We reviewed 31 consecutive patients with Friedreich’s ataxia and scoliosis. There were 24 males and seven females with a mean age at presentation of 15.5 years (8.6 to 30.8) and a mean curve of 51° (13° to 140°). A total of 12 patients had thoracic curvatures, 11 had thoracolumbar and eight had double thoracic/lumbar. Two patients had long thoracolumbar collapsing scoliosis with pelvic obliquity and four had hyperkyphosis. Left-sided thoracic curves in nine patients (45%) and increased thoracic kyphosis differentiated these deformities from adolescent idiopathic scoliosis. There were 17 patients who underwent a posterior instrumented spinal fusion at mean age of 13.35 years, which achieved and maintained good correction of the deformity. Post-operative complications included one death due to cardiorespiratory failure, one revision to address nonunion and four patients with proximal junctional kyphosis who did not need extension of the fusion. There were no neurological complications and no wound infections. The rate of progression of the scoliosis in children kept under simple observation and those treated with bracing was less for lumbar curves during bracing and similar for thoracic curves. The scoliosis progressed in seven of nine children initially treated with a brace who later required surgery. Two patients presented after skeletal maturity with balanced curves not requiring correction. Three patients with severe deformities who would benefit from corrective surgery had significant cardiac co-morbidities.

Aims. Only a few studies have investigated the long-term health-related quality of life (HRQoL) in patients with an idiopathic scoliosis. The aim of this study was to investigate the overall HRQoL and employment status of patients with an idiopathic scoliosis 40 years after diagnosis, to compare it with that of the normal population, and to identify possible predictors for a better long-term HRQoL. Methods. We reviewed the full medical records and radiological reports of patients referred to our hospital with a scoliosis of childhood between April 1972 and April 1982. Of 129 eligible patients with a juvenile or adolescent idiopathic scoliosis, 91 took part in the study (71%). They were evaluated with full-spine radiographs and HRQoL questionnaires and compared with normative data. We compared the HRQoL between observation (n = 27), bracing (n = 46), and surgical treatment (n = 18), and between thoracic and thoracolumbar/lumbar (TL/L) curves. Results. The mean time to follow-up was 40.8 years (SD 2.6) and the mean age of patients was 54.0 years (SD 2.7). Of the 91 patients, 86 were female (95%) and 51 had a main thoracic curve (53%). We found a significantly lower HRQoL measured on all the Scoliosis Research Society 22r instrument (SRS-22r) subdomains (p < 0.001) with the exception of mental health, than in an age-matched normal population. Incapacity to work was more prevalent in scoliosis patients (21%) than in the normal population (11%). The median SRS-22r subscore was 4.0 (interquartile range (IQR) 3.3 to 4.4) for TL/L curves and 4.1 (IQR 3.8 to 4.4) for thoracic curves (p = 0.300). We found a significantly lower self-image score for braced (median 3.6 (IQR 3.0 to 4.0)) and surgically treated patients (median 3.6 (IQR 3.2 to 4.3)) than for those treated by observation (median 4.0 (IQR 4.1 to 4.8); p = 0.010), but no statistically significant differences were found for the remaining subdomains. Conclusion. In this long-term follow-up study, we found a significantly decreased HRQoL and capacity to work in patients with an idiopathic scoliosis 40 years after diagnosis. Cite this article: Bone Joint J 2023;105-B(2):166–171

Clinical, radiological, and Scoliosis Research Society-22 questionnaire data were reviewed pre-operatively and two years post-operatively for patients with thoracolumbar/lumbar adolescent idiopathic scoliosis treated by posterior spinal fusion using a unilateral convex segmental pedicle screw technique. A total of 72 patients were included (67 female, 5 male; mean age at surgery 16.7 years (13 to 23)) and divided into groups: group 1 included 53 patients who underwent fusion between the vertebrae at the limit of the curve (proximal and distal end vertebrae); group 2 included 19 patients who underwent extension of the fusion distally beyond the caudal end vertebra. A mean scoliosis correction of 80% (45% to 100%) was achieved. The mean post-operative lowest instrumented vertebra angle, apical vertebra translation and trunk shift were less than in previous studies. A total of five pre-operative radiological parameters differed significantly between the groups and correlated with the extension of the fusion distally: the size of the thoracolumbar/lumbar curve, the lowest instrumented vertebra angle, apical vertebra translation, the Cobb angle on lumbar convex bending and the size of the compensatory thoracic curve. Regression analysis allowed an equation incorporating these parameters to be developed which had a positive predictive value of 81% in determining whether the lowest instrumented vertebra should be at the caudal end vertebra or one or two levels more distal. There were no differences in the Scoliosis Research Society-22 outcome scores between the two groups (p = 0.17). In conclusion, thoracolumbar/lumbar curves in patients with adolescent idiopathic scoliosis may be effectively treated by posterior spinal fusion using a unilateral segmental pedicle screw technique. Five radiological parameters correlate with the need for distal extension of the fusion, and an equation incorporating these parameters reliably informs selection of the lowest instrumented vertebra. Cite this article: Bone Joint J 2014;96-B:1082–9

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

Aims. We present the results of correcting a double or triple curve adolescent idiopathic scoliosis using a convex segmental pedicle screw technique. Patients and Methods. We reviewed 191 patients with a mean age at surgery of 15 years (11 to 23.3). Pedicle screws were placed at the convexity of each curve. Concave screws were inserted at one or two cephalad levels and two caudal levels. The mean operating time was 183 minutes (132 to 276) and the mean blood loss 0.22% of the total blood volume (0.08% to 0.4%). Multimodal monitoring remained stable throughout the operation. The mean hospital stay was 6.8 days (5 to 15). Results. The mean post-operative follow-up was 5.8 years (2.5 to 9.5). There were no neurological complications, deep wound infection, obvious nonunion or need for revision surgery. Upper thoracic scoliosis was corrected by a mean 68.2% (38% to 48%, p < 0.001). Main thoracic scoliosis was corrected by a mean 71% (43.5% to 8.9%, p < 0.001). Lumbar scoliosis was corrected by a mean 72.3% (41% to 90%, p < 0.001). No patient lost more than 3° of correction at follow-up. The thoracic kyphosis improved by 13.1° (-21° to 49°, p < 0.001); the lumbar lordosis remained unchanged (p = 0.58). Coronal imbalance was corrected by a mean 98% (0% to 100%, p < 0.001). Sagittal imbalance was corrected by a mean 96% (20% to 100%, p < 0.001). The Scoliosis Research Society Outcomes Questionnaire score improved from a mean 3.6 to 4.6 (2.4 to 4, p < 0.001); patient satisfaction was a mean 4.9 (4.8 to 5). . Conclusions. This technique carries low neurological and vascular risks because the screws are placed in the pedicles of the convex side of the curve, away from the spinal cord, cauda equina and the aorta. A low implant density (pedicle screw density 1.2, when a density of 2 represents placement of pedicle screws bilaterally at every instrumented segment) achieved satisfactory correction of the scoliosis, an improved thoracic kyphosis and normal global sagittal balance. Both patient satisfaction and functional outcomes were excellent. Cite this article: Bone Joint J 2017;99-B:1080–7

Scoliosis Clinic and the Department of Human Genetics, Hadassah University Hospital, Jerusalem Scoliosis appeared in fifteen members of a family in three generations. The eight members examined showed an idiopathic type of scoliosis. Father-to-son transmission occurred more than once. Although at first appearance transmission of the scoliosis is as an autosomal dominant trait, the concept of polygenic inheritance, as has been shown in previous population studies, could also explain the inheritance in this family

Aims

The prevalence of scoliosis is not known in patients with idiopathic short stature, and the impact of treatment with recombinant human growth hormone on those with scoliosis remains controversial. We investigated the prevalence of scoliosis radiologically in children with idiopathic short stature, and the impact of treatment with growth hormone in a cross-sectional and retrospective cohort study.

Aims

This study aimed to evaluate rasterstereography of the spine as a diagnostic test for adolescent idiopathic soliosis (AIS), and to compare its results with those obtained using a scoliometer.

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

Aims

To determine the value of scoliosis surgery, it is necessary to evaluate outcomes in domains that matter to patients. Since randomized trials on adolescent idiopathic scoliosis (AIS) are scarce, prospective cohort studies with comparable outcome measures are important. To enhance comparison, a core set of patient-related outcome measures is available. The aim of this study was to evaluate the outcomes of AIS fusion surgery at two-year follow-up using the core outcomes set.

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.

The association between spondylolisthesis and scoliosis was studied in 84 patients who presented during a 30-year period with symptomatic spondylolisthesis. The incidence of scoliosis was 42 per cent, the majority of cases being lumbar or thoracolumbar curves of less than 15 degrees. The incidence was highest in the group of patients with spondylolisthesis at L4--5 where all except one had scoliosis. Scoliosis was present in 47 per cent of patients with dysplastic spondylolisthesis at the lumbosacral junction; in this group, the incidence of scoliosis was greater where the displacement exceeded 25 per cent. The lowest incidence (25 per cent) was found in the group with isthmic spondylolisthesis at the lumbosacral junction. There appeared to be no relationship between excessive lumbar lordosis or tightness of the hamstrings and scoliosis

Aims

Historically, patients undergoing surgery for adolescent idiopathic scoliosis (AIS) have been nursed postoperatively in a critical care (CC) setting because of the challenges posed by prone positioning, extensive exposures, prolonged operating times, significant blood loss, major intraoperative fluid shifts, cardiopulmonary complications, and difficulty in postoperative pain management. The primary aim of this paper was to determine whether a scoring system, which uses Cobb angle, forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and number of levels to be fused, is a valid method of predicting the need for postoperative critical care in AIS patients who are to undergo scoliosis correction with posterior spinal fusion (PSF).

Aims

The aim of this study was to determine whether there is an increased prevalence of scoliosis in patients who have suffered from a haematopoietic malignancy in childhood.

Experimental evidence has accumulated in recent years to suggest that scoliosis can be caused by asymmetrical spinal muscle weakness due to sensorineural loss, though this suggestion has not achieved universal acceptance. The evidence is supported by histopathological observations on cases of clinical idiopathic scoliosis. A study is presented in which cynomolgus monkeys had one, two or three dorsal spinal nerve roots cut. Scoliosis developed, convex to the damaged side; its severity was dependent on the number of nerve roots cut. Section of the first lumbar dorsal spinal nerve root had a marked tendency to cause scoliosis. The study supports the view that scoliosis may be caused by asymmetrical paraspinal muscle weakness acting through loss of proprioception

Aims

To report the outcome of spinal deformity correction through anterior spinal fusion in wheelchair-bound patients with myelomeningocele.

With the identification of literature shortfalls on the techniques employed in intraoperative navigated (ION) spinal surgery, we outline a number of measures which have been synthesised into a coherent operative technique. These include positioning, dissection, management of the reference frame, the grip, the angle of attack, the drill, the template, the pedicle screw, the wire, and navigated intrathecal analgesia. Optimizing techniques to improve accuracy allow an overall reduction of the repetition of the surgical steps with its associated productivity benefits including time, cost, radiation, and safety.

Cite this article: Bone Joint J 2020;102-B(3):371–375.

Aims

Magnetically controlled growing rods (MCGRs) allow non-invasive correction of the spinal deformity in the treatment of early-onset scoliosis. Conventional growing rod systems (CGRS) need repeated surgical distractions: these are associated with the effect of the ‘law of diminishing returns’.

The primary aim of this study was to quantify this effect in MCGRs over sequential distractions.

Adolescent idiopathic scoliosis affects about 3% of children. Non-operative measures are aimed at altering the natural history to maintain the size of the curve below 40° at skeletal maturity. The application of braces to treat spinal deformity pre-dates the era of evidence-based medicine, and there is a paucity of irrefutable prospective evidence in the literature to support their use and their effectiveness has been questioned.

This review considers this evidence. The weight of the evidence is in favour of bracing over observation. The most recent literature has moved away from addressing this question, and instead focuses on developments in the design of braces and ways to improve compliance.

Cite this article: Bone Joint J 2013;95-B:1308–16.

The purpose of this study was to evaluate the long-term outcome of adults with spina bifida cystica (SBC) who had been treated either operatively or non-operatively for scoliosis during childhood.

We reviewed 45 patients with a SBC scoliosis (Cobb angle ≥ 50º) who had been treated at one of two children’s hospitals between 1991 and 2007. Of these, 34 (75.6%) had been treated operatively and 11 (24.4%) non-operatively. After a mean follow-up of 14.1 years (standard deviation (sd) 4.3) clinical, radiological and health-related quality of life (HRQOL) outcomes were evaluated using the Spina Bifida Spine Questionnaire (SBSQ) and the 36-Item Short Form Health Survey (SF-36).

Although patients in the two groups were demographically similar, those who had undergone surgery had a larger mean Cobb angle (88.0º (sd 20.5; 50.0 to 122.0); versus 65.7º (sd 22.0; 51.0 to 115.0); p < 0.01) and a larger mean clavicle–rib intersection difference (12.3 mm; (sd 8.5; 1 to 37); versus 4.1 mm, (sd 5.9; 0 to 16); p = 0.01) than those treated non-operatively. Both groups were statistically similar at follow-up with respect to walking capacity, neurological motor level, sitting balance and health-related quality of life (HRQOL) outcomes.

Spinal fusion in SBC scoliosis corrects coronal deformity and stops progression of the curve but has no clear effect on HRQOL.

Cite this article: Bone Joint J 2014; 96-B:1244–51

Aims

We report the use of the distal radius and ulna (DRU) classification for the prediction of peak growth (PG) and growth cessation (GC) in 777 patients with idiopathic scoliosis. We compare this classification with other commonly used parameters of maturity.

Structural scoliosis occurs more commonly in patients with juvenile chronic arthritis than in the normal population. We have reviewed 32 patients with both juvenile arthritis and a scoliosis and suggest that structural curves may arise from postural curves associated with asymmetrical involvement of lower limb joints.

1. The equipment used in the correction of scoliosis by the anterior route is described.

2. The important points in surgical technique are emphasised.

3. The results of operation on fifty-one patients are analysed.

4. Attention is drawn to the high rate of fusion, the stability of correction, and the brevity of the period of convalescence.

1. In this analysis of forty-three patients with syringomyelia, twenty-seven (63 per cent) had scoliosis. This association is probably due to the early involvement of the ventro-medial and dorso-medial nuclei of the spinal cord by expanding lesions.

2. The literature makes no reference to the treatment of scoliosis associated with syringomyelia. Two cases are presented of attempts to correct this scoliosis–one because of increasing deformity, the other for increasing backache.

3. Due to the presence of cystic lesions characteristic of syringomyelia, corrective operative treatment of scoliosis may present an added risk.

4. Because of the high incidence of scoliosis in patients with syringomyelia, any patient with scoliosis should be examined for evidence of neurological deficit.

1. Analysis of eighty-one patients with neurofibromatosis showed that sixty-two (76 per cent) had café-au-lait markings; 12 per cent had significant spinal deformity.

2. Thirty-three examples of spinal deformity in neurofibromatosis showed a wide variety of patterns and severity of the adult curve. There was no evidence that there was any recognisable pattern of scoliosis in neurofibromatosis. No evidence was discovered to suggest that any acquired local abnormality of bone contributed to the deformity.

3. Some of the severe deformities showed a pattern similar to that seen in the congenital sco1ioses, and this might be the link between the neurofibromatosis and the spinal deformity.

Four cases are reported in which infantile idiopathic structural scoliosis gradually decreased during the period of active growth.

The etiological factors concerned in paralytic scoliosis are complex. Four main types of paralytic scoliosis can be recognised.

1. The general C-curve due to the body's anatomical attempt to shift its centre of gravity towards the weaker side. Vertebral rotation is not usually marked. This type usually occurs when patients with relatively slight paralysis have been allowed up too early ; it does not usually progress to severe deformity but may occasionally do so, gradually changing into Type 2. This type usually responds well to a period of rest and muscle redevelopment in recumbency. It also responds favourably to correction and fusion because correction is easy and there is little tendency to deterioration. Many of the "successes" of correction and fusion are in this class—almost equal success would often have been gained without "correction." The spine is slightly, but not very, unstable and a relatively localised fusion will give the little extra support that is needed.

2. The "general collapse" type of curve due to extensive spinal weakness. This is the type in which simple head suspension produces marked correction. Rotation is moderate. Provided the patient's general condition is satisfactory extensive spinal fusion is usually the best treatment and produces gratifying improvement.

3. The primary lumbar curve due to a combination of pelvic obliquity, extraspinal imbalance and imbalance of the deep rotator muscles. Rotation is usually marked. Treatment must include the correction of all these factors. In mild cases correction of the pelvic obliquity is enough, but in marked cases the spine must also be corrected. The disability from a lumbar paralytic scoliosis is much greater than that from a lumbar idiopathic scoliosis of the same degree; so correction is necessary in this type. Correction in a Risser-type jacket is often inadequate and recourse to operative correction is usually required.

4. The primary thoracic curve—often associated with weakness of the scapular muscles. The indications for and methods of treatment are practically the same as in primary idiopathic thoracic curves. These curves tend to be progressive and uncompensated. Although the most popular treatment is correction and fusion, wedge osteotomy of the spine gives better correction in intractable cases.

The main need is for further investigation into the etiology of paralytic scoliosis so that adequate preventive measures may be undertaken at an early stage. It is essential that every child who contracts poliomyelitis should have his back muscles examined before he gets up. If there is any suggestion of scoliosis further investigations including radiography and electromyography are essential.

1. The prognosis of paralytic scoliosis has been studied by defining curve patterns and establishing the natural development as seen in fully grown patients who have not had surgical correction.

2. The prognosis, unlike that in idiopathic scoliosis, is related to the age of onset of the curvature and the degree of muscle imbalance rather than the site of the primary curve.

3. Paralysis of limb muscles is shown to be unrelated to the development of scoliosis. The intercostal muscles and the lateral abdominal flexors produce scoliosis when weaker on the convex side of the curve. Gravity and the other trunk muscles certainly play a part in the development of lumbar curves but their importance is difficult to assess.

The evolution of an idiopathic scoliosis is determined by the site of the primary curve and by the age of onset. It is significant that thoracic primary curves are commonly severe and the early onset of this curve accentuates this feature. Early operation based on prognosis is practised but sufficient time has not yet elapsed to justify any conclusions.

1. Five cases of scoliosis with paraplegia are reported, and thirty-six comparable cases from the literature are reviewed. These forty-one cases have been studied with the object of determining the etiology of scoliosis, the reason why cord compression sometimes develops, and the results of conservative and operative treatment of such compression of the cord.

2. The cause of paraplegia is nearly always compression of the spinal cord by the dura, which, in severe scoliosis, is under longitudinal tension because of its firm attachment to the foramen magnum above and the sacrum below. Such tension, resisting displacement of the spinal cord from the straight line, may be shown to cause incomplete spinal block even when there is no paralysis.

3. When paralysis occurs it usually develops during the years of most rapid growth, the tight dura being unable to accommodate itself to the rate of growth of the spinal column; cord compression is probably increased by narrowing of the dural sac by rotational displacement.

4. The most striking results have been secured by laminectomy with section of the dura and sometimes division of dentate ligaments and tight nerve roots. After such division there is evidence of release of compression: the cord herniates through the dural slit; and spinal pulsation returns.

5. It is important to control bleeding in order to avoid post-operative compression by blood clot; and to prevent leakage of cerebro-spinal fluid through the arachnoid.

6. It is unwise to perform spinal fusion at the same time as decompression because it increases the danger of haematoma formation. Moreover the improvement gained by decompression is maintained even if no fusion of the spine is performed.

7. Conservative treatment of scoliosis with paraplegia should not be continued for long periods unless there is evidence of early and progressive improvement because prolonged compression causes irreversible changes in the cord.

8. In three cases, paraplegia was not due to dural compression: one turned out later to be a case of syringomyelia; one, reported by Heyman, was due to the pressure of a bone spur; and one, reported in this series, was due to a congenital tight band of developmental origin which might have caused the scoliosis as well as the paralysis, and in which, after resection of the band, recovery from the paralysis was complete.

Conventional growing rods are the most commonly used distraction-based devices in the treatment of progressive early-onset scoliosis. This technique requires repeated lengthenings with the patient anaesthetised in the operating theatre. We describe the outcomes and complications of using a non-invasive magnetically controlled growing rod (MCGR) in children with early-onset scoliosis. Lengthening is performed on an outpatient basis using an external remote control with the patient awake.

Between November 2009 and March 2011, 34 children with a mean age of eight years (5 to 12) underwent treatment. The mean length of follow-up was 15 months (12 to 18). In total, 22 children were treated with dual rod constructs and 12 with a single rod. The mean number of distractions per patient was 4.8 (3 to 6). The mean pre-operative Cobb angle was 69° (46° to 108°); this was corrected to a mean 47° (28° to 91°) post-operatively. The mean Cobb angle at final review was 41° (27° to 86°). The mean pre-operative distance from T1 to S1 was 304 mm (243 to 380) and increased to 335 mm (253 to 400) in the immediate post-operative period. At final review the mean distance from T1 to S1 had increased to 348 mm (260 to 420).

Two patients developed a superficial wound infection and a further two patients in the single rod group developed a loss of distraction. In the dual rod group, one patient had pull-out of a hook and one developed prominent metalwork. Two patients had a rod breakage; one patient in the single rod group and one patient in the dual rod group. Our early results show that the MCGR is safe and effective in the treatment of progressive early-onset scoliosis with the avoidance of repeated surgical lengthenings.

Cite this article: Bone Joint J 2013;95-B:75–80.

This is a study of children who first attended as infants with either progressive infantile idiopathic scoliosis or congenital scoliosis. All had a pattern of scoliosis in which early and damaging deterioration is inevitable. The infants were treated from before the age of three, initially by plaster casts and then a Milwaukee brace, followed at about the age of ten by correction and fusion. The cases were then observed to the end of growth or near that point. In the main study there were twelve cases, six of progressive infantile idiopathic scoliosis and six of congenital scoliosis, which were followed through this long period. Only one of the twelve had a curve worse at the end of growth compared with the initial radiograph as an infant; this one curve had increased only 16 degrees in almost as many years. Although small, the series does show that it is nearly always possible to control even the most serious scoliosis in an infant, if it is tackled early and unremittingly. There are supportive studies of children who have partially completed this regime, and interim results in a newer group of children with spina bifida and scoliosis.

An attempt to produce scoliosis in young baboons by excision of the heads of ribs failed in thirteen growing animals observed for up to a year after operation. Other investigators have failed to produce scoliosis in primates by similar and other techniques that had successfully produced scoliosis in quadruped animals. The possible reasons for this are discussed, especially in the light of clinical trials that are being carried out with techniques transposed from the quadruped experimental animal to the scoliosis clinic.

An attempt has been made to determine the aetiological factors in infantile idiopathic scoliosis from a clinical, genetic and epidemiological survey of 134 infants, ninety-seven of whom developed a curve in the first six months of life. Plagiocephaly was present in all cases; mental retardation occurred in 13 per cent of males with progressive scoliosis; congenital dislocation of the hip occurred in 3.5 per cent of cases and congenital heart disease in 2.5 per cent; and inguinal hernia was found in 7.4 per cent of males. Approximately 3 per cent of parents and 3 per cent of sibs had the same deformity, thirty times the general population frequency for the Edinburgh area. Other positive findings included an excess of breech presentations and of premature, low birthweight males, and a preponderance of curves developing in the winter months. Infants with progressive scoliosis tended to have older mothers and to come from poorer families. Only three children, all with resolving scoliosis, habitually lay prone in early infancy, in marked contrast to North American infants where this posture is usual. The almost complete absence of infantile idiopathic scoliosis in North America is noted and it is thought that the two facts may be related. The aetiology is likely to be multifactorial, with a genetic tendency to the deformity which is either "triggered off" or prevented by external factors.

1. Eleven patients with juvenile rheumatoid arthritis, most of them young adults at a terminal stage, were found to have structural scoliosis with curves measuring between 20 and 80 degrees.

2. The common feature was severe and protracted rheumatoid disease.

3. The characteristics of the spinal curves are analysed; the longer curves may have been caused by muscle imbalance and the shorter curves possibly by asymmetrical involvement of the inter-apophyseal joints.

4. It is suggested that juvenile rheumatoid arthritis is an unusual etiological factor of scoliosis.

1. The results of 243 thoracoplasty operations are discussed. It was found that scoliosis developed in over 99 per cent of cases and that the curve was convex towards the side of operation. The angle of curvature correlated closely with the number of ribs removed.

2. If the head, neck and tubercle of the rib and the transverse process of the corresponding vertebra were all removed the degree of scoliosis was increased, whereas if a thoracoplasty was performed with apicolysis and embedding of the scapula the spinal deformity was less. Possible explanations for these phenomena are discussed.

3. No evidence was found to support the theory of causation by imbalance between the paired "pneumatic cylinders" (lungs and thoracic cage) supporting the thoracic spine.

4. The scoliosis was found to progress for many years; some factors influencing this course are discussed.

5. No correlation was found between the age of the patient at the time of thoracoplasty and the degree of subsequent scoliosis, but severe spinal deformity can arise even in older adults.

1. Idiopathic scoliosis is a familial condition.

2. The findings suggest either dominant or multiple gene inheritance, but a larger series is needed before a firm conclusion can be drawn.

3. The infantile and adolescent types of scoliosis seem to share the same basic etiology, because their families contain instances of each.

4. Infants with resolving scoliosis have affected relatives in the same proportions as in the main group, suggesting this is a mild form of the same disorder.

5. In this series all infants seen with scoliosis under one year of age had plagiocephaly, which was usually transient.

6. Mental defect and epilepsy are the commonest findings associated with scoliosis.

7. In adolescent scoliosis the age of the mother is significantly raised by comparison with the expected figure for the normal population.

1. Evidence is presented that the basic lesion in scoliosis is relative lengthening of the anterior components of the spine compared with the posterior elements.

2. The logical treatment is to reduce this relative lengthening either by lengthening the posterior elements or shortening the anterior elements. This may be achieved by anterior lumbar wedge osteotomy or by epiphysiodesis; and correction of lumbar lordosis can improve a thoracic scoliosis.

"Wedge excision" of the apex of the curve is the rational way of correcting a scoliosis. It is a straightforward procedure which is successful in practice.

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.