Klippel-Feil Syndrome in Congenital Scoliosis
Klippel-Feil Syndrome in Congenital Scoliosis
复制标题
先天性脊柱侧弯中的 Klippel-Feil 综合征
DOI：
10.1097/brs.0000000000000587
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发表时间：
2014-11-01
期刊：
影响因子：
3
通讯作者：
Qiu, Guixing
中科院分区：
文献类型：
--
作者：
Xue, Xuhong;Shen, Jianxiong;Qiu, Guixing
研究设计。回顾回顾：客观的。目的探讨先天性脊柱侧弯(CS)合并Klippel-Feil综合征(KFS)的临床表现和放射学特点。确定KFS脊柱或椎外异常的发生率。背景资料摘要。KFS是一种罕见的疾病，其特征是一个或多个颈椎节段分割不当。“脊柱侧弯”可能是KFS最常见的表现。然而，有关CS患者的KFS的临床表现和放射学特征的报道较少。从2009年1月至2013年3月，从一个机构确认了总共516名CS患者。收集人口学分布、临床和放射学资料。颈部也被指定为高(O-C2)、中(C2-C4)和低(C4-T1)。调查与KFS相关的椎管内和椎外异常的发生率。共确诊KFS患者28例(5.42%)，其中男性8例，女性20例。颈椎冠状位平均为20.6°，矢状位为29.9°。KFSI型14例(50.0%)，II型6例(21.4%)，III型8例(28.6%)。先天性颈段融合多见于中、下颈段(85.7%，24/28)。28例KFS患者中，椎管内异常11例(32.1%)，骨外异常6例(21.4%)。肋骨异常13例(46.4%)。合并KFS和不伴KFS的CS患者肋骨异常发生率无显著差异(P>；0.05)。一半的KFS患者有半椎体，而CS患者有KFS组和无KFS组的半椎体发生率差异无统计学意义(P>；0.05)。CS患者中KFS的发生率为5.42%。先天融合的颈椎模式在中、下颈椎区域更为常见。在合并KFS的CS患者中，肋骨异常、椎管内异常和半椎体的发生率没有增加。
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 degrees and sagittal alignment was 29.9 degrees. 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.