Abstract

Background: Vertebral artery dissection due to dynamic compression by abnormal C1-C2 anatomy is under-recognized in childhood stroke. Our aim was to describe underlying pathologic variants of C1-C2 soft tissue or bone in children with vertebral artery dissection and posterior circulation strokes. Methods: A series of five children diagnosed with vertebral artery dissection from compression by C1-C2 soft tissue or bone were identified at a single pediatric stroke center. Diagnostic evaluation, anatomic cause of dissection, and treatment were abstracted from medical records. Results: Five children (all boys, ages 6 - 14) were identified with a diagnosis of posterior circulation embolic strokes due to focal vertebral artery dissection and presumed compression against adjacent C1-C2 abnormalities. Four had recurrent events, including basilar artery thrombosis, before the cervical abnormality was identified. Vertebral artery dissections were caused by bony (congenital arcuate foramen, post-traumatic os odontoideum) and soft tissue pathology (ruptured atlantoaxial bursa, anomalous C1-C2 connective tissue bands) (Table). Vertebral artery compression at dissection sites during head rotation was confirmed by angiography with head in neutral and rotated in children with soft tissue anomalies. No other etiology for stroke or dissection was found in any of the cases. For secondary stroke prevention, four children underwent direct surgical decompression of the vertebral artery or spinal fusion; one opted for activity restriction and aspirin. None had further recurrences after treatment with a median follow-up of 9 months (range 1 to 61). Conclusions: In children with recurrent vertebral artery dissection and strokes, C1-C2 bone imaging and dynamic angiogram with neck rotation can identify cervical pathology that may otherwise be missed. Cervical stabilization or resection of the compressive pathology should be considered to prevent stroke recurrence.

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