CEREBELLAR VERMIAN DEFECTS - ANTENATAL SONOGRAPHIC APPEARANCE AND CLINICAL-SIGNIFICANCE

A partial or complete defect of the cerebellar vermis may occur sporad ically or as a component of the Dandy-Walker syndrome, the Dandy-Walke r variant, Down's syndrome, or Joubert-syndrome. We identified a defec t of the cerebellar vermis in nine fetuses on routine antenatal sonogr aphic studies. In five of the nine fetuses other abnormalities, includ ing central nervous system and non-central nervous system lesions were identified. Three of these five fetuses died and two infants have rem ained well on follow-up. Four of nine fetuses (or infants) had no othe r abnormalities and have remained well on follow-up at 8 weeks to 4 ye ars of age. Although cases of fatal and serious nonfatal outcomes have been reported by other investigators, in this small series the findin g of an isolated inferior vermian defect was associated with a good pr ognosis.