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1.
Ophthalmic Genet ; 43(4): 543-549, 2022 08.
Article in English | MEDLINE | ID: covidwho-2062673

ABSTRACT

BACKGROUND: Coats plus syndrome or cerebroretinal microangiopathy with calcifications and cysts (CMCC) is an exceedingly rare autosomal recessive disorder that predominantly affects the microvasculature in the retina, brain, bones, and gastrointestinal system. Unlike Coats disease, CMCC is bilateral and affects multiple organ systems. MATERIALS AND METHODS: Case report. RESULTS: We report the case of two brothers with Coats Plus syndrome who presented with variable phenotypic expression. One sibling (Patient 1) was thought to have atypical retinopathy of prematurity and was only diagnosed with Coats plus after his older brother (Patient 2) presented with a seizure and a left upper extremity tremor at 4 years of age. The CTC1 mutation was confirmed in both patients. Aggressive treatment with laser photocoagulation and intravitreal bevacizumab dramatically improved the retinal vascular and exudative changes. CONCLUSION: Coats Plus syndrome can have a variable phenotypic presentation, including retinal vascular findings. This rare genetic disease should be in the differential diagnosis in patients who present with atypical retinal pathology, including Retinopathy of Prematurity, Familial Exudative Vitreoretinopathy, or Coats disease associated with non-specific multiorgan abnormalities.


Subject(s)
Central Nervous System Cysts , Leukoencephalopathies , Retinal Telangiectasis , Retinopathy of Prematurity , Ataxia , Brain Neoplasms , Calcinosis , Central Nervous System Cysts/genetics , Humans , Infant, Newborn , Laser Coagulation , Leukoencephalopathies/genetics , Male , Muscle Spasticity , Retinal Diseases , Retinal Telangiectasis/diagnosis , Retinal Telangiectasis/genetics , Retinal Telangiectasis/therapy , Retinopathy of Prematurity/diagnosis , Retinopathy of Prematurity/genetics , Seizures
2.
Ophthalmic Surg Lasers Imaging Retina ; 53(6): 326-331, 2022 06.
Article in English | MEDLINE | ID: covidwho-1903576

ABSTRACT

BACKGROUND AND OBJECTIVE: The most common clinical features of X-linked retinoschisis (XLRS) include macular schisis in a spoke wheel pattern and peripheral schisis, though other findings such as vitreous veils, vascular attenuation, and subretinal fibrosis have been described. This is the first report to describe retinal folds as a characteristic feature in patients with XLRS. PATIENTS AND METHODS: This was a case series of patients presenting to the retina service at Bascom Palmer Eye Institute with genetically confirmed XLRS. Patients included in this report underwent examination under anesthesia with multimodality imaging. RESULTS: Three patients with XLRS were found to have retinal folds, including a newly characterized "retinal scroll" seen on examination and multimodality imaging. CONCLUSIONS: The presence of a retinal fold should yield a differential diagnosis that includes XLRS in the correct clinical context. Panel-based genetic testing and multimodal imaging are useful in guiding clinical management. [Ophthalmic Surg Lasers Imaging 2022;53(6):326-331.].


Subject(s)
Retinoschisis , Eye Proteins/genetics , Humans , Multimodal Imaging , Retina , Retinoschisis/diagnosis , Retinoschisis/genetics , Tomography, Optical Coherence/methods , Visual Acuity
3.
Am J Ophthalmol Case Rep ; 26: 101522, 2022 Jun.
Article in English | MEDLINE | ID: covidwho-1797276

ABSTRACT

Purpose: To report a case of a central retinal vein occlusion (CRVO) associated with COVID-19 in a patient predisposed to clotting due to a genetic mutation in methylenetetrahydrofolate reductase (MTHFR). Observations: A 15-year-old male presented with 1 day of painless blurry vision in the left eye. Exam disclosed trace anterior chamber cell, anterior vitreous cell, optic nerve head edema, temporally displaced macular star, dilated tortuous veins, and diffuse intraretinal hemorrhages. Exam and FA was consistent with CRVO, however the macular star and OCT were suggestive of a neuroretinitis.The patient then presented to a children's hospital for further evaluation. A routine screen for COVID-19 via nasopharyngeal swab was positive with a high viral load. He also had a known history of an MTHFR mutation. Extensive laboratory and neuroradiologic evaluation excluded other infectious, inflammatory, and coagulopathic etiologies. Conclusions and Importance: This is a case of CRVO associated with COVID-19 infection and an underlying systemic hypercoagulable mutation, with an initial presentation that mimicked neuroretinitis. This case provides valuable diagnostic learning points and expands our knowledge of possible ocular complications of COVID-19.

4.
J Vitreoretin Dis ; 4(5): 420-429, 2020 Oct 01.
Article in English | MEDLINE | ID: covidwho-1295335

ABSTRACT

PURPOSE: To detail the rationale behind recommendations recently published by the American Society of Retina Specialists (ASRS) outlining best practices for safety of vitreoretinal surgeons and staff while performing vitreoretinal surgery during the coronavirus disease (COVID)-19 pandemic. METHODS: The committee for ASRS Best Practices for Retinal Surgery during the COVID-19 Pandemic reviewed existing evidence and information on SARS-CoV-2 transmission, and risk factors during vitreoretinal surgery. Recommendations were based on best available published data, cumulative clinical experiences, and recommendations and policies from other organizations. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to assess the strength of recommendations and confidence in the evidence. These serve as interim recommendations which are routinely updated given gaps of knowledge and lack of high-quality data on this evolving subject. RESULTS: Relevant existing literature related to methods of transmission, and ocular manifestations of SARS-CoV-2 are summarized. The data and clinical experiences driving recommendations for pre-operative, intraoperative and post-operative surgical considerations, anesthesia choice, as well as considerations for intravitreal injections are provided. CONCLUSION: Recommendations are provided with the goal of protecting vitreoretinal surgeons and associated personnel from exposure to SARS-CoV-2 during interventional vitreoretinal procedures. This is a rapidly evolving topic with numerous remaining gaps in our current knowledge. As such, recommendations will evolve and the current manuscript is intended to serve as a foundation for continued dialogue on best practices.

5.
JAMA Ophthalmol ; 139(4): 456-463, 2021 04 01.
Article in English | MEDLINE | ID: covidwho-1116915

ABSTRACT

Importance: The American Academy of Ophthalmology (AAO) indicated that urgent or emergent vitreoretinal surgical procedures should continue during the coronavirus disease 2019 (COVID-19) pandemic. Although decreases in the frequency of critical procedures have been reported outside the field of ophthalmology, analyses are limited by volume, geography, and time. Objective: To evaluate whether the frequency of ophthalmic surgical procedures deemed urgent or emergent by the AAO changed across the United States during the COVID-19 pandemic. Design, Setting, and Participants: Vitreoretinal practices from 17 institutions throughout the US participated in this multicenter cross-sectional study. The frequency of 11 billed vitreoretinal Current Procedural Terminology (CPT) codes across respective weeks was obtained from each practice between January 1, 2019, and May 31, 2020. Data were clustered into intravitreal injections (code 67028), lasers and cryotherapy (codes 67141, 67145, and 67228), retinal detachment (RD) repairs (codes 67107, 67108, 67110, and 67113), and other vitrectomies (codes 67036, 67039, and 67040). Institutions were categorized by region (Northeast, Midwest, South, and West Coast), practice setting (academic [tax-exempt] or private [non-tax-exempt]), and date of respective statewide stay-at-home orders. Main Outcomes and Measures: Nationwide changes in the frequency of billing for urgent or emergent vitreoretinal surgical procedures during the COVID-19 pandemic. Results: A total of 526 536 CPT codes were ascertained: 483 313 injections, 19 257 lasers or cryotherapy, 14 949 RD repairs, and 9017 other vitrectomies. Relative to 2019, a weekly institutional decrease in injections was observed from March 30 to May 2, 2020, with a maximal 38.6% decrease (from a mean [SD] of 437.8 [436.3] to 273.8 [269.0] injections) from April 6 to 12, 2020 (95% CI, -259 to -69 injections; P = .002). A weekly decrease was also identified that spanned a longer interval, at least until study conclusion (March 16 to May 31, 2020), for lasers and cryotherapy, with a maximal 79.6% decrease (from a mean [SD] of 6.6 [7.7] to 1.5 [2.0] procedures) from April 6 to 12, 2020 (95% CI, -6.8 to -3.3 procedures; P < .001), for RD repairs, with a maximal 59.4% decrease (from a mean [SD] of 3.5 [4.0] to 1.6 [2.2] repairs) from April 13 to 19, 2020 (95% CI, -2.7 to -1.4 repairs; P < .001), and for other vitrectomies, with a maximal 84.3% decrease (from a mean [SD] of 3.0 [3.1] to 0.4 [0.8] other vitrectomies) from April 6 to 12, 2020 (95% CI, -3.3 to -1.8 other vitrectomies; P < .001). No differences were identified by region, setting, or state-level stay-at-home order adjustment. Conclusions and Relevance: Although the AAO endorsed the continued performance of urgent or emergent vitreoretinal surgical procedures, the frequency of such procedures throughout the country experienced a substantial decrease that may persist after the COVID-19 pandemic's initial exponential growth phase. This decrease appears independent of region, setting, and state-level stay-at-home orders. It is unknown to what extent vitreoretinal intervention would have decreased without AAO recommendations, and how the decrease is associated with outcomes. Although safety is paramount during the COVID-19 pandemic, practices should consider prioritizing availability for managing high-acuity conditions until underlying reasons for the reduction are fully appreciated.


Subject(s)
COVID-19/epidemiology , SARS-CoV-2 , Vitreoretinal Surgery/statistics & numerical data , Cross-Sectional Studies , Emergency Medical Services , Humans , Vitrectomy/statistics & numerical data
6.
Ophthalmic Surg Lasers Imaging Retina ; 51(11): 651-652, 2020 11 01.
Article in English | MEDLINE | ID: covidwho-976384

ABSTRACT

Certain pediatric ophthalmology procedures require time-sensitive surgical care to prevent long-term morbidity and mortality. In the era of the COVID-19 pandemic, it has become increasingly important for anesthesia and surgical care providers to protect themselves during medically necessary procedures requiring general anesthesia care. In this study, an intubating viewing system with plexiglass shield and drape was designed. The viewing system allowed effective and safe intubation of pediatric patients for ophthalmological surgery while minimizing the risk of disseminating aerosolized droplets. The authors concluded that this viewing system and modified intubation technique may reduce the risk of transmitting SARSCoV-2 and other highly transmissible pathogens to operating room personnel. The continued study of measures to protect operating room personnel is encouraged to provide more evidence-based recommendations. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:651-652.].


Subject(s)
Anesthesia, General/methods , COVID-19/epidemiology , Disease Transmission, Infectious/prevention & control , Eye Diseases/surgery , Ophthalmologic Surgical Procedures/standards , Personal Protective Equipment , SARS-CoV-2 , Child , Comorbidity , Eye Diseases/epidemiology , Humans , Pandemics
7.
Am J Ophthalmol ; 222: 368-372, 2021 02.
Article in English | MEDLINE | ID: covidwho-739729

ABSTRACT

PURPOSE: This study characterized the delivery of emergent ophthalmic surgical care during April 2020 of the coronarvirus disease-19 (COVID-19) pandemic compared with the same interval the previous year. DESIGN: Retrospective observational before-and-after study. METHODS: This study reviewed and characterized each emergent and/or urgent procedure performed during April 2020 and April 2019 at a single tertiary ophthalmology referral center. Information collected included the details of patient presentation, diagnosis, surgical procedure, and preoperative COVID-19 testing. RESULTS: In total, 117 surgical procedures were performed on 114 patients during the month of April 2020 compared with 1,107 performed in April 2019 (P < .0001). Retinal detachment repair was the most common procedure (n = 37; 31.6%) in April 2020, whereas elective cataract surgery (n = 481; 47.3%) was the most common procedure in April 2019. The mean age of patients was 50.0 years in April 2020 compared with 59.0 years (P < .0001) the previous year. During April 2020, the mean age of surgeons performing procedures was 42.3 years compared with 48.4 years (P < .0001) during April 2019. In April 2020, all but 5 patients (96%) had reverse transcriptase polymerase chain reaction based COVID-19 testing before their procedure. One patient (0.88%) had a positive COVID-19 test. CONCLUSIONS: The COVID-19 pandemic decreased our institution's surgical volume in April 2020 to approximately 10% of the usual volume. The pandemic changed the type of cases performed and led to a statistically significant decrease in both the age of our surgeons and patients relative to the same interval in the previous year. Broad preoperative screening led to 1 positive COVID-19 test in an asymptomatic patient.


Subject(s)
COVID-19/epidemiology , Elective Surgical Procedures/methods , Eye Diseases/surgery , Ophthalmologic Surgical Procedures/methods , Pandemics , Referral and Consultation/statistics & numerical data , SARS-CoV-2 , Tertiary Care Centers/statistics & numerical data , Adult , Comorbidity , Eye Diseases/epidemiology , Female , Humans , Male , Middle Aged , Retrospective Studies
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