Syntelencephaly

Case contributed by A.Prof Frank Gaillard
MRI

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. 

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Case information

rID: 6690
Case created: 5th Aug 2009
Last edited: 6th Apr 2017
Inclusion in quiz mode: Included

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