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Operative treatment of scoliosis in proximal spinal muscular atrophy: results of 41 patients

  • Orthopaedic Surgery
  • Published:
Archives of Orthopaedic and Trauma Surgery Aims and scope Submit manuscript

Abstract

Introduction

The early development of progressive scoliosis with pelvic obliquity is the most significant orthopaedic problem for non-ambulatory children with spinal muscular atrophy (SMA).

Patients

24 SMA patients were operated on for scoliosis using the ISOLA® system and 17 patients using a telescopic rod. The average age at spinal surgery was 12.3 years (6.5–22.7) in the ISOLA® group and 6.7 years (4.8–10.9) in the telescopic rod group.

Results

The Cobb angle was corrected on average from pre-operative 83°(54°–120°) to post-operative 39°(5°–70°) in the ISOLA® group and, respectively, from 62°(28°–86°) to 18°(0°–34°) in the telescopic rod group. Mean loss of correction at mean follow-up of 6 years (3–10) was 6°(0°–33°) in the ISOLA® group and at mean follow-up of 8.6 years (3–12) 13°(0°–49°) in the telescopic rod group excluding the patients with rod failure. The vital capacity is not worsened by these operations in comparison to the natural course. After telescopic rods were implanted, there were some unsatisfactory results due to crankshaft phenomenon and an increase of pelvic obliquity.

Conclusion

Our recommendation for non-ambulatory SMA patients is to have definitive stabilisation using multisegmental instrumentation, starting from the age of 10 to 12 years.

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Conflict of interest

The authors declare that they have no conflicts of interest.

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Authors and Affiliations

  1. Department of Orthopaedic Surgery, Friedrich-Alexander-University Erlangen-Nuremberg, Rathsberger Str. 57, 91054, Erlangen, Germany

    Albert Fujak, Alexander Kreß, Raimund Forst & Jürgen Forst

  2. Medizinische Klinik II-4, Klinikum Nürnberg, Breslauer Str. 201, 90471, Nürnberg, Germany

    Wolfgang Raab

  3. Neumarkt Hospital, Teaching Hospital of the Friedrich-Alexander-University Erlangen-Nuremberg, Nürnberger Str. 12, 92318, Neumarkt i.d. OPf, Germany

    Alexander Schuh

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  1. Albert Fujak
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  2. Wolfgang Raab
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  3. Alexander Schuh
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  4. Alexander Kreß
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Correspondence to Albert Fujak.

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Fujak, A., Raab, W., Schuh, A. et al. Operative treatment of scoliosis in proximal spinal muscular atrophy: results of 41 patients. Arch Orthop Trauma Surg 132, 1697–1706 (2012). https://doi.org/10.1007/s00402-012-1610-8

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  • DOI: https://doi.org/10.1007/s00402-012-1610-8

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