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脊髓脊膜膨出:长期神经外科治疗管理。

Myelomeningocele: Long-Term Neurosurgical Management.

机构信息

Department of Neurosurgery, ASST Niguarda Hospital, Milan, Italy.

Department of Orthopedics, ASST Niguarda Hospital, Milan, Italy.

出版信息

Adv Tech Stand Neurosurg. 2024;49:95-122. doi: 10.1007/978-3-031-42398-7_6.

DOI:10.1007/978-3-031-42398-7_6
PMID:38700682
Abstract

Open spina bifida, also known as myelomeningocele (MMC), is the most challenging and severe birth defect of the central nervous system compatible with life and it is due to a failure in the dorsal fusion of the nascent neural tube during embryonic development. MMC is often accompanied by a constellation of collateral conditions, including hydrocephalus, Arnold - Chiari II malformation, brainstem disfunction, hydrosyringomyelia, tethered cord syndrome and scoliosis. Beyond early surgical repair of the dorsal defect, MMC requires lifelong cares. Several additional surgical procedures are generally necessary to improve the long-term outcomes of patients affected by MMC and multidisciplinary evaluations are crucial for early identification and management of the various medical condition that can accompany this pathology. In this chapter, the most common pathological entities associated with MMC are illustrated, focusing on clinical manifestations, treatment strategies and follow up recommendations.

摘要

开放性脊柱裂,又称脊髓脊膜膨出(MMC),是一种最具挑战性和严重的中枢神经系统出生缺陷,与生命兼容,它是由于胚胎发育过程中新生神经管的背侧融合失败所致。MMC 常伴有一系列的伴发情况,包括脑积水、Arnold-Chiari II 畸形、脑干功能障碍、脊髓空洞症、脊髓栓系综合征和脊柱侧凸。除了早期修复背部缺陷的手术外,MMC 还需要终身护理。通常需要进行几次额外的手术来改善 MMC 患者的长期预后,多学科评估对于早期识别和处理可能伴随这种病理的各种医学状况至关重要。在本章中,我们阐述了与 MMC 相关的最常见的病理实体,重点介绍了临床表现、治疗策略和随访建议。

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Int J Dev Neurosci. 2025;85(6):e70051. doi: 10.1002/jdn.70051.
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Neurogenic Bladder in Children with Myelomeningocele.脊髓脊膜膨出患儿的神经源性膀胱
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Detailed analysis of hydrocephalus patterns and associated variables in patients after open fetal repair and postnatal myelomeningocele/myeloschisis closure.

本文引用的文献

1
Neurologic Outcome Comparison between Fetal Open-, Endoscopic- and Neonatal-Intervention Techniques in Spina Bifida Aperta.开放性脊柱裂胎儿干预、内镜干预及新生儿干预技术的神经学结局比较
Diagnostics (Basel). 2023 Jan 9;13(2):251. doi: 10.3390/diagnostics13020251.
2
Intrauterine closure of myelomeningocele defects with primary linear repair versus bipedicle fasciocutaneous flaps: a post-MOMS cohort study with long-term follow-up.原发性线性修复与双蒂筋膜皮瓣修复在脊髓脊膜膨出缺陷宫内闭合中的应用:一项MOMS后队列长期随访研究
J Neurosurg Pediatr. 2022 Nov 25;31(2):143-150. doi: 10.3171/2022.10.PEDS22357. Print 2023 Feb 1.
3
开放性胎儿修复及产后脊髓脊膜膨出/脊髓裂闭合术后患者脑积水模式及相关变量的详细分析。
Childs Nerv Syst. 2025 Apr 16;41(1):160. doi: 10.1007/s00381-025-06819-z.
Hydrocephalus in myelomeningocele.
脑脊膜膨出并发脑积水。
Childs Nerv Syst. 2021 Nov;37(11):3407-3415. doi: 10.1007/s00381-021-05333-2. Epub 2021 Aug 25.
4
Syrinx shunts for syringomyelia: a systematic review and meta-analysis of syringosubarachnoid, syringoperitoneal, and syringopleural shunting.脊髓空洞-脑脊髓液分流术治疗脊髓空洞症:脊髓空洞-蛛网膜下腔、脊髓空洞-腹腔和脊髓空洞-胸腔分流术的系统评价和荟萃分析。
J Neurosurg Spine. 2021 Jul 30;35(4):535-545. doi: 10.3171/2020.12.SPINE201826. Print 2021 Oct 1.
5
Minimally Invasive Surgery for Neuromuscular Scoliosis: Results and Complications at a Minimal Follow-up of 5 Years.微创神经肌肉性脊柱侧凸手术:至少 5 年随访的结果和并发症。
Spine (Phila Pa 1976). 2021 Dec 15;46(24):1696-1704. doi: 10.1097/BRS.0000000000004082.
6
Fetoscopic myelomeningocoele closure: Is the scientific evidence enough to challenge the gold standard for prenatal surgery?经阴道脊膜膨出修补术:现有科学证据是否足以挑战产前手术的金标准?
Prenat Diagn. 2021 Jul;41(8):949-956. doi: 10.1002/pd.5940. Epub 2021 Apr 5.
7
Surgical pathway proposal for severe paralytic scoliosis in adolescents with myelomeningocele.脊髓脊膜膨出青少年重度麻痹性脊柱侧弯的手术路径建议。
Childs Nerv Syst. 2021 Jul;37(7):2279-2287. doi: 10.1007/s00381-021-05079-x. Epub 2021 Feb 14.
8
New surgical paradigm for open neural tube defects.开放式神经管缺陷的新手术模式。
Childs Nerv Syst. 2021 Feb;37(2):529-538. doi: 10.1007/s00381-020-04866-2. Epub 2020 Aug 21.
9
Surgical management of Chiari malformation type II.II型Chiari畸形的外科治疗
Childs Nerv Syst. 2020 Aug;36(8):1621-1634. doi: 10.1007/s00381-020-04675-7. Epub 2020 May 30.
10
Fetal surgery for open spina bifida.开放性脊柱裂的胎儿手术
Obstet Gynaecol. 2019 Oct;21(4):271-282. doi: 10.1111/tog.12603. Epub 2019 Sep 27.