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产前影像学诊断原始脑导水管梗阻的多种表现形式。

The many faces of prenatal imaging diagnosis of primitive aqueduct obstruction.

机构信息

Centre Pluridisciplinaire de Diagnostic Prénatal, Hôpital Femme Mère Enfant, Lyon, France.

Imagerie Pédiatrique et Fœtale, Hôpital Femme Mère Enfant, Lyon, France.

出版信息

Eur J Paediatr Neurol. 2018 Nov;22(6):910-918. doi: 10.1016/j.ejpn.2018.07.015. Epub 2018 Aug 10.

Abstract

OBJECTIVE

To describe the different prenatal imaging patterns related to primary aqueduct obstruction throughout pregnancy and their impact on prenatal counseling.

METHOD

Retrospective review of consecutive prenatal cases of isolated aqueduct obstruction diagnosed over a seven-year period (2010-2016). Prenatal imaging findings, postnatal imaging, pathological data and postnatal outcome, were analyzed.

RESULTS

Twelve cases were included. In four cases, termination of pregnancy was performed, due to early severe ventriculomegaly in three cases suggestive of complete aqueduct obstruction. In eight cases in which pregnancy was continued, three different evolving imaging patterns were demonstrated. In three cases the ventriculomegaly evolved rapidly during third trimester and were subsequently associated with abnormal white matter changes on fetal MRI. Theses cases led to premature delivery in two cases and early surgical care in all, with focal cystic parenchymal damage shown on follow-up MRI in two cases. Slowly evolving ventriculomegaly in three cases diagnosed in the second and the third trimester which required delayed surgery during the first year of life. Stable ventriculomegaly in two cases which did not require any surgical procedure.

CONCLUSION

The diagnosis of primary aqueduct obstruction may be based on different prenatal imaging patterns that include either severe early ventriculomegaly, stable, slowly or rapidly evolving ventriculomegaly.

摘要

目的

描述与先天性中脑导水管狭窄相关的不同产前影像学表现及其对产前咨询的影响。

方法

回顾性分析了 7 年来(2010-2016 年)连续诊断的孤立性先天性中脑导水管狭窄的产前病例。分析了产前影像学表现、产后影像学、病理数据和产后结果。

结果

共纳入 12 例病例。其中 4 例因早期严重的脑室扩大(3 例提示完全性中脑导水管狭窄)而终止妊娠。在继续妊娠的 8 例中,显示出 3 种不同的进展性影像学模式。在 3 例中,脑室扩大在孕晚期迅速进展,并随后伴有胎儿 MRI 上的异常白质改变。这 3 例导致早产和早期手术治疗,2 例在随访 MRI 上显示局灶性囊性实质损伤。3 例在孕中期和孕晚期诊断出的脑室扩大缓慢进展,需要在生命的第一年进行延迟手术。2 例脑室扩大稳定,无需任何手术治疗。

结论

先天性中脑导水管狭窄的诊断可能基于不同的产前影像学表现,包括严重的早期脑室扩大、稳定的、缓慢或迅速进展的脑室扩大。

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