Risk of stroke development following retinal vein occlusion: A systematic review and meta-analysis.

Retinal vein occlusion (RVO) and cerebrovascular disease (CVD) share common risk factors and may be independently associated; however, the strength and nature of this association remain unclear. We conducted a systematic review and meta-analysis, informed by studies from PubMed, Scopus, EMBASE, Web of Science, and Google Scholar until January 6, 2024, aimed to clarify this relationship. Eligible studies included cohorts observing stroke incidence in RVO patients for over a year. Pooled effect estimates were calculated using random-effects models, with subgroup analyses evaluating associations between RVO types (central and branch) and stroke subtypes (ischemic and hemorrhagic). Ten cohort studies with a total of 428,650 participants (86,299 RVO patients) were included. Compared to controls, RVO patients exhibited a significantly increased risk of stroke (pooled risk ratio [RR]=1.38, 95% confidence interval (95%CI)=1.34-1.41). Subgroup analyses indicated elevated risk for both ischemic (RR=1.37, 95%CI=1.32-1.42) and hemorrhagic (RR=1.55, 95%CI=1.08-2.22) strokes in RVO patients. Additionally, both central (RR=1.50, 95%CI=1.27-1.78) and branch (RR=1.41, 95%CI=1.32-1.50) RVO were associated with stroke risk. Sensitivity analyses confirmed consistent results across various criteria, and funnel plots indicated no publication bias. RVO significantly increases the risk of both ischemic and hemorrhagic stroke, regardless of RVO type, suggesting a strong independent association between these conditions.

Case report: A case of unilateral combined central retinal vein occlusion, incomplete central retinal artery occlusion, and papillitis following a third dose of COVID-19 vaccination.

Purpose: The aim of this study was to present a case of severe visual loss due to retinal arteriovenous occlusion and papillitis in one eye following vaccination against coronavirus disease (COVID-19). Methods: A 45-year-old man undergoing treatment for hypertension had severely reduced visual acuity in the right eye 1 day after receiving a third dose of a COVID-19 vaccine manufactured by Moderna. Clinical examination showed that the best-corrected visual acuity in the right eye was counting fingers. Other findings included circumferential retinal hemorrhage, perimacular ischemic color, severe macular edema, and severe optic disc swelling, indicating the presence of central retinal vein occlusion, incomplete central retinal artery occlusion, and papillitis. Based on the possibility of post-vaccination inflammation and/or abnormal immune response, three courses of steroid pulse therapy were administered, and the visual acuity slightly improved to 20/1,000. Results: Three months after the onset of symptoms, macular edema disappeared; conversely, retinal thinning of the macula and extensive non-perfusion areas mainly on the nasal side were noted. Conclusion: The findings in this case suggest that inflammation and abnormal immune response after receiving a COVID-19 vaccination may lead to combined retinal arteriovenous occlusion and papillitis.

Efficacy and Safety of Laser Therapy on Ischemic Central Retinal Vein Occlusion: A Systematic Review and Analysis of Clinical Studies.

Many studies have evaluated different treatments for ischemic central retinal vein occlusion (CRVO). Nevertheless, improvement and complication rates vary significantly. This systematic review aimed to evaluate the efficacy of laser therapy in treating ischemic CRVO compared with a control group using other treatments. The databases of PubMed, Google Scholar, and ClinicalTrials.gov were searched using a variety of keywords, including "ischemic central retinal vein occlusion," "CRVO," "laser," and "panretinal photocoagulation." After data extraction, each study's quality was assessed using the methodological index for nonrandomized studies (MINORS) or grading of recommendations, assessment, development, and evaluation or GRADE standards. A sum of 195 abstracts were reviewed, and seven clinical trials were eventually chosen. Of these, four were prospective studies, two were randomized controlled studies, and only one was a retrospective study. The assessment of potential biases in our included studies revealed that all these studies demonstrated moderate or high quality. Two studies were selected for meta-analysis, and the results showed no significant difference in visual acuity (VA) outcomes between the treated and the control groups (P = 0.17). In the remaining five studies, laser therapy was found to be more effective at neovascular complications, with a higher rate of neovascular glaucoma (NVG), iris neovascularization (NVI), neovascularisation at disc (NVD), and retinal neovascularization in the group without laser treatments. This review suggests that laser therapy is essential in preventing neovascular complications, such as NVG, NVI, NVD, and retinal neovascularization rather than improving VA. In addition, the combination of laser photocoagulation and intravitreal injection (IVI) improved VA, but further studies are required.

The Spectrum of Papillophlebitis.

PURPOSE: Papillophlebitis is a rare condition, manifesting as CRVO in the young adults. We aim to present our experience in managing patients with papillophlebitis. METHODS: Retrospective review of patients' medical files. RESULTS: Included were seven patients with a mean presenting age of 24.86 ± 4.4 years and mean follow-up of 40.4 ± 50.5 months. No pre-existing systemic illness was reported by any patient. One patient was subsequently diagnosed to have Behçet disease and another patient was diagnosed with homozygous mutation to MTHFR C6771. On presentation, fluorescein angiograms showed diffuse vascular and optic disc leakage. Four patients presented with papillophlebitis-associated CME, for which they were treated with systemic steroids and intravitreal anti-VEGF injections. One patient showed full recovery. In 3 patients, due to the protracted course of papillophlebitis and refractory CME, adalimumab was added. All 3 patients eventually showed complete resolution of CME. Two of them eventually developed extensive peripheral capillary non-perfusion that was treated with panretinal photocoagulation. Three patients did not develop CME: In two patients, papillophlebitis resolved after a short course of prednisone while in the third patient, papillophlebitis resolved spontaneously. Mean ± SD presenting log MAR VA was 0.2 ± 0.32 and it was 0.057 ± 0.11 at the last follow-up. CONCLUSION: To the best of our knowledge, this is the first description that suggests a role for TNF-ɑ blockers in the management of patients with recalcitrant papillophlebitis and non-responsive CME. Further studies are needed in order to thoroughly investigate the molecular background of papillophlebitis and clinical outcomes associated with this class of medications.

Concomitant Two-Temporal Cilioretinal Artery Occlusion (CLRAO) With Impending Central Retinal Vein Occlusion (CRVO) in a Young Adolescent With Protein C Deficiency: A Rare Case.

We report structural changes in the retina of an adolescent diagnosed with the concomitant two temporal cilioretinal artery occlusion (CLRAO) with impending central retinal vein occlusion (CRVO) along with mild protein C deficiency. An 18-year-old girl came to the emergency room with sudden onset painless loss of vision in her right eye. On comprehensive ophthalmic examination, she had a pale superior-temporal retina with spongy macular edema corresponding to two temporal CLRAO and blurred disc margins with mild disc swelling and mild tortuosity of retinal veins all over the retina with few superficial hemorrhages in the right eye corresponding to impending CRVO. Optimal coherence tomography (OCT) showed thickening of the nerve fiber layer in the superior-temporal quadrant involving some part of the macula in the right eye. Perimetry showed a right eye visual field defect in the inferior nasal quadrant. Her coagulation profile was normal but her autoimmune profile was suggestive of mild protein C deficiency. Immediately she was started on anticoagulants. After one month, her visual acuity improved from finger counting close to face to 6/9 with treatment. Over a period of one month, retinal and OCT changes recovered with the same perimetry findings as earlier. This case shows how prompt treatment resulted in dramatic improvement in the form of good visual outcomes.

Various Ocular Manifestations in Systemic Lupus Erythematosus: A Case Series.

Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disorder with various systemic and ocular clinical manifestations. In patients with SLE, central retinal vein and artery occlusion, choroidopathy, and occlusive vasculitis are among the most significant and clinically relevant ocular manifestations, although they do not commonly occur. We present a case series of three SLE patients of different races and genders who developed ocular-related clinical features of SLE during the course of their systemic disease. The clinical outcomes of each patient were different, affecting their vision in bilateral eyes, with some patients having better visual recovery while others having permanently poor vision. These outcomes were not significantly related to the patients' age, gender, or race.

Efficacy, Safety, and Treatment Burden of Aflibercept 2 mg and Ranibizumab in Retinal Vein Occlusion: A Systematic Review and Meta-analysis.

INTRODUCTION: This systematic review and meta-analysis aimed to provide an updated evidence base for clinical decision-making by comparing the efficacy and safety of aflibercept 2 mg and ranibizumab in treating retinal vein occlusion (RVO). METHODS: A systematic search was conducted using eight databases up to December 2021. Randomized controlled trials (RCTs) and real-world studies (RWSs) comparing aflibercept and ranibizumab in patients with RVO were evaluated. The primary outcomes assessed were efficacy, number of injections administered, and adverse events. RESULTS: Three RCTs (424 patients) and 11 RWSs (1415 patients) were included. For central RVO (CRVO), RCTs demonstrated a comparable efficacy, whereas RWSs showed that mean changes from baseline in best-corrected visual acuity (BCVA) and central retinal thickness (CRT) were significantly greater with aflibercept compared to ranibizumab; the number of injections of aflibercept was fewer than that of ranibizumab in RCTs, but similar in RWSs. For branch RVO (BRVO), no statistically significant difference in efficacy between the two drugs in RCTs/RWSs was observed, with fewer injections of aflibercept at 12 months in RWSs. The safety profiles of both drugs were similar for both CRVO and BRVO. CONCLUSIONS: For CRVO, aflibercept had similar efficacy and safety profile but with fewer injections versus ranibizumab in RCTs; RWSs showed greater BCVA improvement and CRT reduction with aflibercept than ranibizumab. For BRVO, RCTs showed similar in efficacy, safety, and injection numbers for both drugs, while RWSs demonstrated that aflibercept required fewer injections at 12 months of follow-up. Overall, this study provides updated evidence for clinical decision-making in the treatment of RVO.

From Hair Loss to Vision Loss: Minoxidil-Associated CRVO in a Young Female.

Introduction: Central retinal vein occlusion (CRVO) is a common retinal vascular disorder that is most often seen in older adults and individuals with vascular risk factors. Case Presentation: We report a case of CRVO with cystoid macular edema (CME) in a young, otherwise healthy patient taking minoxidil for hair loss. The patient had no known vascular risk factors, and a comprehensive coagulability workup was negative. The CRVO with CME resolved without intervention upon cessation of minoxidil. Conclusion: Possible mechanisms for minoxidil-associated retinal vascular disorders are explored. Thorough medication histories and the consideration of possible adverse drug events in patients without traditional risk factors are recommended.

A Multicentric Cross-Sectional Observational Study to Analyze the Effects of Moderate High Altitude on Ocular Health.

Objectives: Prolonged exposure to hypoxia at high altitudes can affect the eyes through various mechanisms, culminating in reduced vision. Multiple studies on this subject have focused on the ocular effects of high altitude on intraocular pressure (IOP), corneal thickness, and the retina. However, there is a lack of literature on the effects of 'moderate high altitude (2000-3000 m)' on the eyes. Hence, we designed our study to determine the impact of moderate high altitude on the eyes. Methods: We recruited two age and gender-matched groups of 250 research participants, each at moderate high altitude (Group A) and plains (Group B), respectively, to determine the effects of moderate high altitude on ocular health, if any. Results: We found a statistically significant increase in dry eye in Group A. We also found four cases of central serous retinopathy and one case of central retinal vein occlusion in Group A. There was no statistically significant change in visual acuity, IOP, or central corneal thickness. Conclusion: There is a vast lacuna in the literature on this particular altitude range, and further studies are required to confirm our findings. It is also recommended that lubricant eye drops be advised as a standard prophylactic measure for all individuals traveling and staying at moderate high altitude.

Combined Central Retinal Artery Occlusion (CRAO) and Central Retinal Vein Occlusion (CRVO) in a Celiac Disease Patient: A Case Report.

Celiac disease (CD) is a digestive disorder caused by an abnormal immune reaction to gluten, leading to severe malabsorption syndrome. Central retinal vein occlusion (CRVO) was reported in a couple of cases worldwide in patients with this disease entity. Herein, we are reporting a rare case of combined central retinal vein and artery occlusion in a young female celiac disease patient presented with a counting finger vision at six feet and improved to 20/60 vision after conservative management.

Changes in wider field swept-source OCT angiography vascular metrics with anti-vascular endothelial growth factor therapy in central retinal vein occlusion.

PURPOSE: To investigate the impact of anti-VEGF therapy on vascular metrics in eyes with macular edema secondary to central retinal vein occlusion (CRVO) using wider field swept-source OCT angiography (WF SS-OCTA). METHODS: We included 23 eyes with macular edema associated with non-ischemic CRVO from 22 patients treated with anti-VEGF therapy (median number of injections: 5 [2-9]). Changes in vessel density (VD), vessel skeletonized density (VSD), and foveal avascular zone (FAZ) parameters were measured using WF SS-OCTA. Visual acuity (VA) and central subfield thickness (CST) were also measured. RESULTS: Median CST decreased significantly from 369 µm (305-531) to 267 µm (243-300, p < 0.001). VD and VSD parameters in 12 × 12 mm images showed significant reductions. For instance, VSD in the whole retina decreased from a median of 13.37 (11.22-13.74) to 11.29 (9.36-12.97, p = 0.013). Additionally, a significant increase in FAZ circularity was found, suggesting improved microvascular integrity. Significant inverse correlations were found between the number of anti-VEGF injections and all VSD and VD parameters on the 12 × 12 mm images (p < 0.05). Notably, the reductions in VSD and VD on 12 × 12 mm angiograms in the deep capillary plexus (DCP) after each injection significantly correlated with increased logMAR VA (worse VA). CONCLUSION: Anti-VEGF therapy in CRVO patients not only mitigates macular edema but also alters the overall microvascular morphology and functionality as revealed by WF SS-OCTA.

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.

Visit Adherence and Visual Acuity in Study of COmparative Treatments for REtinal Vein Occlusion 2 (SCORE2).

PURPOSE: We quantify the association between visit adherence and visual acuity (VA) in retinal vein occlusions (CRVO). METHODS: The SCORE2 protocol included a visit every 4 weeks (every 28-35 days) during the first year. Visit adherence was measured as follows: number of missed visits, average and longest (avg and max days) visit interval, and average and longest (avg and max missed days) and unintended visit interval. Avg and max missed days were categorized as on time (0 days), late (>0-60 days), and very late (>60 days). The primary outcome was a change in the Early Treatment Diabetic Retinopathy Study (ETDRS) visual acuity letter score (VALS) between baseline study visit and last attended visit during Year 1, using multivariate linear regression models controlling for numerous demographic and clinical factors. RESULTS: After adjustment, for each visit missed, patients lost 3.0 letters (95% CI: -6.2, 0.2) of vision (p = .07). On average, the 48 patients who missed at least 1 visit lost 9.4 letters (95% CI: -14.4, -4.3, p < .001) of vision after adjustment. Average days and maximal intervals between visits were not associated with changes in VALS (p > .22) for both comparisons. However, when a visit was missed, the average missed days between missed visits and the max missed interval were both associated with loss of VALS (both variables: 0 days missed as reference, late [1-60 days] -10.8 letters [95% CI: -16.9, -4.7], very late [>60 days] -7.3 letters [95% CI: -14.5, -0.2]; p = .003 for both). CONCLUSIONS: Visit adherence is associated with VALS outcomes in CRVO patients.

Real-world effectiveness of intravitreal dexamethasone implants - Comparison between eyes eligible and ineligible for clinical trials and their associated outcomes.

BACKGROUND: Concerns about the generalizability of pivotal randomized controlled trials (pRCTs) findings have been raised. We aimed to compare intravitreal dexamethasone implants' (IDIs) effectiveness for diabetic macular edema (DME) and central retinal vein occlusion (CRVO), between eyes eligible and ineligible for pRCTs. METHODS: This retrospective cohort study analyzed Taiwan's Chang Gung Research Database, including DME or CRVO eyes initiating IDIs during 2015-2020. We classified all treated eyes as eligible or ineligible for pRCTs following major selection criteria of the MEAD and GENEVA trials, and evaluated three-, six-, and twelve-month changes in central retinal thickness (CRT) and visual acuity (VA) after initiating IDIs. RESULTS: We included 177 IDI-treated eyes (DME: 72.3%; CRVO: 27.7%), of which 39.8% and 55.1% were ineligible for DME and CRVO pRCTs, respectively. LogMAR-VA and CRT changes at different times were comparable in DME eyes eligible (LogMAR-VA difference: 0.11 to 0.16; CRT difference: -32.7 to -96.9 µm) and ineligible (LogMAR-VA difference: -0.01 to 0.15; CRT difference: -54.5 to -109.3 µm) for the MEAD trial. By contrast, CRVO eyes ineligible for the GENEVA trial had greater LogMAR-VA changes (0.37 ~ 0.50) than those eligible (0.05 ~ 0.13), with comparable CRT reductions (eligible eyes: -72.3 to -106.4 µm; ineligible eyes: -61.8 to -110.7 µm) (all p-values <0.05 of the mean differences between eligible and ineligible CRVO eyes for all follow-ups). CONCLUSIONS: IDIs had similar VA and CRT outcomes among DME eyes, regardless of pRCT-eligibility. However, among CRVO eyes, those ineligible for pRCTs showed greater deterioration in VA than those eligible.

Real-World Management of Macular Edema Secondary to Retinal Vein Occlusion with Intravitreal Aflibercept: 24-month Results from the AURIGA Observational Study.

INTRODUCTION: AURIGA is the largest real-world study to date to evaluate intravitreal aflibercept (IVT-AFL) treatment of diabetic macular edema or macular edema secondary to retinal vein occlusion (RVO) in routine clinical practice. Here, we report the 24-month outcomes in the RVO cohort from France, Germany, Italy, and Taiwan. METHODS: AURIGA (NCT03161912) was a prospective observational study. Eligible patients with RVO were enrolled for whom the decision to treat with IVT-AFL had already been made by the attending physician. Patients were treated with IVT-AFL for up to 24 months at physician discretion according to local practice. The primary endpoint was mean change in visual acuity (VA; Early Treatment Diabetic Retinopathy Study [ETDRS] letters) from baseline to month (M) 12. All statistical analyses were descriptive. RESULTS: In 554 treatment-naïve and 65 previously treated patients with RVO, the respective mean (95% confidence interval) change in VA from baseline was + 12.5 (10.8, 14.3) and + 7.9 (3.3, 12.6) letters by M12 and + 11.4 (9.4, 13.3) and + 4.4 (- 0.6, 9.5) letters by M24 (baseline mean ± standard deviation: 51.0 ± 21.9 and 51.9 ± 20.4 letters); 44.0% of treatment-naïve and 27.9% of previously treated patients reported ≥ 15-letter gains by M24. By M24, the mean change in central retinal thickness from baseline was - 247 (- 267, - 227) µm in treatment-naïve patients and - 147 (- 192, - 102) µm in previously treated patients. From baseline to M6, M12, and M24, treatment-naïve patients received a total of 4.0 ± 1.3, 5.5 ± 2.5, and 6.9 ± 4.2 injections, respectively, and previously treated patients received 3.8 ± 1.5, 5.0 ± 2.2, and 6.3 ± 3.7 injections, respectively. The safety profile of IVT-AFL was consistent with that of previous studies. CONCLUSIONS: In AURIGA, patients with RVO experienced clinically relevant functional and anatomic improvements following IVT-AFL treatment in routine clinical practice. These improvements were largely maintained in treatment-naïve patients over the 24-month study despite the decreasing treatment frequency, suggesting long-term durability of IVT-AFL treatment outcomes. Infographic available for this article. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT03161912 (May 19, 2017). INFOGRAPHIC.

Central retinal vein occlusion : an uncommon complication in sarcoidosis.

Sarcoidosis is a multi-system granulomatosis of unknown etiology, defined by the presence of epithelioid and gigantocellular granulomas, without caseous necrosis. Ocular sarcoidosis manifests mainly as bilateral granulomatous anterior uveitis. Occlusion of the central retinal vein in sarcoidosis is a rare manifestation, which is the particularity of our observation. We report the case of a patient presenting with unilateral central retinal vein occlusion associated with granulomatous anterior uveitis on the same side. Systemic manifestations and further investigations led to the diagnosis of sarcoidosis.

Acute onset of decreased vision after violently vomiting: A case report.

PURPOSE: Reported cases of central retinal vein occlusion (CRVO) associated with violent vomiting are scarce all over the world. In this case, we reported a male patient with an acute decreased vision right after violently vomiting. METHODS: A 55-year-old male patient found himself with a sudden, painless decline in visual acuity in his left eye (LE) after violently vomiting due to alcohol consumption for 1 day. His best corrected visual acuity (BCVA) was 20/2000 in the LE. He has a history of moderate myopia in both eyes and well-controlled hypertension. After evaluating multimodal images, non-ischemic CRVO retinopathy was diagnosed. RESULTS: During the follow-up period, the patient's BCVA in the LE improved to 20/40 and the ocular examination data confirmed that the previous abnormal performances were nearly back to normal without any treatment. CONCLUSION: Non-ischemic CRVO retinopathy following violent vomiting suggests that vomiting-related mechanisms like Valsalva maneuvers and dehydration may contribute to CRVO through effects on ocular blood flow and thrombosis.

Foveal Thickness Fluctuations in Anti-VEGF Treatment for Central Retinal Vein Occlusion.

Purpose: The aim of this study was to examine the effects of foveal thickness (FT) fluctuation (FTF) on 2-year visual and morphological outcomes of eyes with central retinal vein occlusion (CRVO) undergoing anti-VEGF treatment for recurrent macular edema (ME) based on a pro re nata regimen. Design: Retrospective, observational case series. Participants: We analyzed 141 treatment-naive patients (141 eyes) with CRVO-ME at a multicenter retinal practice. Methods: We assessed FT using OCT at each study visit. Patients were divided into groups 0, 1, 2, and 3 according to increasing FTF. Main Outcome Measures: We evaluated the logarithm of the minimal angle of resolution (logMAR) best-corrected visual acuity (BCVA), the length of the foveal ellipsoid zone (EZ) band defect measured using OCT, and the association of FTF with VA and EZ band defect length. Results: The mean baseline logMAR BCVA and FT were 0.65 ± 0.52 (Snellen equivalent range: 20/20-20/2000) and 661.1 ± 257.4 µm, respectively. The mean number of anti-VEGF injections administered was 5.6 ± 3.6. At the final examination, the mean logMAR BCVA and FT values were significantly improved relative to the baseline values (both P < 0.01). During the observation, BCVA longitudinally improved in Groups 0 and 1, remained unchanged in Group 2, and worsened in Group 3. Likewise, the length of the foveal EZ band defect did not increase in Group 0; however, it gradually increased in Groups 1, 2, and 3. Foveal thickness fluctuation was significantly and positively associated with the logMAR BCVA and length of the foveal EZ band defect at the final examination (P < 0.01). The final logMAR BCVA of patients developing neovascular complications was 1.27 ± 0.72 (Snellen equivalent range: 20/50-counting fingers), which was significantly poorer than that of patients without complications (P < 0.001). There was no significant difference in the neovascular complication rate among the FTF groups (P = 0.106, Fisher exact test). Conclusions: In eyes receiving anti-VEGF treatment for CRVO-ME, FTF can longitudinally impair the visual acuity and foveal photoreceptor status during the observation period, thus influencing the final outcomes. However, neovascular complications, which would also lead to a poor visual prognosis, may not be associated with FTF. Financial Disclosures: The authors have no proprietary or commercial interest in any materials discussed in this article.

Hyperviscosity Syndrome Induced Bilateral Visual and Auditory Impairment in Therapy Resistant Waldenström Macroglobulinemia with MYD88 and CXCR4 Mutations.

Hyperviscosity syndrome (HVS) is an emergent complication of Waldenström macroglobulinemia (WM) characterized by visual, neurologic, and rarely auditory impairment. We report a 69-year-old female with MYD88 and CXCR4-mutant WM who developed HVS resulting in bilateral blindness and deafness associated with neurologic manifestations including confusion, severe generalized weakness, and imbalance. Ophthalmologic evaluation revealed bilateral central retinal vein occlusion (CRVO), diffuse retinal hemorrhages, macular edema, and serous macular detachments (SMD). Magnetic resonance imaging of the brain showed bleeding in the inner ears. Management was challenging as her WM was resistant to systemic therapies including bendamustine + rituximab (BR) and rituximab + bortezomib + dexamethasone (RVD). Bruton's tyrosine kinase inhibitors could not be used initially due to ongoing lower gastrointestinal bleeding. She required five total sessions of plasma exchange and was finally initiated on zanubrutinib, achieving a partial response. She also received intravitreal bevacizumab with rapid resolution of the retinal hemorrhages but with little improvement of the SMD. She had partial restoration of her hearing in the right ear and only slight improvement in her bilateral visual deficits. The management of HVS in frail, elderly patients with therapy-resistant WM can be challenging. In these cases, plasma exchange is required until an effective systemic therapy can be safely instituted. Genomic profiling is important in the management of WM as it can predict treatment resistance and guide therapeutic decisions.

Severe Macular Ischemia Is Associated with a Poor Visual Prognosis and Serious Complications in Eyes with Central Retinal Vein Occlusion.

PURPOSE: This study aims to investigate the factors influencing post-treatment visual acuity (VA) in patients with central retinal vein occlusion (CRVO) with macular edema (ME). METHODS: The subjects of this study were patients who visited our clinic from May 2013 to July 2019 and who could be followed up with for at least 12 months. Cases with hemi CRVO were excluded from this study. Factors considered in the evaluation of visual prognosis at the 12 months included initial best-corrected VA, central subfoveal thickness, CRVO subtype (nonischemic, ischemic, or converted from nonischemic to ischemic), time taken for the first treatment, number of anti-vascular endothelial growth factor agent injections, structural changes in the inner and outer retinal layers, and the presence of macular ischemia in a multiple regression analysis. RESULTS: There were 41 patients with 41 eyes, 27 males and 14 females. The mean age of the patients was 70.5 ± 12.2 (mean ± standard deviation) years. The mean VA was 0.544 ± 0.576, 0.456 ± 0.568, and 0.586 ± 0.665 at the initial visit, 12 months later, and time of last observation, respectively. There were no significant differences in VAs observed between the baseline, month 12, and final visit. Multiple regression analysis revealed that the external limiting membrane score at month 12 (p = 0.030), the VA at initial visit (p < 0.001), and the presence of severe macular ischemia (p < 0.001) were the key factors associated with VA at month 12. Moreover, severe macular ischemia was identified as the only factor affecting decimal VA less than 20/200 at the last observation (p = 0.0092). CONCLUSIONS: Severe macular ischemia is strongly linked to a poor visual prognosis in patients with ME associated with CRVO.