Pediatric Idiopathic Intracranial Hypertension Research Articles

Magnetic Resonance Imaging Features and Clinical Findings in Pediatric Idiopathic Intracranial Hypertension: A Case-Control Study.

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.

Management of idiopathic intracranial hypertension in children utilizing venous sinus stenting.

Venous sinus stenting (VSS) is an accepted and minimally invasive treatment for adult idiopathic intracranial hypertension (IIH) associated with lateral sinus stenosis (LSS). The efficacy and safety of venous sinus stenting (VSS) in children with IIH has not been established. This is a retrospective analysis of IIH patients 18 years of age or younger with LSS treated with VSS at our institution. Included patients have fulminant disease course or are refractory or intolerant to medical management. Eight patients were identified; 4 males and 4 females. Mean age is 13.4 years (range 4-18). All patients had severe headaches, 5 had blurred vision, 3 had diplopia and 3 had pulsatile tinnitus. Papilledema was present in 4 patients. Three patients had prior surgical procedures. Four patients were intolerant to medical management, 3 were refractory and 1 had fulminant course. Cerebral venography demonstrated severe stenosis of the dominant sinus in 6 patients and of bilateral co-dominant sinuses in 2 patients. Six patients had intrinsic stenosis and 2 had extrinsic stenosis. Venous sinus stenting (VSS) resulted in improvement of symptoms, papilledema and normalization of CSF opening pressure in 7 patients. No immediate complications were observed. Mean follow-up period is 21 months (range 6-42). Two patients required re-stenting; one responded well and the other had persistent symptoms and underwent subsequent surgical procedures of CSF diversion, suboccipital decompression and duraplasty which were also ineffective. VSS may provide a viable option for pediatric IIH patients who are intolerant to medication, have failed conservative management or prior surgical interventions, or present with fulminant disease.

Association of pediatric idiopathic intracranial hypertension with olfactory performance.

ObjectiveTo assess the association between pediatric Idiopathic intracranial hypertension (IIH) and olfactory performance.MethodsA cross-sectional comparative study was conducted including 17 patients under 18 years diagnosed with IIH at a tertiary hospital and 17 healthy age- and sex-matched subjects. All participants underwent the semi-objective chemosensory Sniffin’ Sticks test for evaluation of odor threshold (OT), indicative of peripheral olfactory function, and odor identification (OI), reflecting higher cognitive olfactory processing. Scores were compared and referred to the updated normative values. Demographic, clinical, and neuroimaging data were collected from the medical files. The patients with IIH were reassessed for olfactory function and clinical state at the subsequent follow-up, under treatment.ResultsCompared to controls, the IIH group had a significantly lower mean OT score (6.41 ± 3.43 vs 10.21 ± 2.79, p = 0.001) and higher rate of OT score below the 10th percentile for age and sex according to the normative values (47.1% vs 0%, p = 0.001). There was no significant between-group difference in mean OI scores (9.82 ± 1.63, vs 10.59 ± 1.84, p = 0.290). OT scores were not associated with sex, age, body mass index, neuroimaging abnormalities, or lumbar puncture opening pressure. At the follow-up assessment, the OT scores were improved (9.36 ± 4.17 vs 6.7 ± 3.32, p = 0.027) whereas the OI scores were unchanged (9.88 ± 2.5 vs 9.69 ± 1.58, p = 0.432).ConclusionsAs reported in adults, children and adolescents with IIH appear to have a selective reversible deficit in olfactory detection threshold, which may imply a reduction in peripheral olfactory perceptual ability. Future studies should examine the predictive value of olfactory function for IIH.

Dural venous sinus stenting for treatment of pediatric idiopathic intracranial hypertension

BackgroundDural venous sinus stenting (VSS) is an effective treatment for idiopathic intracranial hypertension (IIH) in adult patients. There are no published series to date evaluating safety and efficacy of VSS...

Influence of Optic Nerve Appearance on Visual Outcome in Pediatric Idiopathic Intracranial Hypertension.

To determine whether optic disc hemorrhages (ODH) and cotton wool spots (CWS) at presentation are associated with worse visual outcomes in pediatric patients with idiopathic intracranial hypertension (IIH). Retrospective institutional review of 100 eyes of 50 consecutive pediatric IIH patients (aged 16 years or less) who had baseline optic disc photographs before or within 30 days of their diagnostic lumbar puncture and initiation of medical treatment. Optic disc photographs were independently graded by three ophthalmologists in a standardized manner. Visual function was assessed using visual acuity (VA) and visual field grade (VFG). At least one ODH was found in 41% of eyes, at least one CWS was found in 27% of eyes, and 20% of eyes had both ODH and CWS. At presentation, Frisén grade was associated with the presence of CWS (p = 0.013) and showed no association with ODH (p = 0.060). When controlling for Frisén grade, ODH and CWS were not associated with worse VA or VFG at final follow-up. Severe ODH were associated with worse VA and VFG at presentation (p < 0.03), but not at final follow-up. Severe CWS at presentation was strongly associated with a worse Humphrey mean deviation of 5.0 dB (95% confidence interval 1.6-8.3) at final follow-up (p = 0.002). When controlling for the severity of papilledema, ODH do not provide any additional prognostic value in pediatric IIH patients. Frisén grade and severe CWS at presentation were independently associated with worse visual outcomes at the final follow-up.

The role of ICP monitoring in paediatric IIH.

Diagnosis of idiopathic intracranial hypertension (IIH) in children is an extrapolation of the guidelines suggested for adult population. Lumbar puncture (LP) plays a crucial role in the diagnosis. The diagnosis of IIH at times is solely dependent on the interpretation of the opening pressure (OP). Unfortunately, LP-OP can vary due circumstantial parameters and therefore may be an unreliable form of intracranial pressure (ICP) measurement. Confirming the diagnosis based (as suggested by guidelines) on LP-OP in a doubtful clinical situation would be inappropriate. The aim of our study was to analyse the reliability of LP-OP and importance of ICP monitoring in situations where diagnosis of IIH was questionable. Retrospective review of all children with diagnosis of IIH over a 10-year period was conducted. Children who underwent ICP monitoring (ICPM) were selected. We considered 2 LP-OP values-last LP (lLP) and the mean LP (mLP) for analysis. ICPM and LP-OP were compared. Follow-up till last clinic visit was also considered for long-term outcome. Eleven children (male 3; female 8) were included in the study. Mean delay between LP and ICPM was 112.8days (17-257days). There was lack of correlation between LP-OP and ICP in 9 children. ICP monitoring refuted the diagnosis of IIH in 80% of children and prevented exposure to unnecessary medical and surgical intervention. There was 1complication with ICPM. In 90% of children, there was no progression of symptoms following a decision based on ICP monitoring on long-term follow-up (mean, 36.5months). When the diagnosis of IIH is in doubt, LP may be unreliable and formal ICP monitoring is advised.

Optic nerve ultrasonography in monitoring treatment efficacy in pediatric idiopathic intracranial hypertension.

Measurement of optic nerve sheath diameter (ONSD) with ocular ultrasonography (USG) is a noninvasive technique that can be readily used to determine clues of increased intracranial pressure. In this study, we aimed to determine the role of optic nerve sheath diameter measurements in the diagnosis and follow-up of pediatric patients with idiopathic intracranial hypertension (IIH). Eight patients with a diagnosis of IIH with a median age of 11.7 (range 4.5-17) years were examined prospectively. During follow-up, orbital ultrasonography (USG) was performed immediately prior to lumbar puncture (LP) and at 24h, at 1week, and between 1 and 18months after LP. Cranial MRI examinations and automated visual field assessments were performed at baseline and at 3months, and both measurements were compared with each other. The mean cerebrospinal fluid opening pressure (37.75 ± 12.64cm H2O) and the mean ONSD (5.94 ± 0.46mm) were correlated. The median follow-up was 16 (range, 12-18months), and ONSD regressed gradually consistent with clinical and radiologic improvement during follow-up. To the best of our knowledge, this is the first prospective pilot study performed on pediatric patients with IIH using orbital USG for ONSD measurements. Despite the small sample size, the present study indicated that orbital USG may be used as a promising noninvasive tool to diagnose increased intracranial pressure and for monitoring treatment efficacy in this special patient population.

The United Kingdom Neuro-Ophthalmology Superheroes Day 2019

ABSTRACTA key theme of the United Kingdom Neuro-ophthalmology Superheroes Day was to address emerging topics in Neuro-Ophthalmology and the meeting included speakers from around the world. Rapid cases introduced topics which included non-arteritic anterior ischaemic optic neuropathy, imaging in optic nerve disorders, paediatric optic neuritis, mitochondrial disorders and idiopathic intracranial hypertension.

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.

Idiopathic intracranial hypertension in pediatric and adolescent patients

ObjectivesPediatric idiopathic intracranial hypertension is a rare condition, but inappropriate diagnosis and management may lead to devastating outcome with loss of vision and lifelong handicap. Dandy criteria are used for diagnosis of idiopathic intracranial hypertension (IIH) in adult, but these criteria cannot be applied in all pediatric patients.The aim of this study is to evaluate the diagnostic criteria and outcome of management of IIH in pediatric patients.MethodsNineteen patients with IIH and age less than 16 years old have been evaluated for the diagnostic criteria and outcome of management. The patients were classified according to the secondary sexual criteria into adolescent IIH and pediatric IIH. Full neurological and ophthalmological evaluation was completed in all patients. The patients were managed and followed up for a mean period of 12 ± 8.6 months at Zagazig University hospitals or Kingdom Hospital in the time period from 2009 to 2018.ResultsNineteen patients (16 females and 3 males) had been diagnosed and treated with idiopathic intracranial hypertension, their age is between 4 and 15 years, and the patients have been divided into two groups. Group I (pediatric IIH) involved 9 patients (6 females and 3 males), and their age is ranging between 4 and 9 years with mean age 5.56 ± 1.9 years while group II (adolescent IIH) involved 10 patients, all of them are females and their age ranges between 12 and 15 years with mean age 13.5 ± 1.3. Diagnostic criteria of the patients are papilledema, symptoms and signs of intracranial hypertension, and elevated CSF opening pressure with normal MRI. Headache, delayed school performance, and sixth nerve palsy are the most common clinical finding in the patients of group I while headache is the most common presenting symptom in group II. Anemia and otitis media are the most common associated risk factors in group I while obesity and female gender were the most common associated risk factors in group II. The minimum value for opening pressure in group I is 180 mmH2O while the minimum value in group II is 250 mm. 16 patients improved after the first lumbar puncture and drainage of CSF; two patients improved after repeated lumbar puncture while lumbo-peritoneal shunt was inserted for two patients, both of them were in group II. Recurrence was diagnosed in four patients (21%), one of them (11.1%) was in group I while three of them (30%) were in group II.ConclusionPediatric idiopathic intracranial hypertension can be classified into two subtypes: pediatric type and adolescent type according to the secondary sexual criteria.Modified Dandy criteria can be applied for the diagnosis of adolescent type, but pediatric type is not associated with obesity, has no female predominance, and usually responds to the initial lumbar puncture with a low rate of recurrence.

Influence of optic nerve appearance on final visual outcome in pediatric patients with idiopathic intracranial hypertension (IIH)

In adult IIH patients, Frisén grade, but not optic disc hemorrhages (ODH) or cotton wool spots (CWS), was an independent risk factor for worse visual outcomes, allowing for early intervention. We investigated these variables in pediatric IIH (PIIH) patients.

Asymptomatic Versus Symptomatic Idiopathic Intracranial Hypertension in Children.

Idiopathic intracranial hypertension is a rare neurologic condition characterized by elevated intracranial pressure with normal cerebrospinal fluid analysis and neuroimaging. A subset of pediatric idiopathic intracranial hypertension patients are coincidentally found to have papilledema and elevated intracranial pressure without symptoms (eg, headache, visual blurring, tinnitus). This study aims to investigate the features of asymptomatic pediatric idiopathic intracranial hypertension. Retrospective case-control study of patients aged 0 to 18 years who received idiopathic intracranial hypertension diagnosis from 2005 to 2016. Subjects were included if they met established diagnostic criteria for idiopathic intracranial hypertension diagnosis. Subjects were classified as symptomatic if they presented with 1 symptom related to elevated intracranial pressure, and asymptomatic if no symptoms were present. Statistical analysis was performed to compare the 2 groups. 12 (22.6%) of 53 pediatric idiopathic intracranial hypertension subjects were asymptomatic. Compared to symptomatic idiopathic intracranial hypertension, asymptomatic idiopathic intracranial hypertension had younger age of onset, lower initial opening pressure on lumbar puncture, lower optic nerve edema grades bilaterally, lower likelihood of globe flattening on magnetic resonance imaging (MRI), and smaller required dose of acetazolamide for resolution of papilledema (all P < .05). Asymptomatic idiopathic intracranial hypertension is common among pediatric patients with papilledema and is an important disease entity that requires special clinical management. It may exist as a milder version of idiopathic intracranial hypertension that occurs in younger children, or as a precursor state that later evolves into symptomatic disease.

Clinical features of pediatric idiopathic intracranial hypertension.

Introduction: Adult idiopathic intracranial hypertension (IIH) is often linked to obesity, however, causes of IIH in children are not well understood. This project identifies potential risk factors and features of pediatric IIH.Methods: This study consisted of a retrospective chart review of patients ages 5–17 years who were seen at a tertiary care children’s hospital and diagnosed with IIH. Diagnostic criteria included the presence of papilledema, normal neurological exam, normal neuroimaging, normal cerebrospinal fluid composition, and an opening pressure of a lumbar puncture >28 mmHg.Results: Of the 26 cases of IIH, 19 met all diagnostic criteria for this study, while seven patients were probable IIH, as they lacked papilledema. Intracranial pressure ranged from 28 to 66 mmHg, with a mean of 40.23 mmHg (±10.74). Overall, 50.0% (95% CI: 29.9–70.1%) of IIH patients were obese, with patients 12 years of age and younger exhibiting an overall obesity rate of 30.7% and patients 13 years of age and older having an obesity rate of 69.2%. The overall allergy rate in this IIH patient population was 46.2% (95% CI: 26.6–66.6%).Conclusion: Obesity appears to have no association with IIH in younger cases, but it is a more common feature in older children. An autoimmune component may play a role in pediatric IIH, given the high rate of atopy observed in this pediatric IIH patient cohort. Because a diagnosis of IIH can have an absence of optic nerve edema, taking a detailed history and performing a thorough examination are keys to diagnosing IIH in the pediatric population.

Clinical Features of Pediatric Idiopathic Intracranial Hypertension and Applicability of New ICHD-3 Criteria.

Idiopathic intracranial hypertension (IIH) is characterized by intracranial pressure >28 cmH2O in the absence of identifiable causes. Aim of this paper is to describe the clinical phenotype of pediatric IIH and to analyze the applicability of ICHD-3 criteria in comparison to the ICHD-2. We conducted a retrospective analysis of full clinical data of pediatric patients diagnosed with IIH between January 2007 and June 2018. Diagnostic evaluation included neuroimaging (all patients) and ultrasound-based optic nerve sheath diameter measurement (9 patients). Diagnosis of IIH was verified according to both ICHD-2 and ICHD-3 criteria for headache attributed to IIH, to verify the degree of concordance. We identified 41 subjects with suspected IIH; 14 were excluded due a diagnosis of secondary IH or lack of data. We therefore selected 27 subjects (age 4–15 years, mean 11). All patients presented with headache and bilateral papilloedema. Headache was daily in 22% cases, with diffuse gravative pain in 41%. In 4%, pain was exacerbated by cough, stress or tension. The most common presentation symptoms, in addition to headache, were blurred vision or diplopia (70%), vomiting (33%), and dizziness (15%). Twenty patients (74%) were obese. In 6 patients (22%) neuroimaging showed empty sella. Optic nerve sheath distension was detected in 6 out of 9 patients. Regarding the applicability of the ICHD-2 criteria, 18/27 (71%) patients have criterion A; 24/27 (89%) criterion B; 27/27 (100%) criterion C; 27/27 (100%) criterion D. When the ICHD-3 criteria were used, 27/27 (100%) fitted criterion A; 24/27 (89%) criterion B; 27/27 (100%) criterion C; and 27/27 (100%) criterion D. Our study suggests that, as compared with the ICHD-2, the new ICHD-3 criteria for headache attributed to IIH are better satisfied by pediatric patients with IIH. This is mainly due to the fact that qualitative headache characteristics are no longer considered in ICHD-3. Although the risk of under-rating the symptom of headache in IIH should not be disregarded, in pediatric population headache characteristics are usually less defined than in adults and obtaining a precise description of them is often very difficult.

Temporary Lumbar Drain as Treatment for Pediatric Fulminant Idiopathic Intracranial Hypertension: Comment.

Temporary Lumbar Drain as Treatment for Pediatric Fulminant Idiopathic Intracranial Hypertension: Comment.

Temporary Lumbar Drain as Treatment for Pediatric Fulminant Idiopathic Intracranial Hypertension: Response.

Temporary Lumbar Drain as Treatment for Pediatric Fulminant Idiopathic Intracranial Hypertension: Response.

Pediatric neuro-ophthalmology: not simply neuro-ophthalmology for small adults

Pediatric neuro-ophthalmology is a subspecialty within neuro-ophthalmology. Pediatric neuro-ophthalmic diseases must be considered separate from their adult counterparts, due to the distinctive nature of the examination, clinical presentations, and management choices. This manuscript will highlight four common pediatric neuro-ophthalmic disorders by describing common clinical presentations, recommended management, and highlighting recent developments. Diseases discussed include pediatric idiopathic intracranial hypertension (IIH), pseudopapilledema, optic neuritis (ON) and optic pathway gliomas (OPG). The demographics, diagnosis and management of common pediatric neuro-ophthalmic disease require a working knowledge of the current research presented herein. Special attention should be placed on the differences between pediatric and adult entities such that children can be appropriately diagnosed and treated.

Pediatric idiopathic intracranial hypertension - Is the fixed threshold value of elevated LP opening pressure set too high?

Idiopathic intracranial hypertension (IIH) in children is a rare condition of unknown etiology and various clinical presentations. The primary aim of this study was to evaluate if our pediatric IIH study group fulfilled the revised diagnostic criteria for IIH published in 2013, particularly with regard to clinical presentation and threshold value of an elevated lumbar puncture opening pressure. Additionally we investigated the potential utilization of MR-based and fundoscopic methods of estimating intracranial pressure for improved diagnosis. Clinical data were collected retrospectively from twelve pediatric patients diagnosed with IIH between 2008 and 2012 and revised diagnostic criteria were applied. Comparison with non-invasive methods for measuring intracranial pressure, MRI-based measurement (MR-ICP) and venous ophthalmodynamometry was performed. Only four of the twelve children (33%) fulfilled the revised diagnostic criteria for a definite diagnosis of IIH. Regarding noninvasive methods, MR-ICP (n=6) showed a significantly higher mean of intracranial pressure compared to a healthy age- and sex-matched control group (p=0.0043). Venous ophthalmodynamometry (n=4) showed comparable results to invasive lumbar puncture. The revised diagnostic criteria for IIH may be too strict especially in children without papilledema. MR-ICP and venous ophthalmodynamometry are promising complementary procedures for monitoring disease progression and response to treatment.

Clinical characteristics and outcome of pediatric idiopathic intracranial hypertension

Clinical characteristics and outcome of pediatric idiopathic intracranial hypertension

Update on the evaluation of pediatric idiopathic intracranial hypertension.

Papilledema associated with idiopathic intracranial hypertension (IIH) may result in irreversible, progressive visual loss. The development of tools for the evaluation of pediatric patients with IIH is particularly relevant as many patients may not be able to comply with the detailed clinical evaluation utilized in adults for the treatment and management of this disease. The purpose of this review is to summarize relevant articles on the diagnostic tools used in evaluation and management of pediatric IIH. Studies suggest that characteristic pediatric IIH MRI findings include empty sella turcica, decreased pituitary gland size, optic nerve tortuosity, perioptic subarachnoid space enlargement, posterior globe flattering, and intraocular protrusion of the optic nerve head. On optical coherence tomography (OCT), increased retinal nerve fiber layer and macular thickness may be observed in children with IIH compared with controls. The retinal nerve fiber layer thickness seems to coincide with the severity of papilledema and may be more sensitive than funduscopy for detecting optic nerve head elevation. Research on ultrasound of the optic nerve shows increased size of the optic nerve sheath diameter in pediatric IIH patients, and this may correlate with increased opening pressure on lumbar puncture. There appears to be characteristic findings on MRI, OCT, and ultrasound studies in pediatric IIH patients. Although ultrasound is rarely used for monitoring these patients nowadays, MRI and OCT can be useful in the evaluation and management of these individuals.