Pulmonary function and scoliosis in children with spinal muscular atrophy types II and III

Chng, Seo Yi; Wong, YW; Hui, James; Wong, Hee Kit; Ong, Hian Tat; Goh, Daniel Yam Thiam

doi:10.1046/j.1440-1754.2003.00266.x

Cited by 70 publications

(49 citation statements)

References 18 publications

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“…Other studies have shown that an average correction of 40-64% in spinal curvature can be achieved, without much progression in the curve postoperatively [11,32,35]. Scoliosis in patients with flaccid neuromuscular pathology (DMD and SMA) is actually a kypho-scoliosis deformity, in comparison to other forms of neuromuscular scoliosis with spasticity where lumbar lordosis is usual [47].…”

Section: Discussionmentioning

confidence: 99%

Treatment and complications in flaccid neuromuscular scoliosis (Duchenne muscular dystrophy and spinal muscular atrophy) with posterior-only pedicle screw instrumentation

et al. 2009

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Literature has described treatment of flaccid neuromuscular scoliosis using different instrumentation; however, only one article has been published using posterior-only pedicle screw fixation. Complications using pedicle screws in paralytic neuromuscular scoliosis has not been described before. To present results and complications with posterior-only pedicle screws, a retrospective study was carried out in 27 consecutive patients with flaccid neuromuscular scoliosis (Duchenne muscular dystrophy and spinal muscular atrophy), who were operated between 2002 and 2006 using posterior-only pedicle screw instrumentation. Immediate postoperative and final followup results were compared using t test for Cobb angle, pelvic obliquity, thoracic kyphosis and lumbar lordosis. Perioperative and postoperative complications were noted from the hospital records of each patient. Complications, not described in literature, were discussed in detail. Average follow-up was 32.2 months. Preoperative, immediate postoperative and final follow-up Cobb angle were 79.8°, 30.2°(63.3% correction, p \ 0.0001) and 31.9°, respectively; and pelvic obliquity was 18.3°, 8.9°(52% correction, p \ 0.0001) and 8.9°. Postoperative thoracic kyphosis remained unchanged from 27.6°to 19.9°(p = 0.376); while lumbar lordosis improved significantly from ?15.6°t o -22.4°lordosis (p = 0.0002). Most patients had major to moderate improvement in postoperative functional and ambulatory status compared to the preoperative status. Thirteen (48.1%) perioperative complications were noted with five major complications (four respiratory in the form of hemothorax or respiratory failure that required ventilator support and one death) and eight minor complications (three UTI, two atelectasis, two neurological and one ileus). Postoperatively, we noted complications, such as coccygodynia with subluxation in 7, back sore on the convex side in 4 and dislodging of rod distally in 1 patient making a total of 12 (44.4%) postoperative complications. Of 12 postoperative complications, 6 (50%) required secondary procedure. We conclude that although flaccid neuromuscular scoliosis can be well corrected with posterior-only pedicle screw, there is a high rate of associated complications.

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Section: Discussionmentioning

confidence: 99%

Treatment and complications in flaccid neuromuscular scoliosis (Duchenne muscular dystrophy and spinal muscular atrophy) with posterior-only pedicle screw instrumentation

et al. 2009

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“…However, the severity of progression of scoliosis and pelvic obliquity seems more pronounced in SMA II than in SMA IIIa [44]. Studies on the course of pulmonary function after surgery for scoliosis are conflicting and seem to confirm a progressive decline in lung function despite spinal stabilization due to the progressive neuromuscular weakness associated with the disease [45].…”

Section: Scoliosismentioning

confidence: 99%

1st Italian SMA Family Association Consensus Meeting:

Sansone

Racca

Ottonello³

et al. 2015

Neuromuscular Disorders

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“…While numerous reports are available on respiratory involvement in Duchenne Muscular Dystrophy (DMD) or Spinal Muscle Atrophy (SMA) [1][2][3][4][5][6][7] only few studies have focused specifically on slowly progressive neuromuscular disorders such as Limb-Girdle Muscular Dystrophy (LGMD), Becker Muscular Dystrophy (BMD) and FacioScapulo Humeral Dystrophy (FSHD). LGMD patients generally show a mild global respiratory muscle weakness that usually affects expiratory more than inspiratory muscles, in the absence of significant involvement of the diaphragm [8,9].…”

Section: Introductionmentioning

confidence: 99%