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

Dysphagia and aspiration seem to be rare in Duchenne muscular dystrophy, but cachexia can be associated with early death. Commonly, weight loss can be attributed to inadequate caloric intake caused by loss of ability to self-feed and/or fatigue. Our objective was to determine whether scoliosis repair is associated with malnutrition. A retrospective chart review was undertaken of patients with Duchenne muscular dystrophy, including those who underwent operative repair of scoliosis. We identified nine boys who lost > 5% body weight within 12 months of surgery. Eight patients who gained weight after surgery and eight patients of comparable age who had no surgery served as control subjects. All patients had no change in biceps strength after surgery, but those who lost weight were unable to self-feed. We found that weight loss after surgery was associated with loss of self-feeding. We conclude that pre- and postoperative management of patients with Duchenne muscular dystrophy should include feeding evaluation and determination of postural changes.
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PMID:Postoperative malnutrition in Duchenne muscular dystrophy. 1266 33

Steroids may have a beneficial effect on the course of Duchenne muscular dystrophy (DMD). However, results vary in different studies. This study consisted of 66 DMD boys who were in the therapy group and 22 DMD boys in the control group. The mean ages were 6.8 +/- 2.1 years (range 2.5-12.5) and 7.0 +/- 1.3 years (range 5.0-9.0), respectively. We assessed muscle strength, 10-m walking, ankle contracture, and loss of independent walking ability age and onset of scoliosis. Treatment regimen was oral prednisolone 0.75 mg/kg on alternate days, plus vitamin D 600-1200 units/day and a calcium-enriched diet. After a follow-up period of 2.75 +/- 1.1 years (range 1.5-5) and when compared with controls, there was a statistically significant change in muscle strength between the two groups after 12 months (P < 0.05). Although 10-m walking time decreased in therapy group (P < 0.05), there was not significance between the groups in the end. Boys in the control group developed significantly less ankle contractures (P < 0.05). None of the therapy group had scoliosis during the follow-up period (mean age 10.8 +/- 1.2 years), whereas seven boys of the control group had scoliosis at a mean age of 11.7 +/- 2 years. Loss of walking ability age was statistically different between groups (P < 0.05). Our results indicate that, alternate-day prednisolone regimen may prolong ambulation and scoliosis can be delayed or prevented.
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PMID:Prednisolone therapy in Duchenne muscular dystrophy prolongs ambulation and prevents scoliosis. 1527 99

This article reviews the extent of blood loss in spine surgery for scoliosis corrections in the pediatric age group. An extensive literature review presents blood loss values in surgery for adolescent idiopathic scoliosis, cerebral palsy, Duchenne muscular dystrophy, spinal muscular atrophy, and myelomeningocoele. The underlying disorder plays a major role in determining the extent of blood loss. Blood loss is considerably higher in those patients with a neuromuscular scoliosis compared with adolescent idiopathic scoliosis. Within the neuromuscular group those with Duchenne muscular dystrophy demonstrate the highest mean levels of blood loss. Blood loss is also shown to be progressively greater with increasing numbers of vertebral levels incorporated into the fusion, with posterior fusions compared to anterior fusions, and in those patients having both anterior and posterior fusions.
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PMID:Blood loss in pediatric spine surgery. 1531 83

We compare the long-term benefits and side effects of deflazacort using two treatment protocols from Naples (N) and Toronto (T). Boys with Duchenne muscular dystrophy between the ages of 8 and 15 years and who had four or more years of deflazacort treatment were reviewed. Diagnostic criteria included males with proximal muscle weakness evident before 5 years, increased serum creatine kinase and genetic testing and/or a muscle biopsy consistent with Duchenne muscular dystrophy. Thirty-seven boys were treated with protocol-N using deflazacort at a dose of 0.6 mg/kg per day for the first 20 days of the month and no deflazacort for the remainder of the month. Boys with osteoporosis received daily vitamin D and calcium. Deflazacort treatment started between 4 and 8 years of age. Thirty-two were treated with protocol-T using deflazacort at a dose of 0.9 mg/kg per day, plus daily vitamin D and calcium. Treatment started between 6 and 8 years of age. All boys were monitored every 4-6 months. The results were compared with age-matched controls in the two groups (19 for protocol-N and 30 for protocol-T). For the boys treated with protocol-N, 97% were ambulatory at 9 years (control, 22%), 35% at 12 years (control, 0%), 25% at 15 years (control, 0%). For the 32 boys treated with protocol-T, 100% were ambulatory at 9 years (control, 48%), 83% at 12 years (control, 0%) and 77% at 15 years (control, 0%). No aids or leg braces were used for ambulation. In boys 13 years and older, a scoliosis of >20 degrees developed in 30% of the boys on protocol-N, 16% on protocol-T and 90% of controls. For protocol-N, no cataracts were observed while in protocol-T, 30% of boys had asymptomatic cataracts that required no treatment. Fractures occurred in 19% (control 16%) of boys on protocol-N and 16% (control, 20%) of boys on protocol-T. This report illustrates: (a) the importance of collaborative studies in developing treatment protocols in Duchenne muscular dystrophy and (b) the long-term beneficial effects of deflazacort treatment in both protocols. However, the protocol-T seems to be more effective and frequently is associated with asymptomatic cataracts.
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PMID:Deflazacort in Duchenne muscular dystrophy: a comparison of two different protocols. 1533 88

A defective, normal or enhanced hemostasis has been reported in Duchenne muscular dystrophy (DMD). A retrospective analysis of intra-and postoperative (up to 36 h) estimated blood losses was performed in 156 patients undergoing spinal surgery for: DMD (n = 31), idiopathic scoliosis (IS) (n = 70), poliomyelitis (n = 10), cerebral palsy (CP) (n = 28), spinal muscular atrophy (SMA) (n = 17). Platelet aggregation and bleeding times were also investigated in DMD patients. Immunohistochemistry for dystrophin was performed in platelets, megakaryocytes and blood vessels of normal tissues. DMD patients showed significantly higher intraoperative estimated blood losses (DMD: 3495+/-890 ml; IS: 2269+/-804 ml; poliomyelitis: 2582+/-1252 ml; CP: 2071+/-683 ml; SMA: 2464+/-806 ml; P < 0.05), while postoperative blood losses were similar among different groups. Higher estimated blood losses in DMD were independent of the duration of surgery, body weight, gender, age, vertebral levels or preoperative Cobb angle. DMD children had significantly prolonged bleeding times, but retained normal platelet function. From control samples dystrophin was expressed in vascular smooth muscle cells, but not in platelets. DMD appears to be characterized by immediate bleeding during highly-invasive surgery and increased bleeding time without platelet abnormalities. Considering dystrophin expression in normal vascular smooth muscle cells, these results altogether suggest a selective defect of primary hemostasis in DMD, likely to be due to impaired vessel reactivity.
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PMID:Impaired primary hemostasis with normal platelet function in Duchenne muscular dystrophy during highly-invasive spinal surgery. 1600 51

A tracheo-arterial fistula is a serious and life threatening potential complication of a tracheostomy. Since 1984, we experienced nine fatal cases of tracheo-arterial fistula among 60 Duchenne muscular dystrophy (DMD) patients who underwent a tracheostomy. Representative cases included a patient with lordosis (Case 8), in whom the fistula was located in the brachiocephalic artery close to the trachea, and another with severe scoliosis (Case 9), which caused the aorta to compress the trachea. Such anatomical changes can be the cause of a fistula between the trachea and brachiocephalic artery. The anatomical locations between the trachea and brachiocephalic artery are modified by thoracic deformities in DMD patients, and should be confirmed using computed tomography (CT) prior to a tracheostomy procedure. Further, during such a procedure, the tracheal stoma must be placed in a location clearly away from the arteries, and should be followed by regular post-operative examinations using CT and careful management to avoid a tracheo-arterial fistula.
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PMID:Tracheo-arterial fistula in tracheostomy patients with Duchenne muscular dystrophy. 1636 6

Scoliosis affects 75 to 90% of patients with non-ambulant Duchenne muscular dystrophy (DMD). Spinal surgery is the treatment of choice but the indication varies among centres. Some offer surgery to all non-ambulant patients, irrespective of scoliosis severity. Early surgery has the advantage of targeting DMD when cardiorespiratory function is preserved, but not all patients develop scoliosis. We report our 10-year experience of scoliosis management in 123 patients with DMD who were at least 17 years old at the time of the study. Scoliosis was absent in 10%, and mild, non-progressive (at least 30 degrees ) in 13% of patients. Another 13% had moderate scoliosis (31-50 degrees ) and were managed conservatively. Surgery was considered in 57% (70/123) of patients with scoliosis greater than 50 degrees and eventually performed in 35%. The remaining patients either refused surgery (9%) or were unfit because of cardiorespiratory compromise (13%). In a further 7%, scoliosis (greater than 50 degrees ), first noted after 14 years of age, was progressing slowly and surgery was not performed. At 17 years there was no difference in survival, respiratory impairment, or sitting comfort among patients managed conservatively or with surgery. One-third (44/123) of our patients were managed satisfactorily without receiving spinal surgery. We provide insight into the natural history of scoliosis in DMD that should help families and clinicians with decision-making when surgery is considered.
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PMID:Management of scoliosis in Duchenne muscular dystrophy: a large 10-year retrospective study. 1720 69

Duchenne muscular dystrophy (DMD) is an X-linked recessive disease that affects approximately 1 in 3500 male births. Boys with Duchenne have a progressive and predictable muscle deterioration: muscles lack dystrophin, a protein essential for membrane stability, whose absence induces contraction-related membrane damage and activation of the inflammatory cascade leading to muscle failure, necrosis, fibrosis. Although DMD is present at birth, clinical symptoms are not evident until 2-6 years of age. Initial symptoms include leg weakness, increasing spine kyphosis, and a waddle-like gait. Continuous muscle wasting leads to progressively weaker muscles, usually leading DMD patients on wheelchair by the age of 8-12. Scoliosis develops in 90% of boys who use a wheelchair full-time. Progression of muscle degeneration and worsening clinical symptoms lead to death in the late twenties from respiratory/cardiac failure.
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PMID:[Duchenne muscular dystrophy: rational basis, state of the art]. 1701 93

This report describes the spinal fixation with pedicle-screw-alone constructs for the posterior correction of scoliosis in patients suffering from Duchenne muscular dystrophy (DMD). Twenty consecutive patients were prospectively followed up for an average of 5.2 years (min 2 years). All patients were instrumented from T3/T4 to the pelvis. Pelvic fixation was done with iliac screws similar to Galveston technique. The combination of L5 pedicle screws and iliac screws provided a stable caudal foundation. An average of 16 pedicle screws was used per patient. The mean total blood loss was 3.7 l, stay at the intensive care unit was 77 h and hospital stay was 19 days. Rigid stabilisation allowed immediate mobilisation of the patient in the wheel chair. Cobb angle improved 77% from 44 degrees to 10 degrees, pelvic tilt improved 65% from 14 degrees to 3 degrees. Lumbar lordosis improved significantly from 20 degrees to 49 degrees, thoracic kyphosis remained unchanged. No problems related to iliac fixation, no pseudarthrosis or implant failures were observed. The average percentage of predicted forced vital capacity (%FVC) of the patients was 55% (22-94%) preoperatively and decreased to 44% at the last follow-up. There were no pulmonary complications. One patient with a known cardiomyopathy died intraoperatively due to a sudden cardiac arrest. The rigid primary stability with pedicle screws allowed early mobilisation of the patients, which helped to avoid pulmonary complications.
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PMID:Scoliosis correction with pedicle screws in Duchenne muscular dystrophy. 1805 66

The development of scoliosis in boys with Duchenne Muscular Dystrophy (DMD) is a significant, morbid event in the progression of the disease caused by progressive spinal musculature weakness. As an alternative to muscle activity, the spine can also be stabilised by locking the articular facet joints, which is achieved when the body is supported on a seat tilted anteriorly using a 'wedge', of the kind commonly recommended for low back pain. We tested spinal stability when using a seat tilted 15 degrees anteriorly in eight boys with DMD, without significant scoliosis, by measuring the ability to support a lateral load applied to the thorax through a sling and hawser. All eight boys tolerated lateral loading better with wedged seating and were able to support an average additional load of 95 g per kilogram of body weight compared to normal seating. Lateral load bearing was improved in 10 normal control boys by an average of 40 g per kilogram of body weight. These encouraging pilot findings indicate that there is a need for further studies on the effectiveness of passive mechanical factors in spinal stabilisation to delay the development of scoliosis in boys with DMD.
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PMID:Spinal stability is improved by inducing a lumbar lordosis in boys with Duchenne Muscular Dystrophy: a pilot study. 1809 90


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