Although neurofibromatosis type 1 (NF1) is a common genetical disorder, symptoms due to neural involvement are rare among infants and children. Ganglioneuromas are slow-growing tumors and can be seen in patients with NF1. However, bilateral symmetrical involvement is very rarely reported in the literature. We present a rare pediatric case with NF1 who presented with upper cervical bilateral symmetrical ganglioneuroma. A 7-year-old male presented with progressive gait disturbance, falls, and bilateral limb and trunk numbness that started several weeks ago. Physical examination revealed generalized café au lait spots, neurofibromas, and axillary and inguinal freckling. Family history for NF1 was positive. Neurological examination showed slight paraparesis and hypoesthesia of the upper extremities with C4–C5 dermatomas being more distinct. Cervical magnetic resonance imaging (MRI) revealed contrast enhancing dumbbell-shaped symmetrical lesions originating from both C3 nerve roots extending to C2–C5 vertebral body levels. There was a significant spinal cord compression causing myelopathy as well as dystrophic changes and kyphotic deformity at those levels. The patient was operated and the lesions were totally excised via C2–3–4 laminoplasty. Histopathological evaluation reported the lesion as ganglioneuroma. Following the surgery for excision of the lesions, the patient presented with rapidly progressing kyphotic deformity and underwent rigid craniocervical fusion. Ganglioneuromas are seen among children, tend to occur in the mediastinum, abdomen, and retroperitoneal area, and may accompany NF1. Cervical spinal ganglioneuroma, however, is a very rare entity. Co-existence of NF1 and bilateral symmetrical cervical ganglioneuroma is highly unusual. Like neurofibromas, ganglioneuromas are slow-growing tumors and therefore they are not expected to cause myelopathy and related symptoms during childhood. Symmetrical nature of the lesions results in myelopathy and early onset of symptoms. Due to the risk of postoperative increase of kyphotic angulation, laminoplasty should be considered. Although laminoplasty alone does not prevent the progression of kyphotic changes, the procedure may allow the use of sublaminar wires and laminar hooks for future surgeries to correct the deformity.

中文翻译：

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小儿神经纤维瘤病1型患者的双侧对称哑铃型颈神经节神经瘤引起的脊髓病和后路僵硬器械-病例报告

尽管1型神经纤维瘤病（NF1）是一种常见的遗传性疾病，但在婴幼儿中很少有由于神经受累引起的症状。神经节神经瘤是生长缓慢的肿瘤，可见于NF1患者。但是，文献中很少报道双侧对称受累。我们介绍了一个罕见的儿科病例，伴有上颈双侧对称性神经节神经瘤的NF1。一名7岁的男性表现出进行性步态障碍，跌倒以及几周前开始的双侧肢体和躯干麻木。体格检查发现全身咖啡色斑，神经纤维瘤，腋窝和腹股沟雀斑。NF1家族史为阳性。神经系统检查显示上肢轻度轻瘫和感觉不足，C4-C5皮肤瘤更为明显。子宫颈磁共振成像（MRI）显示对比增强的哑铃状对称病变，起源于两个C3神经根，延伸至C2-C5椎体水平。在这些水平上，脊髓压迫感明显，引起脊髓病，营养不良和脊柱后凸畸形。病人已经手术，并且通过C2-4椎板成形术完全切除了病灶。组织病理学评估报告该病变为神经节神经瘤。手术切除病灶后，患者出现快速发展的后凸畸形并进行了刚性颅颈融合术。儿童中可见神经节神经瘤，往往发生在纵隔，腹部和腹膜后区域，并可能伴有NF1。颈椎神经节神经瘤是非常罕见的实体。NF1与双侧对称性宫颈神经节神经瘤并存是非常罕见的。像神经纤维瘤一样，神经节神经瘤是生长缓慢的肿瘤，因此预计在儿童时期不会引起脊髓病和相关症状。病变的对称性质导致脊髓病和症状的早期发作。由于术后后凸角度增加的风险，应考虑椎板成形术。尽管仅靠椎板成形术不能防止后凸变化的发展，但该程序可能允许在以后的手术中使用层下线和层状钩来矫正畸形。病变的对称性质导致脊髓病和症状的早期发作。由于术后后凸角度增加的风险，应考虑椎板成形术。尽管仅靠椎板成形术不能防止后凸变化的发展，但该程序可能允许在以后的手术中使用层下线和层状钩来矫正畸形。病变的对称性质导致脊髓病和症状的早期发作。由于术后后凸角度增加的风险，应考虑椎板成形术。尽管仅靠椎板成形术不能防止后凸变化的发展，但该程序可能允许在以后的手术中使用层下线和层状钩来矫正畸形。