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The effect of posterior spinal fusion on respiratory function in Duchenne muscular dystrophy

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Abstract

Purpose

Posterior instrumented spinal fusion is indicated for progressive scoliosis that develops in Duchenne muscular dystrophy (DMD) patients. Whilst spinal fusion is known to improve quality of life, there is inconsistency amongst the literature regarding its specific effect on respiratory function. Our objective was to determine the effect of scoliosis correction by posterior spinal fusion on respiratory function in a large cohort of patients with DMD. Patients with DMD undergoing posterior spinal fusion were compared to patients with DMD not undergoing surgical intervention.

Methods

An observational study of 65 patients with DMD associated scoliosis, born between 1961 and 2001: 28 of which underwent correction of scoliosis via posterior spinal fusion (Surgical Group) and 37 of which did not undergo surgical intervention (Non-Surgical Group). Pulmonary function was assessed using traditional spirometry. Comparisons were made between groups at set times, and by way of rates of change over time.

Results

There was no correlation between the level of respiratory dysfunction and the severity of scoliosis (as measured by Cobb angle) for the whole cohort. The Surgical Group had significantly worse respiratory function at a comparable age pre-operatively compared to the Non-Surgical Group, as measured by per cent predicted forced vital capacity (p = 0.02) on spirometry. The rate of decline of forced vital capacity and per cent predicted forced vital capacity was not slowed following surgery compared to the non-operated cases. There was no significant difference in survival between the two groups.

Conclusions

Severity of scoliosis was not a key determinant of respiratory dysfunction. Posterior spinal fusion did not reduce the rate of respiratory function decline. These two points suggest that intrinsic respiratory muscle weakness is the main determinant of decline in respiratory function in DMD.

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Notes

  1. FVC maximum volume of air exhaled from lungs, following maximal inspiration.

  2. %FVC FVC as a percentage of that predicted for a person of the same sex, age, and height.

References

  1. Hoffman EP, Fischbeck KH (1988) Characterization of dystrophin in muscle-biopsy specimens from patients with Duchenne’s or Becker’s muscular dystrophy. N Engl J Med 318(21):1363–1368

    Article  PubMed  CAS  Google Scholar 

  2. Marsh A, Edge G, Lehovsky J (2003) Spinal fusion in patients with Duchenne’s muscular dystrophy and a low forced vital capacity. Eur Spine J 12(5):507–512

    Article  PubMed  CAS  Google Scholar 

  3. Kennedy JD, Staples AJ, Brook PD et al (1995) Effect of spinal surgery on lung function in Duchenne muscular dystrophy. Thorax 50(11):1173–1178

    Article  PubMed  CAS  Google Scholar 

  4. Emery A, Muntoni F (2003) Duchenne muscular dystrophy. Oxford University Press, Oxford

    Google Scholar 

  5. Eagle M, Bourke J, Bullock R et al (2007) Managing Duchenne muscular dystrophy–the additive effect of spinal surgery and home nocturnal ventilation in improving survival. Neuromuscul Disord 17(6):470–475

    Article  PubMed  Google Scholar 

  6. Smith AD, Koreska J, Moseley CF (1989) Progression of scoliosis in Duchenne muscular dystrophy. J Bone Joint Surg Am 71(7):1066–1074

    PubMed  CAS  Google Scholar 

  7. Oda T, Shimizu N, Yonenobu K et al (1993) Longitudinal study of spinal deformity in Duchenne muscular dystrophy. J Pediatr Orthop 13(4):478–488

    Article  PubMed  CAS  Google Scholar 

  8. Khan Y, Heckmatt JZ (1994) Obstructive apnoeas in Duchenne muscular dystrophy. Thorax 49(2):157–161

    Article  PubMed  CAS  Google Scholar 

  9. Smith PE, Calverley PM, Edwards RH (1988) Hypoxemia during sleep in Duchenne muscular dystrophy. Am Rev Respir Dis 137(4):884–888

    PubMed  CAS  Google Scholar 

  10. Manzur AY, Kinali M, Muntoni F (2008) Update on the management of Duchenne muscular dystrophy. Arch Dis Child 93(11):986–990

    Article  PubMed  CAS  Google Scholar 

  11. Velasco MV, Colin AA, Zurakowski D et al (2007) Posterior spinal fusion for scoliosis in Duchenne muscular dystrophy diminishes the rate of respiratory decline. Spine (Phila Pa 1976) 32(4):459–465

    Article  Google Scholar 

  12. Simonds AK, Muntoni F, Heather S, Fielding S (1998) Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy. Thorax 53(11):949–952

    Article  PubMed  CAS  Google Scholar 

  13. Eagle M, Baudouin SV, Chandler C et al (2002) Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation. Neuromuscul Disord 12(10):926–929

    Article  PubMed  Google Scholar 

  14. Granata C, Merlini L, Cervellati S et al (1996) Long-term results of spine surgery in Duchenne muscular dystrophy. Neuromuscul Disord 6(1):61–68

    Article  PubMed  CAS  Google Scholar 

  15. Miller RG, Chalmers AC, Dao H et al (1991) The effect of spine fusion on respiratory function in Duchenne muscular dystrophy. Neurology 41(1):38–40

    Article  PubMed  CAS  Google Scholar 

  16. Sussman MD (1984) Advantage of early spinal stabilization and fusion in patients with Duchenne muscular dystrophy. J Pediatr Orthop 4(5):532–537

    PubMed  CAS  Google Scholar 

  17. Arun R, Srinivas S, Mehdian SM (2010) Scoliosis in Duchenne’s muscular dystrophy: a changing trend in surgical management: a historical surgical outcome study comparing sublaminar, hybrid and pedicle screw instrumentation systems. Eur Spine J 19(3):376–383

    Article  PubMed  Google Scholar 

  18. Rideau Y, Glorion B, Delaubier A et al (1984) The treatment of scoliosis in Duchenne muscular dystrophy. Muscle Nerve 7(4):281–286

    Article  PubMed  CAS  Google Scholar 

  19. Brook PD, Kennedy JD, Stern LM et al (1996) Spinal fusion in Duchenne’s muscular dystrophy. J Pediatr Orthop 16(3):324–331

    Article  PubMed  CAS  Google Scholar 

  20. Galasko CS, Delaney C, Morris P (1992) Spinal stabilization in Duchenne muscular dystrophy. J Bone Joint Surg Br 74(2):210–214

    PubMed  CAS  Google Scholar 

  21. Miller F, Moseley CF, Koreska J (1992) Spinal fusion in Duchenne muscular dystrophy. Dev Med Child Neurol 34(9):775–786

    Article  PubMed  CAS  Google Scholar 

  22. Gayet LE (1999) Surgical treatment of scoliosis due to Duchenne muscular dystrophy. Chirurgie 124(4):423–431

    Article  PubMed  CAS  Google Scholar 

  23. Kinali M, Messina S, Mercuri E et al (2006) Management of scoliosis in Duchenne muscular dystrophy: a large 10-year retrospective study. Dev Med Child Neurol 48(6):513–518

    Article  PubMed  CAS  Google Scholar 

  24. Brooke MH, Fenichel GM, Griggs RC et al (1989) Duchenne muscular dystrophy: patterns of clinical progression and effects of supportive therapy. Neurology 39(4):475–481

    Article  PubMed  CAS  Google Scholar 

  25. Kurz LT, Mubarak SJ, Schultz P et al (1983) Correlation of scoliosis and pulmonary function in Duchenne muscular dystrophy. J Pediatr Orthop 3(3):347–353

    Article  PubMed  CAS  Google Scholar 

  26. Cobb J (1948) Outline for the study of scoliosis. Instr Course Lect 5:261–275

    Google Scholar 

  27. Tsiligiannis T, Grivas T (2012) Pulmonary function in children with idiopathic scoliosis. Scoliosis 7(1):7

    Article  PubMed  Google Scholar 

  28. Miller F, Mosely CF, Koreska J, Levison H (1988) Pulmonary function and scoliosis in Duchenne dystrophy. J Pediatr Orthop 8(2):133–137

    PubMed  Google Scholar 

  29. Young HK, Lowe A, Fitzgerald DA et al (2007) Outcome of non-invasive ventilation in children with neuromuscular disease. Neurology 68(3):198–201

    Article  PubMed  CAS  Google Scholar 

  30. Toussaint M, Steens M, Soudon P (2007) Lung function accurately predicts hypercapnia in patients with Duchenne muscular dystrophy. Chest 131(2):368–375

    Article  PubMed  Google Scholar 

  31. Bourke SC, Gibson GJ (2002) Sleep and breathing in neuromuscular disease. Eur Respir J 19(6):1194–1201

    Article  PubMed  CAS  Google Scholar 

  32. Kennedy JD, Martin AJ (2009) Chronic respiratory failure and neuromuscular disease. Pediatr Clin North Am 56(1):261–273 xii

    Article  PubMed  Google Scholar 

  33. Modi HN, Suh SW, Hong JY et al (2010) Treatment and complications in flaccid neuromuscular scoliosis (Duchenne muscular dystrophy and spinal muscular atrophy) with posterior-only pedicle screw instrumentation. Eur Spine J 19(3):384–393

    Article  PubMed  Google Scholar 

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Acknowledgments

AJ Martin (Respiratory Physician, Women’s and Children’s Hospital, Adelaide, South Australia), The Department of Sleep Medicine (Women’s and Children’s Hospital, Adelaide, South Australia).

Conflict of interest

None of the authors or their family members have received financial benefits from any commercial party for this manuscript.

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Authors and Affiliations

  1. Departments of Orthopaedic Surgery and Respiratory Medicine, Women’s and Children’s Hospital (WCH), North Adelaide, SA, Australia

    W. M. Alexander, M. Smith, B. J. C. Freeman, L. M. Sutherland, J. D. Kennedy & P. J. Cundy

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  1. W. M. Alexander
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  2. M. Smith
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  3. B. J. C. Freeman
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  4. L. M. Sutherland
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  5. J. D. Kennedy
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  6. P. J. Cundy
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Correspondence to W. M. Alexander.

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Alexander, W.M., Smith, M., Freeman, B.J.C. et al. The effect of posterior spinal fusion on respiratory function in Duchenne muscular dystrophy. Eur Spine J 22, 411–416 (2013). https://doi.org/10.1007/s00586-012-2585-4

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  • DOI: https://doi.org/10.1007/s00586-012-2585-4

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