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

Thirteen patients with dystrophic spinal deformities from neurofibromatosis treated by anterior and posterior fusion have been reviewed. The shortest follow-up was five years, the average seven years. Combined fusion produced satisfactory results in patients with a smooth kyphoscoliosis or with scoliosis without kyphosis, but it was unsatisfactory in patients with an angular kyphoscoliosis. Of the five patients with angular kyphoscoliosis, one had a persistent pseudarthrosis after operation and all had progression of the kyphosis despite the treatment. The morbidity rate also was high in this group of patients. Many of the complications were related to soft-tissue manifestations of the disease. It is recommended that very special attention be paid to the dystrophic angular deformity in neurofibromatosis; even anterior and posterior spinal fusion may fail to control its progression

1 . Young Wistar rats fed on a diet containing 0·3 per cent semicarbazide hydrochloride developed the characteristic lesions of osteolathyrism. This consisted of kyphoscoliosis, displacement of epiphyses and dislocations of joints. A pathological study of the skeletal lesions showed widening, disorganisation and tears of the epiphysial plate with the zone of maturing cartilage showing the greatest increase in width. The severe kyphoscoliosis was due to a derangement and displacement at and through the epiphysial plates of the twelfth thoracic or first lumbar vertebra. 2. Some of the compounds that are known to produce osteolathyrism in laboratory animals are beta aminopropionitrile, amino acetonitrile, mercaptoethylamine and semicarbazide. The mode of action of the lathyrogenic compounds was analysed in the light of a number of experiments done in this laboratory. 3. lt is possible that they interfere with the metabolism of the epiphysial plate. There appears to be a decreased polymerisation of the ground substance of the epiphysial cartilage, but the exact metabolic reaction involved is not known. Other chemicals of similar structure, with the same reactive groups, did not show lathyrogenic properties. It is concluded that it is not a particular chemical structure, configuration or specific reactive group that is responsible for the production of osteolathyrism. The chemistry of the lesions still remains to be solved

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

1. Kyphosis and kyphoscoliosis associated with myelomeningocele are discussed. 2. It is suggested that the condition be treated by osteotomy of the spine, with removal of one or more vertebral bodies. 3. Sixteen patients treated by this method of spinal osteotomy are presented. 4. Although almost a third of the patients died, a high operative and post-operative mortality in this condition is considered acceptable

Sublaminar wiring provides strong and effective fixation of the scoliotic or unstable spine, but its long-term effects on the spinal canal remain unknown. An animal model was developed to observe these effects on the growth and development of the immature spine over a two-year period. Laminar overgrowth occurred both longitudinally to produce a kyphoscoliosis and in the transverse plane to cause significant laminar thickening and growth into the spinal canal. However, the cross-sectional area of the spinal canal was not significantly compromised

1. The true deformity of kyphoscoliosis has received little attention. Twenty-one deformities of congenital origin, ten idiopathic, and two secondary to neurofibromatosis, are discussed. The diagnosis is established and usually first suspected by radiography. 2. The deformity was severe and progressive except in three cases; paraplegia occurred in five congenital cases. 3. Early correction and fusion are advocated in the hope of preventing paraplegia and because correction of the old-established deformity is difficult or impossible

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.

A clinical, cadaveric, biomechanical and radiological investigation of the pathogenesis of idiopathic scoliosis indicates that biplanar asymmetry is the essential lesion. Many normal children have coronal plane asymmetry (an inconsequential lateral curvature of the spine), and certainly all have vertebral body asymmetry in the transverse plane, but when median plane asymmetry (flattening or more usually reversal of the normal thoracic kyphosis at the apex of the scoliosis) is superimposed during growth, a progressive idiopathic scoliosis occurs. Idiopathic kyphoscoliosis cannot and does not exist, from the mildest cases in the community to the most severe cases in pathology museums. Median plane asymmetry is crucial for progression and the lateral profile of the spine must be carefully scrutinised. Increased anterior vertebral height at the apex of the curve with posterior end-plate irregularity characterises the median plane asymmetry and suggests that idiopathic scoliosis is the reverse of Scheuermann's disease

The development of spinal deformity in children with underlying neurodisability can affect their ability to function and impact on their quality of life, as well as compromise provision of nursing care. Patients with neuromuscular spinal deformity are among the most challenging due to the number and complexity of medical comorbidities that increase the risk for severe intraoperative or postoperative complications. A multidisciplinary approach is mandatory at every stage to ensure that all nonoperative measures have been applied, and that the treatment goals have been clearly defined and agreed with the family. This will involve input from multiple specialities, including allied healthcare professionals, such as physiotherapists and wheelchair services. Surgery should be considered when there is significant impact on the patients’ quality of life, which is usually due to poor sitting balance, back or costo-pelvic pain, respiratory complications, or problems with self-care and feeding. Meticulous preoperative assessment is required, along with careful consideration of the nature of the deformity and the problems that it is causing. Surgery can achieve good curve correction and results in high levels of satisfaction from the patients and their caregivers. Modern modular posterior instrumentation systems allow an effective deformity correction. However, the risks of surgery remain high, and involvement of the family at all stages of decision-making is required in order to balance the risks and anticipated gains of the procedure, and to select those patients who can mostly benefit from spinal 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.

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 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.

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.

The April 2012 Children’s orthopaedics Roundup³⁶⁰ looks at osteonecrosis of the femoral head and surgery for dysplasia, femoral head blood flow during surgery, femoroacetabular impingement and sport in adolescence, the Drehmann sign, a predictive algorithm for septic arthritis, ACL reconstruction and arthrofibrosis in children, spinal cord monitoring for those less than four years old, arthroereisis for the flexible flat foot, fixing the displaced lateral humeral fracture, and mobile phones and inclinometer applications

A comprehensive study of osteology remains a cornerstone of current orthopaedic and traumatological education. Osteology was already established as an important part of surgical education by the 16^th century. In order to teach anatomy and osteology, the corpses of executed criminals were dissected by the praelector anatomiae of the Amsterdam Guild of Surgeons. Magnificent anatomical atlases preserve the knowledge obtained from these dissections. We present an overview of the most authoritative works of Vesalius, Bidloo, Cheselden, and Albinus authored in the 16^th, 17^th and 18^th centuries. At that time a knowledge of osteology was necessary to pass the ‘master-exam’ in order to become a surgeon, and anatomical teaching was traditionally based on the practice of dissection. In the modern era, anatomical dissection and illustrations are largely being replaced by three-dimensional imaging and computer simulations, with an unfortunate trend in current curricula away from the established teaching technique of dissection. Education through the practice of dissection, particularly for future surgeons, remains integral to the development of tissue handling techniques, understanding of anatomical variation, and furthering of spatial awareness skills. With this review, we seek to remind contemporary surgeons of the lessons we can learn from our predecessors who valued education through anatomical dissection.

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.

The Morquio syndrome is a rare disorder which presents with a number of musculoskeletal problems. The literature describing total knee replacement in these patients is sparse. We describe the management of a patient with bilateral instability and pain in the knees using bilateral constrained knee replacements, and followed up for five years with pre- and postoperative knee scores. We highlight the difficulties encountered and discuss the end results.

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.

In order to treat painful subluxation or dislocation secondary to cerebral palsy, 11 patients (12 hips) underwent combined femoral and Chiari pelvic osteotomies with additional soft-tissue releases at a mean age of 14.1 years (9.1 to 17.8). Relief of pain, improvement in movement of the hip, and in sitting posture, and ease of perineal care were recorded in all, and were maintained at a mean follow-up of 13.1 years (8 to 17.5). The improvement in general mobility was marginal, but those who were able to walk benefited the most.

The radiological measurements made before operation were modified afterwards to use the lateral margin of the neoacetabulum produced by the pelvic osteotomy. The radiological migration index improved from a mean of 80.6% (61% to 100%) to 13.7% (0% to 33%) (p < 0.0001). The mean changes in centre edge angle and Sharp’s angle were 72° (56° to 87°; p < 0.0001) and 12.3° (9° to 15.6°; p < 0.0001), respectively. Radiological evidence of progressive arthritic change was seen in one hip, in which only a partial reduction had been achieved, and there was early narrowing of the joint space in another. Painless heterotopic ossification was observed in one patient with athetoid quadriplegia. In seven hips the lateral Kawamura approach, elevating the greater trochanter, provided exposure for both osteotomies and allowed the construction of a dome-shaped iliac osteotomy, while protecting the sciatic nerve.