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In Colombia, congenital anomalies are the second leading cause of death in children aged less than 1 year, and central nervous system (CNS) anomalies are the second most common cause within this group. The aim of the study is to determine the frequency of perinatal mortality attributable to CNS anomalies in Colombia between 2005 and 2014. Using data from the Integral Information System of Social Protection (SISPRO), we determined the perinatal mortality rate associated with CNS anomalies; we also determined frequency of mortality according to age, type of abnormality, year of presentation, and georeferencing. A total of 4706 deaths were recorded to be primarily caused by CNS anomalies (anencephaly and hydrocephalus) in departments and prominent urban centers. The perinatal mortality rate associated with CNS defects has remained relatively constant over the past several years. Major referral centers in the country registered the highest mortality rates. The impact of CNS defects increased with the decrease in infant mortality rates and other health issues. Reinforcement of primary and secondary prevention strategies is warranted for reducing its incidence.

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