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Neuromuscular Scoliosis

Neuromuscular scoliosis
Objectives
  1. Define neuromuscular scoliosis.
  2. List causes of neuromuscular scoliosis. Include upper motor neuron, lower motor neuron, and myopathic causes.
  3. Describe the genesis and development of neuromuscular spinal deformity.
  4. Describe the role of pelvic obliquity in neuromuscular scoliosis.
  5. Discuss the role of bracing for neuromuscular scoliosis.
  6. Describe factors contributing to the higher complication rate following surgery for neuromuscular spine deformity.

Discussion points
  1. What sagittal deformities accompany neuromuscular scoliosis? Why?
  2. What differences present with a neuropathic etiology as opposed to a myopathic etiology?
  3. How can one evaluate nutritional status prior to operative intervention?

Discussion
Neuromuscular spinal deformity comprises some of the most challenging problems encountered in pediatric orthopaedics. The Scoliosis Research Society has classified neuromuscular scoliosis into neuropathic and myopathic causes. Neuropathic lesions include upper motor neuron lesions such as cerebral palsy, Frederich's ataxia, and spinal cord trauma and tumors. Even in this group there is considerable variety in the effect on the spine -cerebral palsy usually includes a spastic component, while spinal cord trauma and tumors may or may not depending on the site and nature of the lesion. Neuropathic causes also include lower motor lesions such as poliomyelitis, and spinal muscular atrophy. Myopathic lesions include arthrogryposis, muscular dystrophy, and myotonia; the spinal deformity patterns from these etiologies also vary considerably. Despite these differences, the common factor is an inability to provide muscular support to the spinal column. Kyphosis commonly accompanies neuromuscular deformity secondary to forward tilt of the head and/ or poor head control. Once the curve is present, gravity and posturing provide the impetus for worsening deformity. Age of onset obviously varies with etiology, for example, spinal muscular atrophy produces deformity at a younger age than muscular dystrophy.
It is important to view the sacrum (and the rigidly attached pelvis as the last vertebral segment. The lumbosacral joint can deform in any plane as the rest of the spine, or more proximal deformity can throw the pelvis into an oblique position without lumbosacral deformity. Contrarily, hip contracture can secondarily deform the spine dynamically when the patient attempts to accommodate the hip deformity while sitting. It is important to carefully assess hip motion and contracture in any patient with neuromuscular spinal deformity. Small amounts of pelvic obliquity (< 10-15 degrees) are compatible with comfortable seating. Larger fixed pelvic obliquities are not, and must be corrected by operative or (if not fixed) wheelchair modifications.

The goal of treatment is a balanced spine and pelvis, generally for the purpose of sitting; although some patients with, for example, arthrogryposis or Frederich's ataxia, can ambulate into adult life. Modalities used to treat neuromuscular scoliosis include wheelchair modifications in addition to the usual bracing and surgery. Bracing has traditionally been felt to be ineffective, although recent reports are more encouraging. It is technically difficult and time consuming, but success has been reported with both cerebral palsy and muscular dystrophy patients. The ultimate role of bracing is unclear.

Non-ambulatory patients with muscular dystrophy with curves over the 20-30 degree range have been regarded as candidates for surgery, the rationale being that the curve will inevitably progress and pulmonary function will decrease. Technical success has been routine in recent reports. Blood loss is higher in children with muscular dystrophy, hypotensive anesthesia has been helpful. Patient satisfaction is generally high after scoliosis correction in the muscular dystrophy patient.

The role of surgery for the cerebral palsy patient has been less well defined. A study of institutionalized patients did not clearly define benefit, although the patients were reported as being more comfortable. Higher complication rates have often been reported, and nutritional assessment has been recommended prior to surgery. An albumin level of >3.5/ dL and a total lymphocyte count of >1500/ mm3 were regarded as minimum requirements. Another study regarded the severity of overall involvement of the patient, recent medical problems, and severity of curve to be more significant. Both may well be correct, as the most impaired non-ambulatory patients with cerebral palsy develop the most severe curves.

The state of knowledge regarding neuromuscular scoliosis is presently undergoing some revision; this process is dynamic and current literature review is essential. The somewhat large bibliography for this subject reflects the divergent opinions currently presented.

References
  1. Alman BA, Kim HK. Pelvic obliquity after fusion of the spine in Duchenne muscular dystrophy. Journal of Bone & Joint Surgery -British Volume 1999; 81( 5): 821-4.
  2. Banta JV, Drummond DS, Ferguson RL. The treatment of neuromuscular scoliosis. Instructional Course Lectures 1999; 48: 551-62.
  3. Benson ER, Thomson JD, Smith BG, Banta JV. Results and morbidity in a consecutive series of patients undergoing spinal fusion for neuromuscular scoliosis. Spine 1998; 23( 21): 2308-17; discussion 18.
  4. Bridwell KH, Baldus C, Iffrig TM, Lenke LG, Blanke K. Process measures and patient/ parent evaluation of surgical management of spinal deformities in patients with progressive flaccid neuromuscular scoliosis (Duchenne's muscular dystrophy and spinal muscular atrophy). Spine 1999; 24( 13): 1300-9.
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  6. Comstock CP, Leach J, Wenger DR. Scoliosis in total-body-involvement cerebral palsy. Analysis of surgical treatment and patient and caregiver satisfaction. Spine 1998; 23( 12): 1412-24; discussion 24-5.
  7. Fox HJ, Thomas CH, Thompson AG. Spinal instrumentation for Duchenne's muscular dystrophy: experience of hypotensive anaesthesia to minimise blood loss [see comments]. Journal of Pediatric Orthopedics 1997; 17( 6): 750-3.
  8. Heller KD, Forst R, Forst J, Hengstler K. Scoliosis in Duchenne muscular dystrophy: aspects of orthotic treatment. Prosthetics & Orthotics International 1997; 21( 3): 202-9.
  9. Jevsevar DS, Karlin LI. The relationship between preoperative nutritional status and complications after an operation for scoliosis in patients who have cerebral palsy [published erratum appears in J Bone Joint Surg Am 1993 Aug; 75( 8): 1256]. Journal of Bone & Joint Surgery -American Volume 1993; 75( 6): 880-4.
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  14. Mubarak SJ, Morin WD, Leach J. Spinal fusion in Duchenne muscular dystrophy--fixation and fusion to the sacropelvis? Journal of Pediatric Orthopedics 1993; 13( 6): 752-7.
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  16. Olafsson Y, Saraste H, Al-Dabbagh Z. Brace treatment in neuromuscular spine deformity. Journal of Pediatric Orthopedics 1999; 19( 3): 376-9.
  17. Saito N, Ebara S, Ohotsuka K, Kumeta H, Takaoka K. Natural history of scoliosis in spastic cerebral palsy. Lancet 1998; 351( 9117): 1687-92.
  18. Smith AD, Koreska J, Moseley CF. Progression of scoliosis in Duchenne muscular dystrophy. Journal of Bone & Joint Surgery -American Volume 1989; 71( 7): 1066-74.
  19. Stricker U, Moser H, Aebi M. Predominantly posterior instrumentation and fusion in neuromuscular and neurogenic scoliosis in children and adolescents. European Spine Journal 1996; 5( 2): 101-6.
  20. Szoke G, Lipton G, Miller F, Dabney K. Wound infection after spinal fusion in children with cerebral palsy. Journal of Pediatric Orthopedics 1998; 18( 6): 727-33.
  21. Terjesen T, Lange JE, Steen H. Treatment of scoliosis with spinal bracing in quadriplegic cerebral palsy. Developmental Medicine & Child Neurology 2000; 42( 7): 448-54.
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  23. Winter S. Preoperative assessment of the child with neuromuscular scoliosis. Orthopedic Clinics of North America 1994; 25( 2): 239-45.
  24. Yazici M, Asher MA, Hardacker JW. The safety and efficacy of Isola-Galveston instrumentation and arthrodesis in the treatment of neuromuscular spinal deformities. Journal of Bone & Joint Surgery -American Volume 2000; 82( 4): 524-43.

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