Cerebellar vermian defects: antenatal sonographic appearance and clinical significance.

Abstract

A partial or complete defect of the cerebellar vermis may occur sporadically or as a component of the Dandy-Walker syndrome, the Dandy-Walker variant, Down's syndrome, or Joubert-syndrome. We identified a defect of the cerebellar vermis in nine fetuses on routine antenatal sonographic studies. In five of the nine fetuses other abnormalities, including central nervous system and non-central nervous system lesions were identified. Three of these five fetuses died and two infants have remained well on follow-up. Four of nine fetuses (or infants) had no other abnormalities and have remained well on follow-up at 8 weeks to 4 years of age. Although cases of fatal and serious nonfatal outcomes have been reported by other investigators, in this small series the finding of an isolated inferior vermian defect was associated with a good prognosis.

Dandy-walker Variant In A Fetus Of An Icsi Twin Gestation: A Case Report

“…Isolated inferior vermian defect was reported to be associated with a good prognosis (2). Obstetric management and also the neonatal outcome depends on the gestational age at diagnosis, karyotype analysis and associated intracranial or extracranial abnormalities.…”

“…A partial or complete cerebellar vermian defect may occur sporadically or as a component of the Dandy-Walker syndrome (DWS), Down syndrome or Joubert Syndrome (2). The Dandy-Walker malformation (DWM), Dandy-Walker variant (DWV), and mega cisterna magna (MCM) seem to represent steps on a continuum of developmental anomalies of the posterior fossa (3).…”

Neurodevelopmental outcome after antenatal diagnosis of posterior fossa abnormalities.

“…In contrast to DWM and its variant, arachnoid cyst is generally associated with favorable outcome after decompression of the cyst by cystoperitoneal shunting or fenestration. [14][15][16][17][18][19][20][21][22] Isolated MCM has been associated with normal pregnancy and neonatal outcome. 23 One recent study has shown prenatal ultrasonographic diagnosis of central nervous system anomalies to have a high concordance with the autopsy diagnosis (89%).…”

Signs on Obstetric Ultrasound Images in the Second Trimester

“…The absence of the lower lobe of the vermis creates the "keyhole" observed ultrasonographically which is very useful in DWV diagnosis (Fig. 4) [12,13].…”