This is a retrospective study of 76 children who had had malignant tumours treated with laminectomy or laminoplasty and/or radiation therapy affecting the spine. Spinal tumours in children are extremely rare. However, their treatment can result in progressive spinal deformity. Radiation therapy affecting the growing spine can lead to asymmetric vertebral growth, causing kyphosis and/or scoliosis. These spinal deformities pose one of the most challenging problems for the spine surgeon. The aim of this article is to describe late-onset post-laminectomy/post-radiation spinal deformities and to evaluate the results of their treatment. Seventy-six children, with a mean age of 4 years and 7 months (range, 2 months to 16 years), underwent surgical removal of malignant tumours, between 1961 and 1995. Sixty-seven of them developed post-laminectomy/post-radiation spinal deformity. Conservative treatment consisted of bracing and corrective plaster casts. In 46 cases the deformity was treated surgically. A distraction plaster cast was used as preoperative preparation in the more severe and rigid curves, with or without neurological impairment. Surgery consisted of combined anterior and posterior fusion in 39 cases and posterior fusion in seven cases. Posterior instrumentation was used in 38 cases. The mean follow-up period was 6 years and 7 months (range, 9 months to 20 years and 2 months). Nine children did not develop deformity following the primary tumour treatment. One of them underwent laminectomy with posterolateral fusion and eight had laminoplasty combined with external immobilisation. Forty-six children developed iatrogenic kyphosis and underwent surgical correction from a mean of 75 degrees pre-correction to a mean of 32 degrees . The mean scoliotic angle correction was 66 degrees preoperatively to 34 degrees postoperatively. At follow-up, the mean correction loss was 7 degrees in the sagittal plane and 5 degrees in the coronal plane. Preoperative distraction plaster cast treatment resulted in a correction of 39% in kyphosis and of 58% in scoliosis, and in a partial or complete recovery of neurological deficits in all but one patient. In severe and rigid curves that develop following treatment of paediatric spinal tumours, preoperative application of a distraction plaster cast can reduce deformity and facilitate surgical correction. Furthermore, in the case of pure bony compression of the spinal cord due to the apical vertebra of the deformity, treatment with the distraction plaster can result in recovery from the neurological impairment. The prevention of post-laminectomy/post-radiation spine deformities is emphasised. Rigid external immobilisation for a period of 4 months in the cervical spine and of 6 months in the thoracic spine is recommended after both laminoplasty and laminectomy with posterolateral fusion.

A cross-sectional study was carried out to obtain data on the bone mineral density status of a group of neurofibromatosis-1 patients with spinal deformities, and to search for possible accompanying changes in the bone mineral turnover. Neurofibromatosis-1 is a heredofamiliar disorder that is associated with a variety of skeletal anomalies (mostly spinal deformities) in 10-50% of patients. Intraoperatively, a poor vertebral bone quality has been observed. Efforts have been made to identify factors preventing curve progression, to optimize operational planning and to explain the pathomechanism. As part of the preoperative evaluation, dual-energy X-ray absorptiometry was used to assess the bone mineral density of the lumbar spine in 12 patients with neurofibromatosis-1, supplemented by laboratory blood/urine investigations. A significant decrease in bone mineral density of the lumbar spine was measured. An inverse relation was suggested between the severity of scoliosis and the lumbar spine Z-scores. No pivotal alterations were identified in the laboratory measurements. The bony tissue abnormality observed intraoperatively in neurofibromatosis-1 patients may be described as a diminution of the axial bone mineral density. The biochemical parameters do not support the presence of hyperparathyroidism, renal disorders or other associated diseases influencing the bone mineral turnover. The evaluation of bone mineral density in the course of the preoperative planning is proposed in neurofibromatosis-1; the exact background and the role of a possible osteoporosis in the prognosis remain to be elucidated.

The Cotrel-Dubousset technique ensures considerable sagittal correction of the spine. In the course of scoliosis correction, it is possible to preserve the normal preoperative sagittal profile of the spine, to correct the slightly flattened thoracic kyphosis, to increase materially the kyphosis of the frankly hypokyphotic spine, to preserve or restore normal lumbar lordosis in a considerable percentage of the cases, to avoid angular segmental hyperlordosis at the level of the first disc below the fusion, and to avoid retrolisthesis of the last fused vertebra.

Object. In 10 to 50% of cases with neurofibromatosis, skeletal disorders are present, mainly as various deformities of the spine. These deformities can be divided into dystrophic and nondystrophic groups depending on the absence or presence of bone dystrophy. The nondystrophic curves are highly similar to those in idiopathic scoliosis, whereas the dystrophic curves are manifested early and, by progressing inexorably, may lead to neurological symptoms. In this article the authors report on a series of 12 patients (11 with dystrophic and one with nondystrophic deformities) who underwent surgical treatment. Methods. In the case with a nondystrophic curve, posterolateral instrumentation-assisted fusion was performed. A curvature correction of 70% was achieved in the frontal plane, and at the 2-year follow-up examination neither bone dysplasia nor pseudarthrosis was observed. In the cases with dystrophic curves, preoperative traction for 3 weeks was applied; anterior surgical release was then performed, as was two-stage posterior instrumentation-assisted fusion. In the cases of thoracic kyphoscoliosis in which this treatment protocol was performed, the mean scoliosis correction was 66%, whereas the mean decrease in kyphotic angle was 34.5°. In the cases with thoracolumbar and lumbar curves, the mean correction in the frontal plane was 69.8°, whereas the mean preoperative lumbar kyphosis of 42° was corrected to a mean lordotic angle of 23°. Postoperatively, no hook dislocation was detected. A neurological complication was observed in one case. Conclusions. The surgical treatment of dystrophic curves always included 360° fusion and the use of a tibial corticocancellous graft, which must be placed on the concave side of the curve in the frontal plane, the graft thereby providing biomechanical support.

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