoid zone recovery was noted in all patients. The median visual field loss improved from -6.38 dB to -3.41 dB (p = .035). The median of total area of PIC/MFC lesions enlarged from 6.82 mm2 to 8.77 mm2 (p = .005). Recurrent disease was noted in 4 eyes and maintenance steroid was needed in 3 eyes.Conclusion: With steroid therapy, most patients with PIC/MFC and ZOR had good visual prognosis.

Collaborative Ocular Tuberculosis Study Consensus Guidelines on the Management of Tubercular Uveitis-Report 1: Guidelines for Initiating Antitubercular Therapy in Tubercular Choroiditis.

TOPIC: An international, expert-led consensus initiative organized by the Collaborative Ocular Tuberculosis Study (COTS), along with the International Ocular Inflammation Society and the International Uveitis Study Group, systematically developed evidence- and experience-based recommendations for the treatment of tubercular choroiditis. CLINICAL RELEVANCE: The diagnosis and management of tubercular uveitis (TBU) pose a significant challenge. Current guidelines and literature are insufficient to guide physicians regarding the initiation of antitubercular therapy (ATT) in patients with TBU. METHODS: An international expert steering subcommittee of the COTS group identified clinical questions and conducted a systematic review of the published literature on the use of ATT for tubercular choroiditis. Using an interactive online questionnaire, guided by background knowledge from published literature, 81 global experts (including ophthalmologists, pulmonologists, and infectious disease physicians) generated preliminary consensus statements for initiating ATT in tubercular choroiditis, using Oxford levels of medical evidence. In total, 162 statements were identified regarding when to initiate ATT in patients with tubercular serpiginous-like choroiditis, tuberculoma, and tubercular focal or multifocal choroiditis. The COTS group members met in November 2018 to refine these statements by a 2-step modified Delphi process. RESULTS: Seventy consensus statements addressed the initiation of ATT in the 3 subtypes of tubercular choroiditis, and in addition, 10 consensus statements were developed regarding the use of adjunctive therapy in tubercular choroiditis. Experts agreed on initiating ATT in tubercular choroiditis in the presence of positive results for any 1 of the positive immunologic tests along with radiologic features suggestive of tuberculosis. For tubercular serpiginous-like choroiditis and tuberculoma, positive results from even 1 positive immunologic test were considered sufficient to recommend ATT, even if there were no radiologic features suggestive of tuberculosis. DISCUSSION: Consensus guidelines were developed to guide the initiation of ATT in patients with tubercular choroiditis, based on the published literature, expert opinion, and practical experience, to bridge the gap between clinical need and available medical evidence.

The efficacy of corticosteroid-sparing immunomodulatory therapy in treating patients with central multifocal choroiditis.

PURPOSE: To evaluate the efficacy of corticosteroid-sparing immunomodulatory therapy (IMT) in patients with recurrent and/or sight-threatening central multifocal choroiditis (MFC). METHODS: This was a retrospective cohort study in a tertiary uveitis centre including all patients with MFC who have been treated with IMT for at least 12 months. Clinical data and imaging results were collected regarding the period prior to the start of IMT and at 3, 6, 12 and - where available - 24 months after the start of IMT. Main outcome measure was the number of annual recurrences of choroiditis with or without active choroidal neovascularization before and after the start of IMT. Secondary outcomes were the percentage of patients with (steroid-free) remission and the median time between the start of IMT and (steroid-free) remission. RESULTS: Thirty-two patients (39 eyes) were included. At the start of IMT, none of the patients were in (steroid-free) remission. At 24 months, the probability of achieving remission and steroid-free remission was 88,5% and 50%, respectively. The median time to achieve remission and steroid-free remission was 21 and 83 weeks, respectively. In 17 patients (20 eyes) with available clinical data and imaging results for ≥ 12 months prior to the start of IMT, the mean number of recurrences/year decreased significantly from 1.40 ± 0.81 at baseline to 0.49 ± 0.47 (p = 0.001) after the start of IMT. CONCLUSIONS: Preventive therapy with IMT should be considered in patients with recurrent and/or sight-threatening MFC to decrease the number of recurrences/year and to increase the prospects of achieving either remission or steroid-free remission.

Dual lesion margins on fundus autofluorescence associated with paradoxical worsening following treatment for tubercular serpiginous-like choroiditis.

A 31-year-old male presented with decreased vision in the right eye associated with an active plaque-like serpiginoid choroiditis. The lesion showed a unique feature of dual margins of hyperautofluorescence of the lesion on fundus autofluoresence (FAF) imaging. Systemic investigations suggested a tubercular etiology. He was started on antitubercular treatment and a conventional dose of oral corticosteroids (1mg/kg body weight). However, the lesions showed paradoxical worsening and required increased immunosuppression in the form of local steroids and oral immunomodulators. The presence of dual margins of hyperautofluorescence could suggest increased inflammatory activity leading to paradoxical worsening on treatment requiring increased immunosuppression.

Differentiating Multifocal Choroiditis and Punctate Inner Choroidopathy: A Cluster Analysis Approach.

PURPOSE: To develop a robust approach to clinical phenotyping of multifocal choroiditis (MFC) and punctate inner choroidopathy (PIC). DESIGN: Cross-sectional and longitudinal observational study. METHODS: This multicenter study included sites in the United Kingdom and Israel. The study population included 343 eyes of 185 subjects with hospital record diagnoses of MFC or PIC. Eyes were observed over a period of 5 years for clinically relevant characteristics, including demographics and multimodal imaging features, by observers masked to the original diagnoses. Multivariate 2-step cluster analysis was used to identify clusters of eyes in the database with similar clinical phenotypes, which were then analyzed for between-group differences. The primary outcome measure was the difference between clinical phenotype clusters identified using clinical criteria from the multivariate cluster analysis. RESULTS: Subjects ranged from 11 to 89 years of age, with a baseline best-corrected visual acuity of 2.3 to -0.2 logarithm of minimal angle of resolution. Eighty-two percent of eyes were from females, 74% were myopic with a refractive error of +3.00 to -17.00 diopters (spherical equivalent). Cluster analysis prioritized clinical criteria of chorioretinal lesion location and intraocular inflammation and identified 2 distinct phenotype clusters resembling the original descriptions of MFC and PIC. During the 5-year period of observation, the initial clinical diagnosis remained stable for most eyes and only 1 eye (0.3%) changed diagnosis from PIC to MFC because of newly developed peripheral lesions. There were significant between-group differences in clinical characteristics, for example, in choroidal neovascular membrane development and treatment received. CONCLUSIONS: Cluster analysis of this large cohort of eyes identified peripheral lesions and intraocular inflammation as distinct clinical phenotypes of MFC and PIC. The initial diagnosis remained stable for most eyes. This methodology could be useful for future uveitis classification and management.

Long-Term Outcome of Punctate Inner Choroidopathy or Multifocal Choroiditis with Active Choroidal Neovascularization Managed with Intravitreal Bevacizumab.

Purpose: To evaluate the long-term outcome of active choroidal neovascularization (CNV) in punctate inner choroidopathy (PIC) or multifocal choroiditis (MFC) after intravitreal bevacizumab treatment.Methods: Retrospective study of consecutive patients of PIC/MFC complicated with active CNV. Outcome measures included best-corrected visual acuity (BCVA), total number of intravitreal injections of bevacizumab and recurrence of CNV. Correlation analysis was performed to find the correlation of various clinical factors and final BCVA.Results: There were 23 eyes in 22 patients with a mean age of 33.22 years included in this study. The mean duration of follow-up was 6.48 years. Improvement of BCVA was noted through the first 3 years and at the final follow-up. BCVA at 1, 6, 12 months and recurrence of CNV were correlated with final BCVA.Conclusion: Most patients of PIC/MFC complicated with CNV managed with intravitreal bevacizumab had improved BCVA over 4 years.

Masqueraders of multiple evanescent white dot syndrome (MEWDS).

PURPOSE: To describe disorders that can masquerade as multiple evanescent white dot syndrome (MEWDS). DESIGN: Retrospective, multicenter case series. PARTICIPANTS: Patients who presented with clinical findings compatible with a diagnosis of MEWDS but were ultimately diagnosed with an alternative inflammatory, infectious, or neoplastic disorder. METHODS: Clinical records and multimodal imaging findings including fundus photography, fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), optical coherence tomography (OCT), and OCT angiography (OCTA) were analyzed. MAIN OUTCOME MEASURES: Inclusion criteria to be defined as a masquerade syndrome for MEWDS included the presence of disseminated grayish-white outer retinal spots that were hyperautofluorescent on FAF and associated with ellipsoid zone (EZ) disruption on OCT. RESULTS: Twenty-two eyes of 13 patients were identified. All patients presented with the classic findings of MEWDS listed above. A MEWDS-like presentation was bilateral in nine of 13 patients (69%). Final diagnosis was determined on the basis of additional investigations including serologies and biopsy. These diagnoses included syphilis (three patients), lymphoma (three patients), idiopathic multifocal choroiditis (two patients), idiopathic retinal phlebitis (one patient), idiopathic acute zonal occult outer retinopathy (one patient), sarcoidosis (one patient), tuberculosis (one patient), and cancer-associated retinopathy (one patient). The outer retinal lesions and imaging findings resolved with treatment for the associated systemic disorders. CONCLUSIONS: Widespread grayish-white outer retinal spots associated with hyperautofluorescence on FAF and disruption of the EZ on OCT are not pathognomonic for MEWDS. A high index of suspicion must be maintained for masqueraders of MEWDS, which can include serious inflammatory, infectious, and neoplastic disorders.

Ultra-Wide Field Imaging in Paradoxical Worsening of Tubercular Multifocal Serpiginoid Choroiditis after the Initiation of Anti-Tubercular Therapy.

Purpose: To evaluate role of ultra-wide field (UWF) versus conventional imaging in the follow-up and paradoxical worsening (PW) of tubercular (TB) multifocal serpiginoid choroiditis (MSC). Methods: Prospective observational study of patients with TB MSC undergoing UWF imaging, autofluorescence and fluorescein angiography was performed. A circle simulating central 75° field representing conventional imaging was drawn on UWF images. The information yielded by the two modalities, progression of choroiditis lesions and PW was compared. Results: 44 eyes (29 patients, mean age: 30.7 ± 9 years; 23 males) were included. UWF imaging showed additional lesions in 39/44 eyes (88.6%). Overall, 16/44 eyes (36.4%) showed PW; 3/16 eyes (18.7%) showed only peripheral PW, while 10/16 eyes showed both central and peripheral PW. Management was altered in 11 patients (37.93%) based on UWF imaging. Conclusions: UWF is more useful than conventional imaging in identifying additional choroiditis lesions, PW and altering the course of therapy in TB MSC.

Multimodal Imaging of Post-Infectious Unilateral Outer Retinopathy and Choroiditis.

Purpose: To describe with multimodal imaging a case of post-infectious unilateral outer retinopathy with choroiditis. Methods: Retrospective chart review of a case of a 67-year old male who presented following the onset of viral symptoms with an acute onset outer retinopathy, small vessel leakage on fluorescein angiography, and choroidal involvement evident on indocyanine green angiography and near infrared fundus autofluorescence (NIR-AF). Work up for infectious and autoimmune etiologies was negative. Results: Treatment with IV methylprednisolone followed by high dose oral prednisone resulted in improvement in visual acuity, outer retinal reconstitution and resolution of the choroidal changes. Conclusions: Despite this presentation sharing features with both acute zonal occult outer retinopathy (AZOOR) and multifocal choroiditis (MFC), the case is highly atypical of both entities.

Coroidite multifocal / Multifocal choroiditis

Resumo A coroidite multifocal é uma doença inflamatória idiopática pouco comum na prática oftalmológica, que usualmente acomete mulheres jovens. Os autores visam relatar um caso de coroidite multifocal em seguimento ambulatorial em que o paciente foi submetido a injeção subtenoniana de triancinolona associada a corticoterapia via oral com manejo da terapia imunossupressiva. São discutidos os aspectos clínicos, diagnósticos e tratamento. A injeção de triancinolona subtenoniana apresentou bons resultados quando associada à terapia imunossupressiva via oral sobre o edema macular, em consonância com os registros obtidos na literatura médica atual.

Abstract Multifocal choroiditis is an uncommon idiopathic inflammatory ophthalmological disease, which usually affects young women. The authors report a case of multifocal choroiditis in which patient underwent subtenonian triamcinolone injection associated with oral corticosteroid and management of immunosuppressive therapy. The clinical, diagnostic and treatment aspects are discussed. The subtenonian triamcinolone injection presented good results on macular edema when associated with oral imunosuppressive therapy, in agreement with the records obtained in the current medical literature.