先天性脊柱侧凸中的克利佩尔-费尔综合征。
Klippel-Feil syndrome in congenital scoliosis.
作者信息
Xue Xuhong, Shen Jianxiong, Zhang Jianguo, Tian Ye, Zhao Hong, Wang Yipeng, Liang Jinqian, Li Zheng, Qiu Guixing
机构信息
From the Department of Orthopedics, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Peking Union Medical College, Beijing, China.
出版信息
Spine (Phila Pa 1976). 2014 Nov 1;39(23):E1353-8. doi: 10.1097/BRS.0000000000000587.
STUDY DESIGN
Retrospective review.
OBJECTIVE
To investigate the clinical manifestations and radiological characteristics of Klippel-Feil syndrome (KFS) in congenital scoliosis (CS). To identify the incidence of spinal or extraspinal abnormities in KFS.
SUMMARY OF BACKGROUND DATA
KFS is an uncommon condition, characterized as improper segmentation of one or more cervical spine segments. "Scoliosis" is potentially the most common manifestation associated with KFS. However, the clinical manifestations and radiological characteristics of KFS in patients with CS are less reported.
METHODS
A total of 516 patients with CS from January 2009 to March 2013 were identified from a single institution. The demographic distribution and clinical and radiographical data were collected. Cervical regions were also designated as high (O-C2), mid (C2-C4), and low (C4-T1). The incidence of intra- and extraspinal abnormalities associated with KFS was investigated.
RESULTS
In total, 28 patients (5.42%) had been identified with KFS, which included 8 males and 20 females. The mean coronal cervical alignment was 20.6° and sagittal alignment was 29.9°. KFS type I was found in 14 patients (50.0%), type II in 6 (21.4%), and type III in 8 (28.6%). Congenitally fused cervical segment is more common in the mid and lower cervical spine region (85.7%, 24/28). In the 28 patients with KFS, 11 have intraspinal anomalies (32.1%) and 6 have extraskeletal anomalies (21.4%). Thirteen patients (46.4%) exhibited rib anomalies. The incidence of rib anomalies showed no significant difference in CS patients with KFS and without KFS (P>0.05). A half of the patients with KFS have hemivertebrae; however, the incidence of hemivertebrae showed no significant difference in CS patients with KFS and without KFS (P>0.05).
CONCLUSION
The incidence of KFS was 5.42% in patients with CS. Congenitally fused cervical patterns are more common in the mid and lower cervical spine region. The incidence of rib anomalies, intraspinal abnormities, and hemivertebra was not increased in CS patients with KFS.
LEVEL OF EVIDENCE
研究设计
回顾性研究。
目的
探讨先天性脊柱侧凸（CS）中Klippel-Feil综合征（KFS）的临床表现及影像学特征。确定KFS患者脊柱或脊柱外异常的发生率。
背景资料总结
KFS是一种罕见疾病，特征为一个或多个颈椎节段的分割异常。“脊柱侧凸”可能是与KFS相关的最常见表现。然而，CS患者中KFS的临床表现及影像学特征报道较少。
方法
从单一机构中确定了2009年1月至2013年3月期间的516例CS患者。收集了人口统计学分布以及临床和影像学数据。颈椎区域也分为高位（枕骨-颈2）、中位（颈2-颈4）和低位（颈4-胸1）。调查了与KFS相关的脊柱内和脊柱外异常的发生率。
结果
总共28例患者（5.42%）被诊断为KFS，其中男性8例，女性20例。颈椎冠状面平均对线角度为20.6°，矢状面平均对线角度为29.9°。发现14例（50.0%）为I型KFS，6例（21.4%）为II型，8例（28.6%）为III型。先天性融合颈椎节段在颈椎中下段更常见（85.7%，24/28）。在28例KFS患者中，11例有脊柱内异常（32.1%），6例有骨骼外异常（21.4%）。13例患者（46.4%）表现出肋骨异常。KFS的CS患者和无KFS的CS患者中肋骨异常的发生率无显著差异（P>0.05）。一半的KFS患者有半椎体；然而，KFS的CS患者和无KFS的CS患者中半椎体的发生率无显著差异（P>0.05）。
结论
CS患者中KFS的发生率为5.42%。先天性融合颈椎模式在颈椎中下段更常见。KFS的CS患者中肋骨异常、脊柱内异常和半椎体的发生率没有增加。
证据水平
4级。
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