In 17 patients (eleven males, six females) with Morquio-Brailsford syndrome (mucopolysaccharidosis IV) we have used onlay femoral and tibial autografts placed posteriorly and secured to the laminae of C1 and C2 to obtain satisfactory occipito-C1/C2 posterior fusion. They were immobilised postoperatively in a halo-plaster body jacket for four months. The age at operation varied between three and 28 years. Those with myelopathic symptoms of recent onset made some recovery, but severely myelopathic patients showed little or no recovery. We advise prophylactic occipitocervical fusion in these patients since the cartilaginous dens is not strong enough to ensure atlanto-axial mechanical stability

We present seven children with atlantoaxial rotatory fixation (AARF) of more than three months’ duration after an injury to the upper cervical spine. The deformity was irreducible by skull traction. MRI and MR angiography (MRA) of the vertebral arteries were performed in four children. The patients were neurologically intact. Thrombosis of the ipsilateral vertebral artery was noted in two patients. The deformity was gradually corrected and stabilised after transoral release of the atlantoaxial complex, skull traction and posterior atlantoaxial fusion. Soft-tissue interposition and contractures within the atlantoaxial complex prevented closed reduction. MRI and MRA of the vertebral arteries were useful in elucidating the pathology of chronic atlantoaxial rotatory fixation

The accurate assessment of skeletal maturity is essential in the management of orthopaedic conditions in the growing child. In order to identify the time of peak height velocity (PHV) in adolescents, two systems for assessing skeletal maturity have been described recently; the calcaneal apophyseal ossification method and the Sanders hand scores.

The purpose of this study was to compare these methods in assessing skeletal maturity relative to PHV. We studied the radiographs of a historical group of 94 healthy children (49 females and 45 males), who had been followed longitudinally between the ages of three and 18 years with serial radiographs and physical examination. Radiographs of the foot and hand were undertaken in these children at least annually between the ages of ten and 15 years. We reviewed 738 radiographs of the foot and 694 radiographs of the hand. PHV was calculated from measurements of height taken at the time of the radiographs.

Prior to PHV we observed four of six stages of calcaneal apophyseal ossification and two of eight Sanders stages. Calcaneal stage 3 and Sanders stage 2 was seen to occur about 0.9 years before PHV, while calcaneal stage 4 and Sanders stage 3 occurred approximately 0.5 years after PHV.

The stages of the calcaneal and Sanders systems can be used in combination, offering better assessment of skeletal maturity with respect to PHV than either system alone.

Cite this article: Bone Joint J 2015;97-B:1710–17.

Aims

Although atlantoaxial rotatory fixation (AARF) is a common cause of torticollis in children, the diagnosis may be delayed. The condition is characterised by a lack of rotation at the atlantoaxial joint which becomes fixed in a rotated and subluxed position. The management of children with a delayed presentation of this condition is controversial. This is a retrospective study of a group of such children.

We report five children who presented at the mean age of 1.5 years (1.1 to 1.9) with a progressive thoracolumbar kyphosis associated with segmental instability and subluxation of the spine at the level above an anteriorly-wedged hypoplastic vertebra at L1 or L2. The spinal deformity appeared to be developmental and not congenital in origin. The anterior wedging of the vertebra may have been secondary to localised segmental instability and subsequent kyphotic deformity.

We suggest the term ‘infantile developmental thoracolumbar kyphosis with segmental subluxation of the spine’ to differentiate this type of deformity from congenital displacement of the spine in which the congenital vertebral anomaly does not resolve. Infantile developmental kyphosis with segmental subluxation of the spine, if progressive, may carry the risk of neurological compromise. In all of our patients the kyphotic deformity progressed over a period of three months and all were treated by localised posterior spinal fusion. At a mean follow-up of 6.6 years (5.0 to 9.0), gradual correction of the kyphosis was seen on serial radiographs as well as reconstitution of the hypoplastic wedged vertebra to normality. Exploration of the arthrodesis was necessary at nine months in one patient who developed a pseudarthrosis.

Our aim was to review the efficacy of the wound vacuum-assisted closure (VAC) system in the treatment of deep infection after extensive instrumentation and fusion for spinal deformity in children and adolescents.

A total of 14 patients with early deep spinal infection were treated using this technique. Of these, 12 had neuromuscular or syndromic problems. Clinical and laboratory data were reviewed. The mean follow-up was 44 months (24 to 72). All wounds healed. Two patients required plastic surgery to speed up the process. In no patient was the hardware removed and there was no loss of correction or recurrent infection.

We believe that the wound VAC system is a useful tool in the armamentarium of the spinal surgeon dealing with patients susceptible to wound infections, especially those with neuromuscular diseases. It allows for the retention of the instrumentation and the maintenance of spinal correction. It is reliable and easy to use.

Fractures of the odontoid in children with an open basilar synchondrosis differ from those which occur in older children and adults. We have reviewed the morphology of these fractures and present a classification system for them.

There were four distinct patterns of fracture (types IA to IC and type II) which were distinguished by the site of the fracture, the degree of displacement and the presence or absence of atlantoaxial dislocation. Children with a closed synchondrosis were classified using the system devised by Anderson and D’Alonzo. Those with an open synchondrosis had a comparatively lower incidence of traumatic brain injury, a higher rate of missed diagnosis and a shorter mean stay in hospital. Certain subtypes (type IA and type II) are likely to be missed on plain radiographs and therefore more advanced imaging is recommended. We suggest staged treatment with initial stabilisation in a Halo body jacket and early fusion for those with unstable injuries, severe displacement or neurological involvement.