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1.
Ophthalmol Retina ; 8(6): 566-570, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38154618

ABSTRACT

PURPOSE: To assess visual acuity (VA) outcomes in a large cohort of patients diagnosed with nonarteritic central retinal artery occlusion (CRAO), and to ascertain whether time from symptom onset to presentation, presenting VA, or conservative treatment delivery (anterior chamber paracentesis, ocular massage, intraocular pressure lowering drugs, hyperventilation, or some combination of those) impacted ultimate VA outcomes. DESIGN: Retrospective cohort study. SUBJECTS: The study included 794 patients who presented with CRAO between 2011 and 2020. Within this cohort, 484 individuals presented within 30 days of symptom onset and had comprehensive documentation regarding the details of their presentation, management, and follow-up ≥ 90 days postdiagnosis. METHODS: Retrospective chart review was conducted for all patients with a diagnosis of CRAO initially identified via International Classification of Diseases coding, followed by confirmation of diagnosis by 2 retina specialists. Cases of arteritic CRAO were excluded. MAIN OUTCOME MEASURES: Visual acuity recovery, defined as improvement from ≤ 20/200 or worse at presentation to ≥ 20/100 ≥ 90 days after diagnosis. RESULTS: Of the 794 identified patients, 712 (89.7%) presented with VA of ≤ 20/200. Similarly, 447 (92.4%) of the 484-patient subset that presented within 30 days and had comprehensive documentation presented with VA ≤ 20/200. Of the 441 of those patients with documented follow-up, 380 (86.2%) remained at that level. Of the 244 patients who presented within 4.5 hours of symptom onset, 227 (93%) presented ≤ 20/200 and 201 (92.6%) of the 217 of those with follow-up data did not improve beyond that threshold. There was no significant difference (P < 0.05) in final VA between patients presenting before versus after 4.5 hours from time of vision loss. There was also no significant difference (P < 0.05) in VA outcomes between patients who did or did not receive conservative treatment. CONCLUSIONS: This large retrospective study further highlights the poor visual prognosis for patients with CRAO. Earlier time to presentation did not seem to impact final VA outcome, nor did conservative treatment efforts. Efficacious evidence-based treatment options are needed for this patient population. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.


Subject(s)
Retinal Artery Occlusion , Visual Acuity , Humans , Retinal Artery Occlusion/diagnosis , Retinal Artery Occlusion/physiopathology , Retinal Artery Occlusion/therapy , Retrospective Studies , Male , Female , Aged , Middle Aged , Follow-Up Studies , California/epidemiology , Aged, 80 and over
2.
Article in English | MEDLINE | ID: mdl-38117865

ABSTRACT

PURPOSE: To describe a patient who developed retinal degeneration associated with autoimmune retinopathy (AIR) and who was also found to have anti-glutamic acid decarboxylase (GAD65) autoantibodies and the diagnosis of stiff-person syndrome (SPS). METHODS: Ophthalmologic workup consisted of clinical examination, multi-modality retinal imaging, and electrophysiologic testing. Further neurologic assessment including relevant serum and cerebrospinal fluid studies was also conducted. RESULTS: We highlight the case of a 45-year-old patient who developed subacute, sequential vision loss, along with bilateral lower extremity weakness. On initial presentation, optical coherence tomography (OCT) of the left eye was notable for diffuse attenuation of the outer retinal bands. Fundus autofluorescence demonstrated a ring of hyper-autofluorescence encircling the fovea of the left eye. At fifteen-month follow-up, the right eye also became similarly affected. He was found to have elevated serum and cerebrospinal anti-GAD65 autoantibodies and was diagnosed with both SPS and AIR. CONCLUSION: There is a potential association of anti-GAD65 autoantibodies with the development of AIR.

3.
J Neuroophthalmol ; 43(4): 491-498, 2023 Dec 01.
Article in English | MEDLINE | ID: mdl-37974364

ABSTRACT

BACKGROUND: Parainfectious optic neuritis is an inflammatory reaction that occurs shortly after an infection without direct invasion by a pathogen. The clinical profile depends on the infectious organism. Cases of SARS-CoV-2 parainfectious optic neuritis have been reported in the literature, but there are no reviews that have applied strict inclusion criteria to more definitively establish the clinical profile associated with SARS-CoV-2. METHODS: We present 3 new cases of SARS-CoV-2 parainfectious optic neuritis. We also review the literature for definite cases by selecting only those with unambiguous clinical features and MRI findings of optic neuritis, positive SARS-CoV-2 polymerase chain reaction or serology, and the absence of myelin oligodendrocyte-glycoprotein or aquaporin-4 antibodies or other diseases associated with optic neuritis. RESULTS: We report 2 cases of monophasic, unilateral SARS-CoV-2 parainfectious optic neuritis with optic disc edema and nadir visual acuities of finger counting. We report 1 case of mild SARS-CoV-2 parainfectious optic neuritis that featured cotton wool spots, peripapillary wrinkles and hemorrhages, and recurrence after an initial steroid taper. We identified 6 cases of unambiguous SARS-CoV-2 parainfectious optic neuritis from the literature. Combining our case series with the case reports in the literature, the average age was 42.8 years, 3/9 had bilateral disease, 6/8 had optic disc edema, 8/9 had nadir visual acuity of finger counting or worse, and all recovered visual acuity to 20/40 or better after therapy with steroids. CONCLUSIONS: SARS-CoV-2 parainfectious optic neuritis has a clinical profile that is atypical for idiopathic optic neuritis but fairly typical of parainfectious forms of optic neuritis with a severely reduced nadir visual acuity, high likelihood of bilaterality, high incidence of optic disc edema, and prompt and significant response to corticosteroids. Further study with long-term follow-up and epidemiologic investigation will be needed to further characterize this clinical entity.


Subject(s)
COVID-19 , Optic Nerve Diseases , Optic Neuritis , Papilledema , Humans , Papilledema/etiology , Papilledema/complications , SARS-CoV-2 , Retrospective Studies , COVID-19/complications , Optic Nerve Diseases/etiology , Optic Nerve Diseases/complications , Optic Neuritis/diagnosis , Optic Neuritis/drug therapy , Optic Neuritis/etiology , Vision Disorders/diagnosis , Vision Disorders/etiology
4.
JAMA Ophthalmol ; 141(6): 602-603, 2023 06 01.
Article in English | MEDLINE | ID: mdl-37140908

ABSTRACT

A 72-year-old woman with a history of kidney transplant was referred for an eye examination because of visual changes in the left eye. Both optic discs were anomalous in appearance, with central excavation in the left eye greater than the right eye. What would you do next?


Subject(s)
Kidney Transplantation , Vision, Ocular , Aged , Humans , Kidney Transplantation/adverse effects
5.
Ophthalmol Retina ; 7(6): 527-531, 2023 06.
Article in English | MEDLINE | ID: mdl-36639057

ABSTRACT

OBJECTIVE: To evaluate the presentation patterns of patients diagnosed with central retinal artery occlusion (CRAO) from 2011 to 2020. DESIGN: Retrospective cohort study SUBJECTS: The present study was conducted in 484 patients presenting within 30 days of symptom onset with accurate documentation of time of symptom onset, time of presentation to the health care system, and time of presentation to an ophthalmologist. METHODS: An independent chart review of patients with CRAO was conducted. MAIN OUTCOME MEASURES: Demographic information including age, sex, and race were collected. Presentation patterns such as time of first symptoms, time of first contact with the health care system, and time of evaluation by an ophthalmologist were analyzed. Additionally, information regarding the medical venue or specialty of initial patient contact was collected. RESULTS: A total of 247 (51%) patients contacted the health care system within 4.5 hours of system onset, whereas 86 (17.8%) patients waited over 24 hours. Only 81 (32.8%) of the 247 patients who presented within 4.5 hours saw an ophthalmologist within that time frame, whereas 172 (35.5%) of the entire cohort of 484 did not present to an ophthalmologist within 24 hours of vision loss. There was significant variability with regards to medical specialty of initial patient contact, with 292 (60.3%) patients first presenting to an emergency department and 133 (27.5%) patients first presenting to an ophthalmologist. Black and Hispanic patients presented later than patients of White, Asian, or other racial backgrounds (40.4 ± 10.2 hours versus 23.0 ± 3.4 hours, P = 0.05). CONCLUSIONS: Although no level 1 evidence-based treatment is currently available for CRAO, thrombolytic therapy may be promising. Even though over half of patients with CRAO within our institution connected with the health care system within a potential window for thrombolytic therapy, most did not receive a definitive ophthalmic diagnosis within that time frame. Public health educational campaigns and infrastructure optimization must speed up presentation times, decrease the time to ophthalmic diagnosis, and target vulnerable populations to offer and research timely administration of thrombolytic therapy. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any materials discussed in this article.


Subject(s)
Retinal Artery Occlusion , Humans , Retrospective Studies , Retinal Artery Occlusion/diagnosis , Retinal Artery Occlusion/drug therapy , Thrombolytic Therapy , Visual Acuity
6.
Surv Ophthalmol ; 68(4): 821-829, 2023.
Article in English | MEDLINE | ID: mdl-36150481

ABSTRACT

A 43-year-old woman presented with decreased vision in the right eye associated with painful eye movements 10 days after receiving her first dose of Pfizer-BioNTech coronavirus disease 2019 (COVID-19) vaccine (Pfizer Inc, New York, NY). Two days later she developed painful loss of vision in the left eye. Clinical presentation and magnetic resonance imaging findings were consistent with bilateral optic perineuritis transitioning to optic neuritis. Extensive evaluation including aquaporin-4 immunoglobin G (IgG), myelin oligodendrocyte glycoprotein IgG, and lumbar puncture was unrevealing. Visual acuity at nadir was counting fingers in both eyes, but after receiving intravenous steroids and plasma exchange vision eventually improved to 20/20 in each eye, although she was left with inferior visual field defects and bilateral optic disc pallor. This case highlights the diagnostic challenge in the evaluation of atypical optic neuritis with a review of post-COVID-19 vaccination-associated optic neuritis.


Subject(s)
COVID-19 , Optic Neuritis , Female , Humans , COVID-19 Vaccines/adverse effects , Immunoglobulin G/therapeutic use , Optic Nerve , Optic Neuritis/diagnosis , Optic Neuritis/etiology , Vision Disorders , Adult
7.
J Neurol Sci ; 437: 120262, 2022 06 15.
Article in English | MEDLINE | ID: mdl-35489173

ABSTRACT

OBJECTIVE: To investigate the clinical and magnetic resonance imaging (MRI) characteristics of patients with varicella zoster virus (VZV) reactivation involving the cranial nerves and central nervous system (CNS). METHODS: This is a retrospective, multi-center case-series of 37 patients with VZV infection affecting the cranial nerves and CNS. RESULTS: The median age was 71 years [IQR 51.5-76]; 21 (57%) were men. Cerebrospinal fluid (CSF) was available in 24/37 (65%); median CSF white blood cell count was 11 [IQR 2-23] cells/µL and protein was 45.5 [IQR 34.5-75.5] mg/dL. VZV polymerase chain reaction (PCR) assays were positive in 6/21 (29%) CSF and 8/9 (89%) ocular samples. Clinical involvement included the optic nerve in 12 (32%), other cranial nerves in 20 (54%), brain parenchyma in 12 (32%) and spinal cord or nerve roots in 4 (11%). Twenty-seven/28 immunocompetent patients' MRIs were available for review (96%). Of the 27, 18 had T1 postcontrast fat saturated sequences without motion artifact to evaluate for cranial nerve enhancement and optic perineuritis (OPN). Eight/18 (44%) demonstrated OPN. All 8 experienced vision loss: 3 optic neuritis, 1 acute retinal necrosis, and 3 CNS vasculitis with 1 central and 1 branch retinal artery occlusion and 1 uveitis. Diplopic patients had cranial nerve and cavernous sinus enhancement. All immunosuppressed patients were imaged. Seven/9 (88%) had extensive neuraxis involvement, including encephalitis, vasculitis and transverse myelitis; one case had OPN. CONCLUSION: OPN is a frequent manifestation in VZV-associated vision loss among immunocompetent patients. Immunosuppressed patients had greater neuraxis involvement. Optimizing MRI protocols may improve early diagnosis in VZV reactivation.


Subject(s)
Encephalitis, Varicella Zoster , Encephalitis , Herpes Zoster , Aged , Central Nervous System/pathology , Encephalitis, Varicella Zoster/complications , Encephalitis, Varicella Zoster/diagnostic imaging , Female , Herpesvirus 3, Human/genetics , Humans , Male , Polymerase Chain Reaction , Retrospective Studies
8.
Curr Opin Ophthalmol ; 32(6): 499-503, 2021 Nov 01.
Article in English | MEDLINE | ID: mdl-34419978

ABSTRACT

PURPOSE OF REVIEW: The COVID-19 public health emergency accelerated the adoption of telemedicine in neuro-ophthalmology. This review surveys the current telemedicine landscape and discusses associated limitations and opportunities. RECENT FINDINGS: Logistic considerations and regulatory changes related to the COVID-19 pandemic have spurred a proliferation of tele-neuro-ophthalmic practice. One significant benefit of telemedicine is increased access to the limited number of neuro-ophthalmologists in the country. Certain elements of the neuro-ophthalmic examination can be ascertained during a video visit or through the use of mobile applications. However, data quality can be limited and more direct evaluation of the fundus currently requires the implementation of imaging techniques, such as fundus photography and/or optical coherence tomography. For cases that require it, a 'hybrid' model can be adopted in which patients physically present to designated locations for testing and subsequently participate in televisit with the physician to discuss the assessment and plan. SUMMARY: Telemedicine provides an alternate pathway for patients to access the limited resource of neuro-ophthalmic care, and it will likely persist beyond the current COVID-19 pandemic. It has some limitations currently but continued progress in technologic, legal, and reimbursement strategies will hopefully facilitate further adoption.


Subject(s)
COVID-19 , Ophthalmology , Telemedicine , Humans , Pandemics , SARS-CoV-2
9.
Life (Basel) ; 11(6)2021 May 27.
Article in English | MEDLINE | ID: mdl-34071738

ABSTRACT

The purpose of this study is to identify salient magnetic resonance imaging (MRI) findings of pediatric IIH, to determine the relevance of these findings with regard to disease pathogenesis, and to relate these findings to the clinical presentation towards identification of risk factors of disease. A retrospective, a case-control study of 38 pediatric patients with and 24 pediatric patients without IIH from the ophthalmology department at a tertiary care center was performed. Clinical data, including ophthalmic findings and lumbar puncture results, were recorded. Neuroimaging, including both MRI and magnetic resonance venography (MRV), was evaluated for perioptic subarachnoid space diameter enlargement, posterior globe flattening, optic nerve head protrusion, empty or partially empty sella turcica, dural venous sinus abnormalities, skull base crowding, and prominent arachnoid granulations. Compared with controls, IIH patients had larger perioptic subarachnoid space diameters, higher incidences of posterior globe flattening, protrusion of the optic nerve heads, an empty sella turcica, and dural venous sinus abnormalities. A perioptic subarachnoid space diameter of ≥5.2 mm was identified as an independent predictor of IIH (p < 0.001) with sensitivity of 87% and specificity of 67%. Several significant MRI findings in pediatric IIH were identified. Using a model that uniquely incorporated clinical and MRI findings at presentation, we provide a framework for risk stratification for the diagnosis of pediatric IIH which may be utilized to facilitate diagnosis. Future prospective work is needed to further validate the model developed in this study.

10.
J Neuroophthalmol ; 41(2): 217-223, 2021 Jun 01.
Article in English | MEDLINE | ID: mdl-32235230

ABSTRACT

BACKGROUND: Addison disease, corticosteroid withdrawal, and taking synthetic growth hormone have been linked with development of intracranial hypertension, but there is still debate on whether administration of other exogenous hormones plays a role in precipitating elevated pressure. The growing use of hormonal therapy for gender affirmation provides an opportunity to explore this possibility. METHODS: All transgender patients taking exogenous hormones for female-to-male (FTM) and male-to-female (MTF) transitions who were diagnosed with intracranial hypertension at Massachusetts Eye and Ear Infirmary, Massachusetts General Hospital and Beth Israel Deaconess Medical Center between August 2014 and November 2018 were included in a retrospective review. Visual acuity, type, and dose of exogenous hormone, visual field testing, clinical exam, results of neuroimaging and lumbar puncture, and treatment modalities were catalogued and analyzed. RESULTS: Six transgender individuals were identified. Five were FTM, with an average hormone treatment time of 18.4 months, and one was MTF who had been treated with hormones for 4 years. The average age of all patients was 23.5 years. The average time between onset of symptoms and presentation was 5 months. Fifty percent of the patients reported pulse-synchronous tinnitus, 83% reported positional headache, 33% reported transient visual obscurations, and 16% reported diplopia. Lumbar punctures performed on 4 of the patients revealed elevated opening pressures and normal cerebrospinal fluid constituents. MRI findings consistent with elevated intracranial pressure (ICP) were present in the other 2 patients in whom lumbar puncture was unsuccessful. Four patients were treated with acetazolamide and one was treated with topiramate, with an average follow-up time of 15.7 months. All patients demonstrated bilateral optic disc swelling, and all maintained normal acuity and color vision. Performance on visual field testing was not significantly affected in any patient. CONCLUSIONS: This is the largest reported series to date of gender-transitioning patients with intracranial hypertension, including one novel MTF conversion. These observations warrant further investigation into the possible link of exogenous hormonal therapy and elevated ICP and any mechanisms or confounders underlying this potential association.


Subject(s)
Gonadal Steroid Hormones/adverse effects , Intracranial Hypertension/chemically induced , Intracranial Pressure/drug effects , Sex Reassignment Procedures/methods , Transgender Persons , Adult , Female , Humans , Intracranial Hypertension/physiopathology , Male , Retrospective Studies , Young Adult
11.
J Neuroophthalmol ; 41(1): 24-28, 2021 03 01.
Article in English | MEDLINE | ID: mdl-31985565

ABSTRACT

BACKGROUND: Embolic events leading to retinal ischemia or cerebral ischemia share common risk factors; however, it has been well documented that the rate of concurrent cerebral infarction is higher in patients with a history of transient ischemic attack (TIA) than in those with monocular vision loss (MVL) due to retinal ischemia. Despite the fact that emboli to the ophthalmic artery (OA) and middle cerebral artery share the internal carotid artery (ICA) as a common origin or transit for emboli, the asymmetry in their final destination has not been fully explained. We hypothesize that the anatomic location of the OA takeoff from the ICA may contribute to the differential flow of small emboli to the retinal circulation vs the cerebral circulation. METHODS: We report a retrospective, comparative, case-control study on 28 patients with retinal ischemia and 26 patients with TIA or cerebral infarction caused by embolic events. All subjects underwent either computed tomography angiography or MRA. The location of the ipsilateral OA origin off the ICA was then graded in a blinded fashion and compared between cohorts. Vascular risk factors were collected for all patients, including age, sex, hypertension, hyperlipidemia, arrhythmia, diabetes, coronary artery disease, and smoking. RESULTS: We find that in patients with retinal ischemia of embolic etiology, the ipsilateral OA takeoff from the ICA is more proximal than in patients with cerebral infarcts or TIA (P = 0.0002). We found no statistically significant differences in demographic, vascular, or systemic risk factors. CONCLUSIONS: We find that the mean anatomical location of the OA takeoff from the ICA is significantly more proximal in patients with MVL due to retinal ischemia compared with patients with TIA or cerebral ischemia. This finding contributes significantly to our understanding of a long observed but poorly understood phenomenon that patients with MVL are less likely to have concurrent cerebral ischemia than are patients with TIA.


Subject(s)
Embolism/etiology , Intracranial Embolism/etiology , Ophthalmic Artery/anatomy & histology , Retinal Artery/pathology , Aged , Brain Ischemia/diagnostic imaging , Brain Ischemia/etiology , Carotid Artery, Internal/anatomy & histology , Case-Control Studies , Computed Tomography Angiography , Embolism/diagnostic imaging , Female , Humans , Intracranial Embolism/diagnostic imaging , Ischemia/diagnostic imaging , Ischemia/etiology , Magnetic Resonance Angiography , Male , Middle Aged , Retinal Artery/diagnostic imaging , Retinal Diseases/diagnostic imaging , Retinal Diseases/etiology , Retrospective Studies , Risk Factors
12.
Neurology ; 95(2): e111-e120, 2020 07 14.
Article in English | MEDLINE | ID: mdl-32554760

ABSTRACT

OBJECTIVE: Myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) associated disorder (MOGAD) often manifests with recurrent CNS demyelinating attacks. The optimal treatment for reducing relapses is unknown. To help determine the efficacy of long-term immunotherapy in preventing relapse in patients with MOGAD, we conducted a multicenter retrospective study to determine the rate of relapses on various treatments. METHODS: We determined the frequency of relapses in patients receiving various forms of long-term immunotherapy for MOGAD. Inclusion criteria were history of ≥1 CNS demyelinating attacks, MOG-IgG seropositivity, and immunotherapy for ≥6 months. Patients were reviewed for CNS demyelinating attacks before and during long-term immunotherapy. RESULTS: Seventy patients were included. The median age at initial CNS demyelinating attack was 29 years (range 3-61 years; 33% <18 years), and 59% were female. The median annualized relapse rate (ARR) before treatment was 1.6. On maintenance immunotherapy, the proportion of patients with relapse was as follows: mycophenolate mofetil 74% (14 of 19; ARR 0.67), rituximab 61% (22 of 36; ARR 0.59), azathioprine 59% (13 of 22; ARR 0.2), and IV immunoglobulin (IVIG) 20% (2 of 10; ARR 0). The overall median ARR on these 4 treatments was 0.3. All 9 patients treated with multiple sclerosis (MS) disease-modifying agents had a breakthrough relapse on treatment (ARR 1.5). CONCLUSION: This large retrospective multicenter study of patients with MOGAD suggests that maintenance immunotherapy reduces recurrent CNS demyelinating attacks, with the lowest ARR being associated with maintenance IVIG therapy. Traditional MS disease-modifying agents appear to be ineffective. Prospective randomized controlled studies are required to validate these conclusions.


Subject(s)
Immunotherapy/methods , Myelin-Oligodendrocyte Glycoprotein/immunology , Steroids/therapeutic use , Adolescent , Adult , Age of Onset , Antirheumatic Agents/therapeutic use , Child , Child, Preschool , Demyelinating Diseases/immunology , Demyelinating Diseases/prevention & control , Demyelinating Diseases/therapy , Female , Humans , Immunization, Passive , Immunosuppressive Agents/therapeutic use , Male , Middle Aged , Multiple Sclerosis/complications , Multiple Sclerosis/therapy , Recurrence , Retrospective Studies , Steroids/administration & dosage , Young Adult
13.
Am J Ophthalmol Case Rep ; 17: 100594, 2020 Mar.
Article in English | MEDLINE | ID: mdl-32395666

ABSTRACT

PURPOSE: To present a case of glioblastoma multiforme which initially presented with only ophthalmic manifestations. OBSERVATIONS: A 48-year-old man presented with decreased vision and pain with eye movements of the right eye. MRI of the brain showed increased T2/FLAIR signal involving the right optic nerve with no other identified abnormalities. He was treated with intravenous steroids for presumed optic neuritis. His visual acuity then rapidly worsened to no light perception, with new orbital apex symptoms including central retinal artery and vein occlusions and inferior division third and fourth nerve palsies. Repeat MRI with contrast showed perineural enhancement surrounding the right optic nerve and markedly reduced diffusion along its entire course. After an unrevealing initial workup and then a 7 month period during which the patient refused follow up, he re-presented with left sided weakness, headache, and confusion. Repeat brain MRI revealed a large mass involving the right optic nerve, optic chiasm, basal ganglia, corpus callosum and brainstem. Biopsy led to a diagnosis of WHO grade IV glioblastoma multiforme. The disease was poorly responsive to temozolomide, bevacizumab and external beam radiation, and the patient passed away 5 months later. CONCLUSIONS AND IMPORTANCE: Malignant optic glioma of adulthood is a challenging diagnosis with a poor prognosis. This rare case highlights the importance of maintaining neoplasm in the differential for optic neuritis masqueraders.

14.
J AAPOS ; 23(5): 283-285, 2019 10.
Article in English | MEDLINE | ID: mdl-31520721

ABSTRACT

We analyzed clinical and histopathologic data of 97 pediatric patients who underwent excision of dermoid cysts. On review, 16.5% of the sample population demonstrated localized chronic inflammatory changes, including the presence of giant cells and epithelial disruption. These features were considered indicative of prior cyst rupture. Age at time of initial presentation was significantly older and cyst size was significantly larger in patients with histopathologic signs of previous rupture. Longer time to presentation and time to excision were associated with increased odds of spontaneous rupture.


Subject(s)
Dermoid Cyst/pathology , Orbital Neoplasms/pathology , Rupture, Spontaneous/diagnosis , Adolescent , Age Factors , Child , Child, Preschool , Dermoid Cyst/surgery , Epithelial Cells/pathology , Female , Giant Cells/pathology , Humans , Infant , Male , Orbital Neoplasms/surgery , Risk Factors
15.
Curr Opin Ophthalmol ; 30(6): 467-471, 2019 Nov.
Article in English | MEDLINE | ID: mdl-31449087

ABSTRACT

PURPOSE OF REVIEW: To review the most recent literature on the relationship of spontaneous cerebrospinal fluid (CSF) leak with idiopathic intracranial hypertension (IIH) and considerations in management of these conditions. RECENT FINDINGS: A link has been proposed between spontaneous CSF leak and IIH based on similar demographics, radiologic, and clinical findings in these patients, and on a plausible mechanism of skull base erosion in the setting of high CSF pressure over time. IIH patients with CSF leak may not present with classic IIH signs and symptoms as the leak can alleviate excess pressure; however, they may develop these after a leak is repaired. There may also be a higher risk of leak recurrence if intracranial hypertension is not treated postoperatively. SUMMARY: A growing body of evidence supports an association between IIH and spontaneous CSF leak. However, this relationship is still not fully elucidated, and there is no current agreement on how to incorporate screening, management, or counseling guidelines for CSF leak into the care of IIH patients. There are also no specific guidelines for evaluation/management of IIH in patients with spontaneous CSF leak. Further interdisciplinary research is needed to explore this connection and to establish screening, evaluation, and management guidelines.


Subject(s)
Cerebrospinal Fluid Leak/physiopathology , Intracranial Hypertension/physiopathology , Cerebrospinal Fluid Leak/therapy , Cerebrospinal Fluid Pressure , Humans , Intracranial Hypertension/therapy , Pseudotumor Cerebri/physiopathology , Recurrence , Skull Base
16.
Neurol Clin ; 37(1): 85-102, 2019 02.
Article in English | MEDLINE | ID: mdl-30470277

ABSTRACT

The physiologic changes that accompany pregnancy can have important implications for neuro-ophthalmic disease. This article discusses pregnancy-related considerations for meningioma, pituitary disorders, demyelinating disease, myasthenia gravis, thyroid eye disease, idiopathic intracranial hypertension, cerebral venous sinus thrombosis, stroke, migraine, and cranial neuropathies. The article also details the potential neuro-ophthalmic complications of preeclampsia and eclampsia and reviews the use of common diagnostic studies during pregnancy.


Subject(s)
Eye Diseases/complications , Nervous System Diseases/complications , Pregnancy Complications , Female , Humans , Pregnancy
17.
PLoS One ; 13(6): e0199793, 2018.
Article in English | MEDLINE | ID: mdl-29953490

ABSTRACT

PURPOSE: Non-arteritic anterior ischemic optic neuropathy (NAION) is the most common cause of non-glaucomatous optic neuropathy in older adults. Optical coherence tomographic angiography (OCT-A) is an emerging, non-invasive method to study the microvasculature of the posterior pole, including the optic nerve head. The goal of this study was to assess the vascular changes in the optic nerve head and peripapillary area associated with NAION using OCT-A. DESIGN: Retrospective comparative case series. METHODS: We performed OCT-A in 25 eyes (7 acute and 18 non-acute) in 19 patients with NAION. Fellow, unaffected eyes were analyzed for comparison. Patent macro- and microvascular densities were quantified in the papillary and peripapillary regions of unaffected, acutely affected, and non-acutely affected eyes and compared across these groups according to laminar segment and capillary sampling region, and with respect to performance on automated visual field testing. RESULTS: In acutely affected eyes, OCT-A revealed a reduction in the signal from the major retinal vessels and dilation of patent superficial capillaries in the peripapillary area. By contrast, non-acutely affected eyes showed attenuation of patent capillaries. The peripapillary choriocapillaris was obscured by edema in acute cases, but was similar between non-acute and unaffected eyes. The degree of dilation of the superficial microvasculature in the acute phase and attenuation in the non-acute phase each correlated inversely with visual field performance. The region of reduced patent capillary density correlated with the location of visual field defects in 80% of acute cases and 80% of non-acute cases. CONCLUSIONS: OCT-A reveals a dynamic shift in the superficial capillary network of the optic nerve head with strong functional correlates in both the acute and non-acute phases of NAION. Further study may validate OCT-A as a useful adjunctive diagnostic tool in the evaluation of ischemic optic neuropathy.


Subject(s)
Angiography , Optic Neuropathy, Ischemic , Retinal Vessels , Tomography, Optical Coherence , Vision, Ocular , Adult , Aged , Follow-Up Studies , Humans , Middle Aged , Optic Neuropathy, Ischemic/diagnostic imaging , Optic Neuropathy, Ischemic/physiopathology , Retinal Vessels/diagnostic imaging , Retinal Vessels/physiopathology
18.
Am J Ophthalmol ; 189: 160-165, 2018 05.
Article in English | MEDLINE | ID: mdl-29476725

ABSTRACT

PURPOSE: To determine whether botulinum toxin augments the effect of strabismus surgery in pediatric patients with large-angle infantile esotropia. DESIGN: Retrospective, comparative, case series. METHODS: Setting: Tertiary-care pediatric hospital. STUDY POPULATION: Patients with large-angle infantile esotropia. INTERVENTION: Treatment with botulinum toxin-augmented bilateral medial rectus muscle recessions ("augmented-surgery group") or traditional bilateral medial rectus muscle recessions ("surgery-only group"). MAIN OUTCOME MEASURE: The effect of surgery on ocular alignment at 4 months, measured in prism diopters of change per mm of surgery (PD/mm). RESULTS: There were 14 patients in the augmented-surgery group and 16 patients in the surgery-only group. The mean effect on alignment was significantly greater in the augmented-surgery group compared to the surgery-only group at 4 months (5.7 ± 1.3 vs 4.0 ± 1.4 PD/mm, P = .002) and at 1 year (5.4 ± 1.2 vs 3.7 ± 1.2 PD/mm, P = .002). There was a partial loss of treatment effect between 4 months and 1 year in both groups, which was similar in magnitude (P = .57). On linear regression, there was a trend toward a positive correlation between botulinum toxin dose and treatment effect, but this was not statistically significant (P = .09). CONCLUSIONS: Botulinum toxin augments the surgical effect of medial rectus muscle recession. Botulinum toxin-augmented surgery may be an alternative to traditional options for large-angle infantile esotropia. A surgical dosing table is proposed for this technique.


Subject(s)
Botulinum Toxins, Type A/administration & dosage , Esotropia/therapy , Oculomotor Muscles/drug effects , Oculomotor Muscles/surgery , Ophthalmologic Surgical Procedures , Combined Modality Therapy , Drug Synergism , Esotropia/drug therapy , Esotropia/surgery , Female , Follow-Up Studies , Humans , Infant , Injections, Intramuscular , Male , Retrospective Studies , Vision, Binocular/physiology
19.
Semin Ophthalmol ; 33(1): 36-41, 2018.
Article in English | MEDLINE | ID: mdl-29420144

ABSTRACT

There are a number of surgical options for treatment of idiopathic intracranial hypertension (IIH) when it is refractory to medical treatment and weight loss. Optic nerve sheath fenestration (ONSF) is one of these options. Use of this procedure varies among centers due to experience with the procedure and concern for associated complications that can result in severe loss of vision. This review summarizes the literature concerning post-surgical complications of ONSF for IIH.


Subject(s)
Blindness , Decompression, Surgical/adverse effects , Neurosurgical Procedures/adverse effects , Optic Nerve Injuries/etiology , Optic Nerve/surgery , Papilledema/surgery , Pseudotumor Cerebri/complications , Blindness/epidemiology , Blindness/etiology , Blindness/physiopathology , Humans , Optic Nerve/pathology , Optic Nerve Injuries/diagnosis , Optic Nerve Injuries/physiopathology , Papilledema/diagnosis , Papilledema/etiology , Postoperative Complications , Pseudotumor Cerebri/physiopathology , Pseudotumor Cerebri/surgery , Visual Acuity
20.
Semin Ophthalmol ; 33(1): 17-22, 2018.
Article in English | MEDLINE | ID: mdl-28881162

ABSTRACT

Perioperative vision loss (POVL) may cause devastating visual morbidity. A prompt anatomical and etiologic diagnosis is paramount to guide management and assess prognosis. Where possible, steps should be undertaken to minimize risk of POVL for vulnerable patients undergoing high-risk procedures. We review the specific risk factors, pathophysiology, and management and prevention strategies for various etiologies of POVL.


Subject(s)
Blindness , Postoperative Complications , Surgical Procedures, Operative/adverse effects , Blindness/epidemiology , Blindness/etiology , Blindness/physiopathology , Humans , Incidence , Risk Factors , Visual Acuity
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