By Dr. James Wright (formerly of The Hospital for Sick Children in Toronto)
Parents of children with spina bifida are overwhelmed at birth with multiple health concerns. Unfortunately those concerns never go away. The initial focus is usually on the closure of the spina bifida and the possible need for a shunt. However as those issues resolve, inevitably at some point parents want to understand how their child will function in their environment; will they walk, run, or play sports? Every child is different and we never want to extinguish hope but there are some general expectations based largely on the function of the nerves to the legs.
Orthopaedics is a branch of surgery focused on muscles and tendons, ligaments and joints including the spine. As an orthopaedic surgeon, I have had the privilege of attending the clinic at the Holland Bloorview Kids Rehabilitation Centre on Fridays for the past 24 years. I have watched babies grow into mature and wonderful adults. Over that time, not only have orthopaedic treatments improved, but overall, children receive less orthopaedic surgery with better outcomes.
Quality of life and function are key objectives for children, parents, and care givers. Quality of life refers to general well-being. However, everyone defines their own quality of life; for example some people like lots of friends and if they have lots of friends they are happy, whereas other people like a few close friends and if they have a few close friends they are happy. A big component of an individual’s quality of life is their health. When people are healthy they feel well. Health can be defined in multiple ways but in general, includes mental/emotional function, physical function, and social function. Mental/emotional function is about how people feel; happy or sad, angry or calm. Social function is what people do; do they go to school or work, do they have a few or a lot of friends. Physical function is how people do what they need to do in their daily lives; do they walk or use a chair, do they use crutches or orthotics? Of course, these different parts of functions cannot be separated. If a person feels sad they may not be active, and if a person can’t do the things they want to do they may feel sad.
Physical function also depends on other aspects of health and the individual’s personality; if individuals have many health issues they may not be physically active; also some people like to get out and do stuff and others like to stay home. Finally, physical function is affected by the things that surround us. Do braces or orthotics fit well or is the movie theatre or restaurant wheel-chair accessible? All these factors affect physical function. Walking is about how fast, how much energy it takes, and in some cases, how it looks (if that is important to a child). While everyone focuses on walking, walking is not the only way of getting around; other ways include running, hopping, crawling, or using a wheelchair.
Orthopaedic surgeons aim to improve physical function. However orthopaedic surgeons are only one member of a large team including physical therapists, occupational therapists, and orthotists, all aiming to improve and maximize physical function and quality of life. Physiotherapy teaches skills and stretches joints. Occupational therapists help with seating. Orthotists make braces that are used to support joints when muscles are not present.
How the nerves function is a major determinant of physical function. Nerves have two main functions. One is to feel things like pain and cold. Second is to make muscles contract. While feeling or sensation is important, how the muscle works is the main influence on physical function. In addition, when muscles do not contract with normal strength, joints often become stiff or move out of place. Orthopaedics is directed towards preventing a reduction in physical function at some point in the future, and maintaining or restoring physical function. For example, surgery is sometimes performed on a baby’s foot before they start to walk so that the feet are in a good position for standing and walking when they are ready to stand; surgery might also be performed on a stiff joint to allow a child to stand straight to maintain and for others to return to walking or standing. As surgeons, we aim to minimize surgery and maximize function.
The spinal cord contains the nerves between the body and the brain. The higher the level of the spina bifida, the more affected is the spinal cord and the more muscles that are weaker or paralyzed. The nerves to the arms come from part of the neck called the cervical spine; the nerves to the trunk come from the middle part of the back called the thoracic spine; the nerves to the legs come from the lower part of the back called the lumbar spine; and the nerves to the bowel and bladder come from the lowest part of the back called the sacrum.
If the spina bifida is in the middle part of the spine then all the muscles in the legs do not work, the individual cannot feel their legs, and the individual do not have voluntary control of their bowel and bladder. Children with this level, called thoracic level, can stand in childhood with the use braces or orthotics called standing frames or a reciprocating gait orthotics (RGO). A standing frame provides support from trunk to feet with a wide base to allow the child to stand upright. If the standing frame has hinges at the hips and knees allowing the child to sit, it is called a parapodium. A reciprocating gait orthosis, a brace that also spans trunk to feet, has a hinge at the hip that allows one foot in front of the other but with stiff knees. Compared to standers or parapodium, an RGO allows a child to walk around more easily but the brace is heavy, requires the use of a walker, and is more complicated to put on and off. Some children like walking and standing while others like using to use a wheelchair. A wheelchair is faster and easier for children to keep up with their friends. While standing may help stretch joints, it probably does not prevent weak bones, reduce fracture risk, improve bowel function, or prevent pressure sores. Thus, standing is encouraged based primarily on whether the child likes to stand. Function at one point in time may change - for example, most children with no muscle function in their legs even if they stand as a child will shift to using a wheelchair full time in late childhood. Orthopaedic surgery is sometimes performed on stiff joints to support a child and family in their wish to stand. However, as noted above physical function is much more than standing or walking, and for many children physical function means sitting, crawling, using a wheelchair, or playing sports such as sledge hockey. Thus any decision about surgery must consider all aspects of physical function.
If the spina bifida is in the upper part of the lumbar spine then some of the muscle in the hip and knee work but there is usually no sensation below the thighs, and the bowel and bladder are not under voluntary control. Children with this level, called high lumbar, may need orthotics similar to thoracic level and some may walk with long leg braces from hips to feet and use a walker. These children also usually shift to full time wheelchair use in late childhood.
If the spina bifida is in the lower part of the lumbar spine then some of the hip, knee, and foot muscles work but there is no sensation below the knee and the bowel and bladder are not under voluntary control. Children with this level of spina bifida, called low lumbar, walk using braces below the knee. These children often continue to walk at least some of the time as adults. Some choose to use a chair some or much of the time depending on what they want to do. For these children, orthopaedic surgery may be needed to keep their knees straight, their feet in a good position and sometimes to bring their feet in line with the knees. Foot surgery is often needed for the feet to be flat to the ground and allow stable walking and prevents foot ulcers.
If the spina bifida is in the lowest part of the spine then most of the muscles in the legs work but there may be no sensation in the feet and the bowel and bladder are usually not under voluntary control. Children with this level, called sacral, may need braces during childhood, sometimes below the knee and sometimes only in their shoes. These children usually walk as adults with little support. Orthopaedic surgery is sometimes needed for their feet.
Two other areas are frequent topics for discussion between the orthopaedic surgeon and the families are the dislocated hip and scoliosis. The hip joint, a ball that fits in a socket, often dislocates (comes out of the joint) in children with spina bifida. While it may seem to make sense that the ball needs to back into the joint, a dislocated hip doesn’t seem to have an effect on standing or walking. Also a dislocated hip does not appear to cause hip pain as adults. If surgery is needed it is usually because the hip joint is stiff and surgery may help with standing, self-catheterization or riding a horse.
Normally when viewed from the back, the spine from a top to bottom, forms a straight line. Curvature of the spine is called scoliosis. At the bottom of the spine is the pelvis. Associated with scoliosis, the pelvis may no longer be level shifting weight onto a single buttock. Thus, significant scoliosis can influence sitting. If the curve is high up in the back, because the ribs are attached to the spine, the rib cage also can become crooked. Research done in Toronto (by E. Wai et al.) did not find a relationship between the amount of scoliosis and either self-perception of appearance or physical function. Other research from Toronto (Khoshbin et al.) did not find that surgery on the spine improved physical function. Thus the decision to have surgery for scoliosis is a difficult decision based on current function and how the spine will affect future function. If scoliosis is corrected, the surgery is performed differently than in the past. The operation is done with screws and a rod, rather than hooks and a rod. Most of the time a single operation from the back is performed, rather than two operations from the front and back (as was performed in the past). While surgery can make the spine straighter it also stiffens the spine permanently and that may make some movements such as twisting to the side or self-catheterization more difficult.
In conclusion, spina bifida affects sensation, weakens muscles, and stiffens joints, and may cause curvature of the spine. The role of the orthopaedic surgeon is to define which if any of these problems is affecting physical function that is important to the children. Every child is different and their needs for function vary. Orthopaedics is all about helping child achieve their best physical function to best suit their needs and improve their quality of life.