Aims. Clinical and radiological data were reviewed for all patients with mucopolysaccharidoses (MPS) with thoracolumbar kyphosis managed non-operatively or operatively in our institution. Methods. In all 16 patients were included (eight female: eight male; 50% male), of whom nine had Hurler, five Morquio and two Hunter syndrome. Six patients were treated non-operatively (mean age at presentation of 6.3 years; 0.4 to 12.9); mean kyphotic progression +1.5. o. /year; mean follow-up of 3.1 years (1 to 5.1) and ten patients operatively (mean age at presentation of 4.7 years; 0.9 to 14.4); mean kyphotic progression 10.8. o. /year; mean follow-up of 8.2 years; 4.8 to 11.8) by circumferential arthrodesis with posterior instrumentation in patients with flexible deformities (n = 6). Results. In the surgical group (mean age at surgery of 6.6 years; 2.4 to 16.8); mean post-operative follow-up of 6.3 years (3.5 to 10.3), mean pre-operative thoracolumbar kyphosis of 74.3. o. (42. o. to 110. o. ) was corrected to mean of 28.6. o. (0. o. to 65. o. ) post-operatively, relating to a mean deformity correction of 66.9% (31% to 100%). Surgical complications included a deep wound infection treated by early debridement, apical non-union treated by posterior re-grafting, and stable adjacent segment spondylolisthesis managed non-operatively. Thoracolumbar kyphosis > +38. o. at initial presentation was identified as predicting progressively severe deformity with 90% sensitivity and 83% specificity. Discussion. This study demonstrates that severe thoracolumbar kyphosis in patients with MPS can be effectively treated by circumferential arthrodesis. Severity of kyphosis at initial presentation may predict progression of thoracolumbar deformity. Patients with MPS may be particularly susceptible to post-operative complications due to the underlying connective tissue disorder and inherent immunological compromise. Take home message: Clinical and radiological data were reviewed for all patients with mucopolysaccharidoses with thoracolumbar kyphosis managed non-operatively or operatively in our institution. Cite this article: Bone Joint J 2016;98-B:229–37

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. 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. Whether a combined anteroposterior fusion or a posterior-only fusion is more effective in the management of patients with Scheuermann’s kyphosis remains controversial. The aim of this study was to compare the radiological and clinical outcomes of these surgical approaches, and to evaluate the postoperative complications with the hypothesis that proximal junctional kyphosis would be more common in one-stage posterior-only fusion. Methods. A retrospective review of patients treated surgically for Scheuermann’s kyphosis between 2006 and 2014 was performed. A total of 62 patients were identified, with 31 in each group. Parameters were compared to evaluate postoperative outcomes using chi-squared tests, independent-samples t-tests, and z-tests of proportions analyses where applicable. Results. There were six postoperative infections in the two-stage anteroposterior group compared with three in the one-stage posterior-only group. A total of four patients in the anteroposterior group required revision surgery, compared with six in the posterior-only group. There was a significantly higher incidence of junctional failure associated with the one-stage posterior-only approach (12.9% vs 0%, p = 0.036). Proximal junction kyphosis (anteroposterior fusion (74.2%) vs posterior-only fusion (77.4%); p = 0.382) and distal junctional kyphosis (anteroposterior fusion (25.8%) vs posterior-only fusion (19.3%), p = 0.271) are common postoperative complications following both surgical approaches. Conclusion. A two-stage anteroposterior fusion was associated with a significantly greater correction of the kyphosis compared with a one-stage posterior-only fusion, with a reduced incidence of junctional failure (0 vs 3). There was a notably greater incidence of infection with two-stage anteroposterior fusion; however, all were medically managed. More patients in the posterior-only group required revision surgery. Cite this article: Bone Joint J 2020;102-B(10):1368–1374

We report five children who presented at the mean age of 1.5 years (1.1 to 1.9) with a progressive thoracolumbar kyphosis associated with segmental instability and subluxation of the spine at the level above an anteriorly-wedged hypoplastic vertebra at L1 or L2. The spinal deformity appeared to be developmental and not congenital in origin. The anterior wedging of the vertebra may have been secondary to localised segmental instability and subsequent kyphotic deformity. We suggest the term ‘infantile developmental thoracolumbar kyphosis with segmental subluxation of the spine’ to differentiate this type of deformity from congenital displacement of the spine in which the congenital vertebral anomaly does not resolve. Infantile developmental kyphosis with segmental subluxation of the spine, if progressive, may carry the risk of neurological compromise. In all of our patients the kyphotic deformity progressed over a period of three months and all were treated by localised posterior spinal fusion. At a mean follow-up of 6.6 years (5.0 to 9.0), gradual correction of the kyphosis was seen on serial radiographs as well as reconstitution of the hypoplastic wedged vertebra to normality. Exploration of the arthrodesis was necessary at nine months in one patient who developed a pseudarthrosis

This review of the literature presents the current understanding of Scheuermann’s kyphosis and investigates the controversies concerning conservative and surgical treatment. There is considerable debate regarding the pathogenesis, natural history and treatment of this condition. A benign prognosis with settling of symptoms and stabilisation of the deformity at skeletal maturity is expected in most patients. Observation and programmes of exercise are appropriate for mild, flexible, non-progressive deformities. Bracing is indicated for a moderate deformity which spans several levels and retains flexibility in motivated patients who have significant remaining spinal growth. The loss of some correction after the completion of bracing with recurrent anterior vertebral wedging has been reported in approximately one-third of patients. Surgical correction with instrumented spinal fusion is indicated for a severe kyphosis which carries a risk of progression beyond the end of growth causing cosmetic deformity, back pain and neurological complications. There is no consensus on the effectiveness of different techniques and types of instrumentation. Techniques include posterior-only and combined anteroposterior spinal fusion with or without posterior osteotomies across the apex of the deformity. Current instrumented techniques include hybrid and all-pedicle screw constructs

The spinal manifestations of neurofibromatosis include cervicothoracic kyphosis, in which scalloping of the vertebral body and erosion of the pedicles may render conventional techniques of fixation impossible. We describe a case of cervicothoracic kyphosis managed operatively with a vascularised fibular graft anteriorly across the apex of the kyphus, followed by a long posterior construct using translaminar screws, which allow segmental fixation in vertebral bodies where placement of the pedicle screws was impracticable

To determine the effect of cordotomy on the function of the bladder during surgical correction of congenital kyphosis in myelomeningocele, we reviewed 13 patients who had this procedure between 1981 and 1996. The mean age of the patients at operation was 8.9 years (3.7 to 16) and the mean follow-up was 4.8 years (1.3 to 10.8). Bladder function before and after operation was assessed clinically and quantitatively by urodynamics. The mean preoperative kyphosis was 117° (52 to 175) and decreased to 49° (1 to 89) immediately after surgery. At the latest follow-up, a mean correction of 52% had been achieved. Only one patient showed deterioration in bladder function after operation. Eight out of the nine patients who had urodynamic assessment had improvement in bladder capacity and compliance, and five showed an increase in urethral pressure. One patient developed a spastic bladder and required subsequent surgical intervention. Cordotomy, at or below the level of the kyphosis, allows excellent correction of the structural deformity

Cardiac disease in patients with ankylosing spondylitis (AS) has previously been studied but not in patients with a kyphosis or in those who have undergone an operation to correct it. . The aim of this study was to measure the post-operative changes in cardiac function of patients with an AS kyphosis after pedicle subtraction osteotomy (PSO). The original cohort consisted of 39 patients (33 men, six women). Of these, four patients (two men, two women) were lost to follow-up leaving 35 patients (31 men, four women) to study. The mean age of the remaining patients was 37.4 years (22.3 to 47.8) and their mean duration of AS was 17.0 years (4.6 to 26.4). Echocardiographic measurements, resting heart rate (RHR), physical function score (PFS), and full-length standing spinal radiographs were obtained before surgery and at the two-year follow-up. The mean pre-operative RHR was 80.2 bpm (60.6 to 112.3) which dropped to a mean of 73.7 bpm (60.7 to 90.6) at the two-year follow-up (p = 0.0000). Of 15 patients with normal ventricular function pre-operatively, two developed mild left ventricular diastolic dysfunction (LVDD) at the two-year follow-up. Of 20 patients with mild LVDD pre-operatively only five had this post-operatively. Overall, 15 patients had normal LV diastolic function before their operation and 28 patients had normal LV function at the two-year follow-up. The clinical improvement was 15 out of 20 (75.0%): cardiac function in patients with AS whose kyphosis was treated by PSO was significantly improved. Cite this article: Bone Joint J 2015;97-B:1405–10

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

Between 1969 and 1989, we performed posterior segmental instrumentation on 38 patients with thoracic Scheuermann's kyphosis. We used a dynamic system without sublaminar fixation, and a kyphosis of 50 degrees was the main indication for surgery. The mean initial angle was 68 degrees (50 to 100) and the mean final kyphosis was 43 degrees at five-year follow-up, with a mean final loss after surgery of 3.7 degrees. Reconstruction of the vertebral bodies, vertebral wedging and the anterior-body height ratio were observed even in skeletally mature patients. There were no medical complications. There were three cases of loss of correction by more than 10 degrees and one of rod fracture with pseudarthrosis. The role of non-operative treatment is evaluated and early surgical treatment is advocated

We evaluated the efficacy of anterior fusion alone compared with combined anterior and posterior fusion for the treatment of degenerative cervical kyphosis. Anterior fusion alone was undertaken in 15 patients (group A) and combined anterior and posterior fusion was carried out in a further 15 (group B). The degree and maintenance of the angle of correction, the incidence of graft subsidence, degeneration at adjacent levels and the rate of fusion were assessed radiologically and clinically and the rate of complications recorded. The mean angle of correction in group B was significantly higher than in group A (p = 0.0009). The mean visual analogue scale and the neck disability index in group B was better than in group A (p = 0.043, 0.0006). The mean operation time and the blood loss in B were greater than in group A (p < 0.0001, 0.037). Pseudarthrosis, subsidence of the cage, and problems related to the hardware were more prevalent in group A than in group B (p = 0.034, 0.025, 0.013). Although the combined procedure resulted in a longer operating time and greater blood loss than with anterior fusion alone, our results suggest that for the treatment of degenerative cervical kyphosis the combined approach leads to better maintenance of sagittal alignment, a higher rate of fusion, a lower incidence of complications and a better clinical outcome

In 21 children with myelomeningocele who underwent kyphectomy for congenital kyphosis of the lumbar spine, aortography revealed no case in which the aorta followed the spinal curvature. Many anomalies of the intercostal and segmental arteries were demonstrated which were only in part associated with deformities of the respective vertebral bodies. The kidneys, which were frequently malformed, often lay within the kyphosis and were therefore at risk of operative damage. We conclude that the aorta is not at risk and that aortography is not usually necessary before kyphectomy, except in patients who have undergone prior abdominal surgery. Non-invasive methods (ultrasound, CT or MRI) should be used to detect malpositions and malformations of the kidneys

Aims. To develop and internally validate a preoperative clinical prediction model for acute adjacent vertebral fracture (AVF) after vertebral augmentation to support preoperative decision-making, named the after vertebral augmentation (AVA) score. Methods. In this prognostic study, a multicentre, retrospective single-level vertebral augmentation cohort of 377 patients from six Japanese hospitals was used to derive an AVF prediction model. Backward stepwise selection (p < 0.05) was used to select preoperative clinical and imaging predictors for acute AVF after vertebral augmentation for up to one month, from 14 predictors. We assigned a score to each selected variable based on the regression coefficient and developed the AVA scoring system. We evaluated sensitivity and specificity for each cut-off, area under the curve (AUC), and calibration as diagnostic performance. Internal validation was conducted using bootstrapping to correct the optimism. Results. Of the 377 patients used for model derivation, 58 (15%) had an acute AVF postoperatively. The following preoperative measures on multivariable analysis were summarized in the five-point AVA score: intravertebral instability (≥ 5 mm), focal kyphosis (≥ 10°), duration of symptoms (≥ 30 days), intravertebral cleft, and previous history of vertebral fracture. Internal validation showed a mean optimism of 0.019 with a corrected AUC of 0.77. A cut-off of ≤ one point was chosen to classify a low risk of AVF, for which only four of 137 patients (3%) had AVF with 92.5% sensitivity and 45.6% specificity. A cut-off of ≥ four points was chosen to classify a high risk of AVF, for which 22 of 38 (58%) had AVF with 41.5% sensitivity and 94.5% specificity. Conclusion. In this study, the AVA score was found to be a simple preoperative method for the identification of patients at low and high risk of postoperative acute AVF. This model could be applied to individual patients and could aid in the decision-making before vertebral augmentation. Cite this article: Bone Joint J 2022;104-B(1):97–102

The progression of post-tubercular kyphosis in 61 children who received ambulatory chemotherapy was studied prospectively. The angles of deformity and kyphosis were measured for each patient at diagnosis, 3, 6, 9, 12 and 18 months later and every year thereafter for 15 years. During the course of the disease signs of instability appeared on the radiographs of some of the children. These were dislocation of the facets, posterior retropulsion of the diseased fragments, lateral translation of the vertebrae in the anteroposterior view and toppling of the superior vertebra. Each sign was allocated one point to create a spinal instability score. The influence on the progression of the deformity of the level of the lesion, the vertebral body loss, the number of segments involved, the angle of deformity before treatment and the spinal instability score was analysed. The mean angle of deformity at the start of treatment was 35°. This increased to 41° at 15 years. Progression occurred during the active phase of the disease and again after cure when variations in progression were observed. Type-I progression showed an increase in deformity until growth had ceased. This could occur either continuously (type Ia) or after a lag period of three to five years (type Ib). Type-II progression showed decrease in deformity with growth. This could occur immediately after the active phase (type IIa) or after a lag period of three to five years (type IIb). Type-III progression showed minimal change during either the active or healed phases and was seen only in those with limited disease. Multiple regression analysis showed that a spinal instability score of more than 2 was a reliable predictor of patients with an increase of more than 30° in deformity and a final deformity of over 60°. Since signs of radiological instability appear early in the disease, they can be reliably used to identify children whose spine is at risk for late progressive collapse. Surgery is advised in these cases

Aims. This study addressed two questions: first, does surgical correction of an idiopathic scoliosis increase the volume of the rib cage, and second, is it possible to evaluate the change in lung function after corrective surgery for adolescent idiopathic scoliosis (AIS) using biplanar radiographs of the ribcage with 3D reconstruction?. Methods. A total of 45 patients with a thoracic AIS which needed surgical correction and fusion were included in a prospective study. All patients underwent pulmonary function testing (PFT) and low-dose biplanar radiographs both preoperatively and one year after surgery. The following measurements were recorded: forced vital capacity (FVC), slow vital capacity (SVC), and total lung capacity (TLC). Rib cage volume (RCV), maximum rib hump, main thoracic curve Cobb angle (MCCA), medial-lateral and anteroposterior diameter, and T4-T12 kyphosis were calculated from 3D reconstructions of the biplanar radiographs. Results. All spinal and thoracic measurements improved significantly after surgery (p < 0.001). RCV increased from 4.9 l (SD 1) preoperatively to 5.3 l (SD 0.9) (p < 0.001) while TLC increased from 4.1 l (SD 0.9) preoperatively to 4.3 l (SD 0.8) (p < 0.001). RCV was correlated with all functional indexes before and after correction of the deformity. Improvement in RCV was weakly correlated with correction of the mean thoracic Cobb angle (p = 0.006). The difference in TLC was significantly correlated with changes in RCV (p = 0.041). It was possible to predict postoperative TLC from the postoperative RCV. Conclusion. 3D rib cage assessment from biplanar radiographs could be a minimally invasive method of estimating pulmonary function before and after spinal fusion in patients with an AIS. The 3D RCV reflects virtual chest capacity and hence pulmonary function in this group of patients. Cite this article: Bone Joint J 2022;104-B(1):112–119

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

The results of surgery in 59 patients with Scheuermann's kyphosis are reported at an average follow-up of 56 months. These show that in skeletally immature patients, in whom the iliac apophysis has not yet fused to the body of the ilium, posterior fusion alone is adequate and is followed by little loss of correction. For skeletally mature patients combined anterior and posterior surgery is recommended. In all cases a period of pre-operative treatment is important. It is stressed that the indications for surgery are limited

An operative technique for correction of the severe kyphosis that may develop in cases of myelomeningocele is described. It includes excision of the apical vertebrae, correction of the full length of the kyphotic segment and of rigid compensatory curves, and extensive fusion aided by internal fixation with longitudinal threaded wires. The technique evolved during the management of eleven children. Of these, in the first two the internal fixation employed was inadequate; two others died; and in the remainder good correction was maintained up to the time of review one to six years later

A radiological study of 50 patients with thoracic Scheuermann's disease revealed two types of lateral spinal curvature. A total of 43 lateral curves was present in 35 of the patients. Thirteen were apical at the same level as the Scheuermann's kyphosis and were due to vertebral-body wedging in the coronal plane; these curves had a mean Cobb angle of 15 degrees, occurred with equal prevalence in boys and girls and were directed equally to right and left. Thirty curves occurred in regions of compensatory lordosis (mean 5.6 degrees) situated above or, more commonly, below the Scheuermann's kyphosis. These scolioses had a mean Cobb angle of 16 degrees, were more often convex to the right than to the left and were significantly more prevalent in girls than in boys. The presence of these kyphoses and scolioses in the same spine, separated by only a few vertebrae, emphasises the importance of the sagittal plane in idiopathic spinal deformities and strongly suggests that idiopathic scoliosis and Scheuermann's disease share a common pathological process