Anatomic and visual function outcomes in paediatric idiopathic intracranial hypertension

There is a paucity of literature describing risk factors for vision loss in paediatric idiopathic intracranial hypertension (IIH). We investigate the final visual function, spectral domain optical coherence tomography (SD-OCT) and enhanced depth imaging (EDI)-OCT findings in children with papilledema caused by IIH.

Medical records of 31 patients with paediatric IIH (age ≤17 years) were retrospectively reviewed. Optic disc photographs on presentation and automated perimetry, SD-OCT and EDI-OCT imaging on final follow-up visit were statistically analysed to identify patient characteristics and anatomic findings associated with irreversible vision loss.

Permanent visual acuity or visual field loss developed in 19% of study eyes. Papilledema of modified Frisén grade ≥3 on presentation was highly predictive of permanent vision loss (p<0.001), while associations between pubertal status and visual function outcome failed to reach statistical significance. SD-OCT revealed optic atrophy in 13% and photoreceptor loss in 19% of eyes, with both findings highly associated with vision loss (p<0.0001). Optic disc drusen was noted in 48% of study eyes by EDI-OCT but was not found to be predictive of visual outcome.

Clinical observation of high papilledema grade on presentation is predictive of poor visual outcomes. Vision loss is associated not only with optic atrophy but also with photoreceptor damage. Interestingly, a high proportion of study eyes had optic disc drusen, which was not associated with vision loss, but can be a diagnostic challenge in distinguishing true papilledema from pseudopapilledema.