Aims. To describe the clinical, radiological, and functional outcomes in patients with isolated congenital thoracolumbar kyphosis who were treated with three-column osteotomy by posterior-only approach. Methods. Hospital records of 27 patients with isolated congenital thoracolumbar kyphosis undergoing surgery at a single centre were retrospectively analyzed. All patients underwent deformity correction which involved a three-column osteotomy by single-stage posterior-only approach. Radiological parameters (local kyphosis angle (KA), thoracic kyphosis (TK), lumbar lordosis (LL), pelvic tilt (PT), sacral slope (SS), C7 sagittal vertical axis (C7 SVA), T1 slope, and pelvic incidence minus lumbar lordosis (PI-LL)), functional scores, and clinical details of complications were recorded. Results. The mean age of the study population was 13.9 years (SD 6.4). The apex of deformity was in thoracic, thoracolumbar, and lumbar spine in five, 14, and eight patients, respectively. The mean operating time was 178.4 minutes (SD 38.5) and the mean operative blood loss was 701.8 ml (SD 194.4). KA (preoperative mean 70.8° (SD 21.6°) vs final follow-up mean 24.7° (SD 18.9°); p < 0.001) and TK (preoperative mean -1.48° (SD 41.23°) vs final follow-up mean 24.28° (SD 17.29°); p = 0.005) underwent a significant change with surgery. Mean Scoliosis Research Society (SRS-22r) score improved after surgical correction (preoperative mean 3.24 (SD 0.37) vs final follow-up mean 4.28 (SD 0.47); p < 0.001) with maximum improvement in self-image and mental health domains. The overall complication rate was 26%, including two neurological and five non-neurological complications. Permanent neurological deficit was noted in one patient. Conclusion. Deformity correction employing three-column osteotomies by a single-stage posterior-only approach is safe and effective in treating isolated congenital thoracolumbar kyphosis. Cite this article: Bone Joint J 2021;103-B(7):1309–1316

Diastematomyelia is a rare congenital abnormality of the spinal cord. This paper summarises more than 30 years’ experience of treating this condition. Data were collected retrospectively on 138 patients with diastematomyelia (34 males, 104 females) who were treated at our hospital from May 1978 to April 2010. A total of 106 patients had double dural tubes (type 1 diastematomyelia), and 32 patients had single dural tubes (type 2 diastematomyelia). Radiographs, CT myelography, and MRI showed characteristic kyphoscoliosis, widening of the interpedicle distance, and bony, cartilaginous, and fibrous septum. The incidences of symptoms including characteristic changes of the dorsal skin, neurological disorders, and congenital spinal or foot deformity were significantly higher in type 1 than in type 2. Surgery is more effective for patients with type 1 diastematomyelia; patients without surgery showed no improvement

A combined anterior and posterior surgical approach is generally recommended in the treatment of severe congenital kyphosis, despite the fact that the anterior vascular supply of the spine and viscera are at risk during exposure. The aim of this study was to determine whether the surgical treatment of severe congenital thoracolumbar kyphosis through a single posterior approach is feasible, safe and effective. We reviewed the records of ten patients with a mean age of 11.1 years (5.4 to 14.1) who underwent surgery either by pedicle subtraction osteotomy or by vertebral column resection with instrumented fusion through a single posterior approach. The mean kyphotic deformity improved from 59.9° (45° to 110°) pre-operatively to 17.5° (3° to 40°) at a mean follow-up of 47.0 months (29 to 85). Spinal cord monitoring was used in all patients and there were no complications during surgery. These promising results indicate the possible advantages of the described technique over the established procedures. We believe that surgery should be performed in case of documented progression and before structural secondary curves develop. Our current strategy after documented progression is to recommend surgery at the age of five years and when 90% of the diameter of the spinal canal has already developed. Cite this article: Bone Joint J 2013;95-B:1527–32

The purpose of this cohort study is to determine the incidence of all congenital vertebral anomalies detected antenatally through ultrasound. We also reported on the early mortality rate for this patient cohort, as well as the frequency and type of associated congenital anomalies. The East Midlands and South Yorkshire Anomalies Register consists of data on all voluntary reports of congenital anomalies, from an annual baseline birth rate of 67000 births. We analysed all registered congenital anomalies reported over a 10 year period. Between January 1997 and January 2007, 108 vertebral anomalies were reported, excluding spinal dysraphism (incidence 0.01%). 61 of these were detected antenatally (56%), 17 were detected postnatally (16%) and in 30 patients, the precise time of diagnosis was unclear (28%). At the time of analysis January 2007, 45 of 108 patients had died, either in utero or soon after delivery (42%). 12 fetuses remained in utero and 51 infants were alive. The mortality rate for antenatally diagnosed patients was 41% and the majority were electively terminated (72%). 2 fetuses electively terminated had vertebral anomalies in isolation. There is a relatively high incidence of elective termination of pregnancy as a result of antenatal anomaly ultrasound screening. We have evidence to suggest that foetuses with potentially minor congenital anomalies are being electively terminated at approximately 18 weeks gestation. A structured and timely spinal counselling should be offered once antenatal vertebral anomalies have been identified

We describe two patients with an atypical congenital kyphosis in which a hypoplastic lumbar vertebral body lay in the spinal canal because of short pedicles. There were no defects in the posterior elements, or any apparent instability of the facet joints. Both patients were treated successfully by anterior fusion to the levels immediately above and below the affected vertebra, and posterior fusion which extended one level more both proximally and distally. This gave progressive correction of the kyphotic deformity by allowing some continued anterior growth at the levels which had been fused posteriorly

There are many various possibilities of treatment from observation and conservative treatment, over simple bony fusion to exacting hemivertebrectomies and deformity correction.

Retrospective analysis of various conservative and surgical techniques.

Subjects. The total number of 702 patients treated in our department since 1976 had been evaluated. An average follow up is 17 years.

Correction grade, security and efficiency of treatment methods, clinical results and complication rate were the main observed outcome measures.

Group A (conservative treatment) - 356 pts.

Group B (hemiepiphyseodesis) – 112 pts. Final result of correction was 9,8 degrees (22%).

Group C (posterior instrumentated fusion) – 147 pts. Final result of correction was 25,6° (38%).

Group D1 (anterior strut graft with posterior instrumentated fusion) – 27 pts. Final result of correction was 26° (40%).

Group D2 (anterior osteotomy with posterior instrumentated fusion) – 33 pts. Final result of correction was 28° (43%).

Group D3 (combined hemivertebrectomy with posterior instrumentated fusion) – 22 pts. Final result of correction was 31,3° (61%).

Group D4 (posterior only hemivertebrectomy with instrumentated fusion) – 5 pts. Final result of correction was 30,7° (61%).

Early detection, good timing and choosing of adequate surgical type are the main factors of quality treatment results. The best surgical method for formation failure types seems combined or posterior only instrumentated hemivertebrectomy and early hemiepiphyseodesis for segmentation failure types.

We present a study of ten consecutive patients who underwent excision of thoracic or thoracolumbar hemivertebrae for either angular deformity in the coronal plane, or both coronal and sagittal deformity. Vertebral excision was carried out anteriorly alone in two patients. Seven patients had undergone previous posterior spinal fusion. Their mean age at surgery was 13.4 years (6 to 19). The mean follow-up was 78.5 months (20 to 180). The results were evaluated by radiological review of the preoperative, postoperative and most recent follow-up films.

The mean preoperative coronal curve was 78.2° (30 to 115) and was corrected to 33.9° (7 to 58) postoperatively, a mean correction of 59%. Preoperative coronal decompensation of 35 mm was improved to 11 mm postoperatively. Seven patients had significant coronal decompensation preoperatively, which was corrected to a physiological range postoperatively. There were no major complications and no neurological damage.

We have shown that resection of thoracic and thoracolumbar hemivertebrae can be performed safely, without undue risk of neurological compromise, in experienced hands.

Aims. Spinal deformity surgery carries the risk of neurological injury. Neurophysiological monitoring allows early identification of intraoperative cord injury which enables early intervention resulting in a better prognosis. Although multimodal monitoring is the ideal, resource constraints make surgeon-directed intraoperative transcranial motor evoked potential (TcMEP) monitoring a useful compromise. Our experience using surgeon-directed TcMEP is presented in terms of viability, safety, and efficacy. Methods. We carried out a retrospective review of a single surgeon’s prospectively maintained database of cases in which TcMEP monitoring had been used between 2010 and 2017. The upper limbs were used as the control. A true alert was recorded when there was a 50% or more loss of amplitude from the lower limbs with maintained upper limb signals. Patients with true alerts were identified and their case history analyzed. Results. Of the 299 cases reviewed, 279 (93.3%) had acceptable traces throughout and awoke with normal clinical neurological function. No patient with normal traces had a postoperative clinical neurological deficit. True alerts occurred in 20 cases (6.7%). The diagnoses of the alert group included nine cases of adolescent idiopathic scoliosis (AIS) (45%) and six of congenital scoliosis (30%). The incidence of deterioration based on diagnosis was 9/153 (6%) for AIS, 6/30 (20%) for congenital scoliosis, and 2/16 (12.5%) for spinal tuberculosis. Deterioration was much more common in congenital scoliosis than in AIS (p = 0.020). Overall, 65% of alerts occurred during rod instrumentation: 15% occurred during decompression of the internal apex in vertebral column resection surgery. Four alert cases (20%) awoke with clinically detectable neurological compromise. Conclusion. Surgeon-directed TcMEP monitoring has a 100% negative predictive value and allows early identification of physiological cord distress, thereby enabling immediate intervention. In resource constrained environments, surgeon-directed TcMEP is a viable and effective method of intraoperative spinal cord monitoring. Level of evidence: III. Cite this article: Bone Joint J 2021;103-B(3):547–552

SUMMARY. A retrospective cohort study of 19 patients of EOS who underwent MCGR rod instrumentation with subsequent serial distractions, measured using ultrasonography. The degree of distraction achieved during each session were analyzed and subgroup analysis done. HYPOTHESIS. The degree of distraction/ distractibility of the MCGR rod is closely linked to the etiology of EOS. The degree of distraction achieved at each session progressively diminishes with successive distractions. STUDY DESIGN. Retrospective cohort study. INTRODUCTION. MCGR rods have gained popularity as an alternative to traditional growing rods for the treatment of Early-onset scoliosis (EOS), serially distracting with an aim to mimic the normal spinal growth, which can be achieved in an outpatient setting using external remote controller. The use of ultrasound as a tool to measure the degree of distraction achieved has been validated previously. However the association between the etiological diagnosis of EOS and the degree of distraction achieved has not been studied previously. METHODS. We performed a retrospective cohort study of 19 patients with EOS of varying etiologies who underwent MCGR rod instrumentation at our institution since 2016. Their hospital records were analyzed to assess the demographics, distraction interval and the degree of distraction achieved at each session. RESULTS. 19 patients (12 females and 7 males) were included in the study. The average age of the study population was 7 years (4–13 years). The average follow up was 2.1 years (1–4). The following were the etiological diagnoses: Idiopathic EOS(8), Neuromuscular scoliosis(5), Syndromic(5), Congenital(1). The average interval between each distraction was 109.8days (88.2–140.6). The overall mean distraction was 1.66mm (0.6–3.427). The mean distraction achieved for the concave rod was 1.69mm (0.6–3.03) as against 1.59mm (0.2–3.427) for the convex rod. The degree of distraction achieved per session was the greatest in patients with neuromuscular scoliosis 2.79mm (0.94–4.62), while it was the least in Congenital scoliosis 0.99mm (0.8–2.93). The degree of distraction in Syndromic and Idiopathic scoliosis groups were 2.19mm (0.1–4.2) and 1.50mm (0.2–4.45) respectively. The mean distraction achieved during the first session after MCGR instrumentation was 2.82mm (0.4–9.8) as against 1.98mm (0.1–4.2) and 1.18mm (0.2–2.3), achieved during the 5. th. and 10. th. distractions respectively. CONCLUSIONS. The average distraction of the MCGR rod achieved per session depends upon the etiological diagnosis of EOS. Neuromuscular curves are the most amenable to MCGR distractions, while the congenital curves are the least. The degree of distraction achieved progressively diminishes with each successive distractions. The distraction achieved slumps to 70% of the initial distraction by the 5. th. session and further declines to 41% of the initial distraction by the 10. th. session. TAKE HOME MESSAGE. The distractibility of the MCGR rod is closely linked to the etiology of EOS. MCGR rods are most effective in cases of neuromuscular scoliosis, while least effective in congenital scoliosis. The law of diminishing returns holds true even with MCGR rods

Aims. The outcome following the development of neurological complications after corrective surgery for scoliosis varies from full recovery to a permanent deficit. This study aimed to assess the prognosis and recovery of major neurological deficits in these patients, and to determine the risk factors for non-recovery, at a minimum follow-up of two years. Methods. A major neurological deficit was identified in 65 of 8,870 patients who underwent corrective surgery for scoliosis, including eight with complete paraplegia and 57 with incomplete paraplegia. There were 23 male and 42 female patients. Their mean age was 25.0 years (SD 16.3). The aetiology of the scoliosis was idiopathic (n = 6), congenital (n = 23), neuromuscular (n = 11), neurofibromatosis type 1 (n = 6), and others (n = 19). Neurological function was determined by the American Spinal Injury Association (ASIA) impairment scale at a mean follow-up of 45.4 months (SD 17.2). the patients were divided into those with recovery and those with no recovery according to the ASIA scale during follow-up. Results. The incidence of major deficit was 0.73%. At six-month follow-up, 39 patients (60%) had complete recovery and ten (15.4%) had incomplete recovery; these percentages improved to 70.8% (46) and 16.9% (11) at follow-up of two years, respectively. Eight patients showed no recovery at the final follow-up. The cause of injury was mechanical in 39 patients and ischaemic in five. For 11 patients with misplaced implants and haematoma formation, nine had complete recovery. Fisher’s exact test showed a significant difference in the aetiology of the scoliosis (p = 0.007) and preoperative deficit (p = 0.016) between the recovery and non-recovery groups. A preoperative deficit was found to be significantly associated with non-recovery (odds ratio 8.5 (95% confidence interval 1.676 to 43.109); p = 0.010) in a multivariate regression model. Conclusion. For patients with scoliosis who develop a major neurological deficit after corrective surgery, recovery (complete and incomplete) can be expected in 87.7%. The first three to six months is the time window for recovery. In patients with misplaced implants and haematoma formation, the prognosis is satisfactory with appropriate early intervention. Patients with a preoperative neurological deficit are at a significant risk of having a permanent deficit. Cite this article: Bone Joint J 2022;104-B(1):103–111

Introduction. Lumbosacral transitional vertebrae (LSTV) are increasingly recognized as a common anatomical variant and is the most common congenital anomaly of the lumbosacral spine. Patients can have symptomatic LSTV, known as Bertolotti's Syndrome, where transitional anatomy can cause back, L5 distribution leg, hip, and groin pain. We propose an outline for diagnosis and treatment of Bertolotti's Syndrome. Methods. We retrospectively reviewed over 500 patients presenting to the primary author with low back, buttock, hip, groin and/or leg pain from April 2009 through April 2024. Patients with radiographic findings of an LSTV and clinical presentation underwent diagnostic injections to confirm diagnosis of Bertolotti's syndrome. Treatment was determined based on patient's LSTV classification. 157 patients with confirmed Bertolotti's syndrome underwent surgical treatment. Results. Over 500 patients presented with an appropriate clinical presentation and radiographic findings of an LSTV. Diagnostic injections were targeted into the transitional anatomy confirming the LSTV as the primary pain generator to make the diagnosis of Bertolotti's syndrome. The decision in the type of surgical intervention, resection or fusion, was made based on patient's LSTV anatomy. 157 patients with confirmed Bertolotti's Syndrome underwent surgical treatment (121 fusions (77%), 36 resections (23%)). The classification system and surgical outcomes, in part, have been previously published in World Neurosurgery. Conclusion. We have outlined the best practice of diagnosis and treatment selection for Bertolotti's syndrome. We have shown significant improvement in outcomes based on this method. We hope to aid in both patient education and provide an outline on how clinicians can become knowledgeable on Bertolotti's syndrome. Conflicts of interest. No conflicts of interest. Sources of funding. No funding obtained

Aims. Magnetically controlled growing rods (MCGR) have been gaining popularity in the management of early-onset scoliosis (EOS) over the past decade. We present our experience with the first 44 MCGR consecutive cases treated at our institution. Methods. This is a retrospective review of consecutive cases of MCGR performed in our institution between 2012 and 2018. This cohort consisted of 44 children (25 females and 19 males), with a mean age of 7.9 years (3.7 to 13.6). There were 41 primary cases and three revisions from other rod systems. The majority (38 children) had dual rods. The group represents a mixed aetiology including idiopathic (20), neuromuscular (13), syndromic (9), and congenital (2). The mean follow-up was 4.1 years, with a minimum of two years. Nine children graduated to definitive fusion. We evaluated radiological parameters of deformity correction (Cobb angle), and spinal growth (T1-T12 and T1-S1 heights), as well as complications during the course of treatment. Results. The mean Cobb angles pre-operatively, postoperatively, and at last follow-up were 70° (53 to 103), 35° (15 to 71) and 39° (15 to 65) respectively (p < 0.001). Further, there was a mean of 14° (-6 to 27) of additional Cobb angle correction upon graduation from MCGR to definitive fusion. Both T1-T12 and T1-S1 showed significant increase in heights of 27 mm and 45 mm respectively at last follow-up (p < 0.001). Ten children (23%) developed 18 complications requiring 21 unplanned operations. Independent risk factors for developing a complication were single rod constructs and previous revision surgery. Conclusion. MCGR has the benefit of avoiding multiple surgeries, and is an effective tool in treatment of early-onset scoliosis. It also maintains the flexibility of the spine, allowing further correction at the time of definitive fusion. Cite this article: Bone Joint Open 2020;1-7:405–414

Segmental vessel ligation during anterior spinal surgery has been associated with paraplegia. However, the incidence and risk factors for this devastating complication are debated. We reviewed 346 consecutive paediatric and adolescent patients ranging in age from three to 18 years who underwent surgery for anterior spinal deformity through a thoracic or thoracoabdominal approach, during which 2651 segmental vessels were ligated. There were 173 patients with idiopathic scoliosis, 80 with congenital scoliosis or kyphosis, 43 with neuromuscular and 31 with syndromic scoliosis, 12 with a scoliosis associated with intraspinal abnormalities, and seven with a kyphosis. There was only one neurological complication, which occurred in a patient with a 127° congenital thoracic scoliosis due to a unilateral unsegmented bar with contralateral hemivertebrae at the same level associated with a thoracic diastematomyelia and tethered cord. This patient was operated upon early in the series, when intra-operative spinal cord monitoring was not available. Intra-operative spinal cord monitoring with the use of somatosensory evoked potentials alone or with motor evoked potentials was performed in 331 patients. This showed no evidence of signal change after ligation of the segmental vessels. In our experience, unilateral segmental vessel ligation carries no risk of neurological damage to the spinal cord unless performed in patients with complex congenital spinal deformities occurring primarily in the thoracic spine and associated with intraspinal anomalies at the same level, where the vascular supply to the cord may be abnormal

Aims

Developmental cervical spinal stenosis (DcSS) is a well-known predisposing factor for degenerative cervical myelopathy (DCM) but there is a lack of consensus on its definition. This study aims to define DcSS based on MRI, and its multilevel characteristics, to assess the prevalence of DcSS in the general population, and to evaluate the presence of DcSS in the prediction of developing DCM.

We report the results of vertebral column resection (VCR) for paediatric patients with spinal deformity. A total of 49 VCRs in paediatric patients from four university hospitals between 2005 and 2009 with a minimum two-year follow-up were retrospectively identified. After excluding single hemivertebral resections (n = 25) and VCRs performed for patients with myelomeningocele (n = 6), as well as spondylectomies performed for tumour (n = 4), there were 14 patients who had undergone full VCR at a mean age of 12.3 years (6.5 to 17.9). The aetiology was congenital scoliosis in five, neuromuscular scoliosis in three, congenital kyphosis in two, global kyphosis in two, adolescent idiopathic scoliosis in one and secondary scoliosis in one. A total of seven anteroposterior and seven posterolateral approaches were used. The mean major curve deformity was 86° (67° to 120°) pre-operatively and 37° (17° to 80°) at the two-year follow-up; correction was a mean of 54% (18% to 86%) in the anteroposterior and 60% (41% to 70%) in the posterolateral group at the two-year follow-up (p = 0.53). The mean Scoliosis Research Society-24 total scores were 100 (92 to 108) for the anteroposterior and 102 (95 to 105) for the posterolateral group. There was one paraparesis in the anteroposterior group necessitating urgent re-decompression, with a full recovery. Patients undergoing VCR are highly satisfied after a successful procedure

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

Aims

Only a few studies have investigated the long-term health-related quality of life (HRQoL) in patients with an idiopathic scoliosis. The aim of this study was to investigate the overall HRQoL and employment status of patients with an idiopathic scoliosis 40 years after diagnosis, to compare it with that of the normal population, and to identify possible predictors for a better long-term HRQoL.

Aims

This systematic review aims to identify 3D predictors derived from biplanar reconstruction, and to describe current methods for improving curve prediction in patients with mild adolescent idiopathic scoliosis.

Aims

Lumbar spinal stenosis (LSS) is a common skeletal system disease that has been partly attributed to genetic variation. However, the correlation between genetic variation and pathological changes in LSS is insufficient, and it is difficult to provide a reference for the early diagnosis and treatment of the disease.

Aims

The aim of this study was to compare outcomes after growth-friendly treatment for early-onset scoliosis (EOS) between patients with skeletal dysplasias versus those with other syndromes.