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The congenital malformations of the central nervous system is one of the leading causes of perinatal mortality in this region of the country. It may present as an isolated defect or may be associated with other organ system malformations. Aims and Objectives: Aim of the present study was to find out the pattern of congenital malformations in the central nervous system both in live and still born perinates. Our main objective was to ascertain its association with other organ systems most commonly involved. Materials and Methods: The prospective study was carried out on 76 perinates having congenital anomalies out of 15,970 births (15,614 live born and 356 stillborn) ranging from 28th weeks of gestation to 7 days after birth. Twenty, out of 76 were born with central nervous system malformations. Results and Observations: The congenital malformations of central nervous system were found to be 26.31%. Anencephaly was the most common malformation (50%) observed amongst the central nervous system in the study with a female preponderance 1.5:1, followed by spina bifida with meningomyelocele (35%), hydrocephalus (10%) and holoprosencephaly (5%). Anencephaly was associated with occipital meningoencephalocele in 10% cases. Conclusion: Anencephaly is the most common malformations in the study, followed by spina bifida and meningomyelocele. The other malformations found in the central nervous system are hydrocephalus, meningoencephalocele and holoprosencephaly. The incidence of congenital malformations in present study is comparatively lower than in other parts of India and abroad.

Pattern of malformations in central nervous system and its association with other congenital anomalies in perinates