Atypical bilateral acute retinal necrosis in a coronavirus disease 2019 positive immunosuppressed patient.

PURPOSE: To present the clinical features of a rare case of atypical acute retinal necrosis in a Coronavirus Disease 2019 (COVID-19) positive immunosuppressed patient. METHODS: Retrospective observational case report. RESULTS: A 75-year-old lady presented with a left eye pan uveitis picture with vitritis and extensive peripheral and mid-peripheral necrotising retinitis. In the right eye, she had a very mild superior peripheral retinitis with minimal anterior or vitreous inflammation. Two months prior to her diagnosis she completed a course of rituximab and chlorambucil chemotherapy for a relapse of diffuse large cell B-cell lymphoma (DLBCL). The patient's nasopharyngeal swabs tested positive for COVID-19 in a reverse transcription polymerase chain reaction (RT-PCR) assay. The vitreous sample PCR tested positive for Varicella Zoster Virus and was negative for SARS-CoV-2. CONCLUSION AND SIGNIFICANCE: To the best of our knowledge this is the first description of a case that has undergone vitreous PCR testing for COVID-19. It is interesting to note the high level of vitreous inflammation which would not be expected in an immunosuppressed state. We present a number of possible links between the SARS-CoV-2 virus and the unusual ocular presentation of bilateral VZV viral retinitis in this patient.While extra ocular VZV outbreaks have been reported with rituximab treated patients, this report should also raise the awareness of VZV related viral retinitis in DLBCL patients on rituximab chemotherapy which is a very rare occurrence.This case may provide some evidence to healthcare policy makers who are making decisions regarding the re-introduction of routine Ophthalmic surgery.

Real-World Efficacy and Safety of Mycophenolate Mofetil in Active Moderate-to-Sight-Threatening Thyroid Eye Disease.

PURPOSE: There is no universal consensus on second-line agents for the treatment of moderate/severe to sight-threatening thyroid eye disease (TED) to maintain remission after first-line intravenous methylprednisolone (IVMP). This study investigates the efficacy and safety of mycophenolate mofetil (MMF) in TED patients in a real-world setting and over a longer period than previous randomized controlled trials. METHODS: A retrospective cohort study of TED patients with active moderate/severe to sight-threatening TED seen over a 4-year period. Data collected were visual acuity (VA), Clinical Activity Score (CAS), Gorman Diplopia scores, MMF dosing and side effects at 24, 52 and 78 weeks. Clinical efficacy was defined as an absence of relapse: no decline in best corrected LogMAR VA, no need for further steroids, no increase in CAS of ≥2. RESULTS: Out of 23 patients, 20 patients were included in this study. 10% (2/20) stopped MMF before 24 weeks. Median duration of MMF treatment was 76 weeks (1-140 weeks). 55% (11/20) had dysthyroid optic neuropathy (DON). In those with active moderate-severe TED without DON, clinical efficacy was seen in 100% (8/8) at 24 weeks, 87.5% (7/8) at 52 weeks, and 83.3% (5/6) at 78 weeks, with CAS decreasing from a baseline of 2.78±1.99 to 0.50±0.58 at 24 weeks, 0.50±0.82 at 52 weeks and 1.00±1.30 at 78 weeks. In DON, improvements were seen in 90% (9/10) at 24 weeks, 100% (7/7) at 52 weeks and 100% (4/4) at 78 weeks, with significantly reduced CAS scores from 2.55±1.54 to 0.83±1.27, 1.00±1.17 and 0.63±0.95 at 24, 52 and 78 weeks, respectively. Gorman score, VA and soft tissue inflammation parameters also improved throughout. There were two significant side effects over the treatment period. CONCLUSION: MMF appears to be an effective and safe second-line immunosuppressive agent. Further studies aimed at elucidating optimal dosing regimens and ideal treatment duration will prove helpful.

Atypical presentation of Coats' Syndrome in facioscapulohumeral dystrophy - Reflecting the variation in phenotypic manifestations.

PURPOSE: To report a unique case of atypical Coats' Syndrome in an 80 year old female with facioscapulohumeral dystrophy. OBSERVATIONS: An 80 years old female was diagnosed clinically of retinal telangiectasia with exudation threatening the fovia. She received a successful macular laser photocoagulation with subsequent cessation of leakage. CONCLUSIONS AND IMPORTANCE: This case is in keeping with Coats' syndrome in fascioscapulohumeral dystrophy, which classically affects young male subjects - making this patient an obvious outlier. This once again reflects the variation in phenotypic manifestations of inherited disorders.