多发颈椎不连综合征的影像学特点与外科诊疗策略

Radiological features and treatment strategy of multilevel cervical disconnection syndrome

目的:分析多发颈椎不连综合征(multilevel cervical disconnection syndrome,MCDS)的影像学特点及外科诊疗策略。方法:2004年3月~2021年6月,我院收治MCDS患者共7例,男性3例,女性4例;年龄5~46岁(中位年龄12岁)。7例MCDS患者中,平均椎体发育不良节段数3.6±1.3个节段,平均椎弓不连节段数5.7±1.5个节段,局部后凸角平均-92.2°±20.2°,C2-7 Cobb角平均-68.6°±31.0°,T1倾斜角(T1 slope,T1S)平均-12.5°±12.5°,后凸顶点位于C4节段1例,C5节段5例,T1节段1例;术前改良日本骨科学会评分(mJOA评分)8.5~14分(平均12.6±2.1分),其中1例患者伴有吞咽困难。记录患者预矫形方式及手术方式,入院时、预矫形后、术后及末次随访影像学参数,神经功能及并发症。结果:1例术前接受颅骨牵引,3例接受平衡悬吊牵引,3例接受联合牵引,经术前牵引预矫形后,局部后凸角矫正率为60.8%。1例接受手术松解、Halo-vest外固定治疗,1例接受前路矫形内固定手术,1例接受后路矫形固定融合术,4例接受前-后联合手术治疗,手术固定6.0±2.1个节段,2例患者出现术后神经系统并发症,接受翻修手术。术后随访时间6~84个月(41.2±32.0个月),末次随访局部后凸角平均-27.9°±11.6°,矫正率69.7%,C2-7 Cobb角平均-13.3°±28.4°,T1S平均4.9°±17.9°;术后mJOA评分10.5~17分(15.7±2.3分),改善率78.3%。对比手术前后临床及影像学指标,mJOA评分、C2-7后凸角、局部后凸角及T1S有统计学差异。结论:MCDS影像学上主要表现为前方椎体发育不良伴多节段椎弓不连,继发严重后凸畸形。治疗策略可采取术前牵引预矫形并前路多节段椎体切除重建、后路长节段固定融合。

Objectives:To analyze and discuss the radiological manifestation and surgical treatment strategy of multilevel cervical disconnection syndrome(MCDS).Methods:Between March 2004 and June 2021,7 patients with MCDS were treated in our institute.There were 3 males and 4 females,aging from 5 to 46 years old(median:12 years old).Among them,dysplasia of vertebral body averaged 3.6±1.3 levels and segments with anterior-posterior disconnection averaged 5.7±1.5 levels.The mean local kyphotic angle was-92.2°±20.2°,mean C2-7 Cobb angle was-68.6°±31.0°,mean T1 slope(T1 S)was-12.5°±12.5°.The apex was located at C4 in 1 patient,C5 in 5 patients,and T1 in 1 patient.The mJOA score ranged from 8.5 points to 14 points(average:12.6±2.1 points),with 1 patient accompanied by dysphagia.The methods and techniques for pre-correction and surgical treatment,radiological parameters when admitted,instant after surgery and during the final follow-up,and the neurological functions and perioperative complications were recorded.Results:For preoperative correction,one patient received skull traction,three received suspensory correction,and three received combined traction.The correction rate was 60.8%.The average fusion level was6.0±2.1 levels.One patient received anterior soft-tissue release combined with Halo-vest,one received anterior correction and fusion,one received posterior correction and fusion,and 4 received anterior-posterior combined approach.Two patients encountered neurological complication and received revision surgery.The follow-up period ranged from 6 to 84 months(mean:41.2±32.0 months).During the final follow-up,the mean local kyphotic angle was-27.9°±11.6°,correction rate was 69.7%,mean C2-7 Cobb angle was-13.3°±28.4°,mean T1 S was 4.9°±17.9°.mJOA score ranged from 10.5 to 17 points(15.7±2.3 points),and the recovery rate was 78.3%.Comparing the clinical and radiological parameters before and after operation,the mJOA score,C2-7 Cobb angle,local kyphotic angle,and T1 slope were with significant differences.Conclusions:The radiological features of MCDS were dysplasia of vertebral body and multilevel disconnection of pedicle,causing severe kyphotic deformity.Initial treatment should be pre-correction by traction.Surgical treatment should include anterior multilevel corpectomy and correction,combined with posterior long segment fusion.