Abstract
Neuromuscular scolioses in patients with myopathy span a spectrum of clinical presentations but are typically characterized by a collapsing deformity in a hypotonic patient. The case of a 7-year-old girl with nemaline myopathy and an 80 degree progressive, thoracolumbar scoliosis is presented. The patient was managed uneventfully with a pelvis-to-rib manual distraction construct (“Eiffel Tower”) and underwent two lengthenings over 2 years of follow-up.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Darin N, Tulinius M. Neuromuscular disorders in childhood: a descriptive epidemiological study from western Sweden. Neuromuscul Disord. 2000;10(1):1–9. http://www.ncbi.nlm.nih.gov/pubmed/10677857.
Norwood FLM, Harling C, Chinnery PF, Eagle M, Bushby K, Straub V. Prevalence of genetic muscle disease in Northern England: in-depth analysis of a muscle clinic population. Brain. 2009;132(Pt 11):3175–86. https://doi.org/10.1093/brain/awp236.
Colombo I, Scoto M, Manzur AY, et al. Congenital myopathies: natural history of a large pediatric cohort. Neurology. 2015;84(1):28–35. https://doi.org/10.1212/WNL.0000000000001110.
Nagai T, Tsuchiya Y, Maruyama A, Takemitsu M, Nonaka I. Scoliosis associated with central core disease. Brain and Development. 1994;16(2):150–2.
Sharma S, Wu C, Andersen T, Wang Y, Hansen ES, Bünger CE. Prevalence of complications in neuromuscular scoliosis surgery: a literature meta-analysis from the past 15 years. Eur Spine J. 2013;22(6):1230–49. https://doi.org/10.1007/s00586-012-2542-2.
McElroy MJ, Sponseller PD, Dattilo JR, et al. Growing rods for the treatment of scoliosis in children with cerebral palsy: a critical assessment. Spine (Phila Pa 1976). 2012;37(24):E1504–10. https://doi.org/10.1097/BRS.0b013e31826fabd3.
North KN, Ryan MM. Nemaline myopathy. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. Seattle, WA; 1993.
Zanette G, Robb N, Zadra N, Zanette L, Manani G. Undetected central core disease myopathy in an infant presenting for clubfoot surgery. Paediatr Anaesth. 2007;17(4):380–2. https://doi.org/10.1111/j.1460-9592.2006.02108.x.
Pruijs JE, van Tol MJ, van Kesteren RG, van Nieuwenhuizen O. Neuromuscular scoliosis: clinical evaluation pre- and postoperative. J Pediatr Orthop B. 2000;9(4):217–20.
McCarthy RE, Sucato D, Turner JL, Zhang H, Henson MAW, McCarthy K. Shilla growing rods in a caprine animal model: a pilot study. Clin Orthop Relat Res. 2010;468(3):705–10. https://doi.org/10.1007/s11999-009-1028-y.
Hell AK, Campbell RM, Hefti F. The vertical expandable prosthetic titanium rib implant for the treatment of thoracic insufficiency syndrome associated with congenital and neuromuscular scoliosis in young children. J Pediatr Orthop B. 2005;14(4):287–93.
Crawford CH 3rd, Lenke LG. Growth modulation by means of anterior tethering resulting in progressive correction of juvenile idiopathic scoliosis: a case report. J Bone Joint Surg Am. 2010;92(1):202–9. https://doi.org/10.2106/JBJS.H.01728.
Betz RR, D’Andrea LP, Mulcahey MJ, Chafetz RS. Vertebral body stapling procedure for the treatment of scoliosis in the growing child. Clin Orthop Relat Res. 2005;(434):55–60.
Akbarnia BA. Management themes in early onset scoliosis. J Bone Joint Surg Am. 2007;89(Suppl 1):42–54. https://doi.org/10.2106/JBJS.F.01256.
Smith JT. Bilateral rib-to-pelvis technique for managing early-onset scoliosis. Clin Orthop Relat Res. 2011;469(5):1349–55. https://doi.org/10.1007/s11999-010-1700-2.
Hasler CC, Mehrkens A, Hefti F. Efficacy and safety of VEPTR instrumentation for progressive spine deformities in young children without rib fusions. Eur Spine J. 2010;19(3):400–8. https://doi.org/10.1007/s00586-009-1253-9.
Samdani AF, Ranade A, Dolch HJ, et al. Bilateral use of the vertical expandable prosthetic titanium rib attached to the pelvis: a novel treatment for scoliosis in the growing spine. J Neurosurg Spine. 2009;10(4):287–92. https://doi.org/10.3171/2008.12.SPINE08427.
Abol Oyoun N, Stuecker R. Bilateral rib-to-pelvis Eiffel Tower VEPTR construct for children with neuromuscular scoliosis: a preliminary report. Spine J. 2014;14(7):1183–91. https://doi.org/10.1016/j.spinee.2013.07.484.
Flynn JM, Ramirez N, Emans JB, Smith JT, Mulcahey MJ, Betz RR. Is the vertebral expandable prosthetic titanium rib a surgical alternative in patients with spina bifida? Clin Orthop Relat Res. 2011;469(5):1291–6. https://doi.org/10.1007/s11999-010-1620-1.
Teoh KH, Winson DMG, James SH, et al. Magnetic controlled growing rods for early-onset scoliosis: a 4-year follow-up. Spine J. 2016;16(4):S34–9. https://doi.org/10.1016/j.spinee.2015.12.098.
Rolton D, Thakar C, Wilson-MacDonald J, Nnadi C. Radiological and clinical assessment of the distraction achieved with remotely expandable growing rods in early onset scoliosis. Eur Spine J. 2016;25(10):3371–6. https://doi.org/10.1007/s00586-015-4223-4.
Flynn JM, Tomlinson LA, Pawelek J, Thompson GH, McCarthy R, Akbarnia BA. Growing-rod graduates: lessons learned from ninety-nine patients who completed lengthening. J Bone Joint Surg Am. 2013;95(19):1745–50. https://doi.org/10.2106/JBJS.L.01386.
Bekmez S, Dede O, Yazici M. Advances in growing rods treatment for early onset scoliosis. Curr Opin Pediatr. 2017;29(1):87–93. https://doi.org/10.1097/MOP.0000000000000432.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2018 Springer International Publishing AG
About this chapter
Cite this chapter
Lee, M.C., Eberson, C.P. (2018). Congenital Myopathy with Early-Onset Scoliosis. In: El-Hawary, R., Eberson, C. (eds) Early Onset Scoliosis. Springer, Cham. https://doi.org/10.1007/978-3-319-71580-3_15
Download citation
DOI: https://doi.org/10.1007/978-3-319-71580-3_15
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-71579-7
Online ISBN: 978-3-319-71580-3
eBook Packages: MedicineMedicine (R0)