ObjectiveTo present an established practice protocol for safe and effective hospital-setting ophthalmic practice during the coronavirus disease 2019 (COVID-19) pandemic.Methods and AnalysisLiterature was reviewed to identify articles relevant to COVID-19 pandemic and ophthalmology. The following keywords were used: COVID-19, SARS-CoV-2 and telemedicine, combined with eye, ophthalmology, conjunctivitis and tears. Data were extracted from the identified manuscripts and discussed among subspecialists to obtain consensus evidence-based practice.ResultsA protocol for ophthalmic practice in the era of COVID-19 pandemic was established. The protocol covered patient screening, clinic flow, required personal protective equipment and modifications of ophthalmic equipment for improved safety.ConclusionImportant literature emerged with respect to the practice of ophthalmology in the era of COVID-19. An evidence-based ophthalmic practice protocol was established and should be modified in the future to accommodate new insights on the COVID-19 pandemic.
Purpose:RPGRIP1 encodes a ciliary protein expressed in the photoreceptor connecting cilium. Mutations in this gene cause ∼5% of Leber congenital amaurosis (LCA) worldwide, but are also associated with cone–rod dystrophy (CRD) and retinitis pigmentosa (RP) phenotypes. Our purpose was to clinically characterize RPGRIP1 patients from our cohort, collect clinical data of additional RPGRIP1 patients reported previously in the literature, identify common clinical features, and seek genotype–phenotype correlations.Methods: Clinical data were collected from 16 patients of our cohort and 212 previously reported RPGRIP1 patients and included (when available) family history, best corrected visual acuity (BCVA), refraction, comprehensive ocular examination, optical coherence tomography (OCT) imaging, visual fields (VF), and full-field electroretinography (ffERG).Results: Out of 228 patients, the majority (197, 86%) were diagnosed with LCA, 18 (7%) with RP, and 13 (5%) with CRD. Age of onset was during early childhood (n = 133, average of 1.7 years). All patients but 6 had moderate hyperopia (n = 59, mean of 4.8D), and average BCVA was 0.06 Snellen (n = 124; only 10 patients had visual acuity [VA] > 0.10 Snellen). On funduscopy, narrowing of blood vessels was noted early in life. Most patients had mild bone spicule-like pigmentation starting in the midperiphery and later encroaching upon the posterior pole. OCT showed thinning of the outer nuclear layer (ONL), while cystoid changes and edema were relatively rare. VF were usually very constricted from early on. ffERG responses were non-detectable in the vast majority of cases. Most of the mutations are predicted to be null (363 alleles), and 93 alleles harbored missense mutations. Missense mutations were identified only in two regions: the RPGR-interacting domain and the C2 domains. Biallelic null mutations are mostly associated with a severe form of the disease, whereas biallelic missense mutations usually cause a milder disease (mostly CRD).Conclusion: Our results indicate that RPGRIP1 biallelic mutations usually cause severe retinal degeneration at an early age with a cone–rod pattern. However, most of the patients exhibit preservation of some (usually low) BCVA for a long period and can potentially benefit from gene therapy. Missense changes appear only in the conserved domains and are associated with a milder phenotype.
To present our hybrid telehealth medical retina clinic service with intravitreal injections (IVI) treatment as a safe alternative to in-person visits and examination during COVID-19 pandemic disease. Methods: Due to exposure to a COVID-19 positive retina fellow, our retina service, in quarantine, evaluated patients' medical files and retinal scans using a telemedicine approach. A different protocol for patients coming for IVI during the COVID-19 pandemic was established for IVI administration. Results: During the 14-day quarantine period (between March 18th and March 31st 2020), the hybrid telehealth medical retina clinic performed 523 IVI to 394 patients with a mean age ± SD 70.96 ± 14.4 years. IVI were administered for neovascular age-related macular degeneration in 50.5% of the cases (199 patients), diabetic macular edema in 21.3% (84 patients), retinal vein occlusion in 17.5% (69 patients), and 10.7% for other retinal pathologies (42 patients). No ocular or systemic complications were observed. Conclusion: During disasters and pandemics, IVI can be provided safely using a hybrid telehealth medical retina clinic approach but only in the appropriate patient and health care system.
Premacular membranes developing following pars plana vitrectomy (PPV) can cause significant anatomical and functional deficits to the macula. Recent reports showed that postoperative premacular membranes are a localized presentation of macular proliferative vitreoretinopathy (mPVR). Here, we report retrospectively a case series of 5 patients with severe mPVR which developed following uneventful PPV and were followed up to 32 months in the Department of Ophthalmology, Hadassah-Hebrew University Medical Center, Jerusalem, between October 2016 and February 2020. All patients underwent primary repair of rhegmatogenous retinal detachment (RRD) before mPVR developed. Mean best-corrected visual acuity (BCVA) at presentation was 20/76 Snellen (0.58 LogMAR). Median duration of the retinal detachment time until surgery was 1.5 days (range 1–21 days). Mean interval time from last normal follow-up exam to diagnosis of mPVR was 19 days (range 10–28). BCVA dropped from a mean of 20/38 Snellen (0.28 LogMAR) prior to mPVR development to 20/166 Snellen (0.92 LogMAR) following its development, recovering to 20/57 Snellen (0.45 LogMAR) after peeling of membranes. Mean central macular thickness measured by optical coherence tomography decreased from 711 to 354 μm postsurgery. In conclusion, short-term mPVR is a different entity from macular pucker in terms of rapid development, structural distortion, and visual compromise. Surgical treatment significantly restores macular function and anatomy.
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