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Neuromuscular scoliosis: causes of deformity and principles for evaluation and management.

Scoliosis is commonly associated with a variety of neuromuscular disorders including conditions affecting upper and lower motor neurons as well as myopathies. Contained herein is a discussion of the spectrum of neuromuscular disorders that have been associated with scoliosis and related spinal deformities. Management, including surgical treatment in such patients, is summarized including indications, expectations, and impact on trunk balance, pulmonary function, and appearance.

Adult↗

Five-year follow-up of intermittent distracting rod correction in congenital scoliosis.

Severe progressive curve deformities in children's congenital scoliosis often require an early operative intervention. As spinal fusion interferes with longitudinal growth, a "growth enabling" operative device may represent an alternative method to delay a progressive curve deformity until an eventual operative intervention can be carried out. We report on the five-year follow-up of a seven-year-old girl who presented at our clinic with severe congenital scoliosis of 62 degrees with a unilateral unsegmented bar between T7 and T12 and an anomaly of segmentation between L2 and L4. An intermittent distracting rod was implanted with transpedicular fixation from T3/T5 to L3/L4. The correction was obtained by distraction of the mobile segments and also by hemiepiphyseodesis between L2 and L4. The rod's longitudinal extension could be distracted using a special thread. Three successive operations to obtain a minimal access to the thread were necessary to achieve a correction to an angle of 30 degrees with a total rod distraction of 4.5 cm.

Child↗

[Hemivertebra resection in congenital scoliosis -- early correction in young children].

BACKGROUND: Congenital scoliosis due to hemivertebrae usually progresses during further growth and leads to severe deformities. Early correction in young children is therefore required. PATIENTS: Thirty-six hemivertebrae in 33 children aged 1 to 6 years (average 3 years 5 months) underwent surgical intervention. Mean follow-up was 4.5 years (2 months to 13 years). METHODS: The hemivertebra was resected by a posterior approach. The gap after resection was closed by compression via a transpedicular instrumentation, thus correcting the scoliotic deformity. RESULTS: Mean Cobb angle of the main curve was 45.9 degrees preoperatively, 11.9 degrees postoperatively, and 9.9 degrees at latest follow-up. The compensatory cranial curve improved spontaneously from 18.4 degrees preoperatively to 5.0 degrees postoperatively and 3.7 degrees at latest follow-up. The compensatory caudal curve improved from 21.3 degrees to 6.7 and 5.4 degrees. The angle of kyphosis was 22.8 degrees preoperatively, 8.9 degrees postoperatively, and 6.8 degrees at latest follow-up. There was one infection, 2 pedicle fractures, and 3 implant failures. In 3 patients additional operations were performed due to new developing deformities. CONCLUSION: Correction surgery of congenital scoliosis should be performed early before the development of severe local deformities and secondary structural changes. Posterior resection of the hemivertebrae with transpedicular instrumentation allows for early intervention in very young children. Excellent correction in both the frontal and sagittal planes, and a short segment of fusion allow for normal growth in the unaffected parts of the spine.

Age Factors↗

[Electrocardiography changes in scoliosis and kyphosis deformities of the thorax].

Preoperative electrocardiograms and lung function tests in patients with severe scoliosis were compared. The electrocardiograms were analysed by recognizing signs of right ventricular hypertrophy and signs of right ventricular strain. The results are as follows: Dates of electrocardiography and lung function testing show a very good correlation. Scoliotic and kyphotic deformities show a clear dependency on grade of scoliosis and age. Electrocardiographic changes do not depend on additive kyphotic deformity.

Adolescent↗

[Praying for the hunchback man. On the cultural history of scoliosis and kyphosis].

Numerous ancient burial sites contain spines with scoliotic deformities. However, very little is known about the lives of these people, the "hunchbacks". Their everyday life, how they saw themselves and were seen by others, their social status and perspectives can usually only be deduced indirectly from a variety of sources. These include fairy-tales and myths, beliefs and superstitions, classical and light literature, and accounts by theological and philosophical writers. The present paper proves that severely deformed scoliosis patients were generally burdened with a pitful fate in ancient times and during the Middle Ages. There was no effective treatment, healthy citizens showed little compassion, hunchbacks were said to have a vile and base character, and they were usually stigmatised outsiders. The sociopsychological situation for these patients did not improve until modern times, beginning with increased public awareness during the last century, in parallel with the slow development of orthopaedic treatment methods for scoliosis.

Adult↗

[Course and prognosis of congenital scoliosis].

75 patients (33 male, 42 female) with congenital scoliosis, diagnosed first time between birth and 40 years were examined. Type and site of the anomaly were classified and evaluated in accordance with a standardized scheme. The prognosis of congenital scoliosis depends strongly on the type of the anomaly. The differences of progression in the site of the abnormality are therefore minor. Unilateral unsegmented bars showed the most severe progression: the yearly rate of deterioration of the curve averages 7.6 degrees. Unilateral hemivertebrae showed 1.5 degrees (upper thoracic region) and 2.3 degrees (lower thoracic region). Two contralateral hemivertebraes and block vertebraes had a yearly rate of deterioration less than 1 degree. Operation is the treatment of choice in case of unilateral unsegmented bars. A conservative treatment is sufficient in most cases of other abnormalities.

Abnormalities, Multiple↗

[Multi-segmental fusion of scoliosis in Duchenne's muscular dystrophy].

Operations in scoliosis in patients suffering from an advanced stage of Duchenne muscular dystrophy are associated with a higher risk due to the extent of the curves, the respiratory insufficiency and frequent cardiomyopathia. Progressive scolioses in 20 wheelchair patients with an age between 10.5-18.3 years (mean 14.6 years) were treated by CDI. The mean preoperative angle in this group was 70.6 degrees, the postoperative angle 31.2 degrees (mean correction 39.4 degrees or 55.8%). The preoperative lordosis of the lumbar spine (mean angle 4.1 degrees) was corrected to 17.8 degrees postoperatively. The average intraoperative blood loss (2300 ccm) was evident more compared with idiopathic scoliosis. One neurologic complication (postoperative disturbance of bladder function) was observed. Nowadays early surgical correction and stabilization (Cobb angle > 20 degrees) has to be recommended as the treatment of choice for scolioses in Duchenne muscular dystrophy using multisegmental instrumentation methods to enable rapid mobilisation and a postoperative care without brace or cast. This conception allows a prophylactic operation including the following targets: prolongation of life expectancy, improvement of sitting position and prevention of rapid deterioration of lung function including assisted mechanical ventilation in late stages.

Adolescent↗

[Experiences with surgical therapy of scoliosis in patients with myelomeningocele].

This study reports about the experiences in surgical correction of scoliosis, which were performed between 1984 and 1989 in 32 children with myelomeningocele. The average age at time of surgery was 14 years, 1 month; the average degree of scoliosis was 96 degrees preoperatively. 6 different types of surgical techniques were used. In comparison the best results were obtained by a combination of ventral osteodiscectomy with or without instrumentation and dorsal CD-instrumentation; an improvement of 56% was found. Furthermore we sent questionnaires to all patients, who were operated more then one year ago. The majority of them judges the improved sitting stability of the trunk to be the greatest advantage. There is a high rate of postoperative complications. Beside one death, which occurred intraoperatively, especially pseudarthroses of the lumbosacral junction must be mentioned. These pseudarthroses and a to short selected area of fusion are the main reasons for the loss of correction, which was noticed after a mean follow-up period of 24.6 months. The necessity of postoperative bracing was not reduced by using CD-instrumentation.

Adolescent↗

[Proton spin tomography in children and adolescents with so-called idiopathic scoliosis].

Somato-sensory evoked potentials (SSEP) were found to be pathological mostly in the lower extremities in 26 out of 45 children suffering from so called idiopathic scoliosis. We examined the vertebral spine and the spinal cord of 8 of them (with controlled SSEP-findings) by use of conventional MR-imaging and (where necessary) 3-dimensional-data-set following the Fournier-procedure. 6 of the 8 children showed alterations as follows: 1. A lipoma spreading partly extra-, partly intraspinally. 2. Subligamentous protrusions of the intervertebral disc (2 patients). 3. Dysraphic processes (2 patients). 4. An abnormally cranial ending myelon surrounded by a widened spinal channel. The findings are demonstrated and discussed concerning the questions whether the pathological SSEP and, furthermore, the deformity of the vertebral spine could be explained thereby. We are at least able to prove that some of the children with so called idiopathic scoliosis show pathological evoked potentials and MRI-findings.

Adolescent↗

[Relations between development of the apophyses of the ilial crest (Risser's sign) and development of the hand skeleton in girls with scoliosis].

The maturation of the iliac apophysis (Risser sign) and of the bones of the hand and wrist of 34 girls with scoliosis with 46 pairs of data were analyzed to get information upon their predictive value for further growth and development of the scoliotic curve. A strong correlation was found between the Risser sign and the maturation of the hand and wrist (r = 0.74, p less than 0.005). The prognostic value of the Risser sign (r = -0.57) for further height gain is superior to chronological age only (r = -0.52), the maturation of the hand and wrist (r = -0.62) however is superior to the Risser sign. It can be assumed that this also holds for their prognostic value for the progression of the scoliotic curve. We feel that in the clinician routine for girls with scoliosis the determination of the skeletal maturity from X-rays of the hand is sufficient and with its probably unavoidable irradiation risks to the gonads the determination of the Risser sign should be abandoned.

Adolescent↗

[Results of lateral electrical surface stimulation in scoliosis].

A group of 26 out of 32 patients with idiopathic scoliosis treated by lateral external electric stimulation within the last 4 years was examined. In about 80% an arrest of progression or an improvement of the spinal curvature was found within 2 years. The average improvement was 7.6 degrees Cobb. A good effect can be expected in scoliosis with a Cobb angle up to 25 degrees. Analysis showed that good flexibility, low rotation, low Risser sign and Harrington factor seem to be decisive for a successful treatment. Even if the range of electrostimulation can be extended in the future up to higher degrees of spinal curvature it is doubtful that electrostimulation can substitute orthotic treatment in the whole established spectrum.

Adolescent↗

[The progression of untreated idiopathic scoliosis in the x-ray image].

The natural history of scoliosis, or lateral curvature of the spine, was followed up in 135 patients (111 girls, 24 boys) for a total average period of 52.4 months. We observed patients with a curvature of between 5 degrees and 30 degrees none of whom had been treated specifically as orthotics or with electrical stimulation or by surgery. Two groups of patients with progressive curvature were differentiated: 1) in 62.2% of the patients the curvature progressed by more than 5 degrees during the entire observation period; 2) in 36% of the patients we found an increase in curvature by more than 5 degrees within one year. Another result of our study was that idiopathic scoliosis is particularly dangerous in young patients with a "0" Risser sign. Thoracic curvatures and double major curvatures were more liable to progress than lumbar and thoracolumbar curvatures. Even a small angle of curvature in young patients must be taken seriously.

Adolescent↗

[Ventral derotation spondylodesis. Results of treatment of cases of idiopathic lumbar scoliosis (author's (author's transl)].

Of nearly 300 VDS operations, performed at the German Scoliosis Center, the first 50 cases have been analysed. Their follow-up was conducted over a period of two years minimally. Patients with lumbar scoliosis and double major curves will be analysed in detail separately. In suitable cases use of the ventral derotation spondylodesis offers considerable advantages as compared to other methods. Bending tests are decisive for determining the fusion area. Rotation and obliquity of the third or fourth lumbar vertebra are of critical importance. The author considers the Harrington operation no longer justified in those cases in which ventral derotation spondylodesis can be successfully used. Furthermore, the Dwyer procedure has to be considered obsolete.

Adolescent↗

[On the incidence of thoracal scoliosis in juveniles (author's transl)].

Mass radiography in juveniles between 10 and 19 years of age in Westphalia pointed to a probable incidence of thoracal scoliosis of more than 10 degrees in about 4 per cent of the girls and 1.0--1.5 per cent among boys. In both girls and boys the convexity of the scoliosis is chiefly in the right direction, especially if the apex of the curvature is positioned in cranial direction or if the findings are very pronounced. In a few exceptional cases only the apex of curvature is above Th7. Hence, most of the patients can be adequately treated by means of one of the more recent methods of orthesis.

Adolescent↗

[Defective type I collagen in a woman patient with juvenile idiopathic scoliosis (author's transl)].

The molecular causes of idiopathic scoliosis have not yet been clarified to any appreciable extent and are probably heterogeneous. The occurrence of scolioses in congenital diseases of the connective tissue points to changes in connective tissue metabolism. Collagen is the most essential structural connective tissue component of bone. It was the aim of our investigations to examine the synthesis of collagen of Types I and II in order to identify a possible molecular defect. In the woman patient examined by the authors, a pathologically changed alpha-chain of the collagen of Type I was found as the probable cause of this particular case of idiopathic scoliosis.

Adolescent↗

[Morphometric dual-energy roentgen absorptiometry in follow-up of scoliosis].

PURPOSE: Taking pictures of the spine with Morphometric X-ray Absorptiometry (DXA) leads to a radiation dose of just 1% of conventional X-ray. Purpose of this study was to evaluate if DXA is a suitable method for sufficient measuring of the scoliosis angle. METHOD: We examined 20 patients with scolioses I. Control-pictures were made with conventional X-rays and compared to those made by DXA, measuring of scoliosis angle was performed by the method of Lippmann/Cobb. RESULTS: The difference between both methods was -10.5% DXA to conventional X-ray-each angle examined on DXA was smaller. CONCLUSION: It is possible to measure scolioses I on DXA.

Absorptiometry, Photon↗

Central nervous system lesions and developmental scoliosis in myelomeningocele.

Hydrocephalus, spinal cord tethering, cord atresia, hydromyelia, syringomyelia and various manifestations of the Arnold-Chiari malformation occur in association with scoliosis in patients with myelomeningocele. While the causes of the cord lesions remain debatable, these lesions appear to be progressive and causally related to the scoliotic deformity. We have reviewed seventeen myelomeningocele patients with developmental scoliosis and have found that aggressive orthopaedic treatment combined with neurosurgical treatment can satisfactorily control spinal deformity in most cases. Before undertaking orthopaedic treatment of severe spinal deformities in patients with myelomeningocele a careful investigation of the central nervous system with myelography and cord and head CT scans should be carried out.

Arnold-Chiari Malformation↗

Chiropractic and pilates therapy for the treatment of adult scoliosis.

OBJECTIVE: To describe the use of Pilates therapy and sacro-occipital technique in the management of a 39-year-old woman with scoliosis who had undergone spinal fusion many years earlier. CLINICAL FEATURES: The patient had progressive severe low back pain that had worsened over the years after her surgery and had prevented her from activities such as carrying her son or equipment necessary for her job as a photographer. INTERVENTION AND OUTCOME: The patient was provided a series of Pilates exercises used to overcome her chronic habituation and muscle weakness. Although this therapy went on for some time, she did begin to stabilize and increase physical activity. At present, she is no longer limited in her physical activity, although she still exhibits some symptoms from her scoliosis. CONCLUSION: The addition of Pilates therapy can be useful to care for patients with chronic low back pain and deconditioning.

Activities of Daily Living↗