伴有肋骨侵入椎管的Ⅰ型神经纤维瘤病营养不良型脊柱侧后凸的手术治疗

Posterior correction without rib head resection for dystrophic kyphoscoliosis complicated with rib head protrusion into the central canal in type 1 neurofibromatosis

中文关键词:  营养不良型脊柱侧后凸  Ⅰ型神经纤维瘤病  肋骨  椎管

英文关键词:Dystrophic kyphoscoliosis  Neurofibromatosis type 1  Rib  Central canal

中文摘要:

  【摘要】 目的：探讨对伴有肋骨侵入椎管的Ⅰ型神经纤维瘤病营养不良型脊柱侧后凸（neurofibromatosis kyphoscoliosis type 1，NFK-1）患者行单纯后路矫形手术的安全性和早期临床治疗效果。方法：2003年2月~2013年4月共收治8例伴肋骨侵入椎管的Ⅰ型神经纤维瘤病患者，男6例，女2例；年龄7~24岁，平均12.9岁。所有病例肋骨侵入椎管内节段均在侧凸顶点附近1个椎体节段，术前肋骨椎管占位比平均32.86%。其中7例接受单纯后路矫形融合术，1例接受生长棒矫形，均未对突入椎管内肋骨进行直接干预。回顾性分析患者术前、术后及随访时的X线片、CT、脊髓造影后CT（CTM）或MRI，对侧后凸Cobb角、躯干偏移等参数进行测量和分析；同时复习病历，记录围手术期的并发症。结果：手术时间平均为3.3h，术中出血量平均为460ml。固定节段平均为10.1个节段。手术前后胸段冠状面Cobb角分别为67.00°和34.38°，平均矫形率为48.7%。矢状面Cobb角分别为62.50°和31.25°，平均矫形率为49.9%。平均随访时间22.9个月，末次随访时主胸弯冠状面Cobb角及矢状面Cobb角分别为35.75°和33.38°。手术前、后及随访时冠状面躯干平衡分别为35.88mm、15.63mm和14.00mm；矢状位躯干平衡分别为35.13mm、18.13mm和15.50mm。手术前、后椎体旋转度分别为2.25°和1.88°，顶椎偏距分别为49.38mm和35.81mm。7例患者术后复查CT肋骨椎管占位比由术前33.36%减小为术后26.57%；2例肋骨位置未见明显变化，5例肋骨不同程度复位。2例患者术前有胸痛症状，术后胸痛症状均缓解；1例术前右下肢巴氏征（+）、踝阵挛（+），术后3个月随访病理征转阴性，无神经系统并发症。结论：对于无神经损害症状伴有肋骨侵入椎管内的Ⅰ型神经纤维瘤病脊柱侧后凸患者，对胸段脊柱直接矫形是安全、有效的。

英文摘要:

  【Abstract】 Objectives: To discuss the safety and early outcome of posterior correction while remaining the rib head for dystrophic kyphoscoliosis complicated with rib head protrusion into the central canal in type 1 neurofibromatosis. Methods: 8 NF-1 patients with rib head displacement into spine canal underwent posterior correction from Febuary 2003 to April 2013. Data such as the Cobb angle of the scoliosis and kyphosis, trunk balance during pre- post- operation and follow-up were collected, the perioperative complications were also collected. Results: 8 cases (6 males and 2 females) with an average of 12.9 years(7-24 years) were included in our review. All except one experienced posterior fusion surgery, while the remaining underwent the growing rod correction. Both methods had rib head intact. The average follow-up time was 22.9 months and the average number of the instrumented segment was 10.1. The average surgery time was 3.3 hours and the average blood loss was 460ml. The pre- and post- operative thoracic cobb angle was 67.00° and 34.38° respectively and the average correction rate was 48.7%. On the other side, the sagittal plane Cobb angle was 62.50° and 31.25° respectively, the average correction rate was 49.9%. At the final follow-up at and average of 22.9 months, Cobb angle from the coronary plane and the sagittal plane was 35.75° and 33.38° respectively. Pre- post- operation and the follow up coronary trunk balance was 35.88mm, 15.63mm and 14.00mm respectively, and 35.13mm, 18.13mm and 15.50mm respectively in the sagittal plane. The rotation degree of the vertebrae before and after the surgery was 2.25°and 1.88° respectively, the apical vertebral offset was 49.38mm and 35.81mm. All rib head displacements were located in one level and around the scoliotic apex, pre-operative rib head displacement into spine canal accounted for 32.86%. Seven patients decreased from 33.36% before the surgery to 26.57% after the surgery despite of no significant change in the location of the rib head in two cases, the other five cases experienced incomplete recovery. Two cases had chest pain relieved after surgery. One case had pathological sign, which disappeared after surgery. Conclusions: Direct correction in the thoracic spine is safe and effective for NF1 patients with rib head displacement into spine canal and with no neurological deficit.