The treatment of scoliosis in duchenne muscular dystrophy

Rideau, Y; Glorion, B.; Delaubier, Anne; Tarlé, O.; Bach, John R.

doi:10.1002/mus.880070405

Cited by 103 publications

(51 citation statements)

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“…There has been much debate about the indications and timing of surgery in DMD but there is now a general concurrence that spinal deformity correction should be undertaken prior to onset of cardio-respiratory compromise [3,4]. There have been suggestions that surgery should be undertaken if the Cobb angle is between 20 and 50° [5][6][7]. Early operation has been shown to improve outcome in long term follow up studies [3,4,8,9].…”

Section: Introductionmentioning

confidence: 99%

Scoliosis in Duchenne’s muscular dystrophy: a changing trend in surgical management

Arun

Srinivas²,

Mehdian³

2009

Eur Spine J

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A non-randomised retrospective study to compare the results of surgical correction of scoliosis in Duchenne's muscular dystrophy (DMD) patients using three different instrumentation systems-Sublaminar instrumentation system (Group A), a hybrid of sublaminar and pedicle screw systems (Group B) and pedicle screw system alone (Group C). Between 1993 and 2003, 43 patients with DMD underwent posterior spinal fusion and instrumentation. Group A (n = 19) had sublaminar instrumentation system, Group B (n = 13) had a hybrid construct and Group C (n = 11) was treated with pedicle system. The mean blood loss in Group A was 4.1 l, 3.2 l in Group B and 2.5 l in Group C. Average operating times in Group A, B and C were 300, 274 and 234 min, respectively. Mean pre-operative, post-operative and final Cobb angle in Group A was 50.05 ± 15.46°, 15.68 ± 11.23°and 21.57 ± 11.63°, Group B was 17.76 ± 8.50°, 3.61 ± 2.53°and 6.69 ± 4.19°and Group C was 25.81 ± 9.94°, 5.45 ± 3.88°, 8.90 ± 5.82°, respectively. Flexibility index or the potential correction calculated from bending radiographs were 60 ± 6.33, 70 ± 4.65 and 67 ± 6.79% for Group A, Group B and Group C respectively. The percentage correction achieved was 72.5 ± 14.5% in Group A, 82 ± 6% in Group B and 82 ± 8% in Group C. The difference between percentage correction achieved and the flexibility index was 12.45 ± 8.22, 12.05 ± 1.3 and 15.00 ± 1.21% in Group A, B and C, respectively The percentage loss of correction in Cobb angles at final follow-up in Group A, B and C was 12.5 ± 3.5, 16.5 ± 1. and 12.5 ± 2.5%, respectively. Complications seen in Group A were three cases of wound infection and two cases of implant failure; Group B had a single case of implant failure and Group C had one patient with wound infection and one case with a partial screw pull out. Early surgery and smaller curve corrections appears to be the current trend in the management of scoliosis in DMD. This has been possible due to early curve detection and surgery thus having the advantage of less post-operative respiratory complications and stay in paediatric intensive care. Also, early surgery avoids development of pelvic deformity and extension of instrumentation to the pelvis thereby reducing blood loss. This trend reflects the advent of newer and safer instrumentation systems, advanced techniques in anaesthesia and cord monitoring. Sublaminar instrumentation system group had increased operating times and blood loss compared to both the hybrid and pedicle screw instrumentation systems due to increased bleeding from epidural vessels and pelvic instrumentation. Overall, the three instrumentation constructs appear to provide and maintain an optimal degree of correction at medium to long term follow up but the advantages of lesser blood loss and surgical time without the need for pelvic fixation seem to swing the verdict in favour of the pedicle screw system.

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Section: Introductionmentioning

confidence: 99%

Scoliosis in Duchenne’s muscular dystrophy: a changing trend in surgical management

Arun

Srinivas²,

Mehdian³

2009

Eur Spine J

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“…It is the most common of the dystrophies (1 in 5000 live male births) and is characterised by a progressive muscle wasting and weakness leading to a loss of ambulation by the early teens. Muscle weakness is due to lack of the protein dystrophin in skeletal muscle cell membranes, and this can be tested for by immunohistochemical techniques or by western blotting electrophoresis on a muscle biopsy specimen [3,7,9,10,13,15,16,17].…”

Section: Introductionmentioning

confidence: 99%

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

2003

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“…According to many authors, spinal fusion has no effects on the natural involution of the respiratory function [5, 33,35,36,47], both at short-term and at a 5-year follow-up [38]. In contrast, Rideau et al [44] and Galasko et al [17] reported a period of VC stabilisation in surgically treated patients of 24 and 36 months, respectively (Fig. 3).…”

Section: Introductionmentioning

confidence: 97%

“…Rideau et al [44] classified patients with DMD into three groups by their VC. They asserted that VC increases until the age of 10-12 years, until a plateau is reached; then it progressively decreases over the following years (Fig.…”

Section: Introductionmentioning

confidence: 99%

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Surgical treatment of spinal deformities in Duchenne muscular dystrophy: a long term follow-up study

et al. 2004

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IntroductionA spinal deformity is almost never observed in children with Duchenne muscular dystrophy (DMD) who can walk [48], and therefore its onset is usually registered between the ages of 12 and 13 years and rarely before the age of 11.Numerous factors are responsible for the onset of the deformity. According to Gibson and Wilkins [22] the main cause of spinal deformity is the paralysis of the extensor muscles.Rideau [44], Siegel [48] and Dubousset [10,11] considered the inclination of the pelvis to be the major cause of the deformity -in particular the asymmetry of the scar retractions at level with the lower limbs. Ledoux [30] foAbstract Background. Surgical treatment of spinal deformities in Duchenne muscular dystrophy (DMD) is influenced by a number of factors which have proven to be a difficult challenge. Each case should be carefully evaluated, considering not only the natural history of the spinal deformity, but also the patient's general condition. These should be thoroughly assessed through clinical and radiographic investigations together with other medical specialists. Life expectancy should be determined according to the cardio-respiratory function, and both preoperative and postoperative quality of life should be taken into consideration, trying to imagine the functional status of each patient after surgery. Methods. From February 1985 to February 2000, 58 patients with spinal deformity in DMD were surgically treated. Of 25 patients that were operated on between 1985 and 1995, only 20 were followed-up after 5 years because 5 of them had died during this time. Therefore, the present study focuses on the results obtained in 20 cases. The 20 cases reviewed presented with a mean angular value of scoliosis equal to 48°(range 10-92°). Spinal fusion with our modified Luque technique [6] was performed in 19 cases, whereas CD instrumentation was applied in only one case. Results. At the 5 year follow-up (range 5.6-10 years), the age ranged from 18 to 24 years and averaged 20.4 years. The postoperative angular value of scoliosis averaged 22°(58%, range 0-43°), the mean correction at follow-up was 28°(range 0-60°), and the mean loss of correction was equal to 6°(range, 0-11°). Vital capacity showed a slow progression, slightly inferior to its natural evolution in untreated patients. The severest complication was the death that occurred in one of the patients. Conclusions. According to the present study, an early surgery (angular value lower than 35-40°) dramatically reduces the rate of risk factors associated with spinal deformities in DMD, and its advantages far exceed the disadvantages, above all in terms of quality of life.

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The treatment of scoliosis in duchenne muscular dystrophy

Cited by 103 publications

References 12 publications

Scoliosis in Duchenne’s muscular dystrophy: a changing trend in surgical management

Scoliosis in Duchenne’s muscular dystrophy: a changing trend in surgical management

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

Surgical treatment of spinal deformities in Duchenne muscular dystrophy: a long term follow-up study

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