Introduction: Among all the congenital anomalies, disorders of the CNS are the most severe, difficult to understand its etiology, and predict its clinical presentation and course. 75% of fetal deaths and 40% of deaths within the first year of life are secondary to CNS malformations. Aims & Objectives: To study the incidence, pattern and outcome of CNS anomalies in newborns. To established the possible etiological factors and relationship of various antenatal factors. Materials & Methods: All babies were examined within 24hrs of birth & followed up to 72hrs, any anomaly detected confirmed by required investigations. Study Period and design: January 2012 to June 2013. Cross-sectional study (prevalence study). Statistical analysis: Analyzed by simple statistical techniques and tests of significance including Chi-square tests were applied. Results: There were total of 10294 newborn of which CNS malformations were 151 patients with 188 anomalies(1.46%). Most frequent CNS malformations was anencephaly 55/188 (29.25%) followed by Hydrocephalus 51/188 (27.13%), spina bifida (19.15%), Meningocele/meningomyelocele (12.77%), encephalocele (3.19%), agenesis of corpus callosum (3.72%). Male were most commonly involved than female (84% v/s 67%). 107/151 (70%) mother did not have preconceptional and antenatal folic acid supplementation. 15/151 (30%) mother had antenatal history of anaemia.  Incidence was more in maternal age > 30 years and multiparity. Incidence of congenital anomalies was more in preterm (41.8/1000) as compared to full term (10/ 1000). 50 newborns (33.11%) expired within few hrs. Conclusion: Maternal age and parity are important risk factor andpreterm and low birth weight babies are at high risk of CNS Malformations.

Keywords: Congenital central nervous system; Malformations; Newborn; Prevention; Prenatal diagnosis.