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Idiopathic intracranial hypertension without papilledema in children: A case series

Kaliopy Matheos, Shuan Dai

Papilledema has long been considered a hallmark of idiopathic intracranial hypertension, a disease defined by elevated intracranial pressure with indiscernible etiology. Papilledema is often seen in the pediatric population, and as such can lead to delays in diagnosis, and often misdiagnosis. Here, we describe three children who were confirmed to have idiopathic intracranial hypertension with raised intracranial pressure by repeated lumbar puncture or intracranial pressure monitoring, normal neuroimaging and absence of papilledema. All three cases had atypical clinical presentations with visual disturbances or photophobia. The patients had a normal body mass index. This case series demonstrates that idiopathic intracranial hypertension can manifest in the absence of clinically obvious papilledema, and has, as such, the potential to cause permanent visual loss if the diagnosis is missed.