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1型严重神经纤维瘤病合并先天性脊柱侧弯伴胸椎旋转半脱位的术前头环重力牵引

Pre-operative halo-gravity traction in severe neurofibromatosis type 1 and congenital scoliosis with thoracic rotatory subluxation.

作者信息

Shi Benlong, Xu Liang, Li Yang, Liu Zhen, Sun Xu, Zhu Zezhang, Qiu Yong

机构信息

Spine Surgery, the Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, China.

Spine Surgery, the Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, China.

出版信息

Clin Neurol Neurosurg. 2019 Dec;187:105548. doi: 10.1016/j.clineuro.2019.105548. Epub 2019 Oct 19.

Abstract

OBJECTIVES

To evaluate the efficacy and safety of pre-operative Halo-gravity traction in the treatment of severe neurofibromatosis type 1 and congenital scoliosis patients with thoracic rotatory subluxation.

PATIENTS AND METHODS

Patients with neurofibromatosis type 1 and congenital scoliosis undergoing Halo-gravity traction were reviewed. Radiographic parameters were measured at pre-, post-traction and post-operation. The forced vital capacity and forced expiratory volume in 1 s were recorded at pre- and post-traction. The neurologic function were assessed according to the Frankel score. The complications during Halo-gravity traction, operation and post-operative follow-up were recorded.

RESULTS

A total of 35 patients (21M and 14F) with rotatory subluxation including 18 neurofibromatosis type 1 and 17 congenital scoliosis patients were included, of whom the average age was 14.9 ± 4.8 years. The average duration of Halo-gravity traction was 72.3 ± 11.2 days, during which the average Cobb angle improved from 105.4 ± 34.2° to 81.7 ± 32.6° (P < 0.001), and the global kyphosis decreased from 79.2 ± 22.5° to 59.7 ± 23.0° (P = 0.003). At pre-traction, the values of coronal and sagittal rotatory subluxation were 9.3 ± 5.2 mm and 7.5 ± 3.5 mm, which significantly improved to 6.7 ± 3.6 mm (P < 0.001) and 4.9 ± 2.3 mm (P < 0.001), respectively. The average improvement in forced vital capacity and forced expiratory volume in 1 s were from 43.6% to 54.2% predicted and from 40.4% to 48.8% predicted, respectively. After Halo-gravity traction, the Frankel scores improved from C to D in 3 patients, from D to E in 2 patients.

CONCLUSION

Halo-gravity traction can improve the coronal and sagittal curvature, and the rotatory subluxation in neurofibromatosis type 1 and congenital scoliosis patients. The pre-operative Halo-gravity traction is a safe option for severe neurofibromatosis type 1 and congenital scoliosis patients with rotatory subluxation.

摘要

目的

评估术前头环重力牵引治疗重度1型神经纤维瘤病合并先天性脊柱侧凸并伴有胸椎旋转半脱位患者的疗效和安全性。

患者与方法

对接受头环重力牵引的1型神经纤维瘤病和先天性脊柱侧凸患者进行回顾性研究。在牵引前、牵引后及术后测量影像学参数。记录牵引前和牵引后的用力肺活量和1秒用力呼气量。根据Frankel评分评估神经功能。记录头环重力牵引、手术及术后随访期间的并发症。

结果

共纳入35例(男21例,女14例)伴有旋转半脱位的患者,其中18例为1型神经纤维瘤病患者,17例为先天性脊柱侧凸患者,平均年龄为14.9±4.8岁。头环重力牵引的平均持续时间为72.3±11.2天,在此期间,平均Cobb角从105.4±34.2°改善至81.7±32.6°(P<0.001),整体后凸从79.2±22.5°降至59.7±23.0°(P=0.003)。牵引前,冠状面和矢状面旋转半脱位值分别为9.3±5.2mm和7.5±3.5mm,分别显著改善至6.7±3.6mm(P<0.001)和4.9±2.3mm(P<0.001)。用力肺活量和1秒用力呼气量的平均改善幅度分别从预测值的43.6%提高至54.2%,从预测值的40.4%提高至48.8%。头环重力牵引后,3例患者的Frankel评分从C级提高至D级,2例患者从D级提高至E级。

结论

头环重力牵引可改善1型神经纤维瘤病和先天性脊柱侧凸患者的冠状面和矢状面弯曲以及旋转半脱位。术前头环重力牵引对于重度1型神经纤维瘤病合并先天性脊柱侧凸并伴有旋转半脱位的患者是一种安全的选择。

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