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The surgical management of spinal deformity in children with a Fontan circulation: The development of an algorithm
S Evans1, A Ramasamy, D S Marks
1Royal Orthopaedic Hospital, Spinal Unit, Bristol Road, Birmingham B31 2AP, UK.
Insights
Spinal surgery in children with univentricular heart conditions is complex. Growing rod instrumentation offers a safer, staged approach to correct scoliosis, reducing complications compared to traditional fusion methods.
Area of Science:
- Pediatric Orthopedics
- Pediatric Cardiology
- Cardiothoracic Surgery
Background:
- Children with univentricular heart disease often develop spinal deformities.
- Surgical management is challenging due to complex physiology.
- Previous posterior instrumented fusion techniques have high complication rates.
Purpose of the Study:
- To evaluate the safety and efficacy of growing rod instrumentation versus posterior instrumented fusion for spinal deformities in children with univentricular hearts.
- To compare outcomes between cavopulmonary shunt and Fontan completion stages.
Main Methods:
- Retrospective review of six pediatric patients with univentricular circulation undergoing spinal corrective surgery.
- Patients received either growing rod instrumentation or posterior instrumented fusion.
- Outcomes assessed included complications, need for revision, deformity correction, and hemodynamic stability.
Main Results:
- No complications were reported with either technique.
- Growing rod instrumentation achieved successful lengthening.
- Patients post-Fontan completion showed trends toward greater blood loss and hemodynamic instability.
- Median deformity correction was 24.2% at a median follow-up of 87.6 months.
Conclusions:
- Early surgical intervention with growing rod instrumentation facilitates staged correction and minimizes hemodynamic stress in these vulnerable patients.
- Spinal surgery is best performed during the cavopulmonary shunt stage before Fontan completion due to hemodynamic changes.
Abstract:
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.
