scholarly journals Acute presentation of idiopathic intracranial hypertension with severe vision deficits

2020 ◽  
Vol 8 ◽  
pp. 2050313X2094557
Author(s):  
Jacqueline K Shaia ◽  
Carrie Elzie
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Vision Loss ◽  
Initial Symptom ◽  
Symptom Presentation ◽  
Rare Presentation ◽  
Acute Presentation ◽  
Severe Vision Loss ◽  
Vision Deficits ◽  
Visual Disturbances

Fulminant idiopathic intracranial hypertension is a rare presentation of idiopathic intracranial hypertension in which visual decline occurs within 4 weeks of initial symptom presentation. Idiopathic intracranial hypertension usually presents with headaches, visual disturbances, and in women who are overweight. We present a case of idiopathic intracranial hypertension in which a female patient presented with rapid, severe vision loss within 8 days of symptom presentation and no other idiopathic intracranial hypertension symptoms. This case highlights a rare presentation of Fulminant idiopathic intracranial hypertension and the need for a quick diagnosis and treatment to preserve vision.

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

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).

External lumbar drainage in progressive pediatric idiopathic intracranial hypertension

2021 ◽  
pp. 1-7
Author(s):  
Gad Dotan ◽  
Natalie Hadar Cohen ◽  
Hanya M. Qureshi ◽  
Mika Shapira Rootman ◽  
Yoram Nevo ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Vision Loss ◽  
Symptom Relief ◽  
Visual Outcome ◽  
Fiber Layer ◽  
Lumbar Drainage ◽  
Lumbar Drain ◽  
Rnfl Thickness ◽  
External Lumbar Drainage

OBJECTIVE Pediatric idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure despite normal cerebrospinal fluid and neuroimaging findings. Initial management is typically medical; however, nearly 10% of children will eventually require surgery for persistent headache and/or vision loss. External lumbar drainage, which is a considerably safer treatment option, has not been adequately analyzed in children with medically refractory IIH. METHODS The authors conducted a single-institution retrospective analysis of children with medically refractory IIH who had undergone external lumbar drain (ELD) placement because of worsening papilledema, reflected as increased retinal nerve fiber layer (RNFL) thickness on optical coherence tomography (OCT) testing. The main outcome measures were effects of external lumbar drainage on papilledema resolution, symptoms, and vision. RESULTS The authors analyzed the medical records of 13 children with IIH (11 girls, mean age 15.0 ± 2.3 years) whose mean CSF opening pressure was 45.5 ± 6.8 cm H2O. In all children, the average global RNFL thickness in both eyes significantly increased at ELD placement (right eye 371.8 ± 150.2 μm, left eye 400.3 ± 96.9 μm) compared with presentation thickness (right eye 301.6 ± 110.40 μm, left eye 350.2 ± 107.7 μm) despite acetazolamide medical therapy (20–30 mg/kg/day), leading to ELD placement after 9.5 ± 6.9 days (range 3–29 days). After ELD insertion, there was headache resolution, gradual and continuous improvement in optic disc thickness, and preservation of good vision. CONCLUSIONS ELD placement in children with medically refractory IIH who demonstrated worsening papilledema with increased RNFL thickening on OCT testing typically results in symptom relief and disc edema resolution with good visual outcome, often preventing the need for additional definitive surgeries that carry greater failure and morbidity risks.

Rapidly progressive vision loss due to fulminant idiopathic intracranial hypertension: a diagnostic and management dilemma

2020 ◽  
Vol 13 (11) ◽  
pp. e236188
Author(s):  
Jagadeesh Sutraye ◽  
Mohan Kannam ◽  
Rajat Kapoor ◽  
Virender Sachdeva
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Vision Loss ◽  
Obese Woman ◽  
Visual Fields ◽  
Csf Analysis ◽  
Contrast Enhanced ◽  
Grade Ii ◽  
The Right ◽  
The Brain

A 44-year-old obese woman presented with decrease in vision in the right eye (RE) for 3 days. She reported a simultaneous onset of holocranial headache that worsened on bending forward. She denied eye pain, pain on eye movements, and other ocular or neurological complaints. On examination, her distance best-corrected visual acuity was counting fingers at 1 m in the RE and 20/20 in the left eye (LE). Colour vision was subnormal in both eyes (BE). There was grade II relative afferent pupillary defect in the RE. Fundus examination showed disc oedema in BE . Visual fields in the LE showed central scotoma extending nasally. A provisional diagnosis of papillitis was considered. However, contrast-enhanced MRI of the brain and orbits showed evidence of elevated intracranial pressure. Cerebrospinal fluid (CSF) opening pressure was 42 cm H2O while rest of the CSF analysis was normal. Diagnosis was revised to fulminant idiopathic intracranial hypertension. Management with medical therapy and urgent thecoperiteoneal shunt improved visual function in BE.

Treatment of idiopathic intracranial hypertension via stereotactic placement of biventriculoperitoneal shunts

2018 ◽  
Vol 130 (1) ◽  
pp. 136-144 ◽  
Author(s):  
Michael Karsy ◽  
Hussam Abou-Al-Shaar ◽  
Christian A. Bowers ◽  
Richard H. Schmidt
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Vision Loss ◽  
Single Case ◽  
Distal Catheter ◽  
Shunt Placement ◽  
Presenting Symptoms ◽  
Small Series ◽  
Shunt Survival

OBJECTIVEIdiopathic intracranial hypertension (IIH), or pseudotumor cerebri, is a complex and difficult-to-manage condition that can lead to permanent vision loss and refractory headaches if untreated. Traditional treatment options, such as unilateral ventriculoperitoneal (VP) or lumboperitoneal (LP) shunt placement, have high complication and failure rates and often require multiple revisions. The use of bilateral proximal catheters has been hypothesized as a method to improve shunt survival. The use of stereotactic technology has improved the accuracy of catheter placement and may improve treatment of IIH, with fewer complications and greater shunt patency time.METHODSThe authors performed a retrospective chart review for all patients with IIH who underwent stereotactic placement of biventriculoperitoneal (BVP) shunt catheters from 2008 to 2016 at their institution. Bilateral proximal catheters were Y-connected to a Strata valve with a single distal catheter. We evaluated clinical, surgical, and ophthalmological variables and outcomes.RESULTSMost patients in this series of 34 patients (mean age 34.4 ± 8.2 years, mean body mass index 38.7 ± 8.3 kg/m2; 91.2% were women) undergoing 41 shunt procedures presented with headache (94.1%) and visual deficits (85.3%). The mean opening pressure was 39.6 ± 9.0 cm H2O. In addition, 50.0% had undergone previous unilateral shunt placement, and 20.6% had undergone prior optic nerve sheath fenestration. After BVP shunt placement, there were no cases of proximal catheter obstruction and only a single case of valve obstruction at 41.9 months, with a mean follow-up of 24.8 ± 20.0 months. Most patients showed improvement in their headache (82.4%), subjective vision (70.6%), and papilledema (61.5% preoperatively vs 20.0% postoperatively, p = 0.02) at follow-up. Additional primary complications included 4 patients with migration of their distal catheters out of the peritoneum (twice in 1 patient), and an infection of the distal catheter after catheter dislodgment. The proximal obstructive shunt complication rate in this series (2.9%) was lower than that with LP (53.5%) or unilateral VP (37.8%) shunts seen in the literature.CONCLUSIONSThis small series suggests that stereotactic placement of BVP shunt catheters appears to improve shunt survival rates and presenting symptoms in patients with IIH. Compared with unilateral VP or LP shunts, the use of BVP shunts may be a more effective and more functionally sustained method for the treatment of IIH.

What is New About Idiopathic Intracranial Hypertension? An Updated Review of Mechanism and Treatment

Cephalalgia ◽  
2006 ◽  
Vol 26 (4) ◽  
pp. 384-399 ◽  
Author(s):  
M Skau ◽  
J Brennum ◽  
F Gjerris ◽  
R Jensen
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Clinical Laboratory ◽  
Treatment Strategies ◽  
Disease Course ◽  
Causal Treatment ◽  
Associated Conditions ◽  
Patients Experience ◽  
Visual Disturbances ◽  
Neuroimaging Techniques

Idiopathic intracranial hypertension (IIH) is the syndrome of raised intracranial pressure without clinical, laboratory or radiological evidence of intracranial pathology. IIH is a relatively rare disease but rapidly increasing incidence is reported due to a global increasing incidence of obesity. Disease course is generally said to be self-limiting within a few months. However, some patients experience a disabling condition of chronic severe headache and visual disturbances for years that limit their capacity to work. Permanent visual defects are serious and not infrequent complications. The pathophysiology of IIH is still not fully understood. Advances in neuroimaging techniques have facilitated the exclusion of associated conditions that may mimic IIH. No causal treatment is yet known for IIH and existing treatment is symptomatic and rarely sufficient. The aim of this review is to provide an updated overview of this potentially disabling disease which may show a future escalating incidence due to obesity. Theories of pathogenesis, diagnostic criteria and treatment strategies are discussed.

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

2014 ◽  
Vol 7 (1) ◽  
pp. 5-9
Author(s):  
Manish Modi ◽  
Karan Gupta
Keyword(s):  
Optic Nerve ◽  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Vision Loss ◽  
Fluid Pressure ◽  
Case Series ◽  
Invasive Procedure ◽  
Positive Outcome ◽  
Benign Condition ◽  
Work Up

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.

scholarly journals Complete ophthalmoplegia: A rare presentation of idiopathic intracranial hypertension

2015 ◽  
Vol 0 (0) ◽  
pp. 0
Author(s):  
IrfanYousuf Wani ◽  
Sawan Verma ◽  
Mushtaq Wani ◽  
Ravouf Asimi ◽  
Saleem Sheikh ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Rare Presentation ◽  
Complete Ophthalmoplegia

scholarly journals The patients’ perspective on the burden of idiopathic intracranial hypertension

2021 ◽  
Author(s):  
Max Witry ◽  
Christine Kindler ◽  
Johannes Weller ◽  
Andreas Linder ◽  
Ullrich Wüllner
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Sleep Disturbances ◽  
Online Survey ◽  
Cross Sectional Study ◽  
Increased Intracranial Pressure ◽  
Opening Pressure ◽  
Cross Sectional ◽  
Patients Perspective ◽  
Visual Disturbances

Abstract Background: Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure without evidence of a tumor or any other underlying cause. Headache and visual disturbances are frequent complaints of IIH patients, but little is known about other symptoms. In this study, we evaluated the patients’ perspective on the burden of IIH. Methods: For this cross-sectional study, we developed an online survey for patients with IIH containing standardized evaluations of headache (HIT-6), sleep (PROMIS Sleep Disturbance Scale) and depression (MDI) in relation to BMI, lumbar puncture opening pressure (LP OP) and treatment. Results: Between December 2019 and February 2020, 306 patients completed the survey. 285 (93%) were female, mean age was 36.6 years (± 10.8), mean BMI 34.2 (± 7.3) and mean LP OP at diagnosis was 37.8 cmH2O (± 9.5). 219 (72%) of the participants were obese (BMI ≥ 30); 251 (82%) reported severe impacting headaches, 140 (46%) were suffering from sleep disturbances and 169 (56%) from depression. Higher MDI scores correlated with higher BMI and increased sleep disturbances. Patients with a normalized LP opening pressure reported less headaches, less sleep disturbances and less depression than those with a constantly elevated opening pressure. Conclusion: In addition to headaches and visual disturbances, sleep disturbances and depression are frequent symptoms in IIH and contribute to the patients’ burden. Structured questionnaires can help to identify IIH patients’ needs and can lead to personalized and better treatment.

Unilateral papilledema in idiopathic intracranial hypertension: A rare entity

2020 ◽  
pp. 112067212096904
Author(s):  
Mousumi Banerjee ◽  
Swati Phuljhele Aalok ◽  
Deepti Vibha
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Pseudotumor Cerebri ◽  
Clinical Syndrome ◽  
Unknown Etiology ◽  
Obese Women ◽  
Mechanical Compression ◽  
Childbearing Age ◽  
Diagnostic Challenge ◽  
Rare Presentation

Idiopathic Intracranial Hypertension (IIH), also known as pseudotumor cerebri is a clinical syndrome of unknown etiology, which typically affects young, obese women of childbearing age and is characterized by increased intracranial pressure and bilateral papilledema. Unilateral and highly asymmetrical papilledema is a rare presentation in IIH occurring in less than 4% of patients with definite IIH and can poses a diagnostic challenge for the treating physician as it usually raises the suspicion of localized ocular pathology. As per current consensus, papilledema results in stasis of axoplasmic transport due to mechanical compression, leading to secondary vascular changes of venous dilation and hemorrhage. Given this mechanism, the underlying reasons for unilateral and asymmetrical papilledema remain unclear.

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