ABSTRACT
Klippel-Feil syndrome (KFS) classically describes the patient with fused cervical vertebrae and the clinical triad of a short neck, low posterior hairline, and decreased range of motion of the neck. Although this constellation of attributes was originally recognized in 1912 (31,32), identification of the syndrome in additional patients facilitated development of a classification scheme based upon the extent of spinal fusion segments. Since only half of patients with KFS will have the classic triad, congenital fusion of two or more vertebrae has become synonymous with KFS. For example, patients with type I KFS have an extensive cervical and upper thoracic segmentation failure. In contrast, patients with type II KFS have only an isolated one-or two-segment fusion. Finally, patients with type III KFS have associated thoracic or lumbar fusion.
