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.

Keywords: headache; optic nerve; optical coherence tomography; paediatric idiopathic intracranial hypertension; papilloedema; pseudo tumour cerebti; vision.

Figure 1

Colour optic nerve photographs showing Paton’s lines and exudates bilaterally.

Figure 2

Humphrey 24-2 visual field test demonstrating bilaterally enlarged blind spots.

Figure 3

Showing Frisen scale Grade 4 Papilloedema on the left. Same disc following resolution of papilloedema resulting in optic atrophy and pallor resulting in permanent sight loss.

Figure 4

Optos fundus autofluorescence; optic disc drusen show as autofluorescence.

Figure 5

Magnetic resonance venography with blue arrow demonstrating transverse venous sinus stenosis.