Fifteen-minute consultation: the child with idiopathic intracranial hypertension

Abstract

Idiopathic intracranial hypertension (IIH) is a rare condition where intracranial hypertension is found in the context of normal brain parenchyma and no mass lesion, ventriculomegaly, underlying infection, or malignancy. Our understanding of this condition has greatly improved in the recent years with neuroimaging features and normal values for lumbar puncture opening pressure now well defined. This article provides a review of IIH in children and revised diagnostic criteria based on recent evidence and published opinion. We have also presented an algorithmic approach to the child with possible IIH.

Idiopathic intracranial hypertension in pediatric and adolescent patients

“…The clinical findings of IIH are variable [4,15], but headache and papilledema are the main clinical findings in adult with or without additional symptoms and signs of increased ICP [4,9,14,19,20]; however, a typical IIH was diagnosed without headache [12] or papilledema [21]. In this study, the patients of group II have quite similar clinical findings and diagnostic criteria to adult IIH as headache is the most common symptom while non-specific symptoms of increased ICP as deterioration of the school performance, irregular sleeping and irritability, and loss of appetite were significant in group I (Table 1).…”

“…In this study, the mean opening pressure is 291 ± 79.5 cmH 2 O and the minimum value for opening pressure in group I is 180 cmH 2 O, while the minimum value in group II is 250 cm (Table 1); however, the diagnostic limit of the CSF opening pressure is considered a matter of debate in the diagnosis of pediatric IIH, but in the presence of typical IIH, symptoms and signs with papilledema low opening pressure could not exclude the diagnosis as the lumbar puncture may be done during the nadir of a pressure wave [12,14]. Intracranial hypertension is considered in children with age less than 8 years if there is papilledema and cerebrospinal fluid opening pressure greater than 18 cmH 2 O or no papilledema with opening pressure greater than 25 cmH 2 O while in children above 8 years old the diagnosis is confirmed if the opening pressure is greater than 25 cmH 2 O with papilledema [2].…”

“…The clinical presentation of IIH is variable and the diagnostic criteria have changed over time, but currently, Dandy criteria and its modifications which involve symptoms and signs of increased intracranial pressure with elevated CSF pressure without laboratory or radiological evidence of intracranial lesion, infection, or hydrocephalus are used for diagnosis of IIH in adult patients [2,4,5,12]; however, there is no agreement for specific diagnostic criteria in pediatric IIH due to controversies about the diagnostic value of CSF opening pressure [13][14][15][16], in addition to the difference in the presentation and clinical finding between children and adolescent patients [2,16].…”

How to use lumbar puncture manometry in children

“…It is also known as pseudotumour cerebri syndrome and was previously called benign intracranial hypertension. Patients typically present with headaches and may have visual symptoms and signs on examination, typically including papilloedema, with an otherwise normal neurological examination 19…”

“…Patients typically present with headaches and may have visual symptoms and signs on examination, typically including papilloedema, with an otherwise normal neurological examination. 19 When performing an LP for diagnosis of IIH, if CSF pressure is found to be raised, aim for a closing pressure within the normal range (eg, between 20 cmH 2 O and 25 cmH 2 O in most children above the age of 1 year). 19 Some clinicians suggest not reducing pressure to less than 30 cmH 2 O if CSF pressure is exceptionally high (ie, greater than 60 cmH 2 O) to avoid risks of post-LP headaches, but this is not evidence-based.…”