Children with differences affecting the function and structure of their muscles and skeletal system commonly develop scoliosis as they get older. When this is felt to be because of an underlying condition such as cerebral palsy or muscular dystrophy, it is referred to as neuromuscular scoliosis. This column addresses neuromuscular scoliosis and what families tend to think about when surgery is considered.
How does scoliosis develop?
The spinal column is a stack of more than 25 vertebrae, kind of like building blocks with a ot of clever projections and attachments. From the lumbar spine on up to the base of the skull, each vertebral body connects to the one above and the one below by sets of joints and cushions that allow the spine to bend, twist, and absorb a load of weight. The spinal column is supported by muscles and ligaments that keep it shaped in the best formation for activity, balance and comfort. If a child has differently shaped vertebral bodies, they may not stack straight one on top of the other, and as a result, the spine may be bent or curved at angles different from that children with typically shaped vertebral bodies. This could happen if the vertebral bodies developed differently prior to birth, or if they lost their block-like shape over time because of any of a number of conditions.
The Greek word, “skolios,” means “curved,” or “crooked.” The most common type of scoliosis, known as adolescent idiopathic scoliosis, affects fewer than one in twenty typically developing teens, and serious curves are much rarer. As the term, “idiopathic” suggests, we don’t really know why this happens.
How is neuromuscular scoliosis different?
Most children with neuromuscular scoliosis have typically shaped vertebral bodies. But when the muscles keeping the spine balanced function differently, and unevenly, than they do in children with typically functioning muscles, the spine may curve, bend and twist in ways that are noticeably different. This commonly becomes more pronounced over time.
What sorts of difficulties are experienced by children with neuromuscular scoliosis and their families?
The consequences of neuromuscular scoliosis are several:
- the child’s appearance changes in an obvious way;
- adjustments are needed so the child can sit comfortably and in ways that are supportive;
- breathing can be affected.
The impact scoliosis has on breathing is something families and providers get very concerned about. When scoliosis is significant, one or both sides of the chest can be compressed (“squished”) to some extent, reducing the capacity of the lungs to move air in and out. In addition, in a child with advanced scoliosis, some of the muscles involved in breathing are anchored in ways that put them at a disadvantage, as they may lack the leverage needed they once had to pull air into the body in the way they once did. For some children with neuromuscular scoliosis, the muscles throughout their bodies are not as strong as they would like to start with, or may even weaken with age, depending on the condition they are living with. Although it can look like it might hurt a child, neuromuscular scoliosis is not thought to cause pain.
What treatment options exist?
Neuromuscular scoliosis tends to respond poorly, if at all, to bracing. Once the curve is very advanced, an operation may be recommended to help the child sit more comfortably and, theoretically, improve respiratory function. I am not aware of any randomized, controlled trials to see if operating on children with neuromuscular scoliosis reduces the odds of future respiratory problems or hospitalizations, or improves life expectancy. Theoretically, it could.
The most common operation, posterior spinal fusion, is well established, well studied and performed widely. It is a major operation with significant needs for advanced care afterwards. It should preferably be performed by a pediatric orthopedic surgeon experienced in surgery for children with neuromuscular scoliosis, at a hospital with a well staffed pediatric critical care unit. Typically, the team of professionals planning and caring for a child who will have an operation for neuromuscular scoliosis could also include the primary care provider, a pediatric pulmonary specialist, a pediatric critical care physician, a pediatric anesthesiologist, a pediatric pain specialist and more (some of these roles overlap). Recently, a different set of operations is being performed beginning when the child is younger, when the degree of scoliosis is less, using expandable titanium rods (VEPTR’s) that attach to the rib cage and are lengthened or adjusted over the course of years. These operations, though they happen in many stages, may turn out to be less invasive overall.
What are the alternatives?
The clear alternative to having surgery for neuromuscular scoliosis, simply, is deciding not to have the operation. Although none of the hoped for benefits of an operation will come, forgoing an operation for scoliosis will spare the child a major procedure followed by what can be a long and difficult recovery period, as well as some less common complications. Any parent considering an operation for neuromuscular scoliosis for their child will learn about all these details, outcomes, and options and decide, with the child’s providers, what is in the child’s best interests. As with all treatment decisions for children with special needs, the discussions around them should be longitudinal, repetitive, inclusive and maximally supportive to parents, family caregivers and children.
Dr. Okun is Medical Director of New Alternatives for Children, a child welfare agency serving children with special health care needs and their families from New York City. He is board certified in Pediatrics, Developmental-Behavioral Pediatrics and Hospice and Palliative Medicine. He is a Fellow of the American Academy of Pediatrics and the American Academy of Hospice and Palliative Medicine and involved in committee work with those organizations. He is the recipient of numerous teaching awards. He has presented internationally and written about care for children with special health care needs, child maltreatment and a range of issues in general pediatrics, bioethics, and palliative and end of life care and bereavement.
The views expressed in this column are solely those of Dr. Okun and are not intended to represent those of any agency or organization with which he is employed or otherwise affiliated.