Presentation
Myelomeningocele post operative management + ventriculoperitoneal shunt
Patient Data
Hypoplasia of the corpus callosum and agenesis of the splenium, hypoplasia of the trigone with major involvement on left side, absence of septum pellicidum, posterior and inferior displacement of tectum, aqueductal stenosis, tonsillar herniation, cervicomedullary kink, stenogyria, leukoaraiosis in the white matter of the semioval centers , cortico-subcortical atrophy of the medial convolutions of the frontal lobe bilaterally.
Myelomeningocele, tethered medulla and syringomyelia
Case Discussion
The Chiari II malformation is a complex malformation involving the hindbrain, spine, and mesoderm of the skull base and spinal column. Although there are different theories (and none explain all the features), it involves craniospinal pressure differential, with lower spinal pressures due to loss of CSF through the open spinal defect.
Fetal repair results in near normalization of the hindbrain and bony posterior fossa. If the closure of the myelomeningocele is postnatal (usually within the first 48 hours of life), they are likely to develop hydrocephalus.
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