Treatment of Klippel-Feil syndrome with symptomatic atlantoaxial instability in a 7-year-old boy

Klippel-Feil syndrome (KFS) is a congenital deformity of the cervical spine. Clinical symptoms of KFS are reduced range of motion, short neck and low hairline. In adult KFS patients the deformity can lead to adjacent segmental instability with spinal canal stenosis, radiculopathy and myelopathy. This article reports about the diagnostics and treatment management of juvenile KFS patient with myelopathy due to instability of the C1/C2 segment, subsequent stenosis through the posterior arch of C1 and symptomatic myelopathy. This 7‑year-old boy could be successfully treated with C1 decompression and computer tomography (CT) guided C1/C2 stabilization with pedicle screws under intraoperative neuromonitoring.