Post-varicella arteriopathy of childhood - Natural history of vascular stenosis

Objective: To determine the course of vascular changes in childhood post-varicella arteriopathy (PVA) and its relationship to recurrent arterial ischemic stroke or TIA (AIS/TIA). Methods: Subjects were children with AIS/TIA occurring < 1 year after varicella, ischemic localization consistent with unilateral disease affecting the supraclinoid internal carotid artery or proximal anterior or middle cerebral arteries, and no identified AIS/TIA etiology other than PVA. Charts, brain MRI, and sequential cerebral vessel imaging (selective cerebral angiography or MR angiography [SCA/MRA]) were retrospectively reviewed. Results: Twenty-three children had varicella at age 1.0 to 10.4 years and had single or multiple AIS/TIAs 4 to 47 weeks later. Initial SCA/MRA was performed within 1 month of presentation, and each child had one to five repeat SCA/MRAs during a 4- to 87-month period. There was vascular stenosis in 19 children, maximal on initial studies in 15 of these. Subsequent stenosis regression occurred in 17 children. In 11 of these, one or two additional SCA/MRAs showed further regression as long as 48 months after presentation; there was no restenosis. Eight of 23 children had recurrent AIS/TIA with antithrombotic therapy within 33 weeks of presentation, including 1 of 17 children with documented stenosis regression. Conclusion: Vascular stenosis of childhood post-varicella arteriopathy takes a monophasic course, generally with subsequent stenosis regression and only occasional stenosis progression after arterial ischemic stroke/TIA. Arterial ischemic stroke/TIA rarely recurs with antithrombotic prophylaxis after stenosis regression occurs.