FCY Lam, PD Barnes

Abstract

Rhombencephalosynapsis is a rare cerebellar malformation. It is characterised by dorsal fusion of the cerebellar hemispheres, agenesis or hypogenesis of the vermis, and fusion of the dentate nuclei. A wide variety of associated abnormalities has been reported. To our knowledge, there are less than 50 cases described in the literature so far. However, due to the advent of MRI, this condition has been increasingly reported in recent years. The exact aetiology and associated factors are still not known, therefore, any new case is worth reporting. We describe a case of Rhombencephalosynapsis in a new born and review of the literature.