A step toward understanding pediatric occipital neuralgia: Investigating occipital headaches in a pediatric neurology clinic

BACKGROUND: Occipital neuralgia (ON) is well characterized in adults but remains poorly understood in children. Pediatric ON may present with overlapping headache features and variable treatment responses, creating diagnostic and management challenges. OBJECTIVES: We aimed to (1) characterize pediatric patients presenting to a tertiary outpatient neurology clinic with occipital headache symptoms consistent with ON, (2) describe their overlap with other headache symptom profiles, and (3) summarize documented diagnostic evaluation, treatment patterns, and clinical outcomes. METHODS: We conducted a retrospective cohort study using prospectively collected, standardized headache intake questionnaire data from the Children's Hospital of Philadelphia (CHOP) headache registry, including pediatric patients evaluated in outpatient neurology from July 2016 through August 2024. Self-reported headache characteristics were used to identify a cohort of patients with symptoms consistent with ON, defined as recurrent, short-duration, stabbing or sharp, severe pain involving the occipital region. We excluded patients who also had frontotemporal or holocephalic pain. However, to avoid missing patients with overlapping headache phenotypes, we did not exclude patients whose headaches also met criteria for other diagnoses such as migraine. RESULTS: We identified 89 of 14,876 (0.6%) patients within the registry with symptoms consistent with ON, predominantly male (54%) with a median age of 12.7 years (interquartile range, 9.3-15.5). Sixty of the 89 (67%) patients additionally met symptom-based criteria for another headache disorder, mostly migraine. Forty-five of 89 (51%) patients followed up over a median of 1.1 years (interquartile range, 0.6-4.2), with 35 of 45 attempting treatment. At the most recent follow-up of those treated, 26% reported significant improvement (9 of 35; 95% confidence interval [CI], 12.5%-43.3%), 29% some improvement (10 of 35; 95% CI, 14.6%-46.3%), 26% no change (9 of 35; 95% CI, 12.5%-43.3%), and 20% worsened (7 of 35; 95% CI, 8.4%-36.9%). CONCLUSION: Symptoms consistent with ON are rare, even among children with occipital headaches, and frequently overlap with features of other primary headache disorders. These findings highlight the diagnostic complexity of pediatric occipital headache and the need for improved characterization and standardized evaluation to inform future treatment pathways for children with persistent ON-like symptoms.