Papilledema in the pediatric population is seen as part of idiopathic intracranial hypertension (IIH), but can also be a sign of significant pathology. We investigate the final diagnoses in pediatric patients referred for suspected IIH to characterize findings that may indicate an alternate diagnosis.

All pediatric patients referred for suspected IIH from 2008-2018 were identified. Patients with known structural etiologies were excluded. The resulting patients were categorized based on final diagnosis. The pseudotumor cerebri syndrome (PTCS) group included patients with IIH (true IIH or medication-related). The PTCS vs. non-PTCS groups were analyzed for differences in presentation. For the non-PTCS group, the Diagnosis Error Evaluation and Research (DEER) taxonomy tool was applied.

Of 54 patients with suspected IIH, 34 had IIH and 8 had pseudotumor cerebri due to a medication (PTCS group; 78%). The remaining 12 patients (22%) had alternate diagnoses(non-PTCS group): craniosynostosis (2), malignancy (3), benign tumor (1), hydrocephalus (1), retinitis pigmentosa (1), and inflammatory/infectious etiologies (4). Statistically significant differences between the PTCS and non-PTCS groups included age at presentation (average age 11.9 [CI 10.5-13.2] vs. 8.8 [CI 6.9-9.7]), full fields at presentation (35% [CI 23%-46%] vs. 10% [CI 0%-23%]), BMI (23.8 [CI 21.7-25.9] vs. 17.5 [CI 15.7-21.5]), change in RNFL from presentation to follow up (decrease of 28% [CI -19% to -37%] vs. 45% [CI -40% to -50%]), and ventriculomegaly on MRI (7% [CI 3%-11%] vs. 33% [CI 17%-48%]). The DEER tool revealed the greatest number of cases (8) had errors involving laboratory/radiology testing.