We performed posterior fixation with a Hartshill-Ransford contoured loop in 43 patients with instability at the craniocervical junction. No external bracing was used. Fifteen patients had congenital malformations, ten had tumours, seven had 'bone-softening' conditions (such as osteogenesis imperfecta), five had suffered complicated fractures, three had occipito-C1-C2 hypermobility due to lax ligaments and three had severe degenerative spondylosis with pseudotumours of the transverse ligament. Twenty-nine patients had transoral decompression of the cord before fixation. In most cases, cancellous bone grafts taken from the iliac crest were used to induce fusion; in nine very ill patients, no bone graft was used. In the whole series there was no instance of construct failure, broken wire or laminar fracture. The best results were achieved in patients with tumours or bone-softening conditions. No patient with normal neurology deteriorated after surgery but seven had worse neurological deficits after operation than before. Neck stiffness caused half the patients to change their lifestyle.

Fractures of the atlas constitute 4% to 12% of all bony injuries of the cervical spine; most are treated successfully by a cervical orthosis. Nonunion may be associated with neck or scalp pain on movement and is treated conventionally by some form of craniocervical fusion, which restricts head movement. The authors describe a case in which direct repair of the bony ring with a titanium plate and screws allowed bone healing, relieved the symptoms and maintained a full range of neck movements. The titanium plate interfered little with postoperative MR and CT imaging.

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.

An operation which combined anterior transoral decompression with posterior occipitocervical fixation was used in 68 rheumatoid patients with irreducible anterior neuraxial compression at the craniocervical junction. Fibre-optic laryngoscopy with nasotracheal intubation was less hazardous than tracheostomy. The patients underwent surgery in the lateral position to allow access both to the mouth and to the back of the neck without moving the head. Specially designed instruments allowed visualisation from the front without dividing the soft palate. Posterior stabilisation was achieved by a preformed contoured loop fixed to the occiput, the atlas and the axis by sublaminar wires. The procedure allowed immediate mobilisation and had a very low morbidity in such ill patients.

We report three patients having transoral fusion at C2/3 or C3/4 after fractures, with no infections or surgical complications and sound union. The operative technique and the relative merits of different approaches to the upper cervical spine are discussed and the transoral approach to the anterior aspect of the upper three cervical vertebrae is commended to the specialist surgeon.

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.

Rigid posterior fixation of the skull to the third, fourth and fifth cervical vertebrae was achieved in three patients who, as a result of operation, had gross instability of the craniocervical junction. An anatomically contoured steel loop was secured to the occiput via small burr holes and to the vertebrae by sublaminar wiring. This technique has the advantage over bone grafting, either alone or with cement, in that it affords rigid stabilisation, allows early mobilisation and may contribute to eventual bony fusion.

Basilar impression is a well-recognised though rare complication of osteogenesis imperfecta. Three patients, all members of the same family, with advanced basilar impression complicating osteogenesis imperfecta tarda, are described. The clinical features in these cases illustrate the natural history of this condition: from asymptomatic ventricular dilatation, through the foramen magnum compression syndrome, to death from brain-stem compression. The radiological criteria on which the diagnosis is based, are defined. Review of the literature reveals only seven previously documented cases, all in patients with mild forms of osteogenesis imperfecta. The unusually low incidence of basilar impression in osteogenesis imperfecta and its apparent restriction to patients with mild forms of the disease is discussed. The examination of close relatives of patients with basilar impression and osteogenesis imperfecta is emphasised in order to anticipate the onset of severe neurological complications.