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Preliminary results and worst-case analysis of in patient scoliosis rehabilitation.

The purpose of this study was to assess the effectiveness of a scoliosis-specific rehabilitation programme as it is carried out in the Katharina Schroth Spinal Deformities Rehabilitation Center. Physiotherapy in the treatment of scoliosis patients is still regarded as ineffective since the study by the American Orthopedic Association in 1941, which showed that general exercises could not influence the natural history of scoliosis. However, specific exercise programmes were not known in the USA at that time. This preliminary study started in 1989 with the following inclusion criteria: (1) diagnosis of idiopathic scoliosis; (2) risser sign < 4; (3) no treatment other than physiotherapy; (4) first control after 1-3 years during repeated in patient treatment; (5) standing AP radiograph taken not more than 6 months before the first in patient treatment. A total of 181 scoliosis patients, with an average age of 12.7 years and an average angle of curvature of 27% according to Cobb, were included in this study. The average risser sign was 1.4, the average follow-up 33 months. The Cobb angle of the major curve was measured in a standardized way. The results of our preliminary study were compared to natural history as known from literature. For the worst-case analysis additionally a questionnaire was sent to the non-repeaters treated at our centre at the same time (1989 and 1990) as the patient sample described above, taking into account the same inclusion criteria for this patient sample except point 4. Results showed that progression as usually defined (increase in curvature of 5 degrees or more per year) has not been found in the preliminary study. The patient sample of this study was divided into different age groups and different groups of curve magnitude, for comparison with other studies. Additional to the patient sample of the preliminary study, 116 of the patients from the years 1989 and 1990 fulfilled the inclusion criteria of the preliminary study with the exception of point 4. These patients formed the questionnaire sample for the worst-case analysis showing that the progression rate of the 181 patients from the preliminary study and the 116 patients of the questionnaire sample together was still better than natural history even if all drop-outs were considered to be failures. The fact that there was no relative progression in our patients sample treated solely by physiotherapy (preliminary study), seems to show the effectiveness of the inpatient rehabilitation programme even in cases with a bad prognosis, severe angles of curvature and unfavourable curvature patterns. A worst-case analysis does not prevent this conclusion, even if all dropouts from the non-repeaters group were considered as failures.

Adolescent↗

Scoliosis in pediatric Chiari malformations without myelodysplasia.

A prospective study was undertaken in 1985 to better understand how the surgical manipulation of hindbrain herniation affected abnormal spinal curvature. Eleven patients under 16 years of age with Chiari malformation (not associated with myelodysplasia) and scoliosis of at least 15 degrees were studied. The mean curve angle at the time of original treatment was 29 degrees, with the convexity to the right in seven patients. The curvature was rapidly progressing in four patients. The most common presenting signs were myelopathy and weakness. Investigative procedures included spine radiographs with the patient standing and magnetic resonance (MR) imaging of the brain, spinal cord, and craniovertebral junction. Eight children had associated hydrosyringomyelia. Surgical intervention consisted of a dorsal posterior fossa decompression in all patients and a transoral ventral decompression of the cervicomedullary junction in five. All patients were followed at 3, 6, and 12 months, and at yearly intervals thereafter with clinical evaluations, spine radiographs in the standing position, and postoperative MR imaging. The mean follow-up period was 35 months. The scoliosis improved in eight patients, stabilized in one, and progressed in two. Only one child required postoperative spinal fusion and instrumentation for progression of scoliosis. Hematomyelia or hematobulbia was associated with persistent scoliosis in two patients. The presence of hydrosyringomyelia and bone erosion did not preclude curve improvement. All patients under 10 years of age had resolution of their scoliosis, despite preoperative curves of more than 40 degrees. These findings emphasize the importance of early surgical intervention, with the restoration of normal cerebrospinal fluid dynamics at the craniovertebral junction in children with symptomatic Chiari malformations.

Adolescent↗

Neurosurgical causes of scoliosis in patients with myelomeningocele: an evidence-based literature review.

OBJECT: Scoliosis in children with myelomeningocele occurs frequently and is multifactorial in origin. The aim of this paper is to review the evidence regarding the causal association, if any, between isolated scoliosis and spinal cord tethering, Chiari malformation, or syringomyelia in this population. METHODS: A Medline search of the English-language literature from 1966 to 2002 was undertaken. All of the pertinent articles that were identified underwent careful review to determine the strength of the evidence supporting the claim that isolated scoliosis in children with myelomeningocele is related to spinal cord tethering, Chiari malformation, or syringomyelia. The strength of the evidence was graded using the Oxford Centre for Evidence-Based Medicine Levels of Evidence (May 2001). A survey was also sent to practicing pediatric neurosurgeons and the returned responses were evaluated. CONCLUSIONS: All available studies were classified as Level 4 studies (case series and flawed cohort and case-control studies). Based on the strength of the available data, there may be an association between spinal cord tethering and scoliosis, particularly in those patients with upper lumbar lesions and spinal curves less than 45 degrees. There is little evidence to support a causal relationship between scoliosis and Chiari malformation or syringomyelia in this population.

Adolescent↗

A study on platelet function in idiopathic scoliosis.

Because platelets and muscle cells share the same contractile proteins--actin and myosin--platelets may serve as a model for muscle research. To study the functional abnormalities and ultrastructural changes of platelets and to determine whether or not abnormalities in muscle contractile proteins and collagen play an important role in the pathogenesis of idiopathic scoliosis, the bleeding time, the platelet aggregation test, and the titers of platelet plasminogen activator inhibitors were measured and the electron microscopic findings were examined in 52 idiopathic scoliosis patients aged 7 to 28 years and in 49 normal individuals aged 8 to 38 years as a control group. We found no statistically significant difference between the two groups in the bleeding time, the platelet aggregation test, and the titers of platelet plasminogen activator inhibitors. In the electron microscopic findings, no specific abnormalities were found in platelets of idiopathic scoliosis patients. We concluded that idiopathic scoliotic patients have normal morphology and function of platelets, and there is no important role of contractile proteins in the pathogenesis of idiopathic scoliosis. In addition, since the bleeding time was in the normal range, no evidence of subendothelial collagen dysfunction was found in the idiopathic scoliosis patients.

Adolescent↗

[Screening for adolescent idiopathic scoliosis: is current knowledge sufficient to support its use?].

OBJECTIVE: The aim of this study was to re-examine the basis of screening for adolescent idiopathic scoliosis. PATIENTS AND METHODS: Retrospective analysis of scoliosis screening of patients with a positive test was performed. The screening was performed in children between 10 and 15 years by visual inspection of the back and the Adams forward-bending test. RESULTS: In 31.6% of the children, the screening test detected the presence of abnormal findings on initial physical examination. 19% of the total population was sent to a traumatology service. In 11% the diagnosis was confirmed (16 scoliosis, 12 of them idiopathic and 4 due to other conditions), PPV: 58%. In 8% of the adolescents exercise was recommended. In 1.2% braces were recommended. There were no differences in prevalence between sex, but the height of the children with scoliosis was greater (p < 0.001). CONCLUSIONS: Because of the potential severe adverse effects and because the studies of the efficacy of the screening are not reliable, the U.S. Preventive Services Task Force, Canadian Task Force and the Program de Actividades Preventivas y Promoción de la Salud have excluded the routine screening of the asymptomatic adolescent for idiopathic scoliosis.

Adolescent↗

[Idiopathic scoliosis. Definition--pathology--classification--etiology].

Scoliosis is a symptom, it is not a disease. Mechanically, scoliosis is a torsion of the basic elements of the spine developed according to an helicoidal axis. Scoliosis is called idiopathic when no inductive disorder like paralysis, congenital malformation, or metabolic disease, have been established. Idiopathic scoliosis arises during infancy or childhood and gives spontaneously as big deformity as earlier started. The thoracic location gives not only a rib hump, but also respiratory impairment when a severe deformity is obtained. Lumbar location gives mainly imbalance and pain. Etiology is still unknown, but recent research lead to a neuromuscular disorder in relation with the neuro-transmitter involved with the bipedal condition with very often a genetic input. With time degenerative lesions of the discal and ligaments components increase the deformity established during childhood and adolescence, but also these degenerative disorders can create scoliosis deformity only in adulthood arising on normally aligned spines.

Child↗

Adolescent idiopathic scoliosis. Is low melatonin a cause?

BACKGROUND: Although the cause of adolescent idiopathic scoliosis remains unclear, pinealectomy is followed by the development of scoliosis in chickens. Melatonin is the only hormone secreted by pineal gland. OBJECTIVE: To assess serum melatonin levels in adolescents with idiopathic scoliosis. METHODS: Serum melatonin was assayed once, at 12:00 hours, in each of 20 adolescents with idiopathic scoliosis and 10 age-matched siblings. In the patient group, mean age was 14.3 years and the mean Cobb angle was 54.5 degrees (range, 20 degrees-110 degrees). Mean age in the control group was 14 years. RESULTS: Serum melatonin was significantly lower in the patients (mean, 7.7 pg/mL; range, 4 to 13 pg/mL) than in the controls (mean, 29.9 pg/mL; range, 19.3 to 46 pg/mL) (P < 0.00001). The 12 surgically treated patients had a mean melatonin level of 8.1 pg/mL, versus 7.2 pg/mL in the eight other patients (nonsignificant P and regression coefficient values). Serum melatonin was not significantly correlated with the Cobb's angle (regression coefficient, 0.18; P < 0.44). CONCLUSION: Our data suggest that serum melatonin levels may contribute to the pathogenesis of idiopathic scoliosis.

Adolescent↗

Unilateral absence of the clavicle with rapidly progressive scoliosis in an 8-year-old.

We report a possible association of unilateral absence of a clavicle with rapidly progressive scoliosis. Cleidocranial dysplasia (CCD) is an autosomal dominant disorder that is characterized by defective bone formation. The clavicle, pelvis, and skull are the most commonly affected bones. A review of the literature found two cases of CCD and scoliosis. Unilateral absence of the clavicle in association with rapidly progressing scoliosis has not been previously reported. Review of the patient's charts and radiographs from age 8 to 17 years, 5 years after treatment with posterior spinal instrumentation is presented, together with a review of the literature. Our patient initially presented without any spinal deformity until age 9, when she had a 10 degree curve between C-8 and L-T. Eighteen months later, the curve progressed to 52 degrees, Risser 1. Associated anomalies include posterior-element hypoplasia of the thoracic spine and posterior fusion of C4-6. She was treated with posterior spinal instrumentation from C-8 to L-4 without complications. Correction was maintained at 5-year follow-up. There may be an association between unilateral absence of the clavicle and rapid progression of scoliosis in immature children. We hypothesize that the asymmetrical influence of the unilateral absent clavicle may have played a causative role in her rapidly progressive scoliosis.

Cervical Vertebrae↗

Surgical management of paralytic scoliosis in myelomeningocele.

A retrospective analysis of 54 patients with paralytic scoliosis due to myelomeningocele, who underwent surgical treatment, was performed. The aim of this study was to compare different surgical techniques and to identify clinical parameters influencing primary and midterm results. Three surgical techniques were used: 1) group I, posterior fusion/instrumentation; 2) group II, anterior fusion/no instrumentation combined with posterior fusion/instrumentation; and 3) group III, anterior and posterior fusion/instrumentation. Average age at surgery was 13.1 years. A preoperative scoliosis angle of 90 degrees [interquartile range (25th-75th percentile) (IQR), 76-106 degrees] was primarily reduced to 38 degrees (IQR, 30-50 degrees). At final follow-up (mean, 3.3 years), correction deteriorated to 44 degrees (IQR, 38-65 degrees). The group III procedure resulted in a better midterm correction of scoliosis compared with group I (P = 0.02). The extension of anterior fusion correlated with primary and midterm correction of scoliosis (P < 0.03). Patients with a thoracic level of paralysis had a higher relative loss of correction compared with patients with a lumbar level (P < 0.06). This finding can be attributed mostly to group I patients (P = 0.011). Hardware complications occurred in 16 patients (30%). Relative loss of correction among these patients was high (P < 0.01) and relative midterm correction low (P = 0.001). We recommend anterior and posterior fusion, each with instrumentation for the treatment of paralytic scoliosis in myelomeningocele. In patients with a thoracic level of paralysis, the two-stage procedure is mandatory to reduce the risk of hardware complications and subsequent major loss of correction.

Female↗

Infantile idiopathic scoliosis. Causative factors, particularly in the first six months of life.

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.

Female↗

[Possible role of the pineal gland in the pathogenesis of idiopathic scoliosis. Experimental and clinical studies].

The unexpected finding in 1959 by Marie-Jeanne Thillard that pinealectomy in young chickens gives way to spinal deformities was confirmed by the authors. In another experiment they found that injected melatonine to the chick at adequate dose and at the same time as surgery, lessen or even totally prevents the occurrence of deformities. On the other hand, at too low dose or delayed after pinealectomy melatonine injection, may not prevent the deformity which will be persisting or even increasing. In a subsequent series of experiments on the rat, pinealectomy results in decreasing the plasmatic amount of melatonine as well as giving way to spinal deformities. The nature of these deformities observed here is dependent on the stature between of the animal. The normal quadrupede rat develops after pinealectomy a standard scoliosis. Inversely the scoliotic deformity occurs when the animal has been forced to a bipede condition, which may be achieved by removing its forelimbs when baby, then forcing it to stand and remain in erect posture by high enough feeding. Melatonine depressing and erect position are in two conditions, when associated, likely to give way to experimental scoliosis. In human, a low nycthemeral level of plasmatic melatonine is correlated with progressive scoliosis. The level of platelets calmoduline, when is normally modulated by melatonine, has been proved by Kindsfater to be increased in progressive scoliosis. Then raises the hypothesis that human idiopathic scoliosis may be due to an inherited disorder of neuro-transmitters from neuro-hormonal origin, associated with bipedal condition, where an horizontal localized neuro-muscular imbalance starts and produces the scoliotic deformity of the fibro-elastic and bony structures axial spinal pilar.

Animals↗

Adolescent idiopathic scoliosis: a new classification to determine extent of spinal arthrodesis.

BACKGROUND: The lack of a reliable, universally acceptable system for classification of adolescent idiopathic scoliosis has made comparisons between various types of operative treatment an impossible task. Furthermore, long-term outcomes cannot be determined because of the great variations in the description of study groups. METHODS: We developed a new classification system with three components: curve type (1 through 6), a lumbar spine modifier (A, B, or C), and a sagittal thoracic modifier (-, N, or +). The six curve types have specific characteristics, on coronal and sagittal radiographs, that differentiate structural and nonstructural curves in the proximal thoracic, main thoracic, and thoracolumbar/lumbar regions. The lumbar spine modifier is based on the relationship of the center sacral vertical line to the apex of the lumbar curve, and the sagittal thoracic modifier is based on the sagittal curve measurement from the fifth to the twelfth thoracic level. A minus sign represents a curve of less than +10 degrees, N represents a curve of 10 degrees to 40 degrees, and a plus sign represents a curve of more than +40 degrees. Five surgeons, members of the Scoliosis Research Society who had developed the new system and who had previously tested the reliability of the King classification on radiographs of twenty-seven patients, measured the same radiographs (standing coronal and lateral as well as supine side-bending views) to test the reliability of the new classification. A randomly chosen independent group of seven surgeons, also members of the Scoliosis Research Society, tested the reliability and validity of the classification as well. RESULTS: The interobserver and intraobserver kappa values for the curve type were, respectively, 0.92 and 0.83 for the five developers of the system and 0.740 and 0.893 for the independent group of seven scoliosis surgeons. In the independent group, the mean interobserver and intraobserver kappa values were 0.800 and 0.840 for the lumbar modifier and 0.938 and 0.970 for the sagittal thoracic modifier. These kappa values were all in the good-to-excellent range (>0.75), except for the interobserver reliability of the independent group for the curve type (kappa = 0.74), which fell just below this level. CONCLUSIONS: This new two-dimensional classification of adolescent idiopathic scoliosis, as tested by two groups of surgeons, was shown to be much more reliable than the King system. Additional studies are necessary to determine the versatility, reliability, and accuracy of the classification for defining the vertebrae to be included in an arthrodesis.

Adolescent↗

Dynamic measurement of axial vertebral rotation and rotational flexibility in scoliosis by flouroscopic method.

The Pedriolle torsion meter is an established method of vertebral rotation assessment in scoliosis. However, the assessment of scoliosis by this method is static and indirect. The objective of this study is to compare the accuracy of a direct method of assessing scoliosis rotation by fluoroscopy compared to the Pedriolle torsion meter. Secondly, to determine that vertebral body rotation changes with supine posture compared to erect position. Eight volunteers with idiopathic scoliosis were assessed for the apical vertebral rotation with this method and the Pedriolle torsion meter. These patients were also assessed in the supine and erect position with the fluoroscopic method to determine if the apical vertebral rotation would change with posture. The mean Cobb angle of the curves was 62.8 degrees (range 45 degrees to 86 degrees). The mean apical vertebral rotation in a standing position was assessed to be 21.5 degrees by Pedriolle torsion meter and 29 degrees by the fluoroscopic method. This difference was not statistically significant by the student t-test. In most patient, the rotation of vertebrae improved by a varying degree ranging from none to 24 degrees in the supine position. In conclusion, the fluoroscopic method is an alternate mean of measuring vertebrae rotation in idiopathic scoliosis, with comparable accuracy to the Pedriolle torsion meter method. The amount of vertebral rotation changes with posture of the patient.

Adolescent↗

Sagittal profiles of the spine in scoliosis associated with an Arnold-Chiari malformation with or without syringomyelia.

The sagittal spine of children with Arnold-Chiari I malformation with or without syringomyelia and associated scoliosis (ACS) has been poorly studied. A retrospective review of scoliosis secondary to ACS from three centers was undertaken. Sagittal and coronal plane variables were measured from standing radiographs. There were 30 ACS children (19 girls, 11 boys) with an average curve of 50 +/- 20 degrees and age of 11.2 +/- 3.2 years. Syringomyelia was present in 26 (87%). The scoliosis was thoracic in 25, thoracolumbar in 3 and lumbar in 2; 18 curves were right and 12 were left. A positive correlation was noted between cervical lordosis (CL) and thoracic kyphosis (TK). The 30 children with ACS scoliosis were compared with 26 children with adolescent idiopathic scoliosis (AID). The ACS group had more left curves (40% vs. 0%, P = 0.0002), more boys (37% vs. 8%, P = 0.01), and was younger (11.3 +/- 3.7 years vs. 14.2 +/- 1.8 years, P = 0.004). TK and CL were increased in ACS (TK: 40 +/- 13 degrees vs. 30 +/- 13 degrees, P = 0.005; CL: 16 +/- 21 degrees vs. -5 +/- 12 degrees, P < 0.0001). The ACS and AID groups were subdivided by CL >0 degrees and <0 degrees. In the ACS group, 19% (5 of 21) had CL <0 degrees, in the AID group 77% (20 of 26) had CL <0 degrees (P = 0.0001). When cervical lordosis is >0 degrees or thoracic kyphosis is >40 degrees (Cobb method), the clinician should strongly suspect the presence of an Arnold-Chiari I malformation with or without syringomyelia.

Adolescent↗

Management of scoliosis and syringomyelia in children.

Scoliosis is often a characteristic of an underlying syrinx. Surgical treatment, in particular instrumentation, of scoliosis in a child with an unrecognized syrinx may be associated with an increased risk of neurologic complications. The effect of syrinx treatment on scoliosis is yet to be clearly determined. Since 1976, nine children with scoliosis and a syrinx have been treated at a major pediatric referral center. Follow-up averaged 4 years 9 months. The average increase in curve size was 12 degrees (range 2 degrees -43 degrees ). The syrinx was treated operatively in five children. One child, who underwent foramen magnum decompression, required surgical stabilization of the spine. No neurologic complications were encountered. A compilation of reported cases in the literature was performed and analyzed. This analysis of 98 cases revealed that treatment of the syrinx resulted in no further progression of the scoliosis in 35 of 80 (48%) cases in which the curve history was reported. Surgical stabilization of the curve was required in 27 (31%) cases without neurologic complications.

Adolescent↗

Scoliosis in velo-cardio-facial syndrome.

Velo-cardio-facial (VCF) syndrome caused by 22q11.2 deletion is a common genetic condition with variable features including congenital heart defects, facial anomalies, palatal anomalies, and cognitive problems. Besides the main characteristics, various other anomalies have been noted, including musculoskeletal problems. Scoliosis has been mentioned but not emphasized as a common feature. The authors evaluated 20 patients for scoliosis and connective tissue anomalies who were consecutively diagnosed with VCF syndrome and 22q11.2 deletion at their clinics. The authors describe three children with VCF syndrome who had significant scoliosis and connective tissue findings. Two of these patients were initially referred to genetics for possible Marfan syndrome. The authors suggest that scoliosis should be considered a relatively common finding in patients with 22q11.2 deletion, and they should be monitored for this problem. In addition, 22q11.2 deletion should be among the diagnostic considerations in patients with unexplained scoliosis and developmental delay.

Abnormalities, Multiple↗

[Surgical treatment of degenerative scoliosis].

OBJECTIVE: To investigate the techniques and indications of treatment of degenerative scoliosis. METHODS: The clinical data of 45 degenerative scoliosis patients, 18 males and 27 females, aged 41 - 77, all suffering from severe low back pain, were analyzed. Twenty of them underwent posterior lumbar interbody fusion with pedicle screws combined with correction of scoliosis. Sixteen patients underwent depression of vertebral canal and pure internal fixation and fusion of pedicle. Eight patients underwent pure depression, such as laminectomy or incision of intervertebral foramen. The corrective effects of scoliosis, condition of intervertebral fusion, and complications were observed. RESULTS: All operations were performed successfully and all the patients made a remarkable recovery. During the 13 months' follow-up, 88% of the patients showed remarkable relief from pain and claudication and improved life quality. No loss in correction angle and height of disc space was found among the patients who had undergone internal fixation and interbody fusion. The residual symptom of low back pain was more manifest in the patients who had not undergone internal fixation than in those who had. CONCLUSION: Posterior lumbar interbody fusion with pedicle screws is safe and effective in the treatment of severe degenerative scoliosis.

Adult↗

[Scoliosis and congenital cardiopathies].

The authors have followed up 26 children suffering from severe scoliosis associated with congenital heart disease up to the end of puberty. The curve was usually a very severe idiopathic scoliosis developing early and requring surgical treatment. Two types may be distinguished: I. Scoliosis without excessive surgical risk, in patients in whom the heart disease is not associated with cyanosis or where the heart condition has already been treated surgically and in which there are no clinical, radiological or electrical signs of cardiac failure. 2. Scoliosis with considerable surgical risk because of heart disease with cyanosis not treated surgically, or with signs of heart failure or pulmonary hypertension. In such cases, the surgical treatment of the scoliosis is likely to endanger life.

Abnormalities, Multiple↗