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

中文关键词:  重度先天性颈椎后凸畸形  牵引预矫形  多发颈椎不连综合征

英文关键词:Severe congenital cervical kyphosis  Pre-correction by traction  Multilevel cervical disconnection syndrome

中文摘要:

  【摘要】 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。

英文摘要:

  【Abstract】 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(T1S) 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 was 6.0±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 T1S 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.