Aims

The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management.

1. The indications for correction of severe rigid kyphosis by lumbar osteotomy are described. 2. The fatal complications in a series of a hundred cases are listed. With more experience of this operation they should be considerably reduced. 3. Among the non-fatal complications the low incidence of recurrence of the deformity severe enough to require further operation is noteworthy

1. Some of the factors responsible for vertebral growth have been discussed. 2. In kyphosis and scoliosis it is important to prevent progressive epiphysial damage. 3. In selected cases of progressive scoliosis, epiphysiodesis on the convex side will correct unequal growth. 4. The technique of spinal epiphysiodesis is described and the results that may be expected are discussed

1 . The theoretical requirements for optimal correction of thoracic scoliosis and kyphosis are discussed, and observations are made on the limitations of methods in current use for their ambulant correction. 2. The experimental use of hydrostatic pressure in an endeavour to satisfy these clinical requirements is described. 3. It is suggested that the use of hydrostatic pressure as the source of corrective force has considerable advantages, and that, with refinements, the techniques described may prove a useful addition to rigid mechanical methods of correction

This retrospective study assesses the complications affecting the cervical spine after halo-pelvic traction in 83 patients who were followed up for a minimum of five years. Forty-four patients (53 per cent) had significant cervical complications such as radiological degenerative changes, avascular necrosis of the dens, loss of movement, pain or spontaneous fusion. The most important predisposing factors were a long period in the halo-pelvic apparatus, tuberculous kyphosis, stiffness of the spinal deformity and an age of 15 years or more at the time of application

Acute myelopathy is a rare complication of Scheuermann's disease. Three patients are reported where spinal cord compression occurred at the apex of a kyphos. All were male, aged 14, 18 and 20 years, and each had a profound neurological defect associated with a short, sharp kyphos in the low thoracic region. Each patient underwent anterior decompression and all made an almost full recovery. It is deduced that factors which may influence the onset of cord compression include the angle of kyphosis, the number of segments involved, the rate of change of the angle, local anatomical variations, trauma, and possible secondary impairment of the vasculature of the cord

Five patients with classical Ehlers-Danlos syndrome developed severe spinal deformities. Two were shown to have type-VI collagen abnormalities. Three had a double structural scoliosis of the thoracic and lumbar regions, one had a single thoracic scoliosis and one had a thoracic kyphosis. The curves first developed before the age of four years, and were not controlled by bracing. Major corrective surgery with posterior fusion was performed at a mean age of 11 years 8 months. Excessive blood loss could be controlled and although wound haematoma and dehiscence were common, they did not provide major problems. The spinal fusions healed satisfactorily

Eight patients with neurofibromatosis presented with symptoms of cervical spine involvement over a period of 17 years, five of them within the second decade of life. The symptoms included neurological deficit in five, a neck mass in four, and deformity in three; only two complained of pain. Osteolysis of vertebral bodies with kyphosis of more than 90 degrees was the most common radiological feature. Posterior fusion failed in the one patient in whom it was performed. Good results were achieved by anterior fusion, alone, or combined with posterior fusion. Surgical complications included one death in a patient with a malignant neurofibroma, and one case of transient neurological deterioration

Twenty-eight patients with adolescent idiopathic scoliosis treated by anterior spinal fusion with Dwyer instrumentation were reviewed. The average length of follow-up was 6.9 years. This technique produced better correction of lateral curvature and rotation than Harrington instrumentation, particularly in the thoracolumbar and lumbar region. The length of spine requiring fusion was also shorter. There is, however, a tendency for Dwyer instrumentation to lead to kyphosis. Morbidity was significant and included one case of paraplegia, four cases of deep infection and one case of instrument failure. All of these complications, except one case of deep infection, occurred in patients with curves with an apex above the seventh thoracic vertebra

1. The etiology and natural progress of lumbar kyphosis in children from three to twelve years of age with myelomeningocele are reviewed. 2. The indications for operation have included intractable or recurrent skin ulceration, inability to wear calipers for walking, inability to sit in a wheel-chair and inability to perform ileal conduit operations. 3. The technique of osteotomy-excision of lumbar vertebrae used in eighteen cases is described. 4. The results in fourteen children are described. The primary aims of operation have been achieved in all patients. 5. A comparison is made with the results of neonatal osteotomy-excision of the spine in the newborn. Recurrence of deformity, but at a much reduced rate, must be anticipated after either operation

Between 1980 and 1988, displacement bone-marrow transplantation was performed on 25 children with Hurler's syndrome (type-1 mucopolysaccharidosis). We describe the musculoskeletal development of 11 of the 12 surviving children and the orthopaedic procedures undertaken to treat progressive thoracolumbar kyphosis, hip subluxation and carpal tunnel syndrome. We found abnormal bone modelling, focal failures of ossification and an avascular disorder of the femoral head in every patient and offer an explanation for these phenomena. Increasing valgus deformity of the knees and progressive generalised myopathy caused loss of mobility as the children entered adolescence. The benefit of bone-marrow transplantation as a treatment for the skeletal disorders of Hurler's syndrome is limited by the poor penetration of the musculoskeletal tissues by the enzyme derived from the leucocytes

Late-onset idiopathic scoliosis is associated with a rib hump in the thoracic region, and surgery is indicated when this deformity becomes unacceptable. Fifty patients with this deformity were treated by the Leeds procedure, which consists of segmental wiring to a kyphotically-contoured square-ended Harrington rod; this procedure not only derotates the spine but restores the natural thoracic kyphosis, thus avoiding subsequent buckling. All patients were followed up for a minimum of two years. Forty-two of these, who had a pre-operative Cobb angle of less than 60 degrees, were treated by one-stage instrumentation and fusion, while the remaining eight with greater curves underwent preliminary anterior multiple discectomy to provide flexibility with shortening. Postoperative loss of correction was not observed and there were no neurological complications

A study of 130 scoliotic children with curves measuring 10 degrees or more has been performed in order to elucidate the importance of stature, growth and development. Girls with adolescent idiopathic curves measuring 15 degrees or more were taller than girls with smaller idiopathic curves and taller than those whose scoliosis was secondary to leg-length inequality (pelvic tilt scoliosis). No differences were observed as regards growth velocity or development. The increased standing height may be genetic but the uncoiling effect of the normal kyphosis to give a flat lateral profile is a more likely cause. The familial trend in idiopathic scoliosis may therefore be explained by the genetically determined shape of the spine in the median (sagittal) plane

1. The management of severe kyphosis of the lumbar spine in association with myelomeningocele is discussed. 2. Neonatal spinal osteotomy-resection has been performed in six patients with partial correction of the deformity and a greatly improved ease of closure and healing of the skin defect. The severity of lower limb paralysis has been diminished compared with the complete paraplegia that almost always results from conservative management of closure of the defect without osteotomy. 3. In an older child who has not had the benefit of neonatal osteotomy and who has complete lower limb paralysis, transverse spinal osteotomy or excision of the prominent laminae and pedicles on each side of the midline makes possible the fitting of apparatus for walking and diminishes the liability to recurrent ulceration of the skin

A review is presented of 13 young patients with congenital scoliosis who were treated by epiphysiodesis of part of the vertebral bodies combined with posterior fusion, both on the convex side; the plan was to arrest growth on the convexity which, combined with growth of the concave side, would result in progressive correction of the curve. The first patient was operated on at the age of four years and has now reached skeletal maturity with complete correction of her curve. Several others, still growing, are showing progressive correction. Only three curves, in which kyphosis was more severe than scoliosis, have deteriorated since operation. Although full assessment must await skeletal maturity of all the patients, this approach appears to have sufficient potential to justify an early report

Sixty patients with congenital deformities of the spine were operated upon in the past fifteen years using a two-stage procedure. In the fifty patients with scoliosis half of the deformities were due to hemivertebrae and half to unilateral bars. The average correction of the deformity was 47 per cent. Early neurological signs observed in two patients with a diastematomyelia resolved. Of the ten patients with kyphosis nine had neurological signs of impending paraplegia and one was completely paraplegic before operation; all improved markedly. Posterior spinal fusion alone in the rapidly progressing congenital deformity may not prevent further progression, particularly in those cases iwth unilateral bars. Anterior resection of the vertebral body with later posterior fusion with Harrington instrumentation is safe and effective

A clinical, cadaveric, biomechanical and radiological investigation of the pathogenesis of idiopathic scoliosis indicates that biplanar asymmetry is the essential lesion. Many normal children have coronal plane asymmetry (an inconsequential lateral curvature of the spine), and certainly all have vertebral body asymmetry in the transverse plane, but when median plane asymmetry (flattening or more usually reversal of the normal thoracic kyphosis at the apex of the scoliosis) is superimposed during growth, a progressive idiopathic scoliosis occurs. Idiopathic kyphoscoliosis cannot and does not exist, from the mildest cases in the community to the most severe cases in pathology museums. Median plane asymmetry is crucial for progression and the lateral profile of the spine must be carefully scrutinised. Increased anterior vertebral height at the apex of the curve with posterior end-plate irregularity characterises the median plane asymmetry and suggests that idiopathic scoliosis is the reverse of Scheuermann's disease

Fifteen patients with ankylosing spondylitis who had developed a severe flexion deformity of the cervical spine which restricted their field of vision to their feet, were treated by an extension osteotomy at the C7/T1 level. The operation was performed under general anaesthesia with the patient in the prone position and wearing a halo-jacket. Three had internal fixation using a Luque rectangle and wiring. Their mean age was 48 years. Before operation the mean cervical kyphosis was 23°; this was corrected to a mean of 31° of lordosis, a mean correction of 54°. All the patients were able to see straight ahead. One patient with normal neurology soon after operation became quadraparetic after one week; two others had unilateral palsy of the C8 root, which improved. There was subluxation at the site of osteotomy in four patients, and two of them developed a pseudarthrosis which required an anterior fusion

From 1990 to 1993 we treated 22 consecutive patients who had progressive spinal kyphosis due to ankylosing spondylitis by a closing-wedge posterior vertebral osteotomy with partial corporectomy of L4 and transpedicular fixation. The average correction was 32 degrees (24 to 52) with a mean loss of correction after operation of 2.7 degrees (0 to 13). The average operating time was 185 minutes (135 to 240) and blood loss was 2500 ml (1200 to 5000). The osteotomy corrected all patients sufficiently to allow them to see ahead and their posture was improved. There were no fatal complications, but in two cases there was failure of the instrumentation and one patient needed reoperation for nerve compression. Two deep wound infections required removal of the implant and six patients had superficial skin infections under the plaster. The use of a circoelectric bed and intermittent prone lying eliminated this problem