The surgical management of spinal deformity in children with a Fontan circulation

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.

Surgical Treatment for Severe Scoliosis in Patients with Reduced Cardiorespiratory Function after Surgery for Congenital Heart Disease: A Report of Two Cases

“…Evans et al also suggested that patients with Fontan circulation have high venous pressure, particularly while in the prone position. Hence, operations requiring the prone position in these patients may result in significant blood loss [10]. Considering these concerns, we took extra care during the operation to avoid excessive blood loss in case 1.…”

“…Hypotensive anesthesia is often useful to prevent bleeding, although CHD patients with unstable hemodynamics can easily develop hypoperfusion. Evans et al reported that operations during which the patient must remain prone for more than 4 h were associated with an increased risk of bleeding and hypotension, even when the patient was initially hemodynamically stable [10]. Patients with Fontan circulation (as in case 1) receive lifelong anticoagulant therapy, which makes the perioperative use of hemostatic agents difficult.…”

Surgical Correction of Scoliosis in Children with Severe Congenital Heart Disease and Palliated Single Ventricle Physiology

“…Spinal surgery in patients with CHD has become safer with time, however the risk of perioperative complications remains high 17–21 . This risk is greatest in patients with single ventricle physiology and requires multidisciplinary care and preparation for adverse perioperative events 21–27 . Our institution recently reviewed 78 patients with CHD undergoing scoliosis surgery and found patients with cyanotic single ventricle physiology had the highest risk for adverse perioperative events 21 .…”

“…[17][18][19][20][21] This risk is greatest in patients with single ventricle physiology and requires multidisciplinary care and preparation for adverse perioperative events. [21][22][23][24][25][26][27] Our institution recently reviewed 78 patients with CHD undergoing scoliosis surgery and found patients with cyanotic single ventricle physiology had the highest risk for adverse perioperative events. 21 Our single ventricle physiology cohort has grown since this research and an earlier review of Fontan patients.…”