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°) 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: