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.

This is a retrospective study describing four patients who developed symptomatic subluxation of the hip after stabilisation to the pelvis for myopathic scoliosis in Duchenne Muscular Dystrophy (DMD). Fusion to the pelvis is recommended for treatment of scoliosis in DMD. Non-spinal extra-pulmonary complications following this have not been described. 4 patients (average age: 14 years) out of a cohort of patients who have undergone spinal stabilisation for DMD between 1991 and 1998 developed symptomatic subluxation of the hip at an average of three months after fusion from the upper thoracic spine to the pelvis. All four had pain and three noticed clicking in the hip. X-rays revealed subluxation of the hip in all patients, and conservative treatment by adjustment of seating position in the wheelchair was successful in reducing the symptoms in all patients. Flexion-abduction contractures of the hip, which are a feature of DMD, are known to cause uncovering of the contralateral hip. We postulate that the spine compensates for this uncovering to a large degree, and that spinopelvic fusion for scoliosis in patients with pre-existent abduction contractures negates the capacity of the spine to provide compensation. This leads to uncovering of the hip with the lesser degree of contracture, and the resultant symptoms. We recommend screening for, and treatment of, flexion-abduction contractures of the hip in all patients undergoing spinal fusion for DMD, to avoid the possibility of development of symptomatic subluxation of the hip

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

Study Design: Retrospective case series. Objective: To evaluate the clinical outcome, radiographic results and complications associated with single rod anterior instrumentation in neuromuscular thoracolumbar scoliosis. Methods: Retrospective study with mean follow up of 35 months. Subjects: Nine patients (6F, 3M), mean age 15 years, were operated on between 1994–2000. This heterogeneous patient group consisted of five cases of spinal dysraphism, one prune belly syndrome, one arthrogryposis, one myotonic dystrophy and one congenital myopathic dystrophy (muscle-eye-brain-syndrome). All patients were ambulatory and had minimal pelvic obliquity (< 15degrees). Outcome measures: Pre-operative, post-operative and final follow-up measurements of Cobb angles, apical vertebral translation (AVT), thoracic kyphosis, lumbar lordosis, sagittal and coronal balance were recorded along with operative complications, pseudarthrosis, metalwork failure and loss of correction. Results: There was one rod breakage and one case of proximal thoracic curve progression requiring supplementary posterior surgery. For the remaining 7 patients, the average corrections for Cobb angle was 62% (52 to 20 degrees), AVT was 53% (5.7 to 2.7cms), and both thoracic kyphosis and lumbar lordosis remained unchanged. No pseudarthrosis, vascular or neurological complications were encountered. Subjectively results were excellent in six and good in one. Conclusions: Selective anterior instrumentation for neuromuscular scoliosis using a single rod resulted in acceptable clinical and radiographic outcomes in this highly selected series. Advantages include preservation of distal lumbar motion segments whilst maintaining sagittal and coronal alignment. We believe that this method of scoliosis correction has a definite yet select role in patients who are ambulatory, have minimal pelvic obliquity (< 15degrees), non-progressive pathology and near normal mental function

Objective: To evaluate the subjective clinical outcomes, radiographic results and complications associated with single solid rod anterior instrumentation in neuromuscular scoliosis. Design: Retrospective clinical case series with a mean follow up of 30 months (range 24 – 42 months). Subjects: 9 consecutive cases (6F, 3M) with a mean age 15 years (range 11 – 24 years), underwent single solid rod anterior instrumentation of their neuromuscular thoracolumbar scoliosis between 1994 and 2000. The heterogeneous patient group consisted of 5 spinal dysraphism, and 1 each of prune belly syndrome, arthrogryposis, myotonic dystrophy and congenital myopathic dystrophy (muscle eye brain syndrome). All patients were ambulatory and had minimal pelvic obliquity (< 15 degrees). Outcome measures: Pre-operative, post-operative and final follow up measurements were collected for 1) Cobb angles, 2) apical vertebral translation (AVT), 3) thoracic kyphosis (T5-12) and 4) lumbar lordosis (L1-5). Operative complications, pseudarthrosis, metalwork failure and loss of correction were also recorded. Results: There was 1 each of rod breakage and upper thoracic curve progression requiring supplementary posterior surgery. For the remaining 7 patients, the average follow-up corrections for Cobb angle was 56% (49 to 22 degrees), AVT was 49% (5.1 to 2.6 cms), and both the thoracic kyphosis and lumbar lordosis remained unchanged. No significant loss in correction occurred during the post-operative period to final follow-up in all the above parameters. No pseudarthrosis, vascular or neurological complications were encountered. Subjectively, there were 6 excellent and 1 good results. Conclusions: In this limited case review, selective anterior instrumentation for neuromuscular scoliosis using a single solid rod system resulted in acceptable clinical and radiographic outcomes. Our results appear to compare favourably with those published for the recommended method of posterior instrumentation. Advantages include preservation of distal lumbar motion segments whilst maintaining segmental saggital and coronal alignment. We believe that this method of scoliosis correction has a definite yet select role in patients who are ambulatory, have minimal pelvic obliquity (< 15 degrees), non-progressive pathology and near normal mental function

Aims

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