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Dandy-Walker variant: prenatal sonographic features and clinical outcome
J A Estroff1, M R Scott, B R Benacerraf
1Department of Radiology, Children's Hospital, Harvard Medical School, Boston, MA.
Radiology
|December 1, 1992
Summary
Dandy-Walker variant, a posterior fossa anomaly, presents varied outcomes. Prenatal diagnosis aids in genetic testing and planning for infant care, as prognosis is uncertain.
Area of Science:
- Medical Science
- Fetal Medicine
- Pediatric Neurology
Background:
- Dandy-Walker variant is a posterior fossa malformation.
- It is considered less severe than classic Dandy-Walker malformation.
- Prenatal diagnosis and associated anomalies require further investigation.
Purpose of the Study:
- To evaluate associated defects, karyotypic anomalies, and outcomes in fetuses with Dandy-Walker variant.
- To assess the prognostic implications of Dandy-Walker variant.
- To highlight the importance of prenatal recognition.
Main Methods:
- Retrospective analysis of 17 consecutive fetuses diagnosed with Dandy-Walker variant via sonography.
- Evaluation of associated central nervous system (CNS) and non-CNS anomalies.
- Review of karyotypic findings and postnatal outcomes.
Main Results:
- 24% of fetuses had mild ventriculomegaly.
- 47% had concurrent non-CNS anomalies.
- 29% had abnormal karyotypes, including trisomies 18, 13, 21, and 11q+.
- 35% of fetuses experienced in utero or neonatal demise.
- 9 infants developed normally, with 53% lacking non-CNS findings.
Conclusions:
- Prognosis for Dandy-Walker variant is uncertain.
- Prenatal diagnosis allows for fetal karyotyping.
- Early recognition facilitates planning for postnatal care and follow-up.