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.

STUDY DESIGN. Retrospective review of outcome of submuscular rod placement without apical fusion for the treatment of scoliotic deformities in children with severe co morbidities (ASA IV). METHODS. 6 children with progressive scoliosis (2 severe cerebral palsy, 2 congenital cyanotic heart disease, 1 Worster Drought syndrome, 1 Leigh's disease), underwent a serial and limited exposure of the lower and then the upper end of the spine, and insertion of pedicle screws, hooks and clamps. Two submuscularly rods were connected and distracted. Mean age was 13 years old, the mean preoperatively Cobb angle was 87° and the mean postoperatively Cobb angle was 62°. The mean operation time was 120 min and the peri-operative blood loss was 410 ml. Mean follow up is 15 months. RESULTS. There were no adverse peri-operative events. We anticipated late implant failure and revisions but to date only one construct has failed and revised without difficulties (clamps to pedicle screws). 1 patient complicated with increased spasms 1 year postoperatively. The benefits from this procedure were. a) the decreased peri-operative:. blood loss. operation time. respiratory stress. b) the faster patients recovery. c) the significant improvement regarding pain relief and sitting positioning. CONCLUSION. We believe that this procedure can be offered as a safe and viable alternative in selected patients who present with predicted high peri-operative mortality. To the best of our knowledge this technique is not described before