Spine II: Adolescent Scoliosis and Kyphosis

John M. (Jack) Flynn, MD

David L. Skaggs, MD, MMM

Michael G. Vitale, MD, MPH

John B. Emans, MD¹

¹Guru:

Adolescent Idiopathic Scoliosis

Adolescent idiopathic scoliosis (AIS) is a very common condition presenting to the pediatric orthopaedist. About 3 million new cases are diagnosed in the United States each year. Most of these children just need a thorough evaluation, complete (but low radiation) imaging, monitoring through the rapid adolescent growth spurt, and reassurance. A smaller subset will need bracing and counseling to prevent surgery. A very small subset will need their serious spine deformity corrected surgically.

The pediatric orthopaedist needs to be mindful of a few key principles when dealing with AIS:

Be sure you are actually treating idiopathic scoliosis, not an imposter (Fig. 22-1).

• Remind yourself repeatedly that puberty is a high stress, high drama time for humans, when body image and peer pressure are at a lifetime maximum. This dominates all treatment discussions.

• AIS is highly genetic. In clinic, you may be walking into a room with parental guilt hiding in the background. Be particularly alert if one of the parents or grandparents had a “Harrington rod” or a “Milwaukee brace.”

• There are many unscrupulous characters out there spreading misinformation and trying to bilk the families out of large sums of money for useless treatments. They run $5000 per week “scoliosis cure” camps. They make braces out of cloth straps. They offer growth-driven surgical treatments for teens who are done growing (as long as the family pays in cash). Some of them are orthopaedists.

• What we offer for treatments (plastic body braces or screws in the vertebra) work really well, have strong scientific evidence behind them and a well-established track record of success, but are enough to make a teenager collapse with anxiety when they think about the consequences.

• The results of modern spine deformity correction are so spectacular and durable that few changes have been made in the past two decades. However, there are persistent risks of infection, neurologic injury, and need for reoperation
that are unacceptably high. Some of these complications are more preventable than others. Pediatric spine surgeons need to be mindful of the factors driving a great long-term result, rather than just the factors that drive a great-looking postoperative X-ray.

Figure 22-1 A: This boy presented with a 33° left thoracic curve and an asymmetric umbilical reflex. B: MRI revealed Chiari I malformation and holocord syrinx, requiring neurosurgical intervention.

In the pages ahead, we’ve collected many decades of wisdom to allow the pediatric orthopedist to navigate these very common clinic visits and spine deformity correction operations. In all these areas—evaluation, imaging, bracing, and surgery—we’ve seen lots of trouble resulting in patient harm and lawsuits and are determined to continue the downward trend in “trouble” for the many adolescents whose lives are affected by scoliosis.

HISTORY

There are several key pieces of information that should be collected on every single AIS new patient visit:

Who discovered the scoliosis—primary care doctor, school nurse, parent, patient, peer, boyfriend/girlfriend?
Is there a family history of scoliosis? Parse out kyphosis caused by osteoporosis that many will say their grandmother has. Also learn if anyone needed treatment for scoliosis, which suggests a much more significant family history.
Are there any concerning symptoms (neurologic, pain, etc.)? NEWSFLASH! Although you must ask about back pain, be careful not to open a Pandora’s Box into the teenage psyche. Scoliosis (when mild) does not cause back pain (if it did, they wouldn’t have to screen for it). But, most teenagers have some back aches, especially the ones whose primary form of exercise is video gaming or who are depressed or anxious. Most back pain in teenagers is caused by weak core muscles, not small curvatures of the spine. Of course, pain that awakens the teen at night, or that is localized to a specific part of the spine (especially the lumbosacral junction in the midline), is worth noting and evaluating.

PHYSICAL EXAMINATION

Although a “complete physical exam” is the motherhood and apple pie of medicine, and is always the right answer, realize that you’re not expected to be documenting the volume of cerumen in each ear canal for every one of your idiopathic scoliosis patients. So be thorough, but focus on a few key elements: a careful neurologic exam, a complete documentation of the deformity (Adams forward bend test, flank creases, shoulders and pelvis, spinal balance), abnormal skin findings (especially café au lait spots), gait, and leg lengths. Also, look at the bare feet and be sure there is no significant cavus deformity or claw toes. To stay out of trouble with a teenager of the opposite sex from you, keep them as well covered as possible.

Announce your need to do certain aspects of the exam (such as the abdominal reflex) and be considerate of privacy and exposure concerns in front of parents, trainees who are shadowing you, and others. NEWSFLASH! Do the abdominal reflex with their finger not yours (nobody can tickle themselves).

One very helpful trick is to do the Adams forward bend test so that the teen is bending their head directly toward their seated parents. This allows the parent to “sight down” their teenagers back, and get a real appreciation for the magnitude and location of the rotational deformity. Especially for less sophisticated families who were told by the pediatrician to be seen quickly for deformity that on first visit may be in surgical range, it is helpful for the mother to see the deformity before treatment is discussed.

IMAGING

Full-length PA and lateral radiographs of the entire spine are the standard of care for initial images. The recent availability of slot scanning technology has revolutionized pediatric spine imaging. With microdose slot-scanning on follow-up, the radiographs are often less than 5% of the radiation that a child would get from a standard radiograph. In addition, with proper positioning of the hands beside the face (Fig. 22-2), a free bone age can be obtained on every visit, dramatically improving the ability to make high-quality, data-driven decisions for the rapidly growing patient.

Look at those initial PA and lateral radiographs carefully (Fig. 22-3). Look for congenital anomalies of the spine. Look for spondylolysis or spondylolisthesis. Look at the shoulders and pelvis on the radiographs and consider issues such as leg length inequality.

One big red flag is the spine that looks like it is “windblown.” Instead of the typical idiopathic right thoracic or left lumbar spine deformity with significant associated rotation, the “windblown” spine is often a curve to the left with very little rotation and involving both the thoracic and lumbar spine. If it seems to have a neurologic look, you are likely right: this is the appearance of a spine deformity caused by a huge syringomyelia, Chiari malformation, spinal cord tumor, or some other rare but dangerous interest spinal condition (Fig. 22-4).

Figure 22-2 Using this positioning innovation, every scoliosis patient gets a free Sanders bone age score. This saves time and money for the family and greatly enhances the skeletal maturity information needed to make sound clinical decisions.

Figure 22-3 Remember to look beyond the spine on each radiograph. The need for this is aptly demonstrated here: the child’s right hip was noted to be at risk (arrow). This is easy to overlook during a scoliosis evaluation, but may be made to appear obvious to a jury years later.

Figure 22-4 A: Although this is not the infamous “left-sided curve,” it has several concerning features—long sweeping pattern and little or no rotation. It looks like the wind is blowing the spine. As soon as we saw this girl’s X-ray, we sent her for an MRI. B: The MRI revealed a pilocytic astrocytoma of the spinal cord, requiring urgent neurosurgical management.

SKELETAL MATURITY AND GROWTH REMAINING

Skeletal maturity and growth remaining are absolutely essential to proper scoliosis care. Historically, Risser sign and menarche were all we had, and while they offered basic milestones for growth, wild variability led to some bad medical decisions. In general, females have their peak growth acceleration the year before menarche and continue to grow at a slower pace for about 2 years after menarche; however, menarche can be altered (endocrine abnormality, female athlete Triad). The Risser sign was useful mostly because we could see the pelvis on scoliosis radiographs. However, the most dangerous time for scoliosis progression occurs before Risser 1, and lots of growth can occur after Risser 4.

Fortunately, the work of Jim Sanders and others has greatly improved our skeletal maturity information precision in the last decade. Now, tracking Sanders scores and growth since last visit help us decide when a brace should be started, when it should be tapered down, when it should be finished, and when it should be avoided altogether. NEWSFLASH! Beware: height measurements done by busy MAs can be inaccurate, or recorded inaccurately.

One way to know that a family has stopped worrying about the scoliosis: they turn their full attention and passionate emotion into arguing that the Sanders score and the growth chart are wrong, because their son or daughter could not possibly be finished growing: “I kept growing well into college.” A simple scoliosis visit can be turned into a tense and emotional realization about final adult height.

At that moment, when the boy realizes he’s only going to be 5 feet 6 inches tall, the wise clinician reminds him he will never have to pay extra for legroom on an airplane, perhaps saving many thousands of dollars throughout his life. It seems to resonate every time, and the visit ends with everyone happy and laughing, rather than depressed and crying.

TREATMENT

Compared to many other pediatric orthopaedic conditions, treatment decisions are quite simple in idiopathic scoliosis (although families might find that fact depressing):

Observation for curves less than 25° (or 25°-50° in skeletally mature teens)
Bracing for growing (Sanders <8) children between 25° and 45° to 50°
Spine deformity correction surgery for scoliosis greater than 50° (in some rare cases of documented rapid progression, 45°-50°)

From the very first visit, it’s important to communicate the limited options clearly to patient and family. Explain that there are no foods, medicines, mattresses, exercises, or musical playlists that can stop scoliosis from progressing, or make it go away. Explain that it is a genetic and growth condition, and neither the child nor the parents can control either of those factors. Once everyone in the room is on the same page about the options: observation/bracing/surgery, then it is simply a matter of using the maturity and radiographic data to help the family make a good decision.

Bracing

We’ve experienced a nonoperative scoliosis care revolution in the last decade. There is now overwhelming evidence that a well-made corrective scoliosis brace, that is worn snugly and for at least 16 hours a day, can prevent the progression of AIS to surgical range for most children. It is essential that in the treatment decision discussion, the teenager with scoliosis voices a commitment to wearing the brace. It cannot be the doctor’s decision or the parent’s decision—that will fail in most cases. The teenager has to wrap their head around an amazing fact: scoliosis bracing is one of the rare chances that a human gets to use a treatment which will prevent surgery. A Sanders 3 girl who presents with 35° of scoliosis can choose to have her spine fused in a year or two, or she can “wear the stupid brace” and graduate high school with scoliosis in the 30s and no rods and screws in her back (in most cases). Taking the teenager and the family on this journey can be time-consuming. NEWSFLASH! It’s not unusual for a successful brace conversation to take even longer than a surgical conversation. The pediatric orthopedist and the parents need to demonstrate empathy to the patient about the sensitivity of wearing a brace to school, the body image issues, and how cruel teen peers can be. Encourage the patient to engage other local teens who are going through the same bracing care, and to consider joining scoliosis support groups which can be extremely helpful. A nurse who is an expert in scoliosis can provide education, and ongoing support is worth their weight in gold and frees the surgeon from countless hours of counseling.

The most successful brace initiation protocol: measurement for the brace on the day it is prescribed, then a follow-up visit in 6 to 8 weeks (after the patient has reached fulltime wear) with an X-ray in the brace to measure correction, and an initial analysis of the compliance button data to see exactly how many hours a day the patient is actually wearing a brace. More counseling is often necessary at that visit. Thereafter, bracing patients are usually seen twice a year, with an X-ray taken with the brace removed for a day or two before the visit (“brace holiday”).
To stay out of trouble, make sure your X-rays during bracing use this brace holiday concept. If every X-ray is taken in the brace, the actual deformity progression and size will be masked.

Scoliosis-specific exercises are becoming increasingly popular; the exercises improve posture, core strength, and breathing. The impact on deformity progression is under investigation, but it is much harder to study than the effect of bracing, because the “dose effect” of the exercises is hard to quantify. We’ve found that the exercises help many kids tolerate the brace better. These exercise programs can be very expensive and time-consuming, so they do not work for every family.