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Pulmonary function and scoliosis in children with spinal muscular atrophy types II and III.

AIMS: The objectives were to evaluate the clinical course of spinal muscular atrophy (SMA) types II and III patients necessitating scoliosis surgery at the National University Hospital, Singapore. METHODS: A retrospective review of SMA types II and III patients, born over a 10-year period between 1983 and 1992, was conducted. RESULTS: There were eight patients: four with SMA type II and four with SMA type III. The mean age at scoliosis surgery was 9 years 7 months (range 7 years 6 months-12 years 4 months). The mean preoperative Cobb angle was 65.4 degrees (range 43-90 degrees ) and the mean postoperative Cobb angle was 22.6 degrees (range 12-45 degrees ), with a mean correction of 64.8% (range 47.7-77.8%). The decline in percentage predicted forced vital capacity (FVC) was 7.7% (95% CI: 12.4% to 3.0%) per year preoperatively and this was reduced to 3.8% (95% CI: 5.8% to 1.9%) per year postoperatively. The mean length of preoperative and postoperative lung function follow-up was 6.3 months (range 0.03-31 months) and 44 months (range 0-110 months), respectively. CONCLUSIONS: This study suggests that pulmonary function in SMA types II and III continues to decline after scoliosis surgery, though the rate of decline is less marked. Overall, the combined results from this study and all other previously published studies are conflicting in regard to the effect of scoliosis surgery on pulmonary function in SMA types II and III, though half of the studies (3 of 6) did demonstrate a continued decline in lung function postoperatively. This decline in pulmonary function despite spinal stabilization is likely secondary to the progressive neuromuscular weakness of the disease.

Adolescent↗

[Scanning stereographic surface measurement in idiopathic scoliosis after VDS (ventral derotation spondylodesis)].

INTRODUCTION: So far only radiometric and clinical methods have been available for the evaluation of results after anterior scoliosis surgery. Rasterstereography has proved to be a reliable method for three-dimensional surface measurement of conservatively treated idiopathic scoliosis patients. Therefore, patients treated operatively with anterior instrumentation were examined using rasterstereography to determine the three-dimensional correction of the spinal deformity. The aim was to measure back shape deformity, in particular derotation, and thus cosmetic improvements. METHODS: 31 patients with idiopathic thoracic, thoracolumbar and lumbar scoliosis (Cobb angle 57.2 degrees) were examined with raster stereography preoperatively, postoperatively and after follow-up (25.2 months) in a standardized standing posture. Standing radiographs were compared with raster stereography. RESULTS: The mean Cobb angle was reduced from 57.2 degrees to 17.2 degrees, the rasterstereographic maximal surface rotation from 16.5 degrees to 10.8 degrees, and the vertebral rotation according to Perdriolle from 29.2 degrees to 16.7 degrees. During follow-up the Cobb angle increased to 20.8 degrees, and surface rotation to 11.3 degrees. Vertebral rotation remained constant. Lordosis and kyphosis angles changed only slightly. CONCLUSION: Rasterstereography is a suitable tool for analyzing the three-dimensional correction of spinal deformities after anterior scoliosis surgery. In particular, the cosmetic improvement is clearly demonstrated. The measurement of surface rotation allows objective quantification of the obtained derotation.

Adolescent↗

[Calculation of 3-D deformity in scoliosis by MRI of the total spine in two perpendicular reconstructed planes].

AIM: Scoliosis is a spinal deformity that is more complex and does not exist in one plane only. There have been many attempts to analyse three-dimensional spinal deformity, however, these procedures necessitate higher radiation doses. METHOD: In this study we define angles according to the Cobb Definition. By means of trigonometrical evaluation, 3D calculation of spinal deformity is demonstrated using MRI of the total spine in two reconstructed perpendicular planes. 3D spinal analysis was performed on 41 female and 7 male patients with scoliosis. RESULTS: 79 angles were measured by using the Cobb angle in reconstructed coronal plane of MRI of the total spine and, in addition, by using our method. The scoliosis Cobb angles ranged from 11 - 59 degrees (mean: 23 degrees +/- 9 degrees ), the real angles ranged from 12 - 70 degrees (mean: 32 +/- 14 degrees ). There was a poor correlation between Cobb angles and the 3D calculated angles (r = 0.37; p < 0.0001). CONCLUSION: Our method enables us to determine the real angle of scoliosis and to avoid techniques with any radiation risk for the patient.

Adolescent↗

[Brace effect in scoliosis in the sagittal plane - an MRI study].

AIM: Using magnetic resonance (MR) imaging we studied the brace effect in scoliosis in the sagittal plane. METHOD: In 38 patients with idiopathic scoliosis (mean age 13.4 years) MR total spine imaging was carried out to investigate the immediate effect of bracing in the sagittal plane. There were 19 thoracic, 13 S-shaped and 6 lumbar scoliosis. On conventional radiographs the mean Cobb angle of the thoracic curves was 31 degrees and of the lumbar curves 26 degrees. MR imaging was performed in the supine position with and without the brace in direct sequence. On the sagittal MR projection the Cobb angle was measured between T 4 and T 12 and between T 12 and L 5. RESULTS: On the coronal MR images the mean correction with brace was 23 % of the thoracic curves and 29 % of the lumbar curves. The mean, sagittal Cobb angle (T 4 - T 12) was 14 degrees without brace and 12 degrees with brace. For the lumbar curves the mean sagittal Cobb angle (T 12 - L 5) was 32 degrees without brace and 31 degrees with brace. In the paired t-test these differences were significant. CONCLUSION: Using MR total spine imaging the brace effect in scoliosis could be depicted in the sagittal plane. In the thoracic spine a correction of the lordotic deformity could not be observed.

Adolescent↗

[One- or two-step instrumentation for thoracolumbar scoliosis due to myelomeningocele?].

AIM: This investigation evaluates patients with MMC who underwent a two-stage anterior-posterior correction and stabilisation of thoracolumbar scoliosis due to myelomeningocele. The data were compared with the few reported series of one-stage versus two-stage surgery in the literature. METHOD: From 1.7.1992 to 30.6.1995, 11 consecutive patients with severe thoracolumbar scoliosis due to myelomenigocele were admitted at our hospital. The mean age at operation was 12 years nine months (range nine years nine to 14 years six months). All patients underwent a two-stage anterior and posterior spinal instrumentation. The patients were pre- and postoperatively evaluated. RESULTS: All patients were followed for a mean of 4 years 11 months (range 42 months to 88 months) from the time of the second stage procedure. Preoperatively the mean scoliosis angle was 82 degrees (range 55 degrees to 110 degrees ), this was reduced to a mean of 31 degrees (range 8 degrees - 70 degrees ), at final follow-up, the correction had deteriorated slightly to a mean of 35 degrees (range 12 degrees - 80 degrees ). No patient had increased neurological deficit or showed other major complications, i. e., infection, sepsis due to immunologic disorders at the time of the operation. CONCLUSION: We believe that with the two-stage anterior and posterior instrumentation an effective correction of the scoliosis can be achieved. Compared to other studies this report confirms the low morbidity rate and emphasise the good results of a two staged procedure.

Adolescent↗

[Selective ventral derotation spondylodesis in idiopathic thoracic scoliosis: a prospective study].

AIM: Radiometric curve analysis of instrumented primary and spontaneous secondary curve correction after anterior correction and fusion of idiopathic thoracic scoliosis. METHOD: Sixty-four patients with idiopathic thoracic scoliosis were prospectively evaluated. All patients were operated either with the Zielke-VDS or with a primary stable double rod instrumentation with selective fusion of the thoracic curve from end-to end-vertebra. Follow-up averaged 29 months (24 - 52 months). RESULTS: The Cobb angle of the primary curve averaged 63.2 degrees preoperatively and was corrected to 21.4 degrees postoperatively with an average loss of correction of 5.3 degrees (58 % final curve correction). Apical thoracic vertebral rotation was corrected by 48 %. The secondary lumbar curve measured 38.2 degrees preoperatively (72 % correction on the bending films) and was spontaneously corrected by 57 % to 16.4 degrees without significant loss of correction in the final follow-up. Apical vertebral rotation averaged 11.3 degrees in the lumbar curve and was corrected spontaneously by 24 % to 8.6 degrees without significant loss of correction. Lumbar apex vertebra deviation showed no significant reduction. There was no case of lumbar curve decompensation in either frontal or sagittal plane. Implant related complications were observed in 7 patients (rod breakage), but no pseudarthrosis occurred. There were no neurological complications noted. CONCLUSION: Selective anterior correction and fusion in idiopathic thoracic scoliosis enables a satisfactory correction of both primary and lumbar secondary curves. The advantage of selective anterior correction and fusion of thoracic scoliosis is the short fusion length, better derotation and satisfactory correction of the secondary lumbar curve. The disadvantages of single threaded rod techniques in terms of lack of primary stability and a kyphogenic effect have been eliminated by the development of a primary stable, small size double rod instrumentation.

Adolescent↗

[Experience in operations for scoliosis in patients with cerebral palsy].

AIM: Experiences in operation of scoliosis in cerebral palsy are very different. Therefore a therapeutic regiment does not exist. In this study we want to report our experiences. METHODS: This retrospective-clinical study (1986-2003) includes 46 patients with cerebral palsy who had been operated for scoliosis. The data focus in particular on the therapeutic strategies and its success and complications. Publications from 1946 to 2003 were reviewed and served for comparison. RESULTS: 28 female and 18 men during one period by median 3.4 years were after-observed. The post office-operationally reached correction of the curvature of all operation procedures was appropriate for median between 51 and 68 % depending upon localization of the curvature. In the last re-examination median a correction loss of 3-25 % existed. A fusion to S1 corrected a thoraco-lumbale or lumbale scoliosis significantly better than a fusion to L5. The posterior and the combined posterior-anterior operation procedure showed comparable correction results. The total complication rate was with 35 %. CONCLUSIONS: Due to good correction successes and smaller complication rate we favor a posterior-anterior operation procedure. A fusion to S1 is indicated with thoraco-lumbale and lumbale fusion. Altogether complication rate was reduced in patients with scoliosis and cerebral palsy after operation at the spinal column in the last years.

Cerebral Palsy↗

[Idiopathic scoliosis in the sagittal plane].

Idiopathic scoliosis in sagittal plane is characterised by hypokyphosis and the restriction of anteflexion. This we have to take into consideration when we start an early treatment. The results are the best in children beyond 8 years of age with an angle of Cobb below 20 degrees and in thoracolumbar scoliosis. For the beginning idiopathic scoliosis the definition as an error of form is useless. The thesis that restriction of anteflexion has the priority in the development of idiopathic scoliosis will be supported by the examination of pupils.

Adolescent↗

[How idiopathic is idiopathic scoliosis? Results of neurological studies with somatosensory evoked potentials (SSEP) in children and adolescents].

UNLABELLED: About 90% of all the scolioses are called "idiopathic". Various neurological diseases (for example poliomyelitis, etc.) are frequently accompanied by deformities of the spine. The so-called somatosensory evoked potentials are at our disposal being a very sensitive and a non-problematic neurological diagnostic tool. Formation of the question: Are there clinically non-detectable neurological changes demonstrable by use of evoked potentials in children with so-called idiopathic scoliosis? 45 non-operated patients suffering from scoliosis and 21 healthy children were examined clinically, neurologically, and by use of evoked potentials. RESULTS: 26/45 children with idiopathis scoliosis showed pathological evoked potentials (right-left-side-difference concerning latency and amplitudes of the potentials and generally delayed transmission from peripheral nerves to the somatosensory cortex), mostly concerning the lower extremities. 19/45 children showed normal evoked potentials (EP). There was no correlation between EP and direction respectively degree of the scoliosis. Neurological affections mostly located caudally of the cervical spine are to be discussed (for example protrusion of the intervertebral disc, dysrhaphic processes etc.).

Adolescent↗

[Scoliosis in infancy].

1. The paper deals with 101 patients (52 male, 49 female) who have been treated in the first and second years of life because of resolving scoliosis and could be re-examined later at the age of 15 between 17 years. 2. The clinical and roentgenological findings of seven patients only had been normalized, where as in all other cases low lateral deviations of the spinal column (77 times), frequently at a modified type of curvature (44 times) or structural modifications (77 times, 17 spinal columns without lateral deviations), or not rarely, even both of them, had been found. 3. During development it came to a displacement of the vertex-vertebra on a average of 1-2 vertebrae to caudal. 4. No inspected resolving scoliosis had change over into a progressive scoliosis, a coherence consists not with idiopathic scoliosis of the prepuberty and puberty.

Adolescent↗

[Determination of minerals and trace elements in idiopathic scoliosis].

In a preliminary study the spinal processes of the curve and the parietal bone in idiopathic scoliosis were analyzed in 10 patients by atom absorption spectrophotometry during dorsal fusion operations using the Harrington instrumentarium. In addition to calcium and magnesium, the trace elements iron, copper, manganese and zinc were determined. For comparison, bone samples were taken from the pelvic ridges of the patients, as well as control biopsies from the pelvic ridges of 21 healthy patients in the clinic who were of the same age. The bone samples had been taken from the latter during surgery for pseudarthrosis. The statistical analysis of the mineral and trace elements showed that there is no local disturbance of mineral and trace element metabolism in idiopathic scoliosis; a comparison of the bone samples from the scoliosis patients with the normal values also largely ruled out a generalized disturbance of mineral and trace element metabolism in bone tissue in idiopathic scoliosis.

Adolescent↗

Presentation of GTB orthoses for hyperlordotic treatment of idiopathic scoliosis.

Screening of scoliosis means it is possible to make an early diagnosis. Treatment is started when the angle reading is still quite low but as soon as it has been proven that the scoliosis is progressive. A 3-D examination of the scoliosis makes it possible to have a better understanding of its development, which is in kyphosis at lumbar level and in lordosis at thoracic level. It would seem logical that in order to prevent a lumbar scoliosis becoming kyphotic, lordosis must be maintained and if necessary, a hyperlordosis created. The same applies to the thoracic curve when attempting to restore a thoracic kyphosis with a lumbar hyperlordosis. We have developed new orthoses with the correction principle based on creating a lumbar hyperlordosis. Correction of the translation is the second correction principle. In theory, correction involves: a translation (convex lateral pressure) and a hyperlordosis which puts back in place the convex articular facet (posterior pressure). With these two principles in mind, the aim of the orthosis is to invert the torsion movement and to avoid pressure in the opposite direction of the deformity. We have maintained the principle of a single shell made of ribbed polypropylene to produce a brace which is both light and strong. There are three types of models depending on the spine curve: the short GTB 1 brace for lumbar curves, the long GTB 2 brace for thoracic and double curves, the long or short GTB 3 brace depending on the upper end vertebra for thoraco-lumbar curves.

Adolescent↗

Transverse process wiring for thoracic scoliosis: a new technique.

We describe a method of reducing neurological risk by avoiding neural elements when performing segmental spinal fixation for scoliosis. This was done by making use of the transverse processes of the thoracic vertebrae 1-11 and wired plates. The wires are prevented from cutting the bone by attaching them to rectangular plates. These wired plates on the two sides of each segment should be firmly fixed to the base of the transverse process by tying them to each other. Maximum correction is obtained by spreading the force applied by stabilizing each thoracic vertebra from both sides. Derotation is attempted when all the wires have been tightened. Tension forces are spread to all fixated vertebrae simultaneously and the correction should be carried out at all levels at the same time. On the convex side, each wire connects over the rod to the next one above it and, tightened one by one, provides compression. We treated 15 patients having a thoracic curve with this method and followed them for 25-39 months. In 2 cases of congenital scoliosis, a 50% correction was obtained. In 3 cases of neurofibromatosis scoliosis, a 55% correction was achieved. In 10 cases of idiopathic scoliosis, the correction was 60%. When the thoracic sagittal contour was evaluated, thoracic kyphosis, which had been between -20 degrees and +90 degrees, had improved to between +8 degrees and +43 degrees in all cases. No infection or neurological complications occurred.

Adolescent↗

The use of dynamic Lycra orthosis in the treatment of scoliosis: a case study.

Treatment of scoliosis has been under discussion in relation to surgical intervention since the Boston brace was presented by Hall in 1976 (Hall et al. 1976; Watts et al. 1977). The effects of rigid bracing on thoracic skeletal integrity and the possible deformation of ribs due to the high localized pressure due to prolonged wear (Biorthex 1999; Coillard et al. 1999) have been highlighted. The lack of compliance (Houghton et al. 1987) has encouraged clinicians to investigate other options for non-surgical treatment. The Spinecor and Triac bracing systems have been developed as a result of this research; however, both of these orthoses had been designed with idiopathic scoliosis in mind. Little research has been done into the effects of bracing on the neuropathic curve. The use of dynamic Lycra garments in the treatment of neurological scoliosis offers the advantage of deformity correction without the bulk and discomfort of rigid braces. Recent clinical experience has shown that the Lycra suits have a positive effect in the treatment of scoliosis. This study discusses the treatment of a child presenting with a spinal tumour and although not truly of neurological presentation indicates that the garment can be used for the different scoliotic presentations.

Braces↗

The influence of seat adjustment and a thoraco-lumbar-sacral orthosis on the distribution of body-seat pressure in children with scoliosis and pelvic obliquity.

PURPOSE: To determine the effect of a thoraco-lumbar-sacral orthosis (TLSO) on the distribution of body-seat interface pressure in children with concomitant scoliosis and pelvic obliquity and to determine the effects of two methods commonly used in customized seating--elevation (push up) of the lower side of the pelvis or a wedge insertion beneath the raised pelvis--on the distribution of body-seat interface pressure. METHODS: The study population comprised 15 children with an underlying neuromuscular disorder. All had scoliosis and pelvic obliquity when seated, and used a TLSO during sitting. Body-seat interface pressure was measured using the QA Pad. Maximum pressure, mean pressure and contact area were recorded at baseline and at 10 degrees 'push up' and 10 degrees wedge insertion, with and without the TLSO. X-rays were performed with and without the orthosis at baseline position. RESULTS: The TLSO reduced the scoliosis deformity by a mean of 5.3 degrees and significantly (p < 0.05) reduced the mean pressure and contact area in the sub-group of patients whose pelvic obliquity was contralateral to the side of the curve. Seat adjustment did not have any significant effect on pressure readings. CONCLUSION: Application of a TLSO in a child with scoliosis and contralateral pelvic obliquity significantly reduced the spinal curvature and interface sitting pressure. Manipulation of sitting by use of wedges under the pelvis had no significant effect on pressure distribution.

Adolescent↗

Developmental psychological aspects of scoliosis treatment.

The diagnosis and treatment of idiopathic adolescent scoliosis can have significant psychological consequences for affected individuals. This article aims to provide an overview of the current state of empirical knowledge concerning the psychosocial aspects of scoliosis in adolescence. These research results are set against experience gained from practical work. Adolescence, as a sensitive phase of a young person's development, requires a special degree of adaptation in the event of a chronic illness. For scoliosis patients this means, for instance, facing up to cosmetic impairments and subjectively significant physical defects. Cognitively the patient must come to terms with a commitment of time-consuming, confining, and sometimes uncomfortable treatment for a condition that does not always cause physical symptoms and to achieve success which is not necessarily defined as an improvement in the state of health. Scoliosis is a risk factor for impairment of the quality of life of children and adolescents. Its impact is particularly marked if brace-wearing is indicated. Particular attention needs to be paid to aspects of brace compliance. Support for patients within the context of in-patient rehabilitative treatment has proved to be both necessary and helpful. Here, within the setting of psychological group sessions and individual discussions, the possibility exists for preventing psychosocial impairment.

Adolescent↗

Horizontal postrotatory nystagmus response in female subjects with adolescent idiopathic scoliosis.

Quantitative and qualitative assessment of vestibular function was made in 18 female subjects with adolescent idiopathic scoliosis and a control group of 25 female subjects without scoliosis. The Southern California Postrotary Nystagmus Test, which examines postrotatory nystagmus response, was used to assess the vestibular function of subjects in both groups. The results indicated that subjects with adolescent idiopathic scoliosis had a significantly decreased duration of postrotatory nystagmus as well as irregularities in nystagmus form. The recommendation was made that a neurological examination, including assessment of vestibular function, be incorporated into screening methods for scoliosis.

Adolescent↗

Current principles in the nonoperative management of structural adolescent idiopathic scoliosis.

Physical therapists routinely identify and manage adolescents with idiopathic scoliosis. Therefore, it is essential that we maintain a current understanding of both the condition and the treatment principles. This paper includes both a discussion of the personal impact on those left untreated and a review of current concepts concerning the following: the pathological findings associated with structural scoliosis; the prevalence of adolescent idiopathic scoliosis; and the evaluation techniques, the nonoperative treatment procedures, and the long-term treatment results for idiopathic scoliosis. The final section presents research topics relevant to the etiology, the detection, and the treatment of moderate scoliotic curves.

Adolescent↗