Idiopathic intracranial hypertension in childhood: pitfalls in diagnosis

Krishnakumar, Deepa; Pickard, John D.; Czosnyka, Zofia; Allen, Louise; Parker, Alasdair

doi:10.1111/dmcn.12475

Cited by 16 publications

(14 citation statements)

References 25 publications

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“…In adults and children the assessment of average CSF pressure over more than 20 minutes, 'steady state', is reported to be more reliable than a single opening pressure measurement using the height of a fluid column (34)(35)(36).…”

Section: Diagnosismentioning

confidence: 99%

Idiopathic Intracranial Hypertension and Associated Optic Neuropathy in Pediatric Patients

Merticariu¹,

Baltă²,

Merticariu³

et al. 2017

JTMR

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While idiopathic intracranial hypertension (IIH) is more commonly recognized as a disorder of adults, it affects children of all ages and can have distinctive characteristics when presenting in the prepubertal age group. It is characterized by raised intracranial pressure (ICP) in the absence of brain parenchymal lesion, vascular malformations, hydrocephalus, or central nervous system (CNS) infection. The diagnosis is usually confirmed by a high opening pressure of cerebrospinal fluid (CSF) with exclusion of secondary causes of intracranial hypertension. Objectives: The purpose of this review is to summarize relevant articles on the diagnostic tools used in evaluation and management of pediatric IIH. A summary of demographics, clinical presentation, diagnosis, neuroimaging, as well as existing evidence of treatment strategies is presented. Method: We conducted a bibliographic search in PubMed using the terms idiopathic intracranial hypertension (IIH), Pseudotumor Cerebri syndrome (PTCS). The review of the literature revealed a lack of published consistent data on the diagnosis criteria and management of idiopathic intracranial hypertension in children. Conclusion:This article provides a review of IIH in children and revised diagnostic criteria based on recent evidence and published opinion. Relative to the adult population, the demographic features and clinical presentation of IIH as well as the diagnosis and treatment guidelines for children are quite different. This review highlights the importance of early recognition and management of IIH to prevent permanent visual loss. Key words: idiopathic intracranial hypertension, pseudotumor cerebri syndrome Idiopathic intracranial hypertension (IIH), also referred to as Pseudotumor Cerebri, is characterized by elevated intracranial pressure without discernable etiology, with normal cerebrospinal fluid content, and normal contrast-enhanced computed tomography (CT) or magnetic resonance irradiation (1,2,3).

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Section: Diagnosismentioning

confidence: 99%

Idiopathic Intracranial Hypertension and Associated Optic Neuropathy in Pediatric Patients

Merticariu¹,

Baltă²,

Merticariu³

et al. 2017

JTMR

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“…(22) In adults and children the assessment of average CSF pressure over more than 20 minutes, 'steady state', is reported to be more reliable than a single opening pressure measurement using the height of a fluid column. (23,24,25) Diagnostic errors resulted in overdiagnosis of IIH in 39.5% of patients referred for presumed IIH to a neuro-ophthalmology service of a tertiary center and prompted unnecessary tests, invasive procedures, and missed diagnoses. The most common errors were inaccurate ophthalmoscopic examination in headache patients and thinking biases, reinforcing the need for rapid access to specialists with experience in diagnosing optic nerve disorders.…”

Section: Neuroimagingmentioning

confidence: 99%

Optic Neuropathy in Idiopathic Intracranial Hypertension

Merticariu¹,

Baltă²,

Merticariu³

et al. 2017

JTMR

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Adult patients who present with papilloedema and symptoms of raised intracranial pressure need urgent multidisciplinary assessment including neuroimaging, to exclude life-threatening causes. Where there is no apparent underlying cause for the raised intracranial pressure, patients are considered to have idiopathic intracranial hypertension (IIH). Objectives: This review encompasses the newly revised diagnostic criteria for PTCS in adults and children and the role of obesity in the epidemiology, etiology, and management of IIH.Furthermore, this paper tries to offer a practical approach to assessing patients with papilloedema, the investigations and the subsequent management of patients with IIH. Methodology: PubMed was used to search for IIH and Pseudotumor Cerebri. The vast majority of the literature consisted of cohort studies, case control studies, systematic reviews and other narrative reviews. Conclusion: Idiopathic intracranial hypertension (IIH), also known as primary Pseudotumor Cerebri syndrome (PTCS), is a condition of unknown etiology which affects primarily overweight, reproductive-aged women and causes increased intracranial pressure (ICP). This review discusses the recently revised diagnostic criteria for IIH for adults and children and the controversial issues in its diagnosis and management.

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“…Although ICP can reach levels that, if not relieved, can cause papilledema leading to optic atrophy and severe visual loss, impaired cognitive function has not been described. 2,44,66,69,87 That is not to say that IIH arising in childhood does not affect NCI-it may not have been looked for, as it has been in a study (in adults) by Yri et al, 88 who found that "patients with IIH performed significantly worse than controls in four of six cognitive domains. .…”

Section: Idiopathic Intracranial Hypertensionmentioning

confidence: 99%