ABSTRACT

Stroke and cerebrovascular disease (CVD) in childhood is the subject of rapidly growing awareness and research in the past decade. Advances in availability and technical quality of noninvasive brain and vascular imaging with magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA) have greatly expanded the capacity to detect and characterize CVD in infants and children. Concurrent rapid growth in the techniques and discoveries of molecular biology and genetics provide great opportunities to understand the basic molecular and cellular biology of numerous inherited and malformative vascular disorders affecting the developing nervous system. Neurointerventionalists are increasingly being asked to participate in the diagnosis, and sometimes the treatment, of children and adults with uncommon congenital and inherited forms of CVD. This chapter provides an overview of inherited and congenital cerebrovascular disorders for which expertise of an interventional neuroradiologist may be requested for comprehensive diagnosis or treatment. Each disorder is briefly summarized with illustrative images. In most conditions, treatment includes supportive and medical measures that are common across the age spectrum and among different diseases. Acquired cerebrovascular disorders in children and young adults such as thromboembolic disease, focal cerebral arteriopathy, vasculitis, and dissection comprise a large proportion of the spectrum of childhood-onset CVD, and are beyond the scope of this chapter. Excellent comprehensive reviews of the general approach to diagnosis and treatment of these disorders are available elsewhere (1-3).