Chiari II malformation

Case contributed by Amr Farouk
Diagnosis certain


Neonate presenting with myelomeningocele for CNS survey.

Patient Data

Age: 1 month
Gender: Female

Infra-tentorial findings:

  • Crowded foramen magnum with the cerebellar tonsils and vermis seen displaced inferiorly.
  • Small posterior fossa with a low attachment of the tentorium and low torcula.
  • Pulled down brainstem with an elongated and low lying fourth ventricle.
  • Tectal plate beaking.
  • Angulation of the aqueduct.
  • Concavity of clivus

Supratentorial findings:

  • Hydrocephalus with colpocephaly.
  • Thinned out corpus callosum.
  • fenestration of the falx with interdigitated gyri.
  • Heart shape incisura.
  • Large massa intermedia of the thalamus.

Case Discussion

The constellation of the supra and infra-tentorial CNS findings in addition to the myelomeningocele are of a Chiari II malformation.

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