Patient Delay, Lead Times, and Adherence to Diagnostic Guidelines in Children and Adolescents With Idiopathic Intracranial Hypertension.

Aims: In a cohort of 45 children and adolescents diagnosed with idiopathic intracranial hypertension (IIH), our main aims were to investigate patient delay, lead time to final diagnosis, and adherence to current diagnostic guidelines.

Methods: This population-based, retrospective, single-center cohort study was performed at Uppsala University Children's Hospital, Sweden, a tertiary referral center for children and adolescents with rare and/or complicated neurologic disease. Patient data were retrieved from the local registries for patients filling the following criteria: age (0-17.99 yr), study period (2000-2020), and International Classification of Diseases code G93.2 (IIH). Medical records from pediatric, neuropediatric, ophthalmology, and neurosurgery departments were scrutinized. All included patients met the Friedman criteria.

Results: Fifty-one percent of the patients sought medical advice within 1 month of symptom debut, 23% were seen within 1 to three months, and 26% after three months. A final diagnosis of IIH was reached within 48 hours in 60%, within two weeks in 80%, and within four weeks in 89% of patients. Visual fields, color vision, and complete ancillary laboratory investigations to exclude secondary etiologies were performed in 62%, 47%, and 59% of patients, respectively.

Conclusion: The clinical presentation of IIH in children and adolescents may range from acute fulminant symptoms, to a more insidious or even chronic presentation with long-term headache. Although a majority of patients received a correct and prompt diagnosis, lead time to final diagnosis and adherence to diagnostic care guidelines might be improved. A higher awareness and knowledge of the condition may achieve this.