Global prevalence of congenital anencephaly: a comprehensive systematic review and meta-analysis

Plain Language summary Anencephaly is a fatal congenital anomaly characterized by the absence of brain hemispheres and cranial arch. Cochran’s seven-step instructions were used as the guideline. Having determined the research question and inclusion and exclusion criteria, we studied MagIran, SID, Science Direct, WoS, Web of Science, Medline (PubMed), Scopus, and Google Scholar databases. Moreover, the search strategy in each database included using all possible keyword combinations with the help of “AND” and “OR” operators with no time limit to 2021. Out of 1141 initial articles found, and after excluding repetitive ones in various databases and those irrelevant to inclusion criteria, 360 studies with a sample size of 207,639,132 people were considered for the meta-analysis. Overall estimate of the prevalence, incidence and attenuation of anencephaly worldwide were 5.1 per ten thousand births (95% confidence interval 4.7–5.5 per ten thousand births), 8.3 per ten thousand births (95% confidence interval 5.5–9.9 per ten thousand births), 5.5 per ten thousand births (95% confidence interval 1.8–15 per ten thousand births) respectively the highest of which according to the subgroup analysis, belonged to the Australian continent with 8.6 per ten thousand births (95% confidence interval 7.7–9.5 per ten thousand births). The overall prevalence of anencephaly in the world is significant, indicating the urgent need for preventive and treating measures.