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脊髓脊膜膨出与矢状缝早闭并存:病例展示

Concurrent myelomeningocele and sagittal craniosynostosis: illustrative case.

作者信息

Stegmann Kyle P, Nawabi Noah L A, Suresh Rishishankar, Saway Brian F, Miller Mark A, Eskandari Ramin, Kosnik Infinger Libby

机构信息

College of Medicine, Medical University of South Carolina, Charleston, South Carolina.

Department of Neurosurgery, Medical University of South Carolina, Charleston, South Carolina.

出版信息

J Neurosurg Case Lessons. 2025 Jan 6;9(1). doi: 10.3171/CASE24616.

DOI:10.3171/CASE24616
PMID:39761544
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11705678/
Abstract

BACKGROUND

Myelomeningocele and sagittal craniosynostosis are 2 neurosurgical pathologies with complications such as increased intracranial pressure (ICP) and hydrocephalus. While the 2 defects commonly occur independently, their simultaneous occurrence is exceptionally rare.

OBSERVATIONS

The authors report the case of a newborn male diagnosed with a simultaneous myelomeningocele and sagittal craniosynostosis. The patient underwent surgical repair of the myelomeningocele 2 days after birth. Wound breakdown and ventriculomegaly were observed 13 days after birth. Surgical wound repair was performed, and a neonatal reservoir was inserted because of concerns for wound healing and increased ICP. The reservoir was tapped daily, and the ventricle size was monitored via biweekly ultrasounds. Due to the increased risk of ICP presented by the sagittal craniosynostosis, an open strip craniectomy was performed at 34 days of life. Following surgery, cranial ultrasound studies demonstrated improving ventricle size and neonatal reservoir tapping was spaced to weekly. The patient was discharged at 68 days of life in stable condition, with no permanent cerebrospinal fluid diversion needed at the 8-month follow-up.

LESSONS

Surgical repair of sagittal craniosynostosis in the presence of a myelomeningocele should be considered earlier in life than generally indicated to limit the risk of developing elevated ICP, hydrocephalus, and subsequent complications. https://thejns.org/doi/10.3171/CASE24616.

摘要

背景

脊髓脊膜膨出和矢状缝早闭是两种神经外科疾病,可伴有颅内压升高(ICP)和脑积水等并发症。虽然这两种缺陷通常独立发生,但它们同时出现极为罕见。

观察结果

作者报告了一例同时诊断为脊髓脊膜膨出和矢状缝早闭的新生儿男性病例。患儿出生后2天接受了脊髓脊膜膨出修补手术。出生后13天观察到伤口裂开和脑室扩大。进行了手术伤口修复,并因担心伤口愈合和ICP升高而插入了新生儿储液囊。每天抽取储液囊,并通过每两周一次的超声监测脑室大小。由于矢状缝早闭导致ICP升高的风险增加,患儿在出生34天时进行了开放性条带颅骨切除术。术后,头颅超声检查显示脑室大小改善,新生儿储液囊抽取间隔延长至每周一次。患儿在出生68天时病情稳定出院,8个月随访时无需永久性脑脊液分流。

经验教训

对于存在脊髓脊膜膨出的患儿,矢状缝早闭的手术修复应比通常建议的时间更早进行,以降低发生ICP升高、脑积水及后续并发症的风险。https://thejns.org/doi/10.3171/CASE24616

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d04/11705678/1cac4d51a84c/CASE24616_figure_5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d04/11705678/cab402b1c930/CASE24616_figure_1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d04/11705678/fb282b5585b7/CASE24616_figure_2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d04/11705678/5985ad183382/CASE24616_figure_3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d04/11705678/ce34a0fc2ab0/CASE24616_figure_4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d04/11705678/1cac4d51a84c/CASE24616_figure_5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d04/11705678/cab402b1c930/CASE24616_figure_1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d04/11705678/fb282b5585b7/CASE24616_figure_2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d04/11705678/5985ad183382/CASE24616_figure_3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d04/11705678/ce34a0fc2ab0/CASE24616_figure_4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d04/11705678/1cac4d51a84c/CASE24616_figure_5.jpg

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本文引用的文献

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Isolated Sagittal Craniosynostosis: A Comprehensive Review.孤立性矢状缝早闭:综述
Diagnostics (Basel). 2024 Feb 16;14(4):435. doi: 10.3390/diagnostics14040435.
2
Intracranial Pressure, Autoregulation, and Cerebral Perfusion in Infants With Nonsyndromic Craniosynostosis at the Time of Surgical Correction.非综合征性颅缝早闭婴儿手术矫正时的颅内压、自动调节和脑灌注
Neurosurgery. 2023 Dec 13. doi: 10.1227/neu.0000000000002797.
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Risk factors and time trends for isolated craniosynostosis.孤立性颅缝早闭的危险因素和时间趋势。
Birth Defects Res. 2021 Jan 1;113(1):43-54. doi: 10.1002/bdr2.1824. Epub 2020 Oct 22.
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Myelomeningocele-associated hydrocephalus: nationwide analysis and systematic review.脊髓脊膜膨出相关脑积水:全国性分析和系统评价。
Neurosurg Focus. 2019 Oct 1;47(4):E5. doi: 10.3171/2019.7.FOCUS19469.
5
Incidence of Familial Craniosynostosis Among Patients With Nonsyndromic Craniosynostosis.非综合征性颅缝早闭患者中家族性颅缝早闭的发病率
J Craniofac Surg. 2019 Sep;30(6):e514-e517. doi: 10.1097/SCS.0000000000005419.
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Nonsyndromic Craniosynostosis.非综合征性颅缝早闭
Clin Plast Surg. 2019 Apr;46(2):123-139. doi: 10.1016/j.cps.2018.11.001. Epub 2019 Jan 30.
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Age at Time of Craniosynostosis Repair Predicts Increased Complication Rate.颅骨缝早闭修复时的年龄预示并发症发生率增加。
Cleft Palate Craniofac J. 2018 May;55(5):649-654. doi: 10.1177/1055665617725215. Epub 2017 Dec 14.
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Estimates of global and regional prevalence of neural tube defects for 2015: a systematic analysis.估计全球和区域神经管缺陷患病率 2015 年:系统分析。
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Semin Fetal Neonatal Med. 2017 Dec;22(6):360-366. doi: 10.1016/j.siny.2017.08.004. Epub 2017 Oct 12.
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