Systematic review of pediatric cerebral proliferative angiopathy

OBJECTIVE: Cerebral proliferative angiopathy (CPA) is a rare, low-flow, diffuse vascular malformation distinct from a classic cerebral nidal arteriovenous malformation. Its clinical course, imaging features, and optimal management in pediatric patients remain unclear, and existing evidence is limited to small case series. The authors performed a systematic review to characterize the presentation, radiological findings, treatment, and outcomes in children with CPA. METHODS: They performed a systematic search of the MEDLINE, Scopus, and Web of Science databases for English-language studies published between January 2008 and June 2024 and reporting angiographically confirmed CPA in patients with an age ≤ 18 years. Two reviewers independently screened and extracted data on demographics, symptoms, imaging (angiography, perfusion imaging), management (conservative methods, endovascular therapy, cerebral revascularization, resection, radiosurgery), and outcomes. Descriptive statistics were applied for detailed clinical insights into pediatric CPA. RESULTS: The search yielded 557 studies, with 25 papers eligible for study inclusion and a final total of 29 pediatric patients with CPA (median age 11 years, 54% female). Presentations included headaches in 59% of cases (including stroke and transient ischemic attacks), followed by new neurological deficits (55%), hemorrhage (38%), and seizures (28%). Eleven patients (65%) were initially managed conservatively; of these, 5 (45%) later underwent treatment because of lesion progression or clinical deterioration. Forty-eight percent of the patients required intervention (12/25; note that management and follow-up data were available for 25/29 patients), including indirect surgical revascularization (ISR; 5/12), ISR plus radiosurgery (1/12), endovascular therapy (1/12), endovascular therapy plus radiosurgery (1/12), resection (3/12), and microsurgical clipping (1/12). During an overall median 36-month follow-up, 73% (8/11 patients initially managed conservatively) of patients presented with clinical deterioration; ISR led to clinical and perfusion improvement in 66% (4/6) of patients. CONCLUSIONS: Pediatric CPA exhibits a dynamic, progressive course in which conversative management often leads to clinical deterioration. ISR was the most effective treatment for improving ischemic symptoms, highlighting the role of hypoperfusion in CPA. Collaborative prospective studies are needed to determine optimal management strategies for pediatric CPA.