A new appraisal of abnormalities of the odontoid process associated with atlanto-axial subluxation and neurological disability.

It is well known that abnormalities of the odontoid process may be associated with subluxation and neuraxial compression. Modern computerized imaging is demonstrating that existing classifications are in need of revision since they no longer reflect current views on development, nor do they adequately explain the patterns of abnormality commonly encountered in clinical practice. This study is a description of 62 varied clinical cases examined by high definition computerized myelography or MRI in flexion and extension, some before and after stabilization procedures. From these descriptions, and a review of the old and more recent literature concerning phylogenesis and ontogenesis of the odontoid, the following conclusions were drawn. The dens within the atlas ring is not morphologically a centrum of the first cervical vertebra, but a projection arising from it. In os odontoideum the dens forms normally, but ossifies abnormally because of abnormal motion; it is a result rather than the cause of instability. Hypoplasia of the dens is usually incorrectly diagnosed. When present it is associated with atlanto-occipital assimilation, fusion of the second and third, or more, cervical vertebrae, loss or reduction in axial rotation of the head, basilar invagination and hindbrain deformity of Chiari type. Therefore it is part of a regional anomaly involving hypoplasia of the derivatives of the occipital and upper cervical somites. Recognition of these aspects simplifies both diagnosis and the formulation of therapeutic strategies for different types of clinical presentation.