Rhomboencephalosynapsis

Case contributed by A.Prof Frank Gaillard

Presentation

History of birth injury.

Patient Data

Age: 35 years
Gender: Male
MRI

Features of aqueduct stenosis are seen. CSF flow studies confirm markedly decreased flow across the aqueduct. A right frontal burrhole likely relates to a previous ventriculostomy, although a floor of third ventricle defect cannot be appreciated, nor is there convincing flow across the floor on CSF studies. Despite this, notable lack of transependymal edema implies compensated hydrocephalus.

Gross gliosis and encephalomalacia affects both temporal lobes, resulting in ex vacuo dilatation of the temporal horns both lateral ventricles in addition to the aforementioned hydrocephalus likely relates to known 'birth injury'.

There are several small foci of FLAIR hyperintensity in both frontal lobes, involving white matter and to a lesser extent cortex. 

The cerebellar hemispheres are fused across the midline without a well-formed vermis, consistent with rhombencephalosynapsis.

PlayAdd to Share

Case information

rID: 38107
Published: 4th Aug 2018
Last edited: 14th Aug 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.