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先天性导水管狭窄产前手术管理的进展:从实验台到临床的方法。

Advances in prenatal surgical management of congenital aqueductal stenosis: A bench to bedside approach.

作者信息

Aydın Emrah, Duru Soner, Oria Marc, Peiro Jose L

机构信息

The Center for Fetal and Placental Research, Cincinnati Fetal Center, Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center (CCHMC), Cincinnati, OH, USA.

The Center for Fetal and Placental Research, Cincinnati Fetal Center, Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center (CCHMC), Cincinnati, OH, USA.

出版信息

Semin Fetal Neonatal Med. 2024 Nov;29(2-3):101540. doi: 10.1016/j.siny.2024.101540. Epub 2024 Oct 19.

Abstract

Congenital aqueduct stenosis AS is a significant cause of fetal obstructive hydrocephalus, characterized by the obliteration of the cerebral aqueduct, leading to cerebrospinal fluid (CSF) accumulation in the ventricular system and secondary brain damage and cerebral maldevelopment. This review explores the progression from basic science to clinical applications of antenatal surgical interventions for AS, emphasizing historical efforts, current research, and translational studies. Despite advances in prenatal imaging and genetic screening, challenges remain in achieving appropriate fetal candidates, consistent ventricular decompression, and standardized surgical protocols. This review highlights the need for further research and innovation to improve prenatal treatment and outcomes for AS-affected fetuses.

摘要

先天性中脑导水管狭窄(AS)是胎儿梗阻性脑积水的一个重要原因,其特征是中脑导水管闭塞,导致脑室系统中脑脊液(CSF)积聚,并继发脑损伤和脑发育异常。本综述探讨了AS产前手术干预从基础科学到临床应用的进展,重点介绍了以往的努力、当前的研究和转化研究。尽管产前影像学和基因筛查取得了进展,但在确定合适的胎儿候选者、持续脑室减压和标准化手术方案方面仍存在挑战。本综述强调需要进一步研究和创新,以改善AS患儿的产前治疗和预后。

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