Aims. To report the outcome of spinal deformity correction through anterior spinal fusion in wheelchair-bound patients with myelomeningocele. Methods. We reviewed 12 consecutive patients (7M:5F; mean age 12.4 years (9.2 to 16.8)) including demographic details, spinopelvic parameters, surgical correction, and perioperative data. We assessed the impact of surgery on patient outcomes using the Spina Bifida Spine Questionnaire and a qualitative questionnaire. Results. The mean follow-up was 5.4 years (2 to 14.9). Nine patients had kyphoscoliosis, two lordoscoliosis, and one kyphosis. All patients had a thoracolumbar deformity. Mean scoliosis corrected from 89.6° (47° to 151°) to 46.5° (17° to 85°; p < 0.001). Mean kyphosis corrected from 79.5° (40° to 135°) to 49° (36° to 65°; p < 0.001). Mean pelvic obliquity corrected from 19.5° (8° to 46°) to 9.8° (0° to 20°; p < 0.001). Coronal and sagittal balance restored to normal. Complication rate was 58.3% (seven patients) with no neurological deficits, implant failure, or revision surgery. The degree of preoperative spinal deformity, especially kyphosis and lordosis, correlated with increased blood loss and prolonged hospital/intensive care unit stay. The patients reported improvement in function, physical appearance, and pain after surgery. The parents reported decrease in need for everyday care. Conclusion. Anterior spinal fusion achieved satisfactory deformity correction with high perioperative complication rates, but no long-term sequelae among children with high level myelomeningocele. This resulted in physical and functional improvement and high reported satisfaction. Cite this article: Bone Joint J 2021;103-B(6):1133–1141

Introduction. Kyphoscoliosis is defined by a structural lateral curvature of the spine of 10° or more and an excessive thoracic kyphotic curve of 40° or more. Genetic analyses of families in which two or more members had kyphoscoliosis identified a 3·5 Mb area on chromosome 5p containing three genes of the Iroquois (IRX) homeobox family, IRX1, IRX2, and IRX4, which were then sequenced. Methods. Exons and highly conserved non-coding regions (HNCRs) 500 kb upstream and downstream fromIRX1, IRX2, and IRX4 were sequenced in 46 individuals from six families. Selection of these elements was based on PhastCons Placental Mammal Conserved Elements, Multiz Alignment. Single-nucleotide polymorphism (SNP) genotypes and sequence variants were obtained from all individuals. There were 431 SNPs, 61 in IRX4 regions, 80 in IRX2 regions, and 290 in IRX1 regions. 137 SNPs were novel. Mendelian inconsistencies were detected with PEDCHECK (inconsistency rate: 1·4%; missing data: 2·8%). SNPs and individuals with greater than 10% missing rate were excluded. Association analyses (ASSOC [SAGE version 6.0.1]) of the quantitative trait with patient's largest curve, were undertaken on 391 SNPs. Results. Association analyses resulted in 12 SNPs with p values less than 0·025, 11 of which were located upstream and downstream from IRX1. The most significant p value (p=0·000382) was obtained for rs35710183 (table). Multiple variants were found surrounding IRX1. The most prominent is a single base-pair deletion in all affected individuals genotyped in one family. All individuals with kyphoscoliosis and those with scoliotic curves greater than 35° had genotypes differing from the reference (unaffected) genotype for 23 SNPs. Several of these SNPs had significant p values for the association analyses done previously. Conclusions. The phenotype of kyphoscoliosis has been linked to sequence variants that lie within regulatory regions of the IRX homeobox gene family. Further analyses to establish the relevance of these findings will be done through in-vivo and in-vitro assays. The identification of spinal genetic determinants related to axial growth and maturation will help with the understanding of spinal pathology and potentially allow for development of directed therapeutic interventions

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

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.

To describe complications and reoperation rates associated with degenerative spinal deformity surgery. A retrospective review of prospectively collected data from a single spinal surgeon in the United Kingdom. A total of 107 patients who underwent surgery, of 5 or more levels, for primary degenerative kyphoscoliosis between 2006 and 2012 were identified. Clinical notes were reviewed and post-operative complications, reoperation rates, length of follow up and mortality were analysed. A total of 107 patients, average aged 66.5 years (range 52 – 85), with 80% women. 105 patients underwent posterior surgery, two patients required both anterior and posterior surgery. The average number of instrumented levels was 8.3; 10% 5 levels, 15% 6 levels, 11% 7 levels, 14% 8 levels, 15% 9 levels and 35% had fusions of 10 levels and above. 58% included fixation to sacrum or pelvis. 93% had a decompression performed and 30% had an osteotomy. There were 40 complications recorded within the follow-up period. Infection occurred in 7 patients (6.5%). All were successfully managed with debridement, antibiotic therapy and retention of implants. There were 4 dural tears (3.7%). One patient developed a post-operative DVT (0.9%). No patients sustained cord level deficits. Prevalence of mechanical complications requiring re-operation was 26% (28 patients). 5 patients (4.7) required revision surgery for symptomatic pseudarthrosis, 7 patients (6.5%) underwent revision fixation for metal work failure (broken rods/screw pull-out) and 16 patients (14.9%) underwent revision surgery to extend fixation proximally or distally due to adjacent segment disease (symptomatic proximal junction kyphosis 4.7%; osteoporotic fracture 3.7% and junctional/nerve root pain 6.5%). Overall reoperation rate was 32.5% at an average of 1.9 years following primary surgery (range 1 week–6 years). 37% patients remain on regular outpatient review (average 3.8 years following first surgery; range 2–6 years). 52% have been discharged after a mean follow-up of 2.3 years. 11 patients had died since their surgery (10.2%) at an average 4.1 years following their spinal surgery (range 1 –5.9 years). Overall complication rate was 37.3%. 32.5% of patients were re-operated for infective or mechanical complications. 52% of patients had been discharged at an average of 2.3 years following their surgery. 10.2% of patients had died within 6 years of surgery

Study design. Prospective clinical and radiological analysis of children with complex cervical deformities for the safety of cervical pedicle screw insertion. Objectives. To analyse the possibility, safety and efficacy of cervical pedicle screw insertion in complex pediatric cervical deformities, where conventional stabilisation techniques would not have provided rigid fixation. Summary of Background Data. Although the usage of cervical pedicle screws (CPS) in adults has become established, the feasibility and safety of its application in children has not been described previously in the literature. Methods. Sixteen children of mean age 9.7 ± 2.6 years (range: 3 - 13) requiring spinal stabilization for cranio-vertebral junction anomalies (n=10), cervico-thoracic kyphosis/ kyphoscoliosis (n=5) and cervical tumor excision (n=1) formed the study group. Feasibility of CPS insertion was assessed by computerised tomography images. Standard 3.0 mm titanium pedicle screws were inserted using intraoperative Iso-C C arm based 3 D computer navigation and the containment was post operatively evaluated with CT scan. Results. Based on preoperative CT imaging, 55 pedicles were selected for screw fixation. Intra operatively CPS was successfully inserted at 51 levels and at four sclerosed pedicles (7.3%), screws could not be inserted. At 42 levels, the screws were inserted in the classical description of pedicle screw application and in nine deformed vertebra, the screws were inserted in a non-classical fashion, taking purchase in the three columns of the cervical vertebra. Forty five (88.3%) screws were fully contained, six (11.7 %) had a non-critical breach and none had a critical breach. No perioperative complications related to pedicle screw insertion were noted. Conclusion. Safe insertion of cervical pedicle screws is possible in children. Iso-C navigation provides real time virtual imaging and improves the safety and accuracy of successful pedicle fixation even in altered vertebral anatomy. Pedicle width morphometrics do not restrict screw insertion

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.

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.

Aims

The aim of this study is to test the hypothesis that three grades of sagittal compensation for standing posture (normal, compensated, and decompensated) correlate with health-related quality of life measurements (HRQOL).

We determined the frequency, rate and extent of development of scoliosis (coronal plane deformity) in wheelchair-dependent patients with Duchenne muscular dystrophy (DMD) who were not receiving steroid treatment. We also assessed kyphosis and lordosis (sagittal plane deformity). The extent of scoliosis was assessed on sitting anteroposterior (AP) spinal radiographs in 88 consecutive non-ambulatory patients with DMD. Radiographs were studied from the time the patients became wheelchair-dependent until the time of spinal fusion, or the latest assessment if surgery was not undertaken. Progression was estimated using a longitudinal mixed-model regression analysis to handle repeated measurements.

Scoliosis ≥ 10° occurred in 85 of 88 patients (97%), ≥ 20° in 78 of 88 (89%) and ≥ 30° in 66 of 88 patients (75%). The fitted longitudinal model revealed that time in a wheelchair was a highly significant predictor of the magnitude of the curve, independent of the age of the patient (p <  0.001). Scoliosis developed in virtually all DMD patients not receiving steroids once they became wheelchair-dependent, and the degree of deformity deteriorated over time.

In general, scoliosis increased at a constant rate, beginning at the time of wheelchair-dependency (p < 0.001). In some there was no scoliosis for as long as three years after dependency, but scoliosis then developed and increased at a constant rate. Some patients showed a rapid increase in the rate of progression of the curve after a few years – the clinical phenomenon of a rapidly collapsing curve over a few months.

A sagittal plane kyphotic deformity was seen in 37 of 60 patients (62%) with appropriate radiographs, with 23 (38%) showing lumbar lordosis (16 (27%) abnormal and seven (11%) normal).

This study provides a baseline to assess the effects of steroids and other forms of treatment on the natural history of scoliosis in patients with DMD, and an approach to assessing spinal deformity in the coronal and sagittal planes in wheelchair-dependent patients with other neuromuscular disorders.

Cite this article: Bone Joint J 2014;96-B:100–5.

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.

Aims

There is a paucity of information on the pre-operative coronal imbalance in patients with degenerative lumbar scoliosis (DLS) and its influence on surgical outcomes.

Aims

The aim of this study is to introduce and investigate the efficacy and feasibility of a new vertebral osteotomy technique, vertebral column decancellation (VCD), for rigid thoracolumbar kyphotic deformity (TLKD) secondary to ankylosing spondylitis (AS).

We describe 13 patients with cerebral palsy and lordoscoliosis/hyperlordosis of the lumbar spine who underwent a posterior spinal fusion at a mean age of 14.5 years (10.8 to 17.4) to improve sitting posture and relieve pain. The mean follow-up was 3.3 years (2.2 to 6.2).

The mean pre-operative lumbar lordosis was 108^° (80 to 150^°) and was corrected to 62^° (43^° to 85^°); the mean thoracic kyphosis from 17^° (-23^° to 35^°) to 47^° (25^° to 65^°); the mean scoliosis from 82^° (0^° to 125^°) to 22^° (0^° to 40^°); the mean pelvic obliquity from 21^° (0^° to 38^°) to 3^° (0^° to 15^°); the mean sacral slope from 79^° (54^° to 90^°) to 50^° (31^° to 66^°). The mean pre-operative coronal imbalance was 5 cm (0 cm to 8.9 cm) and was corrected to 0.6 cm (0 to 3.2). The mean sagittal imbalance of -8 cm (-16 cm to 7.8 cm) was corrected to -1.6 cm (-4 cm to 2.5 cm). The mean operating time was 250 minutes (180 to 360 minutes) and intra-operative blood loss 0.8 of estimated blood volume (0.3 to 2 estimated blood volume). The mean intensive care and hospital stay were 3.5 days (2 to 8) and 14.5 days (10 to 27), respectively. Three patients lost a significant amount of blood intra-operatively and subsequently developed chest or urinary infections and superior mesenteric artery syndrome.

An increased pre-operative lumbar lordosis and sacral slope were associated with increased peri-operative morbidity: scoliosis and pelvic obliquity were not. A reduced lumbar lordosis and increased thoracic kyphosis correlated with better global sagittal balance at follow-up. All patients and their parents reported excellent surgical outcomes.

Lordoscoliosis and hyperlordosis are associated with significant morbidity in quadriplegic patients. They are rare deformities and their treatment is challenging. Sagittal imbalance is the major component: it can be corrected by posterior fusion of the spine with excellent functional results.

Cite this article: Bone Joint J 2014;96-B:800–6.

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.

Osteoporotic vertebral compression fractures (VCFs) are an increasing public health problem. Recently, randomised controlled trials on the use of kyphoplasty and vertebroplasty in the treatment of these fractures have been published, but no definitive conclusions have been reached on the role of these interventions. The major problem encountered when trying to perform a meta-analysis of the available studies for the use of cementoplasty in patients with a VCF is that conservative management has not been standardised. Forms of conservative treatment commonly used in these patients include bed rest, analgesic medication, physiotherapy and bracing.

In this review, we report the best evidence available on the conservative care of patients with osteoporotic VCFs and associated back pain, focusing on the role of the most commonly used spinal orthoses. Although orthoses are used for the management of these patients, to date, there has been only one randomised controlled trial published evaluating their value. Until the best conservative management for patients with VCFs is defined and standardised, no conclusions can be drawn on the superiority or otherwise of cementoplasty techniques over conservative management.

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.