Spinal fusion in patients with Duchenne's muscular dystrophy and a low forced vital capacity

Marsh, Andrew; Edge, G.; Lehovsky, Jan

doi:10.1007/s00586-003-0545-8

Cited by 67 publications

(54 citation statements)

References 27 publications

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“…Duchenne muscular dystrophy (DMD) is one of the most common and severe muscular dystrophies [11,18,22,28]. Cardio-respiratory complications lead to death in late adolescence or early adulthood.…”

Section: Introductionmentioning

confidence: 99%

“…The resulting pelvic obliquity together with weakness of the spinal musculature impairs the sitting ability in wheelchair. Bracing is known to be ineffective to stop the progression of scoliosis in these children [9,28]. Spinal stabilisation is recommended to correct the spinal deformity, pelvic obliquity and to prevent further progression.…”

Section: Introductionmentioning

confidence: 99%

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Scoliosis correction with pedicle screws in Duchenne muscular dystrophy

et al. 2007

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This report describes the spinal fixation with pedicle-screw-alone constructs for the posterior correction of scoliosis in patients suffering from Duchene 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°to 10°, pelvic tilt improved 65% from 14°to 3°. Lumbar lordosis improved significantly from 20°to 49°, 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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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Scoliosis correction with pedicle screws in Duchenne muscular dystrophy

et al. 2007

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“…Duchenne muscular dystrophy (DMD) is the most common and severe form of muscular dystrophy and is a flaccid neuromuscular disorder in which gradual deterioration is the hallmark [7,11,19,22,27,29,33]. Spinal deformity such as scoliosis is almost universal in patients with DMD [7,11,19,22,27].…”

Section: Introductionmentioning

confidence: 99%

“…Natural history studies on neuromuscular scoliosis demonstrated an almost invariable progression [2,18,31,33]. The progression of untreated spinal deformity in this population causes increasing pain [14,35], difficulties in sitting [2,9,22,27], decreased pulmonary function [4,27] and an increase in mortality [28]. Surgical management is the most reliable treatment for this deformity.…”

Section: Introductionmentioning

confidence: 99%

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Can the caudal extent of fusion in the surgical treatment of scoliosis in Duchenne muscular dystrophy be stopped at lumbar 5?

et al. 2010

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Instrumentation and fusion to the sacrum/pelvis has been a mainstay in the surgical treatment of scoliosis in Duchenne muscular dystrophy (DMD) and is recommended to correct pelvic obliquity. The caudal extent of instrumentation and fusion in the surgical treatment of scoliosis in DMD has remained a matter of considerable debate, and there have been few studies on the use of segmental pedicle screw instrumentation for this pathology. From 2004 to 2007, a total of 28 patients with DMD underwent segmental pedicle screw instrumentation and fusion only to L5. Assessment was performed clinically and with radiologic measurements. All patients had a curve with the apex at L2 or higher preoperatively. Preoperative coronal curve averaged 74°, with a postoperative mean of 14°, and 17°at the last follow-up. The pelvic obliquity improved from 17°pre-operatively to 6°postoperatively, and 6°at the last follow-up. Good sagittal plane alignment was recreated after surgery and maintained long term. In 23 patients with a preoperative L5 tilt of less than 15°, the pelvic obliquity was effectively corrected to less than 10°and maintained by adequately addressing spinal deformity, while five patients with a preoperative L5 tilt of more than 15°had a postoperative pelvic obliquity of more than 15°. Segmental pedicle screw instrumentation and fusion to L5 was effective and safe in patients with DMD scoliosis with a minimal L5 tilt (\15°) and a curve with the apex at L2 or higher, both initially and long term, obviating the need for fixation to the sacrum/pelvis. Segmental pedicle screw instrumentation and fusion to L5 was safe and effective in patients with DMD scoliosis with stable L5/ S1 articulation as evidenced by a minimal L5 tilt of less than 15°, even though pelvic obliquity was significant. There was no major complication. With rigid segmental pedicle screw instrumentation, the caudal extent of fusion in the treatment of DMD scoliosis should be determined by the degree of L5 tilt. This method in appropriate patients can be a viable alternative to instrumentation and fusion to the sacrum/pelvis in the surgical treatment of DMD scoliosis.

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Surgery for scoliosis in Duchenne muscular dystrophy

Cheuk

Wong

Wraige

et al. 2007

Cochrane Database of Systematic Reviews

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Background Scoliosis in people with Duchenne muscular dystrophy is usually progressive and treated with surgery. However, it is unclear whether the existing evidence is sufficiently scientifically rigorous to support a recommendation for spinal surgery for most people with Duchenne muscular dystrophy and scoliosis. This is an updated review and an updated search was undertaken in which no new studies were found. Objectives To determine the effectiveness and safety of spinal surgery in people with Duchenne muscular dystrophy with scoliosis. We intended to test whether spinal surgery is effective in increasing survival, improving respiratory function, improving quality of life and overall functioning; and whether spinal surgery is associated with severe adverse effects.

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Spinal fusion in patients with Duchenne's muscular dystrophy and a low forced vital capacity

Cited by 67 publications

References 27 publications

Scoliosis correction with pedicle screws in Duchenne muscular dystrophy

Scoliosis correction with pedicle screws in Duchenne muscular dystrophy

Can the caudal extent of fusion in the surgical treatment of scoliosis in Duchenne muscular dystrophy be stopped at lumbar 5?

Surgery for scoliosis in Duchenne muscular dystrophy

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