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.

Reviewing our experience of scoliosis in children with a Cavopulmonary Shunt or Fontan circulation and the cardiovascular challenges that this presents.

A notes and x ray review was performed. Special attention was paid to the changes in cardiovascular status whilst prone. The review was from first presentation to latest follow up.

There were 6 patients who underwent 7 major procedures between 2001 and 2009. All had cardiac procedures in early life. Both definitive fusion and growing instrumentation was used. All procedures were successful. Growing instrumentation allowed earlier primary surgery before completion of the Fontan circulation. All have been subsequently lengthened in a lateral position. The mean follow up is 56 months. There was one death 40 months following last surgery, cause unrelated to spinal surgery.

In the older patients with a completed Fontan significant blood loss was seen, due to the raised venous pressure required to run the Fontan, and occult hypotension seen as a climbing difference between Pulmonary Artery Wedge Pressure and Central Venous Pressure were common when prone.

We recommend early intervention, using instrumentation without fusion to correct the deformity over time and allow intervention prior to completing the Fontan circulation. As haemodynamic instability increases with increasing time in the prone position, surgery should be expedited rapidly.