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婴儿期多发性脊髓髓内海绵状血管畸形切除术:病例说明

Resection of multiple intramedullary spinal cord cavernous malformations in infancy: illustrative case.

作者信息

Silveira Luke, Limoges Natalie

机构信息

Neurosurgery Department, The University of Vermont Medical Center, Burlington, Vermont.

Neurosurgery Division, Valley Children's Healthcare, Madera, California.

出版信息

J Neurosurg Case Lessons. 2025 Aug 11;10(6). doi: 10.3171/CASE2557.


DOI:10.3171/CASE2557
PMID:40789216
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12337979/
Abstract

BACKGROUND: Cavernous malformations (CMs) are vascular malformations characterized by enlarged, thin-walled blood vessels composed of dysplastic endothelial cells and pericytes within a loose extracellular matrix. Endothelial cell dysplasia leads to increased permeability and portends an increased risk of hemorrhage within these malformations, and hemorrhage often manifests with acute-onset neurological deficits due to mass effect and irritation of surrounding neural tissue within the brain and spinal cord. OBSERVATIONS: A 5-month-old male presented with concern for possible tethered cord syndrome in the setting of forked gluteal cleft and lower extremity hyperreflexia. He was ultimately determined to have three intramedullary spinal CMs. These were resected with laminoplasty performed. Follow-up imaging demonstrated preservation of spinal alignment and no intraspinal lesion recurrence, with corresponding improvement in the patient's neurological strength examination despite some persistent motor developmental milestone delay. LESSONS: Hemorrhage and rehemorrhage rates of intramedullary spinal CMs are higher in the pediatric population than in the adult population. Hence, early resection of these lesions, when feasible, reduces the risk of long-term neurological deficit. Resection in infancy can be augmented by laminoplasty to preserve the posterior tension band within the spinal column, promote bony regrowth, and significantly reduce the risk of delayed deformity. https://thejns.org/doi/10.3171/CASE2557.

摘要

背景:海绵状血管畸形(CMs)是一种血管畸形,其特征是由发育异常的内皮细胞和周细胞构成的薄壁血管在疏松的细胞外基质中扩张。内皮细胞发育异常导致通透性增加,并预示着这些畸形内出血风险增加,而出血常因脑和脊髓内的占位效应及对周围神经组织的刺激而表现为急性发作的神经功能缺损。 观察结果:一名5个月大的男性因臀裂分叉和下肢反射亢进被怀疑可能患有脊髓栓系综合征。最终确定他患有3个脊髓内CMs。对这些病变进行了切除并实施了椎板成形术。随访影像学检查显示脊柱排列保持良好,脊髓内病变无复发,尽管患者的运动发育里程碑仍有一些持续延迟,但神经功能强度检查有相应改善。 经验教训:小儿脊髓内CMs的出血和再出血率高于成人。因此,在可行的情况下早期切除这些病变可降低长期神经功能缺损的风险。婴儿期切除时可通过椎板成形术来保留脊柱后张力带,促进骨再生,并显著降低延迟畸形的风险。https://thejns.org/doi/10.3171/CASE2557

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/30aa/12337979/b0d7c41c1980/CASE2557_figure_2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/30aa/12337979/7f34a78906ab/CASE2557_figure_1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/30aa/12337979/b0d7c41c1980/CASE2557_figure_2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/30aa/12337979/7f34a78906ab/CASE2557_figure_1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/30aa/12337979/b0d7c41c1980/CASE2557_figure_2.jpg

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[1]
Resection of multiple intramedullary spinal cord cavernous malformations in infancy: illustrative case.

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本文引用的文献

[1]
Clinical Features and Treatment of Pediatric Cerebral Cavernous Malformations.

J Korean Neurosurg Soc. 2024-5

[2]
Biomechanical evaluation on a new type of vertebral titanium porous mini-plate and mechanical comparison between cervical open-door laminoplasty and laminectomy: a finite element analysis.

Front Bioeng Biotechnol. 2024-2-5

[3]
Natural history of familial cerebral cavernous malformation syndrome in children: a multicenter cohort study.

Neuroradiology. 2023-2

[4]
A systematic review on the outcome of intramedullary spinal cord cavernous malformations.

Eur Spine J. 2022-11

[5]
Rapamycin in Cerebral Cavernous Malformations: What Doses to Test in Mice and Humans.

ACS Pharmacol Transl Sci. 2022-4-25

[6]
Comparison of Laminoplasty vs. Laminectomy for Cervical Spondylotic Myelopathy: A Systematic Review and Meta-Analysis.

Front Surg. 2022-1-17

[7]
Cerebral Cavernous Malformation: From Mechanism to Therapy.

Circ Res. 2021-6-25

[8]
Spinal Cavernoma in an Infant: A Rare Pathological Entity with an Atypical Presentation.

Pediatr Neurosurg. 2020

[9]
Clinical features and long-term outcomes of pediatric spinal cord cavernous malformation-a report of 18 cases and literature review.

Childs Nerv Syst. 2021-1

[10]
Idiopathic Scoliosis in Children and Adolescents: Emerging Techniques in Surgical Treatment.

World Neurosurg. 2019-7-5

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