Branch Retinal Artery Occlusion Research Articles

Prevalence of SARS-CoV-2 RNA and antibody-detection, and vaccination status in patients with ocular vascular occlusion.

To analyze the annual prevalence of ocular vascular occlusion in relation to COVID-19 infection and vaccination status in a prospective study. All patients were examined for an active SARS-CoV-2 infection by RNA detection, for a previous infection by virus-specific antibody detection (ECLIA), their vaccination status was documented. Data from pandemic year 2020 and previous years, before COVID-19 (2019, 2018, 2017), were retrospectively analyzed. In 2021, a total of 103 patients with the first diagnosis of ocular vascular occlusion were treated. Most frequent sub-diagnoses were central retinal vein occlusion (20.4%), non-arteriitic anterior ischemic optic neuropathy (18.4%), central retinal artery occlusion (13.6%) and branch retinal artery occlusion (12.6%). Thereof, only 3 patients (2.9%) presented with virus-specific SARS-CoV-2-antibodies, none was PCR-positive. Patients with preceded SARS-CoV-2 vaccination (59.2%) presented with comparable characteristics as unvaccinated patients with vascular occlusion regarding age, gender distribution, systemic risk factors, duration of symptoms, visual acuity and the present sub-diagnoses (p>0.05). The total number of cases in 2021 (103 cases) was comparable to the pandemic year 2020, at which no vaccination was available (114 cases), and to earlier years 2017, 2018, and 2019 without COVID pandemic (100, 120 and 119 cases). Furthermore, we did not reveal any differences between pandemic and reference years regarding patients' characteristics (p>0.05). Our study did not reveal an increased annual prevalence of ocular vascular occlusions during COVID-19 pandemic years 2020 and 2021. Patients with previous COVID vaccination did not present differences regarding the risk profile nor symptoms, compared to unvaccinated individuals.

Olfactory function in Susac syndrome

ObjectivesSusac syndrome is a rare autoimmune endotheliopathy involving the brain, retina, and inner ear. Olfactory dysfunction is a common early manifestation of several central nervous system diseases, including neurodegenerative diseases and autoimmune-mediated diseases such as Multiple Sclerosis. While the literature is abundant about the Susac syndrome classic triad of encephalopathy, branch retinal artery occlusion, and low-frequency sensorineural hearing loss, little is known about the extent of olfactory sense involvement. MethodsUsing the Sniffin' Sticks test, this study evaluated olfactory function (identification and threshold) in ten recovering Susac syndrome patients under our clinic surveillance with a median of 3.1 (SD=1.53) years post-disease onset. Resultsolfactory assessment by threshold and odor identification were within the normal range. No differences between recovering Susac syndrome patients to standard norms of odor identification and threshold were found. ConclusionsOur findings do not support olfactory dysfunction in Susac syndrome and thereby, do not support olfactory assessment as a reliable biomarker for this condition.

Asymmetric sickle cell retinopathy in a patient with sickle cell hemoglobin D disease: A case report

Sickle cell disease (SCD), the most commonly inherited hemoglobinopathy, can result in vision loss due to sickle cell retinopathy (SCR), vascular occlusions, and retinal atrophy. SCR is more common in heterozygous (HbSc) than homozygous (HbSS) patients. HbD (Punjab) is a less commonly reported form of hemoglobin in SCD, seen in northwestern states of India. Patients with sickle cell hemoglobin D disease (HbSD) can clinically behave like HbSS. We report a case of asymmetric SCR and multiple branch retinal artery occlusions in a patient with sickle cell hemoglobin D disease in central India.

Susac syndrome: The effectiveness of Rituximab monotherapy

Susac syndrome (SS) or retino-cochleo-cerebral vasculopathy is an extremely rare, severe, and potentially disabling condition. Underlying occlusive microangiopathy in SS is clinically characterized by the triad of encephalopathy, sensorineural hearing loss and branch retinal arterial occlusion. SS therapy envisages simultaneous use of high doses of glucocorticoids, intravenous immunoglobulins, cyclophosphamide and rituximab (RTХ). This article presents a case of remitting-relapsing slow-progressive SS with typical clinical manifestations demonstrating successful treatment SS with RTХ monotherapy; it also discuss the focus of RTХ monotherapy should be targeted at SS cases with contraindications for glucocorticoids and cytostatics use, slow-progressive SS or at early stages.

Positive Prediction Value of Retinal Artery Occlusion Diagnoses in the Danish National Patient Registry: A Validation Study.

The hospital registration of retinal artery occlusions in the Danish National Patient Registry has not previously been validated. In this study, the diagnosis codes were validated to ensure the diagnoses had an acceptable validity for research. The validation was performed both for the overall diagnosis population and at the subtype diagnosis level. The medical records for all patients with retinal artery occlusion with an incident hospital record in the years 2017-2019 in Northern Jutland (Denmark) were assessed in this population-based validation study. Furthermore, fundus images and two-person verification were assessed for the included patients when available. The positive prediction values for the overall diagnosis of retinal artery occlusion, as well as for the central or branch subtypes, were calculated. A total of 102 medical records were available for review. The overall positive prediction value for a retinal artery occlusion diagnosis was 79.4% (95% CI: 70.6-86.1%), while the overall positive prediction value at the subtype diagnosis level was 69.6% (95% CI: 60.1-77.7%), with 73.3% (95% CI: 58.1-85.4%) for branch retinal artery occlusion and 71.2% (95% CI: 56.9-82.9%) for central retinal artery occlusion. For the stratified analyses at the subtype diagnosis, age, sex, diagnosis year, and primary or secondary diagnosis, the positive prediction values ranged from 73.5 to 91.7%. In the stratified analyses at the subtype level, the positive prediction values ranged from 63.3 to 83.3%. The differences among the positive prediction values of the individual strata of both analyses were not statistically significant. the validities of the retinal artery occlusion and subtype level diagnoses are comparable to other validated diagnoses and considered acceptable for use in research.

Retinal branch artery occlusion combined with moyamoya disease in young adult.

Retinal branch artery occlusion combined with moyamoya disease in young adult.

Branch Retinal Artery Occlusion Treated With Intraarterial Tissue-Type Plasminogen Activator Within 4 Hours.

1Department of Ophthalmology, Nanjing Lishui People's Hospital, Zhongda Hospital Lishui branch, Southeast University, Nanjing, Jiangsu Province, China 2Department of Ophthalmology, Jiangsu Province Hospital, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu Province, China *Corresponding author: Yuhua Ding, Jiangsu Province Hospital, The First Affiliated Hospital of Nanjing Medical University, 300 Guangzhou Road, Nanjing 210029. Email: [email protected] Proprietary interest statement: The authors have no conflicts of interest to disclose. Financial support: Not applicable.

Ischemic Stroke Secondary to Middle Cerebral Artery Branch Occlusion From a Thrombosed Saccular Aneurysm

Ischemic Stroke Secondary to Middle Cerebral Artery Branch Occlusion From a Thrombosed Saccular Aneurysm

Encefalomielitis diseminada aguda, síndrome de Susac y astrocitopatía anti-PGFA

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease more common in children than in adults, often preceded by a nonspecific infectious process. It is typically characterized by encephalopathy and multifocal symptoms and signs and, in some cases, may take a more aggressive form such as acute hemorrhagic leukoencephalitis. A substantial number of patients will be positive for IgG-MOG (especially in children), and to a lesser extent for IgG-AQP4. ADEM is usually monophasic, but recurrent and/or persistent seropositive forms will require chronic immunomodulation. Susac syndrome is an inflammatory endotheliopathy characterized by occlusion of retinal arterial branches, hearing loss and encephalopathy, with no known biomarker and with neuroimaging findings amenable to differential diagnosis with multiple sclerosis. Anti-glial fibrillary acidic protein astrocytopathy (PGFA) can present with meningitis, encephalitis, myelitis, papillitis and epilepsy, is paraneoplastic in a third of cases and has an exquisite response to corticosteroids.

Rapidly Progressive, Lethal Microangiopathy of the Brain: Two Cases of Malignant Susac Syndrome? (P9-5.015)

<h3>Objective:</h3> To present two fulminant cases of suspected Susac syndrome refractory to immunomodulatory therapy. <h3>Background:</h3> Susac syndrome (SS) is a presumed immune-mediated endotheliopathy characterized by a triad of encephalopathy, hearing loss, and branch retinal artery occlusions (BRAO). SS may vary in clinical presentation and course and yet typically responds to immunosuppressive therapy. Fatal cases of SS have been rarely reported. <h3>Design/Methods:</h3> NA <h3>Results:</h3> We describe two cases, 15 years apart, of suspected malignant SS. Our patients, a 36-year-old man and a 35-year-old woman presented with several symptoms including encephalopathy, hearing loss, and, in one case, blurred vision. Neither demonstrated BRAO. The first patient had a positive toxicology screen for methamphetamines. The second patient had a history of hypertension and morbid obesity. Serial MRIs demonstrated progressive scattered punctate foci of restricted diffusion throughout the brain, including the cerebrum, corpus callosum, brainstem, and cerebellum. An area of leptomeningeal enhancement was appreciated in our second patient. Extensive workup in both cases failed to yield an alternative diagnosis. Our patients were treated with high-dose intravenous methylprednisolone and intravenous immunoglobulin without improvement. The second patient also received intravenous cyclophosphamide, aspirin, clopidogrel, therapeutic heparin, and rituximab. Both declined in the hospital despite treatment and died shortly following withdrawal of life-sustaining treatments: the man on hospital day 25 and woman on hospital day 42. Autopsy in both cases demonstrated innumerable microscopic and lacunar infarcts of varying ages throughout the brain, interpreted as most consistent with SS. <h3>Conclusions:</h3> These cases highlight an uncommon aggressive variant of suspected SS leading to death within weeks of symptom onset despite intensive therapies. SS poses a diagnostic and treatment dilemma particularly in patients with a fulminant course. <b>Disclosure:</b> Dr. Tierney has nothing to disclose. Dr. Kazanci has nothing to disclose. Dr. Weinstein has received intellectual property interests from a discovery or technology relating to health care. The institution of Dr. Longstreth has received research support from NIH. Dr. Davis has nothing to disclose. Desiree A. Marshall, 431 has nothing to disclose. Dr. Yoda has nothing to disclose.

Long-term Clinical Course and Prognostic Factor of Branch Retinal Artery Occlusion

Purpose: To investigate the long-term clinical course and prognostic factors of branch retinal artery occlusion (BRAO).Methods: The medical records of patients diagnosed with BRAO were reviewed retrospectively. Visual acuity (VA) and central retinal thickness (CRT) at diagnosis were compared with those measured at the final visit. Patients with a decimal VA ≥ 0.6 (good prognosis group) were compared with those with a decimal VA ≤ 0.5 (poor prognosis group) at the final visit.Results: Fifty-five patients were enrolled and the mean follow-up period was 45.8 ± 27.8 months. The mean logarithm of minimum angle of resolution improved from 0.53 ± 0.57 at diagnosis to 0.36 ± 0.61 at the final visit (&lt;i&gt;p&lt;/i&gt; = 0.026). The decimal VA was ≤ 0.1 in 13 (23.6%) patients, ≥ 0.2 and ≤ 0.5 in 16 (29.1%) patients, and ≥ 0.6 in 26 (47.3%) patients at diagnosis; the respective values were 9 (16.4%), 8 (14.5%), and 38 (69.1%) at the final visit. The mean CRT significantly decreased from 273.9 ± 34.7 µm at diagnosis to 248.9 ± 27.0 µm at the final visit (&lt;i&gt;p&lt;/i&gt; &lt; 0.001). The poor prognosis group (n = 17) was older (&lt;i&gt;p&lt;/i&gt; = 0.044) and had a higher incidence of papillomacular bundle involvement (&lt;i&gt;p&lt;/i&gt; &lt; 0.001) than the good prognosis group (n = 38).Conclusions: Patients with BRAO generally showed relatively favorable long-term outcomes. However, the final VA was ≤ 0.1 in 16.4% of them, suggesting the need for further treatment modalities to improve the outcome of patients with a poor prognosis.

OPHTHALMOLOGIST AS GATEKEEPERS FOR STROKE PREVENTION – A CASE STUDY

Stroke is a major global public health problem and the prevalence ranged from 44.29 to 559/100,000 persons in different parts of India1. Ophthalmologists come across many patients with retinal artery occlusion who are unaware of harbouring a signicant stenosis in the carotid arteries. In our case report, a 78-year old male having multiple associated systemic diseases presented to the outpatient department with the complaint of vision loss in one eye and was diagnosed with Branch Retinal Artery Occlusion (BRAO). We advised him to go immediately for carotid doppler study and systemic evaluation. Stenosis of carotid arteries were noted which was more on the same side of occlusion in the eye, we urgently referred him to a higher centre and he underwent angioplasty for the stenosis. As per our knowledge, despite the guidelines for referral there is no evident literature on the percentage of such cases being referred by Ophthalmologists to higher centres in India. This case study aims to promote research in this area and attract attention of Ophthalmologists in their critical role in dealing with patients with high risk of Stroke.

Branch retinal artery occlusion in a case of rhino-orbital-cerebral mucormycosis

Branch retinal artery occlusion in a case of rhino-orbital-cerebral mucormycosis

RWC Update: RWC Young Retina Connect; Vitreoretinal Surgery in Uveitis; Ruptured Retinal Arterial Macroaneurysm With Secondary Branch Retinal Artery Occlusion

RWC Update: RWC Young Retina Connect; Vitreoretinal Surgery in Uveitis; Ruptured Retinal Arterial Macroaneurysm With Secondary Branch Retinal Artery Occlusion

Risk and risk factor of ischemic stroke after acute retinal arterial ischemia.

We aimed to confirm the risk and risk factor for ischemic stroke after acute retinal arterial ischemia (ARAI). A retrospective cohort study of patients with a diagnosis of acute retinal arterial ischemia (ARAI) and completing 2-year follow-up was conducted from January 2015 to December 2021 at a general hospital. A total of 69 patients including 43(62.3%) patients of central retinal artery occlusion (CRAO), 11(15.9%) patients of branch retinal artery occlusion (BRAO) and 15(21.7%) patients of ophthalmic artery occlusion (OAO) were included in the study. Patients age was 58.2 ± 13.0(years), male patients accounting for 51 (73.9%) and 22 (31.9%) patients having at least 70% ipsilateral carotid artery stenosis (ICAS). During the 2-years follow-up period, 11(15.9%) patients of ARAI experienced ischemic stroke. Among them, 3(20%) patients of OAO, 6(14%) patients of CRAO and 2(18.2%) patients of BRAO had ischemic stroke. The cumulative probabilities of ischemic stroke were 13.0% at 12.9months and 15.9% at 24months after ARAI. In addition, patients with at least 70% ICAS were more likely than patients without it to have ischemic stroke (p = 0.002). After Cox regression analysis, ICAS (≥ 70%) or occlusion was significantly associated with a high risk of ischemic stroke after ARAI during the 2-years follow-up time (HR,6.769,95%CI [1.792-25.578], p = 0.005). Patients have a high risk of ischemic stroke, particularly those with a diagnosis of ICAS (≥ 70%) or occlusion after the onset of ARAI. Clinical management of ARAI should focus on vascular risk factors control and secondaryprevention for stroke.

Multiple branch retinal artery occlusions following the new facial cosmetic filler (Poly-D, L-lactic Acid) injection a case report

BackgroundPoly-D, L-lactic acid is (PDLLA) a new cosmetic filler. We reported the first case of PDLLA-related devastating complication of multiple branch retinal artery occlusion (BRAO).Case presentationA 23-year-old female had sudden blindness after injection of PDLLA at the glabella. After emergency intraocular pressure-lowering medicine, ocular massage, steroid pulse therapy, heparin and alprostadil infusion, and subsequent treatments including acupuncture and 40 sessions of hyperbaric oxygen therapy, her best-corrected visual acuity improved from hand motion at 30 cm to 0.3 within 2 months.ConclusionAlthough safety of PDLLA was evaluated in animal studies and in 16,000 human cases, it could still cause rare but devastating retinal artery occlusion as in the present case. Proper and immediate therapies could still improve patient’s vision and scotoma. Surgeons should keep in mind the possibility of iatrogenic filler-related retinal artery occlusion.

Abstract Number ‐ 217: Case Study: Snowball strokes

Introduction 55 year old RH female, PMH of hypothyroidism and gastric bypass who presented with complaints of 1–2 weeks of “feeling foggy in the head”, dizziness, and unsteady gait. The patient’s husband said she had delayed responses. She denied headache, vision change, or hearing loss. Exam was without focal neurologic deficit, NIHSS of 0.Though CTA head and neck were unremarkable, MRI Brain showed multiple scattered small acute or subacute infarcts involving the corpus callosum. This distribution was thought to suggest a central embolic source. The patient was admitted for stroke workup which was unrevealing including: Normal echocardiogram including bubble study. Her symptoms progressed and she was unable to find outpatient follow up. Two weeks later, she presented again but this time with progressive generalized weakness. At the time of her initial discharge, the patient was ambulatory with her walker, however upon re‐admission the patient required a wheelchair. Exam showed flaccid bilateral lower extremities, brisk reflexes, bilateral Hoffman’s and Babinski signs.Repeat MRI Brain was concerning for Susac syndrome with new lesions in the corpus callosum. CSF showed pleocytosis with markedly elevated protein (WBC 8, protein 153, glucose 55). Treatment with Solu‐Medrol 1000 mg q12h IV, then IVIG 0.4 kg/mg/day both for 5 days, Rituximab 1000mg for 2 days, then transition to prednisone 80 mg /day and CellCept 1000mg BID. She returned 14 days after with confusion, regression of prior gait improvement, and repeat MRI Brain showed additional new multifocal strokes. She repeated 3 days of 1000mg IV Solu‐Medrol, then was treated with cyclophosphamide due to clinical progression. Fluorescein angiography showed evidence of branch retinal artery occlusions bilaterally. She was placed on a prednisone taper from 80mg/day to 40mg/day over the course of the 2 months, with the plan to continue Cellcept 1000mg BID for 2 years. Methods NA‐ Single case study Results NA‐Single case study Conclusions Susac syndrome is an autoimmune disorder, affecting microvasculature of the brain, retina and cochlea. A triad of symptoms: encephalopathy, branch retinal artery occlusions, and hearing loss is classic, though only 13% have the complete triad at onset. It is often associated with headaches, psychiatric features (paranoia), bilateral cochlear hearing loss, and vertigo. It typically occurs in women 20–40 years of age. Brain MRI WO shows white‐matter disturbance, frequently mistaken for Multiple Sclerosis, however the main difference is that the corpus callosum is always affected with lesions that resemble “snowballs”, or “holes”. Treatment for Susac syndrome is immunosuppression, with high‐dose corticosteroids, also with IVIG, Mycophenolate mofetil, and Cyclophosphamide.

RETINAL FLUORESCEIN ANGIOGRAPHY IN SUSAC SYNDROME: A SHIFTING PATTERN OF ARTERIOLAR WALL HYPERFLUORESCENCE-A REPORT OF TWO CASES.

Susac syndrome consists of the triad of encephalopathy, branch retinal artery occlusion, and hearing loss and is believed to be an immune-mediated endotheliopathy. Arteriolar wall hyperfluorescence (AWH) on retinal fluorescein angiography (FA) has been described in the literature as a classic ophthalmic sign for Susac syndrome. In this article, we report a pattern of distally shifting segments of AWH on FA over time that was observed in two cases. Two cases of Susac syndrome are presented with an emphasis on FA findings, obtained at frequent intervals during follow-up. In both cases, FA displays a shifting pattern of segments of AWH distal to the localization of the AWH observed on the previous FA. In patients with Susac syndrome, FA plays an important role in aiding diagnosis and monitoring the efficacy of treatment and disease activity during follow-up. In these two cases, the angiography revealed how the AWH can migrate along the vessel over time. This is the first published report showing an evolving distally shifting pattern of AWH in patients with Susac syndrome.

Abstract Number ‐ 148: Emergent Carotid Artery Stenting On Acute Stroke Patients With Carotid Occlusion: Benefit Or Harm?

Introduction Almost one out of four patients with acute middle cerebral artery occlusion also have ipsilateral internal carotid artery occlusion (ICAO). The interventions for acute stroke due to extracranial ICAO with or without intracranial occlusions are still a challenge. Case series reported early revascularization reduced stroke recurrence and improved outcomes. The benefits of this intervention on hyperacute ischemic strokes (within 6 hours) were much less known. We reported here two hyperacute stroke patients who emergent CAS on ICAO. Methods Electronic medical charts were reviewed, assessing intracranial hemorrhage (ICH) in two hyperacute stroke patients resulting from emergent carotid artery stenting (CAS) on the occluded internal carotid artery (ICA). Results Case description:The first patient was a 60‐year‐old male who had acute right hemiparesis, aphasia, and left gaze deviation with NIHSS12. The last known normal was five hours ago. Head and neck CT angiography (CTA) showed left anterior M2 branch artery occlusion and left ICAO. Head CT perfusion (CTP) showed a small core infarct with a large perfusion mismatch. Emergent CAS was performed without distal embolic protection (DEP) and followed by distal mechanical thrombectomy (MT). TICI 2B recanalization was achieved. After CAS, aspirin and clopidogrel were administrated. He had a large left MCA and PCA stroke from fetal PCA. A few days later, the patient developed large intraparenchymal hemorrhage (IPH) and intraventricular hemorrhage (IVH). He expired shortly. The second was a 52‐year‐old male had acute right facial droop, aphasia, dysarthria, and decreased consciousness (NIHSS 8). CTA showed left ICAO but patent intracranial arteries. CT perfusion showed a large mismatch without core infarction. He received intravenous tPA and had emergent CAS with a DEP. Aspirin 600 mg was administrated afterward. A few hours later, he had worsened weakness. Head CT showed left IPH, IVH, and subarachnoid hemorrhages with cerebral edema, and midline shift. He was medically managed for a prolonged stay and was discharged to a rehabilitation facility. Conclusions We presented two consecutive cases of emergent revascularization of ICAO in hyperacute stroke carried a high risk of ICH with poor outcome. Our online database search found that only a few case series of emergent CAS on ICAO were reported. Overall, emergent CAS carried about 20% risk of ICH and high mortality. Other series reported angioplasty on stenotic or occluded cervical ICA lesions with MT on distal occlusions had less hemorrhagic risk because there was no need fordual antiplatelet treatment. Most emergent CAS cases were performed on tandem occlusions for faster direct access and better efficacy of distal recanalization. A futurestudy comparing hemorrhagic risk betweenemergent CAS versus angioplasty of ICAO in patients with tandem occlusions can help to establish a standard MT protocol. For isolated ICAO with patent intracranial arteries from good collaterals, CTP may not be a good guidance tool for decision‐making of emergent CAS as it can falsely show mismatch from existing collaterals due to occlusion. A randomized clinical trial of comparison of medical management versus emergent CAS on those patients is warranted.

RWC Update: DRCR Retina Network Protocol AC, Step Therapy, and Clinical Practice Implications; Central Retinal Vein Occlusion with Secondary Branch Retinal Artery Occlusion and Incidental Myelinated Nerve Fiber Layer and CHRPE

RWC Update: DRCR Retina Network Protocol AC, Step Therapy, and Clinical Practice Implications; Central Retinal Vein Occlusion with Secondary Branch Retinal Artery Occlusion and Incidental Myelinated Nerve Fiber Layer and CHRPE