Pediatric Idiopathic Intracranial Hypertension: A not so Rare and Benign Condition

ABSTRACT Objective Pediatric idiopathic intracranial hypertension is an underdiagnosed entity with catastrophic presentations. High index of suspicion with early diagnosis and prompt treatment is the key to successful management. Trans-nasal trans-sphenoid Endoscopic Optic Nerve Fenestration is an effective surgical modality for the reversal of vision loss in pediatric idiopathic intracranial hypertension (IIH). Materials and methods This is a single center observational prospective case series. Five diagnosed pediatric patients of IIH satisfying the modified Dandy criteria and reported to the out-patient services of otolaryngology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India in the year 2012 were included in this study. All children underwent thorough clinical examination, complete Neuro-ophthalmological work-up including Visual acuity (V/A), Visual field charting (V/F), Fundus venogram and Radiological work-up with MRI for special optic nerve sections in sagittal reconstruction. Cerebro-Spinal Fluid pressure (CSF) measured preoperatively for all children. Standard endoscopic optic nerve Sheath Fenestration was performed on all children. visual improvement was assessed by comparing preoperative ophthalmological findings. Results Improvement in vision was taken as a positive outcome. Vision improved in all except one child, who had pre-existing optic nerve atrophy. Conclusion Endoscopic optic nerve fenestration is an effective minimally invasive procedure to revert visual loss in pediatric idiopathic intracranial hypertension. How to cite this article Gupta AK, Gupta K, Modi M, Gupta A. Pediatric Idiopathic Intracranial Hypertension: A not so Rare and Benign Condition. Clin Rhinol An Int J 2014;7(1):5-9.

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Intracranial Venous Sinus Stenting in Idiopathic Intracranial Hypertension: A Case Report and Review of the Literature

Brain Sciences ◽

10.3390/brainsci11030382 ◽

2021 ◽

Vol 11 (3) ◽

pp. 382

Author(s):

Dinesh Ramanathan ◽

Zachary D. Travis ◽

Emmanuel Omosor ◽

Taylor Wilson ◽

Nikhil Sahasrabudhe ◽

...

Keyword(s):

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Vision Loss ◽

Transverse Sinus ◽

Venous Sinus ◽

Subdural Hygroma ◽

Presenting Symptoms ◽

Minimal Improvement ◽

Sinus Stenosis ◽

Work Up

We describe a case of severe headaches, double vision, and progressive vision loss secondary to a ruptured intracranial cyst (IAC) in a 31-year-old woman with no relevant past medical history. The case is peculiar because drainage of the subdural hygroma led to a minimal improvement in vision with persistent elevated intracranial pressure (ICP). Further exploration revealed transverse sinus stenosis necessitating stenting. Evaluation post-stenting showed marked reduction of ICP and improvement in symptoms. This report underscores the importance of comprehensive work-up and suspicion of multiple underlying etiologies that may be crucial to complete resolution of presenting symptoms in some cases. We provide an overview of the clinical indications and evidence for venous sinus stenting in treating idiopathic intracranial hypertension (IIH).

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Optic nerve sonographic examination to predict raised intracranial pressure in idiopathic intracranial hypertension: The cut-off points

The Neuroradiology Journal ◽

10.1177/1971400918789385 ◽

2018 ◽

Vol 31 (5) ◽

pp. 490-495 ◽

Cited By ~ 8

Author(s):

Nirmeen A Kishk ◽

Asmaa M Ebraheim ◽

Amal S Ashour ◽

Nashwa M Badr ◽

Mohamed A Eshra

Keyword(s):

Optic Nerve ◽

Intracranial Pressure ◽

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Fluid Pressure ◽

Area Under The Curve ◽

Raised Intracranial Pressure ◽

Sonographic Examination ◽

Linear Probe ◽

Significant Difference

Purpose Monitoring of raised intracranial pressure (ICP) in patients with idiopathic intracranial hypertension (IIH) is required to prevent secondary optic nerve damage. Sonographic measurement of the optic nerve sheath diameter (ONSD) is a noninvasive method to evaluate intracranial hypertension. Different ONSD cut-off values have been reported probably due to ethnic variations. Our aim was to determine optic nerve sonographic examination cut-off points to predict raised ICP in IIH patients. Methods This case-control study was conducted on 99 IIH post-pubertal female patients (both probable and definite) and 35 age- and sex-matched healthy volunteers. Sonographic ONSD and optic nerve diameter (OND) were obtained 3 mm behind the posterior edge of the globe in a horizontal plane via a 7–13 MHz linear probe. Lumbar puncture was then carried out on the patients. Results The opening cerebrospinal fluid pressure documented in the patient group was 279.64 ± 65.97 mm H2O. A statistically significant difference was found between IIH patients and controls regarding ONSD. The best ONSD cut-off value indicating raised ICP was 6.05 mm with an area under the curve of 0.850 (95% confidence interval 0.805 to 0.894, 73.2% sensitivity and 91.4% specificity). Regarding OND/ONSD ratio, there was an insignificant difference between both groups. Conclusion Sonographic ONSD but not OND/ONSD ratio could offer a bedside adjunct or alternative indicator of elevated ICP in IIH patients. Ethnic differences, however, should be noted when using this parameter.

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Anterior Lamina Cribrosa Surface Position in Idiopathic Intracranial Hypertension and Glaucoma

European Journal of Ophthalmology ◽

10.5301/ejo.5000806 ◽

2016 ◽

Vol 27 (1) ◽

pp. 55-61 ◽

Cited By ~ 13

Author(s):

Jenni M. Villarruel ◽

Xiao Q. Li ◽

Daniella Bach-Holm ◽

Steffen Hamann

Keyword(s):

Optic Nerve ◽

Intracranial Hypertension ◽

Optic Nerve Head ◽

Idiopathic Intracranial Hypertension ◽

Open Angle Glaucoma ◽

Fluid Pressure ◽

Lamina Cribrosa ◽

Enhanced Depth Imaging ◽

Mean Deviation ◽

Control Subjects

Purpose To compare the anterior lamina cribrosa (LC) surface position in patients with idiopathic intracranial hypertension (IIH), primary open-angle glaucoma (high-tension glaucoma [HTG] and normal-tension glaucoma [NTG]), and healthy controls using enhanced depth imaging spectral-domain optical coherence tomography (EDI-OCT). Methods This was a retrospective, cross-sectional observational study of 11 eyes with IIH, 20 eyes with HTG, 20 eyes with NTG, and 37 control eyes. Serial horizontal B-scans of the optic nerve head were obtained using EDI-OCT. The LC depth, defined as the distance from the Bruch membrane opening plane to the anterior LC surface, was manually measured on selected B-scans covering the central three-quarters of the optic nerve head in each eye. Results Mean LC depth in patients with IIH (325.2 ± 92.1 µm) was significantly (p<0.01) decreased compared to control subjects (387.8 ± 53.9 µm). In HTG, the mean LC depth (493.0 ± 115.2 µm) was significantly increased compared to NTG (376.6 ± 27.1 µm, p<0.05) and control subjects (332.7 ± 53.1 µm, p<0.001). The trans-LC pressure difference (TLPD) (intraocular pressure − cerebrospinal fluid pressure) was positively correlated with the LC depth (r = 0.96, p<0.001) and inversely correlated with visual field perimetric mean deviation in patients with IIH (r = -0.88, p<0.001). Conclusions The LC is positioned anteriorly in patients with IIH and posteriorly in glaucomatous eyes compared to normal controls. The positional changes of the LC could be a result of the TLPD between the intraocular and intracranial pressure compartments.

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Abnormal Intracranial Pulse Pressure Amplitude Despite Normalized Static Intracranial Pressure in Idiopathic Intracranial Hypertension Refractory to Conservative Medical Therapy

Life ◽

10.3390/life11060537 ◽

2021 ◽

Vol 11 (6) ◽

pp. 537

Author(s):

Per Kristian Eide

Keyword(s):

Intracranial Pressure ◽

Intracranial Hypertension ◽

Medical Therapy ◽

Idiopathic Intracranial Hypertension ◽

Fluid Pressure ◽

Pressure Amplitude ◽

Lumbar Cerebrospinal Fluid ◽

Work Up ◽

Underlying Pathophysiology ◽

Symptoms And Signs

Idiopathic intracranial hypertension (IIH) incorporates symptoms and signs of increased intracranial pressure (ICP) and is diagnosed by increased lumbar cerebrospinal fluid pressure. However, our knowledge about the characteristics of ICP abnormality, e.g., changes in pulsatile versus static ICP, remains scarce. This study questioned how overnight pulsatile ICP (mean ICP wave amplitude, MWA) associates with static ICP (mean ICP) in IIH patients who were refractory to conservative medical treatment. The material included 80 consecutive IIH patients undergoing ICP monitoring prior to shunt, as part of work-up for failed conservative medical therapy. In this group, the overnight mean ICP was normalized in 52/80 patients, but with abnormal overnight MWA in 45 of the 52 patients. Even though there was a positive correlation between MWA and mean ICP at group level and within individual ICP recordings, the levels of MWA were abnormal in a high proportion of patients despite normalized mean ICP. Taken together, the present results disclosed lasting abnormal pulsatile ICP despite normalized static ICP in IIH patients refractory to conservative medical therapy, which may reflect the underlying pathophysiology. It is tentatively suggested that abnormal pulsatile ICP in IIH may reflect alterations at the glia–neurovascular interface, resulting in impaired astrocytic pulsation absorber mechanisms.

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Sensitivity and specificity of neuroimaging signs in patients with idiopathic intracranial hypertension

The Neuroradiology Journal ◽

10.1177/19714009211000623 ◽

2021 ◽

pp. 197140092110006

Author(s):

Nandita Prabhat ◽

Shivani Chandel ◽

Dr Aastha Takkar ◽

Chirag Ahuja ◽

Ramandeep Singh ◽

...

Keyword(s):

Optic Nerve ◽

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Vision Loss ◽

Visual Outcome ◽

Empty Sella ◽

Primary Role ◽

Clinical Scenarios ◽

Treatment Naïve ◽

Magnetic Resonance Imaging Mri

Background The primary role of neuroimaging in idiopathic intracranial hypertension (IIH) is to exclude secondary causes of raised intracranial pressure. Recently, a few imaging markers have been described which may suggest diagnosis of IIH in atypical cases. We carried out this study to assess the prevalence and accuracy of these neuroimaging signs in predicting the diagnosis of IIH. Methods Eighty treatment-naive patients with IIH and 30 controls were recruited as per a predefined criterion. Magnetic resonance imaging (MRI) brain with detailed sella imaging was done in all patients. Results The most common abnormality noted was optic nerve tortuosity in 82.5% of patients, followed by posterior scleral flattening in 80%, perioptic subarachnoid space (SAS) dilatation in 73.8% and partial empty sella in 68.8% of patients. The presence of optic nerve tortuosity was the most sensitive sign on neuroimaging, though the highest specificity was seen for posterior scleral flattening and perioptic SAS dilatation. The presence of more than three neuroimaging features correlated with severity of vision loss. Conclusion In suggestive clinical scenarios, posterior scleral flattening, perioptic SAS dilatation and optic nerve tortuosity are highly sensitive and specific signs in IIH. This study also highlights the utility of MRI as a valuable tool for prognosis of visual outcome in patients with IIH.

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Noninvasive Monitoring and Neurointerventional Management of Idiopathic Intracranial Hypertension

Journal of Clinical Interventional Radiology ISVIR ◽

10.1055/s-0040-1708578 ◽

2020 ◽

Vol 4 (01) ◽

pp. 42-50

Author(s):

Matthew Lang ◽

Sunu Mathew ◽

Alon Harris ◽

Juan Tejada ◽

Jerry M. Kovoor

Keyword(s):

Optic Nerve ◽

Intracranial Hypertension ◽

Surgical Management ◽

Idiopathic Intracranial Hypertension ◽

Vision Loss ◽

Management Strategies ◽

Venous Sinus ◽

Optic Nerve Sheath ◽

Noninvasive Methods ◽

Nerve Sheath

AbstractIdiopathic intracranial hypertension (IIH) is characterized by isolated rise in intracranial pressure (ICP) leading to chronic, debilitating headaches, tinnitus, and vision loss. Conventional diagnostics and monitoring primarily require the use of invasive procedure like lumbar puncture to measure ICP, while traditional management strategies involve weight reduction and medical treatment with acetazolamide. In an effort to reduce the need for invasive procedures, noninvasive methods of ICP monitoring such as optic nerve sheath diameter measurements and two-depth transcranial Doppler ultrasonography have been developed. In cases of refractory and fulminant IIH, surgical management strategies such as optic nerve sheath fenestration (ONSF), ventriculoperitoneal (VP) and lumboperitoneal (LP) shunting, and transverse venous sinus stenting are used to relieve symptoms via ICP reduction. While ONSF and VP/LP shunting exhibit utility primarily for the treatment of vision loss and headache, respectively, venous sinus stenting may prove to be an effective option in the management of all symptoms of IIH. Most importantly, consideration of the patient’s individual symptoms and values should be taken into account when choosing the optimal surgical management strategy for patients with IIH.

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Idiopathic Intracranial Hypertension – Pathophysiology Based on Case Series

Acta Facultatis Medicae Naissensis ◽

10.1515/afmnai-2016-0021 ◽

2016 ◽

Vol 33 (3) ◽

pp. 199-209

Author(s):

Srdjan Ljubisavljević ◽

Jasna Zidverc Trajković

Keyword(s):

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Fluid Pressure ◽

Case Series ◽

Therapeutic Interventions ◽

Pathological State ◽

Obese Women ◽

Sexual Hormones ◽

Clinical Presentations ◽

Number Of Patients

Summary According to the definition, idiopathic intracranial hypertension (IIH) is a pathological state characterized by an increase in intracranial pressure; however, there are no obvious intracranial pathological processes. The pathophysiology of this disorder is not clear, although there are many reports related to it. We present an overview of possible etiopathogenetic mechanisms, clinical presentations and therapeutic interventions from a series of patients hospitalized with the clinical picture and final diagnosis of idiopathic intracranial hypertension (IIH). All data were collected from the moment of IIH diagnosis as well as three months later. The obtained data showed that IIH is a disease that primarily affects obese women in early and midlife. The positive correlation between values of cerebrospinal fluid pressure and body mass index was observed. The disorders of sexual hormones were identified as a possible etiology for IIH female patients. Headache, papilloedema, decreased visual acuity, vertigo and cranial nerve palsy were identified as the most prevalent IIH clinical presentations. The existence of stenosis and hypoplasia of the sigmoid and transverse sinus were confirmed only in one third of IIH patients. Pharmacotherapy combined with weight loss was efficacious in a large number of patients. In this series, there were no short-term consequences of IIH. The results suggest the importance of early and accurate looking for IIH in obese early and midlife women with any hormonal imbalances having a variety of neurological expression, mostly presented as headaches and visual disturbances. Early detection of IIH might influence the timely treatment and prevent far-reaching and severe clinical consequences.

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Optic nerve sheath fenestration for vision preservation in idiopathic intracranial hypertension

Neurosurgical FOCUS ◽

10.3171/foc-07/11/e7 ◽

2007 ◽

Vol 23 (5) ◽

pp. E7 ◽

Cited By ~ 26

Author(s):

Madhu R. Agarwal ◽

Ji Hye Yoo

Keyword(s):

Optic Nerve ◽

Intracranial Hypertension ◽

Visual Function ◽

Idiopathic Intracranial Hypertension ◽

Vision Loss ◽

Pseudotumor Cerebri ◽

Optic Nerve Sheath ◽

Fluid Composition ◽

Optic Nerve Sheath Fenestration ◽

Nerve Sheath

✓Idiopathic intracranial hypertension, also known as pseudotumor cerebri, is a condition of increased intracranial pressure without the presence of mass lesions and with normal cerebrospinal fluid composition. Patients may experience papilledema and vision loss. Optic nerve sheath fenestration (ONSF) is one method of stabilizing visual function and decreasing optic nerve edema. The authors report on 10 patients who underwent bilateral ONSF and in whom visual function was stable or improved postoperatively. The results obtained in these cases suggest that ONSF plays a role in visual preservation in idiopathic intracranial hypertension patients in the acute setting.

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