congenital short neck syndrome

congenital short neck syndrome,

a rare congenital malformation of the cervical spine in which the cervical vertebrae are fused, usually in pairs, into one mass of bone, causing decreased neck motion and decreased cervical length, sometimes with neurologic involvement. The posterior portion of the laminar arches in the cervical area is not fully developed; the result is spina bifida in the cervical region, usually involving the lower cervical vertebrae and, in some cases, one or more of the upper thoracic vertebrae. Congenital short neck syndrome is often associated with a cervical rib or with hemivertebrae. Neurologic complications, such as nerve-root compression and peripheral nerve symptoms, are secondary to deformities of the vertebral bodies. The extreme shortness of the neck is the most common sign of this deformity, which allows only limited motion, lateral bending, and rotation. When the deformity involves nerve-root compression, symptoms of peripheral nerve involvement, such as pain or a burning sensation, may be evident, accompanied by paralysis, hyperesthesia, or paresthesia. Involvement of the spinal cord may present signs of abnormalities of lower extremities with associated signs of an upper motor lesion. Congenital short neck syndrome may require no treatment. Mild associated symptoms may be alleviated with traction, cast application, or cervical collars. Surgery may be required to relieve neurologic manifestations. Also called Klippel-Feil syndrome.