Study Design: Retrospective study with clinical and radiological evaluation of 29 patients with congenital scoliosis who underwent 31 short segment anterior instrumented fusions of lateral hemivertebrae.

Objective: To evaluate the safety and efficacy of early surgical anterior instrumented fusion with partial preservation of the HV in the treatment of progressive congenital scoliosis in children below the age of 6.

Summary of background data: A variety of treatments have been described in the literature for the treatment of HV. We report the results of a novel technique.

Materials and Methods: Between 1996 and 2005, 29 consecutive patients with 31 lateral HV and progressive scoliosis underwent short segment anterior instrumentation and fusion with preservation of the HV. Mean age at surgery was 2.9 years. Mean follow-up period was 6.3 years.

Results: Preoperative segmental Cobb angle averaging 39°, was corrected to 150 after surgery, being 15º at the last follow up (60% of improvement). Compensatory cranial and caudal curves corrected by approximately 50% and did not change significantly on follow up. The angle of segmental kyphosis averaged 13º before surgery, 12º after surgery, and 12° at follow up. There was 2 wound infection requiring surgical debridment, 1 intraoperative fracture of the vertebral body and 1 case lost correction due to implant failure. All went on to stable bony union. There were no neurologic complications.

Conclusions: Early diagnosis and early and aggressive surgical treatment are mandatory for a successful treatment of congenital scoliosis and to prevent the development of secondary compensatory deformities. Anterior instrumentation is a safe and effective technique capable of transmitting a high amount of convex compression allowing short segment fusion which is of great importance in the growing spine

Cervico-thoracic congenital scoliosis is a difficult deformity to obtain good correction due to its anatomical characteristics and lack of proper instrumentation. Surgical treatments often end up with poor correction by convex epiphysiodesis alone, making hideous residual head tilt. This is a report of 2 cases with cervico-thoracic congenital scoliosis, which underwent total excision of hemivertebra, instrumentation and fusion through posterior approach alone. Case 1. 8y2m old Girl who had T1, T3, T7 hemivertebrae with a left convex curve from C7 to T11. At age 5. she had tilted head and left convex 33 degrees scoliosis. Only regular observation was done. At age 8y2m, the scoliosis had progressed to 49degrees. Total excision of T1 hemivertebra was performed. At age 10y8m, total excision of T7 hemivertebra, extension of instrumentation and fusion to T10 was performed. These procedures brought almost normal alignment on both sagittal and coronal plane. However, lower compensatory curve progressed later on, fusion was extended to L2 at age 13 resulting in excellent balance. Case 2: Girl. 2y7m. Multi-level hemivertebrae. C6-L1 L100 degrees. Total excision of T12 hemi, short fusion and instrumentation reduced the scoliosis to 50 degrees. Five months later, total excision of T9 hemi was done. Four months later, concave side instrumentation from T2 to L2 without fusion was done. At age 4y2m, total excision of T1 hemi was done using cervical pedicle screw. The scoliosis is being controlled at 35 degrees with one extension of the rod later on

Study Design: Retrospective study with clinical and radiological evaluation of 15 patients with congenital kyphosis or kyphoscoliosis who underwent anterior instrumented spinal fusion for posterolateral or posterior hemivertebae (HV).

Objective: To evaluate the safety and efficacy of early surgical anterior instrumented fusion with partial preservation of the HV in the treatment of progressive congenital kyphosis in children below the age of 3. We discuss the management of patients presenting with neurological compromise.

Summary of background data: A variety of treatments have been described in the literature for the treatment of congenital kyphosis due to HV. We report the results of our technique.

Materials and Methods: Between 1997 and 2005 we have treated 15 consecutive patients with progressive congenital kyphosis with anterior instrumented fusion and strut grafting. 13 patients had a single posterolateral HV and 2 patients a single posterior HV. Of the 15 patients in the study, 5 were girls and 10 boys. Mean age at surgery was 22 months (range 8–33). Mean follow-up period was 6.8 years. 13 HV were located in the thoracolumbar junction (T10-L2) and 2 in the thoracic spine.

Results: The average operating time was procedure was 150 minutes (range, 130 to 210 minutes). The average blood loss 180 mL (range, 100 to 330 mL), equivalent to a mean external blood volume loss of 15% (range, 11 to 24%).

Preoperative segmental Cobb angle averaging 34 º at last follow up. Compensatory coronal cranial and caudal curves corrected by 50%. The angle of segmental kyphosis averaged 39º (range, 20º to 80º) before surgery and 21 º (range, 11º to 40º) at last follow up. This represents a 43% of improvement of the segmental kyphosis, and a 64% of improvement of the segmental scoliosis at last followup.

One case with initial kyphosis of 80 º continued to progress and required revision anterior and posterior surgery. There were no neurologic complications.

Key points:

In progressive congenital kyphoisis, early diagnosis and aggressive surgical treatment are mandatory for a successful result.

Purpose: We reviewed our pre-puberty patients whose scoliosis or kyphoscoliosis involved a hemivertebra. The purpose of our work was to evaluate the surgical technique used and evaluate spinal static as well as functional outcome at skeletal maturity. Material and methods: This retrospective analysis included 21 patients who underwent surgery before the age of 10 years and were followed to skeletal maturity. We excluded children with a multiple malformation syndrome or multiple vertebral malformations. The type and localisation of the hemivertebra was noted. Deformation, transversal balance, and radiographic measures were recorded preoperatively. Elements contributing to the indication for surgery, the type of procedure, and complications were also recorded. Events recorded during growth were the clinical course, complementary treatments, and possible surgical revision. Functional and aesthetic outcome was assessed at last follow-up. Spinal deviation was measured and compared with the preoperative angles. Results: Twenty-one children (13 girls, 8 boys), mean age three years ten months (range 10 months – 10 years) met the inclusion criteria. These children had a hemivertebra of the thoracic spine (n=9), the thoracolumbar junction (n=4), the lumbar spine (n=4), or the lumbosacral region (n=4). Surgery was indicated to arrest clinical and radiographic degradation. Several surgical procedures were used: fusion without resection for thoracic vertebrae and resection associated with arthrodesis or epiphysiodesis for other localisations. There were five complications: neurological (n=2), infectious (n=2) and disassembly (n=1). The clinical and radiographic course led to revision in ten children, including two who required a new operation. At mean follow-up of 14 years (9–23 years), the functional outcome was good in 19 patients, poor in one patient with a lumbosacral hemivertebra and in another with a thoracic hemivertebra. The aesthetic result was good in 16 patients. Five of the nine patients with a thoracic hemivertebra remained unsatisfied with the outcome. Mean curvature correction ranged from 26% at the thoracic level to 50% at the thoracolumbar and lumbosacral levels and 75% at the lumbar level. Discussion: The long follow-up of this series is exceptional. Treatment of evolving spinal malformations is a difficult challenge. Early surgery does not guarantee the final outcome and 50% of patients have to be reoperated at the end of growth. The good long-term functional and aesthetic outcome is however encouraging, particularly when hemivertebrectomy can be performed

Purpose of the study: Neurophysiological monitoring during pedicular screw insertion has been used to verity bone integrity of instrumented pedicles. The purpose of this study was to determine, experimentally, whether the EMG thresholds after stimulation of dorsal pedicular screws depend on the distance between the nerve structures and the screw, or on the interposition of different tissues. Material and methods: EMG thresholds were recorded after stimulation of 18 VPD in fivde pigs, by varying the distance between the screws and the spinal cord (2, 6, 10 mm). The thresholds were recorded after rupture of the median pedicular cortical and after interposition of different tissues (blood, muscle, fat and bone) between the screws and the spinal cord. In four patients with a hemivertebra, four pedicular screws sere stimulated at insertion, just after resection of the hemivertebra. Results: The average intensity of the EMG thresholds was 5.60±1.90mA when the screws were in contact with the dural sac. When the distance was 2 mm, the average threshold reached ±3.42 mA, at 6 mm 13.59±6.27 mA and at 10mm, 15.86±5.83 mA (p< 0.05). Rupture of the median pedicular cortical and interposition of different biological tissues in experimental animals did not modify the stimulation thresholds of the dorsal pedicle screws. In the four operated patients with resection of a hemivertebra, the EMG stimulation thresholds exhibited a wide spread but did not provide any evidence for a significant change related to interposition of different tissues. The impedance of the bone material was higher than muscle or adipose tissue. In these patients, the distance from the screw to the spinal cord was not correlated with a modification in the stimulation threshold. Discussion: Further clinical study is needed to better understand the stimulation role of the EMG in the implantation of pedicular screws, considering that this technique does not determine pedicle rupture. Conclusion: In experimental animals, the electrical impedance appears to depend on the distance between the screw and the nerve structures but not on the integrity of the median pedicular cortical. Response to intensity does not appear to be related to the type of interpositioned tissue

Summary of Background Data: The craniofacial malformations described by Goldenhar can be associated with congenital anomalies of the vertebrae. This non-random association of abnormalities represents unilateral errors in the morphogenesis of the spine, as well as the first and second branchial arches. Purpose of the study: The aim of the present study was to determine the prevalence of Goldenhar related conditions in patients with congenital deformities of the spine and to describe the types of vertebral abnormalities and the necessity for treatment. Material-Methods: We performed a retrospective study of 668 consecutive patients with congenital deformities of the spine. The medical records and spinal radiographs were reviewed and patients with a Goldenhar associated condition were identified. The vertebral anomalies causing the spine deformity were detected on antero-posterior and lateral spine radiographs. The type and site of the craniofacial abnormalities, as well as other musculoskeletal deformities and systemic anomalies were recorded. Results: Fourteen patients had Goldenhar associated conditions (7 males and 7 females). A thoracic scoliosis was the most common type of deformity occurring in ten patients (71.5%). Eight of these patients had an isolated hemivertebra and the remaining two had a unilateral unsegmented bar with contralateral hemivertebra at the same level. There was only one patient with a lumbar scoliosis and this was due to a hemivertebra. The side of the vertebral anomaly correlated with that of the hemifacial microsomia in five of the eleven patients who had a scoliosis or kyphoscoliosis. A thoracolumbar kyphosis occurred in four patients; two had posterior hemivertebrae, one had wedge vertebrae, and the remaining patient had an anterior unsegmented bar. A thoracolum-bar kyphoscoliosis occurred in only one patient and was due to a posterolateral quadrant vertebra. Klippel-Feil syndrome occurred in six patients (42.8%). Eight patients (57%) underwent surgical treatment at a mean age of 9.8 years (range: 2.9–19). Four patients had a combined anterior-posterior spine arthrodesis. The remaining four patients had a posterior spinal arthrodesis. Conclusions. The prevalence of Goldenhar associated conditions in patients with congenital deformities of the spine was 2%. Failures of vertebral segmentation were the most frequent abnormality in the cervical spine, whereas failures of vertebral formation most commonly occurred in the thoracic or thoracolumbar spine

Study Design. An observational study in patients with congenital spinal deformity. Objective. To review the relationship between hemivertebrae and the posterior spinal elements in congenital spinal deformity. Summary of background data. The vertebral body anomalies of congenital scoliosis have been classified and their natural history is well known. More recently a variety of posterior element anomalies have been classified. The relationship between posterior and anterior structures is important when posterior excision of a hemivertebrae is undertaken. Methods. Patients recorded as having congenital spinal deformity were identified from a database. All available imaging was obtained and reviewed with particular reference to posterior element anatomy as visualised on CT or MRI scans. Results. In total 36 patients with congenital spinal deformity were identified and their imaging was reviewed. 29 hemivertebrae were identified in 24 patients. Clear images were obtained of the posterior elements in 18 cases. 9 isolated hemilaminae (as visualised on coronal CT or MRI) were seen in 8 patients with associated hemivertebrae. In 7 of 9 cases the hemilamina was found to be one level cranial to the hemivertebrae and in the remaining 2 cases it was at the same level. Conclusion. When planning excision of a hemivertebra, whether this is performed by a posterior approach or by combined anterior and posterior surgery, care must be taken to avoid wrong level surgery. Pre-operative assessment with CT and MRI scans is essential. The surgeon must be aware of the high possibility that the posterior element anomaly may be proximal to the vertebral body anomaly both when considering how to excise the hemivertebra and for planning the fixation levels. Statement of interests: The authors declare that they have no competing interests

Introduction: Anterior convex epiphysiodesis and posterior concave distraction has not been previously described in the literature for the treatment of thoracolumbar hemivertebrae. We describe our experience with long-term follow-up. Methods: Six consecutive patients with a mean age of 3.4 years were operated on using this technique. The levels of fusion extended two levels above and below the hemivertebra, while the instrumentation spanned the full length of the curve. Further concave distraction was carried out when there was evidence of loosening of the hooks. Results: The average follow-up was 10.8 years (range: eight to 14 years). The mean Cobb angle before surgery was 49 degrees, and at the latest follow-up was 26 degrees. There mean improvement in the scoliosis was 41%. In five of these cases, this correction was achieved immediately after surgery and did not significantly change despite repeated distraction. Conclusions: The addition of concave distraction provided better correction than convex epiphysiodesis alone. This method of treatment is recommended for patients with single fully segmented hemivertebrae located at the thoracolumbar junction associated with a significant deformity. This method is technically easier and safer than excision of the hemivertebra in the correction of such deformities

Objective: To assess the clinical and radiological outcome of isolated hemivertebrae and multiple vertebral anomalies in the very young. Design: A cohort of patients with congenital scoliosis were identified on antenatal ultrasound and followed prospectively from twenty weeks in-utero (IU) for a mean of two years (range IU – five years). Subjects: Twenty fetuses with congenital hemivertebrae were identified from 16, 000 routine antenatal scans over a five-year period. Each was X-rayed at six weeks and investigated for renal and cardiac abnormalities. Outcome Measures: Clinical assessment included trunk balance, neurological abnormality and associated congenital abnormalities. Radiological assessment of curve magnitude and curve progression was performed using the Cobb method. The potential for progression (based on site, degree of incarceration, growth potential and presence of a congenital bar) was assessed. In all cases a clinical and radiological assessment was made at maximal follow-up. Results: One of the twenty fetuses aborted spontaneously at 23 weeks, three remain in-utero, leaving 16 live births. Thirteen of 20 had an isolated hemivertebra, and seven of twenty had multiple vertebral abnormalities. Two fetuses had renal agenesis, two had VATER’s syndrome and one had rib and abdominal wall abnormalities. The mean antenatal Cobb angle was 30°. IU curve progression was noted in three. Seven of the 16 live births required surgery based on curve magnitude, curve progression, truncal imbalance and the potential for progression. Surgery included anterior and posterior convex hemi-epiphyseodesis in two, and hemivertebra excision with fusion in five. The mean pre-operative Cobb angle was 36° (range 25–42) reducing to a mean of 21° (range 0–45) at maximal follow-up. Conclusions: Multiple vertebral abnormalities were more commonly associated with renal and cardiac abnormalities. If associated with the oligohyramnios sequence the fetus appeared to be at high risk. In general US detected isolated fetal hemivertebrae carry a good prognosis, nevertheless 38% of these cases at our institution underwent surgery within the first two years of life

The purpose of this study was to ascertain whether early diagnosis through antenatal ultrasound screening and intervention lead to a reduction in morbidity associated with congenital scoliosis. Prospective cohort study. All fetuses with vertebral body anomalies detected over a thirteen years period were included. Maternal risk factors, accuracy of antenatal diagnosis, associated fetal anomalies and ultimate outcome are described. Twenty-four fetuses with congenital hemivertebrae were identified from 39,000 antenatal scans (incidence 0.061%). The mothers' median age at conception was 26 years (range 18-40 years). The median fetal gestational age at diagnosis was 20 weeks (range 18-38 weeks). The median gestational age at delivery was 38 weeks (range 27-40). Antenatal ultrasound identified eighteen fetuses with a single hemivertebra, six fetuses with multiple hemivertebrae. Eleven patients (50%) have undergone early surgical intervention with median post-natal follow up was 4 years (range 0-9 years). The median age at surgery was 12 months (range 1.2 - 47 months). The median pre-operative Cobb angle was 33 degrees and the median Cobb angle at final follow up was 25 degrees. In this series 50% of patients required surgical intervention. We felt that pre-natal diagnosis of congenital vertebra abnormally has resulted in early surgical intervention using less complex surgical technique to halt progressive deformity. However, further studies are required to compare the results with the abnormally not detected antenatally

There is a wide range of reports on the prevalence of neurological injuries during scoliosis surgery, however this should depend on the subtypes and severity of the deformity. Furthermore, anterior versus posterior corrections pose different stresses to the spine, further quantifications of neurological risks are presented. Neuromonitoring data was prospectively entered, and the database between 2006 and 2012 was interrogated. All deformity cases under the age of 21 were included. Tumour, fracture, infection and revision cases were excluded. All “red alerts” were identified and detailed examinations of the neuromonitoring records, clinical notes and radiographs were made. Diagnosis, deformity severity and operative details were recorded. 2290 deformity operations were performed: 2068 scoliosis (1636 idiopathic, 204 neuromuscular, 216 syndromic, and 12 others), 89 kyphosis, 54 growing rod procedures, and 80 operations for hemivertebra. 696 anterior and 1363 posterior operations were performed for scoliosis (8 not recorded), and 38 anterior and 51 posterior kyphosis correction. 67 “red alerts” were identified, there were 14 transient and 6 permanent neurological injuries. 62 were during posterior stage (24 idiopathic, 21 neuromuscular, 15 syndromic (2 kyphosis), 1 growing rod procedure, 1 haemivertebra), and 5 were during anterior stage (4 idiopathic scoliosis and 1 syndromic kyphosis). Average Cobb angle was 88°. 1 permanent injuries were during correction for kyphosis, and 5 were for scoliosis (4 syndromic, 1 neuromuscular, and 1 anterior idiopathic). Common reactions after “red alerts” were surgical pause with anaesthetic interventions (n=39) and the Stagnara wake-up test (n=22). Metalwork was partially removed in 20, revised in 12 and completely removed in 9. 13 procedures were abandoned. The overall risk of permanent neurological injuries was 0.2%, the highest risk groups were posterior corrections for kyphosis and scoliosis associated with a syndrome. 4% of all posterior deformity corrections had “red alerts”, and 0.3% resulted in permanent injuries; compared to 0.6% “red alerts” and 0.3% permanent injuries for anterior surgery. The overall risk for idiopathic scoliosis was 0.06%

Introduction: Anterior convex epiphysiodesis and posterior concave distraction has not been previously described in the literature for the treatment of thoracolumbar hemivertebrae. We describe our experience with long-term follow-up. Method: Six consecutive patients with a mean age of 3.4 years were operated on with this technique. Levels of fusion extended two levels above and below the hemi-vertebra, while the instrumentation span the full length of the curve. Further concave distraction was carried out when there was evidence of loosening of the hooks. Results: The average follow-up was 10.8 years (range 8 to 14). The mean Cobb angle before surgery was 49°, and at the latest follow-up was 26°. There was a mean 41% improvement in the scoliosis. In 5 of these cases, this correction was achieved immediately after surgery and did not significantly change despite repeated distraction. Conclusion: The addition of concave distraction provided better correction than convex epiphysiodesis alone. It is technically easier and safer than hemivertebra excision in the correction of such deformities. This method of treatment is recommended for patients with single fully segmented hemivertebrae located at the thoracolumbar junction that has a significant deformity

Purpose. To report the results of full vertebral column resection (VCR) for paediatric spinal deformity. Methods and Results. All VCR (n=47) for paediatric spinal deformity were retrospectively evaluated from four university hospitals performing these procedures in Finland between 2005 and 2010. After excluding single hemivertebra (n=25) and resections performed for patients with MMC (n=6), 16 patients with full VCR (mean age at surgery 12.9 yrs [6.5-17.9] AIS 1; NMS 3; Congenital scoliosis 3 primary, revision 4; Kyphosis congenital 2, global 2; NF1 scoliosis 1) were identified. Seven procedures were performed anteroposteriorly and nine posterior-only. Mean follow-up time 1.9 (0.6–5.5) years. Major Curve (MC) averaged preoperatively 85 (58–120) degrees, 31 (14-53) degrees at 6 months, and 37 (17-80) degrees at 2-year follow-up. MC correction averaged 61 (46-86)% in the AP and 64 (57-83)% in the PL group at 6 months and 54 (18-86)% and 60 (41-70)% at 2-yr FU, respectively (NS). Blood loss averaged 3400 (500-8200) mL (NS between groups). The mean SRS-24 total scores were 100 (92-108) for AP and 102 (95-105) for PL group. There was one paraparesis in the AP group necessitating urgent re-decompression with full recovery. One peripheral L5 motor deficit resolved fully within few days (PL). Two junctional kyphosis were observed (one in both group). One one-sided partial lower instrumentation pull-out was observed without need for revision. One pseudoarthrosis occurred in AP group needing revision. Conclusions. Full VCR is rarely needed for paediatric spinal deformity with an estimated incidence of 2.9/million/year. Posterior VCR allows better control of neural elements during deformity correction

Introduction. The potential structural effect of the sacral tilt on the development of disc wedging adjacent to lowest instrumented vertebra (LEV), and consequent postoperative coronal trunk decompensation in the surgical treatment of idiopathic scoliosis are well known. The aim of this study is firstly to establish the possible role of sacral tilt, limb length inequality, and associated pelvic girdle anomalies in the cause of idiopathic scolisosis, and secondly to determine the effect of sacral tilt on adjacent disc wedging below LEV in some idiopathic curves such as thoracolumbar/lumbar curves, and double major curves postoperatively. Methods. Between 2006 and 2008, 159 patients with idiopathic scoliosis from outpatient clinics were included in analyses. In all patients, standing posteroanterior and lateral spinal column and Ferguson radiographs were routinely taken. If direct radiographs suggested iliac bone asymmetry, hemi-pelvis volume measurements were done with CT scan. Curve type, sacral tilt, L5 tilt, iliac asymmetry, and limb-length differences were measured. As a second part of study, 87 surgically treated patients with double major curves were examined retrospectively in terms of adjacent subjacent disc wedging below LEV and sacral tilt. Results. In the first part of the study, the mean age of patients was 14·21 years (range 2–25) and all but 14 were female. The curve types were lumbar in 31 patients, thoracolumbar in 42, double major in 55, thoracic in 27, and triple in four. Sacral tilt towards the convex side of TL/L curve was present in 117 (74%) patients with an average of 8° measured from Ferguson graphs. L5 tilt towards the convex side of TL/L curve, and limb length inequality of less than 5 mm on the convex side of TL/L curve were more prevalent in patients with sacral tilt (57%) than in those without sacral tilt (p=0·021). On hemi-pelvis volume measurement, patients with no sacral tilt (n=42) had no difference in both sides whereas patients with sacral tilt (n=117) showed significant difference, with the convex side being smaller. We noted a correlation between L5 tilt and sacral tilt (p=0·048) and between sacral tilt and hemi-pelvis volume (p=0·024). There was no correlation between sacral tilt and pelvic asymmetry, age, sex, curve type, or amount of limb length inequality. In the second part of the study, disc wedging adjacent to LEV was present in 75 of 87 patients and sacral tilt was present in 72 of 75 (96%) (p=0·008). Six patients had limb length inequality of more than 1 cm, showing the postoperative trunk decompensation, which was corrected by elevating the shorter leg by shoe-lift. Conclusions. This study revealed significant association between sacral tilt and hemi-pelvis hypoplasia independent from age. This association may have a primary role in the cause of this disease. Although LEV is paralel to pelvis, intervertebral disc adjacent to LEV is often wedged and there is lumbosacral hemicurve distally below the instrumentation in patients treated by either anterior or posterior surgery. We believe that lumbosacral hemicurve due to sacral tilt behaves like hemivertebra, which is impossible to detect with preoperative standing anteroposterior radiographs. Ferguson view is necessary to detect the sacral tilt. The postoperative trunk decompensation is greater with presence of sacral tilt and limb length inequality