Dandy-Walker variant: prenatal sonographic features and clinical outcome.
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.
Estroff
J A
JA
Department of Radiology, Children's Hospital, Harvard Medical School, Boston, MA.
Scott
M R
MR
Benacerraf
B R
BR
D000015:Abnormalities, Multiple; D002531:Cerebellum / Q000002:abnormalities / Q000000981:diagnostic imaging; D002657:Child Development; D002675:Child, Preschool; D003616:Dandy-Walker Syndrome / Q000000981:diagnostic imaging* / Q000235:genetics; D004453:Echoencephalography; D005260:Female; D005313:Fetal Death; D005315:Fetal Diseases / Q000000981:diagnostic imaging; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007621:Karyotyping; D011247:Pregnancy; D016216:Ultrasonography, Prenatal