Roberts S B, Dryden R, Tsirikos A I
Royal Hospital for Sick Children, Sciennes Road, Edinburgh, EH9 1LF, UK.
Scottish National Spine Deformity Centre, Royal Hospital for Sick Children, Sciennes Road, Edinburgh, EH9 1LF, UK.
Bone Joint J. 2016 Feb;98-B(2):229-37. doi: 10.1302/0301-620X.98B2.36144.
Clinical and radiological data were reviewed for all patients with mucopolysaccharidoses (MPS) with thoracolumbar kyphosis managed non-operatively or operatively in our institution.
In all 16 patients were included (eight female: eight male; 50% male), of whom nine had Hurler, five Morquio and two Hunter syndrome. Six patients were treated non-operatively (mean age at presentation of 6.3 years; 0.4 to 12.9); mean kyphotic progression +1.5(o)/year; mean follow-up of 3.1 years (1 to 5.1) and ten patients operatively (mean age at presentation of 4.7 years; 0.9 to 14.4); mean kyphotic progression 10.8(o)/year; mean follow-up of 8.2 years; 4.8 to 11.8) by circumferential arthrodesis with posterior instrumentation in patients with flexible deformities (n = 6).
In the surgical group (mean age at surgery of 6.6 years; 2.4 to 16.8); mean post-operative follow-up of 6.3 years (3.5 to 10.3), mean pre-operative thoracolumbar kyphosis of 74.3(o) (42(o) to 110(o)) was corrected to mean of 28.6(o) (0(o) to 65(o)) post-operatively, relating to a mean deformity correction of 66.9% (31% to 100%). Surgical complications included a deep wound infection treated by early debridement, apical non-union treated by posterior re-grafting, and stable adjacent segment spondylolisthesis managed non-operatively. Thoracolumbar kyphosis > +38(o) at initial presentation was identified as predicting progressively severe deformity with 90% sensitivity and 83% specificity.
This study demonstrates that severe thoracolumbar kyphosis in patients with MPS can be effectively treated by circumferential arthrodesis. Severity of kyphosis at initial presentation may predict progression of thoracolumbar deformity. Patients with MPS may be particularly susceptible to post-operative complications due to the underlying connective tissue disorder and inherent immunological compromise.
Clinical and radiological data were reviewed for all patients with mucopolysaccharidoses with thoracolumbar kyphosis managed non-operatively or operatively in our institution.
回顾性分析我院非手术或手术治疗的患有胸腰椎后凸畸形的黏多糖贮积症（MPS）患者的临床和影像学资料。
共纳入16例患者（8例女性，8例男性；男性占50%），其中9例患有Hurler综合征，5例患有Morquio综合征，2例患有Hunter综合征。6例患者接受非手术治疗（就诊时平均年龄6.3岁；0.4至12.9岁）；平均后凸进展为每年+1.5°；平均随访3.1年（1至5.1年），10例患者接受手术治疗（就诊时平均年龄4.7岁；0.9至14.4岁）；平均后凸进展为每年10.8°；平均随访8.2年（4.8至11.8年），对柔韧性畸形患者（n = 6）采用后路器械辅助下的环形融合术。
手术组（手术时平均年龄6.6岁；2.4至16.8岁）；术后平均随访6.3年（3.5至10.3年），术前平均胸腰椎后凸为74.3°（42°至1°），术后平均矫正至28.6°（0°至65°），平均畸形矫正率为66.9%（31%至100%）。手术并发症包括通过早期清创治疗的深部伤口感染、通过后路再次植骨治疗的顶椎不愈合以及非手术治疗的稳定的相邻节段椎体滑脱。初始就诊时胸腰椎后凸> +38°被确定为预测畸形逐渐加重的指标，敏感性为90%，特异性为83%。
本研究表明，MPS患者的严重胸腰椎后凸可通过环形融合术有效治疗。初始就诊时后凸的严重程度可能预测胸腰椎畸形的进展。由于潜在的结缔组织疾病和固有的免疫功能受损，MPS患者可能特别容易发生术后并发症。
回顾性分析我院非手术或手术治疗的患有胸腰椎后凸畸形的黏多糖贮积症患者的临床和影像学资料。
