Iniencephaly Apertus - A Rare Neural Tube Defect

Iniencephaly is a rare congenital malformation of the CNS, affecting the nape of the neck, occipital bone and cervical vertebrae.Sometimes it is associated with encephalocoele and in such cases it is called as iniencephaly apertus.We are reporting a case of iniencephaly apertus seen in our institution.It is caused by defective closure of the neural tube at multiple sites.Closure of neural tube requires adequate formation of paraxial mesoderm in the early embryonic stage.Primary paraxial mesodermal insufficiency results in different types of neural tube defects.