Rhomboencephalosynapsis

Case contributed by Dr Tamer O. Abdo

Presentation

Hypoactive infant

Patient Data

Age: 4 months
Gender: Male
MRI

Brain

Absent cerebellar vermis with the fusion of the cerebellar hemispheres.

Absent septum pellucidum. The optic chiasm appears intact.

Mild dilatation of the lateral and third ventricles. The fourth ventricle is normal in caliber. 

Marked uniform thinning of the corpus callosum.

Case Discussion

Rhomboencephalosynapsis is a very rare congenital anomaly in which the cerebellar vermis fails to develop properly leading to the fusion of the cerebellar hemispheres.

It may occur as an isolated anomaly or associated with other malformations, as in this case.

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Case information

rID: 80183
Published: 16th Jul 2020
Last edited: 9th Dec 2020
Inclusion in quiz mode: Included
Institution: Ibn Al Haitham hospital

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