CHOROIDAL NEVUS ASSOCIATED WITH VITELLIFORM DEPOSITION IN A PATIENT WITH AUTOSOMAL DOMINANT BEST DYSTROPHY.

BACKGROUND/PURPOSE: To describe the clinical, optical coherence tomography (OCT), fundus autofluorescence and ultrasound findings of a patient with a choroidal nevus actively exuding vitelliform material in the setting of autosomal dominant Best dystrophy (BD). METHODS: The patient's clinical course was followed over time with ophthalmic examinations and multimodal imaging. RESULTS: A 71-year-old male patient with BD was referred for evaluation of a choroidal nevus in the right eye. Dilated fundoscopic examination showed a small pigmented choroidal nevus in the temporal periphery. Over a 3-year period, the nevus developed progressive deposition of vitelliform material along its inferior border. Meanwhile, OCT and fundus photography showed only slight growth. Ultrasound showed no change in height; basal measurements were confounded by the increased vitelliform deposits. Genetic testing confirmed a heterozygous mutation in the BEST1 gene and electrophysiology was consistent with BD. CONCLUSIONS: Dysfunction of the retinal pigment epithelium associated with BD may cause novel presentations of other conditions such as choroidal nevi. The implication for malignant transformation of a choroidal nevus associated with vitelliform deposit accumulation in this context is unknown.

Histocompatibility Leukocyte Antigen-A29-Associated Retinal Vasculitis without Choroidal Lesions: A Report of 4 Cases.

This study describes a cohort of patients presenting with histocompatibility leukocyte antigen (HLA)-A29-associated retinal vasculitis without choroidal lesions that may share clinical features with birdshot retinochoroiditis. The methods include a retrospective chart review of patients presenting with HLA-A29-associated retinal vasculitis without choroidal lesions. The data on the patients were entered retrospectively into a new database and analyzed. Four patients who had HLA-A29-associated retinal vasculitis without choroidal lesions were identified. The median age at presentation was 40 years (range: 14-71); 75% were female. At presentation, all four patients had a visual acuity of 20/50 or better in both eyes. All the eyes had mild vitritis, three eyes (37.5%) had cystoid macular edema, and two eyes (25%) had optic disc edema. All the patients required treatment with systemic steroids and immunosuppressive therapy. HLA-A29-associated retinal vasculitis without choroidal lesions appears to share many clinical features with birdshot chorioretinitis, including the need for systemic immunosuppressive therapy. Whether this entity represents an early form of birdshot retinochoroiditis or a more localized variant of the disease is a topic for additional studies.

CLINICAL OUTCOMES OF ACUTE ENDOPHTHALMITIS AFTER INTRAVITREAL DELIVERY OF VASCULAR ENDOTHELIAL GROWTH FACTOR INHIBITORS VERSUS STEROIDS.

PURPOSE: To compare patients with acute endophthalmitis after intravitreal injection of vascular endothelial growth factor (VEGF) inhibitors vs. steroids. METHODS: Retrospective single-center, nonrandomized interventional study from 2013 to 2021.Patients underwent vitreous biopsy before initiating treatment and were divided into the following cohorts: (1) anti-VEGF managed medically (T&I-anti-VEGF), (2) anti-VEGF managed by immediate pars plana vitrectomy (PPV-anti-VEGF), and (3) steroid therapy and managed medically or by pars plana vitrectomy (steroid). RESULTS: A total of 141 patients were analyzed. The steroid cohort demonstrated significantly worse presenting (median = 2.80 logarithm of the minimum angle of resolution [logMAR]; P ≤ 0.01) and final (median = 2.30 logMAR) best-corrected visual acuity compared with T&I-anti-VEGF (presenting: median = 2.00 logMAR; final: median = 0.40 logMAR) and pars plana vitrectomy-anti-VEGF cohorts (presenting: median = 2.30 logMAR; final: median = 0.48 logMAR). There was no significant ( P = 0.33) difference in the final best-corrected visual acuity between T&I-anti-VEGF and pars plana vitrectomy-anti-VEGF cohorts. There were no significant ( P ≥ 0.63) differences among cohorts in best-corrected visual acuity before acute endophthalmitis diagnosis (T&I-anti-VEGF: median = 0.40 logMAR; pars plana vitrectomy-anti-VEGF: median = 0.40 logMAR; steroid: median = 0.44 logMAR). Microbial cultures revealed similar profiles for all cohorts. CONCLUSION: Acute endophthalmitis after intravitreal injection steroid therapy had worse outcomes compared with anti-VEGF therapy.

BILATERAL UVEITIS AND HYPOTONY FOLLOWING TREATMENT WITH TOPICAL CIDOFOVIR.

PURPOSE: To report a case of bilateral uveitis and hypotony associated with topical cidofovir treatment. METHODS: Case report. RESULTS: A 59-year-old diabetic man with HIV/AIDS presented with photophobia, ocular pain, and decreased vision. He was found to have bilateral hypotony, anterior uveitis, and serous choroidal detachments. Infectious disease workup, patient-reported history, and review of the patient's electronic medication list did not identify the etiology. Treatment with intensive topical corticosteroids led to resolution of uveitis and choroidal effusions within 3 months and resolution of hypotony within 9 months. Two years after his initial presentation, the patient developed acute recurrence of bilateral hypotony, anterior uveitis, and serous choroidal detachments shortly after intravenous cidofovir treatment. Careful reevaluation of the patient's outside medical records revealed that he had initiated treatment for rectal herpes simplex virus with compounded topical cidofovir one month before his initial presentation. CONCLUSION: To our knowledge, this is the first reported case of topical cidofovir causing ocular toxicity. Compounded and topical medications, like cidofovir in this case, may not appear on a patient's electronic medication list, so a focused review of outside medical records may be beneficial when a particular medication toxicity is suspected.

Sustained Control of Serpiginous Choroiditis with the Fluocinolone Acetonide 0.18 mg Intravitreal Implant.

Purpose: To describe an alternative treatment for a patient with serpiginous choroiditis (SC) who was not tolerant to systemic therapies. Methods: Case report of a patient with serpiginous choroiditis with their clinical course followed with ophthalmic examinations and multimodal imaging overtime. Patients and Results. A 57-year-old female with serpiginous choroiditis was treated for seven years with numerous therapies including systemic steroids, immunosuppressive agents, and repeated dexamethasone intravitreal implants. The patient was intolerant of systemic therapies and would flare if dexamethasone injections were performed less frequently than every 8 weeks, making a viable long-term treatment plan problematic. Following one injection of the fluocinolone acetonide 0.18 mg intravitreal implant, she has experienced sustained control for 20 months. Discussion and Conclusions. Real-world treatment of SC is complex as long-term control is necessary, and associated side effects of the therapies provided may limit sustained use. The fluocinolone acetonide implant lasts 36 months and may be an alternative long-term management option, especially in the setting of systemic medication intolerance for some patients with SC.

Analysis of dose to the macula, optic disc, and lens in relation to vision toxicities - A retrospective study using COMS eye plaques.

PURPOSE: The aim of this study was to relate common toxicity endpoints with dose to the macula, optic disc, and lens for uveal melanoma patients treated with Iodine-125 Collaborative Ocular Melanoma Study (COMS) eye plaque brachytherapy. METHODS: A cohort of 52 patients treated at a single institution between 2005 and 2019 were retrospectively reviewed. Demographics, dosimetry, and clinical outcomes were recorded. Univariate, relative risk, and Kaplan-Meier analyses were performed to relate dose to toxicity endpoints including retinopathy, vision decline, and cataracts. RESULTS: By the end of follow up (Median = 3.6 years, Range = 0.4 - 13.5 years), 65 % of eyes sustained radiation retinopathy, 40 % demonstrated moderate vision decline (>5 Snellen lines lost), and 56 % developed cataracts. Significant (p < 0.05) risk estimates exist for retinopathy and VA decline for doses >52 Gy to the macula and >42 Gy to the optic disc. Moreover, dose to the lens > 16 Gy showed a significant risk for cataract formation. Kaplan-Meier analysis demonstrated significantly different incidence of radiation retinopathy for > 52 Gy to the macula and > 42 Gy to the optic disc. In addition, the Kaplan-Meier analysis showed significantly different incidence of cataract formation for patients with lens dose > 16 Gy. CONCLUSIONS: Dose-effect relationships exist for the macula and optic disc with respect to the loss of visual acuity and the development of retinopathy. To better preserve vision after treatment, further research is needed to reduce macula, optic disc, and lens doses while maintaining tumor control.

Rothia Mucilaginosa Endophthalmitis Associated With iStent Inject Implantation.

Postoperative endophthalmitis caused by Rothia mucilaginosa, a gram-positive, coagulase-negative Micrococcaceae of the oropharyngeal flora, is rare and all previously reported intraocular implant-related cases have resulted in evisceration. R. mucilaginosa endophthalmitis has also not been described with any glaucoma implant device. Here, the authors report a case of subacute R. mucilaginosa endophthalmitis after phacoemulsification with implantation of an intraocular lens and the iStent inject (Glaukos). The infection was managed with intravitreal antibiotic injections, pars plana vitrectomy, and intraocular lens and iStent explantations. This was followed by a second pars plana vitrectomy and silicone oil tamponade for a tractional retinal detachment. Nine months after the initial presentation, vision in the affected eye was partially preserved, and the globe remained intact. R. mucilaginosa infections are associated with grave outcomes due to biofilm formation on infected implants, and as such, removal of infected implants is advised.

Short term visual and structural outcomes of anti-vascular endothelial growth factor (anti-VEGF) treatment delay during the first COVID-19 wave: A pilot study.

Regularly scheduled intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections are essential to maintaining and/or improving many ocular conditions including: neovascular age-related macular degeneration (nAMD), diabetic retinopathy, and retinal vein occlusions with macular edema (RVO). This study aims to assess the effect of unintended delays in anti-VEGF treatment during the first wave of the COVID-19 pandemic. This retrospective case series identified patients receiving regularly scheduled anti-VEGF intravitreal injections based on current procedural terminology (CPT) code at two practices in Minnesota. Diagnoses were limited to nAMD, diabetic macular edema (DME), proliferative diabetic retinopathy, and RVO. Patients were divided into two groups based on whether they maintained or delayed their follow-up visit by more than two weeks beyond the recommended treatment interval during the COVID-19 lockdown. The 'COVID-19 lockdown' was defined as the period after March, 28th, 2020, when a lockdown was declared in Minnesota. We then compared the visual acuity and structural changes to the retina using ocular coherence tomography (OCT) to assess whether delayed treatment resulted in worse visual outcomes. A total of 167 eyes from 117 patients met criteria for inclusion in this study. In the delayed group, the average BCVA at the pre- and post-lockdown visits were 0.614 and 0.715 (logMAR) respectively (p = 0.007). Central subfield thickness (CST) increased from 341 to 447 in the DME delayed group (p = 0.03) while the CST increased from 301 to 314 (p = 0.4) in the nAMD delayed group. The results of this pilot study suggests that treatment delays may have a negative impact on the visual and anatomic outcomes of patients with nAMD and DME. Future studies with larger sample sizes are required for further investigation.

Bilateral Ocular Ischemic Syndrome in the Setting of Chronic Angle Closure Glaucoma.

We report novel case of a 57-year-old woman who developed bilateral ocular ischemic syndrome in the setting of chronic angle closure glaucoma without associated angle neovascularization. Detailed is a course in which markedly prolonged, elevated intraocular pressure led to significantly reduced arterial perfusion at the level of the central retinal artery, leading to the clinical picture of ocular ischemic syndrome.

Comparing fundus autofluorescence and infrared imaging findings of peripheral retinoschisis, schisis detachment, and retinal detachment.

PURPOSE: The primary goal of this study was to identify characteristic features of peripheral degenerative retinoschisis (RS), schisis detachment (SD) and retinal detachment (RD) on both fundus autofluorescence (FAF) and infrared (IR) imaging, using spectral domain optical coherence tomography (SD-OCT) imaging of the peripheral retina as the confirmatory imaging tool. METHODS: This is a descriptive case series study. A total of 27 eyes of 22 patients were included. Thirteen eyes of 10 patients diagnosed with RS, 4 eyes of 3 patients diagnosed with SD, and 10 eyes of 9 patients diagnosed with RD were included. Patients with images of poor quality were excluded. Heidelberg Spectralis HRA + OCT machine (Heidelberg Engineering, Heidelberg, Germany) were used to acquire the images. RESULTS: All conditions appeared as areas of hypo-AF on FAF and hypo-reflectance on IR imaging. Accentuated vasculature of the lesion was noted with IR imaging due to elevation of the RS and RD, which was less frequently observed with FAF. On FAF, a hyper-AF leading edge around the RS lesion indicated the presence of intraretinal or subretinal fluid and an extension of the RS. Retinal breaks/holes were best visualized with IR imaging. SD-OCT confirmed the diagnosis in all performed cases. CONCLUSIONS: We were unable to differentiate between RS and RD based solely on findings from FAF and IR imaging. However, the combination of them with SD-OCT can assist in the diagnosis of RS from RD and in the evaluation of RS progression. OCT remains the main modality imaging to differentiate these conditions.

Presumed mast cell choroidal infiltrate in aggressive systemic mastocytosis.

PURPOSE: To report a rare case of a unilateral choroidal mast cell infiltration in a patient with aggressive systemic mastocytosis (ASM). OBSERVATIONS: The patient is a man in his fifties with a diagnosis of ASM. He developed visual complaints in the right eye associated with an area of subretinal fluid on fundus examination. Visual acuity at presentation was 20/150 in the right eye and 20/25 in the left eye. After ophthalmic and radiologic imaging workup, the patient was diagnosed with presumed choroidal mast cell infiltrate. The index of suspicion was high due to the prior ASM diagnosis. External beam radiation and intravitreal injection treatments were offered but the patient declined. The patient was switched from interferon to a new targeted systemic therapy for ASM, midostaurin. Despite some mixed, temporary response in systemic symptoms/signs of ASM at four months, the choroidal lesion and subretinal fluid were stable with visual acuity at 20/125. CONCLUSION AND IMPORTANCE: Mast cell choroidal infiltration in ASM should be considered as part of the differential with acute/subacute vision changes. Diagnosis requires exclusion of other possibilities with ocular imaging and in this case, monitoring for development of other malignancies in which there were none. Midostaurin's ocular response was not on par with systemic response. Additional localized ocular therapies may be required.

ADVERSE EVENTS OF THE ARGUS II RETINAL PROSTHESIS: Incidence, Causes, and Best Practices for Managing and Preventing Conjunctival Erosion.

PURPOSE: To analyze and provide an overview of the incidence, management, and prevention of conjunctival erosion in Argus II clinical trial subjects and postapproval patients. METHODS: This retrospective analysis followed the results of 274 patients treated with the Argus II Retinal Prosthesis System between June 2007 and November 2017, including 30 subjects from the US and European clinical trials, and 244 patients in the postapproval phase. Results were gathered for incidence of a serious adverse event, incidence of conjunctival erosion, occurrence sites, rates of erosion, and erosion timing. RESULTS: Overall, 60% of subjects in the clinical trial subjects versus 83% of patients in the postapproval phase did not experience device- or surgery-related serious adverse events. In the postapproval phase, conjunctival erosion had an incidence rate of 6.2% over 5 years and 11 months. In 55% of conjunctival erosion cases, erosion occurred in the inferotemporal quadrant, 25% in the superotemporal quadrant, and 20% in both. Sixty percent of the erosion events occurred in the first 15 months after implantation, and 85% within the first 2.5 years. CONCLUSION: Reducing occurrence of conjunctival erosion in patients with the Argus II Retinal Prosthesis requires identification and minimization of risk factors before and during implantation. Implementing inverted sutures at the implant tabs, use of graft material at these locations as well as Mersilene rather than nylon sutures, and accurate Tenon's and conjunctiva closure are recommended for consideration in all patients.

Clinical Characteristics of a Case Series of Ocular Syphilis With 3 Cases Requiring Multiple Vitrectomies After Late Diagnosis.

Purpose: This work describes the characteristics and unique features of ocular syphilis. Methods: Ten serologically proven cases of ocular syphilis were retrospectively analyzed. Results: Eighteen eyes of 10 patients were affected. Nine of 10 patients were male and the mean age was 58 years (range, 36-81 years). HIV antibody testing was positive in 3 patients (30%). Five cases were first diagnosed by an ophthalmologist. One patient presented with a syphilitic rash. The most common ocular findings were panuveitis (n = 6) and cystoid macular edema (n = 4). Ocular involvement was unilateral in 2 cases and bilateral in 8. Best-corrected visual acuity improved in 13 of 18 eyes (72%) after treatment. Three cases developed recurrent retinal detachments that required repair with silicone oil. Conclusions: Most cases were HIV negative. Syphilitic uveitis can be the initial presentation of syphilis without classic systemic manifestation. Ophthalmologists play an important role in the diagnosis and treatment of syphilis.

Hemifacial Spasm in an Argus II Retinal Prosthesis Patient.

Purpose: A 59-year-old man with retinitis pigmentosa, who received an Argus II retinal prosthesis in the left eye 3 years prior, presented with left-sided involuntary facial contractions that occurred only after turning on the Argus II device. Methods: A case report. Results: Given that this patient's reported and visualized lower eyelid twitching corresponds to the location of the external region of the implanted coil, we hypothesize that heat or wirelessly transmitted electrical signals from the external coil to the implanted coil may induce spasm of the facial nerve and thus play a role in hemifacial spasm onset. Conclusions: To our knowledge, this is the first report of hemifacial spasm associated with Argus II use.

RETOUCH: The Retinal OCT Fluid Detection and Segmentation Benchmark and Challenge.

Retinal swelling due to the accumulation of fluid is associated with the most vision-threatening retinal diseases. Optical coherence tomography (OCT) is the current standard of care in assessing the presence and quantity of retinal fluid and image-guided treatment management. Deep learning methods have made their impact across medical imaging, and many retinal OCT analysis methods have been proposed. However, it is currently not clear how successful they are in interpreting the retinal fluid on OCT, which is due to the lack of standardized benchmarks. To address this, we organized a challenge RETOUCH in conjunction with MICCAI 2017, with eight teams participating. The challenge consisted of two tasks: fluid detection and fluid segmentation. It featured for the first time: all three retinal fluid types, with annotated images provided by two clinical centers, which were acquired with the three most common OCT device vendors from patients with two different retinal diseases. The analysis revealed that in the detection task, the performance on the automated fluid detection was within the inter-grader variability. However, in the segmentation task, fusing the automated methods produced segmentations that were superior to all individual methods, indicating the need for further improvements in the segmentation performance.

ENDOGENOUS NOCARDIA ENDOPHTHALMITIS PRESENTING AS A MASS LESION IN A PATIENT WITH METATSTATIC NONSMALL CELL CARCINOMA OF THE LUNG.

PURPOSE: To report a case of Nocardia cyriacigeorgica endophthalmitis. METHODS: Case report. RESULTS: A 71-year-old man on long-term steroid therapy for poor respiratory function from lung carcinoma and presumed recent liver metastases presented with a four week history of blurry vision in the left eye. Ophthalmic examination revealed a white elevated mass in the macula with hemorrhage, concerning for metastasis. The patient was treated for presumed ocular metastases but had poor response to radiotherapy and was diagnosed four weeks later with N. cyriacigeorgica by retinal biopsy. Despite intravitreal and systemic antibiotics, the patient had progression of disease and died of respiratory involvement. CONCLUSION: Nocardia endogenous endophthalmitis can present as a mass retinal lesion in immunosuppressed patients with metastatic disease. Early vitreous and retinal biopsy may be required for definitive diagnosis and treatment. A pulmonary source of infection should be suspected and monitored closely.

OCT Fluid Segmentation using Graph Shortest Path and Convolutional Neural Network.

Diagnosis and monitoring of retina diseases related to pathologies such as accumulated fluid can be performed using optical coherence tomography (OCT). OCT acquires a series of 2D slices (Bscans). This work presents a fully-automated method based on graph shortest path algorithms and convolutional neural network (CNN) to segment and detect three types of fluid including sub-retinal fluid (SRF), intra-retinal fluid (IRF) and pigment epithelium detachment (PED) in OCT Bscans of subjects with age-related macular degeneration (AMD) and retinal vein occlusion (RVO) or diabetic retinopathy. The proposed method achieves an average dice coefficient of 76.44%, 92.25% and 82.14% in Cirrus, Spectralis and Topcon datasets, respectively. The effectiveness of the proposed methods was also demonstrated in segmenting fluid in OCT images from the 2017 Retouch challenge.

Association of Optic Nerve Head Drusen with Best Vitelliform Macular Dystrophy: A Case Series.

PURPOSE: To report the association of optic nerve head (ONH) drusen with Best vitelliform macular dystrophy (BVMD). METHODS: Chart review. PATIENTS: Five patients from 3 families. RESULTS: Multimodal imaging and ophthalmic examination demonstrated findings consistent with ONH drusen, in association with BVMD, in 5 patients. CONCLUSION: We report the association of BVMD with ONH drusen in 5 patients. This combination has previously been reported only once. We recommend that patients with a diagnosis of BVMD undergo autofluorescence and ultrasound imaging of the optic nerve to help facilitate this diagnosis, as some ONH drusen can be buried.

Fully Automated Segmentation of Fluid/Cyst Regions in Optical Coherence Tomography Images With Diabetic Macular Edema Using Neutrosophic Sets and Graph Algorithms.

This paper presents a fully automated algorithm to segment fluid-associated (fluid-filled) and cyst regions in optical coherence tomography (OCT) retina images of subjects with diabetic macular edema. The OCT image is segmented using a novel neutrosophic transformation and a graph-based shortest path method. In neutrosophic domain, an image is transformed into three sets: (true), (indeterminate) that represents noise, and (false). This paper makes four key contributions. First, a new method is introduced to compute the indeterminacy set , and a new -correction operation is introduced to compute the set in neutrosophic domain. Second, a graph shortest-path method is applied in neutrosophic domain to segment the inner limiting membrane and the retinal pigment epithelium as regions of interest (ROI) and outer plexiform layer and inner segment myeloid as middle layers using a novel definition of the edge weights . Third, a new cost function for cluster-based fluid/cyst segmentation in ROI is presented which also includes a novel approach in estimating the number of clusters in an automated manner. Fourth, the final fluid regions are achieved by ignoring very small regions and the regions between middle layers. The proposed method is evaluated using two publicly available datasets: Duke, Optima, and a third local dataset from the UMN clinic which is available online. The proposed algorithm outperforms the previously proposed Duke algorithm by 8% with respect to the dice coefficient and by 5% with respect to precision on the Duke dataset, while achieving about the same sensitivity. Also, the proposed algorithm outperforms a prior method for Optima dataset by 6%, 22%, and 23% with respect to the dice coefficient, sensitivity, and precision, respectively. Finally, the proposed algorithm also achieves sensitivity of 67.3%, 88.8%, and 76.7%, for the Duke, Optima, and the university of minnesota (UMN) datasets, respectively.