The embryologic origins of congenital abnormalities of the odontoid process are reviewed. Four cases of odontoid dysgenesis in childhood are presented. All of our cases were associated with a head injury, which subsequently led to the diagnosis. Pain was the most common presenting complaint in our patients. Decreased cervical mobility was the most consistent physical finding. There was no major neurological defect implicating spinal cord compression in any of our patients. This is not true of adults with congenital abnormalities of the odontoid, particularly those with atlantoaxial instability. The diagnosis of odontoid dysgenesis is a radiographic one. Flexion-extension films, tomograms and cineradiograms are often required to make the diagnosis. C1–C2 subluxation, either anterior or posterior is often found. Prompt posterior cervical fusion is recommended as the treatment of choic
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