Consensus recommendations for the assessment and management of idiopathic intracranial hypertension in children and young people.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a potentially disabling condition. There is a lack of evidence and national guidance on how to diagnose and treat paediatric IIH, leading to variation in clinical practice. We conducted a national Delphi consensus via the Children's Headache Network to propose a best-practice diagnostic and therapeutic pathway. METHODS: The Delphi process was selected as the most appropriate methodology for examining current opinion among experts in the UK. 104 questions were considered by 66 healthcare professionals, addressing important aspects of IIH care: assessment, diagnosis, treatment, follow-up and surveillance. General paediatricians, paediatric neurologists, ophthalmologists, opticians, neuroradiologists and neurosurgeons with a clinical interest or experience in IIH, were invited to take part. RESULTS: The Delphi process consisted of three rounds comprising 104 questions (round 1, 67; round 2, 24; round 3 (ophthalmological), 13) and was completed between March 2019 and August 2021. There were 54 and 65 responders in the first and second rounds, respectively. The Delphi was endorsed by the Royal College of Ophthalmologists, which engaged 59 ophthalmologists for round 3. CONCLUSIONS: This UK-based Delphi consensus process reached agreement for the management of paediatric IIH and has been endorsed by the Children's Headache Network and more broadly, the British Paediatric Neurology Association. It provides a basis for a pragmatic clinical approach. The recommendations will help to improve clinical care while minimising under and over diagnosis.

Assessment of the relationship between structural and functional tests in patients with idiopathic intracranial hypertension.

PURPOSE: To assess the relationship between structural and functional tests in mild and moderate idiopathic intracranial hypertension (IIH). METHODS: Patients with mild and moderate IIH and a control group were enrolled. Best-corrected visual acuity (BCVA), macular ganglion cell layer (MGCL) thickness, peripapillary retinal nerve fiber layer (pp RNFL) thickness, perimetric mean deviation (MD), and photopic negative responses (PhNR) of the electroretinogram were recorded. The associations between structural (pp RNFL and MGCL thickness) and functional (PhNR amplitude, MD and BCVA) parameters were assessed. RESULTS: 154 eyes from 78 subjects (74 eyes from IIH patients and 80 eyes from healthy subjects) were included in this comparative observational study. The MGCL thickness, VA, pp RNFL, and PhNR base-to-trough (BT) amplitude were significantly worse in moderate IIH. The BCVA and MD were associated with MGCL thickness only in moderate IIH. The relationship between MD and MGCL thickness started when MD fell below -5.7 dB. CONCLUSIONS: The association between functional and structural parameters varies between mild and moderate IIH. The MD and MGCL thickness outperformed in assessing disease severity in mild and moderate IIH, respectively. The association between MD and MGCL thickness could be considered in IIH severity categorization.

Social Determinants of Health in Idiopathic Intracranial Hypertension.

BACKGROUND: Idiopathic intracranial hypertension (IIH) disproportionately affects women from socioeconomically disadvantaged communities, but specific social determinants of health have not been examined. METHODS: We used data from the National Institutes of Health's All of Us Research Program, an ongoing nationwide study of more than 300,000 diverse individuals in the United States. Height and weight were measured at baseline, and participants completed questionnaires about demographics, health care access, and quality of life. Women aged 18-50 years with IIH were identified through electronic health record data, excluding those with venous thrombosis, meningitis, hydrocephalus, or central nervous system neoplasms. We used logistic regression to compare questionnaire responses for IIH cases and controls, adjusting for age, race, ethnicity, annual income, and body mass index (BMI). RESULTS: We included 416 women with IIH and 107,111 women without IIH. The mean age was 38 years, and 49.3% identified as non-White. After adjusting for age, race/ethnicity, and BMI, women with IIH were more likely to be unemployed (odds ratio [OR] 1.40, 95% confidential interval [CI]: 1.14-1.71) and report delaying care because of difficulty affording copays (OR 1.47, 95% CI: 1.02-2.10) or specialist care (OR 1.52, 95% CI: 1.06-2.18). They also delayed care because of rural residence (OR 2.08, 95% CI: 1.25-3.47) and transportation limitations (OR 2.23, 95% CI: 1.55-3.20). Although women with IIH were more likely to be non-Hispanic Black (OR 1.66, 95% CI: 1.32-2.09), this association lost significance when controlling for BMI and income (OR 1.27, 95% CI: 0.96-1.68). CONCLUSIONS: Women with IIH experience adverse social determinants of health beyond those associated with obesity alone.

Assessment of peripapillary choroidal thicknesses and optic disc diameters in idiopathic intracranial hypertension.

OBJECTIVE: To evaluate the peripapillary choroidal thickness and optic disc diameter changes in the eyes of patients who had idiopathic intracranial hypertension (IIH) when they had swollen discs and after resolution of the papilledema. DESIGN: This is an observational, cross-sectional study. Optical coherence tomography was performed on both eyes of patients with IIH, who had been divided into 2 groups: 18 patients with acute papilledema and 25 patients with resolved papilledema. Twenty healthy participants were also enrolled in this study. METHODS: In this observational, cross-sectional study, the thickness of the retinal nerve fibre layer, the peripapillary choroidal thickness (PCT), the ganglion cell complex thickness, and the optic disc diameters were measured using optical coherence tomography. RESULTS: In the resolved-papilledema subgroup, PCT in all quadrants was significantly lower than in the control group (p < 0.001 for each). In the acute-papilledema subgroup, PCT in the temporal, inferior, and superior quadrants was significantly less than in the control eyes (p < 0.001, p = 0.003, p = 0.049, respectively). The disc diameters in the vertical and horizontal planes were also significantly larger in the acute-papilledema eyes than in the control eyes and in the resolved papilledema eyes (p < 0.001 for each). CONCLUSION: PCT decreases both at the acute and chronic stages of IIH, and optic disc diameter increases in patients with IIH who have swollen discs.

Predictive Value of International Classification of Diseases Codes for Idiopathic Intracranial Hypertension in a University Health System.

BACKGROUND: Misclassification bias is introduced into medical claims-based research because of reliance on diagnostic coding rather than full medical record review. We sought to characterize this bias for idiopathic intracranial hypertension (IIH) and evaluate strategies to reduce it. METHODS: A retrospective review of medical records was conducted using a clinical data warehouse containing medical records and administrative data from an academic medical center. Patients with 1 or more instances of International Classification of Diseases (ICD)-9 or -10 codes for IIH (348.2 or G93.2) between 1989 and 2017 and original results of neuroimaging (head CT or MRI), lumbar puncture, and optic nerve examination were included in the study. Diagnosis of IIH was classified as definite, probable, possible, or inaccurate based on review of medical records. The positive predictive value (PPV) for IIH ICD codes was calculated for all subjects, subjects with an IIH code after all testing was completed, subjects with high numbers of IIH ICD codes and codes spanning longer periods, subjects with IIH ICD codes associated with expert encounters (ophthalmology, neurology, or neurosurgery), and subjects with acetazolamide treatment. RESULTS: Of 1,005 patients with ICD codes for IIH, 103 patients had complete testing results and were included in the study. PPV of ICD-9/-10 codes for IIH was 0.63. PPV in restricted samples was 0.82 (code by an ophthalmologist n = 57), 0.70 (acetazolamide treatment n = 87), and 0.72 (code after all testing, n = 78). High numbers of code instances and longer duration between the first and last code instance also increased the PPV. CONCLUSIONS: An ICD-9 or -10 code for IIH had a PPV of 63% for probable or definite IIH in patients with necessary diagnostic testing performed at a single institution. Coding accuracy was improved in patients with an IIH ICD code assigned by an ophthalmologist. Use of coding algorithms considering treatment providers, number of codes, and treatment is a potential strategy to reduce misclassification bias in medical claims-based research on IIH. However, these are associated with a reduced sample size.

Predictive Value of International Classification of Diseases Codes for Idiopathic Intracranial Hypertension in a University Health System.

BACKGROUND: Misclassification bias is introduced into medical claims-based research because of reliance on diagnostic coding rather than full medical record review. We sought to characterize this bias for idiopathic intracranial hypertension (IIH) and evaluate strategies to reduce it. METHODS: A retrospective review of medical records was conducted using a clinical data warehouse containing medical records and administrative data from an academic medical center. Patients with 1 or more instances of International Classification of Diseases (ICD)-9 or -10 codes for IIH (348.2 or G93.2) between 1989 and 2017 and original results of neuroimaging (head CT or MRI), lumbar puncture, and optic nerve examination were included in the study. Diagnosis of IIH was classified as definite, probable, possible, or inaccurate based on review of medical records. The positive predictive value (PPV) for IIH ICD codes was calculated for all subjects, subjects with an IIH code after all testing was completed, subjects with high numbers of IIH ICD codes and codes spanning longer periods, subjects with IIH ICD codes associated with expert encounters (ophthalmology, neurology, or neurosurgery), and subjects with acetazolamide treatment. RESULTS: Of 1,005 patients with ICD codes for IIH, 103 patients had complete testing results and were included in the study. PPV of ICD-9/-10 codes for IIH was 0.63. PPV in restricted samples was 0.82 (code by an ophthalmologist n = 57), 0.70 (acetazolamide treatment n = 87), and 0.72 (code after all testing, n = 78). High numbers of code instances and longer duration between the first and last code instance also increased the PPV. CONCLUSIONS: An ICD-9 or -10 code for IIH had a PPV of 63% for probable or definite IIH in patients with necessary diagnostic testing performed at a single institution. Coding accuracy was improved in patients with an IIH ICD code assigned by an ophthalmologist. Use of coding algorithms considering treatment providers, number of codes, and treatment is a potential strategy to reduce misclassification bias in medical claims-based research on IIH. However, these are associated with a reduced sample size.

Characterizing idiopathic intracranial hypertension socioeconomic disparities and clinical risk factors: A retrospective case-control study.

INTRODUCTION: Against the backdrop of the diverse minority-majority state of Hawaii, this study seeks to better characterize associations between idiopathic intracranial hypertension (IIH) with sociodemographic variables and medical comorbidities. METHODS: A retrospective case-control study was conducted by utilizing 54 IIH patients and 216 age-, sex-, and race-matched controls, 216 unmatched controls, and 63 age-, sex-, and race-matched migraine patients. RESULTS: Relative to controls, IIH were 25 years younger (p < 0.0001) and 10.18 kg/m2 heavier (p < 0.0001), as well as exhibited greater odds of the following variables (p < 0.05): female (odds ratio [OR]: 8.87), the lowest income quartile (OR: 2.33), Native Hawaiian or other Pacific Islander (NHPI; OR: 2.23), Native American or Alaskan Native (OR: 16.50), obesity class 2 (35.0-39.9 kg/m2; OR: 4.10), obesity class 3 (>40 kg/m2; OR: 6.10), recent weight gain (OR: 11.66), current smoker (OR: 2.48), hypertensive (OR: 3.08), and peripheral vascular disease (OR: 16.42). Odds of IIH were reduced (p < 0.05) for patients who were Asian (OR: 0.27) or students (OR: 0.30;). Unique from Whites, NHPI IIH patients exhibited greater odds (p < 0.05) for being from lower socioeconomic status and currently smoking, as well as potential association with seizures (p = 0.08). Compared to migraines, IIH headaches were at increased odds of occurring (p < 0.05) occipitally, for greater than 15 days per month, aggravated by postural changes, and comorbid with dizziness and tinnitus. CONCLUSIONS: These results not only better characterize IIH, but also highlight socioeconomic and racial disparities in diagnosis.

Noninvasive Intracranial Pressure Assessment in Patients with Suspected Idiopathic Intracranial Hypertension.

INTRODUCTION: Idiopathic intracranial hypertension (IIH) usually occurs in obese women of childbearing age. Typical symptoms are headache and sight disorders. Besides ophthalmoscopy, lumbar puncture is used for both diagnosis and therapy of IIH. In this study, noninvasively-assessed intracranial pressure (nICP) was compared to lumbar pressure (LP) to clarify its suitability for diagnosis of IIH. METHODS: nICP was calculated using continuous signals of arterial blood pressure and cerebral blood flow velocity, a method previously introduced by the authors. In thirteen patients (f = 11, m = 2; age: 36 ± 10 years), nICP was assessed 1 h prior to LP. If LP was >20 cmH2O (~15 mmHg), lumbar drainage was performed, LP was measured again, and nICP was reassessed. RESULTS: In six patients, LP and nICP were compared after lumbar drainage. In three patients, assessment of nICP versus LP was repeated. In total, LP and nICP correlated with R = 0.82 (p < 0.001; N = 22). Mean difference of ICP-nICP was 0.8 ± 3.7 mmHg. Presuming 15 mmHg as critical threshold for indication of lumbar drainage in 20 of 22 cases, the clinical implications would have been the same in both methods. CONCLUSION: TCD-based ICP assessment seems to be a promising method for pre-diagnosis of increased LP and might prevent the need for lumbar puncture if nICP is low.

Assessment of the optic nerve using strain and shear-wave elastography in patients with pseudotumour cerebri.

AIM: To evaluate the optic nerve using strain elastography (SE) and shear-wave elastography (SWE) in idiopathic intracranial hypertension (IIH) patients in comparison to participants in the control group. MATERIALS AND METHODS: Eighty eyes were evaluated in 40 cases consisting of 20 IIH patients and 20 participants in the control group. This study was conducted using SE and SWE in addition optic nerve sonography measurements of participants in the IIH patient group and the control group. SE patterns were categorised using three main types and two subtypes. Quantitative measurements of optic nerve stiffness with SWE were expressed in kilopascals. RESULTS: In the IIH patient group, type 2 and type 1 elasticity patterns were primarily observed, followed by type 3 patterns. In the control group, type 3 elasticity patterns were most often observed, while type 2 elasticity patterns were seen less frequently. Statistically significance differences in the types of elasticity strain patterns were observed between the groups (p<0.01). Quantitative analysis was also performed, and the SWE moduli were obtained for the control group (10.1±0.28 kPa) and the IIH patient group (26.97±1 kPa). A statistically significant difference in the SWE modulus was found between the groups (p<0.01). CONCLUSION: Biomechanical changes may have occurred in the optic nerve secondary to increased intracranial pressure in IIH patients. Strain and shear elastography may have potential as assistive diagnostic techniques for the detection and follow-up of these changes.

Assessment of the Olfactory Function in Patients With Idiopathic Intracranial Hypertension Using the Sniffin' Sticks Test: A Case-Control Study.

OBJECTIVE: Despite the lack of recognition in clinical practice, there is increasing evidence that patients with idiopathic intracranial hypertension may suffer from hyposmia. The current case-control study aims to evaluate olfactory dysfunction in a large series of patients with idiopathic intracranial hypertension. METHODS: All subjects, 44 idiopathic intracranial hypertension patients and 57 healthy controls, underwent olfactory function assessment using standardized "Sniffin' Sticks" test at a tertiary referral center of a university hospital. Threshold, discrimination, identification, and total threshold-discrimination-identification scores have been determined and analyzed statistically. RESULTS: Idiopathic intracranial hypertension patients had significantly lower threshold (6.5 [3.69] vs 8 [1.88], P < .001, 95% CI [-2.250, -0.750]) and threshold-discrimination-identification scores (29.75 [5.56] vs 32.5 [5.25], P = .003, 95% CI [-4.250, -0.750]). Twenty-five patients (57%) were diagnosed with hyposmia. Test scores of patients with active idiopathic intracranial hypertension (n = 18) were not statistically different from patients with inactive disease (n = 26), except for discrimination score (14 [2.50] vs 11 [2.25], P = .005, 95% CI [-3.000, -1.000]). Although idiopathic intracranial hypertension patients with a cerebrospinal fluid opening pressure of ≥330 mmH2 O had lower test scores, the difference was significant only for total threshold-discrimination-identification scores (28.5 [5.50] vs 30.5 [4.38], P = .044, 95% CI [0.750, 5.500]). Multiple regression analysis revealed that test scores were related to disease activity, cerebrospinal fluid opening pressure, papilledema, headache, and medication. CONCLUSION: Our clinical study revealed significant olfactory dysfunction in patients with idiopathic intracranial hypertension compared with healthy controls. Future research should employ larger samples to search for usability of olfactory testing in clinical management of patients with idiopathic intracranial hypertension.

Emergency department utilization among individuals with idiopathic intracranial hypertension.

PURPOSE: Idiopathic intracranial hypertension (IIH) can be a debilitating disorder that is difficult to identify and treat. Failure to adequately manage IIH symptoms may force patients to present at emergency departments (EDs) seeking symptom relief. The purpose of this paper is to empirically characterize ED use by previously diagnosed IIH patients. DESIGN/METHODOLOGY/APPROACH: Patients diagnosed with IIH, and who registered with the Intracranial Hypertension Registry by 2014, were solicited for study inclusion. A survey was designed to elicit ED use during the period 2010-2012. Information on demographic and socioeconomic characteristics, IIH signs and symptoms, time since diagnosis, perspectives of ED use and quality of life was collected. Quality of life was assessed using an adaptation of the Migraine-Specific Quality of Life Questionnaire. Data were analyzed using descriptive statistics and nonparametric hypothesis tests. FINDINGS: In total, 39 percent of IIH patients used emergency services over the study period; those that did used the services intensely. These patients were more likely to be non-white, live in households making less than $25,000 annually, have public insurance and have received a diversional shunt procedure. Patients who used the ED were less likely to live in households making $100,000, or more, annually and have private insurance. Participants who used the ED had significantly lower quality-of-life scores, were younger and had been diagnosed with IIH for less time. ORIGINALITY/VALUE: ED staff and outside physicians can utilize the information contained in this study to more effectively recognize the unique circumstances of IIH patients who present at EDs.

Bedside Optic Nerve Sheath Diameter Assessment in the Identification of Increased Intracranial Pressure in Suspected Idiopathic Intracranial Hypertension.

OBJECTIVE: We determined whether the bedside assessment of the optic nerve sheath diameter could identify elevated intracranial pressure in individuals with suspected idiopathic intracranial hypertension. METHODS: This was a single-center, prospective, rater-blinded study performed in a freestanding pediatric teaching hospital. Patients aged 12 to 18 years scheduled for an elective lumbar puncture with the suspicion of idiopathic intracranial hypertension were eligible to participate. Optic nerve sheath diameter was measured via ultrasonography before performing a sedated lumbar puncture for measuring cerebrospinal fluid opening pressure. Abnormal measurements were predefined as optic nerve sheath diameter ≥4.5 mm and a cerebrospinal fluid opening pressure greater than 20 cmH2O. RESULTS: Thirteen patients participated in the study, 10 of whom had elevated intracranial pressure. Optic nerve sheath diameter was able to predict or rule out elevated intracranial pressure in all patients. CONCLUSIONS: Noninvasive assessment of the optic nerve sheath diameter could help to identify patients with elevated intracranial pressure when idiopathic intracranial hypertension is suspected.

A practical approach to, diagnosis, assessment and management of idiopathic intracranial hypertension.

Adult patients who present with papilloedema and symptoms of raised intracranial pressure need urgent multidisciplinary assessment including neuroimaging, to exclude life-threatening causes. Where there is no apparent underlying cause for the raised intracranial pressure, patients are considered to have idiopathic intracranial hypertension (IIH). The incidence of IIH is increasing in line with the global epidemic of obesity. There are controversial issues in its diagnosis and management. This paper gives a practical approach to assessing patients with papilloedema, its investigation and the subsequent management of patients with IIH.

Assessment of visual function in idiopathic intracranial hypertension.

PURPOSE: Impaired visual function occurs frequently in patients with idiopathic intracranial hypertension (IIH) and may be the most seriously affected function following raised intracranial pressure. The aim of this review was to overview the methods available for the assessment of visual function in IIH. METHODS: A review of databases, including PubMed, Web of Knowledge and ocular and motility and strabismus was undertaken to identify articles relating to visual function and its assessment in IIH. RESULTS: Options for visual assessment include fundus evaluation, retinal imaging, visual field, visual acuity, contrast sensitivity, colour vision, electrodiagnostic and ocular motility evaluations. CONCLUSIONS: Many aspects of visual function assessment contribute to the monitoring of vision in patients with IIH. However, the assessment of visual field, plus fundus and retinal imaging, are particularly important in providing accurate and repeatable measurements on which to determine the progression of the condition.

Assessment of quality of continuous intracranial pressure recordings in children.

BACKGROUND: Bad quality of continuous intracranial pressure (ICP) recordings may potentially give wrong diagnostic information. METHOD: This study examined the ICP recordings of 33 children undergoing ICP monitoring during a 12-month period. Their ICP recordings were stored as raw data files and analyzed retrospectively. An automatic algorithm applied to every subsequent 6-second time window of each recording assessed the quality of ICP recordings. Time windows containing 4-18 cardiac beat-induced single ICP waves were accepted; time windows containing artifact-induced pressure waves due to noise in the signal were rejected. For every ICP recording, the number of accepted and rejected time windows were determined. In addition, the raw data ICP signal was assessed by visual inspection to verify the automatic algorithm. Two methods of computing mean pressure of a time window are compared; mean ICP was either computed according to current practice independent of the presence or absence of single ICP waves or computed only for single ICP waves within the time window (mean(SW) ICP). RESULTS: In 4 of the 33 patients (12%), more than 70% of the time windows of their ICP recordings were rejected; in 3 patients 95-100% of time windows were rejected. Visual inspection of the raw ICP signals confirmed the absence of single ICP waves. In these cases, computing mean(SW) ICP, not by mean ICP, revealed the bad signal quality. CONCLUSIONS: This retrospective study showed that in 3 of 33 patients (9%) the quality of the ICP recordings was so bad that they gave wrong diagnostic information. One way of obtaining quality control is to compute mean(SW) ICP, determined only for cardiac beat-induced single ICP waves within a time window.

[Quantitative assessment of the cerebrospinal fluid dynamic parameters in patients with the "benign" intracranial hypertension syndrome. 2]. / Kolichestvennaia otsenka parametrov u bol'nykh s sindromom "dobrokachestvennoi" vnutricherepnoi gipertenzii. Chast' 2.

The development of the benign intracranial hypertensive syndrome was shown to be followed by changes in liquorodynamic parameters and elastic properties of the brain. In 99% of patients, the baseline liquor pressure was elevated by 1.5-2.5 times as compared to normal values. Resistance of liquor resorption was enhanced in 82% of cases, suggesting impaired normal liquor outflow. The increased brain elasticity gradient suggests the exhaustion of cerebrospinal compensatory potentialities and correlates with the increased liquor resorption resistance. A correlation analysis of liquorodynamic parameters by taking into account clinical signs yielded a pathogenetic scheme of the development of the benign intracranial hypertensive syndrome.

Assessment of CSF dynamics and venous flow in the superior sagittal sinus by MRI in idiopathic intracranial hypertension: a preliminary study.

A velocity-sensitive magnetic resonance imaging (MRI) phase-mapping method was used for non-invasive study of cerebrospinal fluid (CSF) flow in the cerebral aqueduct, for indirect calculation of supratentorial CSF production, and for measurement of blood flow in the superior sagittal sinus (SSS). We examined 12 patients with idiopathic intracranial hypertension (IIH; pseudotumour cerebri), and 10 healthy volunteers. The peak caudal and rostral CSF flow in the aqueduct during the cardiac cycle did not differ significantly between the patients and the volunteers. A significant correlation was found between the CSF volume flow amplitude and the resistance to cerebrospinal fluid outflow in the patients (p < 0.05). The calculated mean supratentorial CSF production rate was 0.79 ml/min in the patients and 0.70 ml/min in the controls, but this difference was not statistically significant. However, the MRI measurements suggested CSF hypersecretion in three patients, whereas increased transependymal passage of CSF could have been the cause of negative calculated CSF production rates in two others. A tendency towards lower mean blood flow in the SSS (mean 345 ml/min) in the patients than in the controls (mean 457 ml/min) was found, and in two patients showed very low values. We showed that MRI phase-mapping may be used to study the relative importance of the pathophysiological factors thought to play a role in the development of IIH.

[Diagnostic value of cranial computerized tomography in pediatric practice]. / Valoración diagnóstica de la tomografía computarizada craneal en clínica pediátrica.

A total of 100 clinical records have been studied to as certain the diagnostic use of brain CT. Most frequent diagnoses are: hypoxic-ischemic encephalopaty, seizure disorders, psychomotor delay, head enlargement and trauma. The first years of life was the period with a higher frequency of applications and the greater percentage of pathologic results (75-80 por 100) was found among intracranial hypertension, fetal distress, macrocephaly or infections of Central Nervous System and their sequelae. Minus than 50 por 100 of CT scans requested for seizures, psychomotor delay and trauma demonstrated true abnormal findings. Nearly half (53 por 100) of exams were pathological and 87 por 100 of them were useful to confirm or complete the diagnosis. It is possible to predict an increase in the number of examinations to perform in the future, although it many be replaced by alternative techniques.