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

Introduction. Sparing of the spinal growth and scoliotic deformity control in patients with early-onset scoliosis is a challenge in spinal surgery. Loss of the surgical correction, implant breakage, and revision surgeries are the main disadvantages of present treatment methods. The purpose of this study is to investigate whether growing transpedicular instrumentation spares achieved surgical correction during patient's growth. Methods. This is a prospective study of 12 consecutive patients with early-onset scoliosis from one clinical centre. All patients underwent anterior convex growth arrest and posterior transpedicular instrumentation with growing construct. Spinal derotation was used for the correction of the deformity. The diagnoses were infantile idiopathic (n=10) and congenital (n=2) scoliosis (formation failure). Follow-up was 3 years. Mean age at the time of surgery was 9·1 years. Results. Preoperative major Cobb angle was 74·3° (range 52–100°), minor Cobb angle was 32·2° (5–50°), and average preoperative thoracic kyphosis was 27·2° (0–63°). Mean number of the instrumented levels was 12·2. Postoperative major Cobb angle was 22·4° (0–40°), minor Cobb angle was 5·7° (0–23·5°), and postoperative thoracic kyphosis was 26·2° (6–41°). After 3 years of follow-up, primary curve was an average of 22·7° (10–40°), secondary curve was 5·4° (0–25°), and mean thoracic kyphosis was 28° (12–40°). Mean growth of the instrumented spine was 18 mm. We did not observe any gross complications in the patients. Two patients had pleural effusion, and one needed rod exchange 15 months after primary surgery because of insufficient length. Conclusions. The results of this study show that anterior convex growth arrest and polysegmental transpedicular spinal instrumentation with growing construct save spinal growth and anatomical values achieved after surgical correction. Rod derotation for the deformity correction favours spinal growth in skeletally immature patients

Aim:. To Determine The Effect Of Posterior Instrumented Fusion On Lung Function In Patients With Idiopathic Scoliosis Aged 8–11. Method:. Lung Function (Fvc And Fev1) Was Measured Before Surgery In 13 Patients (Aged 8 To 11) With Idiopathic Scoliosis. All Patients Had Curves Greater Than 50 And Had Undergone Posterior Instrumented Scoliosis Correction And Fusion With (3 Patients) Or Without (10 Patients) Same Day Anterior Convex Growth Arrest. Lung Function Tests Were Repeated 1–8 Years (Mean 5.3 Years) After Surgery. The Data Was Normalised To Take Into Account Standing Height And Loss Of Stature Due To Lateral Curvature, Allowing A Direct Comparison Of Percent Predicted Fev1 And Fvc Before And After Surgery. Results:. Mean Fvc Was 75% Before Surgery And 79% At Longest Follow-Up (1.9l To 2.7l). Mean Fev1 Was 73% Before Surgery And 76% At Longest Follow-Up (1.7 To 2.4). The Change In Lung Function After Surgery Did Not Correlate With Length Of Time To Longest Follow-Up. The Ratio Of Fev1 To Fvc Was 0.86 Before And After Surgery. Discussion:. In Idiopathic Scoliosis Below The Age Of 8 Years, Most Would Accept That Instrumentation Should Allow Spinal Growth To Try And Maintain Lung Function. Above The Age Of 11, Lung Function Is Probably Sufficient To Allow Instrumented Fusion With Its Reduced Complication Rate And Reduced Need For Further Surgery. Between The Ages Of 8 And 11 Controversy Exists. This Pilot Study Suggests That A Larger (Possibly Rct) Study Should Be Performed To Evaluate This Group Of Patients As Fusion May Be Appropriate As Percent Lung Function Corrected For Loss Of Height Due To Curvature Appears To Be Maintained. Conflict Of Interest Statement: No conflict of interest

Aims

Spinal fusion remains the gold standard in the treatment of idiopathic scoliosis. However, anterior vertebral body tethering (AVBT) is gaining widespread interest, despite the limited data on its efficacy. The aim of our study was to determine the clinical efficacy of AVBT in skeletally immature patients with idiopathic scoliosis.

We studied 15 patients with healed tuberculosis of the spine and a resultant kyphosis. We selected only those with no neurological deficit and performed a wedge resection of the vertebra using a transpedicular approach. The wedge was removed from the apex of the deformity. For those with a neurological deficit, we chose the conventional anterior debridement and decompression with 360° circumferential fusion. At a mean follow-up of 26.8 months (8 to 46) the outcome was good with an increase in the mean Oswestry Disability Index from 56.26 (48 to 62) pre-operatively to 11.2 (6 to 16) at the latest follow-up.

A combination of hemivertebrae and diastematomyelia is rare. We have identified 12 such patients seen during a period of 11 years in the orthopaedic, spinal and neurosurgical units in Nottingham and analysed their treatment and outcome.