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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 -
Neurosurgery Apr 2024Although venous sinus stenting (VSS) improves cerebrospinal fluid reabsorption and decreases intracranial pressure in patients with idiopathic intracranial hypertension... (Meta-Analysis)
Meta-Analysis
BACKGROUND AND OBJECTIVES
Although venous sinus stenting (VSS) improves cerebrospinal fluid reabsorption and decreases intracranial pressure in patients with idiopathic intracranial hypertension (IIH), the underlying pathophysiology of IIH is not well understood. We present a review and meta-analysis of the literature on VSS for IIH treatment, focusing on the rates of restenosis and symptom recurrence.
METHODS
We performed a systematic review of PubMed and Embase databases between January 1, 2011, and December 31, 2021. Articles including ≥5 patients with IIH and venous sinus stenosis treated with VSS and post-treatment rates of restenosis (de novo stenosis at a different anatomic location along the dural sinuses or restenosis within or adjacent to the stent) were selected. Demographic, procedural, and outcomes data were collected and analyzed. Mean values for variables collected were pooled, and a mean value was calculated with a 95% CI.
RESULTS
Twenty-four articles were included, comprising 694 patients and 781 VSS cases. The mean age was 33.9 (CI, 31.5-36.2) years. The mean body mass index was 35.3 (CI, 32.9-37.7) kg/m 2 . Before VSS, 98.8% (CI, 96.8%-100.0%) of patients experienced headaches, 87.7% (CI, 80.6%-95.5%) had visual acuity issues, 78.7% (CI, 69.9%-88.5%) had papilledema, 58.3% (CI, 46.0%-73.9%) had tinnitus, and 98.8% (96.4%-100.0%) had symptoms refractory to previous therapies. After VSS, 77.7% (CI, 71.1%-84.95%) experienced symptom improvement and 22.3% (CI, 15.1%-29.0%) had persistent or worsened symptoms. Pooled restenosis rate was 17.7% (CI, 14.9%-20.9%).
CONCLUSION
VSS is effective in alleviating IIH signs and symptoms, but the associated high rates of restenosis and persistent symptoms highlight the need for further investigation of this procedure and other adjunctive treatments for IIH.
Topics: Humans; Adult; Pseudotumor Cerebri; Constriction, Pathologic; Treatment Outcome; Intracranial Pressure; Cranial Sinuses; Stents; Intracranial Hypertension; Retrospective Studies
PubMed: 37830801
DOI: 10.1227/neu.0000000000002718 -
Journal of Neuro-ophthalmology : the... Mar 2024There are an increasing number of controlled clinical trials and prospective studies, ongoing and recently completed, regarding management options for idiopathic...
BACKGROUND
There are an increasing number of controlled clinical trials and prospective studies, ongoing and recently completed, regarding management options for idiopathic intracranial hypertension (IIH). We present a Common Design and Data Element (CDDE) analysis of controlled and prospective IIH studies with the aim of aligning essential design and recommending data elements in future trials and enhancing data synthesis potential in IIH trials.
METHODS
We used PubMed and ClinicalTrials.gov to screen for ongoing and published trials assessing treatment modalities in people with IIH. After our search, we used the Nested Knowledge AutoLit platform to extract pertinent information regarding each study. We examined outputs from each study and synthesized the data elements to determine the degree of homogeneity between studies.
RESULTS
The most CDDE for inclusion criteria was the modified Dandy criteria for diagnosis of IIH, used in 9/14 studies (64%). The most CDDE for outcomes was change in visual function, reported in 12/14 studies (86%). Evaluation of surgical procedures (venous sinus stenting, cerebrospinal fluid shunt placement, and others) was more common, seen in 9/14 studies (64%) as compared with interventions with medical therapy 6/14 (43%).
CONCLUSIONS
Although all studies have similar focus to improve patient care, there was a high degree of inconsistency among studies regarding inclusion criteria, exclusion criteria, and outcomes measures. Furthermore, studies used different time frames to assess outcome data elements. This heterogeneity will make it difficult to achieve a consistent standard, and thus, making secondary analyses and meta-analyses less effective in the future. Consensus on design of trials is an unmet research need for IIH.
Topics: Humans; Pseudotumor Cerebri; Prospective Studies; Neurosurgical Procedures; Stents; Intracranial Hypertension
PubMed: 37342870
DOI: 10.1097/WNO.0000000000001902 -
British Journal of Neurosurgery Dec 2023The purpose of this study is to systematically review the literature on the clinical outcomes following different surgical techniques in patients with refractory... (Review)
Review
OBJECTIVE
The purpose of this study is to systematically review the literature on the clinical outcomes following different surgical techniques in patients with refractory idiopathic intracranial hypertension (IIH).
BACKGROUND
IIH is a condition characterised by increased cranial pressure (ICP) in the absence of an intracranial lesion that does not adequately respond to different medical and surgical therapies. Cranial decompression or expansion surgeries are a last resort therapy for patients with refractory IIH.
METHODS
A systematic literature search of the databases of PubMed, Embase and Medline from inception to 2019 was performed. Searches were limited to the English language and to clinical studies. Studies were included if clinical outcomes following different cranial decompression or expansion techniques were reported. We also add one case of our own experience with performing a bilateral frontoparietal expansion craniotomy and subtemporal craniectomy.
RESULTS
Five manuscripts, describing 38 procedures, met the inclusion criteria. Thirty-one patients were female (82%). The mean age was 26.2 years. The techniques studied included subtemporal craniectomy (27/38, 71%), internal cranial expansion (10/38, 26%), and cranial morcellation decompression (1/38, 3%). Thirty-five patients presented with headaches of which 17 noted postoperative improvement or resolution (49%). Visual deficits were documented in 30 patients and 25 reported postoperative improvement (83%). Papilledema disappeared in 23 of 32 patients with this sign at presentation (72%). In our patient, symptoms completely resolved postoperatively and a 6% increase in intracranial volume was measured.
CONCLUSIONS
Cranial vault decompression or expansion surgeries may be an effective last resort therapy for patients with refractory IIH. These surgeries expand the intracranial volume, and thus may normalise ICP, leading to clinical improvement.
Topics: Humans; Female; Adult; Male; Pseudotumor Cerebri; Skull; Papilledema; Craniotomy; Decompression; Intracranial Hypertension
PubMed: 34969345
DOI: 10.1080/02688697.2021.2022097 -
Nutrients Jul 2022Idiopathic intracranial hypertension (IIH) is a neurological disorder characterised by optic disc swelling secondary to raised intracranial pressure (ICP) of unknown... (Review)
Review
Idiopathic intracranial hypertension (IIH) is a neurological disorder characterised by optic disc swelling secondary to raised intracranial pressure (ICP) of unknown cause. Obesity is the most established and prevalent risk factor in developed countries. As obesogenic diets are high in calories and nutrient-poor, there may be associated nutritional deficiencies that contribute to the clinical presentation of IIH. Yet none, aside from iron deficiency, are currently included in the inclusion or exclusion criteria for the diagnosis of IIH. Our primary aim was to determine which micronutrient deficiencies, aside from iron deficiency, could present with optic disc swelling associated with or without intracranial hypertension that could potentially meet current IIH diagnostic criteria. To this end, we conducted a systematic search of articles published between 1 January 1980 and 18 December 2020 reporting cases of optic disc swelling associated with micronutrient deficiencies. In total, 65 cases met the eligibility criteria from initial searches: all were case reports and case series with a high risk of bias. Our findings suggest that patients with IIH or unexplained optic disc swelling ought to be screened, investigated, and treated for associated micronutrient deficiencies in vitamin A, B1 and B12; and weight loss interventions in IIH patients ought to promote better nutrition in addition to overall calorie restriction.
Topics: Humans; Intracranial Hypertension; Malnutrition; Micronutrients; Optic Disk; Papilledema; Pseudotumor Cerebri
PubMed: 35893919
DOI: 10.3390/nu14153068 -
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 -
Journal of Neuroimaging : Official... 2024Transorbital sonography (TOS) provides a noninvasive tool to detect intracranial pressure by assessing optic nerve sheath diameter (ONSD) and optic disc elevation (ODE).... (Meta-Analysis)
Meta-Analysis
BACKGROUND AND PURPOSE
Transorbital sonography (TOS) provides a noninvasive tool to detect intracranial pressure by assessing optic nerve sheath diameter (ONSD) and optic disc elevation (ODE). The utility of TOS in the diagnosis of idiopathic intracranial hypertension (IIH) has been increasingly recognized.
METHODS
A single-center case-control study sought to compare TOS-acquired ONSD and ODE among IIH-cases versus patients with other neurological diseases (controls). Furthermore, a systematic review and meta-analysis was conducted to present pooled mean differences and diagnostic measures of ONSD and ODE between IIH-cases and controls.
RESULTS
In the single-center study, consisting of 31 IIH-cases and 34 sex- and age-matched controls, ONSD values were higher among IIH-cases than controls (p<.001), while ODE was more prevalent in cases (65% vs. 15%; p<.001). The receiver-operating characteristic (ROC)-curve analysis revealed that the optimal cutoff value of ONSD for predicting IIH was 5.15 mm, with an area under the curve (AUC) of 0.914 (95% confidence interval [CI]: 0.861-0.967) and sensitivity and specificity values of 85% and 90%, respectively. In a meta-analysis of 14 included studies with 415 IIH-cases, ONSD and ODE values were higher in IIH-cases than controls (mean difference in ONSD 1.20 mm; 95% CI: 0.96-1.44 mm and in ODE 0.3 mm; 95% CI: 0.33-0.67 mm). With regard to ONSD, pooled sensitivity, specificity, and diagnostic odds ratio were calculated at 85.5% (95% CI: 77.9-90.8%), 90.7% (95% CI: 84.6-94.5%), and 57.394 (95% CI: 24.597-133.924), respectively. The AUC in summary ROC-curve analysis was 0.878 (95% CI: 0.858-0.899) with an optimal cutoff point of 5.0 mm.
CONCLUSIONS
TOS has a high diagnostic utility for the noninvasive diagnosis of IIH and may deserve wider implementation in everyday clinical practice.
Topics: Humans; Pseudotumor Cerebri; Case-Control Studies; Optic Nerve; Ultrasonography; Intracranial Pressure; Intracranial Hypertension
PubMed: 37822030
DOI: 10.1111/jon.13160 -
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 -
Interventional Neuroradiology : Journal... Oct 2023Idiopathic intracranial hypertension (IIH) can result in headaches, pulsatile tinnitus, papilledema, and other neurological symptoms. Weight loss can alleviate some...
INTRODUCTION
Idiopathic intracranial hypertension (IIH) can result in headaches, pulsatile tinnitus, papilledema, and other neurological symptoms. Weight loss can alleviate some symptoms of IIH. This study aims to investigate the effect of venous sinus stenting on post-operative weight and body mass index (BMI) changes among IIH patients. The secondary objective is to investigate the relationship between post-operative weight changes and papilledema resolution.
METHODS
Patients with papilledema undergoing venous sinus stenting for IIH were retrospectively reviewed from two comprehensive North American cerebrovascular centers. Patient demographics, surgical course, post-operative outcomes, and weight were analyzed. A systematic review and meta-analysis of post-operative weight in stented IIH patients was conducted.
RESULTS
Twenty-eight patients with a diagnosis of IIH and papilledema who underwent venous sinus stenting were identified across two institutions. Patients had a mean pre-operative weight of 103.2 kg, which decreased to 101.5 kg at 3-month follow up ( = 0.0757). Patients at 6-month follow-up saw a weight decrease to 97.4 kg ( = 0.0066). Patients who saw papilledema resolution saw a mean greater decrease in weight (-4.5%) at 6-month follow up than those whose papilledema did not resolve (-1.7%), although this was insignificant ( = 0.1091). A total of 41 patients were included in the meta-analysis. Patients had an average of 1.1 increase in BMI at 3-month follow up.
CONCLUSIONS
This study shows that venous sinus stenting leads to modest weight reduction in IIH patients, and those with resolved papilledema experience slightly greater weight loss. Further research is necessary to determine the clinical significance of these findings.
PubMed: 37807823
DOI: 10.1177/15910199231190596 -
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