Klippel–Feil Syndrome with Limited Rotation Range of Motion: Our Observation on Radiographic Evaluation of Cervical Spine Case Series

Abstract

Abstract Background: The aim is to identify radiographic changes in the cervical spine or occipitocervical junction in patients with restricted lateral bending and lateral rotation due to Klippel–Feil syndrome. It is important to consider this altered mobility or fusion when planning surgery for hypermobile segments. Observation: In Klippel–Feil syndrome, restricted rotation is typically associated with occipitocervical involvement, but it can also be caused by long fusion of the cervical vertebrae alone. If left untreated in early childhood, this fusion and the relatively mobile lower segments can lead to ligament fibrosis and degenerative changes. Materials and Methods: Nine patients with Klippel–Feil syndrome were clinically evaluated and underwent plain radiographs. Patients with significant concerns about neck motion underwent CT scans. Results: Two out of the nine patients were below the age of 14 years, while the rest were neglected cases. Radiologically, one patient had a single fused cervical segment, two had multiple contiguous cervical segment fusions, and the remaining patients had multiple non-contiguous segments fused. Conclusion: All included patients had rotational restriction, but not all had occipitalization of the cervical spine. It is believed that this may be due to prolonged fibrosis affecting the musculature. This finding can impact surgical planning for fusion of hypermobile segments, as it could further limit functional motion in neglected elderly patients who were previously doing well.