Van der Veeken Lennart, Russo Francesca M, Litwinska Ewelina, Gomez Olga, Emam Doaa, Lewi Liesbeth, Basurto David, Van der Veeken Stephan, De Catte Luc, Gratacos Eduard, Eixarch Elisenda, Nicolaides Kypros, Deprest Jan
Department of Development and Regeneration, Cluster Woman and Child, Group Biomedical Sciences, KU Leuven University of Leuven, Leuven, Belgium.
Clinical Department Obstetrics and Gynaecology, University Hospitals Leuven, Leuven, Belgium.
Prenat Diagn. 2022 Mar;42(3):330-337. doi: 10.1002/pd.5993. Epub 2021 Jul 10.
Children with congenital diaphragmatic hernia (CDH) are at risk for neurodevelopmental delay. Herein we report on prenatal changes in biometry and brain perfusion in fetuses with isolated CDH.
This retrospective study evaluated fetuses with isolated, left-sided CDH in three European referral centers. Abdominal circumference (AC), femur length (FL), head circumference (HC), transcerebellar diameter (TCD), middle cerebral artery (MCA) Doppler, and ventricular width (VW) were assessed during four gestational periods (<24 weeks, 25-28 weeks, 29-32 weeks, >33 weeks). Z-scores were calculated, and growth curves were created based on longitudinal data.
In 367 fetuses, HC, AC and FL were within normal ranges throughout gestation. The TCD diminished with advancing gestational age to fall below the fifth percentile after 32 weeks. A less pronounced but similar trend was seen in VW. The peak systolic velocity of the MCA was consistently approximately 10% lower than normal. Disease severity was correlated to TCD (p = 0.002) and MCA doppler values (p = 0.002). There were no differences between fetuses treated with FETO and those managed expectantly.
Fetuses with isolated left-sided CDH have a small cerebellum and reduced MCA peak systolic velocity. Follow up studies are necessary to determine the impact of these changes on neurodevelopment.
先天性膈疝（CDH）患儿有神经发育迟缓的风险。在此，我们报告孤立性CDH胎儿的生物测量和脑灌注的产前变化。
这项回顾性研究评估了三个欧洲转诊中心的孤立性左侧CDH胎儿。在四个孕期（<24周、25 - 28周、29 - 32周、>33周）评估腹围（AC）、股骨长度（FL）、头围（HC）、小脑横径（TCD）、大脑中动脉（MCA）多普勒和脑室宽度（VW）。计算Z分数，并根据纵向数据绘制生长曲线。
在367例胎儿中，HC、AC和FL在整个孕期均在正常范围内。TCD随着孕周增加而减小，在32周后降至第五百分位数以下。VW也有不太明显但相似的趋势。MCA的收缩期峰值速度始终比正常低约10%。疾病严重程度与TCD（p = 0.002）和MCA多普勒值（p = 0.002）相关。接受胎儿镜胎儿手术（FETO）治疗的胎儿与期待治疗的胎儿之间没有差异。
孤立性左侧CDH胎儿小脑较小，MCA收缩期峰值速度降低。需要进行后续研究以确定这些变化对神经发育的影响。
