医学
胎儿
尸检
儿科
丹迪-沃克综合征
产前诊断
核型
产科
怀孕
外科
病理
脑积水
染色体
遗传学
生物
生物化学
化学
基因
作者
Recep Has,Hayri Ermiş,Atıl Yüksel,Lemi Ibrahimoglu,Alkan Yildirim,Hande Delier Sezer,Seher Başaran
出处
期刊:Fetal Diagnosis and Therapy
[S. Karger AG]
日期:2004-07-01
卷期号:19 (4): 342-347
被引量:44
摘要
<i>Objective:</i> The purpose of this study was to determine the associated abnormalities and clinical outcomes of fetuses with Dandy-Walker malformations. <i>Methods:</i> 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. <i>Results:</i> 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. <i>Conclusions:</i> 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.
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