Evaluation of the underlying causes of papilledema in children

Purpose: The purpose of this retrospective study was to identify the types and relative frequencies of intracranial disorders in pediatric patients who present with papilledema. Methods: This was a retrospective study conducted in two pediatric ophthalmology clinics, both providing community-based care in a large inner-city urban center in the USA. Pediatric patients aged between 0–16 years diagnosed with papilledema, and who had an underlying etiology identified were included in the study. Patient demographic data, ophthalmologic examination findings and diagnostic work-up results were identified from clinical records. Results: The mean age of 38 study patients (19F, 19M) was 8.6 ± 4.8 years. Of the 38 patients, 16 (42.1%) had idiopathic intracranial hypertension (IIH) as the underlying cause of the papilledema, 7 (18.4%) had a craniosynostosis disorder, 6 (15.8%) had intracranial tumors, 2 (5.3%) had primary hydrocephalus, and 7 (18.4%) had other diagnoses. Of the six intracranial tumors, two (33.3%) presented in the sellar/parasellar region, two (33.3%) in the posterior fossa and two (33.3%) were in cortical locations. Conclusion: Clinicians should have a high index of suspicion for IIH and brain tumors in children presenting with papilledema. Patients with craniosynostosis should have routine eye examinations to monitor for asymptomatic papilledema. Understanding the relative incidence of etiologies for papilledema highlights the urgency of appropriate work-up and the need to consider low frequency etiologies.