Management Of Idiopathic Intracranial Hypertension Research Articles

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

BackgroundIdiopathic 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...

Foundations of the Diagnosis and Management of Idiopathic Intracranial Hypertension and Pulsatile Tinnitus

Venous sinus stenosis has garnered increasing academic attention as a potential etiology of idiopathic intracranial hypertension (IIH) and pulsatile tinnitus (PT). The complex anatomy of the cerebral venous sinuses and veins plays a crucial role in the pathophysiology of these conditions. Venous sinus stenosis, often found in the superior sagittal or transverse sinus, can lead to elevated intracranial pressure (ICP) and characteristic IIH symptoms. Stenosis, variations in dural venous anatomy, and flow dominance patterns contribute to aberrant flow and subsequent PT. Accurate imaging plays a vital role in diagnosis, and magnetic resonance (MR) venography is particularly useful for detecting stenosis. Management strategies for IIH and PT focus on treating the underlying disease, weight management, medical interventions, and, in severe cases, surgical or endovascular procedures. Recently, venous sinus stenting has gained interest as a minimally invasive treatment option for IIH and PT. Stenting addresses venous sinus stenosis, breaking the feedback loop between elevated ICP and stenosis, thus reducing ICP and promoting cerebrospinal fluid outflow. The correction and resolution of flow aberrances can also mitigate or resolve PT symptoms. While venous sinus stenting remains an emerging field, initial results are promising. Further research is needed to refine patient selection criteria and evaluate the long-term efficacy of stenting as compared to traditional treatments.

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.

Undiagnosed sagittal synostosis as cause of "idiopathic" intracranial hypertension.

Idiopathic intracranial hypertension (IIH) is a rare condition in children, but if diagnosed needs to be promptly treated to avoid clinical sequalae. The main purpose of this paper was to test our clinical experience with a cohort of normocephalic children with craniosynostosis who do not present in the routine way to craniofacial services, due to the normal head shape and age, diagnosed with IIH. We retrospectively reviewed all children who were referred to neurosurgery from 2012 to 2022 for management of IIH on our prospectively kept database. We determined what treatments were offered and if there was an associated craniosynostosis. In total, 19 children were identified with an average age at referral of 11.5years (st dev 4.0years) with 11 male and 8 female. The most common presenting symptoms and signs were papilloedema (18/19), headaches (15/19), visual deterioration (9/19), nausea and vomiting (7/19) and diplopia (4/19). Five out of 19 children (26.3%) had a sagittal suture fused that was not identified at the time of treatment and all children were normocephalic. There is a cohort of children with IIH who will have concomitant craniosynostosis and ideally would benefit from cranial vault expansion as primary surgery rather than cerebrospinal fluid (CSF) diversion. We suggest all children with IIH requiring neurosurgical intervention have cross-sectional imaging to look for occult craniosynostosis prior to intervention.

Management and Outcome of Pregnancy in Patients With Idiopathic Intracranial Hypertension: A Prospective Case Series Study.

Idiopathic intracranial hypertension (IIH) is a syndrome of increased intracranial pressure without an identifiable cause that mostly affects obese persons of childbearing age. In this prospective case series, we have evaluated the overall outcome of pregnancy and birth in participants with IIH and their newborn children. We also provide a proposal for the management of pregnant persons with IIH. In this observational study, neuro-ophthalmological findings, the course of IIH-related symptoms, disease management, and pregnancy outcomes were evaluated. The participants were divided into 3 groups according to the course of the disease during pregnancy: stable, worsened, and new diagnosed. Furthermore, the type of delivery and outcome of newborn children such as gestational age, weight at birth, and the presence of asphyxia were compared between the groups. We observed 47 pregnancies in 42 participants; 2 had spontaneous abortions. There were 19 (47%) participants in the stable, 18 (45%) in the worsened, and 3 (8%) in the new diagnosed groups, respectively. A relapse of IIH occurred in 2 (5%). Worsening of IIH-related symptoms was experienced by 18/37 (49%) participants: headache by 17/18 (94%), tinnitus by 11/18 (61%), and vision by 7/18 (39%) (mostly in the first and second trimester). In 8/18 (44%), the symptoms were transient or alleviated in the second and third trimester. Body mass index before and after pregnancy did not significantly differ among the groups. A total of 8 participants were treated with acetazolamide. The frequency of cesarean section was 17/40 (43%). Preterm delivery occurred in 22%. No increased risk of asphyxia was observed, and all infants, but one, were healthy. Worsening of headache, tinnitus, and/or vision were experienced by half of pregnant participants with IIH, mostly transient in the first and second trimester, rarely required specific treatment, and were not identified as a relapse of IIH. There was no difference in gestational age and weight at birth in children among the groups, and no perinatal asphyxia was noted. Weight gain in the participants was not identified as a risk factor for relapse of IIH in pregnancy. The rate of cesarean and preterm delivery was higher than in the non-IIH population. A proposal for the management of IIH in pregnancy is provided.

Workup and Management of Idiopathic Intracranial Hypertension.

Workup and Management of Idiopathic Intracranial Hypertension.

A Case Series on Clinical Profile, Risk Factors and Management of Idiopathic Intracranial Hypertension

Introduction Idiopathic intracranial hypertension (IIH) occurs when there is raised intracranial pressure (ICP) of unknown etiology and is diagnosed when all other causes of raised ICP have been excluded1 . IIH most commonly affects women of child-bearing age and overweight women2 . However, men, women of all ages and children of both genders are affected3 . Previously the terms like pseudo tumor cerebri and benign intracranial hypertension were used; however, in view of impending loss of vision it cannot be considered benign. Hence the term IIH is used nowadays. The diagnosis of IIH was made using modified Dandy criteria which includes presence of raised intracranial pressure, papilloedema, normal magnetic resonance imaging (MRI) and elevated CSF opening pressure with normal CSF composition. In the absence of papilledema, the diagnosis is challenging and requires specific neuroimaging criteria to be fulfilled.

Idiopathic intracranial hypertension: expanding our understanding.

Idiopathic intracranial hypertension (IIH) affects predominantly overweight women of childbearing age, causing chronically disabling headaches and visual loss. Weight loss remains the most effective management strategy, but innovative treatments and randomized control trials (RCTs) remain few. This paper will review recent IIH research. Pregnancy-related complications, but not losses, are increased in IIH, while symptom severity is not affected. Weight loss of 24% results in normalization of intracranial pressure (ICP) and improvement in papilledema. Prolonged periods of papilledema results in delayed thinning of the ganglion cell layer. Less-invasive telemetry has improved understanding of the positional effects on ICP with rises seen in the supine and lateral decubitus. Exenatide, a GLP-1 agonist, may reduce ICP and improve symptoms. Venous sinus stenting is increasingly popular but its benefits over CSF diversion remains unclear. Early involvement of obstetric care is recommended with pregnancy in IIH. Early intervention is required to avoid chronic papilledema that confers worse visual outcomes. Positional changes may affect ICP readings. The use of novel ICP telemetric devices has significant potential in future disease monitoring. The dual benefits of weight loss and ICP reduction with exenatide have significant potential in IIH management. Surgical RCTs are still required.

Endoscopic endonasal optic nerve sheath decompression (EONSD) for idiopathic intracranial hypertension: Technical details and meta-analysis

BackgroundThe neurosurgical management of idiopathic intracranial hypertension (IIH) remains controversial. Although shunting and newer endovascular stenting techniques are part of the neurosurgical armamentarium to treat medically refractory IIH symptoms, optic nerve sheath fenestration has traditionally been performed by ophthalmologists. ObjectiveWe present a detailed cadaveric dissection that simulates the endoscopic endonasal optic nerve sheath decompression (EONSD) technique along with the literature review. MethodsEONSD was performed in four freshly injected cadaveric specimens. Additionally, a systematic review from different electronic databases has been done. ResultsBilateral EONSD was performed in all specimens without significant technical difficulties. Based on our experience, there is no need to expose the periorbita or orbital apex. The primary anatomic landmarks were the optic canal, the lateral opticocarotid recess, the tuberculum, the limbus, and the clinoid segment of the internal carotid artery. Based on the systematic review, 68 patients (77.9% female) underwent EONSD, with a mean age of 33.4 ± 6.9 years in adult patients. Follow-up ranged from 3 to 58 months across different studies. The pooled meta-analysis showed headache, papilledema, and visual disturbance improvement in 78% [95%CI 0.65–0.90], 72% [95%CI 0.61–0.83], and 88% [95%CI 0.80–0.96] of cases who underwent EONSD, respectively. The subgroup analysis showed there was no statistically significant difference between unilateral and bilateral EONSD in terms of different measured outcomes. ConclusionEONSD is a feasible surgical procedure that may obviate the need for shunting in patients with IIH. Although clinical studies showed that EONSD is a safe and effective technique, further studies are required to establish the preferences of either unilateral or bilateral approaches.

Fulminant Idiopathic Intracranial Hypertension Refractory to Treatment Associated to SARS-CoV-2: A Case Report (P14-4.004)

<h3>Objective:</h3> NA <h3>Background:</h3> Idiopathic Intracranial Hypertension (IIH) is characterized by increased intracranial pressure, which most frequently occurs in childbearing-age obese women. Scarce reports of COVID-19 infection associated IIH exist and have only described this in women and children. “Fulminant IIH” is defined as intracranial hypertension without secondary cause, with severe vision loss within 4 weeks of onset, and progressive vision loss over days. A 36-year-old man with obesity came to the Ophthalmology clinic for evaluation of vision loss and headache, the patient tested positive for SARS-CoV-2 one month before this. He was identified to have bilateral grade V papilledema, confirmed with optical coherence tomography (OCT), and admitted to Neurology for IIH management. The patient was started intravenous therapy with high-dose steroids and acetazolamide without relief of headache or vision improvement (20/100). Lumbar puncture was performed, revealing an opening pressure of ≥55 cm H20. The MRI only showed findings of increased intracranial hypertension, and the MRV ruled out thrombosis. The lack of improvement in his headaches and vision loss ultimately required ventriculoperitoneal shunt placement. The patient had immediate subjective visual improvement after this, with unchanged visual acuity. On follow-up 4 weeks later, the patient continued to have headaches and the opening pressure was 30 cm H20. Lumboperitoneal shunt was placed. Both optic nerves became completely atrophic. Despite finally reaching an opening pressure of 15 cm H20, headaches did not resolve, and the patient currently only perceives light. <h3>Design/Methods:</h3> NA <h3>Results:</h3> NA <h3>Conclusions:</h3> This case represents, to our best knowledge, the first description of fulminant IIH associated with SARS-CoV-2. The previous reports described mild to moderate cases in women and children with response to treatment. This occurred to an adult male who despite receiving promptly treatment, had a poor response. Further studies are needed to determine if atypical clinical phenotypes are associated with refractory forms. <b>Disclosure:</b> Dr. Murguia Fuentes has nothing to disclose. Mr. Murguia Fuentes has nothing to disclose. Dr. Adamu has nothing to disclose. Dr. Carvajal- Gonzalez has nothing to disclose. Dr. Arvas has nothing to disclose.

Abstract Number ‐ 12: First‐In‐Human Endovascular Treatment of Idiopathic Intracranial Hypertension Using a Miniature Biomimetic Transdural Shunt.

Introduction Successful percutaneous transvenous deployment of a miniature valved biomimetic transdural endovascular shunt (CereVasc eShunt, Auburndale, MA, USA) via an inferior petrosal sinus approach was recently described for treatment of post‐subarachnoid hemorrhage communicating hydrocephalus.The endovascular shunt replicates the function of the arachnoid granulation by draining cerebrospinal fluid (CSF) from the cerebello‐pontine angle cistern to the ipsilateral internal jugular vein. Idiopathic intracranial hypertension (IIH), usually resulting from venous transverse sinus stenosis, can be treated with pharmacological inhibition of CSF production, surgical ventriculoperitoneal shunting, or using venous stent angioplasty. In IIH, elevated CSF pressure can act to exacerbate venous sinus stenosis, resulting in worsening CSF reabsorption, thereby perpetuating a vicious cycle. The authors sought to evaluate the role of the minimally invasive eShunt approach in IIH management. Methods A 50‐year‐old male patient with history of dyslipidemia and IIH initially presented 6 years ago with diplopia initially treated with poorly tolerated acetazolamide and periodic lumbar punctures with subsequent symptom improvement. The patient was admitted following rapid evolution of sudden onset horizontal diplopia and headache. Brain magnetic resonance revealed flattening of the posterior sclera, partially empty Sella Turcica, enhancement of the prelaminar optic nerves and enlarged Meckel´s cave. Lumbar puncture was performed with opening pressure of 28 cmH2O. Cerebral angiography with 3D venography confirmed bilateral transverse sinus stenosis, though without a significant pressure gradient. Results The patient declined surgical ventriculoperitoneal shunting and was approved for compassionate use of eShunt by regulatory and bioethics committees.He underwent successful endovascular transdural deployment of the eShunt, which he tolerated well and was discharged at 48 hours post‐procedure with rapid symptomatic headache relief. Upon 30‐day follow‐up repeat brain MRI showed improvement of the prominent subarachnoid space around both optic nerves and sustained improvement of his pre‐procedural headache and diplopia.A repeat lumbar puncture revealed a lowed opening pressure of 20 cmH2O and MRI cisternography confirmed maintained patency of the biomimetic valve with accumulation of Gadolinium‐enhanced CSF drainage through the eShunt into the jugular bulb. Conclusions The current report describes the first‐in‐human use of the eShunt device for treatment of IIH resulting in sustained symptomatic relief along with decrease in CSF pressure and pre‐laminar optic nerve edema. The current results, which require confirmation in a larger cohort with longer follow‐up, are encouraging and suggest a possible role for minimally invasive endovascular transdural eshunt placement in the management of IIH.

Current understanding of role of venous sinus stenosis in management of idiopathic intracranial hypertension.

Current understanding of role of venous sinus stenosis in management of idiopathic intracranial hypertension.

Improving pediatric idiopathic intracranial hypertension care: a retrospective cohort study

To describe the clinical course and prognosis of pediatric idiopathic intracranial hypertension (IIH) and examine the preferred management setting.IIH is characterized by increased intracranial pressure and is often associated with headaches and visual complaints. IIH is a preventable cause of vision loss in children. Hence, a rapid diagnosis followed by prompt treatment and follow-up is essential. However, standardization of the management of IIH in the pediatric population is not well established. Computerized medical charts of all 82 pediatric (< 18 years) patients diagnosed with IIH between 2007 and 2018 in the metropolitan area of Jerusalem were reviewed. Comparison was made between children followed in a multidisciplinary clinic in tertiary centers and those followed elsewhere. Detailed demographic and clinical data, as well as data regarding the follow-up setting and clinical course of the disease, were collected and analyzed. Recurrent IIH-related hospital returns were selected as a measurable marker for the uncontrolled disease. Recurrent IIH-related hospital return rate was significantly lower and occurred later among children followed by multidisciplinary teams compared to individual experts. Follow-up in multidisciplinary clinics improve the quality of life, and financial burden and may prevent permanent visual impairment in children with IIH.

228 Idiopathic Intracranial Hypertension and Pregnancy: A Comprehensive Review of Management

Abstract Aim To investigate idiopathic intracranial hypertension (IIH) and its management during pregnancy. Method PubMed and Embase databases were searched for studies on pregnancy and IIH, with the most recent search in July 2021. Mesh terms used were “idiopathic intracranial hypertension” or “pseudotumor cerebri” AND “pregnancy”. Papers were limited to studies with human subjects and written in the English language. There were no restrictions in study design or date of publication. The reference lists of all retrieved articles were reviewed for further identification of potentially relevant publications. Results Management of IIH during pregnancy is similar to those who are non-pregnant. It is recommended that pregnant women with IIH are treated in health care settings that have access to multi-specialty input. Management depends on disease severity and includes conservative management, the use of medication such as steroids and diuretics, lumbar punctures, and surgical management. An indication for surgical management includes worsening visual deterioration despite initial management with medication or serial lumbar punctures. Surgical management includes cerebral spinal fluid diversion procedures and optic nerve sheath fenestration. These operations can be performed in all three trimesters of pregnancy. IIH in pregnancy does not increase morbidity or mortality in the newborn and method of delivery should be based on obstetrical indication. Conclusion This review highlights the role of multi-disciplinary approach in the management of IIH during pregnancy.

Surgical Management of Idiopathic Intracranial Hypertension in Pregnancy.

Surgical Management of Idiopathic Intracranial Hypertension in Pregnancy.

Idiopathic intracranial hypertension imaging approaches and the implications in patient management.

Idiopathic intracranial hypertension (IIH) represents a clinical disease entity without a clear etiology, that if left untreated, can result in severe outcomes, including permanent vision loss. For this reason, early diagnosis and treatment is necessary. Historically, the role of cross-sectional imaging has been to rule out secondary or emergent causes of increased intracranial pressure, including tumor, infection, hydrocephalus, or venous thrombosis. MRI and MRV, however, can serve as valuable imaging tools to not only rule out causes for secondary intracranial hypertension but can also detect indirect signs of IIH resultant from increased intracranial pressure, and demonstrate potentially treatable sinus venous stenosis. Digital subtraction venographic imaging also plays a central role in both diagnosis and treatment, providing enhanced anatomic delineation and temporal flow evaluation, quantitative assessment of the pressure gradient across a venous stenosis, treatment guidance, and immediate opportunity for endovascular therapy. In this review, we discuss the multiple modalities for imaging IIH, their limitations, and their contributions to the management of IIH.

Update on Idiopathic Intracranial Hypertension Management.

Idiopathic Intracranial Hypertension (IIH) is a secondary headache with a steadily growing incidence. Currently, there is little evidence to guide the treatment of IIH. To review the pathophysiology of IIH, with focus on the role of obesity as a risk factor, and the implications for new therapeutic perspectives. in this narrative review, we summarized the current knowledge on treatment options highlighting available evidence for managing intracranial hypertension, obesity, and headache. Clinical Presentation: headache is the most common symptom and a significant cause of quality-of-life impairment. Visual loss is common in the diagnosis. Pathophysiology: there is no unified theory able to explain all symptoms and the evolution of the disease. There is growing data pointing to metabolic changes and obesity with a central role in IIH pathophysiology. Treatment: most published data on IIH treatment is related to pressure control and protection from visual loss. Acetazolamide and cerebrospinal fluid diversion are the best options available. Optic nerve sheath fenestration might be useful to temporally control the pressure over the optic nerve and thus protect from visual deterioration. Recently, venous sinus stenting has proven to be a safe option in selected cases. Finally, bariatric surgery has proven to effectively control elevated intracranial pressure. IIH is a potential cause of high disability. Early recognition is important, and treatment should be tailored to the needs of each case. There is a lack of research on headache management, which might persist after ICP control.

Venous sinus stenting after repair of skull base spontaneous cerebrospinal fluid leaks: a single-center retrospective cohort series with case-control analysis

Managementof idiopathic intracranial hypertension (IIH) is recommended after surgical repair of spontaneous cerebrospinal fluid leaks (sCSF-leaks) of the skull base for prevention of recurrence.Purpose: To assess the feasibility of venous sinus stenting, a treatment commonly used for the treatment of IIH associated with intracranial venous sinus stenosis (VSS), after sCSF-leaks closure. Asingle-center cohort series of consecutive patients who underwent sCSF-leak closure was retrospectively analyzed. Stenting was considered either for leak recurrence or in prophylactic manner after repair in patients with VSS as confirmed by cerebral venous imaging. Leak recurrence, need for new repair or adjunctive treatment of IIH, meningitis, and stenting complications were determined at the last follow-up. Cases who had prophylactic stenting were compared to historical controls before stenting option. Twenty-twopatients had intracranial venous stenting after sCSF-leak closure. Their median age was 58 years (Q1=45; Q3=68), BMI=31 kg.m-2 (Q1=27; Q3=36), and female rate=85%. The overall rate of successful repair after stenting was 95% (95% CI = 87-100%) at a median follow-up of 2.4 years (Q1=1.2; Q3=3.3). Adjunctive treatment for IIH was needed in 4 patients (4/22, 18%) including 2 patients without leak recurrence. No meningitis, permanent morbidity or mortality was observed after stenting. Compared to 18 controls, cases had significantly less recurrence (P = 0.03), and a trend for less adjunctive treatment for IIH (P = 0.06). Our study suggests that stenting might be a valid option for prevention of sCSF-leak recurrences after repair in patients with intracranial venous sinus stenosis. Déclaration de liens d'intérêts Les auteurs n'ont pas transmis leur déclaration de liens d'intérêts.

Pseudo tumor cerebri in children: Diagnosis and management approach

Idiopathic intracranial hypertension (IIH) is a rare neurological disorder in children. It is characterized by raised intracranial pressure in the presence of normal cerebrospinal fluid chemistry, normal neuroimaging, and no localizing signs on neurological exam (with the exception of cranial nerve VI palsy). We review clinical presentation, diagnosis and management of IIH in children.

The Pre-Lumbar puncture Intracranial Hypertension Scale (PLIHS): A practical scale to identify subjects with normal cerebrospinal fluid pressure in the management of idiopathic intracranial hypertension

BackgroundIdiopathic Intracranial Hypertension (IIH) diagnosis requires lumbar puncture to measure cerebrospinal fluid (CSF) pressure. The Pre-Lumbar puncture Intracranial Hypertension Scale (PLIHS) is aimed to detect cases that will show raised or normal CSF opening pressure. MethodsRetrospective analysis of records of patients who underwent lumbar puncture for suspect IIH. The target was CSF opening pressure ≥ 250 mmH2O, whereas a set of known neurological, neuro-ophthalmological and neuro-radiological parameters, plus obesity, were used as predictors in a logistic regression model. The PLIHS was based on significant predictors and a cut-off was validated using chi-squared test around CSF opening pressure ≥ 250 and < 200 mmH2O. ResultsRecords of 162 patients were included: CSF opening pressure was <200 mmH2O in 40 and ≥ 250 mmH2O in 95 patients; 85 fulfilled IIH diagnosis. PLIHS is based on Frisén grade 2 or higher papilledema, tinnitus, empty sella, perioptic subarachnoid space distension, and obesity. Score range is 0–7: correlation with CSF opening pressure is 0.508 (p < .001), and PLIHS score is different between subjects not diagnosed with IIH, and those diagnosed with IIH both with and without papilledema (p < .001). PLIHS score ≤ 2 identifies cerebrospinal fluid pressure < 200 mmH2O; PLIHS score ≥ 3 identifies CSF opening pressure ≥ 250 mmH2O, IIH diagnosis, visual acuity ≤0.7, and optic nerve atrophy. ConclusionsThe PLIHS, can be used to identify patients who will particularly need LP, thus helping with the organization of the diagnostic work-up by optimising healthcare resources and potentially limit the likelihood to incur in LP-related adverse events.