Predictors of disease course and long-term outcomes of idiopathic intracranial hypertension in children and adolescents

Noam Senderowich, Anat Bachar-Zipori, Alexis Mitelpunkt, Itay Tokatly Latzer, Ainat Klein, Daphna Mezad-Koursh, Aviva Fattal-Valevski, Moran Hausman-Kedem*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

This study aimed to identify predictors for unfavorable disease course and clinical and visual outcomes in pediatric patients with idiopathic intracranial hypertension (IIH). Employing a multi-tiered approach, we retrospectively analyzed clinical, ophthalmic, and neuroimaging data from patients diagnosed with IIH between 2003 and 2021. Of the 97 patients included, 56 (58%) were females. The median age was 12 years [Interquartile range (IQR) 9, 14], and the median follow-up time was 39.0 months (IQR 14.8, 90.9). Forty-two (43%) patients had an unfavorable disease course, 28 (29%) had persistence of headache at last follow-up, and 16 (18%) had a poor visual outcome, most of them with mild visual disturbances. Poor visual outcome was more common in females compared to males [16/47 (34%) vs. 0/39, p < 0.001)]. On multivariate regression analysis, female sex and disease recurrence were significantly associated with poor visual outcomes (OR: 18.5, CI:1.3–270, P = 0.03, and OR: 5.1, CI: 1.2–22.5, P = 0.03, respectively). Patients with persistent headaches exhibited lower incidence of papilledema, lower opening pressure, and fewer neuroimaging markers indicating elevated intracranial pressure. Conclusions: This study provides insights into predictive factors for an unfavorable disease course, persistent headaches, and poor visual outcomes in patients with childhood IIH. Patients with persistent headaches may have a variant of a chronic pain syndrome warranting a different therapeutic approach. What is Known: • Childhood-onset Idiopathic Intracranial hypertension (IIH) is a heterogenous disease. The knowledge on disease trajectory and long-term outcomes and its predictors is limited. What is New: • A higher opening pressure and factors suggestive of the metabolic syndrome predict an unfavorable disease course whereas female sex and disease recurrence are significantly associated with poor visual outcomes • A third of the patients diagnosed with IIH experience ongoing headaches despite achieving favorable visual outcomes. This

Original languageEnglish
Pages (from-to)5137-5147
Number of pages11
JournalEuropean Journal of Pediatrics
Volume182
Issue number11
DOIs
StatePublished - Nov 2023

Keywords

  • Headache
  • Idiopathic intracranial hypertension
  • Papilledema
  • Pediatric
  • Pseudotumor cerebri
  • Visual outcome

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