Real-World Experience With Teprotumumab in Patients With Dysthyroid Optic Neuropathy.

BACKGROUND: Teprotumumab, an insulin-like growth factor I receptor inhibitory antibody, improved proptosis, diplopia, inflammatory signs/symptoms, and quality of life in patients with active thyroid eye disease (TED) in clinical trials. The trials excluded patients with dysthyroid optic neuropathy (DON). Recently, many case reports and case series have reported the successful use of teprotumumab to treat DON. Here, we review the data from published cases and our clinical experience in treating patients having DON with teprotumumab. METHODS: A literature search was conducted of patients with DON treated with teprotumumab from January 2020 through September 2022. Data from DON patients from the authors' (M.A.T. and C.A.B.) clinical practice were included. Primary outcome measure was mean (SD) improvements for visual acuity, color vision, and visual fields. Improvements in proptosis and clinical activity score (CAS) and diplopia were compared before and after teprotumumab administration. RESULTS: Ten observational studies/case reports were identified along with 2 patients in our practice. In all, there were 24 active TED patients with DON (37 eyes) who were treated with teprotumumab. Mean (SD) age was 66.5 (13.6) years and 13 (54%) were females, disease duration ranged from 2 months to >15 years. 22/24 patients had none, minimal improvement or progression of visual loss with intravenous/oral corticosteroids, orbital decompression (n = 9), and orbital radiation (n = 2). There were 2 patients who received teprotumumab as the only therapy. Overall, 88% (21/24) reported improvement in visual acuity after teprotumumab and in 75% (18/24), improvement in vision was observed after just 2 infusions of teprotumumab. Three eyes had decompression surgery in close proximity to teprotumumab infusions and were excluded from analyses. Mean (SD) improvement in visual acuity was 3.73 lines (SD 3.74), range 2-15 lines in 33 eyes. The mean (SD) improvement in the mean deviation on visual field testing in 15 eyes was 5.6 db (3.0 db). Mean (SD) improvement in proptosis was 4.37 mm (SD: 2.11) (20 patients, 32 eyes); and clinical activity score: mean reduction of 5.1 (1.3) for 18 patients. Teprotumumab was well tolerated in all but one patient. Adverse events reported included fatigue, dysgeusia, hearing loss, nausea, hyperglycemia, and muscle spasms. CONCLUSIONS: Teprotumumab is an effective treatment for DON in our experience and in published cases in whom treatment with steroids, surgery, or orbital radiation was unsuccessful.

Restoration of vision by combined experimental antithymocyte therapy, and orbital radiation with high-dose steroids for severe, acute, steroid-refractory, congestive thyroid orbitopathy.

PURPOSE: We report diagnostic and therapeutic dilemmas in the difficult case of compressive optic neuropathy with severe visual acuity and visual field loss with subsequent visual recovery in both eyes, in a patient with Graves' orbitopathy (GO) by a combination of experimental antithymocyte therapy, orbital radiotherapy with high-dose steroids. METHODS: A 72-year-old man presented with severe vision loss in both eyes. The visual symptoms had appeared over a year before the GO diagnosis. He was initially misdiagnosed with neuroborreliosis and optic neuritis based on brain and orbital magnetic resonance imaging. There was no exophthalmos. The ophthalmological examination included visual acuity, visual field, tonometry in primary and upgaze eye position, optical coherence tomography (OCT), pattern electroretinogram (PERG), pattern, and flash visual evoked potentials (PVEP and FVEP). The patient received experimental therapy with ATG, followed by high-dose of intravenous steroids and orbital radiotherapy. RESULTS: Delayed VEP peaks became shorter after treatment. After systemic and local therapy lowering of intraocular pressure was achieved. Abnormal PERG has been found three months before ganglion cells atrophy was detected in OCT. Visual acuity and visual field improvement occurred in both eyes after therapy, despite partial left optic nerve atrophy. The patient regained full decimal visual acuity (1.0 right from as poor as 0.3  to 1.0 in the right eye and from hand movements to 0.9 in the left. Severe visual field loss with advanced absolute scotomata has improved to slight relative scotomata. The duration of follow-up time after the treatment was 4 months. CONCLUSIONS: Intensive treatment of steroid-resistant Graves' orbitopathy (GO) may prevent total optic nerve atrophy. Despite severely advanced optic neuropathy, this report emphasizes the necessity of therapy even with nearly complete visual function loss hence there is always a possibility to regain full visual acuity and visual field. Patients with tense orbital septum may not present with significant exophthalmos, thus delaying the correct diagnosis of orbitopathy. A supporting sign of GO was the difference in intraocular pressure in the primary and upgaze eye positions. Electrophysiological examinations are helpful in the diagnosis and monitoring of GO therapy. To our knowledge, this is the first report of this kind presenting visual function restoration and structural recovery in a patient with advanced optic neuropathy in GO.

Vitamin B3 Supplementation for Optic Neuropathies: A Comprehensive Review.

Optic neuropathies, such as glaucoma, are some of the leading causes of irreversible blindness worldwide. There has been a lot of research for potential therapies that could attenuate and even reduce the impact of the pathological pathways that lead to the loss of retinal ganglion cells (RGCs). In recent years, vitamin B3 (nicotinamide) has gained some interest as a viable option for these neurodegenerative diseases due to its fundamental role in enhancing the mitochondria metabolism of the RGCs. This review focuses on elucidating the impact of vitamin B3 on retinal cells, especially when in a dysfunctional state like what happens in optic neuropathies, especially glaucoma. This review also summarizes the existing and future research on the clinical effects of vitamin B3 in these optic neuropathies, and determines appropriate recommendations regarding its dosing, efficacy, and eventual side effects.

Macular blood flow changes in dysthyroid optic neuropathy after high-dose intravenous pulse methylprednisolone (IVMP).

OBJECTIVE: To investigate the changes in macular vessel density (VD) of the superficial layer of retina (SLR) and deep layer of retina (DLR) in dysthyroid optic neuropathy (DON) after high-dose intravenous pulse methylprednisolone (IVMP). MATERIALS AND METHODS: Eighteen DON patients (29 eyes) who completed high-dose IVMP and 16 healthy individuals (32 eyes) were enrolled in this study. Optical coherence tomography angiography (OCTA) image analysis and comprehensive ophthalmic examinations were performed, including the SLR macular whole-image VD (SLR-mwiVD) and DLR-mwiVD, best-corrected visual acuity (BCVA), the mean deviation of visual field (VF-MD), pattern standard deviation of visual field (VF-PSD) and the other parameters. RESULTS: The SLR-mwiVD (41.39 ± 4.71 vs. 48.13 ± 3.68, p < 0.001) and DLR-mwiVD (40.77 ± 5.85 vs. 49.14 ± 7.02, p < 0.001) were decreased in DON compared to control eyes. After IVMP, visual function parameters were improved, and SLR-mwiVD (49.41 ± 3.18, p < 0.001) and DLR-mwiVD (50.41 ± 4.04, p < 0.001) were increased in the DON group compared to pretreatment. The increased SLR-mwiVD and DLR-mwiVD were significantly correlated with improvements in BCVA (Log MAR: from 0.62 ± 0.49 to -0.01 ± 0.03, p < 0.001), VF-MD (from - 6.89 ± 2.89 dB to - 1.75 ± 1.29 dB, p < 0.001) and VF-PSD (from 4.38 ± 2.52 dB to 2.32 ± 1.64 dB, p < 0.001). CONCLUSION: The increase in macular VD was significantly correlated with the improvement in visual function in DON after IVMP. Macular VD changes on OCTA may be a useful indicator for the response in DON after IVMP.

Outcomes of systemic bevacizumab in radiation-induced optic neuropathy, case series.

PURPOSE: Optic neuropathy is a rare, delayed complication after radiation with no universally accepted treatment modality. We report the outcomes of 6 patients with radiation-induced optic neuropathy (RION) who were treated with systemic bevacizumab. METHODS: This is a retrospective series of 6 cases of RION, treated with intravenous (IV) bevacizumab. "Improved" or "worse" visual outcomes were defined as a change in best corrected visual acuity of ≥ 3 Snellen lines. Otherwise, the visual outcome was noted as "stable". RESULTS: In our series, RION was diagnosed 8 to 36 months after radiotherapy. IV bevacizumab was initiated as treatment within 6 weeks of the onset of visual symptoms in 3 cases and after 3 months in the other cases. Although no improvement in visual function was observed, stabilization of vision was noted in 4 of the 6 cases. In the other 2 cases, the level of vision declined from counting fingers to no light perception. In 2 cases, bevacizumab treatment was discontinued prior to completion of the planned course due to renal stone formation or worsening of renal disease. One patient developed ischemic stroke 4 months after bevacizumab completion. CONCLUSION: Systemic bevacizumab may stabilize vision in some patients with RION, though the limitations of our study do not allow us to draw this conclusion definitively. Therefore, the risks and potential benefits of using IV bevacizumab should be considered in each individual case.

Outcomes of Patients With Dysthyroid Optic Neuropathy Treated With Intravenous Corticosteroids and/or Orbital Decompression Surgery: A Systematic Review and Meta-analysis.

CONTEXT: The level of evidence is low for the treatment of patients with dysthyroid optic neuropathy (DON) and there is no consensus on the treatment of DON with intravenous high-dose glucocorticoids (ivGC) or direct surgical decompression. OBJECTIVE: To compare the efficacy of glucocorticoid treatment and orbital decompression (OD) in DON. DATA SOURCES: PubMed, EMBASE, and Cochrane Library were searched along with other sources. STUDY SELECTION: A total of 17 studies met the inclusion criteria. DATA EXTRACTION: Standard methodological guidance of the Cochrane Handbook was used and data were independently extracted by multiple observers. The primary outcomes were the improvement of best corrected visual acuity (ΔBCVA). Secondary outcomes were proptosis reduction, change in diplopia, visual field defects, and intraocular pressure (IOP). DATA SYNTHESIS: The ΔBCVA in the ivGC + OD group was improved 0.26 LogMAR more than in the ivGC group (P = .007). The ΔBCVA in the OD group was better than in the ivGC group (P = .008). Posttreatment proptosis in the ivGC + OD and OD groups were improved further by 3.54 mm and 3.00 mm, respectively, than in the ivGC group (P < .01). The mean deviation (MD) in the ivGC + OD group was improved by an additional 5.33 dB than in the ivGC group (P = .002). The IOP in the ivGC + OD group was improved further than in the ivGC group (P = .03). CONCLUSIONS: Based on the results of the present meta-analysis, OD or ivGC + OD may be more effective in improving BCVA and MD and reducing proptosis compared with ivGC. Compared with ivGC alone, ivGC + OD is more effective in improving IOP than ivGC. Although this study improves the hierarchy of evidence in the treatment of DON, additional randomized controlled trials are needed to confirm this conclusion.

Optic neuropathy after COVID-19 vaccination: a report of two cases.

PURPOSE: We report two cases of optic nerve pathology after the administration of the Pfizer-BioNTech and AstraZeneca-Oxford COVID-19 vaccines, respectively, and describe the implications for management of post-vaccination central nervous system (CNS) inflammation. CASE REPORTS: A 69-year-old woman presented with bilateral optic nerve head oedema, 16 days after the second dose of the Pfizer-BioNTech vaccine. She was diagnosed with post-vaccination CNS inflammatory syndrome and was treated for five days with intravenous methylprednisolone at a dose of 1 gram per day. Her optic disc swelling improved, and her vision stabilised. A 32-year-old woman presented six days after her first dose of the AstraZeneca-Oxford vaccine with two days of sudden onset of progressive blurring of vision in her left eye. Posterior segment examination revealed left optic disc swelling, and an MRI of the brain, orbit, and cervical spine was significant for left optic nerve enhancement. The patient was diagnosed with a unilateral post-vaccination optic neuritis. She was treated with a three-day course of intravenous methylprednisolone followed by oral prednisone. Her optic disc swelling and visual field improved, and she recovered 6/6 vision. CONCLUSIONS: Clinicians and patients should be aware of the potential for post-vaccination CNS inflammatory syndromes associated with COVID-19 vaccine administration. Neuroimaging and cerebrospinal fluid analysis may aid in the diagnosis of the cause of vision loss. Further studies are needed to evaluate the spectrum and frequency of optic nerve involvement associated with COVID-19 vaccination.

Successful Treatment of Optic Neuropathy Associated with Sphenoid Sinus Aspergillosis.

PURPOSE: We report a case of optic neuropathy related to sphenoid sinus aspergillosis which showed good visual recovery with surgery and medical antifungal treatment. METHODS: Observational case study Case Presentation A 62-year-old man presented with decreased visual acuity in the right eye for 3 weeks. His visual acuity was counting fingers in the right eye and 20/20 in the left eye. Relative afferent pupillary defects were detected in the right eye. Optic neuropathy related to invasive fungal sphenoid sinusitis was suspected via radiologic evaluation. Endoscopic sinus surgery was performed and histopathological examination revealed aspergillosis. Amphotericin B combined with ceftriaxone and metronidazole was started. After the fungal culture results were positive for the Aspergillus species, amphotericin B was changed to voriconazole. At 1 month after surgery, visual acuity improved to 20/25. CONCLUSION: Appropriate radiologic evaluation can be helpful when optic neuropathy associated with a fungal infection is suspected, and timely surgical and medial treatment should be considered.

Diversified Treatment Options of Adult Stem Cells for Optic Neuropathies.

Optic neuropathies refer to a group of ocular disorders with abnormalities or dysfunction of the optic nerve, sharing a common pathophysiology of retinal ganglion cell (RGC) death and axonal loss. RGCs, as the retinal neurons in the central nervous system, show limited capacity in regeneration or recovery upon diseases or after injuries. Critically, there is still no effective clinical treatment to cure most types of optic neuropathies. Recently, stem cell therapy was proposed as a potential treatment strategy for optic neuropathies. Adult stem cells, including mesenchymal stem cells and hematopoietic stem cells, have been applied in clinical trials based on their neuroprotective properties. In this article, the applications of adult stem cells on different types of optic neuropathies and the related mechanisms will be reviewed. Research updates on the strategies to enhance the neuroprotective effects of human adult stem cells will be summarized. This review article aims to enlighten the research scientists on the diversified functions of adult stem cells and consideration of adult stem cells as a potential treatment for optic neuropathies in future clinical practices.

Risk of Ocular Adverse Events With Taxane-Based Chemotherapy.

Importance: Taxane-based chemotherapy agents, such as docetaxel and paclitaxel, are used for treating a wide range of cancers. Although much has been published on adverse events related to taxanes, data on ocular outcomes with these very important drugs are scant. Objective: To quantify the risk of 3 mutually exclusive ocular adverse events of epiphora, cystoid macular edema (CME), and optic neuropathy with taxane-based chemotherapy agents by undertaking a large pharmacoepidemiologic study. Design, Setting, and Participants: This retrospective cohort study design used a private health-claims database from the US that captures health information of more than 150 million enrollees. The study team created a cohort of new users of women with cancer who were taking taxane-based chemotherapy (docetaxel or paclitaxel) and new users of tamoxifen as controls. Study members were observed to the first incidence of each of the 3 mutually exclusive outcomes. An analysis of taxane-only users was also undertaken. Exposure: Tamoxifen (unexposed) and taxanes (ie, paclitaxel and docetaxel) as the exposed. Main Outcomes and Measures: First diagnosis of (1) epiphora, (2) cystoid macular edema (CME), or (3) optic neuropathy ascertained using International Statistical Classification of Diseases and Related Health Problems, Ninth Revision or International Statistical Classification of Diseases and Related Health Problems, Tenth Revision. Results: Among the 18 219 users in the epiphora analysis and optic neuropathy analysis, there were 1824 taxane users (paclitaxel and docetaxel) (age, mean [SD], 62.1 [12.7] years) and 16 395 tamoxifen users (age, mean [SD], 54.6 [12.8] years), respectively. The crude hazard ratio (HR) for epiphora was 5.55 (95% CI, 2.99-10.29) and adjusted HR was 5.15 (95% CI, 2.79-9.54). For optic neuropathy, the crude HR was 4.43 (95% CI, 1.10-17.82) and the adjusted HR was 4.44 (95% CI, 1.04-18.87). Among the 18 433 users in the CME analysis, there were 1909 taxane users (paclitaxel and docetaxel) (age, mean [SD], 62.5 years) and 16 524 tamoxifen users (age, mean [SD], 54.6 years). The crude HR for CME comparing taxane users with tamoxifen users was 1.37 (95% CI, 0.72-2.60) and adjusted HR was 1.33 (95% CI, 0.70-2.53). The HRs for epiphora and CME in the taxane cohort during the time of exposure compared with the period prior to use of the drugs were 2.86 (95% CI, 1.11-7.39) and 2.27 (95% CI, 0.68-7.54), respectively. Conclusions and Relevance: In a cohort of women who were using taxane chemotherapy agents, there was an association with elevated risk for epiphora, CME, and optic neuropathy. Ophthalmologists and oncologists should be aware of these adverse events in women with breast cancer who receive these drugs.

A Small Natural Molecule S3 Protects Retinal Ganglion Cells and Promotes Parkin-Mediated Mitophagy against Excitotoxicity.

Glutamate excitotoxicity may contribute to retinal ganglion cell (RGC) degeneration in glaucoma and other optic neuropathies, leading to irreversible blindness. Growing evidence has linked impaired mitochondrial quality control with RGCs degeneration, while parkin, an E3 ubiquitin ligase, has proved to be protective and promotes mitophagy in RGCs against excitotoxicity. The purpose of this study was to explore whether a small molecule S3 could modulate parkin-mediated mitophagy and has therapeutic potential for RGCs. The results showed that as an inhibitor of deubiquitinase USP30, S3 protected cultured RGCs and improved mitochondrial health against NMDA-induced excitotoxicity. Administration of S3 promoted the parkin expression and its downstream mitophagy-related proteins in RGCs. An upregulated ubiquitination level of Mfn2 and protein level of OPA1 were also observed in S3-treated RGCs, while parkin knockdown resulted in a major loss of the protective effect of S3 on RGCs under excitotoxicity. These findings demonstrated that S3 promoted RGC survival mainly through enhancing parkin-mediated mitophagy against excitotoxicity. The neuroprotective value of S3 in glaucoma and other optic neuropathies deserves further investigation.

Erythropoietin in Optic Neuropathies: Current Future Strategies for Optic Nerve Protection and Repair.

Erythropoietin (EPO) is known as a hormone for erythropoiesis in response to anemia and hypoxia. However, the effect of EPO is not only limited to hematopoietic tissue. Several studies have highlighted the neuroprotective function of EPO in extra-hematopoietic tissues, especially the retina. EPO could interact with its heterodimer receptor (EPOR/ßcR) to exert its anti-apoptosis, anti-inflammation and anti-oxidation effects in preventing retinal ganglion cells death through different intracellular signaling pathways. In this review, we summarized the available pre-clinical studies of EPO in treating glaucomatous optic neuropathy, optic neuritis, non-arteritic anterior ischemic optic neuropathy and traumatic optic neuropathy. In addition, we explore the future strategies of EPO for optic nerve protection and repair, including advances in EPO derivates, and EPO deliveries. These strategies will lead to a new chapter in the treatment of optic neuropathy.

The Role of NAD+ and Nicotinamide (Vitamin B3) in Glaucoma: A Literature Review.

Glaucoma is a collection of irreversible optic neuropathies which, if left untreated, lead to severe visual field loss. These diseases are a leading cause of blindness across the globe and are estimated to affect approximately 80 million people, particularly women and people of Asian descent (Quigley HA, Broman AT. 2006. Br J Ophthalmol 90: 262-267). This represents a major burden on healthcare systems worldwide. Recently, there has been increasing interest in the potential of nicotinamide (vitamin B3) as a novel option in the management of glaucoma. This review aims to analyse the currently available literature to determine whether there is evidence of an association between nicotinamide adenine dinucleotide (NAD+) and glaucomatous optic neuropathy, and whether nicotinamide has the potential to prevent or reverse these effects. The literature showed a strong connection between reduced NAD+ levels and retinal ganglion cell dysfunction through multiple different studies. There is also evidence of the positive effect of nicotinamide supplementation on retinal ganglion cell function in models of mouse glaucoma and in a study involving humans. Based on the literature findings, a recommendation has been made that more research into the efficacy, appropriate dosing, and potential side effects of nicotinamide supplementation is needed before it can be definitively determined whether it is appropriate for widespread prophylactic and therapeutic use against glaucoma in humans.

Management of patients with dysthyroid optic neuropathy treated with intravenous corticosteroids and/or orbital decompression surgery.

PURPOSE: To assess the characteristics and long-term outcomes of adult patients with dysthyroid optic neuropathy (DON) who underwent orbital decompression surgery and/or received intravenous (IV) methylprednisolone. METHODS: Retrospective chart review of 98 eyes of 49 patients who were diagnosed and treated with bilateral DON between 2007 and 2018 at the Department of Ophthalmology and Optometry and Oral and Maxillofacial Surgery of the Medical University of Vienna. RESULTS: The mean follow-up period was 4.1 ± 2.7 years. The most common presenting symptoms were eyelid and periorbital swelling (45%) representing active inflammation. Upgaze restriction was the most common clinical finding (73%). At time of diagnosis, the mean clinical activity score was 4 ± 1/4 ± 1 (right/left eye, respectively). Sixty-three percent (31/49) of the patients were treated both with IV methylprednisolone and underwent orbital decompression surgery, 22% (11/49) were treated with IV methylprednisolone alone and 14% (7/49) underwent surgical decompression only. Seventy-one percent (30/42) of the patients underwent 3-wall decompression. The mean reduction of proptosis in patients treated with both IV methylprednisolone and orbital decompression surgery was 4/5 mm. Mean of reduction in proptosis in patients receiving IV methylprednisolone only was 1/0 mm and in patients with surgical decompression only was 5/5 mm. Mean VA was 0.1 ± 0.5/0.1 ± 0.5 logMAR at baseline and 0.05 ± 0.7/0.05 ± 0.7 at final follow-up. In 92% (45/49), VA was preserved or improved at final follow-up. CONCLUSIONS: The majority of patients with DON were treated both with IV corticosteroids and 3-wall decompression surgery. Vision could be successfully preserved in most cases and reduction of proptosis was achieved, especially after orbital decompression surgery.

Remyelination in humans due to a retinoid-X receptor agonist is age-dependent.

Remyelination efficiency declines with advancing age in animal models, but this has been harder to demonstrate in people with multiple sclerosis. We show that bexarotene, a putatively remyelinating retinoid-X receptor agonist, shortened the visual evoked potential latency in patients with chronic optic neuropathy aged under 42 years only (with the effect diminishing by 0.45 ms per year of age); and increased the magnetization transfer ratio of deep gray matter lesions in those under 43 years only. Addressing this age-related decline in human remyelination capacity will be an important step in the development of remyelinating therapies that work across the lifespan.

Advances in the Treatment of Thyroid Eye Disease Associated Extraocular Muscle Myopathy and Optic Neuropathy.

PURPOSE OF REVIEW: To review emerging treatments for thyroid eye disease (TED) associated extraocular muscle myopathy and dysthyroid optic neuropathy (DON). RECENT FINDINGS: Emerging targeted biologic therapies may alter the disease course in TED. Teprotumumab, a type I insulin-like growth factor receptor inhibitor, is the most recent addition to the treatments available for TED-associated extraocular muscle myopathy causing diplopia. Small studies also suggest a potential therapeutic benefit for DON. Various recent studies have also expanded our knowledge on conventional TED therapies. The therapeutic landscape of TED and its sequelae has evolved in recent years. New targeted therapies have the potential to reduce the extraocular muscle and orbital volume expansion which can lead to diplopia and vision loss from optic nerve compression. Longer term efficacy and durability data is needed to determine the role biologics, such as teprotumumab, should play in the treatment of TED patients compared to the current standard of care.

Treatment and prophylaxis of radiation optic neuropathy: A systematic review and meta-analysis.

PURPOSE: Radiation optic neuropathy (RON) generally follows radiation therapy that exceed 50 Gy to the visual axis and occurs within three years of therapy. Currently, there are no universally accepted treatments or prophylaxis for RON. The review aimed to examine the efficacy of all treatments and prophylaxis for RON. METHODS: MEDLINE, Embase, the Cochrane Library, and gray literature were searched to December 2020. Studies on treatment(s) and/or prophylaxis of RON were included. Results were meta-analyzed using a random-effects model. Primary outcomes included the proportions of patients who experienced improvement, no change, or worsening of visual acuity (VA) for each treatment. Secondary outcome was the incidence of RON for studies on prophylaxis. RESULTS: Overall, 50 studies (n = 5397) were included. Meta-analysis (n = 1752) showed significantly lower incidence of RON in patients who underwent intravitreal anti-VEGF prophylaxis compared to control (RR 0.64, 95%CI [0.48, 0.86]) for uveal melanoma. Intravitreal anti-VEGF injections (n = 68), hyperbaric oxygen therapy alone (n = 14), and pentoxifylline (n = 5) resulted in improved or stable vision ≤1 logMAR in 54.5%, 42.9%, and 40.0% of patients, respectively. Systemic corticosteroids (n = 82), anticoagulants (n = 12), and systemic bevacizumab (n = 7) showed improved or stable vision ≤1 logMAR in 17.1%, 33.3%, and 14.3% of patients, respectively. Overall risk of bias was low, but evidence was limited to retrospective studies. CONCLUSION: Intravitreal anti-VEGF injections reduced incidence of RON in irradiated uveal melanoma patients. Systemic corticosteroids, systemic bevacizumab, and warfarin alone are likely ineffective treatments. Early hyperbaric oxygen therapy and intravitreal anti-VEGF injections were most effective among those investigated and require further investigation.

Rare early presentation of bilateral compressive optic neuropathy with complete vision loss from nasopharyngeal carcinoma.

Nasopharyngeal carcinoma is very rarely associated with bilateral vision loss, and only in advanced disease. We report a case of bilateral severe compressive optic neuropathy as a first presentation from massive nasopharyngeal carcinoma with poor visual outcome despite corticosteroid, chemotherapy and radiotherapy. Red flag symptoms and signs of mass lesions in the posterior nasal space should be investigated and treated promptly to prevent devastating visual and prognostic consequences.