Chiari II malformation

Case contributed by Dr Amr Farouk


1 month old neonate presenting with myelomeningocoele for CNS survey.

Patient Data

Age: 1 month old
Gender: Female
  • Infra-tentorial findings:-
  1. Crowded foramen magnum with the cerebellar tonsils and vermis seen displaced inferiorly.
  2. Small posterior fossa with a low attachment of the tentorium and low torcula.
  3. Pulled down brainstem with an elongated and low lying fourth ventricle.
  4. Tectal plate beaking
  5. Angulation of the aqueduct.
  6. Concavity of clivus
  • Supra-tenorial findings:-
  1. Hydrocephalus with colpocephaly
  2. Thinned out corpus callosum
  3. fenestration of the falx with interdigitated gyri.
  4. Heart shape incisura.
  5. Large massa intermedia of the thalamus

Case Discussion

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

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Case information

rID: 58484
Case created: 19th Feb 2018
Last edited: 19th Feb 2018
System: Paediatrics
Inclusion in quiz mode: Included
Institution: National cancer institute, Cairo, Egypt

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