Cornelis Jan Pronk

Aim Breath‐holding spells (BHS) are common in children, but evidence‐based clinical guidelines are lacking. We investigated a large population‐based cohort of BHS patients, to propose a refined description of typical BHS and guidelines for its management. Methods In a cross‐sectional retrospective study, patients diagnosed with BHS in Southern Sweden 2004–2018 were recruited. Disease characteristics and diagnostic data were collected from patient medical records. Results In total, 519 patients, mean age at diagnosis 19.8 ± 13.8 months with equal gender distribution, were included. In 48.3%, BHS had already been diagnosed after one spell. During spells, 78.0% of patients were unresponsive. For 71.5%, atonic, tonic, tonic–clonic or myoclonic seizures were reported, and 78.0% of patients had a spell lasting less than 1 min. Electroencephalography was conducted in 30.4% and Electrocardiography in 45.1%. Six children (3.8%) had a pathological electroencephalogram, four of which had concomitant epilepsy and only 0.9% of children had electrocardiogram findings suggesting pathology, none showing long QT syndrome. Conclusion Children with BHS were frequently subjected to unnecessary diagnostic interventions. We characterise a typical presentation of BHS and propose a management‐algorithm, which is expected to reduce unnecessary usage of electroencephalography and electrocardiography.