Cerebral proliferative angiopathy in pediatric patients: case-based review with an illustrative case

To synthesize pediatric presentations, imaging, and management of cerebral proliferative angiopathy (CPA) and to illustrate surgical decision-making with a representative case.

We conducted a systematic search of CINAHL, Cochrane Library, Embase, Ovid MEDLINE, Scopus, and Web of Science from inception to February 2024 per PRISMA guidance, extracting demographics, presentation, lesion distribution, treatment, and outcomes from pediatric cases. We also detail a 2-year-old with hemispheric CPA treated by pial synangiosis plus burr-hole dural inversion.

Twenty-one studies comprising 29 pediatric CPA cases were included. Common symptoms were focal deficits (n = 17), headache (n = 15), and seizures (n = 6). Nidi most frequently involved frontal, temporal, and parietal lobes, with more bilateral and infratentorial involvement than mixed-age cohorts. Fifteen patients received conservative therapy; nine underwent surgery-most commonly indirect revascularization. Our case showed angiographic collateralization over the right motor cortex and absence of new infarction at 2-year follow-up, with functional gains and no recurrent ischemia.

Pediatric CPA often manifests with ischemia and diffuse, eloquent-intermixed vasculature. In symptomatic children with hypoperfusion, indirect revascularization is a reasonable strategy to enhance perfusion and reduce recurrent ischemic events. Aggregated evidence and our illustrative case suggest robust and durable outcomes following pial-based indirect bypass, supporting early multidisciplinary evaluation and individualized surgical consideration.