To provide patient-specific recommendations for secondary stroke prevention in childhood.

Approximately 10% of children with arterial ischemic stroke (AIS) suffer recurrent stroke, with children with cerebral arteriopathy at greatest risk.  However, long-term secondary stroke prevention recommendations do not account for underlying stroke etiologies.   

A retrospective single-institution study was performed to identify children with initial and recurrent AIS (age 1 month-18 years) between 2009 and 2022. Patients identified using diagnostic codes for AIS were reviewed to confirm diagnosis, clinical and neuroimaging data. 95% risk estimates were calculated using a beta-binomial model (beta(2,10) prior).

Among 219 children with AIS, 8% (17) suffered recurrent stroke, accounting for 3% (2/69) of cardioembolic stroke, 8% (2/24) aortic/cervical arteriopathy, 18% (4/22) moyamoya, 17% (3/18) focal cerebral arteriopathy (FCA), 38% (3/8) small vessel arteriopathy (SVA), 6% (3/53) with “other etiologies” (radiation arteriopathy, lupus , and severe brain malformation). There were no recurrences in children with stroke due to presumed PFO (0/11) or cryptogenic stroke (0/14).   Two patients with adenosine deaminase deficiency, one with moyamoya and one with bow hunter’s syndrome had stroke recurrence prior to diagnosis of underlying arteriopathy.  Twelve of 72 (17%) children with cerebral arteriopathy due to aorto/cervical arteriopathy, moyamoya, FCA, or SVA had recurrent stroke (risk estimate: 9-25%) vs 5/147 (3%) for all other stroke etiologies (risk estimate: 2-8%).   Five patients had recurrent stroke after one year; one each with moyamoya, progressive heart failure, severe brain malformation, ADA2, and recurrent VZV in a patient with HIV. 

This confirms the Vascular effects of Infection in Pediatric Stroke (VIPS) study, which showed that children with arteriopathy, particularly progressive arteriopathy, are at highest risk of stroke recurrence, and underscores the importance of diagnosing underlying arteriopathies. Patient-specific recurrence risk counseling and antithrombotic treatment should be tailored by stroke pathophysiology and time since stroke.