The odontoid process in Morquio-Brailsford's disease. The effects of occipitocervical fusion
- 1 September 1991
- journal article
- Published by British Editorial Society of Bone & Joint Surgery in The Journal of Bone and Joint Surgery. British volume
- Vol. 73-B (5) , 851-858
- https://doi.org/10.1302/0301-620x.73b5.1910048
Abstract
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.Keywords
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