Abstract
Neuromuscular scoliosis proves itself to be a challenge due to many facts. Among these are the usually early age of onset, rapid progression, rigidity, and lack of response to brace treatment. Added to that the inherent problems present in an already sick child, surgical interventions become perilous. Meningomyelocele patients provide an additional challenge by their lack of posterior anchor points for instrumentation and difficulty in achieving soft-tissue coverage. Neurofibromatosis type-1 scoliosis often accompanies preexisting neurological deficits, neurofibromas, rib protrusion into the spinal canal, and others. Arthrogryposis syndromes with multiple joint contractures and micrognathia may make intubation and patient positioning a challenge before surgery is even begun. Growth-retaining constructs such as the VEPTR and double growing rods represent the current mainstay of surgical treatment, barring specific contraindications unique to the disorder at hand.
Keywords
- Adolescent Idiopathic Scoliosis
- Idiopathic Scoliosis
- Spinal Deformity
- Pelvic Obliquity
- Instrument Fusion
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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Accadbled, F., et al.: Complications of scoliosis surgery in Prader-Willi syndrome. Spine (Phila 1976) 33(4), 394–401 (2008)
Canavese, F., Sussman, M.D.: Orthopaedic manifestations of congenital myotonic dystrophy during childhood and adolescence. J. Pediatr. Orthop. 29(2), 208–213 (2009)
Crawford, A.H., et al.: The immature spine in type-1 neurofibromatosis. J. Bone Joint Surg. Am. 89(Suppl 1), 123–142 (2007)
de Lind van Wijngaarden, R.F., et al.: Scoliosis in Prader-Willi syndrome: prevalence, effects of age, gender, body mass index, lean body mass and genotype. Arch. Dis. Child. 93(12), 1012–1016 (2008)
Downs, J., et al.: Guidelines for management of scoliosis in Rett syndrome patients based on expert consensus and clinical evidence. Spine (Phila 1976) 34(17), E607–E617 (2009)
Greggi, T., et al.: Treatment of scoliosis in patients affected with Prader-Willi syndrome using various techniques. Scoliosis 5, 11 (2010)
Herron, L.D., Westin, G.W., Dawson, E.G.: Scoliosis in arthrogryposis multiplex congenita. J. Bone Joint Surg. Am. 60(3), 293–299 (1978)
Holm, V.A., et al.: Prader-Willi syndrome: consensus diagnostic criteria. Pediatrics 91(2), 398–402 (1993)
Kroonen, L.T., et al.: Prader-Willi syndrome: clinical concerns for the orthopaedic surgeon. J. Pediatr. Orthop. 26(5), 673–679 (2006)
Labelle, H., et al.: Natural history of scoliosis in Friedreich’s ataxia. J. Bone Joint Surg. Am. 68(4), 564–572 (1986)
Nguyen, N.H., Morvant, E.M., Mayhew, J.F.: Anesthetic management for patients with arthrogryposis multiplex congenita and severe micrognathia: case reports. J. Clin. Anesth. 12(3), 227–230 (2000)
Odent, T., et al.: Scoliosis in patients with Prader-Willi syndrome. Pediatrics 122(2), e499–e503 (2008)
Samdani, A.F., et al.: The patient with myelomeningocele: is untethering necessary prior to deformity correction? In: 45th annual meeting of the Scoliosis Research Society, Kyoto (2010)
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© 2011 Springer-Verlag Berlin Heidelberg
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Hasler, CC. (2011). Other Neuromuscular Disorders with Scoliosis. In: Yazici, M. (eds) Non-Idiopathic Spine Deformities in Young Children. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-19417-7_8
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DOI: https://doi.org/10.1007/978-3-642-19417-7_8
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