Management, and outcomes of pediatric cerebral cavernous malformations across age groups: A systematic review and meta-analysis of the literature

Objective

Pediatric Cerebral Cavernous Malformations (CCM) are focal neuro-vascular lesions with highly variable symptomatic presentations and natural history. In this study, we explored clinical features, management decisions, and outcomes in specific pediatric CCM populations.

Methods

In accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, an exhaustive review of the literature was conducted using search strings on PubMed, SCOPUS, Google Scholar, Ovid Embase, and Cochrane databases to assess differences in patient demographics, treatment strategies and clinical outcomes specifically between National Institute of Health (NIH) and American Academic of Pediatrics (AAP)-defined age groups: infants (0–1 year), children (1–12 years), adolescents (12–17 years).

Results

Data from 630 individual pediatric cases from 94 publications was analyzed, yielding 45 infants (median: 0.3 years), 280 children (median: 7.0 years), and 305 adolescents (median: 15 years). Comparatively, infants were more likely to present with signs of elevated ICP and isolated lesions than both the children and adolescents’ group (p < 0.05). Infants presenting with multiple lesions had higher rates of conservative management than surgery (46.2 % vs. 24.30 %, p < 0.01). Children with isolated sensory deficits or asymptomatic at presentation were more likely to undergo conservative management (14.3 % vs. 3.70 %, p < 0.05; 20.0 % vs. 0 %, p < 0.001), while those who underwent surgery had higher rates of improvement (81.6 % vs. 51.4 %, p < 0.001) and were more often symptom-free at follow-up (48.2 % vs. 25.7 %, p < 0.05). In adolescents, seizures (47.1 % vs. 14.8 %, p < 0.01), supratentorial lesions (64.6.0 % vs. 45.2 %, p < 0.01), and isolated lesions (75.7 % vs. 53.8 %, p < 0.05) favored surgical management. With increasing age, headaches were more likely (OR:1.05, 95 % CI:1.01–1.10, p < 0.05), whereas motor deficits (OR:0.96, 95 % CI:0.93–0.99, p < 0.05) and signs of elevated intracranial pressure (OR:0.91, 95 % CI:0.87–0.95, p < 0.001) were less likely initial presentations. Furthermore, older patients were more likely to harbor multiple malformations (OR:1.04, 95 % CI:1.01–1.08, p < 0.05). Regardless of management strategy, there was no difference in long-term outcomes between age groups (p > 0.05).

Conclusion

Although presenting symptoms, specific lesion characteristics and symptoms varied with the age of presentation in pediatric CCM patients, there was no significant difference noted in outcomes or long-term follow-up between age groups.