The management of spinal deformity in children with univentricular cardiac pathology poses significant challenges to the surgical and anaesthetic teams. To date, only posterior instrumented fusion techniques have been used in these children and these are associated with a high rate of complications. We reviewed our experience of both growing rod instrumentation and posterior instrumented fusion in children with a univentricular circulation. Six children underwent spinal corrective surgery, two with cavopulmonary shunts and four following completion of a Fontan procedure. Three underwent growing rod instrumentation, two had a posterior fusion and one had spinal growth arrest. There were no complications following surgery, and the children undergoing growing rod instrumentation were successfully lengthened. We noted a trend for greater blood loss and haemodynamic instability in those whose surgery was undertaken following completion of a Fontan procedure. At a median follow-up of 87.6 months (interquartile range (IQR) 62.9 to 96.5) the median correction of deformity was 24.2% (64.5° (IQR 46° to 80°) vs 50.5° (IQR 36° to 63°)). We believe that early surgical intervention with growing rod instrumentation systems allows staged correction of the spinal deformity and reduces the haemodynamic insult to these physiologically compromised children. Due to the haemodynamic changes that occur with the completed Fontan circulation, the initial scoliosis surgery should ideally be undertaken when in the cavopulmonary shunt stage. Cite this article: Bone Joint J 2014;96-B:94–9

Aims

Spinal fusion remains the gold standard in the treatment of idiopathic scoliosis. However, anterior vertebral body tethering (AVBT) is gaining widespread interest, despite the limited data on its efficacy. The aim of our study was to determine the clinical efficacy of AVBT in skeletally immature patients with idiopathic scoliosis.

We studied 15 patients with healed tuberculosis of the spine and a resultant kyphosis. We selected only those with no neurological deficit and performed a wedge resection of the vertebra using a transpedicular approach. The wedge was removed from the apex of the deformity. For those with a neurological deficit, we chose the conventional anterior debridement and decompression with 360° circumferential fusion. At a mean follow-up of 26.8 months (8 to 46) the outcome was good with an increase in the mean Oswestry Disability Index from 56.26 (48 to 62) pre-operatively to 11.2 (6 to 16) at the latest follow-up.

A combination of hemivertebrae and diastematomyelia is rare. We have identified 12 such patients seen during a period of 11 years in the orthopaedic, spinal and neurosurgical units in Nottingham and analysed their treatment and outcome.