evoked potentials in patients with Cleland-Chiari malformation may show
a conduction block in the upper cervical region or a delayed conduction
between the upper cervical region and the cerebral cortex. If hydrocephalus
is not present, the treatment of choice is posterior fossa decompression.
If hydrocephalus is present, the initial treatment of choice is shunting
followed by posterior fossa decompression if apnea reoccurs. Myelomeningocele
should be treated shortly after birth. More
Joubert syndrome presents in
the neonatal period with hyperpnea alternating with apnea. Abnormal eye
movements may be present. These neonates also have flaccid hypotonia.
The diagnosis is established by MRI. The MRI shows complete or partial
absence of the cerebellar vermis and a normal or small posterior fossa.
malformation in the neonatal period usually presents with macrocephaly.
The head is particularly enlarged in the occipital area. Apnea is an infrequent
presentation of Dandy-Walker syndrome in the neonatal period.