Your child’s primary physician can monitor for emerging orthopedic issues. A physical medicine and rehabilitation specialist and/or orthopedist can provide monitoring and offer management options. A radiologist diagnoses scoliosis. Physical and occupational therapists can manage therapy needs and recommend optimal positioning equipment. Complex care and palliative care clinicians can assess baseline medical status and provide support for considering surgical treatment options.
Neuromuscular Scoliosis
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Scoliosis is an irregular curvature of the spine. Children with SNI most often develop neuromuscular scoliosis (NMS), as the curvature is caused by the impaired ability to control the muscles that support the spine. Finding ways to address scoliosis is important, because spine misalignment can cause back pain and impact the position of the body. More severe scoliosis restricts the available space for internal organs and may diminish the capacity of the lungs to expand, making a child more susceptible to infections and even lung collapse.
Children with SNI are regularly monitored for scoliosis, first with routine examinations of the back and then with x-rays. Early signs of scoliosis include unevenness in the hips or shoulders, a leaning or uneven seating posture, and an uneven gait. Curvature of the spine often worsens during growth spurts, particularly during adolescence. Many once-ambulatory children with SNI begin to need a wheelchair during adolescence due to their neurological decline and may either develop neuromuscular scoliosis or experience a rapidly worsening curve.
To diagnose scoliosis a radiologist will take an x-ray and measure the exact degree of the curve of the spine. Typically, anything over a 10-degree curve is considered scoliosis. Curves of smaller degrees are often managed with a specially molded brace that goes around the upper body, wheelchair and seating accommodations that support the spine, and physical and occupational therapy. Neuromuscular scoliosis tends to progress even with these non-surgical interventions.
Surgery will be considered when the measurement of the curve reaches a specific level, but the level tends to vary with the child. It is generally recommended with the following goals in mind:
- Improve a child’s position and comfort in their chair
- Maintain the ability to stand for the benefit of bone health
- Prevent or improve pain that is associated with movement of the back
- Improve lung function
Spinal fusion surgery uses metal rods, screws, hooks, and wires to straighten and stabilize the spine so that it will no longer curve abnormally. Approximately 6-12 months after surgery, the spine begins to fuse, or heal as one bone preventing further curvature. During this time the child will continue to wear a brace. In younger children who are still growing, surgery may involve the use of “growing rods”. These rods provide temporary stabilization of the spine while still allowing the spine, chest, and lungs to grow. The child returns to the doctor every 6-8 months to lengthen the rod and allow for natural growth. When the child is fully grown, the doctor performs a spinal fusion.
The ultimate decision about surgery requires input from you and your child’s entire medical team. Your child’s primary physician and complex care specialist can review your child’s overall health, which is important as the risks of surgery and possible recovery time are assessed. Other specialists can evaluate the stability of each medical problem; for example, a neurologist can assess seizure stability and a pulmonologist can assess respiratory health. Additional issues that are important to review include nutrition (especially for adequate protein intake), seizure management, intestinal function, and bladder function.
Children with stable health and few medical problems may not require surgery or may be able to tolerate the surgery with less risk compared to children who have many different medical problems, particularly if those medical problems are difficult to control. Many children with SNI have other problems that will not improve with spinal surgery, and, in fact, the surgery may put them at greater risk. This full assessment will determine the degree of risk involved in major surgery. If you and the medical team agree that surgery makes sense, this full assessment will guide how to optimize health before and after surgery. For example, a child who has chronic respiratory problems may have difficulty being weaned from a ventilator after surgery, which might introduce questions about tracheostomy. Some questions you might consider include:
- Are there multiple health problems that will not improve with spinal surgery, or that might worsen those problems?
- Can major surgery speed up decline in function or health?
- Will the improved positioning that surgery might offer improve my child’s daily life?
- If we decide against surgery, are there seating options that can improve my child’s comfort?
- What is the timeframe for deciding on surgery? At what point will the option of surgery be lost?
It is difficult to see your child experience even mild discomfort, yet the prospect of major surgery can be very daunting. You may be convinced that your child’s stability means that surgery can be one way to give your child the most comfortable life. Alternatively, you may have been seeing decline and decide that surgery will not reverse this decline–or may add risk, in which case you would prefer to focus on how to make your child as comfortable as possible. If a palliative care team is available, they can work with the orthopedist and others to help you have a sense of the big picture of your child’s health, and make you feel more settled in your decisions about the decision you make.
