Congenital abnormality of the cervical spine characterised by deformity & fusion of adjacent vertebral segments & may be associated with local cervical instability.
Includes all variations of congenital failure of segmentation from simple fusion of adjacent vertebrae to involvement of the entire spine
Aetiology
Developmental abnormality with failure of segmentation occuring during the third to eighth week of intrauterine life & associated with a number of other congenital abnormalities.
Clinically
Classical clinical triad
Short neck
Low hairline
Limitation of neck motion
Associations
scoliosis (60%)
more than 1/2 of these require treatment
Sprengel’s deformity (40%)
Renal anomalies – e.g. horseshoe kidney (35%)
Hearing impairment (30%)
Congenital heart disease (14%)
Motion patterns
Upper cervical spine instability & neurologic problems- most common with upper cervical anomalies e.g. C2-3 fusion
Lower cervical spine degenerative osteoarthritis
X-Rays
Posterior fusion first to be recognised
Flexion extension views best for identifying the fused segment & the site of maximal motion
Vertebral endplates often appear falsely as disc space
Prognosis:
Greater than four fused segments is associated with a higher incidence of problems & instability
