Klippel-Feil Syndrome

Definition: Klippel-Feil Syndrome is a condition characterized by abnormal segmentation of the vertebrae in the cervical spine (neck region). Patients with Klippel-Feil have fused cervical vertebrae. Klippel-Feil was initially described prior to x-rays, therefore the original descriptions of classic Klippel-Feil consisted of the manifestations of:

• Decreased range-of-motion of the neck,

• A short, "webbed" neck, and

• A low hairline.

Patients with Klippel-Feil commonly have associated congenital anomalies such as scoliosis, kidney disease, and Sprengel's deformity. Treatment is usually observational, with surgery needed in a small number of patients who have chronic neck problems. Children with Klippel-Feil are instructed to avoid contact sports.