Common Scoliosis Questions
What is Scoliosis?
Everyone’s spine has natural curves. These curves round our shoulders and make our lower back curve slightly inward. But some people have spines that also curve from side to side, and rotate. Unlike poor posture, these curves can’t be corrected simply by learning to stand up straight. This condition of side-to-side spinal curves is called “scoliosis”. On an x-ray, the spine of a person with scoliosis looks more like an “S” or a “C” than a straight line. These curves can make the person’s shoulders or waist appear uneven. Some of these bones may also be rotated slightly, making one shoulder blade more prominent than the other. Scoliosis is a descriptive term and not a diagnosis. In more than 80% of cases, a specific cause is not known. Such cases are termed “idiopathic”, meaning “of undetermined cause”. This is particularly common in adolescent girls. Idiopathic scoliosis is typically called “infantile” in children 0-3 years old, “juvenile” in children 4-10 years old, “adolescent” in adolescents 11-18 years old, and “adult” in patients over 18 years old. Conditions known to cause spinal deformity are congenital spinal column abnormalities (present at birth – called congenital scoliosis), neurologic disorders (neuromuscular scoliosis), genetic conditions, and many other causes. Scoliosis does not come from carrying heavy things, athletic involvement, sleeping/standing postures, or minor leg length abnormalities.
Do I Have Scoliosis?
Determining whether or not you have scoliosis is best done by a physician who performs a physical examination of your back. The examination is done with you standing in a relaxed position with your arms at your sides. The physician will view you from behind looking for curvature of the spine, shoulder blade asymmetry, waistline asymmetry and any trunk shift. You will then bend forward at the waist and the physician will view your back once again to look for the rotational aspect of the scoliosis in the upper part of the back (rib prominence) or in the lower part of your back (flank or waist prominence). Following this simple examination, the physician will usually initial radiographs of the spine viewed from the back and the side to see the entire spine from the neck to the pelvis. If scoliosis is present, the physician will measure the radiographs and provide you with a numerical value, in degrees, to help describe the scoliosis
What Are My Treatment Options?
This is for curves that have a small degree measurement when you are growing (adolescent scoliosis), or for moderate size curves (< 40-45 degrees) when you are done growing. For adults, observation and physical therapy are for those patients who have mild symptoms and have curves which are not large.
Bracing This is for curves between 25 and 45 degrees in growing children to prevent further progression of the curve while growth of the spine remains. The goal of bracing is to prevent further progression since the brace cannot correct curves.
Surgical Treatment This reserved for curves which are generally greater than 50 degrees for adolescent patients and adults. Surgery can be performed for smaller curves if the appearance of the curvature is bothersome to the patient or if symptoms are associated with the scoliosis in the adult patient. The goals of surgical treatment are to obtain curve correction and to prevent curve progression. This is generally achieved by placing metal implants onto the spine which are then attached to rods which correct the spine curvature and hold it in the corrected position until fusion, or knitting of the spine elements together.
Early Onset Scoliosis
Early onset scoliosis is a lateral (side-to-side) curve of the spine that is diagnosed before age 10. There are several different types of early onset scoliosis, including:
There are other circumstances when the concerns unique to an early onset scoliosis may also apply:
When evaluating a patient with potential early-onset scoliosis, your doctor will first take a detailed history and perform a thorough physical assessment. After this, the next step is obtaining X-rays of the spine and neck. CT and MRI scans are also sometimes done to get a three-dimensional view of the spine and how it is positioned in the body. In children less than 3 months of age, sometimes an ultrasound can be helpful. Your doctor may also recommend an ultrasound of the kidneys or investigations related to the heart, since the kidneys and heart are formed at the same time as the vertebrae. Something that affects the spine may also affect these other organs.
What are the treatment options?
The treatment options for scoliosis fall into three main categories: observation of the scoliosis, non-operative treatment (including bracing, casting and traction), and surgical treatment.
There are several treatment options available to children with early onset scoliosis. It is important to recognize that these treatments are individualized for each patient. Several factors are involved in this decision-making process and your spine surgeon is well suited to help guide you towards the optimal treatment for your child. In addition, not all treatment options are FDA-approved; however, they are sometimes used “off label” if directed by a physician. This section details the different options available for the treatment of early onset scoliosis.
Observation is usually the first method of treatment for a young child with a spinal deformity. The physician will first need to determine if the curvature is progressing — that is, getting worse. Some children will have a curvature of their spine that is stable and unchanging, whereas other children will have a curve that keeps progressing. Just because your spine surgeon is “observing” your child does not mean that he/she is not treating them. During this period of time, not only will your child’s surgeon look for changes in the curve, but they will probably order some special tests to evaluate further the child’s condition and have you see some other doctors. These tests may include an MRI study or a CT study (different kinds of diagnostic imaging). Your child may be referred to other specialists, such as a geneticist, cardiologist, or pulmonologist to make sure there are no other problems in other parts of the body. Your spine surgeon will probably want to see your child regularly and have new front- and side-view X-rays taken. They will then measure the curves and compare them with the previous X-rays, as well as the X-rays from the child’s first visit. It is ideal to have all of the X-rays done at the spine surgeon’s office so that he/she can have similar types of X-rays for comparison and maintain your child’s record. The surgeon will probably continue to observe your child’s curves as long as there is no drastic increase in the size of the curve. In some cases the curve will improve or even resolve (as in infantile scoliosis). If your spine surgeon finds progression of the curve, or determines that progression is highly likely, a different form of treatment will need to be started. He/she may want to obtain bending X-rays of the spine to assess flexibility and help determine the next steps in treatment.
If the curve is progressive, and your child is still growing, your surgeon may want to place your child in a brace. This depends on the flexibility of the curve, as determined by the bending X-rays. If the curve is rigid and does not correct (get smaller) on the bending X-rays, a brace will do little good. Rarely does a brace permanently correct scoliosis, instead the goal of bracing is to allow the child to grow before a surgical procedure is done. It must be re-emphasized that the purpose of the brace is to slow the inevitable progression of the curve, not to correct the curve. The brace that your doctor prescribes may depend on your child’s age and the center you visit. There are several types of braces that have the same success rates, but your doctor will select one based on his/her experience with the different devices. The Kalabas brace has several straps that are applied over the shoulder and bend the child in the opposite direction of the curve (Figure 1). The Wilmington brace is a custom-molded thoracolumbosacral orthosis that has molds to push and correct the curve (Figure 2). The Boston brace is similar, but uses pads inside the brace to push the curve (Figure 3). The Milwaukee brace, one of the first braces developed for scoliosis, is also similar, but includes an extension to the chin (Figure 4). It is the only brace, however, that can manage curves in the top part of the spine. Your doctor will probably recommend that your child wear the brace full-time. Braces are generally removed for bathing and special occasions. As your child grows, new braces will need to be fabricated, approximately every twelve to eighteen months. Braces may not be effective in every child for various reasons. The curve may be stiff and resistant to correction. Braces also have a more difficult time controlling kyphosis (rounded back) and lordosis (sway back). Since most braces work on the curve by putting pressure on the rib cage, concern exists over the effect that the brace has on the rib cage and the subsequent development of the lungs.
Figure 1 – The Kalabas brace has several straps that are applied over the shoulder and bend the child in the opposite direction of the curve
Figure 2 – The Wilmington brace is a custom-molded thoracolumbosacral orthosis that has molds to push and correct the curve.
Figure 3 – Boston brace or thoracolumbar sacral orthosis (TLSO)
Figure 4 – The Milwaukee brace, with an extension to the chin, is able to control curves in the top part of the spine.
A few centers treat young children with a body cast (Figure 5). Placement of the cast on the child may require general anesthesia to increase flexibility of the curve and make the child more comfortable during the application. The cast is generally changed every two to four months, usually under an anesthetic. Casting can offer superior curve management, at the cost of its inconvenience (cannot be removed for bathing).
Serial casting (one cast after another, changing at regular intervals to allow for growth) can be used to delay the need for bracing by correcting the deformity enough to allow bracing to then be re-instituted. Since a cast can be considered a full-time brace that can’t be removed, many parents find it preferable to braces, eliminating the problems of compliance and the difficulties of donning braces in uncooperative young children. Casting can become a definitive method of management rather than simply a delay tactic. Research in the United Kingdom indicates that treating non-congenital scoliosis with serial casts beginning in children at 12 months of age with an average curve of 32° in some cases have their scoliosis reduced to up to <10° at maturity. Patients starting treatment at 18 months or later, with larger curves averaging 52°, achieve less correction, but their deformities can be maintained at a similar degree of magnitude. Casting in children under 2 years of age, where the goal is curing the scoliosis, requires cast changes under anesthesia every 2-3 months (minimum 5 casts) with the goal of achieving a straight spine. Despite the extensive casting a brace will still be needed after the casting treatment. Children over age 2 require cast changes every 3-4 months. Older children demonstrating “recurrence” can be re-casted for four months to re-correct the deformity before continuing with brace management.
Figure 5 – Patients treated with scoliosis casting. Holes are cut along the chest and abdomen to allow for normal breathing and eating. Shoulder straps are optional.
Treatment for patients with a progressive deformity who are not candidates for bracing or casting can be more difficult, for example those with weakness, skin or chest wall intolerance, mental retardation, or with large and stiff curves that do not correct much during serial casting. In these instances, halo-gravity traction is a method to achieve deformity correction, and indirectly, improve breathing mechanics. The traction method of treatment has recently regained popularity in some centers. A halo (metal ring around the head) is applied under general anesthesia. Multiple pins attach the ring to the patient’s skull. The halo is not painful and is well tolerated after the patient becomes used to its presence. Traction is applied the following day with the use of ropes, pulleys, and weights or springs that can be applied to the child’s bed or a wheelchair. Some patients can be treated as outpatients if the family is comfortable. The children are followed with serial X-rays after successive increases in the weight of the traction. Once the spine has shown the maximal amount of improvement, your surgeon will decide the next steps in treatment
An operation is sometimes necessary to address spinal deformity in the young child, and the decision to do these procedures is based on many factors. If the child’s curve has shown progression despite bracing or casting, surgery must be considered. The dilemma faced by the surgeon is how to stop the progression of a curve without adversely affecting future growth. Sometimes this is unavoidable, as most operations work by stopping abnormal spinal growth in a procedure called spinal fusion.
In Situ Spinal Fusion
Spinal fusion is a procedure performed to stop growth of the spine. It can be done from the back (posterior) or through the chest (anterior). The joints of the spine are removed, and a bone graft is placed; when the bone heals there will be a fusion mass, or one solid piece of bone. The goal is for the many vertebrae of the spine to become one segment and stop growing crooked. In situ fusion means that the curve will be fused “where it is” with little or no correction of the spine. Sometimes instrumentation (rods, hooks, and screws) may be placed to help straighten the spine slightly and act as an internal brace for the bone graft that will form the fusion mass. When implants are not used, usually in young children, the child may need to wear a brace following the operation. The goal of an in situ spinal fusion is to address the problem early, before it becomes a serious deformity. For example, if a spine surgeon sees a child with a 40° curve that has a poor prognosis (high chance to progress), he/she may elect to perform a limited spinal fusion to prevent the curve from getting any bigger. It is generally a safer procedure than those that correct the curvature of the spine. The results of a procedure to correct the curve at a young age can be unpredictable, as continued growth of the spine in other areas can cause the curve to progress or rotate (twist around). Spinal fusion is not always a good option for every patient with early onset scoliosis. Because spinal fusion basically stops the growth of one part of the spine, it can restrict growth of the thorax resulting in thoracic insufficiency.
This surgical procedure is aimed at stopping abnormal growth on one side of the spine with the hope that continued growth on the other side will result in correction of the curve over time. Every curve has a concave and convex side (Figure 6). If the growth centers are removed and spinal fusion is performed on the convex side, the concave side might continue to grow, possibly improving the curve. As noted, these procedures can be unpredictable in young children with abnormal vertebrae in their back.
Figure 6 – Hemi-epiphysiodesis is aimed at stopping abnormal growth on one side of the spine with the hope that continued growth on the other side will result in correction of the curve over time.
Some young children with scoliosis may have abnormally shaped vertebrae in their back that causes the curve. Normal vertebrae are shaped like rectangles. A hemivertebra is shaped like a triangle. (Figure 7). When this hemivertebra is located at the bottom of the spine it can tilt the base of the spine and cause the child lean to one side. In other parts of the spine, depending on the number of hemivertebrae present, severe deformity can develop. Depending on your child’s situation, this hemivertebra may be removed from the front, back, or both parts of the spine. Once the hemivertebra is removed the vertebrae above and below it are fused together, often with instrumentation. Most children will wear a brace or cast after the operation until the spine heals. This operation has inherent risks involved, including bleeding and neurologic injury, but good spinal correction is often achieved.
Figure 7 – Hemivertebrae is noted by the bottom of the two arrows.
Growth Friendly Surgery
In the past, spinal fusions were the usual treatment for early onset scoliosis. It is now understood that early fusion of the thoracic spine will limit the growth of the lungs as well as the spine and can lead to severe respiratory problems. Growth friendly surgical procedures, that correct the deformity while avoiding long fusions of the spine, are being developed and refined. Today there are a number of possibilities, each with their own benefits and disadvantages. In general , the type of surgery may be divided classified as (1) Distraction-Based, (2) Guided Growth, and (3) Compression Based
Growth Friendly Surgery
Distraction-based procedures include expandable spinal implants that work by controlling the spinal deformity, while still allowing the spine to grow until the child reaches an appropriate size or age for a more permanent solution, like spinal fusion. There are several different types of posterior distraction systems. The rods are periodically lengthened by relatively minor procedures usually performed every 6 months. Growing rods are a spine-based system where the curve is spanned by one or two rods under the skin to avoid damaging the growth tissues of the spine. The rods are attached to the spine above and below the curve with hooks or screws at either end of the rod (Figure 8). Limited fusion is performed at each of the hook/screw foundation sites. The curve can usually be corrected by fifty percent at the time of the first operation. After the rods are implanted, patients are prescribed a special brace to wear for several months. The child then returns every six months to have the rods “lengthened” each) until the spine is closer to maturity. This is usually an outpatient procedure performed through a small incision. When the child becomes older and the spine has grown, the doctor will remove the instrumentation and perform a formal spinal fusion operation. In the past, this procedure had a very high complication rate, most of which were related to the instrumentation (hook dislodgement, rod breakage). Newer techniques are more promising but treatment with growing rods remains a long, difficult therapy for the child.
Figure 8 – Growing rods are a spine-based system where the curve is spanned by one or two rods under the skin to avoid damaging the growth tissues of the spine. The rods are attached to the spine above and below the curve with hooks or screws at either end of the rod.
Rib Based Systems, such as Vertical Expandable Prosthetic Titanium Rib (VEPTR), are systems used for treatment of thoracic insufficiency syndrome in skeletally immature patients. Thoracic insufficiency syndrome (TIS) is usually associated with uncommon three-dimensional deformities of both the spine and rib cage. Several types of rib-based expansion thoracoplasty operations can be used for different types of deformities to gain chest volume (to allow for growth of the underlying lungs) while indirectly correcting the scoliosis without spine fusion. This surgery can be extensive; devices are placed under the scapula (shoulder blade) and are attached to the ribs near the neck and continue down to either the ribs, spine, or to the pelvis near the waist (Figure 9). This helps to stabilize the surgically expanded chest wall constriction (expansion thoracoplasty). To keep up with a patient’s growth, the devices are expanded twice a year in outpatient surgery through small incisions. Currently, there are a limited number of institutions offering rib-based surgery. Your child’s spine surgeon can advise whether your child’s condition is appropriate for this treatment option and to provide referral information, if needed. Some centers are using the rib-based devices as a means to straighten the spine indirectly via the ribs and chest wall.
Figure 9 – Rib-based devices are placed under the scapula (shoulder blade) and are attached to the ribs near the neck and continue down to either the ribs, spine, or to the pelvis near the waist. This is an example of a rib-rib device and a rib-spine device.
Hybrid growing Rods
This is a newer technology that uses growing rod technology. Here the ribs, not the spine, are used as the upper anchor. The fusion of the upper portion of the spine is avoided.
Magnetically Controlled Growing Rods (MCGR)
Growth rods are currently the most commonly used distraction-based technique and which have the advantage of not interfering with the normal spinal growth and may even have a potential for growth stimulation beyond the normal growth rate. However, the technique requires frequent surgeries for construct lengthening to keep up with normal spinal growth and to maintain scoliosis curve correction. Multiple surgeries, mostly through the same incision site, leave the skin tissue susceptible to infection and other skin problems. Implant related complications are the most common complications in growth rod surgeries. These include rod fracture, anchor failure, or prominent implant, which can cause skin breakdown and even infection. Among the implant-related complications, rod fractures are the most common problem. The idea of non-invasive multiple lengthening without the need for anesthesia and open surgery is appealing given the direct relationship between high complications and repeated surgeries. These devises allow for lengthening to be performed in the doctor’s office. It is composed of an implantable rod, an external remote controller (ERC) and accessories. The titanium rod includes a telescopic actuator portion that holds a small internal magnet. Rotation of the magnet remotely by use of the ERC causes the rod to be lengthened or shortened. The rod is implanted and secured using standard fixation components, such as hooks and/or pedicle screws as anchors. Magnet driven rods are now being used in certain centres outside the United States and preliminary results have shown to be able to reduce morbidities, cost, and decrease stress for patients and parents. They have been implanted in cases of both idiopathic (Figure 10) and neuromuscular scoliosis.
Figure 10 – Magnetically Controlled Growing Rod (MCGR) in a patient with idiopathic early onset scoliosis.
Growth Friendly Surgery
These techniques involve instrumentation that guides spinal growth in a correct direction. Unlike distraction techniques, repeated surgeries are not required. The Luque Trolley uses wires to allow the spine to grow in the desired direction as the wires slide along contoured rods. In the Shilla technique initial correction is obtained by instrumentation and fusion at the apex, or most deformed portion of the spine. Specially designed screws are placed at the ends of the deformity and slide along the contoured rods guiding the direction of the spine as it grows. These children are usually placed in a special brace for 3 months after the surgery.
Early Onset Scoliosis
Growth Friendly Surgery
Compression based systems are intended to produce relative growth inhibition on the convex side of the curve. These techniques can be used in children who are still growing, have a progressing deformity that measures less than 35 degrees, and who are able to tolerate open or endoscopic exposure of the spine. By placing special vertebral body staples or tethers on the convex side of the curve, growth is inhibited on that side. The idea is that the scoliosis may then correct through more growth on the concave side of the curve.
Fusion with Instrumentation Surgery
In some cases, fusion with instrumentation surgery will be advised. This may be indicated when the child is closed to the end of growth or when co-existing medical problems make the growth friendly procedures, which often require multiple surgeries, too dangerous. In most cases this will involve posterior spinal instrumentation and fusion of the entire deformity. When a significant amount of growth remains, a posterior fusion may result in a twisting or “crankshaft” deformity as the growth of the anterior or non-fused front of the spine is tethered by the fused posterior portion. To avoid this problem an anterior fusion is often performed at the time of the posterior procedure in selected children. These procedures are covered elsewhere.