Abstract

Purpose: To evaluate the clinical efficacy of preoperative and postoperative treatment of patients with Klippel-Feil syndrome (KFS).

Methods: 8 patients with KFS with cervical instability were selected as the subjects during April 2008 to June 2015, including 3 males and 5 females, who aged 25 - 45 years. These 8 patients were accompanied by cervical spine instability of C2-3 congenital fusion, surgical treatment, postoperative rehabilitation training and health education, regular review of imaging tests, physical examination, as well as assessment by Japanese Orthopaedic Association (JOA) and American Spinal Injury Association (ASIA) scoring systems.

Results: The 8 patients were successfully followed up for surgery. Statistical analysis results of all patients with lateral cervical spine, no internal fixation failure, were well graft fusion, in which 8 patients were no incontinence, no obvious spinal cord, nerve root, and vertebral artery injury. However, only 1 patient after 5 months showed recurrence of atlantoaxial dislocation, which led to the second hospitalization. The final postoperative score of JOA was significantly increased compared with the preoperative score, as well as the ASIA ensory score and exercise score. In one patient, a three dimensional (3D) printing guide plate was used to assist pedicle screw placement, which was accurate and safe.

Conclusion: Surgical treatment of KFS patients with cervical spine instability is still risky, however, surgical treatment can alleviate or cure the postoperative symptoms. Here, 8 patients were satisfied with the results of surgical treatment, therefore, the surgical treatment with cervical instability in patients with KFS is a highly effective treatment strategy.

Keywords: KFS; Upper Cervical Spine; Surgical Treatment; 3D Printing Guide Plate; Japanese Orthopaedic Association (JOA) Score

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