Objective: To describe a pediatric stroke syndrome with chronic focal vertebral arteriopathy adjacent to cervical abnormalities. 

Methods: At a single pediatric stroke center, we identified consecutive children with stroke and vertebral arteriopathy of the V3 segment with adjacent cervical bony or soft tissue abnormalities. We abstracted clinical presentation, treatment and follow-up data from medical charts. 

Results: From 2005-2016, ten children (all boys, ages 6 – 16 years) presented with posterior circulation strokes and vertebral arteriopathy with adjacent cervical pathology. Two children had bony abnormalities: one had a congenital arcuate foramen and one had os odontoideum with cervical instability. In children without bony pathology, vertebral artery narrowing during contralateral head rotation was visualized by digital subtraction angiography. Eight boys had recurrent ischemic events despite anti-thrombotic treatment (including 5 with multiple recurrences) and were treated surgically to prevent additional stroke. Procedures included vertebral artery decompression (n=6), endovascular stent and spinal fusion (n=1), or vertebral artery endovascular occlusion (n=1). In boys treated with decompression, cervical soft tissue abnormalities (ruptured atlantoaxial bursa, ruptured joint capsule or connective tissue scarring) were directly visualized during open surgery. No other etiology for stroke or dissection was found in any of the cases. Two boys without recurrent stroke were treated with activity restriction and anti-thrombotics. At a median follow-up of 51 months (range 17-84), there have been no additional recurrences.

Conclusions: Children with V3 segmental vertebral arteriopathy frequently have stroke recurrence despite anti-thrombotics. Cervical bone imaging and angiography with neck rotation can identify underlying pathology.

We retrospectively and prospectively identified a consecutive series of patients <18 years of age at the University of California San Francisco (January 2005-December 2016) who had ischemic stroke due to focal V3 segment vertebral arteriopathy and imaging or direct visualization of adjacent bony or soft tissue abnormalities. All data were abstracted from medical records in a standardized fashion. Patient characteristics, disease course and management are summarized in the main manuscript. This supplemental table provides additional individual case data.