Lung function in Duchenne muscular dystrophy

Galasko, C. S. B.; Williamson, J. B.; Delaney, Claire

doi:10.1007/bf00301031

Cited by 82 publications

(48 citation statements)

References 23 publications

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“…Surgical treatment of scoliosis in DMD is mainly performed to restore the balance of the spinal column in both coronal and sagittal planes and to improve table top activities. Surgery has also been advocated to improve lung compliance; however this is controversial [18][19][20]. Surgical treatment can halt progression of deformity in patients with DMD, however, their compromised general condition, quality and requirements of life, and associated medical comorbidities should be taken into account prior to embarking on such major surgical procedures.…”

Section: Discussionmentioning

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: Discussionmentioning

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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“…Most patients with DMD develop a progressive scoliosis starting at the time of loss of ambulatory status [1,3,5,7,9,12,13,15,18,19,20,22,23]. This occurs in 50-95% of patients, and is associated with a deteriorating vital capacity (4% for every 10°Cobb angle).…”

Section: Introductionmentioning

confidence: 99%

“…Independent of the scoliosis, vital capacity decreases by 4% per year following loss of ambulation, and this is due to a progressive respiratory muscle weakness. Progression of the scoliosis and an associated pelvic obliquity cause problems with posture and seating to the extent that quality of life may be severely impaired [1,3,5,6,7,9,12,15,16,17,18,19,20,22,23].…”

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 Galasko et al [17,18], VC decreases by 8% per year in patients with scoliosis secondary to DMD. No brace can prevent the progression of this kind of spinal deformity.…”

Section: Introductionmentioning

confidence: 99%

“…From the onset, progression can be extremely rapid (Fig. 1) and in all cases seriously affects the unsupported sitting ability and deteriorates the respiratory function, already compromised by the disease [18,21,22,27,49].…”

Section: Introductionmentioning

confidence: 99%

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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Lung function in Duchenne muscular dystrophy

Cited by 82 publications

References 23 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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