Cause Of Papilledema Research Articles

The Leading Causes of Optic Disc Edema Seen in Tertiary Care, Academic Neuro-Ophthalmology Clinics.

Optic disc edema (ODE) is one of the most common reasons for referral to a neuro-ophthalmologist. There are various causes that require vastly different workup. Thus, differentiating among each cause is important. Our goal was to determine the causes of ODE and various clinical characteristics of consecutive patients with ODE presenting to neuro-ophthalmology clinics. A retrospective review of consecutive patients with ODE over a period of 5 years were included. Fundus photographs were routinely obtained as part of clinical care. Clinical data including retinal nerve fiber layer thickness, best-corrected visual acuity, and visual field mean deviation were retrieved, and patients were grouped by etiology. A total of 654 patients (n = 462 or 70.6% women and 192 or 29.4% men) with ODE were included with a mean age of 41.2 ± 17.9 years. Female patients were significantly younger than male patients (mean age female participants: 38.7 years, male participants: 47.6; P < 0.001). The top 5 most common causes of ODE in our clinics were idiopathic intracranial hypertension (IIH; 351/654 or 53.7%), nonarteritic anterior ischemic optic neuropathy (NAION; 116/654 or 17.4%), non-IIH papilledema (71/654 or 10.9%), optic neuritis (ON; 46/654 or 7.0%), and uveitis (17/654 or 2.6%). When considering female-only patients, the top 3 causes of ODE were IIH, non-IIH papilledema, and NAION. Among male-only patients, the top 3 causes were NAION, IIH, and non-IIH. Among the top 5 causes of ODE, visual acuity was the worst at presentation in the ON group and the best in the patients with IIH. The Humphrey mean deviation was the worst in ON/NAION groups and best in IIH group. The ODE was most severe in patients with non-IIH papilledema and least severe in ON group. Non-IIH patients with papilledema were not significantly different in visual acuity and visual field parameters at presentation compared with patients with IIH papilledema. Patients with papilledema (both IIH and non-IIH etiologies) had significantly better visual function at presentation compared with other top causes of disc edema (P < 0.001). The most common cause of ODE seen in neuro-ophthalmology clinics was IIH, and these patients were more likely to present with preserved visual function. The higher prevalence of IIH was likely why most patients with ODE were women. Visual function at presentation was not able to differentiate the specific cause of papilledema; however, it was an important differentiating factor for all papilledema cases compared with all other causes of ODE including ON and NAION.

Misconceptions in diagnosis and treatment of papilledema caused by idiopathic intracranial hypertension

Papilledema refers to the swelling of the optic disk due to increased intracranial pressure. In clinical practice, papilledema is often confused with optic disc edema. Idiopathic intracranial hypertension (IIH) is a major cause of papilledema and there are still misconceptions about the etiology of IIH, the classification and examination of papilledema, and the treatment of IIH. This article elaborates on the misunderstandings that may exist in the diagnosis and treatment of papilledema, in order to lay a foundation for the standardized diagnosis and treatment of papilledema in China.

Optic nerve sheath fenestration for treating papilloedema in the era of cerebral venous sinus stenting.

Pseudotumour cerebri (PTC) is the syndrome of intracranial hypertension without intracranial mass or hydrocephalus and is the commonest cause of papilloedema seen in many eye clinics. In the last 10 years, we have increasingly used TSS in patients whose papilloedema was not well controlled with medical treatment and have done fewer ONSFs. Here, we review our experience at Royal Prince Alfred Hospital Sydney with ONSF in 35 patients over the period 2002-2021. Retrospective case series of 35 patients, 30 of whom had primary PTC [i.e., idiopathic intracranial hypertension (IIH)] and 5 with secondary PTC. Eighteen patients had bilateral ONSF and 17 patients unilateral ONSF, in each case of the worse eye. Thirteen patients then underwent transverse sinus stenting (TSS), in each case following ONSF. The primary outcome measures were visual acuity (VA) and mean deviation (MD) on visual field (VF) testing. MD improved by 5 dB or more in 34 of 70 total eyes (48.6%); VA improved by 0.2 logMAR (two lines on Snellen chart) or more in 21 eyes (30%), and by both in 15 eyes (21.4%). Final MD was -10 dB or better in 38 eyes (54.3%); final VA was 0.3 (6/12) or better in 54 eyes (77.1%), and both in 39 eyes (55.7%). The results confirm that ONSF can relieve papilloedoema in both eyes and improve both VF and VA, even in cases of fulminant PTC with severe acute visual impairment.

Bilateral papilledema with vision loss due to post–COVID-19–induced thiamine deficiency: illustrative case

BACKGROUNDBilateral papilledema with vision loss is considered a neurosurgical emergency due to high intracranial pressure. However, it may not be the only cause of papilledema. The authors reported an association among coronavirus disease 2019 (COVID-19), bilateral papilledema, blindness, and Wernicke’s encephalopathy (WE).OBSERVATIONSAn 18-year-old woman presented to the neurosurgery service with rapid profound vision loss and bilateral papilledema. She had COVID-19 3 months earlier with subsequent loss of smell (anosmia) and taste (ageusia), which resulted in hyperemesis and a 43-lb weight loss. Examination revealed ataxia, horizontal nystagmus, and blindness. Magnetic resonance imaging and magnetic resonance venography of her brain were normal. Presumptive diagnosis of WE was made, and she was treated with intravenous thiamine with restoration of vision within 48 hours. Patient’s thiamine level was less than half the normal value.LESSONSNeurosurgeons should be aware of this unique correlation between papilledema and vision loss and its association with WE due to post–COVID-19 hyperemesis and weight loss from anosmia and ageusia.

Papilledema: A Review of the Pathophysiology, Imaging Findings, and Mimics

Increased intracranial pressure is the most common cause of papilledema. Multiple etiologies such as cerebral edema, hydrocephalus, space occupying lesions, infection, and idiopathic intracranial hypertension among others should be considered. Imaging plays a critical role in the detection of pathologies that can cause papilledema. MRI with contrast and CE-MRV, in particular, are key for the diagnosis of idiopathic intracranial hypertension. This review will focus in common and infrequent causes of papilledema, the role of imaging in patients with papilledema as well as its potential mimickers.

Dural Venous Sinus Thrombosis and Papilledema Related to JAK2 Mutation: A Case Series.

Dural venous sinus thrombosis (DVST) is an important cause of papilledema. Patients diagnosed with DVST should undergo work-up for underlying hypercoagulable state, including genetic causes. One important prothrombotic mutation is in the JAK2 gene, which is a driver of myeloproliferative neoplasms including polycythemia vera (PV). We aimed to determine the prevalence of JAK2 mutation in patients in presenting to neuro-ophthalmology clinic with DVST and papilledema. Retrospective case series of patients seen in a tertiary neuro-ophthalmology practice who presented with papilledema due to DVST and were investigated for presence of JAK2 mutation. Four out of 15 patients with DVST (26%) were found to have JAK2 V617F mutation which led to subsequent diagnosis of PV in 2. One additional patient had a known diagnosis of essential thrombocytosis. We describe the clinical presentation of these four patients with papilledema and JAK2 mutation. A significant proportion of patients with papilledema secondary to DVST will harbor mutations in the JAK2 gene. Clinicians should be aware of this mutation as early testing will facilitate timely diagnosis and treatment of myeloproliferative disease to improve prognosis and reduce risk of recurrent thrombotic events.

Dural venous sinus thrombosis after lumbar puncture in a patient with idiopathic intracranial hypertension.

Idiopathic intracranial hypertension (IIH) is one of the most common causes of papilloedema seen by ophthalmologists and neurologists. Patients with IIH routinely undergo lumbar puncture (LP) for diagnosis. Dural venous sinus thrombosis (DVST) is a rare complication of cerebrospinal fluid pressure (CSF)-lowering procedures such as lumbar puncture and epidural and may be an intracranial complication of IIH. A 29-year-old obese woman was diagnosed with severe idiopathic intracranial hypertension (IIH) after she presented with new-onset headache, pulsatile tinnitus and blurred vision. Magnetic resonance imaging (MRI) and venography (MRV) were normal apart from signs of intracranial hypertension. Lumbar puncture (LP) revealed an opening pressure of 40 cm of water. Due to the severity of the papilloedema and vision loss, she was referred for a ventriculoperitoneal shunt and found to have venous sinus thrombosis involving the superior sagittal sinus on the pre-operative computed tomography (CT) head 5 days after the LP. CT venography (CTV) one day later and MRV 3 days later showed significant worsening as the thrombosis extended into the deep venous system. She was started on therapeutic heparin and her symptoms and vision improved and she did not develop any neurological complications. DVST should be considered in IIH patients who have worsening papilloedema or symptoms of intracranial hypertension. Repeat venous imaging can prevent devastating consequences such as venous infarcts of haemorrhage in these patients.

Etiology of Papilledema in Patients in the Eye Clinic Setting

The study of health conditions associated with papilledema will augment the clinical judgment of eye care professionals treating patients with optic disc edema in determining the urgency of additional evaluation and counseling patients accordingly. To determine the incidence, demographic characteristics, and etiologies of papilledema based on a unique records-linkage research platform; and to describe the demographic and clinical differences between patients with idiopathic intracranial hypertension (IIH) and other causes of papilledema. Retrospective population-based cross-sectional study of patients treated for papilledema at outpatient eye clinics in Olmsted County, Minnesota, using the Rochester Epidemiology Project. Data were collected from January 1990 to December 2014 and analyzed from September 2018 to April 2019. Etiologies of papilledema, body mass index, incidence of headache, or localizing neurologic signs. Eighty-six patients were diagnosed with papilledema during the 24-year period, providing an age- and sex-adjusted incidence of 2.5 individuals per 100 000 per year; 68 patients (79%) were women, 73 (85%) were white patients, and the median (range) age was 27.7 (6.2-64.2) years. Nineteen patients (22%) presented with a previously diagnosed attributable cause (eg, trauma or intracranial tumor). Among patients presenting with papilledema without a previously diagnosed attributable cause, 58 patients (87%) had IIH, and 9 patients (13%) were found to have a secondary cause of raised intracranial pressure, such as intracranial tumor, cerebral venous sinus thrombosis, or granulomatous meningitis. Patients with IIH had a higher median (range) body mass index (37.5 [20.4-55.7] vs 27.4 [16.6-40.1]; P = .003) and headache prevalence (54 of 58 patients [93%] vs 6 of 9 patients [67%]; P = .004) than patients with other causes of papilledema. Of 9 patients with papilledema but no IIH, 2 (22%) had localizing neurologic signs, such as gait abnormalities, hearing loss, focal weakness or numbness, visual field defects, or aphasia. Among 42 patients with demographic characteristics typically associated with IIH (female sex, with obesity, aged 15 to 45 years, and absent localizing neurologic signs or symptoms), 40 (95%) had papilledema that was associated with IIH. Conversely, among the 19 patients without these demographic characteristics, 7 (37%) had an alternative cause. In this study, most patients who presented to the eye clinic with papilledema without a previously known cause were found to have IIH. These patients were more likely to present with headaches and had statistically higher body mass index. Clinicians should take these findings into account when determining the pretest probability of a patient having IIH or an alternative cause of papilledema.

Papilledema in Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP): A Pediatric Case and Review of the Literature

To analyze the available literature on papilledema in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), report the first detailed pediatric case, and explore the underlying pathophysiology. First, we conducted a comprehensive literature review of all cases of papilledema in CIDP. Next, we reviewed each case, incorporating only those including cerebrospinal fluid analysis into the results. Finally, we present our pediatric patient. Our literature review yielded a total of 9 adult and no pediatric cases. Cerebrospinal fluid protein and opening pressures were elevated in all cases. They were also elevated in our pediatric case. Prolonged periods of active immune-mediated inflammation is likely a cause of papilledema in adult CIDP, and possibly also in our pediatric case.

Resident Perspectives

Resident Perspectives

Evaluation of the underlying causes of papilledema in children

The purpose of this retrospective study was to identify the types and relative frequencies of intracranial disorders in pediatric patients who present with papilledema. Retrospective case series. This study was conducted in 2 pediatric ophthalmology clinics, both providing community-based care in a large inner-city urban center in the U.S. Pediatric patients aged between 0 and 16 years diagnosed with papilledema and who had an underlying etiology identified were included in the study. Patient demographic data, ophthalmologic examination findings, and diagnostic work-up results were identified from clinical records. The mean age of 38 study patients (19 female, 19 male) was 8.6 ± 4.8 years. Of the 38 patients, 16 (42.1%) had idiopathic intracranial hypertension (IIH) as the underlying cause of the papilledema, 7 (18.4%) had a craniosynostosis disorder, 6 (15.8%) had intracranial tumours, 2 (5.3%) had primary hydrocephalus, and 1 (2.6%) patient each had transverse sinus thrombosis related to sinusitis, hypertensive crisis, subdural hematoma, intracranial abscess, Lyme disease, presumed neurosarcoidosis, and acute disseminated encephalomyelitis. Of the 6 intracranial tumours, 2 (33.3%) presented in the sellar/parasellar region, 2 (33.3%) in the posterior fossa, and 2 (33.3%) were in cortical locations. Clinicians should have a high index of suspicion for IIH and brain tumours in children presenting with papilledema. Patients with craniosynostosis should have routine eye examinations to monitor for asymptomatic papilledema. Understanding the relative incidence of etiologies for papilledema highlights the urgency of appropriate work-up and the need to consider low-frequency etiologies.

Identification of underlying causes of papilledema in children

Identification of underlying causes of papilledema in children

Froin Syndrome

ABSTRACT Introduction Yellow-colored cerebrospinal fluid (CSF) suggestive of xanthochromia can be due to elevated CSF protein and can be one of the causes of papilledema. Observation We observed a 55-year-old lady with papilledema with yellow-colored CSF which was hypercoagulable along with elevated CSF protein and no cells. Her systemic and nervous system examination was unremarkable except for the eye findings. Her contrast-enhanced brain magnetic resonance imaging (MRI) showed multiple meningiomas, and on imaging of the spine, she had multiple contrast-enchancing intradural, extramedullary lesions. Her eye findings were attributed to Froin syndrome found in patients with spinal tumors. Conclusion Our case stresses the need to keep the possibility of Froin syndrome in mind while evaluating patients with papilledema and it may be pertinent to perform a spinal imaging, especially when no apparent cause for papilledema is found. How to cite this article Choudhary A, Goyal MK, Modi M, Mukherjee KK, Ahuja CK, Lal V. Froin Syndrome. J Postgrad Med Edu Res 2018;52(1):34-35.

Successful management of peripapillary choroidal neovascular membrane secondary to idiopathic intracranial hypertension with intravitreal ranibizumab

Idiopathic intracranial hypertension (IIH) is a common cause of papilledema in young females and causes headache with transient visual loss. Severe visual loss occurs due to optic atrophy or peripapillary choroidal neovascular membrane (PCNVM). PCNVM in IIH has an incidence of 0.5% with a benign course in the majority of patients. Intravitreal Anti-vascular endothelial growth factor agents have anecdotally been used to manage these patients, with complete resolution reported in all cases after a single injection. Our case of IIH-associated PCNVM was treated with three injections of intravitreal ranibizumab with no recurrence at 6-month follow-up.

Accuracy of crescent sign on ocular ultrasound in diagnosing papilledema.

AIMTo study the usefulness of orbital ultrasonography in the diagnosis of papilledema.METHODSFifty patients who were referred to the neurophthalmology clinic and clinically suspected to have papilledema were selected. Thorough, clinical examination with slitlamp biomicroscopy and visual acuity assessment was done. These patients underwent ultrasonography to demonstrate the crescent sign. The patients were further evaluated with the neurologist and magnetic resonance imaging (MRI) thus confirming the diagnosis of papilledema. The results of our ultrasonographic evaluation were correlated with final diagnosis after thorough clinical evaluation, imaging and the neurologist’s opinion.RESULTSOut of 50 patients diagnosed having papilledema on MRI, 46 (92%) showed crescent sign on B scan ultrasonography. Headache was most common presenting complaint in 47 (94%) and idiopathic intracranial hypertension was most common underlying cause of papilledema in 30 (60%) cases.CONCLUSION“Crescent sign” seen on ultrasonography is a sensitive tool for diagnosis of papilledema. It is cost effective, less cumbersome and effective tool to differentiate between papilledema and pseudo papilledema before subjecting the patients to costly investigations like MRI. A positive crescent sign should always be followed by MRI to find out the cause of papilledema.

Papilledema Due to Mirtazapine

Mirtazapine is a tetracyclic antidepressant that enhances both noradrenergic and serotonergic transmission. The most common cause of papilledema is increased intracranial pressure due to brain tumor. Also it may occur as a result of idiopathic intracranial hypertension (IIH, pseudo tumor cerebri). Moreover, papilledema may also develop due to retinitis, vasculitis, Graves' disease, hypertension, leukemia, lymphoma, diabetes mellitus and radiation. In this article, a patient who developed papilledema while under treatment with mirtazapine (30 mg/day) for two years and recovered with termination of mirtazapine treatment was discussed to draw the attention of clinicians to this side effect of mirtazapine. Idiopathic intracranial hypertension and papilledema due to psychotropic drugs has been reported in the literature. Mirtazapine may rarely cause peripheral edema. However, papilledema due to mirtazapine has not been previously reported. Although papilledema is a very rare side effect of an antidepressant treatment, fundoscopic examinations of patients must be performed regularly.

Papilledema secondary to a superior sagittal sinus thrombosis. Mantle cell lymphoma paraneoplastic syndrome

Clinical caseA 46-year-old patient presented with visual loss in the left eye during the previous months. Ophthalmoscopic examination and magnetic resonance angiography found the presence of papilledema due to thrombosis in superior sagittal sinus. The examination findings revealed a mantle cell lymphoma. DiscussionCerebral venous thrombosis is an unusual cause of papilledema. This type of thrombosis may be secondary to hyper-viscosity within a context of a paraneoplastic syndrome.

Papiledema secundario a trombosis del seno sagital superior. Cuadro paraneoplásico de un linfoma del manto

Clinical caseA 46 year old patient presented with visual loss in the left eye during the previous months. Ophthalmoscopic examination and magnetic resonance angiography found the presence of papilledema due to thrombosis in superior sagittal sinus. The examination findings revealed a mantle cell lymphoma. DiscussionCerebral venous thrombosis is an unusual cause of papilledema. This type of thrombosis may be secondary to hyper-viscosity within a context of a paraneoplastic syndrome.

POEMS syndrome as an uncommon cause of papilledema.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is an uncommon condition related to a paraneoplastic syndrome secondary to an underlying plasma cell disorder. Among the myriad of manifestations of the disease, ocular signs and symptoms are relatively prevalent, affecting about half of all patients with the disease. To report the ocular manifestations of POEMS syndrome. A 47-year-old lady diagnosed to have POEMS syndrome presented with painless progressive visual diminution. Her color vision was impaired. There was bilateral papilloedema. POEMS syndrome should be considered among the differential diagnoses of all patients with a bilateral papilledema in which no other cause can be readily elucidated.

Unusual causes of papilledema: Two illustrative cases

Background:Neurosurgeons are frequently among the first physicians asked to evaluate patients with papilledema, and the patient is often referred with the implication that they may require shunting. After an initial evaluation to exclude potential neurosurgical emergencies, the physician should carefully consider various etiologies of papilledema to prevent unnecessary neurosurgical operations.Case Description:The authors report two illustrative cases of unusual causes of papilledema: Anemia and leukemic infiltration of the central nervous system. In each case, a complete blood count provided clues for the diagnosis. A review of the literature is also included.Conclusions:Both patients responded to medical management/treatment of the underlying disease and did not require neurosurgical operative intervention. Papilledema may be caused by other etiologies besides increased intracranial pressure. The authors present two unusual cases leading to papilledema and provide an outline for the workup of these conditions.