Dandy-Walker variant: prenatal sonographic features and clinical outcome

Radiology. 1992 Dec;185(3):755-8. doi: 10.1148/radiology.185.3.1438757.


The Dandy-Walker variant is a less severe posterior fossa anomaly than the classic Dandy-Walker malformation. In 17 consecutive fetuses, the Dandy-Walker variant was diagnosed at sonography, and associated defects, karyotypic anomalies, and outcomes were evaluated. Four of the 17 fetuses (24%) had mild ventriculomegaly. Eight of the 17 (47%) had concurrent non-central nervous system (CNS) anomalies. Five fetuses (29%) had an abnormal karyotype (two with trisomy 18, one each with trisomy 13, 21, and 11q+) and associated sonographic anomalies. Six of the 17 fetuses (35%) died in utero or during the neonatal period, two are severely handicapped, and the other nine are developing normally at ages 4 months to 4 years. Six of the nine normally developing infants (53%) lacked non-CNS sonographic findings. Because the prognosis is uncertain for an infant born with the prenatal diagnosis of Dandy-Walker variant, prenatal recognition of the anomaly allows for the option of fetal karyotyping and for arrangement for postnatal follow-up.

MeSH terms

  • Abnormalities, Multiple
  • Cerebellum / abnormalities
  • Cerebellum / diagnostic imaging
  • Child Development
  • Child, Preschool
  • Dandy-Walker Syndrome / diagnostic imaging*
  • Dandy-Walker Syndrome / genetics
  • Echoencephalography*
  • Female
  • Fetal Death
  • Fetal Diseases / diagnostic imaging
  • Humans
  • Infant
  • Infant, Newborn
  • Karyotyping
  • Pregnancy
  • Ultrasonography, Prenatal*