Bilateral central retinal vein occlusion following COVID-19 vaccination: Cause or coincidence?

AIM: To report a case of sequential bilateral ischemic central retinal vein occlusion (CRVO) following the third dose of anti-COVID 19 vaccination. METHODS: Observational case report. RESULTS: A 73-year-old Caucasian male patient, with no known medical history, complained of sudden vision loss in his right eye (RE) 18 days following the third dose of Pfizer-BioNTech anti-COVID 19 vaccination. Ten days later, he suffered from sudden vision loss in his left eye (LE).Best-corrected visual acuity was limited to counting fingers at 50cm in both eyes.Fundus examination of both eyes revealed signs of ischemic central retinal vein occlusion (CRVO) with diffuse superficial and deep retinal hemorrhages in all four quadrants. Diagnosis was confirmed of fluorescein angiography.Optical coherent tomography (OCT) showed an ischemic hyperreflectivity and disorganization of the inner retinal layers in both eyes with significantly increased central macular thickness, associated to intraretinal fluid accumulation in LE.An urgent systemic assessment was requested. A mild hypertension was discovered and the rest of the work up was unremarkable. CONCLUSION: To our knowledge, we report the first case of bilateral CRVO in a healthy patient after anti-COVID 19 vaccination. CRVO occurred few days following third shot of vaccine followed by a sequential CRVO in the fellow eye in a patient with recently diagnosed very mild hypertension and no thrombo-embolic risk factors, strongly suggesting a relationship between both events. Nowadays, CRVO should be kept in mind as a potential side effect of Covid-19 vaccination and should be added to the spectrum of their ophthalmic complications.

Atypical presentation of bilateral iridoschisis with abnormal visibility of iris vessels: Role of anterior segment optical coherence tomography.

PURPOSE: To describe clinical and anterior segment optical coherence tomography (AS-OCT) findings in a patient with bilateral iridoschisis and unilateral angle closure glaucoma (ACG) associated with abnormal visibility of iris vessels. CASE PRESENTATION: A 67-year-old male patient with a history of red and painful left eye (LE) one year earlier, presented to our ophthalmology department for a routine examination.Ophthalmic examination of the right eye revealed narrow anterior chamber with sectorial iris atrophy associated to abnormal visibility of an iris vessel. Intraocular pressure (IOP) was 12 mmHg with normal optic disc appearance. LE anterior chamber was narrow with diffuse iris atrophy and abnormal vessels visibility. IOP was 28 mmHg with an important optic disc excavation. On gonioscopy, angle was narrow without neovessels nor synechiae. AS-OCT of both eyes revealed shallow angles, iris splitting with material release in the anterior chamber, while pigmented epithelium was preservedAnti-glaucoma eye drops were prescribed and peripheral laser iridotomy was performed in both eyes with decreased IOP at 14 mmHg in the LE. CONCLUSION: Iridoschisis is a rare ocular condition characterized by a separation between the anterior and posterior layers of iris stroma with several clinical presentations, and may be associated with abnormal visibility of iris vessels in some cases. The diagnosis of iridoschisis may be challenging and AS-OCT can be a very useful tool to confirm the diagnosis in atypical presentations and to detect associated angle closure.

Widefield oct-angiography-based classification of sickle cell retinopathy.

PURPOSE: To assess the capillary non-perfusion in different concentric sectors on widefield optical coherence tomography angiography (WF-OCTA) and to correlate the ratio of non-perfusion (RNP) to the severity of sickle cell retinopathy (SCR). METHODS: This retrospective, cross-sectional study included eyes of patients with various sickle cell disease (SCD) genotypes having undergone WF-OCTA and ultra-widefield color fundus photography (UWF-CFP). Eyes were grouped as no SCR, non-proliferative SCR or proliferative SCR. RNP was assessed on WF-OCTA montage in different field-of-view (FOV) sectors centered on the fovea: 0-10-degrees circle excluding the foveal avascular zone, the 10-30-degrees circle excluding the optic nerve, the 30-60-degrees circle, and the full 60-degrees circle. RESULTS: Forty-two eyes of twenty-eight patients were included. Within each SCR group, mean RNP of the FOV 30-60 sector was higher than all other sectors (p < 0.05). Mean RNP of all sectors were significatively different between no SCR group and proliferative SCR group (p < 0.05). To distinguish no SCR versus non-proliferative SCR FOV 30-60 had a good sensitivity and specificity of 41.67% and 93.33%, respectively (cutoff RNP > 22.72%, AUC = 0.75, 95% CI 0.56-0.94, p = 0.028). To differentiate non-proliferative versus proliferative SCR, FOV 0-10 had good sensitivity and specificity of 33.33% and 91.67%, respectively (cutoff RNP > 18.09, AUC = 0.73, 95% CI 0.53 to 0.93, p = 0.041). To discern no SCR versus proliferative SCR, all sectors had optimal sensitivity and specificity (p < 0.05). CONCLUSION: WF OCTA-based RNP provides non-invasive diagnostic information regarding the presence and severity of SCR, and correlates with disease stage in certain FOV sectors.

OCT-angiography assessing quiescent and active choroidal neovascularization in retinitis pigmentosa associated with PRPH2 pathogenic variant.

PURPOSE: To report multimodal imaging findings including optical coherence tomography angiography (OCT-A) of a patient presenting with a quiescent choroidal neovascularization (CNV) in one eye and an active CNV in the fellow eye, complicating retinitis pigmentosa (RP) linked to PRPH2 pathogenic variant, with follow-up and management of both eyes. METHODS: Observational case report. RESULTS: A 40-year-old female with history of autosomal dominant RP consulted for acute visual loss in her right eye (RE). Multimodal imaging including OCT-A confirmed the diagnosis of active type 2 CNV in the RE and highlighted an incidental asymptomatic non-exudative "quiescent" CNV in the left eye (LE). This complication was managed by intra-vitreal Bevacizumab injections in the RE and regular monitoring of the LE. Frequent follow-up could detect early CNV activation signs in LE allowing early treatment. Mutation analysis of PRPH2 exons identified a known heterozygous pathogenic missense variation c.646C>T, p.P216S in exon 2. CONCLUSION: Multimodal imaging and especially OCT-A can be of a great help in the diagnosis and the management of CNV complicating RP, even at the stage of quiescent CNV. In presence of neovascular complication, PRPH2 gene should be investigated because of its frequent macular involvement despite high phenotypic variability.

Clinical and Genetic Findings of Autosomal Recessive Bestrophinopathy (ARB).

Mutations in BEST1 cause several phenotypes including autosomal dominant (AD) Best vitelliform macular dystrophy type 2 (BVMD), AD vitreo-retino-choroidopathy (ADVIRC), and retinitis pigmentosa-50 (RP50). A rare subtype of Bestrophinopathy exists with biallelic mutations in BEST1. Its frequency is estimated to be 1/1,000,000 individuals. Here we report 6 families and searched for a genotype-phenotype correlation. All patients were referred due to reduced best-corrected visual acuity (BCVA), ranging from 0.1/10 to 3/10. They all showed vitelliform lesions located at the macula, sometimes extending into the midperiphery, along the vessels and the optic disc. Onset of the disease varied from the age of 3 to 25 years. Electrooculogram (EOG) revealed reduction in the EOG light rise in all patients. Molecular analysis revealed previously reported mutations p.(E35K);(E35K), p.(L31M);(L31M), p.(R141H);(A195V), p.(R202W);(R202W), and p.(Q220*);(Q220*) in five families. One family showed a novel mutation: p.(E167G);(E167G). All mutations were heterozygous in the parents. In one family, heterozygous children showed various reductions in the EOG light rise and autofluorescent deposits. Autosomal recessive Bestrophinopathy (ARB), although rare, can be recognized by its phenotype and should be validated by molecular analysis. Genotype-phenotype correlations are difficult to establish and will require the analysis of additional cases.