Arteriopathies, defined as intrinsic vessel stenosis, irregularity, pseudoaneurysm, banding, or dissection flap, account for 18% to 64% of pediatric ischemic stroke cases [36]. Common causes of arteriopathy include moyamoya (22%), arterial dissection (15% to 20%), vasculitis (12%), and sickle cell disease (SCD) arteriopathy (8%) [27,36]. Genetic mutation constitutes an increasingly represented etiology of cerebral arteriopathy as demonstrated by mutations in the ACTA2 or CERC 1 genes. Arteriopathies are a strong indicator of recurrent stroke risk (66%) [36-38]. Moyamoya refers to the angiographic appearance of a progressive stenosis or occlusion of the internal carotid artery apex and proximal branches of the circle of Willis with the development of stereotypical collaterals. Moyamoya occurs in approximately one in every 1 million children in the United States and accounts for 6% of all pediatric strokes [39]. The underlying cause is unclear, but it is likely that many different factors, both genetic and environmental, contribute to developing the arteriopathy. The moyamoya syndrome may be idiopathic (moyamoya disease) or occur in association with other conditions (moyamoya syndrome). Up to 40% of children with SCD may show moyamoya-like changes on imaging [39]. Moyamoya disease is, by definition, bilateral but may be asymmetric in severity [39]. Unilateral involvement is considered moyamoya syndrome. As many as 12% of patients with sickle cell anemia will have a clinically detected stroke by the age of 20 [40,41]. Importantly, at a minimum, 85% of patients with sickle cell anemia who present with frank stroke will have evidence of cerebral arteriopathy on neuroimaging [41,42]. Most strokes in moyamoya in children are ischemic and most frequently occur in the vascular border zone territories, but cortical infarctions can occur as well.
Cerebrovascular Disease Child PCAs. Arteriopathies, defined as intrinsic vessel stenosis, irregularity, pseudoaneurysm, banding, or dissection flap, account for 18% to 64% of pediatric ischemic stroke cases [36]. Common causes of arteriopathy include moyamoya (22%), arterial dissection (15% to 20%), vasculitis (12%), and sickle cell disease (SCD) arteriopathy (8%) [27,36]. Genetic mutation constitutes an increasingly represented etiology of cerebral arteriopathy as demonstrated by mutations in the ACTA2 or CERC 1 genes. Arteriopathies are a strong indicator of recurrent stroke risk (66%) [36-38]. Moyamoya refers to the angiographic appearance of a progressive stenosis or occlusion of the internal carotid artery apex and proximal branches of the circle of Willis with the development of stereotypical collaterals. Moyamoya occurs in approximately one in every 1 million children in the United States and accounts for 6% of all pediatric strokes [39]. The underlying cause is unclear, but it is likely that many different factors, both genetic and environmental, contribute to developing the arteriopathy. The moyamoya syndrome may be idiopathic (moyamoya disease) or occur in association with other conditions (moyamoya syndrome). Up to 40% of children with SCD may show moyamoya-like changes on imaging [39]. Moyamoya disease is, by definition, bilateral but may be asymmetric in severity [39]. Unilateral involvement is considered moyamoya syndrome. As many as 12% of patients with sickle cell anemia will have a clinically detected stroke by the age of 20 [40,41]. Importantly, at a minimum, 85% of patients with sickle cell anemia who present with frank stroke will have evidence of cerebral arteriopathy on neuroimaging [41,42]. Most strokes in moyamoya in children are ischemic and most frequently occur in the vascular border zone territories, but cortical infarctions can occur as well.