Progress and recognition of idiopathic intracranial hypertension: A narrative review.

BACKGROUND: Idiopathic intracranial hypertension (IIH) mainly affects obese young women, causing elevated intracranial pressure, headaches, and papilledema, risking vision loss and severe headaches. Despite weight loss as the primary treatment, the underlying mechanisms remain unclear. Recent research explores novel therapeutic targets. AIMS: This review aimed to provide a comprehensive understanding of IIH's pathophysiology and clinical features to inform pathogenesis and improve treatment strategies. METHODS: Recent publications on IIH were searched and summarized using PubMed, Web of Science, and MEDLINE. RESULTS: The review highlights potential pathomechanisms and therapeutic advances in IIH. CONCLUSION: IIH incidence is rising, with growing evidence linking it to metabolic and hormonal disturbances. Early diagnosis and treatment remain challenging.

Pulsatile Tinnitus: Differential Diagnosis and Approach to Management.

PURPOSE OF REVIEW: The purpose of this review is to provide an updated approach to the evaluation and management of pulsatile tinnitus (PT), an uncommon but often treatable subtype of tinnitus. RECENT FINDINGS: Secondary PT can be due to either vascular or non-vascular etiologies, including, but not limited to: neoplasm, arteriovenous malformation or fistula, idiopathic intracranial hypertension, dural venous sinus stenosis, otoacoustic etiologies (e.g., otosclerosis, patulous eustachian tube) and bony defects (e.g., superior semicircular canal dehiscence). Computed tomography (CT) and magnetic resonance imaging (MRI) imaging have comparable diagnostic yield, though each may be more sensitive to specific etiologies. If initial vascular imaging is negative and a vascular etiology is strongly suspected, digital subtraction angiography (DSA) may further aid in the diagnosis. Many vascular etiologies of PT can be managed endovascularly, often leading to PT improvement or resolution. Notably, venous sinus stenting is an emerging therapy for PT secondary to idiopathic intracranial hypertension with venous sinus stenosis. Careful history and physical exam can help establish the differential diagnosis for PT and guide subsequent evaluation and management. Additional studies on the efficacy and long-term outcome of venous sinus stenting for venous stenosis are warranted.

Novel Approaches to the Treatment of Idiopathic Intracranial Hypertension.

PURPOSE OF REVIEW: Idiopathic intracranial hypertension (IIH) typically affects women of childbearing age, is associated with recent weight gain, and can result in debilitating headache as well as papilledema that can cause vision loss. There have been advances in the medical and surgical treatment of affected patients with IIH that can improve outcomes and tolerability of therapy. RECENT FINDINGS: Medical treatment with agents that lower intracranial pressure through pathways other than carbonic anhydrase inhibition are being developed, and medically-directed weight loss as well as bariatric surgery now may be considered as primary therapy. New surgical options including venous sinus stenting have shown efficacy even with cases of severe vision loss. Our treatment options for IIH patients are becoming more diverse, and individualized treatment decisions are now possible to address specific components of the patient's disease manifestations and to lead to IIH remission.

Occipital venous sinus stenting for idiopathic intracranial hypertension and pulsatile tinnitus: A case series.

Venous sinus stenting for dural venous sinus outflow obstruction due to an intrinsic filling obstruction or extrinsic stenosis is an increasingly popular treatment strategy for idiopathic intracranial hypertension (IIH) and isolated pulsatile tinnitus (PT). The most common site of stenosis is the lateral venous sinus at the transverse-sigmoid junction. Approximately 10% of the population has a persistent occipital venous sinus (OVS), a variant that may be the dominant venous drainage pathway in the setting of a hypoplastic or aplastic transverse sinus. OVS stenosis has been rarely associated with IIH and isolated PT with only a handful published cases. We herein report a retrospective series of OVS stenting in five patients, four of whom presented with non-IIH PT and one with IIH.

Future Directions and Innovations in Venous Sinus Stenting.

This review explores the future role of venous sinus stenting (VSS) in the management of idiopathic intracranial hypertension and pulsatile tinnitus. Despite its favorable safety profile and clinical outcomes compared with traditional treatments, VSS is not yet the standard of care for these conditions, lacking high-level evidence data and guidelines for patient selection and indications. Current and recently completed clinical trials are expected to provide data to support the adoption of VSS as a primary treatment option. Additionally, VSS shows potential in treating other conditions, such as dural arteriovenous fistula and cerebral venous sinus thrombosis, and it is likely that the procedure will continue to see an expansion of its approved indications. The current lack of dedicated venous stenting technology is being addressed with promising advancements, which may improve procedural ease and patient outcomes. VSS also offers potential for expansion into modulation of brain electrophysiology via endovascular routes, offering exciting possibilities for neurodiagnostics and treatment of neurodegenerative disorders.

Venous sinus stenting for idiopathic intracranial hypertension: An updated Meta-analysis.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is characterized by elevated intracranial pressure and primarily affects obese women of reproductive age. Venous sinus stenting (VSS) is a surgical procedure used to treat IIH, but its safety and efficacy are still controversial. METHODS: A systematic review and meta-analysis were conducted following PRISMA guidelines. Multiple databases were searched for studies evaluating the safety and efficacy of VSS in IIH patients and meta-analysis was performed to pool the data. RESULTS: A total of 36 studies involving 1066 patients who underwent VSS were included. After VSS, a significant reduction in trans-stenotic gradient pressure was observed. Patients also showed significantly lower cerebrospinal fluid (CSF) opening pressure. Clinical outcomes demonstrated improvement in tinnitus (95%), papilledema (89%), visual disturbances (88%), and headache (79%). However, 13.7% of patients experienced treatment failure or complications. The treatment failure rate was 8.35%, characterized by worsening symptoms and recurrence of IIH. The complications rate was 5.35%, including subdural hemorrhage, urinary tract infection, stent thrombus formation, and others. CONCLUSION: VSS appears to be a safe and effective treatment option for IIH patients who are unresponsive to medical therapy or have significant visual symptoms. However, long-term outcomes and safety of the procedure require further investigation.

Prediction of Venous Trans-Stenotic Pressure Gradient Using Shape Features Derived From Magnetic Resonance Venography in Idiopathic Intracranial Hypertension Patients.

OBJECTIVE: Idiopathic intracranial hypertension (IIH) is a condition of unknown etiology associated with venous sinus stenosis. This study aimed to develop a magnetic resonance venography (MRV)-based radiomics model for predicting a high trans-stenotic pressure gradient (TPG) in IIH patients diagnosed with venous sinus stenosis. MATERIALS AND METHODS: This retrospective study included 105 IIH patients (median age [interquartile range], 35 years [27-42 years]; female:male, 82:23) who underwent MRV and catheter venography complemented by venous manometry. Contrast enhanced-MRV was conducted under 1.5 Tesla system, and the images were reconstructed using a standard algorithm. Shape features were derived from MRV images via the PyRadiomics package and selected by utilizing the least absolute shrinkage and selection operator (LASSO) method. A radiomics score for predicting high TPG (≥ 8 mmHg) in IIH patients was formulated using multivariable logistic regression; its discrimination performance was assessed using the area under the receiver operating characteristic curve (AUROC). A nomogram was constructed by incorporating the radiomics scores and clinical features. RESULTS: Data from 105 patients were randomly divided into two distinct datasets for model training (n = 73; 50 and 23 with and without high TPG, respectively) and testing (n = 32; 22 and 10 with and without high TPG, respectively). Three informative shape features were identified in the training datasets: least axis length, sphericity, and maximum three-dimensional diameter. The radiomics score for predicting high TPG in IIH patients demonstrated an AUROC of 0.906 (95% confidence interval, 0.836-0.976) in the training dataset and 0.877 (95% confidence interval, 0.755-0.999) in the test dataset. The nomogram showed good calibration. CONCLUSION: Our study presents the feasibility of a novel model for predicting high TPG in IIH patients using radiomics analysis of noninvasive MRV-based shape features. This information may aid clinicians in identifying patients who may benefit from stenting.

Technical considerations and long-term results of endovascular venous stenting to control venous hypertension from meningiomas invading intracranial venous sinuses.

OBJECTIVE: Meningiomas invading the intracranial venous sinuses may cause intracranial venous hypertension, papilledema, and visual compromise. Sinus resection and graft reconstructions, however, add significant complexity to tumor surgery, with the potential for increased morbidity. In this study, the authors explored whether venous sinus stenting might provide an alternative means of controlling venous hypertension that would be sustainable over the long term. METHODS: The authors performed a retrospective review of all 16 patients with intracranial meningiomas who underwent stenting at their institution for venous sinus compromise. At presentation, all had headache and 9 had papilledema. Thirteen patients had 1 meningioma and 3 had 2 or more. Three patients had had previous tumor resection and radiotherapy. One patient had been treated with a lumboperitoneal shunt and radiotherapy. The median length of clinical follow-up was 8 years (range 4 months-18 years). RESULTS: Venous sinus narrowing was often not confined to the site of meningioma, and bilateral transverse sinus narrowing, reminiscent of that seen in idiopathic intracranial hypertension, was present in 7 patients with sagittal sinus meningiomas. Eleven patients had stents placed solely across sinus narrowing caused by meningioma. Five patients had additional stents placed at other sites of venous narrowing at the same time: in one of these patients, a stent was placed across a defect in the sagittal sinus caused by previous surgery, and in the 4 other patients, stents were placed across nontumor narrowings of the transverse sinuses. In 1 patient, the jugular vein was also stented. Nine patients developed symptomatic in-stent restenosis at the meningioma site. Eight had further stenting procedures with variable success in restoring the in-stent lumen. The remaining patient, with a late partial relapse, is being reinvestigated. Papilledema resolved in all patients after stenting. Six patients experienced prolonged and very substantial relief of all symptoms. Five patients had persistent headache despite restoration of the sinus lumen. Five had persistent symptoms associated with resistant in-stent stenosis. There were no significant complications from any of the diagnostic or therapeutic procedures. CONCLUSIONS: In patients who are symptomatic with meningiomas obstructing the venous sinuses, successful stenting of the affected segment can give a good outcome, especially in terms of relieving papilledema. However, further procedures are often necessary to maintain stent patency, other areas of venous compromise frequently coexist, and some patients remain symptomatic despite apparently successful treatment of the index lesion. Long-term surveillance is a requirement.

Post-operative weight loss in venous sinus stenting patients: A multi-center review, systematic review, and meta-analysis.

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 (p = 0.0757). Patients at 6-month follow-up saw a weight decrease to 97.4 kg (p = 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 (p = 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.

Venous sinus stenting for idiopathic intracranial hypertension patients with functioning ventriculoperitoneal shunts: A case series.

Idiopathic intracranial hypertension (IIH) is a disease defined by increased intracranial pressure and associated with a variety of symptoms ranging from headaches to tinnitus. Ventricular peritoneal shunting has been the mainstay treatment for patients with IIH. Although VPS's have shown efficacy in treating IIH, some patients complain of refractory symptoms even with functioning VPS's. Venus stenting has emerged as a new technique for treating these refractory symptoms. Despite the scarce literature pertaining its efficacy and safety profile, several small studies have shown promising results. In this case series, four patients with IIH complained of refractory symptoms despite functioning VPS's and were treated with venous stenting.

Cerebral venous sinus stenting in idiopathic intracranial hypertension.

Cerebral venous sinus stenting (CVSS) is a minimally invasive procedure using endovascular stent placement to relieve elevated intracranial pressure secondary to venous sinus stenosis. Increased venous sinus pressure secondary to stenosis is commonly associated with elevated intracranial pressure without intracranial lesions on imaging or idiopathic intracranial hypertension (IIH). While the etiology of IIH remains unknown, stenosis of one or more of the dural sinuses has been implicated as a possible underlying mechanism. The manifestations of IIH include headaches, transient vision loss, pulsatile tinnitus, and neck pain. In this review, we discuss the recent studies that have demonstrated the e!ectiveness of CVSS for patients with IIH and also the indications, technical challenges, potential complications, and emerging developments in CVSS.

"Idiopathic" intracranial hypertension: An update from neurointerventional research for clinicians.

BACKGROUND: The recognition of venous sinus stenosis as a contributing factor in the majority of patients with idiopathic intracranial hypertension coupled with increasing cerebral venography and venous sinus stenting experience have dramatically improved our understanding of the pathophysiologic mechanisms driving this disease. There is now a dense, growing body of research in the neurointerventional literature detailing anatomical and physiological mechanisms of disease which has not been widely disseminated among clinicians. METHODS: A literature search was conducted, covering the most recent neurointerventional literature on idiopathic intracranial hypertension, the pathophysiology of idiopathic intracranial hypertension, and management strategies (including venous sinus stenting), and subsequently summarized to provide a comprehensive review of the most recently published studies on idiopathic intracranial hypertension pathophysiology and management. CONCLUSION: Recent studies in the neurointerventional literature have greatly improved our understanding of the pathophysiologic mechanisms causing idiopathic intracranial hypertension and its associated conditions. The ability to make individualized, patient-specific treatment approaches has been made possible by advances in our understanding of how venous sinus stenosis and cerebral venous hypertension fundamentally contribute to idiopathic intracranial hypertension.

Intracranial venous stenting for idiopathic intracranial hypertension.

INTRODUCTION: There is increasing evidence in the literature to support venous sinus stenting in patients with idiopathic intracranial hypertension who fail first-line therapy. Venous sinus stenting is a safe and successful technique compared with cerebrospinal fluid diversion procedures. This study examines the clinical outcomes of patients post intracranial venous stenting for intracranial hypertension across three tertiary hospitals in Western Australia. METHODS: A retrospective analysis was performed on 83 consecutive patients treated with intracranial venous stenting for IIH at three tertiary hospitals from October 2013 to March 2020. Data were collected from outpatient clinic letters, electronic discharge letters, electronic radiological imaging and procedural reports. RESULTS: 89.2% patients were able to cease Acetazolomide post stenting. 78.3% patients reported resolution of headaches. 84.3% patients demonstrated resolution of their papilloedema. 91.6% patients demonstrated improvement of their visual acuity. Compared with a recent meta-analysis by Satti and Chaudry in 2015, our results demonstrated a higher technical success rate and lower complication rates. CONCLUSION: Our study findings support the paradigm shift from CSF diversion procedures to venous sinus stenting in patients with IIH who fail first-line therapy. Venous sinus stenting has a high technical success rate and significantly lower complication rates than other invasive treatments.

Venous sinus stenting for the treatment of acute blindness in a patient with idiopathic intracranial hypertension.

We present a case of acute onset of blindness treated with venous sinus stenting in the hyperacute period (24-48 h). The patient had not been diagnosed previously with idiopathic intracranial hypertension and presented at an outside facility with a short history of headache, nausea, vomiting and visual deficits. Initial management included lumbar punctures for pressure relief. Unfortunately, the patient's condition deteriorated and she experienced the rapid onset of blindness. Within 24-48 h of blindness onset, she was transferred to our institution for emergent endovascular stenting of the venous sinuses. The intervention resulted in improvement of symptoms and recovery of visual acuity over a short-term follow-up period.

Opinion article: Neurosurgical treatment for neuro-ophthalmologic conditions.

A significant role of the neuro-ophthalmologist is to counsel patients on appropriate management and anticipated visual prognosis for conditions affecting the afferent and efferent visual systems, including those requiring neurosurgical treatment. However, the literature regarding anticipated neuro- ophthalmologic prognosis after neurosurgical intervention for cerebral aneurysms, sellar lesions, optic pathway tumors, and elevated intracranial pressure is limited with many key questions unanswered. For example, if a cerebral aneurysm is equally amenable to clipping or endovascular coiling, is there a preferred approach in terms of visual prognosis based on aneurysm location? Is dural venous sinus stenting (VSS) for idiopathic intracranial hypertension (IIH) superior, equivalent or inferior to shunting in terms of visual recovery and safety profile? Landmark studies on pituitary tumors using pre-operative optical coherence tomography (OCT) imaging of the optic nerve head to predict visual recovery after surgical decompression of the optic chiasm have changed neuro-ophthalmologic practice and enabled patients to be better informed regarding expected visual outcomes. 1,2 In order to optimize an interdisciplinary team approach to patient care, further studies of visual outcomes for neuro- ophthalmologic conditions requiring neurosurgical intervention are needed.

Dural Venous Sinus Stenting in Idiopathic Intracranial Hypertension: A National Database Study of 541 Patients.

BACKGROUND: Dural venous sinus stenting (VSS) is an effective intervention for patients with idiopathic intracranial hypertension (IIH) refractory to medical treatment. Our goal was to evaluate the efficacy by utilizing a large multi-institutional sample. METHODS: Five hundred forty-one patients >18 years old who underwent VSS within 3 years of IIH diagnosis were queried using Current Procedural Terminology and International Classification of Diseases, Tenth Revision codes from the TriNetX Analytics Network. Patient demographics, baseline symptoms, procedures, and clinical outcomes were evaluated within 1 year postoperatively. Outcomes examined were headache, tinnitus, blindness/low vision, optic nerve sheath fenestration (ONSF), cerebrospinal fluid (CSF) shunt, and use of medications (acetazolamide, methazolamide, furosemide, topiramate, tricyclic antidepressants, and valproate) for IIH. Prestent and poststent data were compared using Fisher exact test, and the odds ratios were computed using the Baptista-Pike method. RESULTS: The mean age at VSS was 36.7 ± 10.6; 92% were female, 65% of patients were Caucasian, 25% were Black/African American, 1% were Asian, and 9% were of other/unknown race. Within the 1-year follow-up, acetazolamide and topiramate use were significantly reduced post-VSS (P < 0.0001∗; odds ratio, 0.45; confidence interval, 0.35-0.57 and P = 0.03∗; odds ratio, 0.71; confidence interval, 0.52-0.95, respectively). Also, headaches, visual disturbance, dizziness/giddiness, and tinnitus significantly improved post-VSS (P < 0.005∗). Finally, the number of CSF shunt procedures and ONSF procedures demonstrated no significant change post-VSS (P > 0.05). CONCLUSIONS: VSS is an effective and safe procedure resulting in significant improvement of headaches, visual impairment, dizziness, and tinnitus, acetazolamide and topiramate usage were lower after VSS in patients with IIH. The paucity of pre-VSS and post-VSS CSF shunt and ONSF procedure data does not provide enough evidence to establish significance.

Cerebral Venous Outflow Implications in Idiopathic Intracranial Hypertension-From Physiopathology to Treatment.

In this review, we provide an update on the pathogenesis, diagnosis, and management of adults with idiopathic intracranial hypertension (IIH) and implications of the cerebral venous system, highlighting the progress made during the past decade with regard to mechanisms of the venous outflow pathway and its connection with the cerebral glymphatic and lymphatic network in genesis of IIH. Early diagnosis and treatment are crucial for favorable visual outcomes and to avoid vision loss, but there is also a risk of overdiagnosis and misdiagnosis in many patients with IIH. We also present details about treatment of intracranial hypertension, which is possible in most cases with a combination of weight loss and drug treatments, but also in selected cases with surgical interventions such as optic nerve sheath fenestration, cerebral spinal fluid (CSF) diversion, or dural venous sinus stenting for some patients with cerebral venous sinus stenosis, after careful analysis of mechanisms of intracranial hypertension, patient clinical profile, and method risks.

Current Indications for Management Options in Pseudotumor Cerebri.

There are surgical options available for those patients with idiopathic intracranial hypertension (IIH) who have significant visual threat or visual deterioration despite best medical management or whose visual deterioration is rapid enough to warrant urgent intervention. Optic nerve sheath fenestrations, venous sinus stenting, and cerebrospinal fluid diversion via ventriculoperitoneal and lumboperitoneal shunting are useful adjuncts in the management of this condition. Significant resources are used in the care of patients with IIH. Further understanding of the pathophysiology of IIH will likely direct future treatment options to more targeted therapeutics including surgery for IIH in the future.

New Options (or not) for Treatment of Idiopathic Intracranial Hypertension.

PURPOSE OF REVIEW: Idiopathic intracranial hypertension (IIH) is a disorder primarily affecting obese women of childbearing age and, if left untreated, can lead to irreversible vision loss. No consensus exists on the best management strategy for IIH. Weight loss is advocated and few useful medical options exist. There is an unmet need to discover new treatment options for this increasingly prevalent condition. This article reviews the recent advances and research on the treatment of IIH. RECENT FINDINGS: Venous sinus stenting (VSS) is now performed in many experienced centers, and there is growing interest in bariatric surgery as a treatment modality. Newly approved anti-obesity drugs are showing effectiveness in weight loss, and novel targeted disease-modifying IIH therapies are being explored. Further evaluation of these novel therapeutic strategies as well as studies exploring the use of anti-obesity drugs in IIH is needed. While VSS is gaining popularity due to its efficacy and low complication rate, there is insufficient evidence to support any surgical procedure over another. Bariatric surgery is appealing for patients with non-sight-threatening IIH and needs to be further explored.

Neuro-ophthalmological evaluation including optical coherence tomography surrounding venous sinus stenting in idiopathic intracranial hypertension with papilledema: a case series.

Venous sinus stenting (VSS) for idiopathic intracranial hypertension (IIH) has been demonstrated to achieve significant symptom improvement while harboring a low periinterventional morbidity profile. Comprehensive neuro-ophthalmological monitoring represents a cornerstone of disease monitoring. The value of optical coherence tomography (OCT) requires further exploration. Patients with IIH and papilledema who underwent VSS between 04/2018 and 02/2021 were retrospectively reviewed. Clinical and radiological were analyzed. Neuro-ophthalmological data included visual acuity, visual fields, fundoscopy categorized via Frisén scale, and OCT obtained retinal nerve fiber layer (RNFL) thickness were analyzed. Of 39 IIH patients who underwent cerebral angiography with transverse-sigmoid sinus pressure evaluation, 18 patients with IIH and papilledema underwent 21 transverse-sigmoid sinus stenting (TSST) procedures. After TSST, manometry showed a significant reduction of maximum transverse sinus pressures and trans-stenotic gradient pressures (p = 0.005 and p < 0.001, respectively). Chronic headaches, visual disturbance resolved and pulsatile tinnitus improved significantly. Visual fields remained similar, while papilledema Frisén scales and visual acuity significantly improved. The OCT calculated RNFL thickness significantly decreased in all patients. Stratification according to a minimal-low degree (Frisén 1-2) and moderate-marked degree (Frisén 3-4) papilledema demonstrated a significant reduction of RNFL thickness in both groups. Venous sinus stenting provides favorable clinical and neuro-ophthalmological outcomes. This study demonstrates that neuro-ophthalmologic testing augmented with OCT evaluation provides objective data that can be used as a biomarker for treatment success for managing patients with different extents of papilledema and may inform patient management.