Paediatric Idiopathic Intracranial Hypertension (IIH)—A Review

Abstract

Paediatric idiopathic intracranial hypertension (IIH), is a rare but important differential diagnosis in children presenting with papilloedema. It is characterised by raised intracranial pressure in the absence of an identifiable secondary structural or systemic cause and is, therefore, a diagnosis of exclusion. In the adult population, there is a strong predilection for the disease to occur in female patients who are obese. This association is also seen in paediatric patients with IIH but primarily in the post-pubertal cohort. In younger pre-pubertal children, this is not the case, possibly reflecting a different underlying disease aetiology and pathogenesis. Untreated IIH in children can cause significant morbidity from sight loss, chronic headaches, and the psychological effects of ongoing regular hospital monitoring, interventions, and medication. The ultimate goal in the management of paediatric IIH is to protect the optic nerve from papilloedema-induced optic neuropathy and thus preserve vision, whilst reducing the morbidity from other symptoms of IIH, in particular chronic headaches. In this review, we will outline the typical work-up and diagnostic process for paediatric patients with suspected IIH and how we manage these patients.

Idiopathic Intracranial Hypertension in a Malaysian Preschooler

“…Randomized clinical trials are limited in pediatric IIH due to its rare incidence. Thus, the treatment for pediatric IIH is largely based on evidence procured from the adult population [ 11 ]. The mainstay of treatment is lowering intracranial pressure (ICP) to preserve vision and control symptoms.…”

Idiopathic intracranial hypertension in children with obesity

“…9 It is also thought that the IIH mechanism may be different between the pre-and post-pubertal groups due to the different demographics and risk factors between the two cohorts. 7 Levels of androgens and metabolic factors, such as insulin and leptin-resistance, are being considered as possible contributors to the underlying pathophysiology. 7 Data regarding IIH in childhood obesity is limited and it does not appear to be screened for as often as other complications such as non-alcoholic fatty liver disease and impaired glucose tolerance.…”

“…1,3,[5][6][7] The pathophysiology between obesity and IIH remains unclear. 1,7,8 It has been suggested by Salpietro et al that rapid weight gain causes intra-abdominal pressures to rise, which results in increased cerebral venous pressures. 9 It is also thought that the IIH mechanism may be different between the pre-and post-pubertal groups due to the different demographics and risk factors between the two cohorts.…”

Evolving Evidence in Idiopathic Intracranial Hypertension

“…Due to the nature of the disease, 7% have severe visual loss at presentation caused by papilledema, requiring neurosurgical intervention [2,6]. The complexity of the spectrum of this disorder includes people who have raised ICP, but never develop papilledema [7] and pre-puberty the disease shares some clinical characteristics but is expectantly distinct from the adult phenotype [8]. This Special Issue "Idiopathic Intracranial Hypertension" published in Life (ISSN 2075-1729) has highlighted the worldwide research from a multi-professional perspective within the neurosciences.…”

“…Diagnosing and managing IIH can be challenging [2] and selected articles underlined the importance of the multidisciplinary team in joint pathways where specialists worked together to provide the best care for patients [1,6,8]. A number of articles investigated methods to aide diagnostic certainty and enable stratification of the disease [9][10][11][12].…”