Association of Ocular Adverse Events With Inactivated COVID-19 Vaccination in Patients in Abu Dhabi.

Importance: As vaccinations against COVID-19 continue, potential ocular adverse events should be reported in detail to increase awareness among the medical community, although typically, a causal relationship cannot be established definitively. Objective: To describe ocular adverse events that occur soon after receiving an inactivated COVID-19 vaccination (Sinopharm). Design, Setting, and Participants: This case series took place from September 2020 to January 2021 at Cleveland Clinic Abu Dhabi, a tertiary referral center. Patients who reported ocular adverse events and presented within 15 days from the first of 2 doses of an inactivated COVID-19 vaccine were analyzed. Main Outcomes and Measures: Each patient underwent Snellen best-corrected visual acuity that was then converted to logMAR, applanation tonometry, and biomicroscopic examination with indirect ophthalmoscopy. Color fundus photography was obtained with a conventional 9-field fundus photography camera or with a widefield fundus photography system. Optical coherence tomography and optical coherence tomographic angiography images were obtained. Sex, race, age, and clinical data were self-reported. Results: Nine eyes of 7 patients (3 male individuals) presenting with ocular complaints following COVID-19 vaccine were included in the study. The mean (SD) age was 41.4 (9.3) years (range, 30-55 years); the mean best-corrected visual acuity was 0.23 logMAR (range, 0-1 logMAR; approximate Snellen equivalent, 20/32). The mean time of ocular adverse event manifestations was 5.2 days (range, 1-10 days). One patient was diagnosed with episcleritis, 2 with anterior scleritis, 2 with acute macular neuroretinopathy, 1 with paracentral acute middle maculopathy, and 1 with subretinal fluid. Conclusions and Relevance: In this case series study of 7 patients, the timing of transient and ocular complications 5.2 days after vaccination with an inactivated COVID-19 vaccine supported an association with the ocular findings, but a causal relationship cannot be established from this study design.

Acute zonal occult outer retinopathy complex disease: Lessons learned about choroid, photoreceptors, and retinal function.

PURPOSE: Retinal photoreceptors layer integrity is considered essential to visual function. We report a case of acute zonal occult outer retinopathy (AZOOR) complex disease (namely AIBSE: acute idiopathic blind spot enlargement) in which apparently a full anatomic regeneration is not needed for a complete functional recovery. METHODS: Case report with multimodal imaging. REPORTS: Visual field recovery in the presence of photoreceptors layer disruption studied by means of Optical Coherence Tomography. Choroid and photoreceptors layer thickness thinned progressively during recovery. CONCLUSION: This case suggests that anatomical retinal integrity as shown by OCT does not always correspond to visual function. Our case highlights that a complete visual recovery can occur even when structural abnormalities are still observable.

Ellipsoid layer restoration after Ozurdex® treatment in a patient with acute posterior multifocal placoid pigment epitheliopathy.

An atypical case of acute posterior multifocal placoid pigment epitheliopathy with a clear reappearance of the ellipsoid layer of the retina after Ozurdex® intravitreal implantation is presented. A 51-year-old woman reported a 3-week history of left eye photopsia. On slit-lamp examination, yellowish placoid lesions were found on her left eye fundus. Ancillary tests were performed. The patient was diagnosed as a left eye acute posterior multifocal placoid pigment epitheliopathy, and observational approach was decided. Later, the condition started to progress in an ampiginous manner and a decrease of visual acuity caused by an increase in number and size of the lesions was observed. As the disease was progressing with the conservative, observational approach, and the macula was menaced, an intravitreal dexamethasone implant was injected in the left eye with a consequent improvement of the visual acuity and lesion stabilization. The ellipsoid layer, unidentifiable inside the placoid lesions in previous optical coherence tomography tests, reappeared after the treatment. Intravitreal dexamethasone implants can be used to stabilize acute posterior multifocal placoid pigment epitheliopathy lesions and help resolve the condition. Spectral domain optical coherence tomography can also be useful for monitoring these lesions, as the ellipsoid layer may reappear upon resolution.

Longitudinal Retinal Layer Changes and Clinical Outcome in Patients with Multiple Evanescent White Dot Syndrome.

Purpose: To evaluate longitudinal changes in retinal layer thickness and clinical outcome in patients with MEWDS.Methods: In 20 patients with MEWDS, SD-OCT images and BCVA were assessed at baseline, and at months 1, 3, and 12. SD-OCTs were segmented and measurements were performed within the fovea and a MEWDS lesion. Baseline and follow-up values in the affected eye were compared to measurements performed at the corresponding location in the fellow eye.Results: ONL thickness was 4.7% thicker in MEWDS-eyes compared with the baseline, with a significant decrease of 9% at 3 months. Within the lesion, INL thickness was 7.9% increased at baseline and decreased significantly over the follow-up of 12 months. BCVA was decreased at baseline (0.2 ± 0.18logMAR) and at the 3 months but after 12 months had increased to 0.01 ± 0.04 logMAR.Conclusion: MEWDS shows the involvement of different retinal layers and characteristic changes over the disease course.

Optical Coherence Tomography Angiography Changes in Choroidal Vasculature following Treatment in Punctate Inner Choroidopathy.

Purpose: To describe the changes seen on optical coherence tomography angiography [OCTA] in patients with PIC following immunosuppressive therapy.Methods: We reviewed serial OCTA scans from five consecutive PIC patients (5 eyes) with at least 3 months of follow-up, who underwent imaging before and after immunosuppressive therapy. Using ImageJ, superficial and deep retinal vasculature were analyzed for vessel area and foveal avascular zone. Choriocapillaris layer was analyzed for flow signal loss.Results: Five out of five patients received an orbital floor triamcinolone acetonide injection as the initial treatment for periods of activity. Mean choriocapillaris (CC) flow void area obtained after immunosuppressive therapy was significantly lower than the mean CC flow void area obtained prior to treatment (Pre-treatment: 0.270 vs Post-treatment: 0.144; p = .0068). In 2 out of 2 patients with longitudinal visual field testing, CC flow voids were spatially associated with visual field defects, and immunosuppressive therapy was associated with reduced CC flow void area and improved visual function.Conclusion: OCTA can detect alterations in choriocapillaris flow. Longitudinal follow-up demonstrates a centripetal restoration of choriocapillaris flow in response to immunosuppressive therapy. OCTA may be a useful adjunct for monitoring and evaluating treatment of PIC.

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.

Multimodal Imaging Characteristics and Functional Test Findings in a Case of Acute Macular Neuroretinopathy Accompanied by Behçet Disease.

Purpose: To report a case of acute macular neuroretinopathy (AMN) in Behçet Disease.Case: A 23-year-old male presented with a complaint for central scotoma in his right eye. He had been diagnosed with Behçet Disease 3 years ago. Best-corrected visual acuity (BCVA) was 20/20. Anterior chamber and fundus examinations were unremarkable. Optical coherence tomography revealed a paracentral area of outer nuclear layer thinning. Infrared reflectance showed a well-defined, circular, hyporeflective area. Optical coherence tomography angiography revealed an area of capillary dropout in deep retinal capillary plexus corresponding to that hyporeflective lesion. Microperimetry test showed decreased macular sensitivity on the lesion area and the loss of the macular integrity. In multifocal electroretinogram, diminished amplitudes of the central cone responses were detected nasal to fixation.Conclusion: Behçet disease is a cause of occlusive retinal vasculitis. Accompanied retinal microvascular disease may be a possible risk factor of AMN suggesting ischemic etiopathogenesis for AMN.

A Multiple Evanescent White Dot Syndrome-like Reaction to Concurrent Retinal Insults.

PURPOSE: To describe a clinical picture resembling classic multiple evanescent white dot syndrome (MEWDS) potentially triggered by previous or concurrent, apparently unrelated, ocular events and to provide a literature review of similar presentations. DESIGN: Retrospective chart series and literature review. PARTICIPANTS: Consecutive patients diagnosed with MEWDS at the Feinberg School of Medicine, Northwestern University, Chicago, Illinois, and the IRCCS San Raffaele Scientific Institute, Milan, Italy, between July 2019 and June 2020. METHODS: Charts of patients were reviewed. Ophthalmic history, best-corrected visual acuity, spectral-domain OCT results, OCT angiography results, fundus autofluorescence results, ultra-widefield fluorescein angiography results, and indocyanine green angiography results were collected. A PubMed-based search was carried out for similar presentations using the terms MEWDS and white spot syndromes. MAIN OUTCOME MEASURES: An ocular history positive for previous or concurrent ocular events in patients with MEWDS was sought in our cohort and the existing literature. RESULTS: Five eyes of 4 patients (2 females; age range, 16-81 years) were included. The first eye had a history of bilateral Best vitelliform dystrophy and unilateral choroidal neovascularization. The second eye had angioid streaks complicated by choroidal neovascularization and underwent prior thermal laser photocoagulation. The third eye had a history of high myopia and a scleral buckle procedure for retinal detachment. The fourth patient had bilateral idiopathic retinochoroiditis. We identified 16 case reports from 5 previous publications that support a MEWDS-like reaction to previous ocular insults. CONCLUSIONS: We suggest a MEWDS-like reaction may be elicited by ocular events in a subset of susceptible patients. We hypothesize that damage to the outer retina may play a role in triggering the local inflammatory response.

Oguchi disease caused by a homozygous novel SAG splicing alteration associated with the multiple evanescent white dot syndrome: A 15-month follow-up.

PURPOSE: We report a 15-month follow-up case on a Chinese patient with Oguchi disease associated with the multiple evanescent white dot syndrome (MEWDS). METHODS: The patient's clinical presentation and follow-up visits were documented via decimal best-corrected visual acuity, fundus photography, fundus autofluorescence (FAF) imaging, near-infrared FAF, spectral domain optical coherence tomography, Humphrey's visual fields, microperimetry, and multifocal electroretinography. We also performed whole exome sequencing for screening variation in the patient and her relatives. RESULTS: The patient had typical clinical characteristic of Oguchi disease, including night blindness, the Mizuo-Nakamura phenomenon (a golden yellow discoloration of the fundus that disappears in the prolonged dark adaptation [DA]) and typical full-field electroretinogram changes (nearly undetected b-wave in 0.01 and 0.03 ERGs that can partially recover only after prolonged DA). Aside from Oguchi disease, the patient was also diagnosed with the MEWDS based on clinical detections, including suddenly reduced visual acuity, appeared white dots, blurred ellipsoid zone and disrupted interdigitation zone, enlarged blind spot, and reduced macular sensitivity. A series of investigations revealed that along with the 15-month follow-up after onset, the visual acuity enhanced, the numerous white dots disappeared, and the macular structure returned to normal. Moreover, the novel homozygous splicing alteration c.181 + 1G > A was identified in the SAG gene. CONCLUSIONS: This work is the first long-term case study of a patient with Oguchi disease associated with the MEWDS. The recovery period of symptoms caused by the MEWDS was much longer than that in typical patients with MEWDS. Molecular genetics demonstrate that this is the first case of Oguchi disease caused by splicing alterations in the SAG gene.

Changes in intraocular pressure and aqueous flare in eyes with multiple evanescent white dot syndrome.

PURPOSE: To study the changes in intraocular pressure (IOP) and aqueous flare in eyes with multiple evanescent white dot syndrome (MEWDS) during the disease course. STUDY DESIGN: Retrospective observational study. METHODS: Twenty-one patients with unilateral MEWDS were retrospectively evaluated. IOP values were compared between the affected and fellow eyes 2 weeks, 1 month, and 3 months following disease onset in 17 patients, and within 7 days from disease onset in 11 patients. Aqueous flare values measured using a laser flare-cell meter in ten eyes between 1 weeks and 1 month from disease onset were compared between the affected and fellow eyes. RESULTS: IOP values were significantly lower in the affected eyes than in the fellow eyes at both 2 weeks (P=0.002) and 1 month from disease onset (P=0.02). However, IOP values of affected eyes did not show significant differences from the fellow eyes within 7 days ((P=0.11) and 3 months of onset (P=0.30). Aqueous flare values were significantly increased in the affected eyes compared to those in the fellow eyes (P=0.010) and significantly correlated with IOP values (r=-0.67, P=0.035). CONCLUSION: IOP values mildly decreased in association with aqueous flare values in the acute phase in eyes with MEWDS.

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.

RETINAL MICROVASCULATURE ALTERATION IN PARACENTRAL ACUTE MIDDLE MACULOPATHY AND ACUTE MACULAR NEURORETINOPATHY: A QUANTITATIVE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY STUDY.

PURPOSE: To present a series of idiopathic paracentral acute middle maculopathy (PAMM) and acute macular neuroretinopathy (AMN) cases and to compare the microvascular retinal status of PAMM/AMN eyes with those of healthy individuals. METHODS: Six eyes with PAMM and two eyes with AMN in a total of six patients were included in this study. Multimodal retinal imaging, including optical coherence tomography angiography, was performed in all patients. The vessel density of the superficial capillary plexus and deep capillary plexus, as well as the area, circularity, and angle of the superficial foveal avascular zone (FAZ) were measured and compared with the same parameters in age-matched healthy individuals. RESULTS: The optical coherence tomography angiography showed a decreased vessel density of both superficial capillary plexus and deep capillary plexus in PAMM/AMN eyes versus control eyes (12.2 ± 0.74 vs. 14.0 ± 1.62 mm [P = 0.01] in superficial capillary plexus and 18.5 ± 1.2 vs. 20.2 ± 1.0 mm [P = 0.02] in deep capillary plexus). There was no statistically significant change in the FAZ area between PAMM/AMN and healthy eyes. However, FAZ circularity in PAMM/AMN eyes was statistically significantly lower compared with healthy eyes (0.64 ± 0.09 and 0.77 ± 0.07, respectively; P = 0.01). The angle of FAZ was statistically significantly higher in PAMM/AMN eyes compared with healthy eyes (27.6 ± 16.2 and 11.2 ± 5.5°, respectively; P = 0.005). CONCLUSION: Paracentral acute middle maculopathy and AMN eyes demonstrated not only an isolated alteration of deep retinal circulation but also general nonspecific changes in retinal microvasculature, including a decrease in the vessel density of the superficial capillary plexus and distortion of the FAZ.

Optical coherence tomography angiography findings in a case with acute posterior multifocal placoid pigment epitheliopathy.

Twenty-six-year-old male patient admitted to the outpatient clinic with a complaint of decreased vision in both eyes. Visual acuity was 2/10 in the right and 7/10 in the left eye. Fundus examination revealed bilateral yellow-white subretinal lesions involving the macula. Fluorescein angiography showed hypofluorescent lesions in early and hyperflourescent lesions in the late phase. Optical coherence tomography angiography performed in the acute phase showed bilateral hypofluorescency at the choriocapillary level. A diagnosis of acute posterior multifocal placoid pigment epitheliopathy was given. After steroid treatment, visual acuity was improved to 10/10 and earlier lesions in optical coherence tomography angiography were found to be reduced. In conclusion, optical coherence tomography angiography is a noninvasive imaging technique that can be used in the diagnosis and follow-up of acute posterior multifocal placoid pigment epitheliopathy.

Textural Properties of Choriocapillaris on OCTA in Healed Inflammatory Choriocapillaropathies.

BACKGROUND AND OBJECTIVE: To evaluate the flow characteristics and textural properties of choriocapillaris (CC) on optical coherence tomography angiography in eyes with resolved inflammatory choriocapillaropathies and Vogt-Koyanagi-Harada (VKH) disease. PATIENTS AND METHODS: A cohort of eyes with healed acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis (SC), and VKH disease were included. A 3 mm × 3 mm OCT angiogram of CC was acquired and graded for flow characteristics and textural properties. RESULTS: This study included 16 patients. Texture was heterogeneous in all eyes in the SC and VKH groups, and in four eyes (40%) in the APMPPE group. Most of the eyes with VKH disease had severe low flow, whereas most of the SC and APMPPE eyes demonstrated mild low flow. Heal duration had a strong negative correlation with severity of CC low flow and a weak, statistically nonsignificant correlation with texture heterogeneity. CONCLUSION: Despite the resolution of active inflammation, partial CC hypoperfusion and texture disruptions persist for longer durations and may resolve in a time dependent manner. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:566-572.].

Visual acuity loss and development of ocular complications in white dot syndromes: a longitudinal analysis of 3 centers.

PURPOSE: To compare the clinical prognosis among selected white dot syndromes (WDS) (birdshot chorioretinopathy (BRC), multifocal choroiditis, serpiginous choroidopathy (SC), and others) and to identify risk factors of poor visual prognosis. METHODS: Retrospective longitudinal cohort study including 84 patients (143 affected eyes) diagnosed with WDS between 1982 and July 2017, followed up until loss of follow-up or December 2017, and recruited from three Uveitis Clinics (Madrid Community, Spain). Our main outcome measures were temporary or permanent moderate (corrected visual acuity in the Snellen scale < 20/50) or severe (< 20/200) vision losses, and development of new ocular complications. Incidence rates (IR) of the main outcome measures were estimated per 100 eye-years. Bivariate and multivariate Cox robust regression models analyzed the association of demographic- and clinical-related variables with vision loss. RESULTS: SC exhibited the greatest IR of vision loss, even in the multivariate models. Previous events of vision loss, presence of choroidal neovascularization, and cataracts exhibited worse visual prognosis. Monotherapy with immunosuppressive drugs but not combine therapy was also associated with higher IR of visual loss. Regarding new ocular complications, BRC showed the highest IR of epiretinal membrane and macular edema. CONCLUSIONS: SC presents the worst visual prognosis. Some ocular manifestations can identify patients with WDS at risk of a worse clinical evolution.