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Neuromuscular Scoliosis

Neuromuscular scoliosis is 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, a condition in which the child's pelvis is unevenly tilted with one side higher than the other. Frequently, kyphosis is also concurrently present.

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).

Symptoms

  • Children with neuromuscular scoliosis usually do not experience any pain from the condition.
  • Most children with neuromuscular scoliosis have poor balance and poor coordination of their trunk, neck, and head.
  • Neuromuscular scoliosis can lead to thoracic insufficiency syndrome.
  • Seating problems
  • Hygiene challenges

Incidence

Because of the many causes of neuromuscular scoliosis, the incidence is variable (Table 1). The likelihood and severity of the curves tends to increase with the degree of neuromuscular involvement.

Diagnosis 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%
Table 1. Incidence of Neuromuscular Scoliosis

Treatment Options

Nonoperative Treatment

Nonoperative treatment should be prescribed and managed by a multidisciplinary team to ensure that all clinical areas receiving the proper treatment: pulmonary, neurologic, genitourinary, orthopedic, nutritional and gastroenterologic. Nonoperative treatments can include the following:

  • Bracing for smaller curves (most curves from neuromuscular scoliosis do not benefit from bracing)
  • Wheelchair modifications (for non-ambulators) to improve function
  • Physical therapy

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-ambulators)
  • Reduce repositioning
  • Reduce pain