Silveira Luke, Limoges Natalie
Neurosurgery Department, The University of Vermont Medical Center, Burlington, Vermont.
Neurosurgery Division, Valley Children's Healthcare, Madera, California.
J Neurosurg Case Lessons. 2025 Aug 11;10(6). doi: 10.3171/CASE2557.
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.
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
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