Nouri Aria, Martin Allan R, Lange Stefan F, Kotter Mark R N, Mikulis David J, Fehlings Michael G
Krembil Research Institute, Toronto Western Hospital and University of Toronto, University Health Network, Toronto, Canada.
Krembil Research Institute, Toronto Western Hospital and University of Toronto, University Health Network, Toronto, Canada.
World Neurosurg. 2017 Apr;100:531-539. doi: 10.1016/j.wneu.2017.01.048. Epub 2017 Jan 24.
Congenital fusion of cervical vertebrae, including Klippel-Feil syndrome (KFS), is a suspected risk factor for development of degenerative cervical myelopathy (DCM). We aimed to establish prevalence and degenerative patterns of congenital cervical fusion (CCF) among a global cohort of patients with DCM.
Data from 3 prospective DCM studies were merged, including clinical data for 813 patients and imaging for 592 patients. CCF was diagnosed by presence of fused cervical vertebrae without signs of degenerative fusion. A wasp-waist sign was used to define a KFS subgroup. Characteristics of patients with CCF and the KFS subgroup were compared with the remainder of patients with DCM.
Twenty-three patients with CCF (14 KFS) were identified, indicating a prevalence of 3.9% (2.4% KFS). Patients with CCF were older (P = 0.02), had more operated levels (P = 0.01), had higher rates of ossified posterior longitudinal ligament (P = 0.02), and demonstrated worse degenerative changes at C3-4, including spinal cord compression (P = 0.002) and T weighted image T2WI signal hyperintensity (P = 0.04). Levels adjacent to fusions showed a trend toward increased spinal cord compression (P = 0.09), with fusions at C3-4 or above showing cord compression below in 9 of 10 patients, fusions at C5-6 or below having cord compression above in 8 of 8 patients, and fusions at C4-5 showed cord compression above and below in 2 of 2 patients.
The prevalence of CCF and KFS is higher in DCM than for the general population, suggesting that these patients are predisposed to DCM development. Patients with CCF also have an altered pattern of degenerative changes, seemingly related to adjacent segment degeneration that preferentially affects midcervical levels.
包括克-费综合征（KFS）在内的先天性颈椎融合是退行性颈椎病（DCM）发生的一个可疑危险因素。我们旨在确定全球DCM患者队列中先天性颈椎融合（CCF）的患病率和退变模式。
合并了3项DCM前瞻性研究的数据，包括813例患者的临床数据和592例患者的影像学资料。CCF通过存在无退变融合迹象的颈椎融合来诊断。用蜂腰征来定义KFS亚组。将CCF患者和KFS亚组患者的特征与其余DCM患者进行比较。
共识别出23例CCF患者（14例KFS），患病率为3.9%（KFS为2.4%）。CCF患者年龄较大（P = 0.02），手术节段更多（P = 0.01），后纵韧带骨化率更高（P = 0.02），并且在C3-4节段显示出更严重的退变改变，包括脊髓受压（P = 0.002）和T2加权像（T2WI）信号高增强（P = 0.04）。融合相邻节段显示出脊髓受压增加的趋势（P = 0.09），C3-4或以上节段融合的10例患者中有9例在下方出现脊髓受压，C5-6或以下节段融合的8例患者中有8例在上方出现脊髓受压，C4-5节段融合的2例患者在上方和下方均出现脊髓受压。
DCM患者中CCF和KFS的患病率高于一般人群，提示这些患者易患DCM。CCF患者也有退变改变模式的改变，似乎与优先影响颈中段水平的相邻节段退变有关。
