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

Abstract

Abstract Purpose: To identify predictors for unfavorable disease course and clinical and visual outcomes in pediatric patients with idiopathic intracranial hypertension (IIH). Methods: A multi-tiered approach analyzing retrospectively collected clinical, ophthalmic, and imaging findings of patients diagnosed with IIH between 2003-2021. Results: Of the 97 patients, 42 (43%) had an unfavorable disease course, 28 (29%) had persistence of headache at last follow-up, and 16 (18%) had a poor visual outcome. On univariate regression analysis, female sex, overweight/obesity, visual field (VF) defect at presentation, and retinal nerve fiber layer (RNFL) >130μm after treatment initiation served as predictors of an unfavorable disease course as well as poor visual outcome. An unfavorable disease course was significantly associated with higher opening pressure (OP), evidence of polycystic ovary syndrome and higher triglyceride levels whereas optic atrophy at presentation, and disease recurrence were also associated with poor visual outcome. On multivariate regression analysis, female sex and disease recurrence remained 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 a persistent headache had a lower OP, lower papilledema rates and less neuroimaging parameters suggestive of increased intracranial pressure. Conclusions: We provide multi-layered insights into predictive factors for an unfavorable disease course and poor clinical and 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.