Elsevier

Pediatric Neurology

Volume 43, Issue 6, December 2010, Pages 377-390
Pediatric Neurology

Review Article
Idiopathic Intracranial Hypertension in Children: A Review and Algorithm

https://doi.org/10.1016/j.pediatrneurol.2010.08.001Get rights and content

This updated review of pediatric idiopathic intracranial hypertension focuses on epidemiology, clinical presentations, diagnostic criteria, evaluation, clinical course, and treatment. General guidelines for the clinical management of idiopathic intracranial hypertension are discussed. A new algorithm outlines an efficient management strategy for the initial diagnostic evaluation of children with signs or symptoms of intracranial hypertension. This algorithm provides a systematic approach to initial evaluation and management, and identifies important decision-making factors. The risk of permanent visual loss with idiopathic intracranial hypertension necessitates a prompt, thorough collaborative approach in the management of patients. Although idiopathic intracranial hypertension has been recognized for over a century, the need remains for prospectively collected data to promote a better understanding of the etiology, risk factors, evaluative methods, and effective treatments for children with this syndrome.

Introduction

Increased intracranial pressure is classified into two main categories, depending on its idiopathic or secondary etiology. In the literature, the two categories have not always been clearly delineated, leading to difficulties in understanding associations or drawing inferences about the syndrome. Categorizing etiologies is necessary, because characteristics, management, and outcomes may vary considerably. Idiopathic intracranial hypertension is defined increased intracranial pressure without a space-occupying lesion or hydrocephalus and normal cerebrospinal fluid composition [1]. Initially termed “serous meningitis,” this syndrome was first recognized by Quincke in 1897 [2]. With its unclear etiology, the syndrome has assumed different names, including pseudotumor cerebri and benign intracranial hypertension. Especially in children, questions continue to surround the diagnostic criteria and medical management.

A review of the literature indicates that the diagnostic evaluation of these patients often involves several medical subspecialties. Articles have been written by different physicians in many subspecialties, including general practice, emergency medicine, radiology, psychiatry, neurology, ophthalmology, neuro-ophthalmology, and neurosurgery [3], [4], [5], [6], [7], [8]. Although the approach to patients with idiopathic intracranial hypertension is often similar among subspecialties, differences do exist. Coordinating the patient’s care from presentation to follow-up is an enormous challenge. Because a risk of permanent visual loss exists with idiopathic intracranial hypertension, patients require a prompt and thorough collaborative approach in their management.

Wide diversity is evident in the published approaches, and little has been written about children. Therefore, an urgent need exists for a coherent approach to the evaluation and management of children. This updated review of idiopathic intracranial hypertension offers a focus on children, including epidemiology and pathogenesis, clinical presentations, common clinical course, diagnostic criteria and challenges, and treatments. An algorithm for the initial diagnostic evaluation of children with intracranial hypertension is proposed. This algorithm provides a systematic approach to management, identifies important decision-making factors, and illustrates the importance of a coordinated multidisciplinary approach.

Section snippets

Epidemiology

The true incidence of idiopathic intracranial hypertension in children is unknown. In the past, children were often included within adult studies, leaving the pediatric incidence unclear. Moreover, several previous pediatric studies did not exclude children in whom a secondary etiology was discovered for their intracranial hypertension signs. Hence the number of patients with idiopathic intracranial hypertension may have been falsely elevated [9], [10]. Although the incidence of idiopathic

Diagnostic Challenges

Diagnostic criteria were originally outlined by Smith [30] (Table 1), based on characteristics described by Dandy in 1937 [31]. Over the past several years, physicians have proposed further expansions of the criteria, primarily as a result of further research and knowledge, as well as advances in diagnostic technology such as magnetic resonance imaging. For example, physicians have recommended further clarification that a patient is not required to exhibit signs of idiopathic intracranial

Clinical Presentations

In children, the most common presenting symptom is headache [11], [16], [58]. However, the characteristics of these headaches are variable. A headache may begin as moderate and episodic and progress to severe, chronic pain [62]. Patients often report a headache to be exacerbated by Valsalva and postural changes [62]. Patients may describe a headache similar to a migraine, with a pulsatile quality as well as photophobia, nausea, and vomiting [62]. Differentiating between headaches caused by

Approach

The need exists for a systematic approach to the initial diagnostic evaluation of a child with signs of intracranial hypertension. The proposed algorithm provides a systematic approach to initial evaluation and management, and identifies important decision-making points (Figure 1, Figure 2). It also illustrates the importance of a coordinated multidisciplinary approach. Although Class I evidence is lacking, this algorithm could support more consistent practice and provide a foundation for

Collaboration

Collaboration among physicians within the algorithm is very important. The speed of the initial diagnostic evaluation and management depends on a well-coordinated multidisciplinary approach. If urgent outpatient cranial imaging and lumbar puncture are not possible, the neurologist may need to coordinate with a nearby, appropriately equipped facility for a rapid evaluation. This procedure may be particularly beneficial if sedation is necessary to complete cranial imaging and lumbar puncture.

Discussion and Limitations

One limitation of the algorithm pertains to a lack of recommended time frames for diagnostic procedures. At present, no evidence-based data provide guidance for a specific timeline of evaluation. Because visual loss can occur any time during the course of idiopathic intracranial hypertension, the physician should undertake a diagnostic evaluation promptly, to initiate management for preserving vision and reducing signs.

Another limitation involves the lack of evidence regarding magnetic

Chronic Management

The goal of treatment for idiopathic intracranial hypertension is primarily twofold: to prevent visual loss and to eliminate symptoms. At present, no randomized, controlled, double-blind, prospective studies have evaluated possible treatments for children. Because the etiology of idiopathic intracranial hypertension remains unclear, a specific treatment tailored to the cause has not yet developed. Therefore, current treatments are tailored to treat signs. Further studies are desperately needed

Prognosis

Permanent loss of visual function, regardless of treatment, was reported in children. Permanent decreased visual acuity occurs in up to 10% of children [15], [58], [107]. Permanent visual field defects occur in up to 17% [58]. In mixed adult and pediatric studies, blindness in one or both eyes developed in up to 5% of patients [35], [65]. Findings of visual examinations or complaints such as transient visual obscurations, severity of papilledema, duration of signs, or the presence of cranial

Future Directions and Conclusions

Although the etiology of idiopathic intracranial hypertension currently eludes the medical profession, the continued search for that etiology is imperative, because identifying the cause could lead to better treatments. Idiopathic intracranial hypertension is an uncommon problem, and an algorithm to outline an efficient management strategy for children should be helpful. This algorithm serves as a first step toward the consistent management of children with this condition. The lack of

References (108)

  • P.H. Phillips et al.

    Pseudotumor cerebri in children

    J AAPOS

    (1998)
  • P.N. Sylaja et al.

    Differential diagnosis of patients with intracranial sinus venous thrombosis related isolated intracranial hypertension from those with idiopathic intracranial hypertension

    J Neurol Sci

    (2003)
  • J.C. Orcutt et al.

    Factors affecting visual loss in benign intracranial hypertension

    Ophthalmology

    (1984)
  • C.C. Giles et al.

    Intracranial hypertension and tetracycline therapy

    Am J Ophthalmol

    (1971)
  • W.E. Rosenfeld

    Topiramate: A review of preclinical, pharmacokinetic, and clinical data

    Clin Ther

    (1997)
  • G.T. Liu et al.

    High-dose methylprednisolone and acetazolamide for visual loss in pseudotumor cerebri

    Am J Ophthalmol

    (1994)
  • D.I. Friedman et al.

    Diagnostic criteria for idiopathic intracranial hypertension

    Neurology

    (2002)
  • H. Quincke

    Uber Meningitis serosa and verwandte Zustande

    Dtsch Z Nervenheilkd

    (1897)
  • R.S. Baker et al.

    Visual loss in pseudotumor cerebri of childhood. A follow-up study

    Arch Ophthalmol

    (1985)
  • R.F.R. Agid

    Neuroimaging in the diagnosis of idiopathic intracranial hypertension

    Minerva Med

    (2006)
  • D.K. Binder et al.

    Idiopathic intracranial hypertension

    Neurosurgery

    (2004)
  • D. Soler et al.

    Diagnosis and management of benign intracranial hypertension

    Arch Dis Child

    (1998)
  • D.N. Grant

    Benign intracranial hypertension. A review of 79 cases in infancy and childhood

    Arch Dis Child

    (1971)
  • K. Dhiravibulya et al.

    Benign intracranial hypertension in childhood: A review of 23 patients

    J Paediatr Child Health

    (1991)
  • P. Babikian et al.

    Idiopathic intracranial hypertension in children: The Iowa experience

    J Child Neurol

    (1994)
  • L.A. Weisberg et al.

    Pseudotumor cerebri of childhood

    Am J Dis Child

    (1977)
  • L.J. Balcer et al.

    Idiopathic intracranial hypertension: Relation of age and obesity in children

    Neurology

    (1999)
  • L.A. Weisberg

    Benign intracranial hypertension

    Medicine (Baltimore)

    (1975)
  • A. Rose et al.

    Benign intracranial hypertension in children

    Pediatrics

    (1967)
  • J.F. Schoeman

    Childhood pseudotumor cerebri: Clinical and intracranial pressure response to acetazolamide and furosemide treatment in a case series

    J Child Neurol

    (1994)
  • F.J. Durcan et al.

    The incidence of pseudotumor cerebri. Population studies in Iowa and Louisiana

    Arch Neurol

    (1988)
  • K. Radhakrishnan et al.

    Idiopathic intracranial hypertension (pseudotumor cerebri). Descriptive epidemiology in Rochester, Minn, 1976 to 1990

    Arch Neurol

    (1993)
  • D.I. Friedman

    Pseudotumor cerebri presenting as headache

    Expert Rev Neurother

    (2008)
  • JH Garrett et al.

    The incidence of idiopathic intracranial hypertension in Mississippi

    Ann Neuro

    (2004)
  • B. Mokri

    The Monro-Kellie hypothesis: Applications in CSF volume depletion

    Neurology

    (2001)
  • M. Wall

    Idiopathic intracranial hypertension (pseudotumor cerebri)

    Curr Neurol Neurosci Rep

    (2008)
  • I.H. Johnston et al.

    Benign intracranial hypertension: 2. CSF pressure and circulation

    Brain

    (1974)
  • D.G. Karahalios et al.

    Elevated intracranial venous pressure as a universal mechanism in pseudotumor cerebri of varying etiologies

    Neurology

    (1996)
  • D.B. Baryshnik et al.

    Changes in the appearance of venous sinuses after treatment of disordered intracranial pressure

    Neurology

    (2004)
  • J.L. Smith

    Whence pseudotumor cerebri?

    J Clin Neuro Ophthalmol

    (1985)
  • W.E. Dandy

    Intracranial pressure without brain tumor: Diagnosis and treatment

    Ann Surg

    (1937)
  • K.B. Digre et al.

    Idiopathic intracranial hypertension (pseudotumor cerebri): A reappraisal

    Neurologist

    (2001)
  • The international classification of headache disorders

    Cephalalgia

    (2004)
  • S.G. Weig

    Asymptomatic idiopathic intracranial hypertension in young children

    J Child Neurol

    (2002)
  • M. Wall et al.

    Idiopathic intracranial hypertension. A prospective study of 50 patients

    Brain

    (1991)
  • H.L. Lipton et al.

    Pseudotumor cerebri syndrome without papilledema

    JAMA

    (1972)
  • S.J. Wang et al.

    Idiopathic intracranial hypertension without papilledema: A case-control study in a headache center

    Neurology

    (1998)
  • K.B. Digre et al.

    A comparison of idiopathic intracranial hypertension with and without papilledema

    Headache

    (2009)
  • H.H. Merritt

    Cerebrospinal fluid pressure

  • R.A. Fishman

    Intracranial pressure: Physiology and pathophysiology

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