Pediatric Bow Hunter syndrome: clinical, vascular, and longitudinal findings from a single center and systematic review of the literature

Bow Hunter syndrome (BHS) is a rare cause of pediatric vertebral artery dissection (VAD) and posterior circulation arterial ischemic stroke (AIS) due to dynamic compression during head rotation. Imaging features, vascular evolution, and outcomes remain poorly defined. We reviewed children with definite or probable BHS diagnosed at a tertiary pediatric center between 2010 and 2026. A systematic review identified published pediatric cases (0–18 years) of definite/probable BHS. Twelve boys were included (mean age, 7.3 years; mean follow-up, 54.4 months). Posterior circulation AIS occurred in 10/12 (83.3%), with multifocal infarcts of different ages in eight and additional anterior circulation embolic infarcts in three. VAD was bilateral in 6/12 (50%), consistently involving V3. Distal posterior cerebral and/or superior cerebellar artery stenosis or occlusion with collateral networks occurred in 7/12 (58.3%); basilar artery involvement in 4/12. Dynamic vascular imaging demonstrated position-dependent vertebral artery compromise in 9/12 patients (eight by rotational DSA and one by Doppler ultrasound). Craniocervical junction variants were present in 10/12 (83.3%), without flexion-extension instability. Early recurrent AIS occurred in 5/10 despite antithrombotic therapy. Serial imaging showed early vascular evolution followed by stabilization. Two patients underwent surgery; the others were treated conservatively. A rotational mechanical mechanism should be considered in children with posterior circulation AIS and V3 abnormalities, particularly when preceded by overlooked prodromal symptoms or minor trauma- and sport-related triggers. Multimodal neurovascular imaging, especially dynamic angiography, is essential for diagnosis, surveillance, and individualized treatment planning aimed at preventing further vascular injury.