Acute Monocular Vision Loss Research Articles

Dolosigranulum Pigrum Bacteremia in an Injecting Drug User Associated with Methicillin Resistant Staphylococcus Aureus Endocarditis and Fungal Endophthalmitis

Dolosigranulum pigrum is a commensal bacterium of the upper respiratory tract. We present a case of D. Pigrum bacteremia, identified incidentally in a patient with a history of injecting drug use (IDU), chronic hepatitis C (HCV) and partially treated methicillin resistant staphylococcus aureus (MRSA) endocarditis, who presented with acute monocular vision loss. Workup was consistent with unilateral fungal endophthalmitis. The patient was initially treated with intravenous Voriconazole, Ceftriaxone and linezolid for 4 weeks with resolution of his symptoms. This case highlights the emerging role of D. Pigrum as a pathogen in the immune compromised and specifically in the IDU population. This case further illustrates that although identification of fungal growth in vitreous fluid may include culture and PCR analysis, fungal endophthalmitis remains a clinical diagnosis and that fungal cultures have lower sensitivity.

Abstract WMP56: Diagnosis of Acute Central Retinal Artery Occlusion With a Nonmydriatic Ocular Fundus Camera Combined With Optical Coherence Tomography in the Emergency Department: A New Paradigm

Background: Central retinal artery occlusion (CRAO) is a retinal infarction with causes/management similar to cerebral infarction. However, CRAO diagnosis is often delayed, resulting in missed opportunities for rapid treatment. Diagnosis by ophthalmologists is essential prior to management by stroke neurologists and may be challenging within a few hours of vision loss. Our goal is to provide a new paradigm in the emergency department (ED) with the implementation of a nonmydriatic ocular fundus camera (NMFP) combined with optical coherence tomography (OCT) to allow for ultrarapid remote diagnosis and stroke alert as soon as patients present to the ED. Methods: Prospective collection of data from consecutive patients who had NMFP-OCT for acute monocular vision loss in our general ED affiliated with a comprehensive stroke center. We collected timing of photographs; final diagnosis (CRAO/other causes of vision loss); time between vision loss onset and CRAO diagnosis; NMFP-OCT findings. Results: Over 9 weeks, 27 patients had NMFP-OCT obtained for acute monocular visual loss, including 9 CRAO [others included 7 retinal detachments; 8 vitreous hemorrhages; 3 temporal arteritis]. Diagnosis of acute CRAO was made remotely on NMFP-OCT obtained 30, 33, 36 min after arriving in ED in 3 patients presenting to ED within 3.5 hrs of vision loss. In all 3 patients, the ocular color photographs were almost normal and acute CRAO diagnosis was made primarily from the OCT showing inner retinal edema/hyperreflectivity. Conclusion: Incorporating NMFP-OCT into the ED workflow can be used as a substitute for funduscopic examination/pupillary dilation, allowing for remote diagnosis of CRAO by ophthalmologists who can provide real time recommendations before in person visit. These positive preliminary results suggest that this strategy accelerates the diagnosis of urgent vision loss, resulting in improved patient outcomes. Results of the first 6 months will be presented at the conference.

Abstract 14835: A Rare Case of Left Atrial Thrombus Post Extensive Ablation Despite Therapeutic Anticoagulation

Introduction: Persistent Atrial Fibrillation (AF) is often treated with radiofrequency catheter ablations to the pulmonary veins as well as linear ablations of the left atrium. Extensive ablations can increase the risk of clotting at the ablation site. Case: A 47-year-old male with a history of Shone Syndrome, AF on Warfarin with prior ablations presented for acute monocular vision loss found to be due to central retinal artery occlusion. Symptoms were three weeks after repeat PVI and posterior wall isolation. Transesophageal echocardiogram (TEE) showed multiple mobile echodensities suspicious for thrombi in the left atrium but no LAA thrombus. With elevation of INR goal range to 3 to 3.5 and addition of aspirin there was resolution of the left atrial thrombi on repeat TEE 2 months later. Discussion: Visual loss was suspected to be cardioembolic. The cause of thrombi, despite being anticoagulated with an INR of 2.6, was multifactorial from chronic fibrosis, repeated ablation-induced injury yielding endothelial damage, elevated LA pressures due to mitral stenosis, and persistent atrial flutter. Multiple small thrombi visualized adherent to left atrial wall without any thrombus in LAA. Advanced imaging cannot guide anticoagulation strategy directly but can assess fibrotic burden. It is important to consider increasing the INR goal or adding aspirin to high-risk patients. Conclusion: Patients should be risk stratified when extensively ablated and with elevated atrial pressures with multimodal imaging to determine risk and management when undergoing extensive ablation. Further investigation needs to be done to determine if heightened anticoagulation goals are needed based on risk stratification.

Sudden vision loss and neurological deficits after facial hyaluronic acid filler injection

BackgroundThe ongoing expansion of the cosmetic armamentarium of facial rejuvenation fails to uncover the inherent risks of cosmetic interventions. Informed consent to all risks of cosmetic filler injections and potential sequelae, including ocular and neurological complications, should be carefully ensured. We present two cases of complications following facial hyaluronic acid filler injections.Case presentationsCase 1: A 43-year-old woman presented with monocular vision loss of the left eye, associated ptosis, ophthalmoplegia, periocular pain and nausea, cutaneous changes of the glabella region and forehead, and sensory impairment in the left maxillary branch dermatome (V2) after receiving a hyaluronic acid (HA) filler injection into the left glabellar area. On ophthalmological examination, an ophthalmic artery occlusion (OAO) was diagnosed upon identification of a “cherry-red spot”. Magnetic resonance imaging (MRI) revealed a left ischemic optic neuropathy. Supportive therapy and hyaluronidase injections were initiated. A follow-up MRI of the head performed two months after presentation corresponded to stable MRI findings. The patient had irreversible and complete vision loss of the left eye, however, the ptosis resolved.Case 2: A 29-year-old woman was admitted to hospital a few hours after a rhinoplasty and cheek augmentation with hyaluronic acid, presenting with acute monocular vision loss in the right eye, retrobulbar pain, fatigue and vomiting. In addition, the patient presented a harbinger of impending skin necrosis and a complete oculomotor nerve palsy on the right side, choroidal ischemia and vision impairment. Supportive treatment and hyaluronidase injections into the ischemic tissue were initiated. A small scar at the tip of the nose, vision impairment and an irregular pupillary margin on the right side persisted at follow-up.ConclusionThese two case reports and the literature review emphasize the pathophysiological mechanisms leading to potentially devastating complications. In order to reduce the risk of vision loss secondary to cosmetic filler injections, practitioners should possess a thorough knowledge of anatomy and preventive strategies.

Neuro-Ophthalmology

Neuro-ophthalmic disorders are capable of causing significant morbidity, requiring the clinician to develop and maintain nuanced skills that facilitate timely diagnosis and effective management. Virtually any neurologic disease can present with symptoms of either afferent or efferent visual pathway dysfunction. This chapter emphasizes principles of accurate localization and discusses key clinical clues that serve to generate an appropriate differential diagnosis. It specifically addresses acute monocular visual loss from retinal and optic nerve diseases, papilledema, chronicoptic neuropathies, visual field deficits, supranuclear and infranuclear eye movement disturbances, and nystagmus. Figures include an algorithm for the evaluation of acute monocular visual loss, computed tomography scans, and a large gallery of diagnostic photos. This review contains 49 figures, 14 tables, and 171 references Keywords: Neurology, ophthalmology, nystagmus, optic field deficit, neuritis, neuropathy, papilledema, visual pathway

Abstract WP58: Telestroke Assessment Of Acute Monocular Visual Loss Fails To Routinely Diagnose Retinal Artery Occlusion And Recommend Thrombolytic Therapy

Introduction: Central retinal artery occlusion (CRAO) is a neurovascular emergency and acute monocular visual loss should trigger a stroke code activation. As thrombolytic therapy is considered for non-arteritic CRAO in the stroke therapeutic window, we aimed to review all telestroke encounters for acute visual loss in Mayo Clinic national telestroke network, focusing on the ocular diagnosis and the management recommendations. Hypothesis: patients evaluated via telestroke for acute visual loss are less likely to receive thrombolysis Methods: We conducted a retrospective review of the Mayo Clinic Arizona Telestroke/Teleneurology (2010-2017, 6364 encounters) and Mayo Clinic Enterprise Telestroke databases (2018-2020, 3147 encounters), searching for visits requested for acute visual loss. We recorded the subjects’ demographics, time from last seen normal to telestroke evaluation, preliminary diagnosis, and therapeutic/diagnostic recommendation. Results: From a total of 9511 audio-video telestroke encounters, 49 (0.51%) had an ocular diagnosis. Out of those, 6 patients (12.2%) had a provisional diagnosis of CRAO. 4/6 (66.6%) CRAO patients presented within 4.5h from symptom onset (median 2 hours) and none received thrombolytic therapy. All telestroke physicians recommended antiplatelet, ophthalmology consultation for diagnostic evaluation and comprehensive stroke work-up. Conclusions: Telestroke evaluations for CRAO, in routine practice, are less common than the disease prevalence would predict, suggesting that education is urgently mandated to increase the index of awareness. Even when CRAO subjects are evaluated via telestroke within 4.5 hours from symptom onset, they do not receive thrombolytic therapy nor timely ophthalmologic evaluation. Telestroke visits may benefit from the inclusion of tele-ophthalmologic consults to help with establishing the diagnosis in the narrow time-window of intravenous thrombolysis.

Paracentral Acute Middle Maculopathy in Pregnancy.

A 31-year-old previously healthy, pregnant woman presented with acute, unilateral, painless paracentral vision loss in the left eye. The results of neuroimaging studies were normal. Hypercoagulable workup was negative, and fundus examination showed no retinal emboli and no retinal vascular abnormalities. The patient had well-controlled blood pressure and did not have eclampsia/preeclampsia. Although a presumptive referral diagnosis of "optic neuritis" was made, optical coherence tomography (OCT) of the macula showed a hyperreflective band involving the inner nuclear layer, consistent with paracentral acute middle maculopathy (PAMM). Furthermore, OCT angiography (OCT-A) showed flow attenuation of the outer retinal capillary plexus, further supporting a diagnosis of PAMM in pregnancy. Clinicians should be aware of the benefit of OCT-A as a complement to macular OCT in the evaluation of acute monocular vision loss mimicking retrobulbar optic neuropathy, particularly when signs and findings of other retinal vasculopathy are absent. PAMM should be considered in pregnant patients with acute visual changes.

Abstract P16: Alteplase Within 4.5 Hours via Telemedicine and In-Person for Central or Branch Retinal Artery Occlusion vs Medical Management

Introduction: Central or branch retinal artery occlusion (CRAO/BRAO) results in acute monocular vision loss. Only one prospective study of IV tPA use within 4.5 hours with a recent updated patient-level meta-analysis showed favorable visual outcomes but long-term visual outcomes were not reported. We evaluated the use of IV tPA within 4.5 hours for CRAO/BRAO vs medical management and assessed 90-day visual outcomes. Given the increasing use of telemedicine (TM), we also assessed the diagnostic accuracy of CRAO/BRAO evaluated via TM vs in-person. Methods: Data were retrospectively abstracted for CRAO/BRAO treated with IV tPA vs medical management within a large managed healthcare system. 90-day visual outcomes were reported as Low Vision (no light perception, light perception, hand motion, finger counting) and mean LogMAR, which was converted from Snellen, and classified as normal-mild impairment (LogMAR 0.4-0.5), moderate-severe impairment (LogMAR 0.6-1.3) and blind-low vision (LogMAR >1.3). Visual outcomes were analyzed using the Wilcoxon rank-sum and signed-rank tests. Results: Between 2012-2019, 21 patients received IV tPA within 4.5 hours for presumed CRAO/BRAO vs 34 medically managed. One patient evaluated via TM and one patient evaluated in-person were misdiagnosed and excluded from analysis. Of those accurately diagnosed and treated with IV tPA, 15 were evaluated via TM and 4 in-person. At 90 days, intragroup analysis revealed at least a one-point improvement on the Low Vision scale (68% tPA, p= 0.007 vs 30% non-tPA, p=0.63) and the LogMAR scale (32% tPA, p=0.03 vs 12% non-tPA, p=0.38) with 26% of tPA patients having normal-mild impairment in vision vs 9% in non-tPA patients. Initial to 90-day vison on the Low Vision scale showed visual improvement in the tPA group (p=0.001) and a trend towards improvement on the LogMAR scale (p=0.07). Conclusions: Similar to a recent prospective study with patient level meta-analysis, we found that IV tPA within 4.5 hours for CRAO/BRAO may lead to improved visual outcomes at 90 days compared to medical management. Diagnostic accuracy of CRAO/BRAO was similar irrespective of method of being evaluated via TM or in-person. Our study was limited by small sample size and retrospective design.

Neurovascular Causes of Acute Monocular Visual Loss

Acute monocular visual loss may result from a diverse set of neurovascular and systemic disorders and requires urgent evaluation. The goal of this review is to describe the most current understanding of the clinical features, pathophysiology, diagnosis, and treatment of these disorders. 1. Acute central retinal artery occlusion (CRAO) should be treated as an equivalent to acute ischemic stroke. 2. Several recent clinical trials are evaluating the treatment of non-arteritic anterior ischemic optic neuropathy (NAION). 3. Tocilizumab is emerging as a first-line steroid-sparing immunomodulatory therapy for the treatment of giant cell arteritis (GCA). 4. Optical coherence tomography (OCT) imaging of the retina has led to the appreciation that retinal capillary ischemia in the form of paracentral acute middle maculopathy (PAMM) is a distinct form of acute monocular visual loss with relatively normal-appearing fundus examination and good prognosis for recovery. Accurate diagnosis and timely treatment of disorders resulting in acute monocular visual loss may reduce the likelihood of additional visual and neurologic complications.

Neurosarcoidosis presenting as CRVO combined CRAO: a biopsy-proven case report of a Chinese patient

BackgroundNeurosarcoidosis is a rare systemic disorder that can affect the eye and other organs, including the central nervous system. Neurosarcoidosis infiltrating the optic nerve presenting as central retinal vein occlusion combined with artery ischaemia has not been reported in the literature previously. We describe a Chinese patient presenting with acute monocular vision loss, in whom an optic nerve biopsy confirmed the diagnosis of neurosarcoidosis.Case presentationA 47-year-old woman complained of acute decreased vision in her left eye over the course of 1 month. She reported that her vision deteriorated quickly within first 3 days of consulting an ophthalmologist at a local hospital. She was diagnosed with central retinal vein occlusion after funduscopic examination and fundus fluorescein angiography, and the vision in her left eye further deteriorated to no light perception. An orbital magnetic resonance imaging showed an abnormal T1-weighted image of the optic nerve after contrast enhancement. She was referred to a neuro-ophthalmologist for further evaluation. After routine blood tests ruled out infectious and metastatic diseases, she was prescribed 500 mg/d methylprednisolone for 5 days, but her vision did not improve. As she could still not perceive light, an optic nerve biopsy was performed, and the histopathology revealed non-necrotising granuloma that was consistent with neurosarcoidosis.ConclusionsIsolated optic nerve infiltration by neurosarcoidosis without the involvement of the central nervous system or other systemic organs is challenging to diagnose. Biopsy of the optic nerve sheath is crucial for the final diagnosis of neurosarcoidosis. Therefore, a comprehensive ophthalmologic and systemic examination and work-up for inflammation of the eye, chest, and central nervous system should be conducted for atypical cases.

Abstract WP119: Alteplase Given Within 4.5 Hours via Telemedicine and in Person to Patients Presenting With a Central Retinal Artery Occlusion

Introduction: Central or branch retinal artery occlusion (CRAO/BRAO) results in acute monocular vision loss. Studies on the efficacy of IV t-PA, treated patients up to 30 hours from symptom onset. There has only been one prospective study of early IV t-PA use. With the increasing use of telemedicine (TM), we evaluated the use of IV t-PA within 4.5 hours for CRAO/BRAO and assessed visual outcomes up to 90 days amongst patients evaluated via TM versus in-person. Methods: Data were retrospectively abstracted for CRAO/BRAO treated with IV t-PA within a large managed healthcare system. Visual outcomes abstracted at 24 hours were classified as Low Vision (light perception, hand motion, finger counting) and converted to mean LogMAR. Visual outcomes abstracted within 2 weeks and 3 months were classified as normal-mild impairment (LogMAR 0.4-0.5), moderate-severe impairment (LogMAR 0.6-1.3) and blind-low vision (LogMAR >1.3). Descriptive statistics were performed on all variables. Visual outcomes were analyzed using the sign test. Results: Between 2013-2019, 16 patients received IV t-PA within 4.5 hours of symptom onset. 11 were evaluated via TM and 5 in-person. All patients were accurately diagnosed with CRAO/BRAO. 24 hours post t-PA, 37.5% of TM vs 25% of in-person patients had at least a one-point improvement on the low vision scale. Within 2 weeks, 12.5% of TM vs 18.75% of in-person, and within 90 days, 13.33% of TM vs 20% of in-person patients improved from low vision to normal-mild impairment in vision. In all patients, at 24 hours post t-PA 56.25% (p= 0.04) had at least a one-point improvement on the low vision scale. Within 2 weeks, 31.25% (p= 0.03) and 90 days, 33.3% (p= 0.03) of patients continued to improve from low vision to normal-mild impairment in vision. Conclusions: All patients were correctly diagnosed with CRAO/BRAO irrespective of method of evaluation. More patients improved on the low vision scale at 24 hours via TM than in-person. More in-person diagnosed patients improved from low vision to normal-mild impairment in vision within 2 weeks and 90 days of IV t-PA. IV t-PA use within 4.5 hours for CRAO/BRAO may lead to improved visual outcomes at 90 days regardless of TM or in-person diagnosis. Our study was limited by small sample size and no control group.

Reader response: Clinical Reasoning: A case of bilateral orbital mass lesions presenting with acute monocular vision loss.

We read the recent case by Bhatt et al.1 with interest. The 80-year-old patient had a central retinal artery occlusion (CRAO) and bilateral enhancing orbital mass lesions that improved with steroids, with a temporal artery biopsy showing arteritis that was diagnosed as giant cell arteritis (GCA)

Troubles visuels aigus associés aux méningiomes sphénoïdaux

Meningiomas represent about 20% of intracranial tumors. Involvement of the medial sphenoid wing includes anterior clinoid, cavernous sinus and superior orbital fissure meningiomas. Due to the proximity of these tumors to the optic nerve, typically progressive unilateral vision loss, over several months to years, is the classic clinical presentation. We report three cases of acute monocular vision loss, two transient and one permanent, ipsilateral to a sphenoid meningioma. Ophthalmological involvement with sphenoid meningiomas is most often chronic, due to interruption of axoplasmic flow and demyelination of the optic nerve by local compression. However, vascular involvement with ischemia of the optic nerve or transient low blood flow secondary to compression of the carotid branches vascularizing these structures is another possible mechanism. In our series, two patients had amaurosis fugax, and one patient had sudden, persistent visual loss in relation to acute anterior ischemic optic neuropathy on the side of the meningioma. The mean age of patients with acute visual manifestations was 62 years. These ischemic and non-compressive visual symptoms, ipsilateral to sphenoid meningiomas, are difficult to interpret. Whether these temporary visual disturbances of vascular origin should be considered an early sign of future severe or permanent visual impairment when no optic nerve compression is observed is not certain. The place of these acute visual disturbances in the therapeutic decision, particularly surgical, remains to be defined. Larger multicentric prospective studies are needed to better understand the role of local circulatory factors attributable to meningioma in the occurrence of these acute visual signs.

Abstract T P191: Emergent Management of Acute Retinal Ischemia

Background: Retinal artery occlusion (RAO) is characterized by acute monocular visual loss (AMVL). It is unclear how intravenous tissue-type plasminogen activator (IV tPA) affects this stroke subset. Other interventions have been explored, but clear benefit of these has yet to be demonstrated. Given the emergent nature of RAO, and absent uniform guidelines, we investigated the incidence of and approach to management of RAO at our institution. Methods: We retrospectively reviewed medical records at the University of California, San Diego from 1992 through December 2012. Charts were retrieved via the ICD-9 codes 362.30-362.37 (retinal vascular occlusion). We excluded patients with monocular visual loss for more than 24 hours before presentation, spontaneous symptom resolution, traumatic visual loss, and patients with other associated acute neurologic symptoms. We captured time from presentation to initial physician encounter, time to consultant evaluation, consultant specialty, and whether time was <4.5 hours since last known well. Results: Of 132 patient charts reviewed, 111 were excluded as per criteria above (>24 hours = 55, spontaneous resolution = 50, trauma = 2, other symptoms = 4). We identified 16 patients with AMVL: 11 with central RAO, 2 with branch RAO, 1 with temporal arteritis, and 1 with nonarteritic anterior ischemic optic neuropathy. Ophthalmology was consulted first in 12, Neurology and Interventional Radiology (IR) in 2 patients each. Mean time from presentation to consultant evaluation was 282 minutes; for Ophthalmology 308 minutes, for Neurology 90 minutes and for IR 315 minutes. Five patients presented within 4.5 hours from their last known well time; one received IV tPA. Conclusion: Acute central RAO is uncommon. Ophthalmology is most frequently consulted for RAO, but without a designated acute treatment plan, most patients are seen relatively late. Future studies must include predefined treatment and faster assessment algorithms and include multiple study centers to recruit patients.

Acute Monocular Visual Loss in an Elderly Woman

Acute Monocular Visual Loss in an Elderly Woman

Approach to the patient with acute monocular visual loss

Acute visual loss in one eye is a common symptom brought to the attention of the practicing neurologist. In this circumstance, it is critical to identify whether visual loss is due to an optic neuropathy or an ocular disorder (especially retinal disease). This review addresses the elements of the history and examination that are useful in evaluating a patient with visual loss, with the goals of correctly localizing the lesion and constructing a likely differential diagnosis.

Complete recovery of acute monocular visual loss following endoscopic resection of anterior clinoid mucocele: Case report and review of the literature

Mucoceles are chronic non-neoplastic cystic lesions lined by mucus-secreting respiratory epithelium in the paranasal sinuses. Mucocele of the anterior clinoid process is a particularly rare entity most often presenting with rapidly progressive monocular blindness. The authors describe the case of a 32 year-old man who presented with acute painless visual loss in the left eye. Workup revealed an expansile lesion of the left anterior clinoid process with associated optic nerve compression. The patient underwent emergent endoscopic-assisted transnasal decompression of the optic nerve with full recovery of visual function. Early diagnosis and prompt surgical intervention optimizes the chances of functional regain of visual acuity.

Monocular visual loss due to herniation of gyrus rectus: A case report with review of literature

The authors present an unusual case of a polar mass in the frontal lobe of the brain, causing acute monocular visual loss in a 50-year-old woman with history of breast carcinoma treated with surgery, radiation and chemotherapy. Neuroimaging demonstrated herniation of the gyrus rectus into the suprasellar cistern resulting in compression of the anterior visual pathway.

Juxtapapillary Cytomegalovirus Retinitis With Optic Neuritis

A 49-year-old man with AIDS developed acute monocular visual loss and an ipsilateral swollen optic disc with a large right relative afferent pupillary defect, a nerve fiber bundle visual field defect, and a peripapillary retinal infiltrate. Lumbar puncture disclosed cytomegalovirus (CMV) DNA on polymerase chain reaction (PCR). Treatment with oral valganciclovir produced complete resolution of the visual deficits and the fundus abnormality. This case differs from previously reported cases of CMV optic neuritis in which visual function has been irreversibly lost.

Anterior clinoid mucocele causing acute monocular blindness

We report a patient who presented with acute painful monocular visual loss reminiscent of idiopathic optic neuritis. After treatment with corticosteroids was commenced, an anterior clinoid mucocele causing compressive optic neuropathy was demonstrated on imaging studies. The diagnosis was proven histopathologically following excision of the lesion via craniotomy. Although very rare as a cause of optic neuropathy, anterior clinoid mucocele should be excluded by adequately imaging the optic canal, as visual prognosis is poor if surgical decompression is not performed in a timely fashion.