The term "neuromuscular scoliosis" is used to describe curvature of the spine in children with any disorder of the neurological system. Common categories include cerebral palsy, spina bifida, muscular dystrophies, spinal cord injuries and so forth. Most of these children have as a unifying feature weakness of the trunk. As they grow and their trunk gets weaker, there is a progressive, collapsing deformity of the spine producing a long, c-type curve. These curves tend to be progressive, with the rate of progression becoming worse during rapid growth. For children confined to a wheel chair, progressive curves may affect the child's ability to be seated comfortably, thereby affecting their quality of life and function.
The treatment of neuromuscular scoliosis must be individualized. Bracing may provide support for the trunk in the seated position, but is usually not effective at stopping progression of the curve over time. Seating modifications such as inserts into wheelchairs may help with positioning the child, but are also not corrective in terms of the scoliosis. Alternative therapies such as insertion of an Intrathecal Baclophen Pump will produce a reduction in spasticity but will not affect the long-term progression of the scoliosis. Injection of Botox into the paraspinal musculature will temporarily reduce the tone in these muscles but has no proven long-term efficacy in the treatment of neuromuscular curves.
The fundamental question to be addressed by families and their treating doctor is whether the preservation of the ability to sit by invasive surgery will maintain or improve the child's quality of life and function. For some children with cognitive or visual/sensory impairment, these decisions are difficult. Parents often struggle with the fact that they are unable to explain to the child why they are having surgery and help them understand that pain accompanying the procedure. These can be difficult questions to answer given the risks, costs, and recovery associated with this type of surgery. These decisions should be made with great care. Talking with other families who have been through this decision process is very helpful. Ultimately, parents will have to make this difficult decision for their child.
This 3-year-old boy has a 45-degree lumbar scoliosis caused by Myelomeningocele or Spina Bifida. This is a disease caused by failure of the spinal cord and posterior parts of the spine to close completely at birth. Children with this disease have varying degrees of weakness or paralysis in their legs at birth and will often develop scoliosis as they grow.
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