Cryptogenic posterior circulation stroke in children.

We aimed to evaluate the percentage of posterior circulation arterial ischaemic stroke (PCAIS) caused by craniovertebral junction (CVJ) anomalies and describe their clinical course. Children admitted to a tertiary care paediatric hospital with PCAIS between July 2017 and December 2020 were assessed retrospectively for disease aetiology. We reviewed the clinical, radiological, and surgical details of children with evidence of CVJ anomalies. Fourteen (24.1%) of 58 children admitted with arterial ischaemic stroke had posterior circulation involvement. The mean age of patients presenting with posterior circulation stroke was 6 years 6 months (range 3 months-15 years), 11 were male. Six of 14 cases with PCAIS were due to CVJ anomaly, their ages ranged from 4 months to 15 years (two age ranges were noted, 4 months-4 years and 11-15 years), four were male. Two children had atlantoaxial dislocation with basilar invagination, two had Bow Hunter syndrome with Chiari malformation type 1 (one with completed stroke), one had Chiari malformation type 1 alone, and one presented with Farber disease with proatlas segmentation anomaly in CVJ. The time lag to stroke and CVJ diagnosis ranged from 2 weeks to 24 months. A dynamic angiogram was required to evaluate biomechanical changes on scans with inconclusive findings on standard stroke imaging. CVJ anomalies are an important treatable cause of paediatric posterior circulation stroke. Cervical spine x-ray in flexion and extension should be done in all patients with posterior circulation stroke beyond the acute period. In cryptogenic aetiology, provocative angiography with guarded neck rotation should be considered to evaluate possible dynamic vertebral artery compression. WHAT THIS PAPER ADDS: Craniovertebral junction anomalies are an important cause of posterior circulation stroke in children. Evidence of flat occiput, short neck, and short stature in children with posterior circulation stroke should be assessed. Dynamic imaging helps identify dynamic vertebral artery compression.