We reviewed 47 patients with neurofibromatosis and dystrophic spinal deformities; 32 of these patients had been untreated for an average of 3.6 years and in them the natural history was studied. The commonest pattern of deformity at the time of presentation was a short angular thoracic scoliosis, but with progression the angle of kyphosis also increased. Deterioration during childhood was usual but its rate was variable. Severe dystrophic changes in the apical vertebrae and in particular anterior scalloping have a poor prognosis for deterioration. The dystrophic spinal deformity of neurofibromatosis requires early surgical stabilisation which should be by combined anterior and posterior fusion if there is an abnormal angle of kyphosis or severely dystrophic apical vertebrae. Some carefully selected patients can be treated by posterior fusion and instrumentation alone.

Thirty-four patients with adolescent idiopathic scoliosis were assessed by radiography and the integrated shape imaging system (ISIS) both before and after spinal surgery. Twenty-seven patients underwent Harrington instrumentation, after which lateral indices of curvature were significantly improved, but changes in the transverse plane were less pronounced. Sublaminar wiring was carried out in two patients whose thoracic lordosis was corrected by the surgery. Five patients whose severe deformity had persisted after previous spinal surgery underwent costoplasty, which resulted in a significant improvement in back shape measurements. We conclude that the cosmetic deformity of the back in scoliosis is only partially corrected by operations on the spine itself, whilst costoplasty addresses the problem directly, and improves the surface shape.

Four cases are presented of a syndrome of progressive external ophthalmoplegia and scoliosis occurring within one family. These patients were extensively investigated but no biochemical abnormality was detected.

1. The results of posterior spinal fusion for paralytic scoliosis in 118 patients have been reviewed after growth had finished. The criteria for skeletal maturity were both clinical and radiological, with emphasis on ossification of the iliac apophyses.

2. The age of onset of anterior poliomyelitis and the age at which scoliosis was first noticed, as well as the extent of the muscle weakness and the curve patterns, all have a bearing on the severity of the deformity and the indication for operative treatment.

3. The method of treatment including operation is described and the complications detailed. The use of a tibial strut has now been abandoned and Harrington instrumentation has become routine.

4. There were five deaths in the series, three early and two late.

5. The difference in height, changes in respiratory function and eventual functional capacity have been analysed.

1. A family study of sixty-one children with infantile idiopathic scoliosis to determine the relationship of other developmental anomalies to the behaviour of the curve is reported.

2. Of the thirty-nine children with resolving curves only one had another defect.

3. Twelve out of twenty-two children with non-resolving curves had at least one other developmental anomaly.

4. The presence of such defects may indicate that the curve is likely to progress.

It is concluded that the development of scoliosis is due to muscle imbalance, the weaker muscles being on the convex side of the curve. It is suggested that the increased electrical activity in the muscles on the convex side is secondary to the scoliosis, being part of the body's attempt to compensate for the curvature.

The principle of Occam's razor proves nothing. Nevertheless, it is possible to explain all the phenomena of severe scoliosis on the basis of a primary rotation deformity alone. The typical rotation type of scoliotic deformity can be reproduced artificially by fitting vertebrae together in an abnormal rotatory relationship without any element of lateral flexion. From this, certain mechanical factors inevitably come into play which must tend to increase the deformity. Above all, the forces responsible for progressive scoliosis are dynamic and active, not just passive. The spine readily compensates for a passive, non-progressive deformity such as a simple wedge vertebra. It is my belief that rotation is usually the dominant factor and that correction and control of severe scoliosis can only be achieved by concentrating on the rotation deformity. I am well aware that this is an old idea but its essential truth has been insufficiently appreciated in recent years and we have not faced its full implications. Failure to correct rotation invites recurrence. Conversely, even a slight reduction in rotation usually produces a marked cosmetic improvement, often out of all proportion to the radiographic appearances.

1. The natural history and prognosis of progressive infantile idiopathic scoliosis are reviewed and twenty-eight cases are reported.

2. Resolving infantile idiopathic scoliosis is described and seven cases are reported.

3. The length of the curve, the degree of rotation, the age of onset of deterioration, and the rate of progression are the important factors in determining the type and severity of the deformity.

4. Infantile idiopathic scoliosis is briefly compared with congenital scoliosis.

Infantile idiopathic scoliosis is a structural scoliosis seen in infants, usually boys, with the major curve to the left in almost all cases, and almost invariably in the mid-or lower thoracic region. It occasionally disappears, but in general the curve tends to increase. In the absence of any discoverable etiology it is termed "idiopathic" and it is believed not to differ in essentials from the more common adolescent scoliosis.

Lumbar idiopathic scoliosis has a good prognosis as to deformity, but leads more often than any other curvature to degenerative arthritis and pain in later life.

1. The records of forty-nine patients with idiopathic structural scoliosis in infancy treated by a combination of splinting and physiotherapy have been reviewed.

2. Nine curves (18·3 per cent) failed to respond to treatment and five of these progressed.

3. The percentage of good results (81·7 per cent) shows no improvement on the natural course of the condition.

Aims. Closed suction subfascial drainage is widely used after instrumented posterior spinal fusion in patients with a spinal deformity. The aim of this study was to determine the effect of this wound drainage on the outcomes in patients with adolescent idiopathic scoliosis (AIS). This was a further analysis of a randomized, multicentre clinical trial reporting on patients after posterior spinal fusion using segmental pedicle screw instrumentation. In this study the incidence of deep surgical site infection (SSI) and chronic postoperative pain at two years’ follow-up are reported. Methods. We conducted a randomized, multicentre clinical trial on adolescents undergoing posterior spinal fusion for AIS using segmental pedicle screw instrumentation. A total of 90 consecutive patients were randomized into a ‘drain’ or ‘no drain’ group at the time of wound closure, using the sealed envelope technique (1:1). The primary outcomes in the initial study were the change in the level of haemoglobin in the blood postoperatively and total blood loss. A secondary outcome was the opioid consumption immediately after surgery. The aim of this further study was to report the rate of deep SSI and persistent postoperative pain, at two years' follow-up. Results. As previously reported, the mean 48-hour opioid consumption was significantly higher in the no drain group (2.0 mg/kg (SD 0.8) vs 1.4 mg/kg (SD 0.7); p = 0.005). There were no delayed deep SSIs. At two years’ follow-up, the mean Scoliosis Research Society 24-item questionnaire (SRS-24) total score did not differ between the groups (4.11 (SD 0.47) vs 4.16 (SD 0.33); p = 0.910). The mean SRS-24 pain score was 4.28 (SD 0.48) in the no drain group compared with 4.33 (SD 0.66) in the drain group (p = 0.245). Seven patients (19%) in the no drain group and six in the drain group (14%) reported moderate to severe pain (numerical rating scale ≥ 4) at two years’ follow-up (p = 0.484). The general self-image domain score was significantly higher in the no drain group compared with the drain group (4.02 (SD 0.74) vs 4.59 (SD 0.54); p < 0.001). Conclusion. The main finding in this study was that more severe pain immediately after surgery did not result in a higher incidence of chronic pain in the no drain group compared with the drain group. Back pain and health-related quality of life at two years’ follow-up did not differ between the groups. The patient-reported self-image domain was significantly better in the no drain group compared with the drain group. Cite this article: Bone Joint J 2024;106-B(10):1176–1181

1. A study of serial radiographs of 138 children with infantile scoliosis starting under the age of two years has resulted in a method of early differentiation between resolving and progressive curves.

2. The method involves careful measurement of the rib-vertebra angles at the apex of the curve and observation of the relationship of the head of the rib to the body of the vertebra as seen in antero-posterior radiographs.

3. The influence of the infantile growth spurt upon progressive curves is examined and the importance of early diagnosis and treatment is stressed.

1. Twelve patients with suspected pseudarthroses in previous spine grafts were subjected to operation.

2. Correction of the curves was produced by Harrington's instrumentation at the sites of pseudarthroses, osteotomies and fractures.

3. A significant increase in stature was produced in all the patients.

4. Harrington's hooks were safely inserted into cavities cut in intact portions of previous grafts.

5. Most patients were able to leave hospital in less than four weeks.

6. Deliberate bending of the Harrington rods has not led to subsequent fracture of the rod.

7. A sliding scoliosis fusion frame is described. It reduces friction during correction and it reduces haemorrhage during surgery.

1 . The theoretical requirements for optimal correction of thoracic scoliosis and kyphosis are discussed, and observations are made on the limitations of methods in current use for their ambulant correction.

2. The experimental use of hydrostatic pressure in an endeavour to satisfy these clinical requirements is described.

3. It is suggested that the use of hydrostatic pressure as the source of corrective force has considerable advantages, and that, with refinements, the techniques described may prove a useful addition to rigid mechanical methods of correction.

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

Aims. High-grade dysplastic spondylolisthesis is a disabling disorder for which many different operative techniques have been described. The aim of this study is to evaluate Scoliosis Research Society 22-item (SRS-22r) scores, global balance, and regional spino-pelvic alignment from two to 25 years after surgery for high-grade dysplastic spondylolisthesis using an all-posterior partial reduction, transfixation technique. Methods. SRS-22r and full-spine lateral radiographs were collected for the 28 young patients (age 13.4 years (SD 2.6) who underwent surgery for high-grade dysplastic spondylolisthesis in our centre (Scottish National Spinal Deformity Service) between 1995 and 2018. The mean follow-up was nine years (2 to 25), and one patient was lost to follow-up. The standard surgical technique was an all-posterior, partial reduction, and S1 to L5 transfixation screw technique without direct decompression. Parameters for segmental (slip percentage, Dubousset’s lumbosacral angle) and regional alignment (pelvic tilt, sacral slope, L5 incidence, lumbar lordosis, and thoracic kyphosis) and global balance (T1 spino-pelvic inclination) were measured. SRS-22r scores were compared between patients with a balanced and unbalanced pelvis at final follow-up. Results. SRS-22r domain and total scores improved significantly from preoperative to final follow-up, except for the mental health domain that remained the same. Slip percentage improved from 75% (SD 15) to 48% (SD 19) and lumbosacral angle from 70° (SD 11) to 101° (SD 11). Preoperatively, 35% had global imbalance, and at follow-up all were balanced. Preoperatively, 63% had an unbalanced pelvis, and at final follow-up this was 32%. SRS-22r scores were not different in patients with a balanced or unbalanced pelvis. However, postoperative pelvic imbalance as measured by L5 incidence was associated with lower SRS-22r self-image and total scores (p = 0.029). Conclusion. In young patients with HGDS, partial reduction and transfixation improves local lumbosacral alignment, restores pelvic, and global balance and provides satisfactory long-term clinical outcomes. Higher SRS-22r self-image and total scores were observed in the patients that had a balanced pelvis (L5I < 60°) at two to 25 years follow-up. Cite this article: Bone Jt Open 2021;2(3):163–173

Aims. To report the surgical outcome of patients with severe Scheuermann’s kyphosis treated using a consistent technique and perioperative management. Methods. We reviewed 88 consecutive patients with a severe Scheuermann's kyphosis who had undergone posterior spinal fusion with closing wedge osteotomies and hybrid instrumentation. There were 55 males and 33 females with a mean age of 15.9 years (12.0 to 24.7) at the time of surgery. We recorded their demographics, spinopelvic parameters, surgical correction, and perioperative data, and assessed the impact of surgical complications on outcome using the Scoliosis Research Society (SRS)-22 questionnaire. Results. The mean follow-up was 8.4 years (2 to 14.9). There were 85 patients (96.6%) with a thoracic deformity. Posterior spinal fusion with closing-wedge osteotomies and hybrid instrumentation was used in 86 patients; two patients underwent combined anterior and posterior spinal fusion. The mean kyphosis was corrected from 94.5° to 47.5° (p < 0.001). Coronal and sagittal balance returned to normal. The rate of complications was 12.5%: there were no neurological deficits, implant failure, or revision surgery. SRS-22 scores improved from a mean 3.6 (1.3 to 4.1) to 4.6 (4.2 to 5.0) at two years (p < 0.001) with a high rate of patient satisfaction. Non-smokers and patients with lower preoperative SRS-22 scores showed greater improvement in their quality of life. Sagittal pelvic balance did not change after correction of the kyphosis and correlated with lumbar lordosis but not with thoracic or thoracolumbar kyphosis. Conclusion. Posterior spinal fusion using hybrid instrumentation, closing-wedge osteotomies, and iliac bone grafting achieves satisfactory correction of a severe kyphosis resulting in improvements in physical and mental health and a high degree of patient-reported satisfaction. Cite this article: Bone Joint J 2021;103-B(1):148–156

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). Methods. A total of 50 healthy volunteers (normal), 100 patients with single-level lumbar degenerative spondylolisthesis (LDS), and 70 patients with adult to elderly spinal deformity (deformity) were enrolled. Following collection of demographic data and HRQOL measured by the Scoliosis Research Society-22r (SRS-22r), radiological measurement by the biplanar slot-scanning full body stereoradiography (EOS) system was performed simultaneously with force-plate measurements to obtain whole body sagittal alignment parameters. These parameters included the offset between the centre of the acoustic meatus and the gravity line (CAM-GL), saggital vertical axis (SVA), T1 pelvic angle (TPA), McGregor slope, C2-7 lordosis, thoracic kyphosis (TK), lumbar lordosis (LL), pelvic incidence (PI), PI-LL, sacral slope (SS), pelvic tilt (PT), and knee flexion. Whole spine MRI examination was also performed. Cluster analysis of the SRS-22r scores in the pooled data was performed to classify the subjects into three groups according to the HRQOL, and alignment parameters were then compared among the three cluster groups. Results. On the basis of cluster analysis of the SRS-22r subscores, the pooled subjects were divided into three HRQOL groups as follows: almost normal (mean 4.24 (SD 0.32)), mildly disabled (mean 3.32 (SD 0.24)), and severely disabled (mean 2.31 (SD 0.35)). Except for CAM-GL, all the alignment parameters differed significantly among the cluster groups. The threshold values of key alignment parameters for severe disability were TPA > 30°, C2-7 lordosis > 13°, PI-LL > 30°, PT > 28°, and knee flexion > 8°. Lumbar spinal stenosis was found to be associated with the symptom severity. Conclusion. This study provides evidence that the three grades of sagittal compensation in whole body alignment correlate with HRQOL scores. The compensation grades depend on the clinical diagnosis, whole body sagittal alignment, and lumbar spinal stenosis. The threshold values of key alignment parameters may be an indication for treatment. Cite this article: Bone Joint J 2020;102-B(10):1359–1367

Aims. The health-related quality of life (HRQoL) of paediatric patients with orthopaedic conditions and spinal deformity is important, but existing generic tools have their shortcomings. We aim to evaluate the use of Paediatric Quality of Life Inventory (PedsQL) 4.0 generic core scales in the paediatric population with specific comparisons between those with spinal and limb pathologies, and to explore the feasibility of using PedsQL for studying scoliosis patients’ HRQoL. Methods. Paediatric patients attending a speciality outpatient clinic were recruited through consecutive sampling. Two groups of patients were included: idiopathic scoliosis, and paediatric orthopaedic upper and lower limb condition without scoliosis. Patients were asked to complete PedsQL 4.0 generic core scales, Youth version of 5-level EuroQol-5-dimension questionnaire, and Refined Scoliosis Research Society 22-item (SRS-22r) questionnaire. Statistical analyses included scores comparison between scoliosis and limb pathology patients using independent-samples t-test, and correlation tests of PedsQL and SRS-22r. Results. A total of 566 paediatric patients were recruited: 357 (63.0%) having idiopathic scoliosis, and 209 (37.0%) with limb conditions. Patients with limb pathology had lower functioning scale, summary, and total scores of PedsQL than scoliosis patients (p < 0.05 to p < 0.001). No floor or ceiling effects (< 15%) were detected for PedsQL Psychosocial Health Summary and total scores in both groups. PedsQL was sensitive in differentiating patients with/without problems in their daily lives (p < 0.05 to p < 0.01). PedsQL summary and total scores correlated with SRS-22r total score. Conclusion. PedsQL is an effective HRQoL measure for both paediatric orthopaedic groups with minimal ceiling and floor effects, and is capable of detecting worse HRQoL in patients with limb pathology. The multidimensional PedsQL is sensitive in differentiating among those with daily life problems, especially for scoliosis patients. Cite this article: Bone Joint J 2020;102-B(7):890–898