Cerebral Proliferative Arteriopathy Secondary to Extracranial Carotid Stenosis

Cerebral proliferative arteriopathy (CPA) is a rare vascular malformation distinct from arteriovenous malformations. Although arterial stenoses in CPA are thought to develop later, we hypothesize some cases may result from preexisting stenosis causing ischemia and vascular proliferation. A 7-year-old boy with a history of biliary atresia and a traumatic jugular line insertion presented with recurrent left hemiparesis. Magnetic resonance imaging and cerebral angiography revealed a pinhole stenosis in the right internal carotid artery and CPA features. This case suggests CPA may be a secondary maladaptive response to chronic ischemia in certain patients.