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.

Highlights

  • Paediatric idiopathic intracranial hypertension (IIH) is a rare condition affecting between 1 in 100,000–150,000 children in the population [1,2,3]. It is defined as raised intracranial pressure (ICP) in the absence of a site-occupying lesion, normal brain structure and vasculature, and normal metabolic, endocrine, and haematological systemic states

  • A meta-analysis of the epidemiological characteristics of paediatric IIH was reported by Genizi et al showed 44% of those affected under 12 years of age were female

  • The referring hospital carries out a history, documents weight, height, BMI, medications, Tanner/pubertal status, and basic blood profiles alongside neuroimaging (MRI to include orbits, optic nerves, and pituitary fossa and magnetic resonance imaging venogram (MRV) to rule out venous sinus thrombosis) if possible

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Summary

Introduction

Paediatric idiopathic intracranial hypertension (IIH) is a rare condition affecting between 1 in 100,000–150,000 children in the population [1,2,3]. It is defined as raised intracranial pressure (ICP) in the absence of a site-occupying lesion, normal brain structure and vasculature, and normal metabolic, endocrine, and haematological systemic states. Variable clinical signs, and symptoms and the challenges associated with diagnostic investigations, which often require general anaesthesia or sedation, all add to this challenge Despite these difficulties, accurate diagnosis is essential to limit long-term morbidity

Demographics
Risk Factors
Pathophysiology
Symptoms
Work-Up and Diagnosis
Management and Monitoring
Conservative Measures
Medications
Findings
Surgical Options
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