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胎儿镜双层缝合治疗开放性神经管缺陷:前50例病例的产科、手术及围产期结局的前瞻性研究

Fetoscopic two-layer closure for open neural tube defects: prospective study of obstetric, surgical and perinatal outcomes in the first 50 cases.

作者信息

Giné C, Arévalo S, Maiz N, Rodó C, Moreno E, Casas B, Manrique S, Meléndez M, López M, Carreras E

机构信息

Paediatric Surgery Department, Hospital Universitari Vall d'Hebron, Vall d'Hebron Barcelona Hospital Campus, Barcelona, Spain.

Universitat Autònoma de Barcelona, Barcelona, Spain.

出版信息

Ultrasound Obstet Gynecol. 2025 Sep 3. doi: 10.1002/uog.70013.

DOI:10.1002/uog.70013
PMID:40903997
Abstract

OBJECTIVE

Fetoscopic repair for open neural tube defects (ONTDs) has gained acceptance among leading groups, although it remains controversial owing to the lack of a standardized neurosurgical technique. In 2018, our group described a new fetoscopic two-layer procedure with an exteriorized uterus for ONTD reconstruction. This study aimed to report obstetric, surgical and perinatal outcomes for the first 50 cases since the implementation of this technique and to provide comparative data with open fetal surgery studies.

METHODS

This was a single-center, observational, prospective study conducted between February 2017 and September 2024. Patients scheduled for fetoscopic repair of ONTD using the two-layer technique with uterine exteriorization were included, and variables such as maternal characteristics, prenatal diagnosis, surgical technique, obstetric outcome, perinatal outcome and complications were evaluated. We compared these with the outcomes of the Management of Myelomeningocele Study (MOMS) cohort and a post-MOMS cohort.

RESULTS

Fetoscopic repair of ONTD was performed successfully in all 50 (100%) cases, with no conversions to hysterotomy repair. Of these, 48 cases resulted in a live birth, one in stillbirth and one pregnancy was terminated. Presurgical ultrasound identified myelomeningocele in 29 (58.0%) and ventriculomegaly in 27 (54.0%) cases. The mean ± SD gestational age at surgery was 25.0 ± 1.1 weeks, and the mean procedure duration was 178 ± 37.6 min. In 42 (84.0%) cases, the repair was performed using a two-layer technique. Complications included preterm prelabor rupture of membranes in 24/49 (49.0%) cases and chorioamniotic membrane separation in 11/49 (22.4%). Among the live births, delivery occurred at a median gestational age of 36.0 (interquartile range (IQR), 33.9-37.2) weeks, with 14/48 (29.2%) delivering at term. Median birth weight was 2510 (IQR, 2178-2816) g, and no cases of neonatal death were reported. Postnatal motor function was equal or better than the presurgery motor level in 26/34 (76.5%) cases. No case of cerebrospinal fluid leakage at the spinal repair site was reported. Comparison with the MOMS and post-MOMS studies showed a higher gestational age at delivery, improved motor outcome and less respiratory distress syndrome than in the post-MOMS cohort. Vaginal delivery occurred in 47.9% of cases in our cohort, in contrast to the MOMS and post-MOMS cohorts, in which all deliveries were by Cesarean section.

CONCLUSION

The hybrid two-layer closure of ONTDs is a safe procedure, yielding obstetric and perinatal outcomes comparable with those of open surgery. However, it may not be suitable for all types of defect. Long-term data are required to allow for comprehensive comparisons and to determine whether this technique should be recommended over other current surgical options. © 2025 The Author(s). Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

摘要

目的

胎儿镜修复开放性神经管缺陷(ONTDs)已得到主要研究团队的认可,尽管由于缺乏标准化的神经外科技术,该方法仍存在争议。2018年,我们团队描述了一种新的胎儿镜双层手术方法,通过子宫外置进行ONTD重建。本研究旨在报告自实施该技术以来的前50例病例的产科、手术及围产期结局,并提供与开放性胎儿手术研究的对比数据。

方法

这是一项于2017年2月至2024年9月期间在单一中心开展的观察性前瞻性研究。纳入计划采用子宫外置双层技术进行胎儿镜修复ONTD的患者,并评估产妇特征、产前诊断、手术技术、产科结局、围产期结局及并发症等变量。我们将这些结果与脊髓脊膜膨出管理研究(MOMS)队列及MOMS后队列的结果进行比较。

结果

所有50例(100%)ONTD胎儿镜修复手术均成功完成,无中转开腹修复情况。其中,48例活产,1例死产,1例妊娠终止。术前超声检查发现29例(58.0%)有脊髓脊膜膨出,27例(54.0%)有脑室扩大。手术时的平均孕周±标准差为25.0±1.1周,平均手术时长为178±37.6分钟。42例(84.0%)采用双层技术进行修复。并发症包括24/49例(49.0%)早产前胎膜早破和11/49例(22.4%)羊膜绒毛膜分离。在活产儿中,分娩孕周中位数为36.0(四分位间距(IQR),33.9 - 37.2)周,其中14/48例(29.2%)足月分娩。出生体重中位数为2510(IQR,2178 - 2816)克,未报告新生儿死亡病例。26/34例(76.5%)产后运动功能等于或优于术前运动水平。未报告脊髓修复部位脑脊液漏病例。与MOMS及MOMS后研究相比,本队列分娩孕周更大,运动结局改善,呼吸窘迫综合征发生率更低。本队列47.9%的病例经阴道分娩,而MOMS及MOMS后队列均为剖宫产。

结论

ONTDs的混合双层闭合术是一种安全的手术方法,产科及围产期结局与开放性手术相当。然而,它可能并不适用于所有类型的缺陷。需要长期数据进行全面比较,并确定该技术是否应优于当前其他手术选择而被推荐。© 2025作者。《超声妇产科》由约翰·威利父子有限公司代表国际妇产科超声学会出版。

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