Biometry of face and brain in fetuses with trisomy 21
Biometry of face and brain in fetuses with trisomy 21
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DOI：
10.1203/01.pdr.0000190580.88391.9a
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发表时间：
2006-01-01
影响因子：
3.6
通讯作者：
Delezoide, AL
中科院分区：
文献类型：
--
作者：
Guihard-Costa, AM;Khung, S;Delezoide, AL
本研究的目的是指定的特定颅面形态唐氏综合征在胎儿期的尸检检查的基础上的数据的早期设置。该研究包括1277例15-38孕周（GW）的胎儿：922例对照胎儿和355例21三体胎儿，选自巴黎的胎儿病理学单位。记录体重（BW）和面部、颅骨和脑的9个维度：外眦和内眦距离（OCD、ICD）、双顶径（BPD）、头围（HC）、脑重量（BrW）、左右半球枕横径（IOFD、rOFD）、脑幕下部分重量（IBW）和小脑最大横径（CTD）。计算四个比值：BPD/HC、OCD/BPD、BrW/BW、IBW/BrW。通过年龄间隔检验三体胎儿和对照胎儿之间的差异。结果表明，早在15 GW的三体胎儿的BW，rOFD和IOFD较低。小脑发育不全包括三体胎儿的IBW和CTD较低。三体胎儿的IBW/BrW比值较高，表明生长受限对大脑幕下部分的影响小于幕上部分。早期短头畸形见于三体胎儿。BPD和BPD/HC值从15 GW开始升高。ICD和OCD在两组间无显著性差异，但OCD/DBP比值在三体胎儿中较低。这些结果证实了颅面形态学上21三体的早期表型表达，与脑生长的显著限制相关，特别是在幕上部分。
The aim of this study was to specify the early setting of the particular craniofacial morphology in Down syndrome during the fetal period from data based on postmortem examinations. The study included 1277 fetuses at 15-38 gestational weeks (GW): 922 control fetuses and 355 fetuses with trisomy 21, selected from fetopathology units in Paris. Body weight (BW) and nine dimensions of the face, skull, and brain were recorded: the outer and inner canthal distances (OCD, ICD), biparietal diameter (BPD), head circumference (HC), brain weight (BrW), occipitofirontal diameters of left and right hemispheres (IOFD, rOFD), weight of the infratentorial part of the brain (IBW), and maximal transversal diameter of the cerebellum (CTD). Four ratios were computed: BPD/HC, OCD/BPD, BrW/BW, IBW/BrW. Differences between trisomic fetuses and control fetuses were tested by age interval. Results showed that BW, rOFD, and IOFD were lower in trisomic fetuses as early as 15 GW. Cerebellar hypoplasia included lower IBW and CTD in trisomic fetuses. The IBW/BrW ratio was higher in trisomic fetuses, showing that growth restriction affected the infratentorial part of the brain less than the supratentorial part. Early brachycephaly was found in trisomic fetuses. with higher values of BPD and BPD/HC from 15 GW. ICD and OCD were not significantly different in the two groups, but OCD/DBP ratio was lower in trisomic fetuses. These results confirm the early phenotypical expression of trisomy 21 on craniofacial morphology, associated with a marked restriction of brain growth, especially in the supratentorial part.