小儿重度颈后凸畸形患者复发丛状神经纤维瘤的外科治疗一例报告

Solji Jung, Dongho Kang, Donghwan Kim, Sang Hyub Lee
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摘要

大约50%的1型神经纤维瘤病(NF1)患者出现骨科并发症,脊柱畸形是常见的表现。颈后凸在NF1中相对少见;然而,它导致脊髓受压并伴有麻痹和呼吸功能障碍,需要手术矫正。小儿NF1患者通常有小而有缺陷的椎弓根或外侧肿块,单一入路的手术是有限的,以确保足够的脊髓减压和畸形矫正。然而,对于这种具有挑战性的疾病,没有可靠的治疗指南。这个病例报告了一个7岁的NF1患者,他有严重的颈椎后凸伴硬膜内髓外神经纤维瘤。在矫正畸形之前切除肿瘤,以减压脊髓,降低脊髓损伤的风险。此外,我们使用前-后-前入路有效地矫正和稳定了后凸。
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Surgical Management of Recurrent Plexiform Neurofibroma in a Pediatric Patient with Severe Cervical Kyphotic Deformity: A Case Report
Approximately 50% of patients with neurofibromatosis type 1 (NF1) develop orthopedic complications, and spinal deformities are common manifestations. Cervical kyphosis is comparatively rare in NF1; however, it results in spinal cord compression associated with paralysis and respiratory dysfunction, requiring surgical correction. Pediatric patients with NF1 usually have small and defective pedicles or lateral masses, and surgery with a single approach is limited to ensure sufficient spinal cord decompression and deformity correction. However, no reliable treatment guidelines are available for this challenging condition. This case report presents a 7-year-old patient with NF1 who had severe cervical kyphosis with intradural extramedullary neurofibromas. The tumors were removed before correcting the deformity to decompress the spinal cord and reduce the risk of spinal cord injury. Moreover, we effectively corrected and stabilized the kyphosis using the anterior-posterior-anterior approach.
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