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Prader-Willi syndrome and scoliosis.

Of 37 patients with the Prader-Willi syndrome for whom spinal x-rays were available, 32 had a structural scoliosis of 10 degrees or greater. Kyphosis was also found to be more common in older persons with this syndrome, occurring in only one of 14 adolescents but in five of 10 adults. The scoliosis was analyzed in infantile, juvenile, adolescent and adult subgroups. The results suggest that the scoliosis is present from an early age and remains stable during childhood, but progresses in 15 to 20 per cent of cases during adolescence. Scoliosis in patients with Prader-Willi syndrome has many of the clinical characteristics encountered in idiopathic scoliosis. Because it is difficult to detect in these individuals, the authors recommended that baseline spinal X-rays be obtained in all such patients who have suspicious asymmetry of the spine, followed by regular clinical examination, especially during adolescence.

Adolescent↗

Conventional metrizamide myelography (MM) and computed tomographic metrizamide myelography (CTMM) in scoliosis. A comparative study.

A retrospective examination was performed to assess the accuracy of metrizamide myelography (MM) and computed tomographic metrizamide myelography (CTMM) in scoliosis. Of 81 consecutive scoliotic children studied by myelography, 30 had only MM while the remaining 51 had CTMM immediately afterward. CTMM added essential diagnostic information in 13 cases of dysraphism and 4 cases of idiopathic scoliosis; in the remaining cases, both methods gave the same information. The authors conclude that in patients with severe scoliosis, dysraphism, and scoliosis with localized neurological disturbances, CTMM should always be added to MM or be the only examination; while in idiopathic scoliosis with vague neurological disturbances a survey of the entire spine is essential, preferably with MM.

Adolescent↗

Factors determining pulmonary function in adolescent idiopathic thoracic scoliosis.

Adolescent idiopathic thoracic scoliosis may lead to severe pulmonary impairment and early death, but the responsible factors are poorly understood; pulmonary function is only weakly related to the angle of scoliosis. We performed a cross-sectional study using multivariate analysis to identify the individual and additive influence of different features of spinal deformity and nonstructural factors on pulmonary impairment. Pulmonary function was assessed by measuring lung volumes and diffusing capacity, with a priori selection of vital capacity (expressed as percentage of predicted, % VC) as the primary index of pulmonary impairment. Radiologic and physiologic measurements were made independently in 66 subjects who had not previously had spinal surgery. Angle of scoliosis (p = 0.01) was one of four features of spinal deformity associated with reduced % VC; greater number of vertebrae involved (p = 0.007), cephadal location of the curve (p = 0.04), and loss of the normal thoracic kyphosis (p = 0.002) made an equal and additive contribution to pulmonary impairment. Spinal deformity led to reductions in VC, primarily by reducing TLC. Spinal column rotation, respiratory muscle strength, and duration of the curvature were not related to pulmonary function (p > 0.05). We conclude that features of the spinal deformity are the major determinants of pulmonary impairment in idiopathic thoracic scoliosis but that the relationship between deformity and impairment is complex. The severity of pulmonary impairment cannot be inferred to a clinically useful extent from the angle of scoliosis alone.

Adolescent↗

Factors influencing work capacity in adolescent idiopathic thoracic scoliosis.

The factors contributing to reduced work capacity (disability) in adolescent idiopathic thoracic scoliosis are poorly understood. We performed a cross-sectional study using multivariate analysis to identify the individual and additive influence of spinal deformity, pulmonary impairment, and muscular function on work capacity in 79 subjects with idiopathic scoliosis (angle of scoliosis 45 +/- 18.5 degrees, SD). Work capacity was measured using an incremental cycle test, and the cardiorespiratory response to exercise was compared with that of normal subjects. Work capacity was reduced (% Wcap, 86%; 95% CI 81.9 to 89.7), indicating significant disability. The % Wcap was unrelated to the nature and extent of spinal deformity (p > 0.05). Leg muscularity and pulmonary impairment had an additive influence on work capacity, the relationship with muscularity being the stronger of the two. Independently of muscularity and pulmonary impairment, a high heart rate response at submaximal work rates was also associated with a reduced work capacity. Ventilation was normal for metabolic demands. During exercise, the tidal volumes of scoliotic subjects were reduced in proportion to the vital capacity. We conclude that disability occurs with mild to moderate idiopathic scoliosis and appears to be related to a combination of reduced ventilatory capacity, reduced muscularity, and cardiovascular deconditioning. These findings suggest that physical activity should be encouraged in subjects with idiopathic scoliosis to maintain peripheral muscle and cardiovascular conditioning, thereby minimizing disability.

Adolescent↗

Prevention of rapidly progressive scoliosis in Duchenne muscular dystrophy by prolongation of walking with orthoses.

We reviewed the incidence and severity of scoliosis in 93 boys with Duchenne muscular dystrophy who had been rehabilitated in light-weight knee-ankle-foot orthoses at the point of loss of ambulation, between the ages of 6 and 12 years. There was an inverse relationship between the severity of the scoliosis and the age walking was lost in the orthoses. The scoliosis was less severe in the 20 boys (22%) who walked in their orthoses beyond 13 years of age than in those who stopped walking in their orthoses before 13 years. There was also a rapid deterioration in the scoliosis between the ages of 13 and 15 years in boys who had stopped walking in their orthoses before the age of 13 years, while in comparison, boys of the same age who were ambulant in their orthoses beyond 13 years showed a much slower rate of deterioration. These results strongly suggest that walking in orthoses beyond the age of 13 years prevented rapid progression of scoliosis between 13 and 15 years of age, ie, during the pubertal growth spurt.

Adolescent↗

Why do we treat adolescent idiopathic scoliosis? What we want to obtain and to avoid for our patients. SOSORT 2005 Consensus paper.

BACKGROUND: Medicine is a scientific art: once science is not clear, choices are made according to individual and collective beliefs that should be better understood. This is particularly true in a field like adolescent idiopathic scoliosis, where currently does not exist definitive scientific evidence on the efficacy either of conservative or of surgical treatments. AIM OF THE STUDY: To verify the philosophical choices on the final outcome of a group of people believing and engaged in a conservative treatment of idiopathic scoliosis. METHODS: We performed a multifaceted study that included a bibliometric analysis, a questionnaire, and a careful Consensus reaching procedure between experts in the conservative treatment of scoliosis (SOSORT members). RESULTS: The Consensus reaching procedure has shown to be useful: answers changed in a statistically significant way, and 9 new outcome criteria were included. The most important final outcomes were considered Aesthetics (100%), Quality of life and Disability (more than 90%), while more than 80% of preferences went to Back Pain, Psychological well-being, Progression in adulthood, Breathing function, Scoliosis Cobb degrees (radiographic lateral flexion), Needs of further treatments in adulthood. DISCUSSION: In the literature prevail outcome criteria driven by the contingent treatment needs or the possibility to have measurement systems (even if it seems that usual clinical and radiographic methods are given much more importance than more complex Disability or Quality of Life instruments). SOSORT members give importance to a wide range of outcome criteria, in which clinical and radiographic issues have the lowest importance. CONCLUSION: We treat our patients for what they need for their future (Breathing function, Needs of further treatments in adulthood, Progression in adulthood), and their present too (Aesthetics, Disability, Quality of life). Technical matters, such as rib hump or radiographic lateral alignment and rotation, but not lateral flexion, are secondary outcomes and only instrumental to previously reported primary outcomes. We advocate a multidimensional, comprehensive evaluation of scoliosis patients, to gather all necessary data for a complete therapeutic approach, that goes beyond x-rays to reach the person and the family.

Journal Article↗

Neurologic features of horizontal gaze palsy and progressive scoliosis with mutations in ROBO3.

OBJECTIVE: To review the neurologic, neuroradiologic, and electrophysiologic features of autosomal recessive horizontal gaze palsy and progressive scoliosis (HGPPS), a syndrome caused by mutation of the ROBO3 gene on chromosome 11 and associated with defective decussation of certain brainstem neuronal systems. METHODS: The authors examined 11 individuals with HGPPS from five genotyped families with HGPPS. Eight individuals had brain MRI, and six had electrophysiologic studies. RESULTS: Horizontal gaze palsy was fully penetrant, present at birth, and total or almost total in all affected individuals. Convergence, ocular alignment, congenital nystagmus, and vertical smooth pursuit defects were variable between individuals. All patients developed progressive scoliosis during early childhood. All appropriately studied patients had hypoplasia of the pons and cerebellar peduncles with both anterior and posterior midline clefts of the pons and medulla and electrophysiologic evidence of ipsilateral corticospinal and dorsal column-medial lemniscus tract innervation. Heterozygotes were unaffected. CONCLUSIONS: The major clinical characteristics of horizontal gaze palsy and progressive scoliosis were congenital horizontal gaze palsy and progressive scoliosis with some variability in both ocular motility and degree of scoliosis. The syndrome also includes a distinctive brainstem malformation and defective crossing of some brainstem neuronal pathways.

Adolescent↗

Screening for scoliosis: do we have indications for effectiveness?

OBJECTIVE: The effectiveness of screening for scoliosis has not been established. This study investigated whether patients with adolescent idiopathic scoliosis detected by screening are detected in an earlier stage of the clinical course, and whether these patients have better outcome than otherwise detected patients. SETTING: The study is a retrospective follow-up study of patients with adolescent idiopathic scoliosis who had completed treatment with a brace, by surgery, or with a brace followed by surgery. Of the 143 patients (born on or after 1 January 1984) consecutively recruited from 12 hospitals in the Netherlands, 125 (87%) agreed to participate. Of these, 51 patients were treated with a brace only and 74 patients were operated on. Screening for scoliosis is carried out in 80% of Dutch children. METHODS: Data on being screen detected or otherwise detected and Cobb angle at diagnosis were collected using youth health-care files, medical files and interviews by telephone with the patients. RESULTS: About 55% of the patients were detected by screening (programme sensitivity). Screen-detected patients had a significantly smaller Cobb angle at diagnosis (28 degrees versus 40 degrees ; P<0.01) and had a 73% lower chance of having had surgery (45% versus 75%; P<0.01) than otherwise-detected patients. CONCLUSION: In the present study, two essential prerequisites necessary for a screening programme for scoliosis to be effective have been met. However, definite proof of the effectiveness of screening still needs to be established because length bias and over-treatment bias cannot be ruled out using this design.

Adolescent↗

The behaviour pattern of the scoliosis associated with osteoid osteoma or osteoblastoma of the spine.

The behaviour pattern of the scoliosis associated with osteoid osteoma or osteoblastoma of the spine is described. In patients presenting with symptoms at or around skeletal maturity, the scoliosis is postural. Excision of the lesion ensures complete resolution of the curve. In the growing child, however, an initial postural scoliosis may develop vertebral rotation with structural characteristics. The magnitude of the curve and the associated vertebral rotation is dependent on the time interval between the onset of symptoms and the surgical treatment. Although removal of the lesion usually results in regression of the curve, a prolonged delay in treatment may result in a progressive structural scoliosis. A possible mechanism for the behaviour of the scoliosis is discussed.

Adolescent↗

Scoliosis caused by section of dorsal spinal nerve roots.

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.

Animals↗

The potential role of the elastic fiber system in adolescent idiopathic scoliosis.

To assess its possible role in the etiology of adolescent idiopathic scoliosis, the elastic fiber system of the ligamentum flavum was examined in twenty-three patients who had scoliosis and in five age-matched individuals who did not. Elastic fibers are composed of two components: the amorphous core of elastin and microfibrils, of which fibrillin is the primary element. Fresh-frozen histological specimens of ligamentum flavum removed at the time of an operation were examined by Verhoeff staining for elastic fibers and by immunohistochemical staining with use of a monoclonal antibody to fibrillin. Additionally, cultures of fibroblast cells from the ligamentum flavum were used to study the biosynthesis and secretion of fibrillin and its incorporation into the extracellular matrix in vitro. The specimens from one patient did not provide sufficient material for the histological studies; however, fibroblasts were harvested from this specimen. In five (23 percent) of the remaining twenty-two specimens from patients who had adolescent idiopathic scoliosis, Verhoeff staining of elastic fibers showed a marked decrease in fiber density (the number of fibers per unit area) and a non-uniform distribution of fibers throughout the ligament. Eighteen specimens (82 percent) exhibited abnormalities on immunohistochemical staining, including a marked disarrangement of the fibers and a difference in the density of staining, when compared with the control specimens from individuals who did not have adolescent idiopathic scoliosis. Studies of the biosynthesis and secretion of fibrillin and its incorporation into the extracellular matrix in vitro demonstrated that fibroblasts from four (17 percent) of the twenty-three specimens produced normal amounts of fibrillin and secreted it from the cell, but the fibrillin failed to bind to other macromolecules, to form a sedimentable complex, and to incorporate into the extracellular matrix. Collectively, the results suggest the potential role of the elastic fiber system as a component in the pathogenesis of adolescent idiopathic scoliosis in some individuals.

Adolescent↗

Harrington and Cotrel-Dubousset instrumentation in adolescent idiopathic scoliosis. Long-term functional and radiographic outcomes.

BACKGROUND: Previous studies have shown that the long-term clinical outcome does not correlate with the radiographic outcome in patients treated with Harrington instrumentation for adolescent idiopathic scoliosis. Cotrel-Dubousset instrumentation has been reported to provide better correction radiographically, but it is unclear whether it provides better long-term clinical or functional outcomes. We are not aware of any long-term studies comparing Harrington and Cotrel-Dubousset instrumentation. METHODS: Seventy-eight patients in whom adolescent idiopathic scoliosis was treated with Harrington instrumentation and fifty-seven in whom it was treated with Cotrel-Dubousset instrumentation participated in this study. The mean duration of follow-up was 20.8 years for the Harrington instrumentation group and 13.0 years for the Cotrel-Dubousset instrumentation group. The mean age at the time of follow-up was thirty-six years and twenty-eight years, respectively. Radiographs were made preoperatively and at the two-year and final follow-up examinations. The Scoliosis Research Society questionnaire was completed, a physical examination was performed, and spinal mobility and non-dynamometric trunk strength were measured at the final follow-up visit. RESULTS: The mean preoperative Cobb angle of the thoracic curves was 53 degrees in the Harrington instrumentation group and 55 degrees in the Cotrel-Dubousset instrumentation group. The mean numbers of vertebrae included in the instrumentation were 10.7 and 9.9, respectively. At the two-year follow-up evaluation, the mean postoperative Cobb angles were 38 degrees and 25 degrees, respectively (p < 0.0001). At the final follow-up evaluation, the mean angles were 45 degrees and 32 degrees (p < 0.0001). No significant difference in thoracic kyphosis or lumbar lordosis was observed between the study groups at the final follow-up evaluation. The average score on the Scoliosis Research Society questionnaire was 97 points in both groups. Measurements of non-dynamometric trunk strength corresponded with age and sex-adjusted reference values, on the average, but patients with Cotrel-Dubousset instrumentation performed significantly better in the squatting test (p = 0.010). Abnormal lumbar extension and trunk side-bending were significantly more common in the Harrington instrumentation group (p = 0.050 and p = 0.0061, respectively). Complications were recorded for nine (12%) of the patients treated with Harrington instrumentation and fifteen (26%) of those treated with Cotrel-Dubousset instrumentation (p = 0.027). CONCLUSIONS: Cotrel-Dubousset instrumentation yielded better long-term functional and radiographic outcomes in patients with adolescent idiopathic scoliosis than did Harrington instrumentation. However, complications were more common in the Cotrel-Dubousset instrumentation group.

Adolescent↗

Pulmonary function in adolescent idiopathic scoliosis relative to the surgical procedure.

BACKGROUND: The long-term pulmonary function of patients with adolescent idiopathic scoliosis undergoing surgical correction is uncertain. To our knowledge, no report has demonstrated the changes in pulmonary function five years or more following spinal arthrodesis with use of modern segmental spinal instrumentation techniques for the treatment of all types of adolescent idiopathic scoliosis in a similar adolescent population. METHODS: One hundred and eighteen patients with adolescent idiopathic scoliosis undergoing surgical treatment at a single institution were evaluated with pulmonary function tests to assess the absolute and percent-predicted value of forced vital capacity and forced expiratory volume in one second at the preoperative examination and at regular intervals postoperatively. The patients were divided into four groups depending upon the surgical procedure: Group 1 comprised forty-nine patients who had posterior spinal arthrodesis with iliac crest bone graft; Group 2, forty-one patients who had posterior spinal arthrodesis with thoracoplasty; Group 3, sixteen patients who had open anterior spinal arthrodesis with a rib resection thoracotomy; and Group 4, twelve patients who had combined anterior and posterior spinal arthrodesis with a rib resection thoracotomy and iliac crest bone graft, respectively. RESULTS: A comparison of absolute pulmonary function values from the preoperative and final follow-up evaluations demonstrated a significant (p < 0.0001) increase in both the forced vital capacity and the forced expiratory volume in one second for Group 1, whereas no change was seen in those values for Groups 2, 3, and 4. A comparison of the changes in the percent-predicted pulmonary function values demonstrated significant (p < 0.05) decreases in forced vital capacity and forced expiratory volume in one second for Groups 2, 3, and 4, except for the latter value for Group 4, whereas Group 1 had no change. CONCLUSIONS: Patients who have had any type of chest cage disruption during the surgical treatment of adolescent idiopathic scoliosis demonstrate no change in the absolute value and a significant decline in the percent-predicted value of pulmonary functions at five years following surgery. Chest cage preservation is recommended to maximize both absolute and percent-predicted pulmonary function values after surgical treatment of adolescent idiopathic scoliosis.

Adolescent↗

Osteopenia: a new prognostic factor of curve progression in adolescent idiopathic scoliosis.

BACKGROUND: Studies have shown that 27% to 38% of girls with adolescent idiopathic scoliosis have systemic osteopenia. The aim of this study was to investigate whether osteopenia could serve as one of the important prognostic factors in predicting curve progression. METHODS: A prospective study was performed in 324 adolescent girls with adolescent idiopathic scoliosis who had a mean age of thirteen and a half years. Bone mineral density of the spine and both hips was measured at the time of the clinical diagnosis of scoliosis. All patients were followed longitudinally until skeletal maturity or until the curve had progressed > or =6 degrees . The univariate chi-square test and stepwise logistic regression were used to predict the prevalence of curve progression, and a receiver operating characteristic curve was plotted. RESULTS: The overall prevalence of curve progression was 50%. The prevalence of osteopenia at the spine and hips was 27.5% and 23.1%, respectively. A larger initial Cobb angle (odds ratio = 4.6), a lower Risser grade (odds ratio = 4.7), premenarchal status (odds ratio = 2.5), osteopenia in the femoral neck of the hip on the side of the concavity (odds ratio = 2.3), and a younger age at the time of diagnosis (odds ratio = 2.1) were identified as risk factors in predicting curve progression. A predictive model was established, and the area under the receiver operating characteristic curve of the model was 0.80 (p < 0.01). CONCLUSION: Osteopenia may be an important risk factor in curve progression. The measurement of bone mineral density at the time of diagnosis may serve as an additional objective measurement in predicting curve progression in adolescent idiopathic scoliosis. The bone mineral density-inclusive predictive model may be used in treatment planning for patients with adolescent idiopathic scoliosis who are at high risk of curve progression.

Adolescent↗

Thoracoscopic spinal fusion compared with posterior spinal fusion for the treatment of thoracic adolescent idiopathic scoliosis.

BACKGROUND: Posterior spinal fusion with segmental instrumentation is the gold standard for the surgical treatment of thoracic adolescent idiopathic scoliosis. More recently, anterior surgery and video-assisted thoracoscopic surgery with spinal instrumentation have become available. The purpose of the present study was to compare the radiographic and clinical outcomes as well as pulmonary function in patients managed with either anterior thoracoscopic or posterior surgery. METHODS: Radiographic data, Scoliosis Research Society patient-based outcome questionnaires, pulmonary function, and operative records were reviewed for fifty-one patients undergoing surgical treatment of scoliosis. Data were collected preoperatively, immediately postoperatively, and at the time of the final follow-up. The radiographic parameters that were analyzed included coronal curve correction, the most caudad instrumented vertebra tilt angle correction, coronal balance, and thoracic kyphosis. The operative parameters that were evaluated included the operative time, the estimated blood loss, the blood transfusion rate, the number of levels fused, the type of bone graft used, and the number of intraoperative and postoperative complications. The pulmonary function parameters that were analyzed included vital capacity and peak flow. RESULTS: The thoracoscopic group included twenty-eight patients with a mean age of 14.6 years, and the posterior fusion group included twenty-three patients with a mean age of 14.3 years. The percent correction was 54.5% for the thoracoscopic group and 55.3% for the posterior group. With the numbers available, there were no significant differences between the two groups in terms of kyphosis (p = 0.84), coronal balance (p = 0.70), or tilt angle (p = 0.91) at the time of the final follow-up. The mean number of levels fused was 5.8 in the thoracoscopic group, compared with 9.3 levels in the posterior group (p < 0.0001). The estimated blood loss in the thoracoscopic group was significantly less than that in the posterior fusion group (361 mL compared with 545 mL; p = 0.03), and the transfusion rate in the thoracoscopic group was significantly lower than that in the posterior fusion group (14% compared with 43%; p = 0.01). Operative time in the thoracoscopic group was significantly greater than that in the posterior group (6.0 compared with 3.3 hours, p < 0.0001). There were no intraoperative complications in either group. Vital capacity and peak flow had returned to baseline levels in both groups at the time of the final follow-up. Patients in the thoracoscopic group scored higher than those in the posterior group in terms of the total score (p < 0.0001) and all of the domains (p < 0.01) of the Scoliosis Research Society questionnaire at the time of the final follow-up. CONCLUSIONS: Thoracoscopic spinal instrumentation compares favorably with posterior fusion in terms of coronal plane curve correction and balance, sagittal contour, the rate of complications, pulmonary function, and patient-based outcomes. The advantages of the procedure include the need for fewer levels of spinal fusion, less operative blood loss, lower transfusion requirements, and improved cosmesis as a result of small, well-hidden incisions. However, the operative time for the thoracoscopic procedure was nearly twice that for the posterior approach. Additional study is needed to determine the precise role of thoracoscopic spinal instrumentation in the treatment of thoracic adolescent idiopathic scoliosis.

Adolescent↗

Maturity assessment and curve progression in girls with idiopathic scoliosis.

BACKGROUND: Scoliosis progression during adolescence is closely related to patient maturity. Maturity has various indicators, including chronological age, height and weight changes, and skeletal and sexual maturation. It is not certain which of these indicators correlates most strongly with scoliosis progression. The purpose of the present study was to evaluate various maturity measurements and how they relate to scoliosis progression. METHODS: Physically immature girls with idiopathic scoliosis were evaluated every six months through their growth spurt with serial spinal radiographs; hand skeletal ages; Oxford pelvic scores; Risser sign determinations; height; weight; sexual staging; and serologic studies of the levels of selected growth factors, estradiol, bone-specific alkaline phosphatase, and osteocalcin. These measurements were then correlated with the curve-acceleration phase. RESULTS: The period and pattern of curve acceleration began during Risser stage 0 for all patients. Skeletal maturation scores derived with the use of the Tanner-Whitehouse-III RUS method, particularly those for the metacarpals and phalanges, were superior to all other indicators of maturity. Regression of the scores provided good estimates of maturity relative to the period of curve progression (Pearson r = 0.93). The initiation of this period occurred simultaneously with digital changes from Tanner-Whitehouse-III stage F to G. At this stage, curves also separated into rapid, moderate, and low-acceleration patterns, with specific curve types in the rapid and moderate-acceleration groups. The low-acceleration group was not confined to a specific curve type. CONCLUSIONS: The curve-acceleration phase separates curves into various types of curve progression. The Tanner-Whitehouse-III RUS scores are highly correlated with timing relative to the curve-acceleration phase and provide better maturity determination and prognosis determination during adolescence than the other parameters tested. Accurate skeletal maturity determination should be used as the primary maturity measurement in girls with idiopathic scoliosis.

Adolescent↗

Scoliosis in children with juvenile rheumatoid arthritis.

Juvenile rheumatoid arthritis (JRA) is an unusual causative factor of scoliosis in childhood. Among 320 patients with JRA in Sweden, 17 (5.3%) had structural scoliosis of thoracic and lumbar spine. This frequency is considerably higher than in the normal population. The mean age when scoliosis was first noted was 12.4 years, corresponding to a mean duration of the JRA of 7.4 years. A curvature exceeding 20 degrees was found in 12 out of the 17 patients. The scoliosis in our patients was associated with severe and protracted polyarthritis of early onset and involvement of hip joints and cervical spine. Contracture of the hip with pelvic tilt was found in 13 and torticollis in 3 of the patients. These factors, in addition to growth disturbances and probable inflammatory processes of spinal joints, may explain the development of the scoliosis.

Adolescent↗

Costodesis and contralateral rib release in the management of progressive scoliosis.

This paper reports on 41 immature patients whose scoliosis was treated by fixation of ribs on the convexity. All had progressive scoliosis, the curve deteriorating at more than 10 degrees p.a., or the rib-vertebra angle difference being greater than 20 degrees. Of the 24 with infantile idiopathic scoliosis examined 5 years after operation, 10 maintained the improvement obtained at operation and in a further 19 the rate of deterioration had been slowed. Factors leading to a favourable outcome included: 1) An initial rib-vertebra angle difference of less than 30 degrees. 2) Success in achieving convex rib fusion. 3) The use of the operation in patients with infantile idiopathic scoliosis. There was a less favourable outcome in congenital and adolescent scoliosis. Spirometric volumes were diminished immediately after operation. Costodesis is therefore contraindicated in patients with precarious respiratory function.

Child↗