-
Operative Neurosurgery (Hagerstown, Md.) Dec 2023Venous sinus stenosis is believed to play a role in the pathogenesis of idiopathic intracranial hypertension (IIH). Venous stenting has emerged as a promising treatment...
BACKGROUND AND IMPORTANCE
Venous sinus stenosis is believed to play a role in the pathogenesis of idiopathic intracranial hypertension (IIH). Venous stenting has emerged as a promising treatment option for patients with IIH because of venous sinus stenosis refractory to medical management or unsuitable for shunt placement. In this technical note, we present a case of IIH with the highest recorded pressure gradient to date.
CLINICAL PRESENTATION
This technical note presents the successful use of intracranial venous stenting in a patient with IIH because of severe venous sinus stenosis, leading to significant improvement in vision and reduction in intracranial pressure. A meticulous review of the literature revealed that our patient exhibited the highest recorded pressure gradient (70 mm Hg). This remarkable finding underscores the potential effectiveness of venous stenting as a viable treatment approach. The procedure involved the placement of a Zilver stent (Cook Medical) and balloon angioplasty after stenting of the right transverse sinus stenosis, resulting in a substantial decrease in pressure gradient. Following the procedure, another venous manometry showed no more gradient with a uniform pressure in the whole venous system at 18 mm Hg.
CONCLUSION
To our knowledge, this case presents the highest pressure gradient reported in the literature and contributes to the growing evidence supporting venous stenting in patients with IIH and venous sinus stenosis.
Topics: Humans; Pseudotumor Cerebri; Constriction, Pathologic; Cranial Sinuses; Intracranial Pressure; Stents
PubMed: 37589472
DOI: 10.1227/ons.0000000000000858 -
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 -
Indian Journal of Endocrinology and... 2023The data on the characteristics of patients with idiopathic intracranial hypertension (IIH) following levothyroxine (LT4) replacement are limited. Here, we report a case... (Review)
Review
The data on the characteristics of patients with idiopathic intracranial hypertension (IIH) following levothyroxine (LT4) replacement are limited. Here, we report a case and systematically review published cases of idiopathic intracranial hypertension (IIH) following levothyroxine (LT4) replacement. The systematic review was performed as per the PRISMA guidelines. Our patient is a 46-year-old lady with hypothyroidism (thyrotropin: 319 mIU/L, free thyroxine: 0.04 ng/dl), treated with 100 μg.d of LT4 and presented a month later with headache, visual diminution, bilateral lateral rectus palsies, and papilledema. Cerebrospinal fluid (CSF) pressure was 32 cmH2O. Drainage of CSF, oral acetazolamide, and modification of LT4 dose resulted in prompt symptomatic improvement and complete reversal of IIH. In the systematic review (n = 21), the median age of patients (7 males) was 13 (IQR: 8.8- 26.5) years. The median duration of hypothyroid symptoms was 4 (n = 10, IQR: 0.44-6.25) years whereas that from initiation of LT4 replacement to the diagnosis of IIH was 2 (n = 20, IQR: 1.17-4) months. Initial median serum thyrotropin and thyroxine were 100 (n = 14, IQR: 72.5-421.6) mIU/L, and 1.13 (n = 12, IQR: 1.0-2.45) μg/dl which changed to 2.2 (n = 7; IQR: 0.23-3.40) mIU/L and 8.90 μg/dl (n = 8, IQR: 6.43-14.85 μg/dl), respectively at diagnosis of IIH after LT4 treatment with median daily LT4 doses of 0.89 (n = 8, IQR: 0.60 - 1.17) times the maximum recommended dose for age. To conclude, we report an adult woman with IIH following LT4 replacement for primary hypothyroidism, a rare entity. Pediatric age, prolonged symptom duration, and use of higher LT4 replacement dose may be associated with IIH following LT4 replacement.
PubMed: 37215264
DOI: 10.4103/ijem.ijem_439_22 -
Clinical Neurology and Neurosurgery Jun 2023The neurosurgical management of idiopathic intracranial hypertension (IIH) remains controversial. Although shunting and newer endovascular stenting techniques are part... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The 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.
OBJECTIVE
We present a detailed cadaveric dissection that simulates the endoscopic endonasal optic nerve sheath decompression (EONSD) technique along with the literature review.
METHODS
EONSD was performed in four freshly injected cadaveric specimens. Additionally, a systematic review from different electronic databases has been done.
RESULTS
Bilateral 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.
CONCLUSION
EONSD 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.
Topics: Adult; Humans; Female; Male; Pseudotumor Cerebri; Optic Nerve; Papilledema; Decompression, Surgical; Cadaver; Intracranial Hypertension
PubMed: 37146367
DOI: 10.1016/j.clineuro.2023.107750 -
Cureus Aug 2022Pseudotumor cerebri syndrome (PTCS)/idiopathic intracranial hypertension (IIH) is a clinical presentation appertaining to signs/symptoms of raised intracranial pressure,... (Review)
Review
Pseudotumor cerebri syndrome (PTCS)/idiopathic intracranial hypertension (IIH) is a clinical presentation appertaining to signs/symptoms of raised intracranial pressure, like headache and papilledema. It is an uncommon but clinically significant cause of morbidity such as permanent vision loss. It is crucial to understand if idiopathic intracranial hypertension (IIH) is on the rise in adolescents, it is probably due to the rising prevalence of obesity worldwide. Our study aimed to find an association between obesity and IIH in adolescents. We utilized Preferred Reporting Items for Systematic Review and Meta-Analysis 2020 (PRISMA) guidelines to run this systematic review. Many publications related to the topic in the discussion were scrutinized through a comprehensive database search. We filtered them down to a final count of 10 articles after utilizing our inclusion/exclusion criteria and assessing the quality of work. In these final papers, we identified several possibilities to explain the link between obesity and IIH in adolescents. Overweight and obese adolescents were found to have a significantly increased risk of IIH development, with a more severe clinical picture seen in morbidly obese female patients.
PubMed: 36127965
DOI: 10.7759/cureus.28071 -
Neurosurgery Oct 2022Cerebrospinal fluid (CSF) shunting is widely used in refractory idiopathic intracranial hypotension (IIH). Although multiple reviews have assessed its efficacy compared... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Cerebrospinal fluid (CSF) shunting is widely used in refractory idiopathic intracranial hypotension (IIH). Although multiple reviews have assessed its efficacy compared with other surgical treatments, there is no detailed analysis that evaluates the clinical outcomes after CSF shunting.
OBJECTIVE
To conduct a meta-analysis of the clinical impact of CSF shunting for refractory IIH and use this in conjunction with existing information on other treatment modalities to develop a modern management protocol.
METHODS
PubMed and Embase were systematically searched for studies describing CSF shunting for medically refractory IIH. Relevant information including study characteristics, patient demographics, clinical outcomes, periprocedural complications, and long-term outcomes were subjected to meta-analysis.
RESULTS
Fifteen studies published between 1988 and 2019 met our inclusion and exclusion criteria, providing 372 patients for analysis. The mean age was 31.2 years (range 0.5-71) with 83.6% being female. The average follow-up was 33.9 months (range 0-278 months). The overall rate of improvement in headache, papilledema, and visual impairment was 91% (95% CI 84%-97%), 96% (95% CI 85%-100%), and 85% (95% CI 72%-95%), respectively. Of 372 patients, 155 had 436 revisions; the overall revision rate was 42% (95% CI 26%-59%). There was no significant correlation between average follow-up duration and revision rates in studies ( P = .627). Periprocedural low-pressure headaches were noted in 74 patients (20%; 95% CI 11%-32%).
CONCLUSION
CSF shunting for IIH is associated with significant improvement in clinical symptoms. Shunting rarely causes periprocedural complications except overdrainage-related low-pressure headache. However, CSF shunting has a relatively high revision rate.
Topics: Adolescent; Adult; Aged; Cerebrospinal Fluid Shunts; Child; Child, Preschool; Clinical Protocols; Female; Headache; Humans; Infant; Intracranial Hypertension; Male; Middle Aged; Neurosurgical Procedures; Papilledema; Pseudotumor Cerebri; Young Adult
PubMed: 35916578
DOI: 10.1227/neu.0000000000002086 -
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 -
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 -
Cephalalgia : An International Journal... May 2022Idiopathic intracranial hypertension is characterized by increased intracranial pressure without any pathological findings on neuroimaging, except for signs of high...
BACKGROUND
Idiopathic intracranial hypertension is characterized by increased intracranial pressure without any pathological findings on neuroimaging, except for signs of high intracranial pressure. Before diagnosing idiopathic intracranial hypertension secondary causes of increased intracranial pressure should be excluded.
OBJECTIVE
to characterize the phenotype of patients with secondary intracranial hypertension and to identify possible risk factors for secondary intracranial hypertension.
METHODS
We have systematically searched the PubMed database. The publications were analyzed according to the patient phenotype, age, gender, comorbidities, body mass index/weight status, and additional medication. The results are summarized in four categories: medication, infection, hormonal induced intracranial hypertension and miscellaneous groups of diseases related to sIH.
RESULTS
We identified 105 eligible papers which included 272 cases. There were 49.6% pediatric cases. Among the adult group,70.9% were women. A total of 40.4% of all cases were obese or overweight, 27% among adults and 13.4% among pediatric cases. Increased BMI and recent weight gain, anemia, renal diseases and hypertension were the most frequent comorbidities related to sIH.
CONCLUSION
Among sIH patients, 40.4% were obese or overweight; two thirds were women. We recommend that even patients with a typical IIH phenotype should be screened for secondary causes.
Topics: Body Mass Index; Child; Female; Humans; Hypertension; Intracranial Hypertension; Male; Obesity; Overweight; Pseudotumor Cerebri
PubMed: 34822742
DOI: 10.1177/03331024211056580 -
Journal of Neuro-ophthalmology : the... Mar 2022Idiopathic intracranial hypertension (IIH) is a condition typically affecting young, obese women. Although anemia is recognized as a risk factor of IIH from case... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Idiopathic intracranial hypertension (IIH) is a condition typically affecting young, obese women. Although anemia is recognized as a risk factor of IIH from case reports, their relationship remains controversial as several comparative studies showed no significant association. This study aimed to examine the relationship between anemia and IIH.
METHODS
MEDLINE, Embase, Cochrane Library, and grey literature were searched to September 2020. Primary studies on patients with diagnoses of anemia of any kind and IIH were included. Primary outcomes included the total number of cases of anemia and IIH. A meta-analysis on the prevalence of anemia in IIH compared with control patients was conducted. Grading of Recommendations, Assessment, Development and Evaluations (GRADE) was used to rate the quality of evidence.
RESULTS
Overall, 62 cases and 5 observational or case-control studies were included. Pooled incidence of anemia in patients with IIH was 195/1,073 (18.2%). Patients with IIH (n = 774) had a significantly higher prevalence of anemia compared with controls (n = 230,981) (RR 1.44 [95% confidence interval 1.08-1.92]). Patients were 67.7% females and had a mean age of 22.4 years. The mean opening pressure was 37.9 cmH2O. Anemia was most commonly caused by iron deficiency (51.6%) and aplastic anemia (19.4%). Most patients (59.7%) showed improvement or resolution with anemia treatment only without intracranial pressure-lowering therapy. Evidence was limited because of high risk of reporting bias from the large number of case reports and case-control studies.
CONCLUSIONS
Anemia is significantly more common in IIH compared with control patients, and case reports suggest a direct relationship. Complete blood counts should be considered in all patients with papilledema, particularly in atypical presentations (male, nonobese, nonperipapillary retinal hemorrhages, prominent risk factor for anemia) or in treatment-refractory IIH.
Topics: Adult; Anemia; Case-Control Studies; Female; Humans; Intracranial Hypertension; Intracranial Pressure; Male; Papilledema; Pseudotumor Cerebri; Young Adult
PubMed: 34812762
DOI: 10.1097/WNO.0000000000001408