不伴神经损害的I型神经纤维瘤病性脊柱侧凸矫形术中保留脱入椎管的肋骨头的安全性

Security analysis of the preservation of the intraspinal rib head when correcting the spinal deformity in neurologically intact patients with scoliosis secondary to Neurofibromatosis type 1

目的探讨对不伴神经损害的I型神经纤维瘤病性脊柱侧凸患者在保留脱入椎管内肋骨头的同时行脊柱矫形术的安全性与有效性。方法对1998年8月至2012年3月行脊柱后路矫形内固定植骨融合术的9例I型神经纤维瘤病性脊柱侧凸伴肋骨头椎管内脱位患者的临床及影像学资料进行回顾性研究。男4例，女5例；年龄7~33岁，平均（15．4±7．6）岁；冠状面胸弯Cobb角平均70．7°±17．7°；矢状面后凸Cobb角平均59．7°±17．6°。神经功能均为Frankel E级。行脊柱后路矫形内固定植骨融合术，术中不切除脱入椎管内的肋骨头。测量患者术前和术后肋骨头脱入椎管内程度、肋骨进入椎管角度、双侧肋骨成角、冠状面及矢状面Cobb角。结果9例患者均获得随访，随访时间0．5-4．8年，平均2．4年。与术前比较，术后即刻肋骨头脱入椎管内平均程度减小（术前32．8％±9．9％，术后16．8％±15．2％，t=3．269，P=-0．026）；肋骨进入椎管平均角度增大（术前34．7°±16．4°，术后47．8°±17．5°，t=5．423，P=0．001）；双侧肋骨成角减小（术前83．0°±19．5°，术后67．9°±13.3°，t=3．441，P=0．009）。术后即刻冠状面Cobb角和矢状面Cobb角较术前减小（术前70．7°±17．7°，术后35．4°±17．0°，t=6．850，P=0．000；术前59．7°±17．6°，术后24．7°±10．8°，t=5．986，P=0．001）；术后即刻与末次随访比较冠状面及矢状面Cobb角的差异均无统计学意义。术后及随访期间患者神经功能均保持为FrankelE级。结论对有肋骨头脱入椎管但未压迫脊髓的I型神经纤维瘤病性脊柱侧凸患者，在不切除肋骨头的情况下行脊柱侧凸矫形术安全有效。

Objective To observe the safety and efficacy of the posterior spinal instrumentation （PSI） with preserving of the intraspinal rib head in neurologically intact patients with scoliosis secondary to Neurofibromatosis type 1 （NF1）. Methods The clinical and radiographic data of nine NF1 scoliosis patients with rib head protrusion into the spinal canal, who had undergone PSI from August 1998 to March 2012, were retrospectively investigated. The average age of these patients （4 males, 5 females） was 15.4±7.6 years, and their neurological status all were Frankel grade E preoperatively. The intraspinal rib head was not resected in all patients intraoperatively. The following parameters, including the magnitude of rib head penetration into the spinal canal （MRPC）, the angle between the bilateral rib （ABR）, the angle between the dislocated rib and the posterior vertebral wall （ARV）, the coronal Cobb angle and the sagittal Cobb angle, were measured before and after surgery. Results The follow-up period was 0.5 to 4.8 years. The average MRPC decreased from preoperative 32.8%±9.9% to postoperative 16.8%±15.2% （P= 0.026）; the average ARV increased from preoperative 34.7°±16.4° to postoperative 47.8°±17.5° （P=0.O01）; the average ABR decreased from preoperative 83.0°±19.5° to postoperative 67.9°±13.3° （P=0.009）; the average coronal Cobb angle decreased from preoperative 70.7°±17.7° to 35.4°±17.0° immediately after operation （P=0.000） and the sagittal Cobb angle decreased from preoperative 59.7°±17.6° to 24.7°±10.8° immediately after operation （P=0.001）. The coronal Cobb angle and sagittal Cobb angle had no significant change during follow-up period. The neurological status was Frankel grade E in all patients immediately after operation and at final follow-up. Conclusion For NF1 scoliosis patients with rib head penetration into the spinal canal without impingement of the spinal cord and neurological deficits, the deformity can be corrected safely and effectively without resecting the intraspinal rib head.