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Influence of surgical treatment of scoliosis in children with spina bifida on ambulation and motoric skills.

The influence on motoric skills of surgical treatment of scoliosis in 14 children with myelomeningocele was studied. Fifty percent of the children had increased flexion contractures of the hips at follow-up, and all but one patient had impaired motor ability. There was no influence on activities of daily life, but 57% of children had lost some of their ambulation capacity. Postoperative physiotherapy is highly advisable. Intensive attempts to treat these children conservatively to prevent scoliosis progression is suggested.

Activities of Daily Living↗

Congenital scoliosis in a quarter horse filly.

A 4-week old Quarter Horse filly was evaluated for abnormal gait and lateral deviation of the cervical spine. Physical examination findings prompted radiographs and computed tomography of the thoracic vertebral column which revealed hypoplasia of several thoracic vertebral bodies and resultant scoliosis of the thoracic vertebral column and deviation of the left and right hemithoraces and associated ribs. Collectively, radiography and computed tomography provided an accurate description of the vertebral malformations resulting in scoliosis in this foal.

Animals↗

Simulation of progressive deformities in adolescent idiopathic scoliosis using a biomechanical model integrating vertebral growth modulation.

While the etiology and pathogenesis of adolescent idiopathic scoliosis are still not well understood, it is generally recognized that it progresses within a biomechanical process involving asymmetrical loading of the spine and vertebral growth modulation. This study intends to develop a finite element model incorporating vertebral growth and growth modulation in order to represent the progression of scoliotic deformities. The biomechanical model was based on experimental and clinical observations, and was formulated with variables integrating a biomechanical stimulus of growth modulation along directions perpendicular (x) and parallel (y, z) to the growth plates, a sensitivity factor beta to that stimulus and time. It was integrated into a finite element model of the thoracic and lumbar spine, which was personalized to the geometry of a female subject without spinal deformity. An imbalance of 2 mm in the right direction at the 8th thoracic vertebra was imposed and two simulations were performed: one with only growth modulation perpendicular to growth plates (Sim1), and the other one with additional components in the transverse plane (Sim2). Semi-quantitative characterization of the scoliotic deformities at each growth cycle was made using regional scoliotic descriptors (thoracic Cobb angle and kyphosis) and local scoliotic descriptors (wedging angle and axial rotation of the thoracic apical vertebra). In all simulations, spinal profiles corresponded to clinically observable configurations. The Cobb angle increased non-linearly from 0.3 degree to 34 degrees (Sim1) and 20 degrees (Sim2) from the first to last growth cycle, adequately reproducing the amplifying thoracic scoliotic curve. The sagittal thoracic profile (kyphosis) remained quite constant. Similarly to clinical and experimental observations, vertebral wedging angle of the thoracic apex progressed from 2.6 degrees to 10.7 degrees (Sim1) and 7.8 degrees (Sim2) with curve progression. Concomitantly, vertebral rotation of the thoracic apex increased of 10 degrees (Sim1) and 6 degrees (Sim2) clockwise, adequately reproducing the evolution of axial rotation reported in several studies. Similar trends but of lesser magnitude (Sim2) suggests that growth modulation parallel to growth plates tend to counteract the growth modulation effects in longitudinal direction. Overall, the developed model adequately represents the self-sustaining progression of vertebral and spinal scoliotic deformities. This study demonstrates the feasibility of the modeling approach, and compared to other biomechanical studies of scoliosis it achieves a more complete representation of the scoliotic spine.

Adolescent↗

Scoliosis in chickens: responsiveness of severity and incidence to dietary copper.

The severity and incidence of spinal lesions were manipulated in a line of chickens susceptible to scoliosis by varying their dietary intake of copper. A decrease in expression of the lesion was related to increased intake of copper. The change in expression, however, appeared to be related only indirectly to the defects in collagen cross-linking, maturation, and deposition known to be associated with dietary copper deficiency. Thus, a dietary constituent in the range of normal intakes may act as an environmental factor in the expression of scoliosis.

Animals↗

Virus-like particles in paraspinal muscle in scoliosis.

Biopsy material from the skeletal muscle (paraxials) of 21 patients with scoliosis was examined by light and electron microscopy. Virus-like particles, 17 nm in diameter with a crystalline structure, were identified in the skeletal muscle fibres of four patients. Associated changes in the sarcoplasm included swelling of mitochondria, presence of lipid droplets, and vesicular structures. Serological studies and culture for virus isolation gave negative results. An excess of lipid (predominantly in type 1 fibres) was noted in the skeletal muscle of several other cases. The significance of these findings is obscure, but the morphology of the paraxial muscles of patients with scoliosis and controls is currently being investigated in greater detail.

Adolescent↗

School screening for scoliosis: cohort study of clinical course.

A visual examination of 1764 Oxford schoolchildren for scoliosis was followed by low-dose radiography of the spine in those who showed evidence of asymmetric body topography; radiography was repeated a year later to assess progression. Forty-four children had curves of 10 degrees or more. Two had a congenital abnormality and the remaining 42 were classified according to the type of curve: sacral tilt (compensatory), spinal (idiopathic), or combined (sacral tilt and spinal). Progression occurred in 6 (14%) children, none of whom had only a sacral tilt. These results suggest that only by measuring sacral tilt can benign compensatory curves be differentiated from true idiopathic scoliosis.

Adolescent↗

A study of pulmonary artery pressure, electrocardiography, and mechanocardiography in thoracic scoliosis.

Cardiac catheterisation was carried out in 40 patients with thoracic scoliosis in order to measure the pulmonary artery pressure. Statistical correlations were calculated between these results and the electrocardiographic and mechanocardiographic findings determined on a separate occasion. The pulmonary artery pressure was normal in 72% of subjects. It was inversely correlated with arterial oxygen tension but not with the aetiology, severity, or age at onset of the scoliosis. The accuracy of electrocardiography and mechanocardiography in predicting the pulmonary artery pressure was assessed. The closet correlates were found to be a tall P wave in lead II or III and a prolonged interval between pulmonary valve closure and tricuspid valve opening.

Adolescent↗

Pulmonary function in adolescents with mild idiopathic scoliosis.

Spirometric indices, lung volumes, maximum voluntary ventilation, and maximum inspiratory and expiratory pressures were measured in 44 adolescents with mild idiopathic scoliosis (spinal curvature less than 30 degrees). All were symptom free, but six (13.6%) showed a restrictive defect with forced vital capacity less than 80% of predicted. In 12 subjects (27.3%) maximum voluntary ventilation was reduced to less than 80% of predicted normal. Forced vital capacity was significantly correlated with maximum inspiratory pressure and with maximum expiratory pressure, measures of respiratory muscle strength, but was not related to the degree of thoracic curvature. When maximum inspiratory pressure and forced vital capacity were corrected for differences in body size these variables remained positively correlated, most significantly in the girls. These data indicate that ventilatory function may be impaired in mild, idiopathic scoliosis and that the force developed by the respiratory muscles is a more important determinant of this impairment than the radiologically determined degree of spinal curvature.

Adolescent↗

Pulmonary restrictive effect of bracing in mild idiopathic scoliosis.

The use of bracing in the treatment of mild idiopathic scoliosis is controversial. A study of 33 adolescents showed that bracing significantly decreased lung volumes. Functional residual capacity was reduced by a mean of 26%, 18% of children showing a reduction of greater than 40%. The mean reduction in total lung capacity was 16% and in forced vital capacity 18%. This restriction of lung function by bracing might have a deleterious effect on lung growth or might impose an additional risk factor in the presence of other disorders, such as asthma and diaphragmatic weakness. The use of bracing in individuals with mild scoliosis should be judiciously reassessed.

Adolescent↗

An improved film cassette for scoliosis radiography.

An improved film cassette for scoliosis radiography was constructed by joining two smaller cassettes for use with two 35 x 35-cm radiographic films. The new cassette has five advantages over previous scoliosis cassettes: a) lighter weight for easier handling, b) better film-screen contact, c) easier marking for patient identification, d) easier film loading and unloading, and e) lower film cost.

Adolescent↗

Atypical idiopathic scoliosis: MR imaging evaluation.

The authors analyzed the clinical and imaging findings in 30 consecutive pediatric, adolescent, and young adult patients who underwent MR imaging because of atypical features of idiopathic scoliosis. Atypical clinical and plain radiographic features included early onset (n = 4), rapid progression (n = 19), pain (n = 17), other neurologic symptoms or signs (n = 12), associated syndromes (n = 4), a convex left thoracic or thoracolumbar curve (n = 18), a kyphotic component (n = 7), and pedicle thinning (n = 4). MR imaging demonstrated 17 abnormalities in 11 patients: lumbar disk protrusions (n = 1), patulous intradural space (dural ectasia) (n = 3), hydrosyringomyelia (n = 7), Chiari I malformation (n = 5), and cord astrocytoma (n = 1). Significant associations with abnormal MR imaging findings were shown for patients with pain, weakness, abnormal neurologic findings, and atypical curvatures. Furthermore, there was a striking association of convex left thoracic or thoracolumbar scoliosis with hydrosyringomyelia (six of seven cases). On the basis of these results, "atypical" spinal curvatures at radiography and "atypical" clinical features should prompt performance of additional diagnostic studies.

Adolescent↗

The scoliosis (sco) mouse: a new allele of Pax1.

We describe the spontaneous mutant mouse scoliosis (sco) that carries a new allele of Pax1 (un-i, undulated intermediate). The Pax1(un-i) allele is lacking the 5'-flanking region and exon 1 to 4 which is mapped to nt -2636 to -640 and -272 to 4271 of the Pax1 gene. Homozygous mice show a mild form of the known phenotypes of other Pax1 mutants. Adult mice have a lumbar scoliosis and kinky tails. In homozygous embryos the skeleton ossifies early, ossification centers of the vertebral bodies are fused with the ossification centers of the pedicles. Neural arches and spinous processes are underdeveloped but the pedicles and transverse processes are overdeveloped which is in contrast to other Pax1 mutants. In the scapula, the acromion is missing and the deltoid tuberosity of the proximal humerus is shortened and thickened. Among the inner organs the thymus development is affected. In late embryos, the thymus is small and thymocyte numbers are reduced. T-cell development from CD4- and CD8- double negative (DN) to CD4+ and CD8+ double positive (DP) is decelerated. The percentage of CD90+ cells is also reduced but in contrast to other Pax1 mutants no alteration of the expression level of the CD90 (Thy-1) could be found.

Animals↗

Anatomy of the extrinsic spinal muscles related to the deformities of scoliosis.

The spatial displacement of vertebrae in idiopathic scoliosis is not explicable by forces created by the muscles which act upon the spine only (intrinsic muscles). The trapezius and latissimus muscles are attached to the spinous processes and the upper limb. In keeping with Newton's third law, these muscles act on the spine as well as upon the upper extremity. The peculiarity of the vertebral anatomy, together with the direction of pull of these muscles, permits an explanation of the biomechanics of the development of 'idiopathic' scoliosis.

Biomechanical Phenomena↗

A comparison of the innervation characteristics of the lateral spinal ligaments between normal subjects and patients with adolescent idiopathic scoliosis.

Evidence is rapidly accumulating to suggest that general proprioceptive dysfunction might be a major contributing factor in the development of adolescent idiopathic scoliosis (AIS). The innervation of appropriate ligaments which has been shown to be involved in proprioceptive feedback mechanisms, has also been suggested to play a part in this sensory dysfunction. Accordingly, this study compared the innervation characteristics of lateral spinal ligaments from patients with AIS to similar measurements from control subjects. Using an antibody to neurofilament protein, Ruffini corpuscles, small and large nerve bundles, and free nerve endings were identified and their numbers and distribution patterns compared. In the control group, the innervation was found to be symmetrical between left and right sides but was more concentrated in the ventral portion of each ligament. No apparent morphological defect of the innervation was found in the lateral spinal ligaments of the scoliosis patients but the innervation densities of Ruffini corpuscles, single nerve fibres and total neural elements were significantly lower (p<0.01) than those found in normal subjects. These results suggest a possible mechanism for the production of AIS and warrant further study.

Adolescent↗

Contractile protein studies on platelets from patients with idiopathic scoliosis.

Measurements of total proteins, myosin, actin, actin-binding protein, and ATPase activity of myosin were examined in platelets from patients with idiopathic scoliosis and from healthy individuals. Abnormalities in the distribution of total and contractile proteins were revealed after fractionations. The insoluble fraction of the patients' platelets had a higher, and the cytosol fraction had a lower than normal protein content. Similar differences were observed in the specific activity of myosin ATPase. These findings show that in patients with idiopathic scoliosis platelet defects exist and that their study might be useful in research of the disease.

Actins↗

Neck and abdominal muscle activity in patients with severe thoracic scoliosis.

Patients with severe chronic obstructive pulmonary disease (COPD) do not use the sternocleidomastoid muscles when breathing at rest, but have a greater than normal neural drive to the rib-cage inspiratory muscles, the abdominal muscles, and the diaphragm. Yet the increased activation of the abdominal muscles and diaphragm in such patients has only limited mechanical effects, and this has led to the suggestion that the overall increase in neural drive is simply an automatic response of the respiratory system to a greater than resting stimulation. To test this hypothesis, we examined the pattern of respiratory-muscle activation in eight patients with severe thoracic scoliosis (Cobb angle between 100 degrees and 136 degrees). We recorded electromyograms of the sternocleidomastoid, scalene, rectus abdominis, external oblique, and transversus abdominis muscles; esophageal (Pes) and gastric (Pga) pressures; and the anteroposterior (AP) diameter of the abdomen during resting breathing in the seated posture. All patients had invariable phasic inspiratory activity in the scalenes; and five patients had invariable phasic expiratory activity in the transversus; intermittent expiratory activity in the transversus was also recorded in three patients. In contrast, only one patient had invariable phasic inspiratory activity in the sternocleidomastoid, and only one patient had invariable phasic expiratory activity in the external oblique. The decrease in abdominal AP diameter during expiration was commonly associated with a rise in Pga. These observations therefore indicate that the pattern of respiratory-muscle activation in patients with severe thoracic scoliosis is essentially similar to that seen in patients with severe COPD. This supports the concept that the order of recruitment of the respiratory muscles during breathing is an automatic response of the central controller.

Abdominal Muscles↗

Idiopathic scoliosis. Mechanical properties of the respiratory system and the ventilatory response to carbon dioxide.

The aims were to examine the effects of scoliosis (angle), and age on lung volumes, elastic properties of the respiratory system, and the ventilatory response to CO2. The mean age of the 55 patients was 25.4 plus or minus SEM 2.5 yr, and the mean angle was 80 plus or minus SEM 4.2. The mean plus or minus SEM percent predicted lung volumes were vital capacity (VC), 60.5 plus or minus 2.7; total lung capacity (TLC), 70,2 plus or minus 2.6; functional residual capacity (frc), 79.3 plus or minus 3.2; and residual volume (RV), 99.7 plus or minus 5.2. The correlation coefficients between the angle of scoliosis and each of the following were significant: TLC (-0.548), percent predicted TLC (-0.547), VC (-0.485), percent predicted VC (-0.523), FRC (-0.533), percent predicted FRC (-0.338), RV (-0.438), and percent predicted RV (-0.318). The mean compliance of the total respiratory system (Crs) was 0.049 litter/cm H2O plus or minus SEM 0.004, and the mean compliance of the chest wall (Ccw) was 0.080 liter/cm H2O plus or minus SEM 0.012. The Crs and Ccw were inversely proportional to the angle (r-0.620 and -0.721) and directly proportional to the height and the weight. The mean deltaV/deltaPco2 was 1.32 liter/min per mm Hg (SEM 0.171), and the mean deltaVt/deltaPco2 was 28.9 ml/mm Hg (SEM 3.64). The correlation coefficients between deltaV/deltaPco2 and the following were height, 0.499; VC, 0.792; TLC, 0.632; AND Crs, 0.520; and between the deltaTt/deltaPco2 and the following were height, 0.500; VC, 0.878; TLC, 0.802; and Crs, 0.590. We conclude that body size and the deformity were the determinants of the lung volumes and the mechanical properties of the respiratory system, and that these variables were the major factors in both the magnitude and pattern of the ventilatory response to CO2. The correlations between age and the mechanical properties of the respiratory sytem, deltaV/deltaPco2, and deltaVt/deltaPco2, were not significant, but the correlation coefficients between age and several of the derivatives of deltaV/deltaPco2 and deltaVt/deltaPco2 were significant.

Adolescent↗

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