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产前脊髓脊膜膨出修补术——改善生活质量的契机

[Prenatal myelomeningocele repair - a chance to improve the quality of life].

作者信息

Pastuszka Agnieszka, Bohosiewicz Janusz, Olejek Anita, Zamłyński Jacek, Horzelska Ewa, Koszutski Tomasz

机构信息

Katedra i Zakład Anatomii Opisowej i Topograficznej, Wydział Lekarski z Oddziałem Lekarsko-Dentystycznym w Zabrzu, Śląski Uniwersytet Medyczny w Katowicach, Katowice, Polska.

Wyższa Szkoła Planowania Strategicznego w Dąbrowie Górniczej, Dąbrowa Górnicza, Polska.

出版信息

Wiad Lek. 2019;72(7):1380-1386.

PMID:31398173
Abstract

Spina bifida (myelomeningocele) is the most prevalent developmental defect of the nervous system. In different regions of the world it affects from 0.3 to 5 per 1000 of living neonates. It is known that the defect develops during the early fetal life however no explicit cause has been found yet. Recently it has been accepted that one of the major factors contributing to spina bifida is deficiency of folic acid in the mother's body before and during pregnancy. The first attempts to cure myelomeningocele were taken in ancient times. More extensive knowledge of aetiology and effects of spina bifida and development of new surgical techniques allowed for management not only of the defect itself but also the consequent complications. Prenatal spina bifida repair has been performed in the USA for 20 years now while European surgeons (Poland and Switzerland) have operated myelomeningocele for 12 years. Comparative analysis carried out to evaluate the patients operated for spina bifida prenatally and postnatally pointed that those after fetal repair demand the use of ventriculoperitoneal shunt for hydrocephalus twice less frequently. Thanks to shortened exposure of the spinal cord and the spinal nerves to the toxic effect of amniotic fluid and reduced exposure to mechanical injuries, prenatal myelomeningocele repair offers better opportunities to improve motor, urinary and alimentary functions, contributing then statistically to higher quality of lives of children with spina bifida.

摘要

脊柱裂(脊髓脊膜膨出)是最常见的神经系统发育缺陷。在世界不同地区,每1000名活产新生儿中受其影响的人数为0.3至5人。已知该缺陷在胎儿早期发育过程中形成,但尚未发现明确病因。最近,人们公认导致脊柱裂的主要因素之一是母亲在怀孕前及怀孕期间体内叶酸缺乏。古代就有治疗脊髓脊膜膨出的首次尝试。对脊柱裂病因及影响的更广泛了解以及新手术技术的发展,不仅使对该缺陷本身的治疗成为可能,也使对随之而来的并发症的治疗成为可能。在美国,产前脊柱裂修复手术已开展20年,而欧洲的外科医生(波兰和瑞士)开展脊髓脊膜膨出手术已有12年。对产前和产后接受脊柱裂手术的患者进行的比较分析表明,胎儿修复术后的患者需要进行脑室腹腔分流术治疗脑积水的频率降低了一半。由于脊髓和脊神经暴露于羊水毒性作用的时间缩短,以及受到机械损伤的暴露减少,产前脊髓脊膜膨出修复术为改善运动、泌尿和消化功能提供了更好的机会,从而在统计学上有助于提高脊柱裂患儿的生活质量。

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Wiad Lek. 2019;72(7):1380-1386.
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First 60 fetal in-utero myelomeningocele repairs at Saint Louis Fetal Care Institute in the post-MOMS trial era: hydrocephalus treatment outcomes (endoscopic third ventriculostomy versus ventriculo-peritoneal shunt).在“MOMS”试验后时代,圣路易斯胎儿护理研究所进行的前60例胎儿宫内脊髓脊膜膨出修补术:脑积水治疗结果(内镜下第三脑室造瘘术与脑室腹腔分流术)
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Fetal surgery for myelomeningocele: history, research, clinical trials, and future directions.脊髓脊膜膨出的胎儿手术:历史、研究、临床试验及未来方向。
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