Lumbar fixation to L5 is adequate if the surgery is performed early, soon after becoming wheelchair bound, and with smaller curves and minimal pelvic obliquity. Use of pedicle screws in lumbar spine provides a solid foundation to maintain the correction over the period of relatively short life expectancy of these children. Pelvic fixation may be necessary in older children, who have larger curves and established pelvic obliquity. In the presence of deteriorating lung function, this is associated with a greater morbidity and higher complication rate.
A previously well 2-year-old female infant was admitted as an emergency with status epilepticus. She was apyrexial and general examination was unremarkable. Initial investigation revealed profound hypoglycaemia (glucose < 0.6 mmol/l). The seizures settled following administration of anticonvulsant medication and intravenous glucose. She remained comatose for 24 h. Mid-Chain Acyl-CoA Dehydrogenase Deficiency (MCAD) was suspected, and later confirmed on blood tests. Following the intravenous administration of L-carnitine she became more alert and responsive. At that stage she had an extensor response to painful stimuli with generalised spasticity. Electroencephalogram tests revealed a diffuse encephalopathy.Ten days after admission she developed a pyrexia of 38.4°C; blood cultures were negative. Further pyrexial episodes continued over the next 2 weeks, at the end of which she developed a varicella infection. The skin vesicles became secondarily infected with Staphylococcus aureus, which progressed to a septicaemia and pneumonitis. Her condition deteriorated; although her white cell count was elevated at 16.1 × 10 9 /l, she was profoundly neutropoenic (1.0 × 10 9 /l). Scalp and praecordial abscesses erupted and required formal surgical drainage.Immediately after the above operation there was no voluntary movement of the lower limbs. She had a pyrexia of 39°C and neurological examination showed absent deep tendon reflexes and bilaterally positive Babinski reflexes. Her general condition was poor with inspiratory stridor and low blood saturation even with supplemental oxygen. Plain radiographs were initially interpreted as normal; in retrospect the lateral radiograph of the thoracic spine showed a kyphosis between T5 and T7 (Fig. 1). MRI revealed complete vertebral collapse of T6 and localised kyphosis at this level. There was an extensive epidural abscess and extrusion of granulation tissue posteriorly causing predominant anterior compression of the spinal cord. There was marked marrow oedema of the adjacent vertebrae (Fig. 2).Despite her condition (ASA grade 4), surgery was deemed necessary but was delayed for 12 h for further resuscitation and chest physiotherapy.A left thoracotomy was performed and the MRI findings were confirmed. There was marked paravertebral thickening of the somatic pleura. Frank pus was obtained from within the canal. Decompression of the cord was achieved by means of a T6 vertebrectomy. A rib strut graft was used to maintain correction of the kyphosis. Confirmatory cultures of Staphylococcus aureus were obtained from the intraoperative specimens.Post-operatively she was transferred to the paediatric intensive care unit. High-dose intravenous flucloxacillin, fucidin and gentamicin antibiotic therapy was commenced. The pyrexia rapidly settled and she was successfully weaned from assisted ventilation at 48 h. Spontaneous movements of the legs returned 5 days after the spinal surgery. Oral antibiotic treatment was maintained for 6 weeks. A moulded polypropylene thoracolumbar support orthosis w...
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