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Epidemiological patterns of scoliosis in a spinal center in Saudi Arabia.

OBJECTIVE: To study the patterns of scoliosis seen in a major Spinal Unit in Saudi Arabia. METHODS: Medical records of 192 Saudi patients with scoliosis seen between 1992 and 1997 at the Spinal Unit of the Riyadh Medical Complex (which is a major spinal unit in the Kingdom) were studied and analyzed and epidemiological data was collected. RESULTS: Fifty nine percent of all cases of scoliosis were idiopathic, 7% were secondary to poliomyelitis and 17% were congenital scoliosis. The mean age of discovery of idiopathic scoliosis was 12.5 years and at presentation was 16 years. The mean Cobb's angle at presentation was 58 degrees C. Adolescent type constituted 74% of idiopathic curves with a male to female ratio of 1:3.8. Thoracic curves were the most common followed by the double major curves. Half of the curves were right sided. Infantile idiopathic scoliosis constituted 8% with male to female ratio of 4:1 and here left thoracic curves formed 50%. The juvenile type constituted the remaining 18% with male to female ratio of 1:1.5. CONCLUSION: The pattern of scoliosis in our study is comparable in many aspects with other studies carried out elsewhere, with the exception of delay in patients age at referral. We believe that our data could reflect a possible pattern of the epidemiology of scoliosis in Saudi Arabia, given the fact that there is no National Registry. To avoid delay in management, community education will help in the early detection of cases.

Adolescent↗

[Imaging of chilhood and adolescent scoliosis].

Scoliosis is frequent, especially during the peripubertal period, which corresponds to the period of greatest activity. About 75% of scoliosis are idiopathic, while the remaining 25% can be divided into neuromuscular, congenital and miscellaneous etiologies. When it is associated with pain, a comprehensive work up should be undertaken to identify the underlying etiology of the scoliosis. Idiopathic scoliosis is typically evaluated using standard radiographs. The number of follow-up radiographs and radiation exposure must be maintained to a minimum. Radiation safety guidelines and protocols must be implemented. Large cassettes can be replaced by digital acquisitions using units with reconstruction software. The technique of evaluation of these scoliosis series radiographs as well as their results will be reviewed. Recent data regarding the evaluation of spinal and pelvic balance parameters in the standing position will be introduced. Atypical idiopathic and other scoliosis require additional evaluation. Computed tomography, with 2D and 3D reformations, is ideal for evaluation of bony anomalies. MRI is ideal for evaluation of the spinal canal and cord, from the skull base down to the sacrum, for all patients with surgical scoliosis or patients with neurological symptoms. The increased availability of MR imaging has resulted in an increased number of so-called idiopathic asymptomatic scoliosis where an underlying anomaly can be identified.

Adolescent↗

Conservative management in patients with scoliosis--does it reduce the incidence of surgery?

The effectiveness of conservative scoliosis treatment, including bracing, is widely denied. Like any therapeutic intervention, conservative scoliosis treatment including patient education, techniques of brace moulding, and time-consuming follow-ups is reasonable only if the benefits outweigh the strain placed upon the patient by the treatment. The most important benefit of early intervention in scoliosis is prevention of the need for spinal fusion surgery. Retrospective analysis of the incidence of surgery for patients with scoliosis, by comparison with incidence in an untreated control group. Since 1992 the bracing technique according to Chêneau has been applied in parallel with the scoliosis inpatient intensive rehabilitation programme (SIR). For this study we chose from our data base the scoliosis patients who had conservative treatment at our center between 1993 and 1996. All patients, like those of the control group, were at least 15 years of age at the time they were evaluated for the last time. The incidence of surgery of our group was compared with that reported by a center in Ireland. Of 343 female scoliosis patients with a curve angle of 33.4 AE (SD=18.9), 41 (11.95%) had surgery. The incidence of surgery of our collective was significantly lower than the incidence of surgery of the control group which reported an incidence of 28,1%. The AIS matched group of patients (n = 179) had an incidence of surgery of slightly more than 7%. When compared with a matched control group of untreated patients, incidence of surgery was significantly reduced by SIR combined with bracing. So conservative management is indicated in patients with scoliosis.

Adolescent↗

[Long-term follow-up in patients with idiopathic scoliosis].

The study concludes the results of the clinical and radiological follow-up of 51 patients with idiopathic scoliosis after 20-36 years (mean of 24.5 years). The aim of the study was to find out which parameters can give an answer at the beginning about the expectable prognosis of the scoliosis. Most of all rotation of the apex vertebra, the skeletal maturity and the curve pattern of the scoliosis are a precise factor for prognosis. The term "scoliosis in babies" should be left, because these benign spontaneously resolving type of scoliosis can appear in the second age, too. To separate them from the infantile scoliosis, which often has a bad natural history the term "oblique position scoliosis" should be used instead of scoliosis in babies.

Adolescent↗

Social acceptability of treatments for adolescent idiopathic scoliosis: a cross-sectional study.

BACKGROUND: There are no data on social acceptability of scoliosis. Aim. To elicit evidence-based opinions on therapeutic strategies for adolescent idiopathic scoliosis in a sample of families with not affected children, so to understand the social perception of this issue. METHODS: Design. Cross-sectional study. Setting. Secondary schools in 4 northern Italian regions. Participants. Parents of children in the age group at risk of and not affected by scoliosis (Pre-test group = 100, Study group = 3,162). Interventions. Questionnaire: five specific and evidence-based questions regarding scoliosis treatment options and a socio-demographic section. Methodology. "Role-playing" in which it was required to normal people to answer what they would have chosen if they had been in the situation proposed. Main outcome measures. Perception of acceptability of treatments for adolescent idiopathic scoliosis in the general population (social acceptability). RESULTS: The families support the use of screening (94.8%) at school, immediate bracing (76.4%) for scoliosis with a 60% risk of progression, but also therapeutic exercises (86.9%) in cases with a 25% risk of progression. CONCLUSION: There is a growing tendency to consider not only the efficacy, effectiveness and efficiency of treatments, but also their acceptability. This patient-centred aspect is especially more important in areas (like adolescent idiopathic scoliosis) in which there is some evidence on the efficacy of treatments, but not strong and definitive (RCTs). Adolescent idiopathic scoliosis treatments should thus be carefully considered also in the light of their social acceptability.

Journal Article↗

Biomechanical spinal growth modulation and progressive adolescent scoliosis--a test of the 'vicious cycle' pathogenetic hypothesis: summary of an electronic focus group debate of the IBSE.

There is no generally accepted scientific theory for the causes of adolescent idiopathic scoliosis (AIS). As part of its mission to widen understanding of scoliosis etiology, the International Federated Body on Scoliosis Etiology (IBSE) introduced the electronic focus group (EFG) as a means of increasing debate on knowledge of important topics. This has been designated as an on-line Delphi discussion. The text for this debate was written by Dr Ian A Stokes. It evaluates the hypothesis that in progressive scoliosis vertebral body wedging during adolescent growth results from asymmetric muscular loading in a "vicious cycle" (vicious cycle hypothesis of pathogenesis) by affecting vertebral body growth plates (endplate physes). A frontal plane mathematical simulation tested whether the calculated loading asymmetry created by muscles in a scoliotic spine could explain the observed rate of scoliosis increase by measuring the vertebral growth modulation by altered compression. The model deals only with vertebral (not disc) wedging. It assumes that a pre-existing scoliosis curve initiates the mechanically-modulated alteration of vertebral body growth that in turn causes worsening of the scoliosis, while everything else is anatomically and physiologically 'normal' The results provide quantitative data consistent with the vicious cycle hypothesis. Dr Stokes' biomechanical research engenders controversy. A new speculative concept is proposed of vertebral symphyseal dysplasia with implications for Dr Stokes' research and the etiology of AIS. What is not controversial is the need to test this hypothesis using additional factors in his current model and in three-dimensional quantitative models that incorporate intervertebral discs and simulate thoracic as well as lumbar scoliosis. The growth modulation process in the vertebral body can be viewed as one type of the biologic phenomenon of mechanotransduction. In certain connective tissues this involves the effects of mechanical strain on chondrocytic metabolism a possible target for novel therapeutic intervention.

Journal Article↗

Structural vertebral changes in the horizontal plane in idiopathic scoliosis and the long-term corrective effect of spine instrumentation.

The rotation and structural changes of the apex vertebra in the horizontal plane as well as of the thoracic cage deformity were quantified by measurements on computed tomography (CT) scans from patients with right convex thoracic idiopathic scoliosis (IS). The CT scans were obtained from 12 patients with moderate scoliosis (mean Cobb angle 25.8 degrees, r 13 degrees-30 degrees) and from 33 with severe scoliosis (mean Cobb angle 46.2 degrees, r 35 degrees-71 degrees). In addition, CT scans of thoracic vertebrae from 15 patients without scoliosis were used as reference material. Ten of the scoliotic cases had had Cotrel-Dubousset instrumentation (CDI) and posterior fusion and had entered a longitudinal study on the effect of operative correction on the re-modelling of the apical vertebra. An increasingly asymmetrical vertebral body, transverse process angle, pedicle width and canal width were found in the groups with scoliosis as compared with the reference material. Vertebral rotation and rib hump index were significantly larger in patients with early and advanced scoliosis than in normal subjects. The modelling angle of the vertebral body, the transverse process angle index and the vertebral rotation in relation to the middle axis of the thoracic cage were significantly greater in patients with severe than with moderate scoliosis. The results of this longitudinal study suggest that the structural changes of the apical vertebra regress 2 years or more after CD instrumentation.

Adolescent↗

The development of adolescent idiopathic scoliosis.

There are many conflicting actiological theories for adolescent idiopathic scoliosis. We present a simple new model of scoliosis and a mechanism by which it is initiated and progresses. This mechanism provides a final common pathway for the multiple aetiological factors. A simple model of the spine, incorporating its fundamental mechanical features, was constructed. The model consisted of interconnected anterior compression and posterior tension columns. It allowed normal spinal movements, with flexion limited by the posterior column and rotation centered around the anterior column. It also allowed deformities to develop. The ends of the model were fixed in the position of the vertebrae they represented. Overgrowth of the anterior column relative to the posterior column caused the model to take up the shape of an idiopathic scoliosis. The greater the overgrowth, the more marked the deformity. Normally anterior and posterior column growth are coupled. During the growth spurt the thoracic kyphosis flattens indicating that anterior growth temporarily exceeds posterior growth. If this over-growth is marked a scoliosis will develop, as demonstrated by the model. Once this occurs the coupling is lost, anterior growth further outstrips posterior growth and the deformity progresses. Not all scolioses worsen, as the tendency to progress is balanced by neuromuscular factors and remodelling. Factors that increase the growth rate, induce asymmetry or decrease the inherent stability of the spine all encourage the development and progression of a scoliosis. This explains the complex biomechanics of scoliosis and provides a final common pathway by which the multiple aetiological factors can induce idiopathic scoliosis. It has important implications for the understanding and treatment of this condition.

Adolescent↗

Scoliosis in children with Chiari I-related syringomyelia.

OBJECTIVE: To study the relationship between scoliosis and Chiari I malformation, with reference to the possible role of cranio-vertebral decompression in preventing the need for scoliosis correction. MATERIAL AND METHODS: Out of a total of 36 patients with symptomatic Chiari I, who underwent primary cranio-vertebral decompression by a single paediatric neurosurgeon with an interest in Chiari malformation between 1998 and 2003, 13 had clinically detected scoliosis. Of these, ten had no other structural spine abnormality, which could influence the natural history of scoliosis and were included in this study. RESULTS: In all but one patient, syringomyelia improved significantly after cranio-vertebral decompression. Of the ten patients, eight had levoscoliosis (left convexity), all single curves, and two had curves to the right (both double curves). Six patients did not require corrective scoliosis surgery after cranio-vertebral decompression. The mean Cobb angle for those not requiring scoliosis correction was 29 degrees in contrast to a mean of 76 degrees for those requiring correction [p=0.001, one-way analysis of variance (ANOVA)]. The mean age of patients requiring corrective surgery was 158 months against 125 months for those not requiring correction (p=0.084, one-way ANOVA). These findings were confirmed by multivariate analysis, which also confirmed that symptom duration, syrinx length and site were not significant in predicting the need for corrective surgery following cranio-vertebral decompression. CONCLUSIONS: Cranio-vertebral decompression for Chiari I may prevent the need for corrective scoliosis surgery when performed before the age of ten and below a Cobb angle of 30 degrees.

Adolescent↗

Molecular analysis of congenital scoliosis: a candidate gene approach.

The etiology of congenital scoliosis is largely unknown. The severe vertebral disorder, spondylocostal dysostosis type 1, is associated with a homozygous delta-like 3 (DLL3) mutation. Scoliosis has been observed in a heterozygous DLL3 carrier, raising the possibility of its involvement in congenital scoliosis. We present the first molecular study of congenital scoliosis by analysis of the candidate gene DLL3 and demonstrate one novel missense variant. However, no novel or previously described mutations are present in our cohort, indicating that DLL3 mutations may not be a major cause of congenital scoliosis. Additionally, we have evaluated patients with congenital scoliosis not diagnosed with a known syndrome and identified a significant number of associated renal and cardiac anomalies and familial incidence of idiopathic scoliosis in this group.

Abnormalities, Multiple↗

Progressive scoliosis in central core disease.

Central core disease (CCD) is a rare congenital myopathy with autosomal dominant inheritance. Here, we report on two cases of progressive scoliosis in CCD, pointing out the value of a muscle biopsy to establish the correct diagnosis. The first case involves a 13-year-old boy with severe progressive scoliosis and joint contractures. The patient was initially diagnosed with arthropgryposis multiplex congenita. The second case involves a 45-year-old man with severe scoliosis that had slowly progressed over the years. Both patients suffered from unexplained muscle weakness and severe restriction of pulmonary function. The correct diagnoses were established through muscle biopsies taken from the paravertebral musculature during scoliosis surgery. Correction of the spinal deformities was achieved through posterior instrumentation in both patients, with prior anterior release in one patient. Although scoliosis is a common feature in CCD, the correct diagnosis can be missed in scoliosis patients. Therefore, we recommend a muscle biopsy in patients with scoliosis, unexplained muscle weakness and multiple joint problems.

Adolescent↗

Preoperative evaluation of activity and function in patients with paralytic scoliosis.

Preoperative evaluation of patients with paralytic scoliosis should take into account the consequences of surgery on the every day life of the patient. However, the parameters that are customarily used in these operations relate only to very narrow measures such as the angle of scoliosis or kyphosis. The aim of this study was to introduce a set of instruments appropriate for measuring both function and activities in paralytic scoliosis patients. The study took as its starting point the WHO International Classification of Impairments, Disabilities and Handicaps (ICIDH), in which an activity is described at the level of the individual and function at the level of the organ. A consecutive series of 100 paralytic scoliosis patients with 18 different diagnoses were evaluated preoperatively with a set of instruments that had been specially developed at Linköping hospital, in which the variables are classified according to the system used in the ICIDH. The set of instruments included general information and evaluation of activities and function--sitting, balance, weight distribution to sitting surface, angle of scoliosis, reaching, pain estimation, activities of daily living (ADL) Barthel and ADL Klein and Bell, care given, time spent resting, and seating supports). The results showed that patients with paralytic scoliosis constitute a heterogeneous group in activities and function. Even when the patients were grouped into four subgroups according to the Scoliosis Research Society Classification, they remained very heterogeneous. However, reaching, Klein and Bell Activities of Daily Living and pain could only evaluate patients who could understand verbal instructions. In those who could not, assessment relied more heavily on measures of function and level of dependence. It was concluded that the choice of assessment must be guided by the patient's ability to understand verbal instructions irrespective of his/her disorder. It is important to use the three levels--impairments, disabilities and handicaps-- in order to focus on the different outcomes in the two groups with respect to the patient's total situation.

Activities of Daily Living↗

Activities and functional assessment 1 year after spinal fusion for paralytic scoliosis.

The results of spinal fusion in patients with paralytic deformities are usually presented as the correction of the deformity. When evaluating the surgical results in such patients, it is advantageous to classify the patients into subgroups because of the varying dysfunction and disabilities. The aim of this study was to evaluate the effect of spinal fusion in patients with paralytic scoliosis in relation to function in terms of Impairments, activities in terms of Disabilities, and dependence in terms of Handicaps 1 year postoperatively, with emphasis on subgroups. A total of 94 patients with paralytic scoliosis and 18 different diagnoses were evaluated 1 year after surgery. The patients were classified according to whether or not they could understand verbal instructions. The patients were also grouped according to the Scoliosis Research Society classification of diagnoses. A set of instruments was used with the variables classified according to WHO's International Classification of Impairments, Disabilities, and Handicaps (ICIDH). This consists of the patient's/relative's motives for surgery, persistent skin discoloration, ambulating or use of wheelchair, use of a brace, sitting balance, weight distribution on a sitting surface, angle of scoliosis, reaching, pain estimation, activities of daily living (ADL) Klein and Bell, care given, time spent resting, and seating supports. The set of instrument also included a follow-up questionnaire comprising 12 different areas, where the patients/relatives assessed the results of surgery. The study showed that spinal fusion in paralytic scoliosis led to showed improvements in the whole group of patients at the Impairment level in the angle of scoliosis, sitting balance, weight distribution, and reduced number of patients with persistent skin discoloration, and at the Handicap level in reduced time for resting during the day, reduced number of seating supports in the wheelchair, and in the use of a brace. Most of the parameters were unchanged. The results in the subgroups were almost the same as in the whole group, although pain and reaching at the Impairment level, and ADL at the Disability level, could not be measured in the patients who were unable to understand verbal instructions. The subjectively assessed results showed that seating posture was ranked positively irrespective of the motive for surgery. The study showed that the patients with paralytic scoliosis maintained or improved their function and level of independence in terms of Impairment and Handicap 1 year postoperatively. The subjective results assessed by the patients/relatives also showed a positive outcome of surgery. Weight distribution on a seating surface was improved, but still uneven, and with respect to better sitting balance and increasing time sitting in a wheelchair, this can involve a risk for pressure sores and needs further investigation. When introducing outcomes including the Disability level, one must take the importance of homogeneity in the groups into consideration.

Activities of Daily Living↗

A new MRI technique for imaging scoliosis in the sagittal plane.

The purpose of the present study was to introduce a new magnetic resonance imaging (MRI) procedure showing the whole spine in a coronal and sagittal plane, and to study the assessment of sagittal Cobb angle measurements using this technique. Prospectively we studied 32 patients (average age 14.8 years) with idiopathic scoliosis (mean thoracic Cobb angle 33 degrees on radiograph) and 18 patients (average age 14.5 years) without scoliosis. The MRI investigation was carried out in a standard supine position. The cervical and upper thoracic spine and the lower thoracic and lumbar spine were measured on a 1.5-T Gyroscan ACS-NT Powertrak 6000 system. An algorithm was developed to combine the results of the cranial and caudal scans into a coronal and a sagittal image of the whole spine (MR total spine imaging). Measurement of the sagittal Cobb angle conducted ten times by four independent investigators revealed an intraobserver variance of 1.6 degrees and an interobserver variance of 1.8 degrees. In the group with scoliosis the mean sagittal Cobb angle from T4 to T12 was 12 degrees (range -3 degrees to 24 degrees) and in the group without scoliosis 22 degrees (range 16 degrees to 30 degrees), which was a significant difference. MR total spine imaging makes it possible to image scoliosis in the sagittal plane. On these MR projections, idiopathic thoracic scoliosis was identified by a reduced sagittal Cobb angle. MR total spine imaging would allow monitoring of scoliosis in the sagittal plane, which can reveal relevant clinical data without radiation exposure.

Adolescent↗

[Sagittal Cobb-angle measurements in scoliosis with MRI whole spine imaging].

AIM: A newly developed MR procedure allows imaging of the whole spine in coronal and sagittal planes. We studied the use of total spine MR imaging in measuring sagittal Cobb angles in scoliosis. METHOD: 64 patents with idiopathic scoliosis (mean age 18.1 years, 35 thoracic, 20 double major, and 9 lumbar curves) and 27 patients without scoliosis were consecutively examined. The MR images were acquired in the supine position. The sagittal Cobb angles were measured between T4-T12 and between T12-L5. RESULTS: For the group of the thoracic and double major scoliosis the mean sagittal Cobb angle (T4-T12) was 13 degrees and for the group without scoliosis 23 degrees, which was a significant difference (p < 0.01, Mann Whitney-U-test). There was a negative correlation between the sagittal Cobb angles (T4-T12) and the lateral, thoracic curves. The mean sagittal Cobb angle (T12-L5) of the group with lumbar and double major curves was 35 degrees, which was not a significant difference when compared to 37 degrees of the non-scoliotic group. CONCLUSION: Using total spine MR imaging the lordotic aspect of the thoracic deformation in scoliosis can be reliably measured. Because of the absent radiation exposure the sagittal MR reconstructions could be used as an additional imaging in monitoring scoliosis.

Adolescent↗

[Early results of operative treatment of scoliosis using the anterior derotation spondylodesis technique (VDS-Zielke) (author's transl)].

The paper presents early results of 25 surgical procedures for scoliosis using the instruments and implants of the anterior derotation spondylodesis (VDS-Zielke). The series included 10 dorsal, 3 dorso-lumbar and 12 lumbar curves, whereby up to 8 vertebral bodies were fused. The average postop. follow-up time was 25 months (8--45 months). The average degree of correction was 51 p.c. Emphasis is layed on the fact that aetiology and severity of the scoliotic curves was extremely heterogeneous. Besides excellent corrective potentials of the VDS for scoliosis, this technique offers possibilities for correction of cyphosis or lordosis as well. The results are described and documented in detail. The author puts much emphasis in describing special techniques for correcting lordosis or cyphosis as well as scoliosis in the same patient. The analysis of the results presented shows good indications for VDS in dorso-lumbar and lumbar scoliosis, congenital scoliosis of the dorso-lumbar area and thoracic lordo-scoliosis. For the indications outlined the possibilities of the VDS are superior to any other operative technique for scoliosis (Dwyer, Harrington). The rate of complications can be kept low through good selection of cases, excellent surgical technique and postoperative intensive care.

Adolescent↗

Genetics of scoliosis in chickens.

The genetics of an inherited form of scoliosis in chickens was studied to estimate the number of genes involved, whether they are autosomal or sex-linked, their degree of dominance and penetrance, and the heritability of this trait in this population. Expression of scoliosis and in the progeny was analyzed by radiographs of birds 12 weeks of age or older. Crosses between an inbred line selected for scoliosis expression (incidence of scoliosis - 89 percent) and a highly inbred line displaying normal spinal development provided data for genetic analyses. The incidence of expression of scoliotic parent line implicates three major autosomal, recessive genes. The significantly higher incidence of severe scoliosis found in the homogametic male sex is ascribed to a sex-influenced, on the scoliosis trait rather than to sex-linkage. Variation of expression observed in the scoliotic line is attributed to incomplete penetrance of the major genes, additive effects of minor modifying genes, and primarily to environmental effects. Because of the similarities in the expression of this disease in chickens and humans, the inheritance pattern determined for chickens may provide useful insights into that operating for so-called adolescent idiopathic scoliosis in humans.

Animals↗

Early detection of scoliosis. A neurological approach using the asymmetrical tonic neck reflex.

The purpose of this study was to determine the relationship between the asymmetrical tonic neck reflex (ATNR) and the occurrence of scoliosis in healthy children. One hundred eighty-four children, between the ages of 10 years, 5 months to 13 years, 10 months, who attended a rural elementary school were selected for the study. The quadruped reflex-inhibiting posture was used to assess the ATNR. A standard orthopedic screening tool was used for assessment of scoliosis. The occurrence of the ATNR in children tended to increase the rate of referral for further testing for scoliosis. In those children who were assessed for scoliosis using roentgenograms and who had a positive ATNR, a relationship occurred between the convex side of the curvature and the side of the ATNR. Results did not show significant differences in the occurrence of the ATNR or scoliosis in regard to sex. Based on the results of this study, the addition of the ATNR testing to scoliosis screening may prove beneficial in the early identification of scoliosis.

Adolescent↗