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Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

One hundred and sixty-two patients with Duchenne muscular dystrophy (DMD) were followed over a 10-yr period to provide a profile of impairment and disability. The median height and weight of DMD boys were normally distributed before ages 9-10, but during the second decade height was markedly reduced, and weight was no longer normally distributed. Younger boys gained more weight than normals, whereas older individuals actually showed weight loss. Manual muscle test (MMT) measurements showed loss of strength in a fairly linear fashion from ages 5-13 yr, -0.25 MMT units per year. Upper extremity muscles were stronger than lower extremity muscles, proximal muscle groups were weaker than distal muscle groups, and extensor muscles were weaker than flexor muscles. There was no side dominance. There was a change in the rate of strength loss at 14-15 yr, and the decline slowed to only -0.06 MMT units per year. Although MMT and quantitative strength measurement profiles were similar, the latter were far more sensitive. In general, by the time strength declined to MMT grade 4, isometrically measured strength was 40-50% of normal control values. Joint contractures were rare before age 9, increased in frequency and severity with age, and were present in most individuals older than 13. Lower extremity contractures were strongly related to onset of wheelchair reliance, but there was no association between muscle imbalance around a joint. The prevalence of scoliosis increased between ages 11 and 16, with about 50% of the boys acquiring scoliosis between ages 12 and 15, corresponding to the onset of the adolescent growth spurt. Wheelchair reliance and scoliosis were both age-related. Percent predicted forced vital capacity declined at different yearly rates: ages 7-10, -0.3%; ages 10-20, -8.5%; after age 20, -6.2%. There was a direct relationship between percent predicted FVC and MMT scores. Decreased airway pressures, especially maximal expiratory pressure, appeared earlier than reductions in FVC but followed the same pattern. Thirty percent of the DMD boys had a history of respiratory complications, and the frequency increased with age. Spine deformity did not have a significant additive effect on the age-related decrement in pulmonary function. There was a high occurrence (79%) of abnormal electrocardiograms with age-related progression of some abnormalities, but only 30% of the patients had a history of cardiovascular complications. Functional level grades and timed motor performance measurements had a nonlinear relationship with strength and age.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Profiles of neuromuscular diseases. Duchenne muscular dystrophy. 757 24

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.
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PMID:[Multi-segmental fusion of scoliosis in Duchenne's muscular dystrophy]. 798 96

Somatosensory cortical evoked potentials (SCEPs) monitoring in neuromuscular scoliosis surgery was evaluated in a large consecutive series of spinal reconstructions to define its role in the detection and prevention of spinal cord injury; 141 procedures in 101 patients were evaluated. In 28% a reliable tracing could not be obtained. Two postoperative neurologic deficits were missed by SCEP monitoring. Diagnosis (Duchenne muscular dystrophy and polio) was the only significant factor found to correlate with a reliable tracing. The use of sublaminar wires was associated with a higher rate of change in tracing. Transient changes were common. SCEPs are unreliable and nonspecific in neuromuscular scoliosis surgery and are not efficatious in preventing or detecting spinal cord injury when used alone. Adjunctive techniques using epidural and MEPs must be studied in these patients.
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PMID:Efficacy of spinal cord monitoring in neuromuscular scoliosis. 784 86

To investigate the natural course of the spinal deformity in Duchenne muscular dystrophy (DMD) and its clinical relevance, longitudinal series of spinal radiographs and medical records of 46 patients with DMD were reviewed. The natural course of the deformity was classified into three types; type 1 (n = 21), unremittent progression of scoliosis with kyphosis; type 2 (n = 18), transition from kyphosis to lordosis before age 15 years; and type 3 (n = 7), less deformity without prominent longitudinal changes. Age at loss of ambulatory ability was not a predictor of type. Neither was the age at which the Cobb angle was 30 degrees correlated with the rate of subsequent progression. Because the spinal deformity always progresses, we consider spinal surgery justifiable in type 1, when a certain strict indication exists, such as spinal deformity > 30 degrees and age < 15 years in patients with > 35% predicted value of vital capacity. In type 2, operation may be necessary in patients in whom Cobb angle will progress unremittently. There is no surgical indication for patients with type 3.
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PMID:Longitudinal study of spinal deformity in Duchenne muscular dystrophy. 837 Jul 81

Although the primary defect of Duchenne muscular dystrophy has been found, there is no causal treatment to alter the natural course of this disease. Based on the recommendations by Glorion and Rideau with early treatment of contractures of the hips and the lower limbs we performed a modified release of the spina muscles, resection of tensor fasciae latae muscle and a lengthening of the tendo calcaneus in 32 patients. The mean age of DMD patients at time of operation was 6.1 years. The mean follow-up was 3.4 years. All children underwent mobilisation the day after surgery. Complete correction of all contractures was immediately achieved after operation and kept in all but two cases up to the follow-up examination after 3.4 years. No loss of ambulation was observed. Our results demonstrate that early selective surgery in DMD patients just at or better before the onset of contractures without performing an additional aponeurectomy of the iliotibial band and percutaneous tenotomy of the hamstrings according to the original Glorion-Rideau technique safely prevents severe contractures and thereby delays the progression of scoliosis.
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PMID:Clinical results of early orthopaedic management in Duchenne muscular dystrophy. 855 16

Over 90% of patients with Duchenne muscular dystrophy develop a scoliosis when they become wheelchair bound. The scoliosis is progressive and is associated with deteriorating lung function. The purpose of this study was firstly to assess whether a standing regimen, in patients who had gone off their feet, protected against the development of scoliosis and affected their lung function, and secondly to evaluate the effect of spinal stabilisation in patients who had developed a progressive scoliosis. The results of the first part of this study showed that a standing regimen significantly delayed the progression of scoliosis and that patients who complied with the standing regimen had a significantly better lung function, as measured by vital capacity and peak expiratory flow rate, than those patients who did not stand. Spinal stabilisation prevented deterioration in the scoliosis, whereas the deformity continued to progress relentlessly in patients who did not undergo surgery. The patients who underwent spinal stabilisation maintained a significantly better lung function and had an improved survival compared with the patients who refused surgery.
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PMID:Lung function in Duchenne muscular dystrophy. 858 25

We studied the results in 46 patients with neuromuscular and neurogenic scoliosis (average age 13.5 years, range 6-19 years) who had had posterior fusion with a modified Luque technique between May 1985 and June 1992. The main criteria to recommend surgery were curve progression, loss of balance when sitting, control of the head and difficulties in wearing an external orthotic support. The mean preoperative curve was 63 degrees, the postoperative value was 24 degrees, representing a correction of about 62%. The average number of stabilized segments was 13. In 39 out of 46 patients, lumbosacral fixation was included in the construct. Failure of implants, pseudarthroses and major losses of correction in purely neuromuscular scolioses could be avoided by using rigid segmental fixation and a dorsolateral fusion with a mixture of autologous and allogenous bone. The scoliosis most difficult to influence was found to be Friedreich's ataxia. In Duchenne muscular dystrophy the best method of treatment was surgery performed as early as possible, i.e. at the time of loss of walking capacity in the case of a scoliosis exceeding 20 degrees and with two consecutive X-rays proving curve progression. Analysis of our series does not confirm the morbidity and complication rates of previous studies.
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PMID:Predominantly posterior instrumentation and fusion in neuromuscular and neurogenic scoliosis in children and adolescents. 872 89

This study is designed to analyse the behavior, in the sagittal plane, of a complete human dorso-lumbar rachis, made rigid by the posterior instrumentation used for the treatment of scoliosis, on subjects suffering from DMD (Duchenne Muscular Dystrophy). The object of this analysis is to demonstrate the reliability of early surgery made possible by new instrumentation. Close review of the literature shows that the currently used Harrington or Luque instrumentations lead to mechanical complications, especially rod breaking, at the thoraco-lumbar junction. 8 specimens were non-destructively tested in-vitro. Compression and flexion were applied. For each test, rachis movements with and then without instrumentation, and also rod restraints were noted. The results show a linear stiffness multiplied by 8.3 in flexion and 11.6 in extension. The maximum restraint recorded for physiological displacements is 77 MPa. This remains largely under the fatigue-breaking limit of the metal used (stainless steel hammer-hardened 316 L, Young's modulus = 200,000 MPa, Poisson's ratio = 0.21, endurance limit = 350 MPa at 5 x 10(6) cycles). The results of this study encourage us to continue and develop early surgery in children affected by myopathy, with fixation of the complete rachis, including a lumbo-sacral arthrodesis and a supple dorsal part of the mounting, in the sagittal plane.
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PMID:In-vitro biomechanical study of a dorso-lumbo-sacral posterior supple instrumentation with variable section. 893 35

In Duchenne muscular dystrophy (DMD), sphincter muscles tend to be clinically spared. However, urinary incontinence is occasionally reported, usually late in the course of the disease. We wished to determine the etiology of urinary dysfunction in patients with DMD. Seven boys with DMD and urinary dysfunction were examined by a neurologist and a urologist followed by urodynamic and electrophysiological assessment. Based on the results of these evaluations, patients were defined as having an upper motor neuron (UMN), lower motor neuron (LMN), or myopathic lesion. Five of the patients had UMN abnormalities consisting of either uninhibited contractions or bladder/sphincter dyssynergy. One patient had a LMN lesion with prolonged duration and high-amplitude motor units. No patient demonstrated myopathic motor units. Five boys had undergone spinal fusion for scoliosis. We conclude that urinary incontinence in DMD is most often due to UMN dysfunction and not due to a severe myopathy of the detrusor or external sphincter. The most likely causes of the UMN abnormalities are severe scoliosis or a complication of spinal fusion surgery.
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PMID:Urinary dysfunction in Duchenne muscular dystrophy. 896 33

We studied the functional outcome of spinal fusion for the surgical treatment of scoliosis in 8 patients with Duchenne muscular dystrophy (DMD). The mean age of DMD patients at the time of the surgery and the mean follow-up duration was 13.8 (12.3 to 15.4) and 3.9 (1.5 to 6.8) years, respectively. The average spinal angle (Cobb angle) was corrected from 58.8 to 28.6 degrees with the mean corrective rate of 51.3% by the surgical intervention. The correction rate was higher and the corrected Cobb angle remained unchanged during follow-up period in mildly scoliotic patients. Forced vital capacity (FVC) increased post-operatively in 3 patients with moderate scoliosis (Cobb angle: 50 to 80 degrees), indicating that the correction of spinal alignment is effective for the treatment of decreased thoracic volume in DMD. On the other hand, two cases with low % FVC (16.9% and 30.4%, respectively) had poor prognosis in respiratory status. Namely, one died of pneumonia at 17 months after the surgery and the other required mechanical ventilation via nasal mask at 3 years post surgery. Sitting balance improved in all patients, which resulted in more functional use of their upper extremities. During the follow-up period, all patients except one patient who died of pneumonia could maintain sitting balance without support. Moreover these included 2 patients over 20 year old. No complications related to spinal deformities have been found in these patients. Previous study in our hospital showed that 7 of 48 (14.6%) of DMD patients spent all their lives without apparent scoliosis (Cobb angle less than 30 degrees). These suggest that spinal fusion could be recommended for patients with Cobb angle more than 30 degrees and with % FVC more than 35%. Although the impact of spinal fusion upon the life expectancy remains unclear, favorable effect on respiratory function and quality of life can be expected for carefully selected patients with DMD.
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PMID:[The effects of spinal fusion on respiratory function and quality of life in Duchenne muscular dystrophy]. 916 37


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