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Fetal Diagn Ther. 2004 Jul-Aug;19(4):342-7.

Dandy-Walker malformation: a review of 78 cases diagnosed by prenatal sonography.

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  • 1Department of Obstetrics and Gynecology, University of Istanbul, Faculty of Medicine, Istanbul, Turkey. recephas@superonline.com

Abstract

OBJECTIVE:

The purpose of this study was to determine the associated abnormalities and clinical outcomes of fetuses with Dandy-Walker malformations.

METHODS:

Sonograms and medical reports of 78 cases were reviewed and information regarding each outcome was collected from autopsy records, hospital charts and specialists caring for the surviving infants.

RESULTS:

We identified 64 fetuses with classic Dandy-Walker malformation (DW) and 14 fetuses with Dandy-Walker variant (DWV). A high proportion (44.8%) of the parents were consanguineous. The spectrum and proportion of central nervous system (67.1 vs. 71.4%; p = 1.0) and other malformations (43.7 vs. 64.2%; p = 0.57) associated with DW and DWV were similar. Chromosome abnormalities were found in 9 of the 51 (17.6%) fetuses that underwent karyotype analysis. Only 4 of 64 (6.2%) DW and 3 of 14 (21.4%) DWV infants survived (p 0.14), and all surviving infants with DW or DWV had neurological disorders.

CONCLUSIONS:

DW and DWV cases show so many similarities that a clear-cut distinction is difficult. There was no significant difference in the spectrum of associated anomalies and postnatal prognosis between DW and DWV cases.

PMID:
15192294
DOI:
10.1159/000077963
[PubMed - indexed for MEDLINE]
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