Craniovertebral junction database analysis: incidence, classification, presentation, and treatment algorithms

The initial treatment of bony lesions at the craniocervical junction consisted of posterior decompression with enlargement of the foramen and removal of the posterior arch of the atlas and axis vertebra. This was regardless of the site of compression. Needless to say, the results were poor when an irreducible ventral lesion at the craniocervical junction caused compression of the cervicomedullary area. It was predictable that an adverse outcome would occur in approximately 35–40% of patients treated with dorsal decompression with a fixed anterior abnormality at the craniovertebral junction. Thus, it became essential to identify the causes of failure and recognize the pathology as well as attempt to improve the treatment. The need to identify natural history led to a prospective database. This database now lists 5,300 patients who were analyzed. Of these, 2,000 were children and this has been reviewed here.

Review of symptoms showed that children with craniovertebral abnormalities present with failure to thrive, weakness, basilar migraine, dysphagia, sleep apnea, scoliosis, and the cervical central cord syndrome. Torticollis was seen in the very young.

New imaging techniques were utilized as they became available. Unexplained neurological symptoms and signs associated with craniovertebral abnormalities in children required angiography which was performed with dynamic motion studies. All children with reducible craniocervical abnormalities underwent stabilization. In irreducible abnormalities, decompression was paramount with a fusion to be done immediately. The decompression was accomplished in the direction in which encroachment occurred.

A treatment algorithm was formulated as a result of this database and a classification of the craniovertebral abnormalities; that holds good for the entire spectrum.