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Anencephaly :
Defective closure of the anterior neuropore: defect in skull , meninges
and scalp with rudimentary brain withor without congental heart disease
or polyhydramnios
Management : Symptomatic
Encephhalocele :
Protrusion of cortex and meninges through a midline defect in the skull
covered by skin
Usually occipital but may be frontal
Diagnosed by brain MRI
Management : immediate surgery .
-Defective prosecephalization :
Agenesis of corpus callosum :
Solitary agenesis: usually clinically unapparent
Mental retardation and learning disabilities occur in most of cases
Diagnosed by brain CT ,MRI
Management : Symptomatic
Holoprosencephaly :
Defect in cleavage of the embryonic forebrain → spectrum of
malformations .
Single ventricle , absent falx cerebri and fused basal ganglia
May be associated with craniofacial dysplasia , facial deformities and
their severity is often predictive of the severity of brain malformation
Diagnosed by brain MRI
Management : measures to extend life are inappropriate