Keil Corinna, Sass Benjamin, Schulze Maximilian, Köhler Siegmund, Axt-Fliedner Roland, Bedei Ivonne
Department of Obstetrics and Gynecology, University Hospital Giessen and Marburg, Campus Marburg, Marburg, Germany; Department of Neurosurgery, University Hospital of Giessen and Marburg, Campus Marburg, Marburg, Germany; Department of Neuroradiology, University Hospital Giessen and Marburg, Campus Marburg, Marburg, Germany; Center for Prenatal Medicine and Fetal Therapy, University Hospital Giessen and Marburg, Campus Giessen, Giessen, Germany.
Dtsch Arztebl Int. 2025 Jan 24;122(2):33-37. doi: 10.3238/arztebl.m2024.0239.
Open spinal dysraphism is a congenital malformation that causes major morbidity. Its consequences include sensory and motor impairment as well as bladder- and bowel dysfunction. It is often also associated with prenatal ventriculomegaly, which, in turn, necessitates postnatal treatment with a ventriculoperitoneal shunt in approximately 80% of cases. Prenatal therapy with coverage of neural tube defect can reduce the shunt rate and preserve motor function. In this review, we describe the different surgical procedures and their outcomes.
This review is based on publications that were retrieved by a selective literature search in the MEDLINE, Web of Science, EMBASE, Scopus, and Cochrane databases, employing pertinent keywords. Studies of all types (except case reports) that were published in English or German in the period 2010-2024 were included.
The randomized, controlled MOMS trial showed that intrauterine surgery for defect closure resulted in less progressive neural tissue damage than postnatal surgery and reduced the need for shunting by approximately half (40% vs. 82%). Since the publication of these results, various prenatal surgical procedures have been established, including hysterotomy-assisted, percutaneous fetoscopic, and laparotomy-assisted fetoscopic closure. The individual surgical methods yield comparable results in terms of motor function and shunt rate. A problem with these procedures is that they increase the likelihood of preterm birth, to an extent that varies from one type of procedure to another.
Prenatal surgery improves motor function and reduces the shunt rate but long-term outcomes beyond adolescence are still lacking. Transparent and interdisciplinary counseling is essential in prenatal communication to inform parents not only about the potential benefits of this treatment, but also about its limitations and risks.
开放性脊柱裂是一种导致严重发病的先天性畸形。其后果包括感觉和运动障碍以及膀胱和肠道功能障碍。它通常还与产前脑室扩大有关，而这反过来又使得约80%的病例在出生后需要进行脑室腹腔分流术治疗。神经管缺陷覆盖的产前治疗可以降低分流率并保留运动功能。在本综述中，我们描述了不同的手术方法及其结果。
本综述基于通过在MEDLINE、科学网、EMBASE、Scopus和Cochrane数据库中进行选择性文献检索，使用相关关键词检索到的出版物。纳入了2010年至2024年期间以英文或德文发表的所有类型（病例报告除外）的研究。
随机对照的MOMS试验表明，与出生后手术相比，宫内手术闭合缺损导致的神经组织渐进性损伤更少，并且分流需求减少了约一半（40%对82%）。自这些结果发表以来，已经确立了各种产前手术方法，包括子宫切开术辅助、经皮胎儿镜和剖腹术辅助胎儿镜闭合术。就运动功能和分流率而言，个体手术方法产生的结果相当。这些手术方法的一个问题是它们增加了早产的可能性，其程度因手术类型而异。
产前手术可改善运动功能并降低分流率，但仍缺乏青春期后长期结果。在产前沟通中，透明和跨学科的咨询至关重要，不仅要告知父母这种治疗的潜在益处，还要告知其局限性和风险。
