Cabaret A S, Loget P, Loeuillet L, Odent S, Poulain P
Department of obstetrics and gynecology, University Hospital, Rennes, France.
Prenat Diagn. 2007 Aug;27(8):738-42. doi: 10.1002/pd.1771.
To get information about embryologic mechanisms of neural tube defects (NTD), by studying the associated malformations.
Eighty three cases of NTD, seen at the prenatal diagnosis unit of Rennes University Hospital (France) between May 1999 and December 2002, were retrospectively studied. Cases with chromosomal anomalies (5/83), cases without available karyotype or pathologic examination were excluded. 24 spina bifida, and 27 cephalic forms (anencephalies, exencephalies, and encephaloceles) were thus analyzed.
Only 22/51 cases (43%) were strictly isolated NTD. Anomalies of tissues arising from neural crests were noted in 8/51 fetuses (16%), midline or lateralization anomalies in 12/51 (24%), and anomalies of mesoblastic tissues in 17/51 (33%). An already known syndrome was found in 4/51 cases (8%).
NDT are more extensive congenital damages that would suggest the restrictive terminology. That prompts to assess cautiously prenatal diagnosis of NTD, and to get detailed pathological examination after termination of pregnancy.
通过研究相关畸形来获取有关神经管缺陷(NTD)胚胎学机制的信息。
回顾性研究了1999年5月至2002年12月期间在法国雷恩大学医院产前诊断科就诊的83例NTD病例。排除染色体异常病例(5/83)以及无可用核型或病理检查的病例。对24例脊柱裂和27例头部畸形(无脑儿、露脑畸形和脑膨出)进行了分析。
51例病例中仅22例(43%)为严格意义上的孤立性NTD。51例胎儿中有8例(16%)出现神经嵴来源组织的异常,12例(24%)出现中线或侧化异常,17例(33%)出现中胚层组织异常。51例病例中有4例(8%)发现了已知综合征。
NTD是更广泛的先天性损害,这表明现有术语具有局限性。这促使谨慎评估NTD的产前诊断,并在终止妊娠后进行详细的病理检查。