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Clinical Neurology and Neurosurgery Oct 2021Idiopathic Intracranial Hypertension (IIH) typically occurs in obese (BMI >30 kg/m) females of childbearing age in the absence of any apparent intracranial... (Review)
Review
Idiopathic Intracranial Hypertension (IIH) typically occurs in obese (BMI >30 kg/m) females of childbearing age in the absence of any apparent intracranial space-occupying lesion. Patients typically present with headache, nausea, vomiting, tinnitus, and blurry vision secondary to increased intracranial pressure, with more severe cases involving cranial neuropathies and ophthalmological manifestations. Complete ophthalmoplegia is a rare event in IIH. In such cases, aggressive management with pharmacological, endovascular, and surgical intervention is essential to hasten recovery and limit long-term neurological and visual deficits. Herein, we present a rare case of a patient with IIH associated with third, fourth, and sixth cranial nerve palsies, resulting in complete unilateral ophthalmoplegia, who underwent dural sinus stenting and 2.5-year follow-up revealed complete resolution with full extraocular movements. We also perform a systematic literature review of complete and partial ophthalmoplegia secondary to IIH, highlighting the associated presentations, pathophysiology, management, and outcomes.
Topics: Adult; Cranial Sinuses; Female; Humans; Ophthalmoplegia; Pseudotumor Cerebri; Stents; Treatment Outcome
PubMed: 34560385
DOI: 10.1016/j.clineuro.2021.106910 -
BMJ Open Aug 2021To evaluate the diagnostic capability of optical coherence tomography (OCT) in children aged under 18 years old with intracranial hypertension (IH).
OBJECTIVES
To evaluate the diagnostic capability of optical coherence tomography (OCT) in children aged under 18 years old with intracranial hypertension (IH).
DESIGN
Systematic review.
METHODS
We conducted a systematic review using the following platforms to search the keywords 'optical coherence tomography' and 'intracranial hypertension' from inception to 2 April 2020: Cochrane Central Register of Controlled Trials, EMBASE, MEDLINE, PubMed and Web of Science, without language restrictions. Our search returned 2729 records, screened by two independent screeners. Studies were graded according to the Oxford Centre for Evidence-Based Medicine and National Institutes of Health Quality Assessment Tool for observational studies.
RESULTS
Twenty-one studies were included. Conditions included craniosynostosis (n=354 patients), idiopathic IH (IIH; n=102), space-occupying lesion (SOL; n=42) and other pathology (n=29). OCT measures included optic nerve parameters, rim parameters (notably retinal nerve fibre layer thickness) and retinal parameters. Levels of evidence included 2b (n=13 studies), 3b (n=4) and 4 (n=4). Quality of 10 studies was fair and 11 poor. There was inconsistency in OCT parameters and reference measures studied, although OCT did demonstrate good diagnostic capability for IH in craniosynostosis, IIH and SOL.
CONCLUSIONS
This systematic review identified various studies involving OCT to assist diagnosis and management of IH in children with craniosynostosis, IIH, SOL and other pathology, in conjunction with established clinical measures of intracranial pressure. However, no level 1 evidence was identified. Validating prospective studies are, therefore, required to determine optimal OCT parameters in this role and to develop formal clinical guidelines.
PROSPERO REGISTRATION NUMBER
CRD42019154254.
Topics: Adolescent; Child; Humans; Intracranial Hypertension; Intracranial Pressure; Prospective Studies; Pseudotumor Cerebri; Tomography, Optical Coherence
PubMed: 34380720
DOI: 10.1136/bmjopen-2020-046935 -
Survey of Ophthalmology 2022Idiopathic intracranial hypertension (IIH) is increased intracranial pressure without a known cause. Dural venous sinus stenting (DVSS) is a relatively new intervention... (Review)
Review
Idiopathic intracranial hypertension (IIH) is increased intracranial pressure without a known cause. Dural venous sinus stenting (DVSS) is a relatively new intervention for treatment of IIH refractory to medical therapy and lifestyle modifications. In this review, we outline various hypotheses of IIH pathogenesis and describe the role of venous sinus stenosis and the technical details of DVSS. We also present a summary and critique of the available evidence describing the outcomes of DVSS in IIH and review the evidence-based guidelines for this procedure. We conclude that, although many studies have shown generally favorable outcomes of DVSS in patients with IIH, most have serious limitations, the most common one being paucity of pre- and postprocedure ophthalmological data. Thus, there is not enough available evidence to conclude whether DVSS is an effective procedure for treatment of IIH. We also present the most commonly used indications for DVSS as described in the literature and stress the importance of neuro-ophthalmological assessment before and after the procedure to monitor response and potential complications.
Topics: Cranial Sinuses; Humans; Intracranial Hypertension; Pseudotumor Cerebri; Retrospective Studies; Stents
PubMed: 34004224
DOI: 10.1016/j.survophthal.2021.05.002 -
Neurosurgical Review Apr 2021Idiopathic intracranial hypertension denotes raised intracranial pressure in the absence of an identifiable cause and presents with symptoms relating to elevated ICP,...
Idiopathic intracranial hypertension denotes raised intracranial pressure in the absence of an identifiable cause and presents with symptoms relating to elevated ICP, namely headaches and visual deterioration. Treatment of IIH aims at reducing intracranial pressure, relieving headache and salvaging patients' vision. Surgical interventions are recommended for medically refractory IIH and include CSF diversion techniques, optic nerve sheath fenestration, bariatric surgery and venous sinus stenting. Prospective studies on the surgical options for IIH are scant and no evidence-based guidelines for the surgical management of medically refractory IIH have been established. A search in Cochrane Library, MEDLINE and EMBASE from 1 January 1985 to 19 April 2019 for controlled or observational studies on the surgical treatment of IIH (defined in accordance with the modified Dandy or the modified Friedman criteria) in adults yielded 109 admissible studies. VSS improved papilledema, visual fields and headaches in 87.1%, 72.7% and 72.1% of the patients respectively, with a 2.3% severe complication rate and 11.3% failure rate. CSF diversion techniques diminished papilledema, visual field deterioration and headaches in 78.9%, 66.8% and 69.8% of the cases and are associated with a 9.4 severe complication rate and a 43.4% failure rate. ONSF ameliorated papilledema, visual field defects and headaches in 90.5, 65.2% and 49.3% of patients. Severe complication rate was 2.2% and failure rate was 9.4%. This is currently the largest systematic review for the available operative modalities for IIH. VSS provided the best results in headache resolution and visual outcomes, with low failure rates and a very favourable complication profile. In light of this, VSS ought to be regarded as the first-line surgical modality for the treatment of medically refractory IIH.
Topics: Adult; Female; Headache; Humans; Male; Neurosurgical Procedures; Observational Studies as Topic; Prospective Studies; Pseudotumor Cerebri; Stents; Vision Disorders; Visual Fields
PubMed: 32335853
DOI: 10.1007/s10143-020-01288-1 -
Canadian Journal of Surgery. Journal... Mar 2020Idiopathic intracranial hypertension (IIH) is a rare condition typically affecting women with obesity who are of child-bearing age. Patients commonly present with...
BACKGROUND
Idiopathic intracranial hypertension (IIH) is a rare condition typically affecting women with obesity who are of child-bearing age. Patients commonly present with headaches, visual disturbances, pulsatile tinnitus and papilledema. The association between IIH and obesity has been well established in the literature, suggesting that weight loss may contribute to improving IIH. For patients with severe obesity for whom conservative management is not successful, bariatric surgery is an effective modality for weight loss. We aimed to systematically review the literature to determine the efficacy of bariatric surgery in the treatment of IIH
METHODS
We conducted a comprehensive search of MEDLINE, Embase, Scopus, the Cochrane Library and Web of Science (limited to studies in humans published in English between January 1946 and July 2015).
RESULTS
Twelve primary studies (n = 39 patients) were included in the systematic review. All patients had a preoperative diagnosis of IIH. Preoperative body mass index (BMI) was 47.4 ± 3.6 kg/m2 ; BMI improved to 33.7 ± 2.1 kg/m2 and 33.9 ± 11.6 kg/m2 at 6 and 12 months postoperatively, respectively. Lumbar puncture opening pressures decreased from 34.4 ± 6.9 cmH2O to 14.0 ± 3.6 cmH2O after surgery. Common symptoms of IIH improved after bariatric surgery: headaches (100% preoperatively v. 10% postoperatively), visual complaints (62% v. 44%), tinnitus (56% v. 3%) and papilledema (62% v. 8%).
CONCLUSION
Bariatric surgery appears to lead to considerable improvement in IIH. Idiopathic intracranial hypertension is not a well-publicized comorbidity of obesity, but its presence may be considered as an indication for bariatric surgery.
Topics: Bariatric Surgery; Body Mass Index; Headache; Humans; Obesity; Papilledema; Pseudotumor Cerebri; Tinnitus; Vision Disorders
PubMed: 32195557
DOI: 10.1503/cjs.016616 -
European Journal of Radiology Jul 2019To systematically review the potential value of MRI signs in the assessment of intracranial hypertension (IIH). (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To systematically review the potential value of MRI signs in the assessment of intracranial hypertension (IIH).
METHODS
MEDLINE and Embase were systematically searched for original studies investigating the accuracy of MRI signs in diagnosing IIH. Methodologic quality of included studies was assessed. Sensitivity and specificity were pooled with a bivariate random-effects model.
RESULTS
Twenty-one studies, comprising a total of 724 patients with IIH, were included. All studies had a case-control design. "Empty" sella (11 studies), posterior displacement of pituitary stalk (2 studies), meningoceles (2 studies), posterior globe flattening (8 studies), optic nerve head protrusion (6 studies), optic nerve enhancement (3 studies), optic nerve sheath distension (12 studies), optic nerve tortuosity (7 studies), slit-like ventricles (4 studies), tight subarachnoid spaces (3 studies), and inferior position of cerebellar tonsils (4 studies) had pooled sensitivity ranging between 6.1% and 68.6%, and pooled specificity ranging between 84.0% and 99.2%. Transverse sinus stenosis (8 studies) had pooled sensitivity of 84.4%; (95% CI: 65.9-93.9%) and pooled specificity of 94.9% (95% CI: 91.7-96.9%).
CONCLUSION
"Empty" sella, posterior pituitary stalk displacement, meningoceles, posterior globe flattening, optic nerve head protrusion, optic nerve enhancement, optic nerve sheath distension, optic nerve tortuosity, slit-like ventricles, tight subarachnoid spaces, and inferior position of cerebellar tonsils have overall high specificity but low sensitivity. Transverse sinus stenosis appears to be the most useful sign, because it has high specificity and fairly high sensitivity.
Topics: Brain; Female; Humans; Intracranial Hypertension; Magnetic Resonance Imaging; Male; Sensitivity and Specificity
PubMed: 31153551
DOI: 10.1016/j.ejrad.2019.04.023 -
Ultraschall in Der Medizin (Stuttgart,... Apr 2019In patients with idiopathic intracranial hypertension (IIH), transorbital sonography (TOS) may reveal an enlargement of the optic nerve sheath diameter (ONSD) and the... (Meta-Analysis)
Meta-Analysis
In patients with idiopathic intracranial hypertension (IIH), transorbital sonography (TOS) may reveal an enlargement of the optic nerve sheath diameter (ONSD) and the presence of optic disc elevation (ODE), as a sign of an increase in intracranial pressure (ICP). We systematically reviewed the TOS findings in adults with IIH. MEDLINE, EMBASE, Cochrane Library and CENTRAL (1966 - May 2017) were searched to identify studies reporting data on patients with IIH assessed by B mode-TOS. Data were extracted and included in a meta-analysis, and the quality of the included studies was evaluated. 5 studies with 96 patients were included. The values of ODE were 0.8 - 1.2 mm and ONSD was 6.2 - 6.76 mm in IIH patients vs. 4.3 - 5.7 mm in controls. In IIH patients the ONSD was significantly higher compared to controls (overall weighted mean difference of 1.3 mm (95 % CI: 0.6 - 1.9 mm)). The meta-analysis of proportion of papilledema based on results of three studies revealed a pooled estimator of 87 % (95 % CI: 76 - 94 %). IIH patients have higher ONSD values and higher frequency of ODE compared to controls. The indirect, noninvasive ICP assessment using TOS may be useful in supporting the clinical diagnosis of IIH in adults by detecting increased ONSD values and the presence of ODE.
Topics: Adult; Brain; Humans; Intracranial Hypertension; Intracranial Pressure; Optic Nerve; Pseudotumor Cerebri; Ultrasonography
PubMed: 30347420
DOI: 10.1055/a-0719-4903 -
Journal of Neurointerventional Surgery Apr 2019Stenting of the intracranial venous sinuses is used as a treatment in certain cases of idiopathic intracranial hypertension (IIH). Interest in, and experience of, this... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Stenting of the intracranial venous sinuses is used as a treatment in certain cases of idiopathic intracranial hypertension (IIH). Interest in, and experience of, this technique is growing, particularly in recent years. We sought to provide an updated systematic review and meta-analysis of the use of venous stenting in these patients, examining clinical outcomes.
METHODS
A literature search of venous stenting in IIH patients was performed. Using random-effects meta-analysis, we evaluated the following outcomes: clinical resolution of papilledema; headaches and pulsatile tinnitus; recurrence of symptoms after stenting; and complications.
RESULTS
Twenty articles from 18 different centers were included. In a total of 474 patients. 418 were female (88%). The mean age of the patients was 35, while the mean body mass index (BMI) was 35 kg/m. Median follow-up was 18 months. The overall rate of improvement in papilloedema was 93.7% (95% CI 90.5% to 96.9%), while the overall rate of improvement or resolution of headache was 79.6% (95% CI 73.3% to 85.9%). Pulsatile tinnitus resolved in 90.3% (95% CI 83.8% to 96.70%), while the overall rate of recurrence of IIH symptoms after stenting was 9.8% (95% CI 6.7% to 13%). The rate of major complications was 1.9% (95% CI 0.07% to 3.1%).
CONCLUSIONS
Venous sinus stenting in patients with IIH who are refractory to medical therapy appears to have an excellent safety profile and is associated with significant improvements in headaches, pulsatile tinnitus, and papilledema.
Topics: Cranial Sinuses; Female; Headache; Humans; Intracranial Hypertension; Male; Middle Aged; Observational Studies as Topic; Papilledema; Prospective Studies; Pseudotumor Cerebri; Recurrence; Retrospective Studies; Stents; Tinnitus
PubMed: 30166333
DOI: 10.1136/neurintsurg-2018-014172 -
European Journal of Neurology Oct 2018Idiopathic intracranial hypertension (IIH) is positively associated with obesity, mostly in young women. The global increase in obesity may influence the burden of IIH.... (Meta-Analysis)
Meta-Analysis
Idiopathic intracranial hypertension (IIH) is positively associated with obesity, mostly in young women. The global increase in obesity may influence the burden of IIH. Using the PubMed, Embase, MEDLINE and Web of Science databases, a meta-analysis and systematic review of epidemiological studies of IIH were performed up to June 2017. Temporal changes in IIH incidence were measured, and incidence rates of IIH were correlated with country-specific World Health Organization obesity rates. Prevalence data and shunting rates of IIH were recorded. The quality of epidemiological studies was assessed using the Standards of Reporting of Neurological Disorders (STROND) criteria. In 15 identified studies, there were 889 patients (87% women), mean age 29.8 years. The incidence of IIH ranged from 0.03 to 2.36 per 100 000 per year. The pooled incidence of IIH was 1.20 per 100 000 per year although there was very high heterogeneity (I 98%). The incidence rates of IIH were correlated with country-specific prevalence of obesity (Spearman's correlation 0.82, P < 0.01). The prevalence of IIH was rarely recorded. A shunting procedure was reported in 8% of patients. STROND criteria were variably reported, median of 26.5 of 43 (range 16-35). IIH is a public health concern as increased obesity prevalence is associated with increased incidence of IIH. A better quality of epidemiological studies is required to improve understanding of IIH and inform health policy for IIH management.
Topics: Adult; Comorbidity; Databases, Factual; Female; Humans; Incidence; Male; Middle Aged; Obesity; Prevalence; Pseudotumor Cerebri; Young Adult
PubMed: 29953685
DOI: 10.1111/ene.13739 -
Journal of Neurosurgery Mar 2018The role of venous sinus stenting (VSS) for idiopathic intracranial hypertension (IIH) is not well understood. The aim of this systematic review is to attempt to...
OBJECTIVE
The role of venous sinus stenting (VSS) for idiopathic intracranial hypertension (IIH) is not well understood. The aim of this systematic review is to attempt to identify subsets of patients with IIH who will benefit from VSS based on the pressure gradients of their venous sinus stenosis.
METHODS
MEDLINE/PubMed was searched for studies reporting venous pressure gradients across the stenotic segment of the venous sinus, pre- and post-stent pressure gradients, and clinical outcomes after VSS. Findings are reported according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines.
RESULTS
From 32 eligible studies, a total of 186 patients were included in the analysis. Patients who had favorable outcomes had higher mean pressure gradients (22.8 ± 11.5 mm Hg vs 17.4 ± 8.0 mm Hg, p = 0.033) and higher changes in pressure gradients after stent placement (19.4 ± 10.0 mm Hg vs 12.0 ± 6.0 mm Hg, p = 0.006) compared with those with unfavorable outcomes. The post-stent pressure gradients between the 2 groups were not significantly different (2.8 ± 4.0 mm Hg vs 2.7 ± 2.0 mm Hg, p = 0.934). In a multivariate stepwise logistic regression controlling for age, sex, body mass index, CSF opening pressure, pre-stent pressure gradient, and post-stent pressure gradient, the change in pressure gradient with stent placement was found to be an independent predictor of favorable outcome (p = 0.028). Using a pressure gradient of 21 as a cutoff, 81/86 (94.2%) of patients with a gradient > 21 achieved favorable outcomes, compared with 82/100 (82.0%) of patients with a gradient ≤ 21 (p = 0.022).
CONCLUSIONS
There appears to be a relationship between the pressure gradient of venous sinus stenosis and the success of VSS in IIH. A randomized controlled trial would help elucidate this relationship and potentially guide patient selection.
Topics: Blood Pressure; Cranial Sinuses; Humans; Neurosurgical Procedures; Pseudotumor Cerebri; Stents; Treatment Outcome
PubMed: 29498569
DOI: 10.3171/2017.8.JNS17459