Global Epidemiology of Craniosynostosis: A Systematic Review and Meta-Analysis

OBJECTIVE: Craniosynostosis leads to craniofacial deformity and may result in raised intracranial pressure, neurocognitive deficits, and psychosocial issues if left untreated. The global epidemiology of craniosynostosis is unknown. We conducted a meta-analysis to estimate global birth prevalence. METHODS: PubMed, Embase, and Scopus were searched. Articles were screened by title and abstract and then full text. Meta-analysis of birth prevalence was conducted. Birth prevalence figures were combined with metrics detailing the number of births in 2019 to estimate the number of children worldwide born with craniosynostosis annually. RESULTS: Of 1378 resultant articles, 24 studies were included, including 20 providing data for craniosynostosis overall and 9 for nonsyndromic craniosynostosis. World Health Organization regions of included studies were 9 (37.5%) European Region, 8 (33.3%) Region of the Americas, 4 (16.7%) Western Pacific region, 2 (8.3%) African Region, and 1 (4.2%) Eastern Mediterranean Region. Lower middle-income countries represented only 4% of study manuscripts. The overall birth prevalence of craniosynostosis was 5.9 per 10,000 live births (20 studies; 95% confidence interval [CI]: 3.9, 8.4; I-2 = 100%). The birth prevalence of nonsyndromic craniosynostosis was 5.2 per 10,000 live births (9 studies; 95% CI: 3.4, 7.3; I-2 = 98%). The number of children born globally with craniosynostosis in 2019 was estimated to be 84,665 (95% CI: 55,965, 120,540), including 72,857 (95% CI: 47,637, 120,280) with nonsyndromic craniosynostosis. CONCLUSIONS: Craniosynostosis is a common condition that affects the neurocognitive and craniofacial skeletal development of children worldwide. Initiatives to scale up capacity for craniosynostosis epidemiologic research and clinical care are warranted, particularly in low- and middle-income countries.