Presentation
Hypoactive infant
Patient Data
Age: 4 months
Gender: Male
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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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