Cervical instability in Klippel-Feil syndrome: case report and review of the literature

Abstract

Background: The authors present a case of cervical myelopathy and radiculopathy in the setting of multiple Klippel-Feil syndrome abnormalities treated surgically with a single-level C3-C4 anterior cervical discectomy and fusion. We discuss the clinical presentation, radiographic findings, and various treatment options for cervical spine abnormalities in Klippel-Feil syndrome. Case Presentation: This 22-year-old female with Klippel-Feil syndrome presented with intermittent neck pain, left upper extremity weakness, and paresthesias. Preoperative MRI, CT, and X-rays of the cervical spine revealed anterolisthesis at C3/4 with unstable movement on flexion and extension imaging. In addition, there were multiple segmental fusion abnormalities including hemivertebrae and other congenital fusion abnormalities. A C3-C4 anterior cervical discectomy and fusion was performed with intervertebral disc spacer. Adequate decompression was achieved with postoperative resolution of the patient's symptoms and improvement in neurological exam. Conclusions: Single-level anterior cervical discectomy and fusion can be utilized for treatment of cervical myelopathy and radiculopathy in the setting of multiple congenital Klippel-Feil syndrome abnormalities.