Ⅰ型神经纤维瘤病脊柱侧后凸并椎体完全脱位:1例报道与综述

Neurofibromatosis type Ⅰ accompanied with dystrophic kyphoscoliosis and complete spondylolisthesis: a case report and literature review

报告1例Ⅰ型神经纤维瘤病脊柱侧后凸并椎体完全脱位患者的手术治疗方法及效果。患者,女,17岁,行手术治疗此严重畸形。经后入路在3D打印导板引导下置入椎弓根螺钉,行肋段切除胸廓成形,顶椎椎板切除神经减压后,行T5脊椎全切,最后置入双侧棒,矫正畸形、大量植骨,以重建脊柱稳定性。术中未损伤脊髓,术后患者恢复顺利, 12个月随访无复发加重的迹象。Ⅰ型神经纤维瘤病性营养不良型脊柱侧后凸畸形伴椎体完全脱位是罕见的,存在因脊柱畸形缓慢加重导致脊髓损伤的可能性。由于经济、文化等多方面的影响,常常在病情进展后才引起患者重视而进行治疗,采用后入路全脊椎截骨定制个体化减压矫形手术可能是减少术中脊髓损伤并达到令人满意结果的有效方法。

A case of neurofibromatosis type Ⅰ(NF-Ⅰ) accompanied with dystrophic kyphoscoliosis and complete spondylolisthesis was reported in this paper. The patient was a 17-year-old girl who underwent surgical treatment for this severe deformity.Through posterior approach the pedicle screws were placed under navigation by 3 D printed guiders, and then rib transverse resection and thoracoplasty were conducted. After laminectomy and decompression, vertebral column resection(VCR) of T5 was performed, finally the rods were placed to correct the deformity and reconstruct the spinal stability. The patient recovered smoothly without intraoperative spinal cord injury and returned to normal life. During 12-month follow-up, the patient had no sign of recurrence or worsening. The NF-I accompanied with dystrophic kyphoscoliosis and complete vertebral body dislocation is a rare case, which might lead to spinal cord damage due to progressive spinal deformity. However, this problem usually draws attention till it progress to sever condition due to economic and cultural limitation, and is taken seriously to surgical treatment.Individualized decompression, VCR and instrumented fusion may be an effective way to reduce intraoperative spinal cord injury and achieve satisfactory results.