Neuromuscular Scoliosis (Pediatric)

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What is neuromuscular scoliosis?

Neuromuscular scoliosis is the presence of one or more abnormal curvatures of the spine and affects children with neuromuscular disorders. This curvature causes the spinal column to bend to the left or the right in the shape of an S or C. A variety of the types of diseases that may cause neuromuscular scoliosis, the clinical presentation and severity of this condition is extremely variable. However, most children with this disease have poor balance and poor coordination of their trunk, neck, and head. There is also a high frequency of concurrent kyphosis, which is an abnormal forward-bending curve of the spine. The condition is generally classified into two categories based on the type of disease that the child suffers from, and which causes the abnormal curve. Neuropathic scoliosis involves diseases that are primarily afflictions of the nervous system, such as cerebral palsy or spinal cord trauma, whereas myopathic scoliosis involves muscular disorders, like muscular dystrophy or arthrogryposis.

Basic facts about neuromuscular scoliosis are:

• Scoliosis is technically defined by the presence of a curvature in the spine of more than 10 degrees deviation from straight upright.
• The earlier the curve develops in neuromuscular scoliosis, the more likely it is to progress to a more severe form. Likewise, the more severe a curve is when first detected, the faster it will progress on average.
• Unlike most cases of idiopathic scoliosis, in which the curves occur in a limited region of the spinal column and are relatively short, neuromuscular scoliosis is associated with long curves that often extend to the bottom of the spinal column.
• Neuromuscular curves are often associated with a condition known as pelvic obliquity, in which the child’s pelvis is unevenly tilted with one side positioned higher than the other side.
• Children with neuromuscular scoliosis usually do not experience any pain from the condition.

What causes neuromuscular scoliosis?

Defects or abnormalities in the central nervous system (brain and spinal cord) lead to altered proprioception or diminished control of the paraspinal muscles. As a result, abnormal forces are transmitted to the spine's vertebral units either because the muscles show increased motion (termed spasticity, as seen in cerebral palsy); decreased motion (also known as flaccidity, as seen in Friederich’s ataxia); or out-of-sequence motion (called dyskinesia, also seen in cerebral palsy).

Children's spines are still growing when they experience abnormal forces, which generally worsen curves as growth spurts occur.

How is neuromuscular scoliosis diagnosed?

Neuromuscular scoliosis is usually first detected during a standard physical examination by a pediatrician, noticed by a child’s parents, or found during a full workup for a child’s neuromuscular condition. We follow a physical with a series of X-rays for more precise measurement of the possible presence and severity of one or more curves.

A full neurological exam of the back and extremities will be performed to ensure no other spinal conditions are present and the spinal cord is unaffected by the presence of the abnormal curvature (if it is not already affected by the underlying condition).We may also do a spinal MRI.

What is the treatment for neuromuscular scoliosis?

Treatment depends on the severity of the spinal curvature at the time of the diagnosis, the age of the patient, and the symptoms of the existing neurological or muscular disorder. The curves in almost all of the predisposing conditions have a high rate of progression and almost all children will require surgery at some point. However, sometimes braces are used to slow the curve's progression until a later time when surgery can be safely performed.

Bracing Treatment

Braces should improve a patient’s ability to sit or stand and perform certain functions otherwise not possible. The brace should be worn for several years or until surgery is necessary. Wheelchair seating adaptations also exist that can act similarly to bracing and may be appropriate for some patients.

Patients wear an external brace when upright but not when sleeping or lying flat. The semi-rigid molded TLSO brace (thoraco-lumbo-sacral-orthosis) is the preferred design because it is effective when worn periodically and does not significantly constrict breathing. This is also the brace used for patients in wheelchairs.

In ambulatory patients, the more traditional TLSO may be used.

Surgical Treatment

Surgeries for neuromuscular scoliosis are complex because of other biological effects common in underlying neuromuscular diseases, such as respiratory difficulties, weak bones, and poor nutrition. Operations entail instrumentation in which metal rods are attached to the spine to maintain curve correction and spinal fusion, and two or more of the vertebrae are fused together with bone bridges made of bone grafts. Fusion between the spine and the pelvis may also be necessary with cases of pelvic obliquity and particularly in children with muscular dystrophy.

Due to the stability and effectiveness of devices used in spine operations today, patients usually do not require bracing treatment following surgery, and hospital stays are limited to 7-10 days.

The type of surgery depends on the severity of the curve and the age of the patient. If the patient is younger, a growing rod type of technique may be used. If the patient is older, a spinal fusion and instrumentation is most likely what will be recommended. Surgical treatment of neuromuscular scoliosis is quite successful in reducing curves and improving quality of life.

We know the development of scoliosis on top of an existing neuromuscular condition is challenging for children and parents alike. Our doctors are dedicated to providing solid treatment outcomes and helping children enjoy fulfilling lives.