Neuromuscular Scoliosis Dion Heimink & Tony Kotoor

What is NM??? *An irregular spinal curvature caused by disorders of the brain, spinal cord, and muscular system. *Nerves and muscles are unable to maintain appropriate balance / alignment of the spine and trunk. *Neuromuscular curves are often associated with pelvic obliquity. *Frequently, kyphosis is also concurrently present.

Causes NM Scoliosis occurs secondary to many different neurological and muscular disorders. Incidence of Scoliosis: Cerebral palsy (2 limbs involved) 25% Myelodysplasia (lower lumbar) 60% Spinal muscle atrophy 67% Friedreich ataxia 80% Cerebral palsy (4 limbs involved) 80% Duchenne muscular dystrophy 90% Myelodysplasia (thoracic level) 100% Traumatic paralysis (<10 years) 100%

S- curve

Background Can affect the entire spine and not just specific segments Can develop at earlier ages than idiopathic scoliosis Can rapidly progress into adulthood in comparison to idiopathic/congenital scoliosis, which typically progress at slower rate and stop after adolescence.

Prognosis Compared with idiopathic scoliosis, neuromuscular scoliosis is much more likely to produce curves that progress, and continue progressing into adulthood. (Curve progression and trunk imbalances are more severe in patients who are not able to walk).

Treatment Bracing Wheel-Chair Modifications Physical Therapy Surgical Stabilization

Operative Treatment The type of spinal stabilization depends on the age of the patient, ambulatory status, and underlying condition. Surgical goals are: Prevent curve progression Improve sitting balance and tolerance (in non-ambulator) Reduce repositioning Reduce pain

Physical Therapy Literature is lacking high quality evidence that physical therapy and non-surgical interventions are effective for treating scoliosis.

Study 1: “Our results demonstrate that posterior-only pedicle screw fixation with PVCR if necessary is effective in obtaining and maintaining alignment in the neuromuscular scoliosis population.” Modi, Hitesh N., et al. "Treatment of neuromuscular scoliosis with posterior-only pedicle screw fixation." Journal of orthopaedic surgery and research 3.1 (2008): 1.

Study 2: Found that “Bracing significantly decreased progression of high risk curves to the threshold for surgery and the benefit increased with longer hours of brace wear” Weinstein et al, 2013, Effects of Bracing in Adolescents with Idiopathic Scoliosis. NEJM, Sept 2013.

Braces

References Halawi MJ, Lark RK, Fitch RD. Neuromuscular Scoliosis: Current Concepts. Orthopedics. 2015 Jun;38(6):e452-6. doi: 10.3928/01477447-20150603-50. Scherl, S MD. Adolescent idiopathic scoliosis: management and prognosis. UpToDate. March 02, 2016. Topic 6291. Version 23.0. Modi, Hitesh N., et al. "Treatment of neuromuscular scoliosis with posterior-only pedicle screw fixation." Journal of orthopaedic surgery and research 3.1 (2008): 1. Banta JV: Combined anterior and posterior fusion for spinal deformity in Myelomeningocele. Spine. 1990, 15: 946-52. 10.1097/00007632-199009000-00020. Pehrsson K, Larsson S, Oden A: Long term follow-up of patients with untreated scoliosis. A study of mortality, causes of death, and symptoms. Spine. 1992, 17: 1091-6. Weinstein et al, 2013, Effects of Bracing in Adolescents with Idiopathic Scoliosis. NEJM, Sept 2013. Anderson, P. 2013, Bracing avoids surgery for young patients with scoliosis. Medscape, September 19th 2013.