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最小报道新生儿的产后脊髓脊膜膨出修补术:病例说明

Postnatal myelomeningocele closure in smallest reported neonate: illustrative case.

作者信息

Mohis Momin M, Cordeiro Kevin, Larson Sarah, Garland Catharine, Stadler James A

机构信息

1Department of Neurological Surgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin; and.

2Division of Plastic Surgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.

出版信息

J Neurosurg Case Lessons. 2024 Mar 4;7(10). doi: 10.3171/CASE23556.

DOI:10.3171/CASE23556
PMID:38437685
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10916853/
Abstract

BACKGROUND

Myelomeningocele (MMC) is the most serious form of spina bifida, a congenital defect in neural tube development. Defect closure in a patient with an extremely low birth weight presents unique challenges and risks; lower birth weight is associated with multiple organ system concerns, homeostasis is difficult, and local tissue is underdeveloped. To the authors' knowledge, the present case is the lowest reported weight (490 g) for a neonate with postnatal MMC repair.

OBSERVATIONS

A preterm male with a prenatally diagnosed lumbosacral MMC and associated Chiari malformation type II was born at 23 weeks 1 day to a 29-year-old mother, gravidity 6 parity 4. The patient was medically stabilized and underwent MMC closure on day of life 5. His weight was 490 g at the time of this repair, and he did not have any surgical complications. At age 16 months, he underwent endoscopic third ventriculostomy with choroid plexus cauterization; he has not required any further hydrocephalus treatments since the last follow-up at 30 months of age.

LESSONS

To the authors' knowledge, this case is the lowest birth weight ex utero MMC closure reported in the literature. Challenges of prematurity and size required appropriate preoperative stabilization, careful hemostasis and temperature regulation, and meticulous surgical technique.

摘要

背景

脊髓脊膜膨出(MMC)是脊柱裂最严重的形式,是神经管发育的先天性缺陷。极低出生体重儿的缺损闭合面临独特的挑战和风险;较低的出生体重与多器官系统问题相关,内环境稳定困难,局部组织发育不全。据作者所知,本病例是出生后接受MMC修复的新生儿中报告的最低体重(490克)。

观察结果

一名早产男婴,产前诊断为腰骶部MMC并伴有Ⅱ型Chiari畸形,于孕23周1天出生,其母亲29岁,孕6产4。患儿病情稳定后,于出生后第5天接受了MMC闭合手术。修复时他的体重为490克,且未出现任何手术并发症。16个月大时,他接受了内镜下第三脑室造瘘术并烧灼脉络丛;自30个月龄最后一次随访以来,他未再需要任何脑积水治疗。

经验教训

据作者所知,本病例是文献报道的宫外MMC闭合的最低出生体重。早产和体型带来的挑战需要适当的术前稳定、仔细的止血和体温调节以及精细的手术技术。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c8aa/10916853/059280451d56/CASE23556f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c8aa/10916853/793ee2cd83a9/CASE23556f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c8aa/10916853/059280451d56/CASE23556f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c8aa/10916853/793ee2cd83a9/CASE23556f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c8aa/10916853/059280451d56/CASE23556f2.jpg

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本文引用的文献

1
Neural tube defects: a review of global prevalence, causes, and primary prevention.神经管缺陷:全球患病率、病因及初级预防的综述。
Childs Nerv Syst. 2023 Jul;39(7):1703-1710. doi: 10.1007/s00381-023-05910-7. Epub 2023 Mar 8.
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Progressive hydrocephalus despite early complete reversal of hindbrain herniation after prenatal open myelomeningocele repair.尽管产前开放脊髓脊膜膨出修复后后脑疝迅速完全逆转,但仍出现进行性脑积水。
Neurosurg Focus. 2019 Oct 1;47(4):E13. doi: 10.3171/2019.7.FOCUS19434.
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Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on Closure of Myelomeningocele Within 48 Hours to Decrease Infection Risk.
神经外科医师学会系统评价和循证指南:在 48 小时内闭合脊髓脊膜膨出以降低感染风险。
Neurosurgery. 2019 Sep 1;85(3):E412-E413. doi: 10.1093/neuros/nyz264.
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Myelomeningocele closure by unilateral lumbar artery perforator flap: Experience with thirty-eight patients.采用单侧腰动脉穿支皮瓣修复脊髓脊膜膨出:38例患者的经验
Microsurgery. 2018 Oct;38(7):752-757. doi: 10.1002/micr.30348. Epub 2018 Jun 28.
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Fetal surgery for myelomeningocele: After the Management of Myelomeningocele Study (MOMS).胎儿手术治疗脊髓脊膜膨出:脊髓脊膜膨出治疗管理研究(MOMS)之后。
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