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

The x-linked Duchenne muscular dystrophy (DMD) is the most frequent generalized muscle disorder arising from a lack of the sarcolemmic protein "dystrophin". Patients with DMD develop in the majority a progressive scoliosis when they cease walking and/or standing at the age of 10 years and become confined to a wheelchair. Increasing muscle weakness leads to a progression of the curvature, the pelvic tilt and problems in sitting. Together with the simultaneous progressive weakness of the respiratory muscles a restrictive pulmonary insufficiency will occur. Surgical stabilization of the spine (> 20 degrees Cobb, forced vital capacity > 35%) by an adequate multisegmental instrumentation enabling early mobilization is now the treatment of choice. However, orthotic treatment may offer an acceptable compromise in exceptional cases, if the patient rejects surgical intervention or is in the late (inoperable) stages of the disease. Such a treatment is superior to a primary sitting support provision with insufficient possibilities of correction. The authors' experiences with 48 scoliosis orthoses made for 28 patients with DMD are reported. A "double plaster" cast has emerged as the best method to optimize adaption, especially in severe curvatures and the time taken for manufacturing the orthosis. A great deal of experience, patience and the consideration of the patients' individual demands are inevitable for a successful orthotic treatment.
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PMID:Scoliosis in Duchenne muscular dystrophy: aspects of orthotic treatment. 945 95

Patients with Duchenne muscular dystrophy (DMD) tend to bleed more during surgery than do patients with other conditions. A retrospective analysis of blood loss after spinal surgery for scoliosis was therefore undertaken in 102 patients undergoing surgery in the senior author's unit. These included 48 patients with DMD, 26 patients with spinal muscular atrophy, and a miscellaneous group of 28 other patients most of whom had idiopathic scoliosis. For each patient the age at surgery, estimated blood volume, duration of operation, Cobb angle, and number of vertebrae fused were recorded and compared. Expression of dystrophin in skeletal muscle and the underlying gene mutation were also determined. The estimated blood loss in patients with DMD was significantly higher than that in patients with spinal muscular atrophy undergoing the same or similar procedure (P < 0.005) and was also significantly greater than that of the third group, which consisted mostly of patients with idiopathic scoliosis (P < 0.0005). Blood loss in the patient group with DMD showed a significant relationship with duration of surgery (P < 0.05). As most patients expressed no dystrophin, this did not correlate with the estimated blood loss. There was also no correlation between the estimated blood loss and the type of gene mutation found causing DMD. The authors' previous observations confirm the increased blood loss in patients with DMD who undergo scoliosis surgery. Because children with DMD lack dystrophin in all muscle types, including smooth muscle, the excessive blood loss may be because of a poor vascular smooth muscle vaso-constrictive response due to a lack of dystrophin.
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PMID:Blood loss in Duchenne muscular dystrophy: vascular smooth muscle dysfunction? 1039 27

Spinal fusion, ending caudally at L5 rather than at the sacrum, is recommended for selected patients with scoliosis due to Duchenne muscular dystrophy. We present a retrospective review of 48 patients operated on for this condition. Patients having spinal curvature with a Cobb angle of less than 40 degrees and with less than 10 degrees between a line tangential to the superior margins of both iliac crests and a line perpendicular to the spinous processes of L4 and L5, were fused to L5 (38 patients); patients not meeting these criteria were fused to the sacrum (10 patients). Spinal and sitting obliquity increased in patients fused to L5, rather than to the sacrum, but the severity of the worsening obliquity was significantly greater in patients in whom the apex of the curve was below L1. Two of the ten latter patients required revision procedures for worsening obliquity when their pulmonary function deteriorated to less than 25% of predicted values. We recommend fusion to the sacrum for scoliosis in Duchenne muscular dystrophy, especially for patients with an apex to their curve below L1.
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PMID:Pelvic obliquity after fusion of the spine in Duchenne muscular dystrophy. 1053 Aug 43

The principles of the operative treatment of neuromuscular scolioses differ from those of idiopathic scolioses. Depending upon the deformity in the frontal and sagittal plane, the amount of pelvic obliquity and especially the etiology of the curve, consideration of a posterior, an anterior or a combined anterior-posterior procedure is necessary. Statistics demonstrate a higher preoperative angle and a higher rate of complications combined with worse corrections in comparison with idiopathic scolioses. The existing deterioration of vital capacity in patients with Duchenne muscular dystrophy, as in patients with spinal muscle atrophy, makes an anterior approach impossible. The correction of a severe pelvic obliquity combined with a rigid lumbar or thoracolumbar scoliosis requires a combined approach in most patients suffering from myelomeningcele (MMC) and cerebral palsy. In neurofibromatosis Recklinghausen associated with an angulated kyphotic curve the anterior approach is mandatory to avoid further deterioration. Multisegmental primary-stable anterior or posterior instrumentations allow postoperative care without external support.
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PMID:One-stage versus two-stage spinal fusion in neuromuscular scolioses. 1114 65

The aim of this study was to report results of prophylactic spinal stabilization in patients with Duchenne muscular dystrophy. There is still debate regarding the ideal instrumentation. A prospective study of a consecutive series of 31 patients stabilized with the ISOLA system from D2 to S1 will be presented. The mean follow-up was 22 months (range, 1-60 months). The evaluation of the Cobb angle and pelvic obliquity revealed the following: 1) Cobb angle: preoperation, 48.6 degrees (range, 22-82 degrees); postoperation, 12.5 degrees (range, 0-30 degrees); follow-up, 12.5 degrees (range, 0-42 degrees); and 2) pelvic obliquity: preoperation, 18.2 degrees (range, 3-40 degrees); postoperation, 3.8 degrees (range, 0-13 degrees); follow-up, 5.1 degrees (range, 0-14 degrees). Spinal stabilization with the ISOLA system was found to be a suitable treatment for scoliosis owing to Duchenne muscular dystrophy. It should be carried out after loss of ambulation as soon as a progressive curve of more than 20 degrees is documented. The complication rate was found to be high.
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PMID:Spinal stabilization in Duchenne muscular dystrophy: principles of treatment and record of 31 operative treated cases. 1126 6

Although hip subluxation and dislocation occur commonly in neuromuscular diseases such as cerebral palsy, spinal muscular atrophy and myelomeningocele, they are not known to commonly affect patients with Duchenne Muscular Dystrophy. In this study, 15 out of 54 patients whose hip radiographs were reviewed showed unilateral subluxation, one had bilateral subluxation and three had unilateral dislocation. Having established that hip subluxation and dislocation develop frequently in patients with Duchenne Muscular Dystrophy, serial hip radiographs should be taken as has been advocated for cerebral palsy. The possible relationship between subluxation and pelvic tilt also calls for better control of sitting posture to prevent pelvic tilt. Spinal stabilization should be carried out at an early age when any scoliosis and pelvic tilt are still mild, so that progressive subluxation of the hip may be delayed or prevented, in addition to maintaining sitting balance and comfort.
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PMID:Hip subluxation and dislocation in Duchenne muscular dystrophy. 1149 66

Muscular dystrophy is a collective group of inherited, noninflammatory, progressive muscle wasting diseases. The initial pathologic feature is an abnormality in the genetic code for dystrophin or one of its associated glycoproteins, which leads to the various clinical syndromes. Despite minor variations between the different types, all muscular dystrophies have in common progressive muscle weakness, which is best typified by Duchenne muscular dystrophy. The weakness occurs in a proximal to distal direction and can compromise ambulatory status as well as cardiopulmonary function. Additionally, structural soft tissue contractures and spinal deformities may develop from poor posturing secondary to the progressive muscle weakness and imbalance. The rapidly developing scoliosis and its associated pelvic obliquity can even compromise sitting. Recent advances in molecular biology and gene therapy research raise the hope for a cure for muscular dystrophy in the near future. Until that time, however, the role of orthopedic surgeons in treating patients with muscular dystrophy is to preserve or prolong their functional status for as long as possible. This can be achieved by physical therapy, bracing, soft tissue releases for joint contractures, and early stabilization of the spine.
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PMID:Orthopedic management of the muscular dystrophies. 1188 Jul 34

Duchenne muscular dystrophy is an X-linked disease of muscle caused by an absence of the protein dystrophin. Affected boys begin manifesting signs of disease early in life, cease walking at the beginning of the second decade, and usually die by age 20 years. Until treatment of the basic genetic defect is available, medical, surgical, and rehabilitative approaches can be used to maintain patient function and comfort. Corticosteroids, including prednisone and a related compound, deflazacort, have recently been shown to markedly delay the loss of muscle strength and function in boys with Duchenne muscular dystrophy. Surgical release of lower extremity contractures may benefit some patients. Approximately 90% of boys with Duchenne muscular dystrophy will develop severe scoliosis, which is not amenable to control by nonsurgical means such as bracing or adaptive seating. The most effective treatment for severe scoliosis is prevention by intervening with early spinal fusion utilizing segmental instrumentation as soon as curves are ascertained and before the onset of severe pulmonary or cardiac dysfunction.
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PMID:Duchenne muscular dystrophy. 1192 8

A 12-year-old boy with Duchenne muscular dystrophy underwent posterior spinal fusion for progressive scoliosis. Preoperative evaluation was focused on respiratory function as well as cardiac function, which revealed markedly reduced respiratory reserve (FVC 0.77 l, %FVC 25.9%, FEV1.0 0.48 l, %FEV1.0 62%) and well-preserved biventricular function. A possible association between malignant hyperthermia and Duchenne muscular dystrophy has been documented. Thus anesthesia was administered without triggering agents. Propofol and fentanyl were used for induction and maintenance of anesthesia, and the patient was ventilated with O2-nitrous oxide mixture. The anesthesia machine, prepared by using a disposable circuit and fresh CO2-absorbent and disconnecting the vaporizers, was flushed with O2 at a rate of 10 l.min-1 for 20 minutes before use. A small dose of vecuronium was administered while monitoring the train-of-four ratio. The bispectral index (BIS) was utilized to optimize the depth of anesthesia so that the wake-up test could be performed promptly on surgeon's request while avoiding the intraoperative awareness. The BIS was helpful in continuously assessing the wakening process. BIS increased from 40's to 80's in 15 minutes after discontinuation of propofol and nitrous oxide during the test. The patient was kept under close observation postoperatively without any sign of malignant hyperthermia.
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PMID:[Application of bispectral index (BIS) monitoring to anesthetic management of posterior spinal fusion in a patient with Duchenne muscular dystrophy]. 1216 84

Neuromuscular and chest wall disorders are individually uncommon but together form an important group of conditions that can lead to chronic ventilatory failure. This is best recognised in scoliosis, kyphosis, following a thoracoplasty, in muscular dystrophies, such as Duchenne muscular dystrophy (DMD), and myotonic dystrophy, after poliomyelitis and with motor neurone disease (amyotrophic lateral sclerosis). If bulbar function is impaired, tracheostomy ventilation may be required, but in other situations, noninvasive ventilation is preferable. Positive pressure techniques using nasal and face masks are usually the first choice, but negative pressure ventilation is an alternative. There are no randomised-controlled trials regarding the indications for initiating noninvasive ventilation, but this is usually provided if there are symptoms due to nocturnal hypoventilation or right heart failure in the presence of a raised carbon dioxide tension in arterial blood (Pa,CO2) either at night or, more usually, in the daytime as well. There is no evidence that "prophylactic" ventilatory support is of benefit if this is provided before ventilatory failure has appeared. Careful selection of patients is required, especially in the presence of progressive neuromuscular disorders such as DMD and motor neurone disease. There are no randomised-controlled trials concerning the outcome of noninvasive ventilation in these conditions, but studies have shown an improved quality of life, physical activity and haemodynamics, normalisation of blood gases and slight improvement in other physiological measures, such as the vital capacity and maximal mouth pressures. Survival in chest wall disorders is approximately 90% at 1 yr and 80% at 5 yrs, and similar figures have been obtained in nonprogressive neuromuscular conditions. If, however, the underlying disorder is deteriorating, particularly if it involves the bulbar muscles, it may limit survival despite the provision of adequate noninvasive ventilatory support.
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PMID:Noninvasive ventilation for chest wall and neuromuscular disorders. 1508 69


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