Theiss S M, Smith M D, Winter R B
Division of Orthopaedic Surgery, University of Alabama at Birmingham, USA.
Spine (Phila Pa 1976). 1997 Jun 1;22(11):1219-22. doi: 10.1097/00007632-199706010-00009.
This study evaluated the long-term results of Klippel-Feil syndrome in patients with congenital scoliosis.
To determine the incidence of cervical and cervical-related symptoms of patients who have Klippel-Feil syndrome associated with congenital scoliosis.
Many authors have described the association of Klippel-Feil syndrome and congenital scoliosis. In this population of patients, cervical lesions often are discovered incidentally. The significance of these lesions is unknown.
Thirty-two patients with congenital scoliosis and Klippel-Feil syndrome were observed for more than 10 years. They were questioned specifically about cervical and cervical-related symptoms. All patients had sequential cervical radiographs and physical examinations.
Despite rather dramatic radiographic appearances, only seven (22%) of the 32 patients had cervical or cervical-related symptoms, with two patients requiring surgery for their cervical lesions. The extent of the deformities and the average number of cervical vertebrae fused and cervical fusion-patterns were statistically similar between the symptomatic and asymptomatic groups. Patients fused to the cervicothoracic junction for management of their deformities had a significantly increased incidence of cervical symptoms. Also, patients with congenital stenosis had a significantly greater incidence of upper extremity pain.
Only a small number of patients with Klippel-Feil syndrome and congenital scoliosis developed cervical symptoms. No fusion pattern that placed the patient at greater risk for developing symptoms could be identified. Factors that did lead to a greater incidence of cervical symptoms were fusion to the cervicothoracic junction and congenital cervical stenosis.
本研究评估了先天性脊柱侧凸患者中Klippel-Feil综合征的长期结果。
确定患有与先天性脊柱侧凸相关的Klippel-Feil综合征患者的颈部及颈部相关症状的发生率。
许多作者描述了Klippel-Feil综合征与先天性脊柱侧凸的关联。在这类患者群体中，颈部病变常常是偶然发现的。这些病变的意义尚不清楚。
对32例先天性脊柱侧凸合并Klippel-Feil综合征的患者进行了超过10年的观察。专门询问了他们关于颈部及颈部相关症状的情况。所有患者均接受了连续的颈椎X线检查和体格检查。
尽管影像学表现相当显著，但32例患者中只有7例（22%）有颈部或颈部相关症状，其中2例因颈部病变需要手术治疗。有症状组和无症状组在畸形程度、融合的颈椎平均数量以及颈椎融合模式方面在统计学上相似。为治疗畸形而融合至颈胸交界处的患者颈部症状发生率显著增加。此外，患有先天性狭窄的患者上肢疼痛发生率显著更高。
只有少数患有Klippel-Feil综合征和先天性脊柱侧凸的患者出现颈部症状。未发现哪种融合模式会使患者出现症状的风险更高。确实导致颈部症状发生率更高的因素是融合至颈胸交界处和先天性颈椎狭窄。
