Low vision devices for age-related macular degeneration: a systematic review.

PURPOSE: Age-related macular degeneration (AMD) is a degenerative condition impacting central vision. Evaluating the effectiveness of low vision devices provides empirical evidence on how devices can rehabilitate and overcome deficits caused by AMD. This evidence could help to facilitate discussion on necessary future improvements to vision enhancement technology. METHODS: A systematic review of the literature was conducted on low vision device use in AMD populations. Relevant peer-reviewed research articles from six databases were screened. RESULTS: The findings of thirty-five studies revealed a significant positive impact of low vision devices leading to improvements in visual acuity, reading performance, facial recognition, and more. While the studies were found to have moderate risks of bias, a GRADE assessment of the evidence suggested the certainty of the evidence was low-moderate. DISCUSSION: Simple hand-held low vision devices (e.g., magnifiers) appear to currently have greater preferential support than newer visual enhancement technology (e.g., head mounted devices). Financial, comfort or usability reasons may influence preferences more than performance-based findings. However, there is a lack of studies examining newer technologies in AMD populations, which future research should address. Moreover, given the presence of bias across the studies and limited controlled experiments, confidence in the results may be low. CONCLUSIONS: Most studies indicated that low vision devices have positive impacts on reading and visual performance. But, even though they are reported to be a valuable asset to AMD populations, more rigorous research is required to draw conclusive evidence. IMPLICATIONS FOR REHABILITATIONLow vision devices can improve patient outcomes (e.g., vision, reading ability) for age-related macular degeneration populations.A multidisciplinary combination of low vision devices and rehabilitative services (i.e., eccentric viewing training, counselling, education) may enhance quality of life.

Intravenous Fibrinolysis for Central Retinal Artery Occlusion: A Cohort Study and Updated Patient-Level Meta-Analysis.

BACKGROUND AND PURPOSE: Central retinal artery occlusion results in sudden, painless, usually permanent loss of vision in the affected eye. There is no proven, effective treatment to salvage visual acuity and a clear, unmet need for an effective therapy. In this work, we evaluated the efficacy of intravenous tissue-type plasminogen activator (IV alteplase) in a prospective cohort study and an updated systematic review and meta-analysis. METHODS: We enrolled consecutive patients with acute central retinal artery occlusion within 48 hours of symptoms onset and with a visual acuity of <20/200 from January 2009 until May 2019. The primary outcomes were safety and functional visual acuity recovery. We compared rates of visual recovery between those treated with alteplase within 4.5 hours of symptom onset to those who did not receive alteplase (including an analysis restricted to untreated patients presenting within the window for treatment). We incorporated these results into an updated systematic review and patient-level meta-analysis. RESULTS: We enrolled 112 patients, of whom 25 (22.3% of the cohort) were treated with IV alteplase. One patient had an asymptomatic intracerebral hemorrhage after IV alteplase treatment. Forty-four percent of alteplase-treated patients had recovery of visual acuity when treated within 4.5 hours versus 13.1% of those not treated with alteplase (P=0.003) and 11.6% of those presenting within 4 hours who did not receive alteplase (P=0.03). Our updated patient-level meta-analysis of 238 patients included 67 patients treated with alteplase within 4.5 hours since time last known well with a recovery rate of 37.3%. This favorably compares with a 17.7% recovery rate in those without treatment. In linear regression, earlier treatment correlated with a higher rate of visual recovery (P=0.01). CONCLUSIONS: This study showed that the administration of intravenous alteplase within 4.5 hours of symptom onset is associated with a higher likelihood of a favorable visual outcome for acute central retinal artery occlusion. Our results strongly support proceeding to a randomized, placebo-controlled clinical trial.

Arterial Occlusions to the Eye: From Retinal Emboli to Ocular Ischemic Syndrome.

: Abstract: A loss or lack of blood supply to the eye can result in acute loss of vision. The site of ischemia may be at the level of the retinal arterioles, the central retinal artery, or further back at the ophthalmic and internal carotid artery. Recognizing the symptoms and signs are important to help prevent permanent ischemic and irreversible blindness. The objective of this review article is to provide the general ophthalmologists with information on how to recognize the symptoms and to best manage these patients. The management is to investigate for the cause of the transient monocular visual loss and to apply secondary prevention to address atherosclerotic risk factors to prevent further ischemic events like a stroke.

Visual compensation in cervical dystonia.

Introduction: Visual-spatial processing can be affected in people with cervical dystonia (CD). These impairments have almost exclusively been observed in laboratory studies, but the impact of visuo-spatial impairments on daily activities is unclear. Here, we investigated how people living with CD visually explore the environment. Method: 21 participants (10 patients and 11 controls) walked a designated course searching for targets while their eye movements were tracked by the means of eye tracking glasses. In addition, all participants performed an extensive battery of spatial neglect tests. Results: People with CD visually compensated for their lateral head position. That is, they made more eye movements towards the side opposite their lateral head position. No evidence for or against spatial asymmetries were detected in a range of behavioral measures (target detection, standard neglect battery tests) on a group level. Yet, single patients showed a neglect-like pattern. One patient with a right torticollis failed to detect most left-sided targets when walking. Conclusion: In general, participants seem to adequately compensate for the deviated head posture when judging spatial stimuli. However, the insufficient spatial exploration in one patient with a valid driving license should raise awareness in clinicians to potential visuo-spatial problems in people living with CD. An interesting question arising from our findings is whether people with CD and marked visuo-spatial deficits would benefit from scanning training, as is employed in stroke rehabilitation.

Extensive Postradiation Ocular and Diffuse Cranial Neuromyotonia Mimicking Myasthenia Gravis.

BACKGROUND: Ocular neuromyotonia is a rare, but well-recognized, complication of cranial irradiation. CASE REPORT: Using figures and videos, we report a 52-year-old man with extensive ocular, brainstem, and lower cranial nerve neuromyotonia postradiation therapy for a fourth ventricle glioma who, in the context of an apparently positive edrophonium test, was initially misdiagnosed with myasthenia gravis. CONCLUSIONS: This is the first case of postirradiation neuromyotonia to be reported with such extensive cranial nerve and brainstem involvement.

Fingolimod and macular edema: Pathophysiology, diagnosis, and management.

Fingolimod causes macular edema (ME) by acting via the S1P3 receptor agonism, thereby reducing the tight junction between the endothelial cells of the retinal capillaries. This results in the breakdown of the inner blood retinal barrier, causing ME. Ophthalmologic evaluation including optical coherence tomography is recommended at baseline and then at 3 months, 6 months, and annually thereafter in all patients on fingolimod. The risk of ME increases in patients who are diabetic, have had uveitis, or who undergo intraocular procedures such as cataract surgery, and hence these patients need close monitoring. Cessation of the drug results in resolution of the ME. However, ME can also be treated using anti-inflammatory medication (steroids) in patients who opt to remain on fingolimod.

Treatment options for central retinal artery occlusion.

OPINION STATEMENT: Central retinal artery occlusion (CRAO) is an ocular emergency and is the ocular analogue of cerebral stroke. It results in profound, usually monocular vision loss, and is associated with significant functional morbidity. The risk factors for CRAO are the same atherosclerotic risk factors as for stroke and heart disease. As such, individuals with CRAO may be at risk of ischemic end organ damage such as a cerebral stroke. Therefore, the management of CRAO is not only to restore vision, but at the same time to manage risk factors that may lead to other vascular conditions. There are a number of therapies that has been used in the treatment of CRAO in the past. These include carbogen inhalation, acetazolamide infusion, ocular massage and paracentesis, as well as various vasodilators such as intravenous glyceryl trinitrate. None of these "standard agents" have been shown to alter the natural history of disease definitively. There has been recent interest shown in the use of thrombolytic therapy, delivered either intravenously or intra-arterially by direct catheterisation of the ophthalmic artery. Whilst a number of observational series have shown that the recovery of vision can be quite dramatic, two recent randomised controlled trials have not demonstrated efficacy. On the contrary, intra-arterial delivery of thrombolytic may result in an increased risk of intracranial and systemic haemorrhage, while the intravenous use of tissue plasminogen activator (tPA) was not shown to be efficacious within 24 h of symptom onset. Nevertheless, both of these studies have shown one thing in common, and that is for treatment to be effective in CRAO, it must be deployed within a short time window, probably within 6 h of symptom onset. Therefore, while CRAO is a disease that does not have a treatment, nevertheless it needs to follow the same principles of treatment as any other vascular end organ ischaemic disease. That is, to attempt to reperfuse ischemic tissue as quickly as possible and to institute secondary prevention early.

Evaluating the effectiveness of multidisciplinary low-vision rehabilitation.

PURPOSE: To objectively evaluate the changes in vision-specific quality of life (QoL) after multidisciplinary low-vision rehabilitation at the Royal Society for the Blind Low-Vision Clinic. METHODS: The standard care model at the Royal Society for the Blind Low-Vision Clinic is optical management at the initial assessment, with subsequent referral to multidisciplinary services as required. Participants completed the Impact of Vision Impairment Questionnaire (IVI) and Veterans Affairs Low-Vision Visual Functioning Questionnaire (VA LV VFQ-48) before initial assessment, at 30 days (to assess the outcome of optical management) and at 3 months follow-up (to assess the outcome of multidisciplinary services). RESULTS: Seventy-one participants completed the study. Using the VA LV VFQ-48, an improvement (p < 0.05) was seen at 30 days follow-up in overall visual ability, and the reading, visual information, and visual motor subscores. However, at 3 months follow-up, all subscores were not significantly different from pre-rehabilitation levels. In contrast, for the IVI, there was no improvement in both the overall score and the mobility subscale at 30 days follow-up, but a significant improvement at 3 months follow-up. Greater improvements in visual function were seen for those with low vision (<20/60-20/200) compared with those with blindness, those aged >85 years compared with those aged 80 to 85 and <80 years, and those without glaucoma (visual motor subscore). CONCLUSIONS: The VA LV VFQ-48 and IVI demonstrated improvements in QoL after low-vision rehabilitation. The timing of the observed changes varied between the two questionnaires, reflecting the different content of these instruments and the timing of delivery of multidisciplinary services, but also suggests that continued rehabilitation may be warranted to maintain a patient's QoL.

Functional improvements following the use of the NVT Vision Rehabilitation program for patients with hemianopia following stroke.

UNLABELLED: The incidence of visual deficits following stroke ranges from 20%-68% and has significant impact on activities of daily living. The NVT system is a compensatory visual scanning training program that consists of combined static and mobility training and transfer to activities of daily living. The study aims to evaluate functional changes following the NVT program for people who have homonymous hemianopia (HH) following stroke. METHOD: Interventional case series of 13 consecutive participants with HH undergoing NVT vision rehabilitation. The primary outcome measure was the number of targets missed on a standardized Mobility Assessment Course (MAC). Other outcome measures included assessment of visual scanning, vision specific Quality of Life questionnaires and reading performance. RESULTS: The average number of targets (sd) missed on the MAC course was 39.6 ± 20.9% before intervention, 27.5 ± 16.3% immediately post intervention and 20.8 ± 15.5% at 3 months post rehabilitation. The study showed a statistically significant trend in improvement in mobility related subscales of National Eye Institute Visual Function Questionnaire-NEI VFQ-25 (p=0.003) and the Veteran Affairs Low Vision Visual Function Questionnaire-VA LVFQ-48 (p=0.036) at 3 months post rehabilitation. DISCUSSION: The NVT intervention resulted in functional improvements in mobility post rehabilitation. The NVT training showed improvement in vision specific quality of life. There is a need for standardised vision therapy intervention, in conjunction with existing rehabilitation services, for patients with stroke and traumatic brain injury.

Efficacy of intravenous tissue-type plasminogen activator in central retinal artery occlusion: report from a randomized, controlled trial.

BACKGROUND AND PURPOSE: Central retinal artery occlusion is caused by a platelet-fibrin thrombus or embolic occlusion and is a stroke of the eye. Observational studies suggest that thrombolytics may restore ocular perfusion and visual function. We hypothesized that intravenous tissue-type plasminogen activator (tPA) administered within 24 hours of symptom onset might restore ocular perfusion and visual function. METHODS: A placebo-controlled, randomized trial of intravenous tPA versus intravenous saline was performed in patients with clinically defined central retinal artery occlusion within 24 hours of symptom onset. tPA was administered at a total dose of 0.9 mg/kg, with 10% given as a 1-minute bolus and the remainder over 1 hour. An improvement of visual acuity of 3 lines or more was considered significant. RESULTS: Twenty-five percent (2 of 8) of the tPA group experienced the primary outcome at 1 week after tPA versus none of the placebo group. One patient had an intracranial hemorrhage. The visual acuity improvement of these 2 patients was not sustained at 6 months. In both patients, tPA was administered within 6 hours of symptom onset. CONCLUSIONS: Although essentially a negative study, it does add to the evidence base of reperfusion in central retinal artery occlusion by showing that the time window for intervention is likely to be <6 hours. Reocclusion is a potential problem and may require adjuvant anticoagulation. Future studies should concentrate on determining the efficacy of thrombolytics in the <6-hour time window. Clinical Trial Registration- URL: http://www.anzctr.org.au. Unique identifier: 83102.

Study of the efficacy of intravenous tissue plasminogen activator in central retinal artery occlusion.

RATIONALE: Central retinal artery occlusion is a stroke of the eye caused by a blockage of its main blood supply by platelet-fibrin clot. Systemic thrombolysis has been successful in restoring perfusion to ischaemic tissue by fibrin-platelet clot lysis in ischaemic stroke and myocardial infarction. Several open-label studies have demonstrated efficacy of thrombolysis in the treatment of central retinal artery occlusion, with up to 60-70% of treated subjects experiencing an improvement in visual acuity. Most of these are given intraarterially, which is an invasive procedure and not widely applicable to all treatment centres. An alternative is the intravenous infusion of tissue plasminogen activator using existing stroke thrombolysis protocols. A systematic review of all observational studies of intravenous tissue plasminogen activator in acute central retinal artery occlusion showed that 48·5% of subjects had a four line or more visual acuity improvement with an acceptable rate of haemorrhagic complications, creating the equipoise necessary to conduct a randomised controlled trial. AIM: To determine the efficacy of intravenous thrombolysis in acute treatment of central retinal artery occlusion. DESIGN: A phase II, placebo-controlled, double-blind, randomised controlled trial comparing intravenous tissue plasminogen activator at 0·9 mg/kg to placebo (normal saline) 100 ml in a 1:1 block randomisation. STUDY OUTCOME: The primary outcome measure is an improvement of three lines or more on the Snellen visual acuity chart, which signifies a doubling of the visual angle.

Access to low-vision rehabilitation services: barriers and enablers.

BACKGROUND: The current mismatch between the need for and uptake of low-vision services has been attributed to various barriers including different service delivery models and referral pathways. This study evaluates the referral pathway and low-vision service provision of the Royal Society for the Blind (RSB) in South Australia. METHODS: All new referrals from the 2008-2009 financial year to the RSB were reviewed. Initially, patients were contacted by a triage officer within one week of referral. Initial appointments were made in the Low Vision Clinic with a multidisciplinary team. Reasons for declining the appointment or non-attendance were tracked via telephone. RESULTS: There were 1116 patients referred over a 12-month period and 1082 (97 per cent) were reviewed in the Low Vision Clinic. Most attendees (92 per cent) lived within 50 kilometres of the clinic. There were 34 referred patients, who declined or did not attend the assessment. All non-attendees also lived within 50 kilometres of the Low Vision Centre. Concurrent major health problems (27 per cent) and patients not feeling the need for low-vision rehabilitation (27 per cent) were the most common reasons for not accessing the service. Only 125 patients (11.6 per cent) accessed volunteer transport services and only 24 patients (2.2 per cent) needed an interpreter service. CONCLUSION: The attendance rate is significantly higher than in other published studies. The distance to travel or transport difficulties were not significant barriers. Patient perception that either the service was not required or would not help them was the main barrier. The referral and triage process appeared to be a major enabler of low-vision service uptake.

Concurrent central retinal artery occlusion and branch retinal vein occlusion in giant cell arteritis.

Ophthalmic involvement in giant cell arteritis can manifest in a number of ways. Central retinal artery occlusion is one of the common causes of visual loss in giant cell arteritis. On the contrary, branch retinal vein occlusion is rarely associated with the latter. We report an 89-year-old lady with acute left central retinal artery occlusion on a background of progressive decline in vision over a 6-month period with a concurrent right branch retinal vein occlusion. Subsequent investigation confirmed giant cell arteritis on temporal artery biopsy. This is the first reported case of a concurrent central retinal artery occlusion and branch retinal vein occlusion in giant cell arteritis, and highlights the various ocular presentations that can occur in giant cell arteritis.

Visual field defects after stroke--a practical guide for GPs.

BACKGROUND: Visual field defect after stroke can result in significant disability and reduction in quality of life. Visual rehabilitation aims to maximise the residual vision and decrease functional disability. Understanding the rehabilitation options available, and where to refer patients with visual defects after a stroke, can help patients, and their families, in the rehabilitation process. OBJECTIVE: This article provides a review of the functional disability from visual field loss and discusses the various forms of visual rehabilitation. DISCUSSION: Optical therapy, eye movement therapy and visual field restitution are the rehabilitation therapies currently available. Rehabilitation needs to cater to each patient's specific needs. Any patient recognised as having a visual field defect after stroke needs prompt referral for further assessment and consideration for visual rehabilitation.

Multiple sclerosis: patients' information sources and needs on disease symptoms and management.

OBJECTIVE: To investigate the current information sources of patients with multiple sclerosis (MS) in the early stages of their disease and to identify patients' preferred source of information. The relative amounts of information from the different sources were also compared. METHODS: Participants at a newly diagnosed information session organized by the Multiple Sclerosis Society of South Australia were invited to complete a questionnaire. Participants were asked to rate on a visual analog scale how much information they had received about MS and optic neuritis from different information sources and how much information they would like to receive from each of the sources. RESULTS: A close to ideal amount of information is being provided by the MS society and MS specialist nurses. There is a clear deficit between what information patients are currently receiving and the amount of information they actually want from various sources. Patients wish to receive significantly more information from treating general practitioners, eye specialists, neurologists, and education sessions. Patients have identified less than adequate information received on optic neuritis from all sources. CONCLUSION: This study noted a clear information deficit regarding MS from all sources. This information deficit is more pronounced in relation to optic neuritis and needs to be addressed in the future. PRACTICE IMPLICATIONS: More patient information and counselling needs to be provided to MS patients even at early stages of their disease, especially in relation to management of disease relapse.

Ocular neovascularization following central retinal artery occlusion: prevalence and timing of onset.

PURPOSE: Debate exists in the literature on the prevalence and etiology of neovascularization following central retinal artery occlusion (CRAO). The reported prevalence varies from 2.5% to 31.6%. We conducted a retrospective study to determine the prevalence of ocular neovascularization following acute CRAO in our institution. METHODS: A retrospective audit of consecutive patients with nonarteritic/thromboembolic CRAO presenting between 1997 and 2009 in a single tertiary teaching hospital. RESULTS: Thirty-three patients were identified as having nonarteritic CRAO, and of this cohort 6 patients (18.2%) developed ocular neovascularization. Neovascular glaucoma was present in 5 cases (15.2%); 2 of these presented through an emergency department with painful eyes, both at 16 weeks post CRAO. The other cases of neovascularization were detected on scheduled follow-ups. Mean time from retinal occlusive event to observed neovascularization was 8.5 weeks (range 2-16 weeks). One case of neovascularization was associated with hemodynamically significant ipsilateral carotid stenosis; no patient had proliferative diabetic retinopathy or other causes of neovascularization. CONCLUSIONS: The prevalence of neovascularization following acute CRAO in our population was 18.2% at an average of 8.5 weeks post CRAO. There was a temporal relationship between the 2 events and no other causes of neovascularization demonstrable in our cohort of patients. There is no consensus on the follow-up regimen post CRAO to detect ocular neovascularization complications. Our study suggests that neovascularization can occur early and regular follow-up especially in the first 4 months is important post CRAO.

Clinical characteristics and outcome of current standard management of central retinal artery occlusion.

BACKGROUND: To investigate the visual outcomes in acute central retinal artery occlusion (CRAO) with current standard therapy at two university teaching hospitals. METHODS: Retrospective analysis of two cohorts of CRAO patients from John Hopkins Hospital (JHH; USA), and Flinders Medical Centre (FMC; Australia), treated with current standard therapy. The outcome measures were visual acuity, and subsequent ocular and systemic ischaemic events. RESULTS: The mean follow-up period was 11.2 +/- 13.1 months in the JHH cohort and 35.4 +/- 34.9 months in the FMC cohort. The frequency distribution of vascular risk factors and the incidence of subsequent ischaemic events were similar for the patients from both institutions. All patients from JHH were treated as inpatients, whereas 79% of patients from FMC were treated as outpatients. More patients in the JHH cohort underwent paracentesis, ocular massage or were treated with intraocular hypotensive agents (76%) than in the FMC cohort (26%); however, there was no significant difference in visual outcome between the two cohorts (P = 0.114). CONCLUSION: Despite differences in management of CRAO between two institutions in different countries, visual outcomes were similar. This suggests a lack of efficacy of current standard treatment in acute CRAO.

Traumatic vertebral artery dissection presenting with incomplete congruous homonymous quadrantanopia.

BACKGROUND: To describe a rare presentation of vertebral artery dissection (VAD) as a small but congruous incomplete homonymous hemianopia demonstrating use of visual field testing in the diagnosis. CASE PRESENTATION: A 30 year old woman had been unwell for 4 months with difficulty focusing, vertigo, dizziness and a feeling of falling to the right. A small but congruous right inferior homonymous quadrantanopia was found on examination leading to further investigation that uncovered a vertebral artery dissection and multiple posterior circulation infarctions including a left occipital stroke matching the field defect. CONCLUSIONS: We describe an atypical case of VAD presenting with a small congruous quadrantanopia. This is a rare but significant condition that predisposes to multiple thromboembolic infarction that may be easily misdiagnosed and a high index of suspicion is required to make the diagnosis.

Clinicopathological correlation in pituitary gland metastasis presenting as anterior visual pathway compression.

Pituitary gland metastases, albeit rare, remain an important differential in sellar and suprasellar tumours. Clinical and radiological features of pituitary metastases may be indistinguishable from benign suprasellar lesions such as a pituitary adenoma. Histopathology with immunohistochemical assay remains the key to the diagnosis of pituitary metastasis. We describe four patients with sellar lesions presenting with anterior visual pathway compression initially diagnosed as pituitary adenomas who on immunohistochemistry were found to have metastases to the pituitary. Classification of the cell histology determined the primary site of origin in some patients. This series demonstrates the importance of combining histopathology and immunohistochemistry in the diagnosis of suprasellar lesions.