Case contributed by A.Prof Frank Gaillard

MRI of this child demonstrates dysmorphic lateral ventricles with absent septum pellucidum. Abnormal bridging grey matter is shown between the hemispheres along the level of the body of the corpus callosum with resultant thinning and irregular shape of the body of corpus callosum. The genu and splenium of the corpus callosum are relatively enlarged. 

The majority of the falx appears deficient, particularly anteriorly and the anterior cerebral artery is azygous. The pituitary, optic chiasm and hypothalamus appear normal.

Features in keeping with the middle interhemispheric variant of holoprosencephaly, also known as syntelencephaly

Case Discussion

This case demonstrates features of syntelencephaly, a rare malformation along the holoprosencephaly spectrum. 

PlayAdd to Share

Case information

rID: 6690
Published: 5th Aug 2009
Last edited: 29th Oct 2019
Inclusion in quiz mode: Included

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.