The development of spinal deformity in children with underlying neurodisability can affect their ability to function and impact on their quality of life, as well as compromise provision of nursing care. Patients with neuromuscular spinal deformity are among the most challenging due to the number and complexity of medical comorbidities that increase the risk for severe intraoperative or postoperative complications. A multidisciplinary approach is mandatory at every stage to ensure that all nonoperative measures have been applied, and that the treatment goals have been clearly defined and agreed with the family. This will involve input from multiple specialities, including allied healthcare professionals, such as physiotherapists and wheelchair services. Surgery should be considered when there is significant impact on the patients’ quality of life, which is usually due to poor sitting balance, back or costo-pelvic pain, respiratory complications, or problems with self-care and feeding. Meticulous preoperative assessment is required, along with careful consideration of the nature of the deformity and the problems that it is causing. Surgery can achieve good curve correction and results in high levels of satisfaction from the patients and their caregivers. Modern modular posterior instrumentation systems allow an effective deformity correction. However, the risks of surgery remain high, and involvement of the family at all stages of decision-making is required in order to balance the risks and anticipated gains of the procedure, and to select those patients who can mostly benefit from spinal correction.

Aims

Severe spinal deformity in growing patients often requires surgical management. We describe the incidence of spinal deformity surgery in a National Health Service.

1. The results of electrodiagnostic tests reveal the level and progress of nerve lesions. 2. The findings in a myopathic lesion are: a) a normal SD curve; b) no fibrillation on the E.M.G.; c) on volition a full interference pattern-because motor units have not been lost, but only muscle fibres within the units; d) the action potentials are short in duration, polyphasic and of low amplitude; e) conduction times are normal. 3. In peripheral nerve affections these findings are evidence of denervation: a) a sluggish response to a long duration current; b) a kinked SD curve; c) fibrillation at rest on the E.M.G.; d) a reduced interference pattern of motor unit action potentials on volition; e) a preponderance of polyphasic action potentials of long and short duration; f) slowing of conduction times. 4. Evidence of reinnervation is given by: a) a kink in the SD curve; b) the appearance of a response in the muscle to nerve stimulation; c) a progressive increase in conduction velocity; d) the appearance of polyphasic action potentials on volition; e) fibrillation becoming more difficult to detect. 5. The following findings suggest a lesion of the anterior horn cell: a) difficulty in detecting fibrillation, and a relatively normal SD curve in the presence of marked wasting. (Intact axons have "mopped up" denervated fibres.) b) Marked reduction in interference pattern with ‘giant units’. c) Normal conduction velocity