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Is prophylactic formal fusion with implant revision necessary in non-ambulatory children with spinal muscular atrophy and growing rods who are no longer lengthened?

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Abstract

Study design

Single center, retrospective chart review.

Objectives

To determine if routine posterior spinal fusion (PSF) is unnecessary in non-ambulatory growing rod graduates with SMA.

Summary of background data

Most non-ambulatory children with SMA develop early-onset scoliosis (EOS). Posterior growing rods (GR) have been shown safe and effective in managing spinal deformities in these children. The best management of these children, once graduated from their GR, is currently unknown. In this study, we report the clinical results of managing these children without routine definitive fusion following a course of GR treatment.

Methods

A single-center, retrospective chart and radiographic review was performed on children with SMA treated with posterior distraction GR, with a two-year minimum follow-up since final lengthening. Electronic medical records and radiographs were reviewed for demographic variables, Cobb measurements, implant revisions, occult radiographic implant failure, symptomatic failure, and/or conversion to PSF.

Result

12 patients (2 type 1, 9 type 2, 1 type 1/2) met inclusion criteria. Mean age at growing rod insertion was 6.2 years of age (range 4.1–8.2) and age at final lengthening 10.3 years of age (range 9.3–11.9). The mean time between last lengthening and latest clinical or radiographic review was 5.5 (range 2.1–9.0) years. Average mean pre, post, final Cobb angles were 71°, 27° (p < 0.001), 25°. Following final lengthening, only one patient required hardware revision and conversion to definitive fusion in attempts to alleviate chronic hip pain, which was unsuccessful. One additional patient was found to have an occult rod failure that has not required treatment.

Conclusion

While limited by sample size, this single-center cohort of non-ambulatory SMA patients with EOS treated with similar constructs suggests that routine, definitive fusion in SMA GR graduates may be unnecessary.

Level of evidence

Level IV.

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Acknowledgements

This study was approved by the UW-Madison IRB. The authors would like to acknowledge funding from Families of Muscular Atrophy (now CureSMA) Grant Number, MSN164044 and University of Wisconsin ICTR Patient-Centered Outcomes Research Award Grant Number, WPP 3086 Pilot that either directly or indirectly helped make this project possible.

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Authors and Affiliations

  1. School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI, USA

    Rewais Hanna, Mark Sharafinski, Karen Patterson, Kenneth J. Noonan & Sarah Sund

  2. AveXis, Inc., Bannockburn, IL, USA

    Meredith Schultz

  3. Cure SMA, Chicago, IL, USA

    Mary K. Schroth

  4. Department of Biostatistics and Medical Informatics, University of Wisconsin-Madison, Madison, WI, USA

    Scott Hetzel

  5. Children’s Hospital of Omaha, University of Nebraska Medical Center, 8200 Dodge St, Omaha, NE, 68114, USA

    Matthew A. Halanski

Authors
  1. Rewais Hanna
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  2. Mark Sharafinski
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  3. Karen Patterson
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  4. Kenneth J. Noonan
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  5. Sarah Sund
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  6. Meredith Schultz
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  7. Mary K. Schroth
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  8. Scott Hetzel
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  9. Matthew A. Halanski
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Contributions

Rewais Hanna—Data Acquisition, Data Interpretation, Drafting, Revising, Final Approval; Mark Sharafinski—Data Acquisition, Revising, Final Approval; Karen Patterson—Conception/Design, Data Interpretation, Revising, Final Approval; Kenneth Noonan—Data Acquisition, Revising, Final Approval; Sarah Sund—Conception/Design, Data Acquisition, Revising, Final Approval; Meredith Schultz—Revising, Final Approval; Mary Schroth—Conception/Design, Data Acquisition, Final Approval; Scott Hetzel—Data Interpretation, Final Approval; Matthew Halanski—Conception/Design, Data Acquisition, Data Interpretation, Drafting, Revising, Final Approval.

Corresponding author

Correspondence to Matthew A. Halanski.

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Hanna, R., Sharafinski, M., Patterson, K. et al. Is prophylactic formal fusion with implant revision necessary in non-ambulatory children with spinal muscular atrophy and growing rods who are no longer lengthened?. Spine Deform 8, 547–552 (2020). https://doi.org/10.1007/s43390-020-00077-6

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  • DOI: https://doi.org/10.1007/s43390-020-00077-6

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