title: The odontoid process in Morquio-Brailsford's disease. The effects of occipitocervical fusion.
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.
authors.affiliation: National Hospital for Neurology and Neurosurgery, London, England.
authors.forename: J M
authors.initials: JM
authors.lastname: Stevens
authors.forename: B E
authors.initials: BE
authors.lastname: Kendall
authors.forename: H A
authors.initials: HA
authors.lastname: Crockard
authors.forename: A
authors.initials: A
authors.lastname: Ransford
mesh_terms: D000328:Adult; D001268:Atlanto-Axial Joint / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D004204:Joint Dislocations / Q000000981:diagnostic imaging / Q000209:etiology; D008297:Male; D009085:Mucopolysaccharidosis IV / Q000150:complications / Q000000981:diagnostic imaging*; D009192:Myelography; D009809:Odontoid Process / Q000002:abnormalities* / Q000000981:diagnostic imaging* / Q000601:surgery; D013117:Spinal Cord Compression / Q000000981:diagnostic imaging / Q000209:etiology; D013123:Spinal Fusion; D014057:Tomography, X-Ray Computed