[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.

Interdisciplinary Protocol for the Management of Vision-Threatening Papilledema.

BACKGROUND: This study evaluates the effectiveness of a multidisciplinary protocol for management of patients with papilledema and vision loss secondary to increased intracranial pressure. METHODS: Retrospective record review of all adult patients who presented with vision-threatening papilledema (VTPE) and were treated under this protocol. Patients are admitted for lumbar drain placement and diuretics and followed daily to determine if they may be managed medically or require surgery (optic nerve sheath fenestration [ONSF] and/or cerebrospinal fluid [CSF] shunting). RESULTS: Nineteen patients were included. Twelve had body mass index in the obese range and 6 were morbidly obese. Fourteen had idiopathic intracranial hypertension. Five had secondary pseudotumor cerebri syndrome related to medication use, dural venous sinus thrombosis, hypothyroidism, end-stage renal disease, pulmonary disease, and diastolic heart failure. Three patients did not require surgery and were discharged on oral diuretics; 3 patients underwent unilateral ONSF, 9 underwent bilateral ONSF, and 4 underwent bilateral ONSF followed by ventriculoperitoneal shunt placement. The average follow-up was 10.1 months. The visual acuity improved bilaterally in 12 patients and unilaterally in 4 patients. The remaining 3 patients had worsened vision in both eyes. Fifteen patients had bilateral improvement in their visual fields. Five eyes in 3 patients showed further constriction of the visual field at follow-up. CONCLUSIONS: We demonstrate how a multidisciplinary complex care protocol for treating VTPE can expedite and streamline treatment and restore vision. We found that most patients had improved symptoms and signs, including visual acuity, visual fields, and papilledema. We encourage institutions that manage VTPE to adopt similar institutional protocols.

Ocular Trauma Caused by Confetti Cannons.

BACKGROUND: Eye trauma is an unfortunate and often preventable cause of vision loss. Confetti cannons are common causes of injury. Awareness of ocular hazards of confetti cannons remains low because of limited reports describing ophthalmic injuries following their use. OBJECTIVES: To describe outcomes of ocular trauma caused by confetti cannons and to increase recognition of their ocular risks. METHODS: A retrospective analysis was conducted of eye injuries caused by confetti cannons presenting to a single medical center between 2016 and 2020. Data collected included age, gender, eye injured, ocular damage, visual outcome, and details of surgeries performed. RESULTS: Overall, six consecutive patients (2 males, mean age 19.5 ± 9.74 years) were identified and studied. In all patients only one eye was injured (3 right eyes) during a private celebration, most commonly (n=5) to a bystander while in the vicinity of a cannon operated by someone else. Most common eye injuries included corneal erosion (n=4), traumatic hyphema (n=4), and retinal edema (n=3). Mean initial logMAR visual acuity in the injured eye was 0.73 ± 0.18, improving to 0.25 ± 0.16 at the final visit (P = 0.125). Two patients underwent eye surgery due to their trauma: one to repair globe penetration and another to undergo intravitreal injection of tissue plasminogen activator and C3F8 for submacular hemorrhage, followed 8 months later by intravitreal bevacizumab injection for choroidal neovascularization. CONCLUSIONS: Confetti cannons pose hazards that can cause severe ocular trauma resulting in permanent vision loss. Increasing awareness of device hazards is necessary to prevent eye injuries.

Ocular Adverse Effects of Amiodarone: A Systematic Review of Case Reports.

SIGNIFICANCE: Amiodarone is an excellent antiarrhythmic medication; however, it has numerous systemic and ocular adverse effects. PURPOSE: We aimed to improve our understanding of amiodarone and its ocular adverse effects by performing a systematic review and meta-analysis of published case reports. METHODS: This systematic review was reported in accordance with Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. We used the MEDLINE database, primarily through PubMed, and used keywords (amiodarone, eye, eye diseases, visual/ocular adverse effects/manifestations) to identify case reports of ocular adverse effects after amiodarone use. The initial search resulted in 92 total case reports. However, after excluding nonrelevant case reports, 25 cases were selected for the final analysis. RESULTS: Among the patients in the 25 case reports, 18 were male (72%), and the median age was 66 ± 9.9 years. In 15 cases (60%), the patients reported halos around light and/or decrease in vision after amiodarone use. The most common ophthalmic examination findings were cornea verticillata/vortex keratopathy in 19 cases (76%), followed by different patterns of papilledema and retinal hemorrhages in 5 cases (20%). Discontinuation of amiodarone was the most common intervention, followed by application of topical heparin. Outcomes among case reports were variable. CONCLUSIONS: Cornea verticillata/vortex keratopathy was the most common ocular adverse effect in cases where amiodarone was administered. Early recognition of amiodarone-induced ocular adverse effects is imperative to prevent worsening keratopathy or uncommon adverse effects. Collaboration between physicians prescribing amiodarone-to recognize the ocular symptoms-and referral to eye care physicians are important.

Pediatric Idiopathic Intracranial Hypertension.

The presentation of idiopathic intracranial hypertension (IIH) in pediatric populations has several important distinctions from that in adults, especially among prepubertal patients, in which there is no apparent association with gender or obesity. Pediatric patients are more likely to be asymptomatic or present with atypical symptoms than their adult counterparts, posing a diagnostic challenge in some cases. It is important to be aware of the ways in which diagnostic criteria for IIH are modified from that of adults. Ideal treatment practices and the natural history of pediatric IIH remain unclear. Acetazolamide is the mainstay of medical treatment, but some patients with significant visual loss may require surgical intervention. Multicenter studies to accrue a large number of cases and future prospective studies will help to better define pediatric IIH and to formulate consensus guidelines for treatment and management of these patients.

Bilateral Papillitis and Anterior Uveitis in a Pediatric Patient.

We report a case of bilateral papillitis and anterior uveitis in a pediatric patient with preceding viral prodrome. Because this is a rare disease in the pediatric population, work-up in this case was extensive given the wide differential of papilledema in the pediatric setting. The clinical significance of this case is to bring to light the timely recognition, follow-up, and treatment of this disease to minimize the burden to the patient and family.

Syndrome of Transient Headache and Neurologic Deficits With Cerebrospinal Fluid Lymphocitosis Should Be Considered in Children Presenting With Acute Confusional State.

BACKGROUND: Transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL) is benign and self-limited, with neurologic deficits including sensory disturbance of one body side, aphasia, nausea/vomiting, weakness, decreased vision, homonymous hemianopsia, photophobia. Acute confusional state can rarely occur. Papilledema and intracranial hypertension have also been described. It is a rare entity mainly affecting adults; however, it has been sporadically described in children and adolescents. MAIN FINDINGS: In this clinical observational study, we describe a clinical series of three consecutive pediatric patients being diagnosed with HaNDL after presenting with altered consciousness, papilledema, and increased intracranial pressure. They all recovered without relapses. CONCLUSION: Presentation during childhood and adolescence is rare; the majority of pediatric cases presented with altered consciousness, which is infrequent in HaNDL. This may suggest that in childhood this symptom might be more common than in adults. All three patients presented with increased intracranial pressure and papilledema, thus suggesting that these aspects should be investigated in all patients presenting with this clinical pattern. Finally, all our patients began to suffer from migraine. This feature, together with the benign course of the disease, could favor the hypothesis of a migrainous pathophysiology of this syndrome, although this remains a speculative.

Floorball-related eye injuries: The impact of protective eyewear.

Several previous studies have shown that floorball belongs to a high-risk group of sports in terms of eye injuries. Protective eyewear is available, but the extent of its use and impact on eye injuries are unknown. The purpose of this study was to investigate the current incidence of eye injuries caused by floorball and to compare it with the present use of protective eyewear. Medical records were used to identify all eye injuries suffered while playing floorball in Jönköping County from 2008 to 2011 (N = 167). All these patients were sent a questionnaire that included inquiries about the use of protective eyewear. The study shows that floorball caused more eye injuries than all other sports combined (56%). Prolonged decreased visual acuity was very unusual (0.5%), but moderate eye injuries with some risk of future problems were seen in 62% of the sample. More than one fifth of the injured patients reported some kind of vision-related problem 2-7 years after the original injury. Only one player had been using protective eyewear at the time of injury. Our results underline the importance of protective eyewear to prevent floorball-related injuries.

[Treatment of idiopathic intracranial hypertension].

Idiopathic intracranial hypertension(IIH) is a kind of diffuse brain swelling disease. Several causes could lead to IIH. Intracranial venous circulatory disturbance is more common. The clinical manifestations include intracranial pressure increased, headache and papilledema. Since IIH usually has serious impact on vision even blindness, the diagnosis and corresponding treatment are especially important. This review is to discuss the new insights on the therapeutic options of IIH, which cover medical treatment, weight-losing therapy, surgical treatment, interventional therapy and supportive treatment.(Chin J Ophthalmol, 2016, 52: 948-951).

[Non-Arteritic Ischemic Optic Neuropathy (NAION)]. / Die nicht arteriitische anteriore ischämische Optikusneuropathie (NAION).

Non-arteritic ischemic optic neuropathy (NAION) is virtually unknown outside ophthalmology. It is characterised by acute unilateral visual loss, no pain on eye movements and virtually always optic disc swelling. Optic disc oedema resolves within 1 to 2 months, leaving behind optic atrophy. Vision hardly improves. NAION is the product of local abnormalities of the vascular supply to the optic nerve and general vascular risk factors. Of these, diabetes, hypertension and especially sleep apnoea syndrome are the most important. Recurrences in the involved eye are rare; contralateral recurrence occurs in approximately 15 % of patients. There is no clear scientific evidence for any specific therapy. However, there is general agreement that it is reasonable to control risk factors.

The course of headache in idiopathic intracranial hypertension: a 12-month prospective follow-up study.

BACKGROUND AND PURPOSE: Our aim was to prospectively describe the course of headache during the first year of idiopathic intracranial hypertension (IIH). METHODS: Patients with newly diagnosed IIH were consecutively included from December 2010 to June 2013. Treatment according to standard guidelines was initiated. Headache history was obtained by headache diaries and standardized interviews performed at baseline and after 1, 2, 3 and 12 months. Parallel changes in papilledema were assessed by optical coherence tomography (OCT). All patients had comprehensive neuro-ophthalmological examinations including automated perimetry. RESULTS: Forty-four patients were included. Thirty-five patients completed the 12-month follow-up. Dramatic improvement in headache occurred within the first weeks after diagnosis. After 1 year, 15 patients reported no or only infrequent headache. However, 15 of the remaining 20 patients reported sustained chronic headache. Early age of onset and high diagnostic intracranial pressure (ICP) were associated with better headache outcome (≤1 headache days/month) after a year. Papilledema decreased rapidly within the first 2 months of diagnosis. After 1 year, OCT measures had normalized. Visual outcome was excellent in most patients. CONCLUSIONS: Although headache in 43% of patients responded well to ICP management, sustained long-term headache was seen in the remaining patients, despite resolution of papilledema. Headache in IIH may thus be attributed to more complex mechanisms than ICP elevation alone. High ICP and young age were associated with better headache outcome. Early treatment according to standard guidelines seems sufficient to ensure excellent visual outcome in the vast majority of patients.

Managing idiopathic intracranial hypertension in the eye clinic.

Idiopathic intracranial hypertension (IIH) is a neuro-ophthalmological condition characterised by a raised intracranial pressure and papilloedema that causes disabling headaches. The main risk factors of female sex and living with obesity have been known for some time, however the knowledge of the underlying pathophysiology is evolving. Papilloedema can impact the visual function, and the majority of people are offered acetazolamide. Those with sight threatening disease need urgent management, though there is little high quality evidence to recommend any particular surgical intervention. Headache treatment is an unmet clinical need and simple medication overuse advice has the potential to reduce the chronification of migraine-like headaches. IIH is emerging as a systemic metabolic disease distinct from people living with obesity alone. While weight loss is the main stay of disease modifying therapy this is challenging to access and many healthcare professionals that manage the condition have no formal training or accessible pathways for weight management. The aim of this "how to do it" article is to present the latest advances in knowledge of IIH that we pragmatically included in routine clinical care for people living with the condition.

Predicting the Rapid Improvement of Papilledema After Stenting in Idiopathic Intracranial Hypertension.

PURPOSE: This study aimed to identify predictors of rapid improvement of papilledema after stenting and develop a simple preintervention scale. METHODS: A prospective cohort of idiopathic intracranial hypertension (IIH) with venous sinus stenosis (VSS) treated with stenting in a tertiary hospital from January 2014 to December 2019 was reviewed. We categorized papilledema improvement into favorable (grades 0-1) and unfavorable (grades 2-5). We employed logistic regression analysis to find the predictive factors and develop the predictive scale. We then estimated the performance of the scale using the ROC curve and Hosmer-Lemeshow test. RESULTS: There were 110 patients who underwent venous sinus stenting, with a mean age of 37.1 years and a predominance of females (77.3%). A total of 85 patients had a favorable outcome following stenting, while 25 patients had an unfavorable outcome. The results of the multivariate analysis indicate that lower preoperative pressure gradients (odds ratio, OR: 4.01; 95% confidence interval, CI: 1.27-12.68), stenosis rates (OR: 4.16; 95% CI: 1.11-15.56), and preoperative papilledema grades (OR: 2.92; 95% CI: 1.44-5.91) were independently associated with rapid improvement of papilledema following stenting treatment. The 3­item scale exhibited good discrimination with an area under the curve (AOC) of 0.81 (95% CI 0.72-0.89, p < 0.001), as well as acceptable calibration determined by the Hosmer-Lemeshow test (P = 0.42). The optimal cut-off value of the scale (range 0-6 points) was ≥ 4 points, with a sensitivity of 72%, specificity of 73%, and accuracy of 78%. CONCLUSION: The presence of lower preoperative pressure gradients, stenosis severity, and preoperative status of papilledema were identified as positive predictors of rapid improvement of the papilledema following stenting in IIH patients. The 3­item scale provides a promising preintervention predictive model for predicting rapid response following stenting treatment in IIH patients with VSS.

Stenting versus medical treatment for idiopathic intracranial hypertension: a matched-control study.

BACKGROUND: This prospective cohort study compared the outcomes of stenting and medical treatment for patients with idiopathic intracranial hypertension (IIH) and venous sinus stenosis (VSS). METHODS: In this single-center cohort study, patients with IIH and VSS were evaluated between January 2014 and December 2019 with follow-up periods of 1, 3, and 6 months. The patients received either stenting or medical treatment. The two groups underwent 1:1 matching using propensity score analysis, and the clinical outcomes were compared. RESULTS: Following 1:1 matching, 36 patients who underwent stenting and 36 who underwent medical treatment were matched. The median improvements in the papilledema Frisén grade were greater in the stenting group at 1 month (-2 vs 0), 3 months (-3 vs -1), and 6 months (-3 vs -1) than in the medical treatment group. Patients who received stenting treatment had a significantly higher prevalence of complete resolution of their respective symptoms (headache, tinnitus, or visual disturbances) at 3 months (58.3% vs 13.9%, OR 8.68, 95% CI 2.74 to 27.52) and 6 months (80.6% vs 22.2%, OR 14.50, 95% CI 4.64 to 45.32) than those receiving medical treatment. CONCLUSIONS: This matched-control study shows that stenting has a greater efficacy rate and rapid resolution of papilledema and its respective symptoms compared with medical treatment.

Association of post-intervention pressure gradient with symptom-free at 6 months in idiopathic intracranial hypertension with venous sinus stenosis treated by stenting.

OBJECTIVE: This study aimed to identify the key factors that might affect the clinical outcome of patients with idiopathic intracranial hypertension (IIH) and Venous sinus stenting (VSS). METHODS: We performed an analysis of a prospectively collected database of patients with IIH and VSS who underwent stenting. The trans-stenotic pressure gradient was measured before and after intervention. In additional, patients' baseline characteristics, procedure details and clinical outcomes at 6-month follow-up (including changes in headache, visual impairment, papilledema, etc.) were recorded. The effects of post-intervention pressure gradient on symptom-free at 6 months were explored using logistic regression analysis, generalized additive model and receiver operator characteristic (ROC) curve. RESULTS: Of 101 patients included in this study, the median pressure gradient across stenosis decreased from 19 mmHg before intervention to 2 mmHg after intervention. At 6 months, symptom-free was observed in 58 cases (57.4%). Multivariable logistic analysis and generalized additive model showed that post-intervention pressure gradient (increased by 1 mmHg) was independently and linearly correlated with symptom-free (OR = 0.79, 95% CI = 0.67-0.94). Moreover, the post-intervention pressure gradient revealed moderate discrimination with an area under ROC curve of 0.68 (95% CI = 0.57-0.78). Similar associations were observed for the disappearance of headache and papilledema, but not for the visual recovery. CONCLUSION: The post-intervention pressure gradient may be a valid and reliable predictor of 6-month clinical outcome in patients with IIH and VSS treated by stenting. Nevertheless, external validation with blinded outcome is still needed to confirm its performance before clinical application.

Papilledema: are we any nearer to a consensus on pathogenesis and treatment?

Papilledema is a term generally reserved (at least in the English language use of the term) by neuro-ophthalmologists for optic disc edema due to increased intracranial pressure. The etiology for the intracranial hypertension may be known (e.g., brain tumor, meningitis, cerebral venous sinus thrombosis) or may be idiopathic (idiopathic intracranial hypertension [IIH]). IIH is a disorder that predominantly affects overweight women of childbearing age and these epidemiologic factors should offer clues to pathogenesis. The main morbidity of papilledema is visual loss and the major mechanism for permanent optic nerve damage is axoplasmic flow stasis and resultant intraneuronal ischemia. The current initial management of papilledema in IIH includes weight loss and medical therapy (e.g., acetazolamide or furosemide). Patients who fail, are intolerant to, or noncompliant with maximum tolerated medical therapy might require optic nerve sheath fenestration or cerebrospinal fluid diversion (i.e., shunting) procedures.

[Headache from increased cerebrospinal fluid pressure]. / Kopfschmerz durch Liquordrucksteigerung.

Increased cerebrospinal fluid (CSF) pressure is often accompanied by headache. The term idiopathic intracranial hypertension (pseudotumor cerebri) describes an increase in CSF pressure without a space-occupying intracranial lesion or hydrocephalus. After headaches, visual field defects are the second most common feature. Therapeutic measures include both drugs and surgical procedures. In high-pressure hydrocephalus, the volume of the CSF is increased, resulting either from increased production or reduced absorption. If an acquired or congenital obstruction of the ventricular system can be demonstrated, the term non-communicating hydrocephalus is used. In contrast, the CSF passage is blocked outside the ventricles in communicating hydrocephalus. Symptoms include diffuse headache worsening in the morning and with the Valsalva-like maneuver. Treatment is guided by etiology whenever possible; otherwise, drainage of CSF by ventriculostomy or shunt is necessary.

Papilledema: A review of etiology, pathophysiology, diagnosis, and management.

Papilledema is optic nerve head edema secondary to raised intracranial pressure (ICP). It is distinct from other causes of optic disk edema in that visual function is usually normal in the acute phase. Papilledema is caused by transmission of elevated ICP to the subarachnoid space surrounding the optic nerve that hinders axoplasmic transport within ganglion cell axons. There is ongoing controversy as to whether axoplasmic flow stasis is produced by physical compression of axons or microvascular ischemia. The most common cause of papilledema, especially in patients under the age of 50, is idiopathic intracranial hypertension (IIH); however, conditions that decrease cerebrospinal fluid (CSF) outflow by either causing CSF derangements or mechanically blocking CSF outflow channels, and rarely conditions that increase CSF production, can be the culprit. When papilledema is suspected clinically, blood pressure should be measured, and pseudopapilledema should be ruled out. Magnetic resonance imaging of the brain and orbits with venography sequences is the preferred neuroimaging modality that should be performed next to look for indirect imaging signs of increased ICP and to rule out nonidiopathic causes. Lumbar puncture with measurement of opening pressure and evaluation of CSF composition should then be performed. In patients not in a typical demographic group for IIH, further investigations should be conducted to assess for underlying causes of increased ICP. Magnetic resonance imaging of the neck and spine, magnetic resonance angiography of the brain, computed tomography of the chest, complete blood count, and creatinine testing should be able to identify most secondary causes of intracranial hypertension. Treatment for patients with papilledema should be targeted toward the underlying etiology. Most patients with IIH respond to weight loss and oral acetazolamide. For patients with decreased central acuity and constricted visual fields at presentation, as well as patients who do not respond to treatment with acetazolamide, surgical treatments should be considered, with ventriculoperitoneal shunting being the typical procedure of choice.