Aims

Clinical and radiological data were reviewed for all patients with mucopolysaccharidoses (MPS) with thoracolumbar kyphosis managed non-operatively or operatively in our institution.

C5 nerve root palsy is a rare and potentially debilitating complication of cervical spine surgery. Currently, however, there are no guidelines to help surgeons to prevent or treat this complication.

We carried out a systematic review of the literature to identify the causes of this complication and options for its prevention and treatment. Searches of PubMed, Embase and Medline yielded 60 articles for inclusion, most of which addressed C5 palsy as a complication of surgery. Although many possible causes were given, most authors supported posterior migration of the spinal cord with tethering of the nerve root as being the most likely.

Early detection and prevention of a C5 nerve root palsy using neurophysiological monitoring and variations in surgical technique show promise by allowing surgeons to minimise or prevent the incidence of C5 palsy. Conservative treatment is the current treatment of choice; most patients make a full recovery within two years.

Cite this article: Bone Joint J 2014;96-B:950–5.

We reviewed 31 consecutive patients with Friedreich’s ataxia and scoliosis. There were 24 males and seven females with a mean age at presentation of 15.5 years (8.6 to 30.8) and a mean curve of 51° (13° to 140°). A total of 12 patients had thoracic curvatures, 11 had thoracolumbar and eight had double thoracic/lumbar. Two patients had long thoracolumbar collapsing scoliosis with pelvic obliquity and four had hyperkyphosis. Left-sided thoracic curves in nine patients (45%) and increased thoracic kyphosis differentiated these deformities from adolescent idiopathic scoliosis. There were 17 patients who underwent a posterior instrumented spinal fusion at mean age of 13.35 years, which achieved and maintained good correction of the deformity. Post-operative complications included one death due to cardiorespiratory failure, one revision to address nonunion and four patients with proximal junctional kyphosis who did not need extension of the fusion. There were no neurological complications and no wound infections. The rate of progression of the scoliosis in children kept under simple observation and those treated with bracing was less for lumbar curves during bracing and similar for thoracic curves. The scoliosis progressed in seven of nine children initially treated with a brace who later required surgery. Two patients presented after skeletal maturity with balanced curves not requiring correction. Three patients with severe deformities who would benefit from corrective surgery had significant cardiac co-morbidities.

This review of the literature presents the current understanding of Scheuermann’s kyphosis and investigates the controversies concerning conservative and surgical treatment. There is considerable debate regarding the pathogenesis, natural history and treatment of this condition. A benign prognosis with settling of symptoms and stabilisation of the deformity at skeletal maturity is expected in most patients. Observation and programmes of exercise are appropriate for mild, flexible, non-progressive deformities. Bracing is indicated for a moderate deformity which spans several levels and retains flexibility in motivated patients who have significant remaining spinal growth.

The loss of some correction after the completion of bracing with recurrent anterior vertebral wedging has been reported in approximately one-third of patients. Surgical correction with instrumented spinal fusion is indicated for a severe kyphosis which carries a risk of progression beyond the end of growth causing cosmetic deformity, back pain and neurological complications. There is no consensus on the effectiveness of different techniques and types of instrumentation. Techniques include posterior-only and combined anteroposterior spinal fusion with or without posterior osteotomies across the apex of the deformity. Current instrumented techniques include hybrid and all-pedicle screw constructs.

Segmental vessel ligation during anterior spinal surgery has been associated with paraplegia. However, the incidence and risk factors for this devastating complication are debated.

We reviewed 346 consecutive paediatric and adolescent patients ranging in age from three to 18 years who underwent surgery for anterior spinal deformity through a thoracic or thoracoabdominal approach, during which 2651 segmental vessels were ligated. There were 173 patients with idiopathic scoliosis, 80 with congenital scoliosis or kyphosis, 43 with neuromuscular and 31 with syndromic scoliosis, 12 with a scoliosis associated with intraspinal abnormalities, and seven with a kyphosis.

There was only one neurological complication, which occurred in a patient with a 127° congenital thoracic scoliosis due to a unilateral unsegmented bar with contralateral hemivertebrae at the same level associated with a thoracic diastematomyelia and tethered cord. This patient was operated upon early in the series, when intra-operative spinal cord monitoring was not available.

Intra-operative spinal cord monitoring with the use of somatosensory evoked potentials alone or with motor evoked potentials was performed in 331 patients. This showed no evidence of signal change after ligation of the segmental vessels.

In our experience, unilateral segmental vessel ligation carries no risk of neurological damage to the spinal cord unless performed in patients with complex congenital spinal deformities occurring primarily in the thoracic spine and associated with intraspinal anomalies at the same level, where the vascular supply to the cord may be abnormal.