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胎儿镜下脊柱裂修复术。

Fetoscopic spina bifida repair.

作者信息

Miller Jena L, Groves Mari L, Baschat Ahmet A

机构信息

Department of Gynecology and Obstetrics, The Johns Hopkins Center for Fetal Therapy, Johns Hopkins University, Baltimore, MD, USA -

Division of Pediatric Neurosurgery, Department of Neurosurgery, Johns Hopkins University, Baltimore, MD, USA.

出版信息

Minerva Ginecol. 2019 Apr;71(2):163-170. doi: 10.23736/S0026-4784.18.04355-1. Epub 2018 Nov 27.

Abstract

INTRODUCTION

Spina bifida is the most common non-lethal congenital birth defect of the central nervous system that causes chronic disability due to the combined effects of local nerve damage and the sequelae of non-communicating hydrocephalus. This abnormality can be identified early in gestation and the damage can be progressive over the course of pregnancy. Advances in fetal treatment have made minimally invasive prenatal surgery a realistic consideration for spina bifida in order to improve the outcome for children affected this condition.

EVIDENCE ACQUISITION

Prenatal surgery for spina bifida via open fetal surgery with hysterotomy decreases the rate of ventriculoperitoneal shunt placement and improves motor function compared to standard postnatal surgery. Maternal risks of open fetal surgery are primarily related to complications of the hysterotomy including thinning or rupture that begins in the index pregnancy but persists for every future pregnancy. Minimizing maternal risks is the largest impetus to explore and optimize a minimally invasive fetoscopic alternative. Techniques vary from using a complete percutaneous approach to open fetoscopy, which requires laparotomy but is minimally invasive to the uterus. This allows vaginal delivery at term and no scar complications are reported thus far. Fetal short-term neurosurgical outcomes compare favorably with improvement in hindbrain herniation >70% and decreased need for treatment for hydrocephalus between 40-45% after prenatal surgery performed either fetoscopically or through open fetal surgery.

EVIDENCE SYNTHESIS

Maternal obstetric outcomes are superior for fetoscopic spina bifida repair compared to open fetal surgery and avoids the ongoing risk in future pregnancy. Neonatal and infant benefits appear equivalent. The open fetoscopic approach minimizes the risk of ruptured membranes and subsequent preterm delivery as opposed to a completely percutaneous procedure. International collaboration is ongoing to share experience and assess long term treatment effects.

CONCLUSIONS

Continued refinement of a minimally invasive strategy for prenatal treatment of spina bifida is necessary to maximize benefits to the child and further minimize maternal risks and preterm birth.

摘要

引言

脊柱裂是中枢神经系统最常见的非致死性先天性出生缺陷,由于局部神经损伤和非交通性脑积水的后遗症共同作用,导致慢性残疾。这种异常在妊娠早期即可被识别,且损伤在孕期过程中可能会进展。胎儿治疗的进展使微创产前手术成为脊柱裂的一个现实考虑方案,以改善受此病影响儿童的预后。

证据获取

与标准的产后手术相比,通过开腹胎儿手术经子宫切开术进行的脊柱裂产前手术可降低脑室腹腔分流术的放置率,并改善运动功能。开腹胎儿手术的母体风险主要与子宫切开术的并发症有关,包括在首次妊娠时开始出现的子宫变薄或破裂,且在未来每次妊娠中都会持续存在。将母体风险降至最低是探索和优化微创胎儿镜替代方案的最大动力。技术从使用完全经皮方法到开放式胎儿镜检查各不相同,开放式胎儿镜检查需要开腹手术,但对子宫的创伤最小。这允许足月阴道分娩,且迄今为止未报告有瘢痕并发症。胎儿短期神经外科手术结果较好,在进行胎儿镜检查或开腹胎儿手术后,后脑疝的改善率>70%,脑积水治疗需求减少40 - 45%。

证据综合

与开腹胎儿手术相比,胎儿镜下脊柱裂修复术的母体产科结局更好,且避免了未来妊娠中的持续风险。新生儿和婴儿的获益似乎相当。与完全经皮手术不同,开放式胎儿镜检查方法可将胎膜破裂和随后早产的风险降至最低。国际合作正在进行中,以分享经验并评估长期治疗效果。

结论

持续完善脊柱裂产前治疗的微创策略对于最大化儿童获益、进一步降低母体风险和早产风险是必要的。

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