Craniocervical Arterial Dissection in Children: Pathophysiology and Management

Abstract

Background: Craniocervical arterial dissection is a commonly reported arteriopathy associated with stroke in children. It is characterized by a high stroke recurrence rate and variable outcomes. Here we review the pathophysiology, clinical presentation, and diagnostic neuroimaging approaches that are helpful in accurate diagnosis and follow-up of children with arterial dissection.

Methods: MEDLINE searches (2000 to 2018) for articles that contained patients aged less than 18 years with craniocervical arterial dissection was performed, with the goal of analyzing their presenting features, pathophysiological mechanisms, and imaging characteristics and interventions.

Results: Sixteen articles met the study criteria and reported 182 cases of craniocervical arterial dissection, 68% male, average age 8.6 years. Dissection was associated with head and neck trauma in 56% of the cases and frequently involved the posterior (61%) and extracranial locations (64%); the vertebral artery was the most commonly involved artery (60%). The most common clinical presentation was hemiparesis (80/160, 50%), followed by headache (64/164, 39%). Magnetic resonance imaging was the preferred neuroimaging method, followed by cerebral catheter angiography as a gold standard definitive neurovascular imaging modality when the initial vascular imaging revealed nondiagnostic findings.

Conclusions: The diagnosis of arterial dissection requires a high index of suspicion and consideration for detailed neurovascular imaging, including both the cranial and cervical regions. Neurovascular imaging challenges, especially visualization of arterial abnormalities, highlight the importance of appropriate and timely use of specific neurovascular imaging techniques. Magnetic resonance imaging appears to be the preferred neurovascular imaging modality in children with arterial dissection and may obviate the need for invasive cerebral catheter angiography.

Keywords: Arterial imaging; Dissection; Pathophysiology; Stroke.