Nizard Jacky, Bernard Jean-Pierre, Ville Yves
Fetal Medicine Unit, Paris-Ouest University, CHI, Poissy, France.
Fetal Diagn Ther. 2005 Mar-Apr;20(2):146-51. doi: 10.1159/000082440.
Diagnosis of abnormalities of the posterior fossa, such as Dandy-Walker malformation, can be assessed during the first trimester of pregnancy by ultrasonography. We report on 5 cases of posterior fossa abnormalities, 4 Dandy-Walker malformations and 1 Dandy-Walker variant, diagnosed during the first trimester of pregnancy by ultrasound examination. All cases were confirmed later during the pregnancy by further ultrasound examinations or by postmortem examination when parents elected for termination of pregnancy. Two of our Dandy-Walker malformation cases were siblings from consanguineous parents and had a Meckel syndrome variant associated with the posterior fossa malformation, multicystic kidneys and hepatic fibrosis. We believe first trimester diagnosis of Dandy-Walker complex is possible, but needs to be confirmed later during the pregnancy and should prompt a detailed survey for other abnormalities.
后颅窝异常,如丹迪-沃克畸形,在妊娠早期可通过超声检查进行评估。我们报告了5例妊娠早期经超声检查诊断为后颅窝异常的病例,其中4例为丹迪-沃克畸形,1例为丹迪-沃克变异型。所有病例在妊娠后期均通过进一步超声检查或在父母选择终止妊娠时经尸检得以确诊。我们的2例丹迪-沃克畸形病例是近亲结婚父母的子女,伴有与后颅窝畸形、多囊肾和肝纤维化相关的梅克尔综合征变异型。我们认为妊娠早期诊断丹迪-沃克综合征是可能的,但需要在妊娠后期予以确认,并且应促使对其他异常情况进行详细检查。