VOL.25 NO.2 2009

Case Reports

A case of rhombencephalosynapsis ‐fetal MRI

Shigeko Kuwashima
Department of Radiology, Dokkyo Medical University

Abstract

  Rhombencephalosynapsis(RES)is a rare cerebellar malformation of unknown etiology characterized by vermian agenesis or hypogenesis, with fusion of hemispheres and the dentate nuclei. Clinical presentation and prognosis are extremely variable and generally depend on the associated supratentorial anomalies. In this case, the prenatal ultrasound revealed hydrocephalus. We diagnosed this case as RES by MRI that demonstrated fusion of cerebellar hemispheres and horizontal folia orientation without an intervening vermis. Prenatal identification of RES is likely to prove useful for prenatal counseling.

Keywords:Rhombencephalosynapsis, MRI, Fetus

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