Klippel-Feil syndrome: patterns of bony fusion and wasp-waist sign

Cervical spine radiographs of 33 patients with Klippel-Feil syndrome were studied for patterns of bony fusion and presence of wasp-waist sign. Five patients were found to have the classic features of massive cervical fusion. Two patients with two adjacent levels of bony fusion showed a wasp-waist sign. Fusion at one level of the cervical spine accounted for 26 remaining cases. The wasp-waist sign was observed in 14 of the cases in which there was complete vertebral interbody fusion, making this finding a valuable radiologic sign. Partial anterior or posterior vertebral interbody fusion, or isolated fusion of the neural arch, however, may or may not be associated with the wasp-waist sign. Klippel-Feil syndrome, easy to recognize when presenting with classic features or when associated with the wasp-waist sign, may be confused with a variety of other entities.