High definition computed cervical myelograms have been made in flexion and extension in 13 patients with Morquio-Brailsford's disease. We observed that: 1) odontoid dysplasia was present in every case, with a hypoplastic dens and a detached distal portion which was not always ossified; 2) atlanto-axial instability was mild, and anterior atlanto-axial subluxation was absent in most cases; 3) severe spinal cord compression, when present, was due to anterior extradural soft-tissue thickening; 4) this compression was not relieved by flexing or extending the neck and was manifested early in life; 5) posterior occipitocervical fusion resulted in disappearance of the soft-tissue thickening and normalisation of subsequent development of the dens. We conclude that the severity of neurological involvement at the craniovertebral junction was determined by soft-tissue changes, not by the type of odontoid dysplasia nor by subluxation. Posterior occipitocervical fusion proved to be an effective treatment.

Cervical myelopathy is an uncommon but potentially fatal complication of rheumatoid atlanto-axial subluxation. Computerised myelotomography with three-dimensional reconstruction shows that rheumatoid pannus, together with the odontoid peg, contributes significantly to anterior cervico-medullary compression. These findings were the basis for treatment by transoral anterior decompression and posterior occipitocervical fusion, which removes both bony and soft-tissue causes of compression and allows early mobilisation without major external fixation. We report encouraging results from this combined approach in 14 patients who had progressive neurological deterioration.

Recent surveys have shown that idiopathic structural scoliosis of mild degree is generally not progressive. We will propose a mechanism which may be responsible for deterioration in the few. It has been observed that the spinal cord, although displaced towards the concavity, does not rotate in company with the vertebrae, thus exposing the emerging nerve roots to the effects of traction and possibly of entrapment. We suggest that progression occurs when the neuraxis is unable to adjust to the change in the anatomy of vertebral column. Our proposition is based upon our findings in a complete spinal column obtained from a baby with structural scoliosis. Support is provided by intercostal angiography, and by observations upon normal anatomy, the pathological anatomy of mature scoliotic spines and the anatomy of contrived scoliosis in normal spines. Although our histological and electrophysiological investigations are incomplete we can demonstrate a significant increase in degenerate cells in the dorsal root ganglia at the apex on the convex side. Lack of suitable necropsy material prevents us from confirming our observations so that our report is inevitably preliminary. We enter a plea that careful examination of the neuraxis be undertaken whenever a specimen of a scoliotic spine becomes available.