The long-term follow-up of patients with Klippel-Feil syndrome and congenital scoliosis.
METHODS
This study evaluated the long-term results of Klippel-Feil syndrome in patients with congenital scoliosis.


OBJECTIVE
To determine the incidence of cervical and cervical-related symptoms of patients who have Klippel-Feil syndrome associated with congenital scoliosis.


BACKGROUND
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.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Theiss
S M
SM
Division of Orthopaedic Surgery, University of Alabama at Birmingham, USA.
Smith
M D
MD
Winter
R B
RB
D000328:Adult; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007714:Klippel-Feil Syndrome / Q000453:epidemiology*; D008297:Male; D019547:Neck Pain / Q000453:epidemiology / Q000209:etiology; D011859:Radiography; D012600:Scoliosis / Q000151:congenital* / Q000000981:diagnostic imaging / Q000601:surgery; D013123:Spinal Fusion; D013997:Time Factors