Idiopathic intracranial hypertension (IIH) is a rare condition of increased intracranial pressure (ICP) without any identifiable pathology. Despite intervention, the clinical course of IIH is often prolonged and recurring with potentially serious complications of distressing headache and blindness.1^–10 Various therapeutic measures either alone or in combination have been used widely in children with IIH, however, their therapeutic efficacy has not been proven in controlled studies. This article provides a comprehensive review of clinical knowledge in childhood IIH and its various treatment modalities with an emphasis on the drugs used. It also highlights that current management is not evidence based and that well-designed multicentre randomised controlled trials are urgently needed.

TERMINOLOGY

Idiopathic intracranial hypertension was first described in 189711 as “serous meningitis”, followed by various names such as otitic hydrocephalus, toxic hydrocephalus, etc, pseudotumour cerebri,12 and the commonly known benign intracranial hypertension.13 Subsequently, IIH was introduced14 and is now a preferred term due to its identified risk (up to 40%) of visual impairment.2 6 7 15^–19

INCIDENCE

The overall (including children and adult) annual incidence of IIH is estimated at 1–3 per 100 000 population.20^–22 To date, the epidemiological data on childhood IIH, which are limited to hospital-based retrospective case series, vary greatly from 0.1–0.9 per 100 000 children.23 24 Both genders are equally affected in childhood IIH, whereas it is most common in obese young women in adult IIH. However, obesity has become increasingly dominant in recent paediatric reports, affecting 43–91% of teenage IIH cases.1 7 25 This raises the concern that the recent rise in the prevalence of childhood obesity will be associated with an increased incidence of paediatric IIH.

DIAGNOSTIC CRITERIA

Although the key features of IIH are headache and papilloedema (table 1), neither of them is a prerequisite …