I know. I know. The first thing you do is look up scoliosis. You might just as well look up, mmmm, flat feet. The facts just do not carry over. ‘Scoliosis’ is like ‘cough’ in that the underlying process far outweighs the thing itself. Also, most of the scoliosis stuff you read about is generated by healthy teen aged girls who have this inexplicable imperfection. We are NOT talking about that. So put that all away and, please, if you are queasy, leave now.

You just crossed the queasy line.

Scoliosis in neuromuscular conditions has known causes and they are nasty and often multiple. Always remember HOW the neuromuscular disease got there in the first place.

1. Disproportunate and sustained muscle pull can curve the spine. Obvious. It can also damage the way the growth plates and centers grow making a growing deformity on top of a bending deformity. When things over pull that will identify the over pull as being in the concavity (as with the string on an archer’s bow - concave side)

2. Weakness - or failure to initiate normal muscular tone sufficiently or sustain it enough will also allow the spine to fall the other way. Weakness is therefore on the convex side.

Aaaah, but this is not a muscle disease in most cases but rather neuromuscular, the muscles just reflecting what they are told or not told to do. Neurologic imbalances are rarely muscle group specific and more often form larger patterns. Therefore, one side is truly pulling too hard too long while the other is inhibited. That’s a double whammy.

3. There’s a tendency for fibrous tissue to populate improperly neurologically tended muscle. Fibrous tissue, once it spans a segment, does not keep up with normal growth patterns and tends to act as a non-growing tether. That will manifest on the concave side.

4. The spine sits on the pelvis. If the pelvis is skewed by the legs below it will reflect in curvature above. Do that far enough and long enough and it becomes a structural thing of itself.

5. Was the neurologic damage secondary to cardiac anomaly? There is a high statistical spinal growth anomaly associated with cardiac anomalies - neurologic problems or not. The more the cardiac anomaly has blood shunting from the right to the left (bypassing the pulmonary circuit) the higher the chances of this type scoliosis.

6. Was there a disease that required nasty drugs or radiation? Wilms tumor can be cured, but the cure can damage the spine growth cells and leave asymmetrical growth behind. Those who had spinal or brain involvement before the cure may well have difficult scoliosis.

7. It ain’t over even when the phat lady sinks. Those numbers that suggest wellness in adolescent scoliosis just don’t work in neuromuscular scoliosis.

We’ll save statistics and academics for a lecture. Let’s really learn by looking at our reality. In certain diseases, the progression of scoliosis is so utterly certain once it begins, that the numbers don’t mean anything. A ten degree curve is not a less threat than a 30 as it will soon become 30. Muscular dystrophy (not technically a ‘neuro’muscular curve but definitely a paralytic curve - with weakness and fibrosis and relentless underlying generalized progression) it stands out as an example to surgically stop it as soon as possible. Delay only increases the surgical complications. Braces just do not work. They kill.

Fortunately, most paralytic curves manifest a static neurologic imbalance and don’t have the underlying disorder running ahead making everything harder and harder. So the forces take their toll but other medical considerations can be handled as the curve is managed by braces.

Here’s another problem. Just about every big city has a brace with a name. Forget it. The problems of paralytic curves are so utterly complex and with so many permutations and combinations of issues and severity that one brace for the whole group means poor bracing outcomes. It often gets called ‘noncompliance’ as families will refuse to allow choking, or apnea, or skin loss.

Braces that depend on abdominal pressure are doomed to fail as this population cannot handle abdominal compression for reasons of reflux and respiration.

So we start out in a hole. Worse curves with longer duration of progression and less ability to tolerate abdominal and even chest pressure. The brace we recommend is called the THINK brace. We use our brains to think what this specific case needs to nudge it without upsetting those other things.

Bracing does not do what intuition would have you assume when looking at an x-ray.

If you merely push at prominent locations, the youngster becomes a squeezed tube of tooth paste.

Ribs collapse and internal volume gets squished and the boa constrictor properties of the brace are no friendlier on breathing than the snake. Breathing can be impaired. Stomach contents do wind up in lungs.

Lets look up inside the rib cage from the pelvis.

Remember that the scoliotic spine twists horizontally as it bends sideways, and it carries ribs on one side backward as it carries the opposite side ribs forward.

We want to untwist the entire unit. Dr. Cotrel, in France, brought this principle to the forefront first as a casting technique and then later as a surgical dual rod construct
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Others, such as Dwyer from Australia, had come to similar conclusions for anterior surgical work. So rather than pushing from some favorite spot (pick a city), you have to embrace the whole circumference and nudge the entire circumferance in a gentle twist.

From the back, yes some ribs have to go forward, but other ribs need a hollow place to nestle into. This is volume neutral bracing . It is well tolerated because it does not squeeze and leaves the air space intact.

Now think! If you just push on ribs with a hand, or a pad, or foam, or an air bladder ... is it any different?

NO! Duh. There are folks out there hyping air as if air is pixie dust. Force is force. Air is least capable of being directed tangentially. Pads that fit the circumferance and are slinged to rotate as shown do not press IN, but rather rotate one side forward, and the other side back. But the fact is, these braces are HARD TO MAKE! And they don’t lend to mass production. They are fiddle factor high because they work. The shape does change. So, you have to fiddle with them. If they are hard to make and keep perfect, then why do it?

This is why. When you put hocus pokus air bladders in and inflate, you hydraulically squash the rib cage into oblivion and breathing capacity with it.

There has to be a place to go to.

There is an alternative though. Surprised? Curves that are poorly tolerant of pushing, when x-rayed to measure suppleness, sometimes show that they are surprisingly flexible. How were those x-rays taken?

 Side bending x-rays are taken lying down supine (which alone helps) and the patient is simply leaned sideways. The shoulders stay in contact with the table as the bending advances until it just resists. No big force. Curves will drop dramatically. We measure how far surgery can straighten the spine without high tension, using this kind of x-ray. We can do this with a brace. A side bending brace is made with the patient leaning sideways. The correction from the bend is essentially traction, and not compression. At night, lying down, it is well tolerated as the neurologic tightness is least during sleep, and also neurologically less while recumbent in addition to being relieved of gravity.

So difficult paralytic curves can be reclaimed by having a second NIGHT TIME brace that leans. If there are two curves, then we lean to correct the difficult one. The brace leans with a very large cut out that lets the patient fall out of the brace in the direction of correction.  Horizontal derotation is not forgotten. That’s in there too.

<= But look at this (mouse over the image). The front and back views show a ‘nice light’ brace that is called a
“Mold ‘n Hold” brace - a ‘nice comfortable’ brace. We call these Mold ‘n Fail braces. The brace curve matches the child! What’s the point?  There are also similar “soft” braces out there. “Soft”  refers to the thinking behind them. Besides being useless, they are HOT.

Anything can be made light. Brace weight out not even come up. Most ‘soft’ braces dehydrate kids as well as uniformly squeeze them. At least this illustrated firm Mold ‘n Fail brace had an abdominal  relief.

As you run cursor over the above image, notice what two hands can do easily and gently. A brace is just hands left behind when the physician lets go. Make the brace do what the hands were doing when an improvement was seen. Aside from a pelvic base to build on, not much is needed here. This is perfect for a bending brace - where from the physician’s left hand upward there is nothing on the child’s right side. The child’s left side is held high and over and rotated as needed.

If the child can tolerate this brace sitting - big if - then it can serve as a day brace as well. The Charleston Brace - simplified - and liberated from abdominal compression and liberally cut out, makes a good brace. That brace was put forward as a bending night time brace for idiopathic curves in a community where heat and humidity make daytime plastic vests nasty.

In some young kids we can actually overshoot. That’s OK. We’d prefer our bend to theirs. If they’re going to be crooked, be good crooked. Just a bit. This assumes we are countering a known relentless muscular pull. The straighter the body the LESS the  force and point pressure felt. Overcorrections, when you can get them, make the perceived forces near nothing.

It’s in the hands! Play with the child and see what gets it right without crushing force. Bending and turning without squeezing. Then duplicate that. If you wrap the child in wet plaster and repeat the hands-on correction, how can you get it wrong? The key element in a good brace is the time spent figuring out just how to turn or bend and rehearsing it before a cast is made.

But, in this other case, before you dip that plaster, look at the x-ray. Can you see the hidden faces? One is unhappy. The other is a lady wearing a brain head dress.

Hidden things in the x-rays? Yes. They are there. The Cobb angle is already pointed out. But what else lurks?

OK, you DO see the faces. But they are not real. Did you see what was far more important and very real ?

Ahhh, there are several things. The vertebral body well offset from the spinous process showing that this is not just curved but very  rotated. The thoracic vertebrae are trapezoidal (wedged) from pressure and so damaged will grow wedged no matter what. And they seem to overlap meaning that the mid thoracic area is coming at you - lordosis. Not good. And then those evergreen (here red) branches laden with snow. The ribs have lost  their ability to transmit any external forces to the spine. Put the plaster away. These hidden faces are telling you it is time for surgery. There is much more to spines than angles.

And there’s much more to these children than their spines. Let’s just take a quick sampling which is fairly typical:

See a trend? It isn’t that a spine that gets scoliosis causes hips to go bad or that a bad hip will cause the spine to go bad. The central problem causes BOTH to go bad and it is just a matter of what gets there first. So a spine gets fixed and then the hip goes? Could happen. But not because you fixed the spine. Despite it. In fact, leveling the pelvis is a huge protection for hips and can even make a tenuous case go the right way. When the pelvis tilts, usually the higher one gets “uncovered” by the tilt and can more easily go out if the socket mechanics are bad enough. But notice the top middle case, the lower more covered hip was the one going out. Why? Because it is a neuromuscular disease. Ultimately it is the internal muscular imbalance that does it all - tilts the pelvis, curves the spine, and pulls the hip in one way not protected by a counter pull. Look at what this can do to wrists, fingers, ankles, foot or even midfoot.

Muscular imbalance distorts posture. Easy enough. But it also distorts growth patterns. It isn’t just bent like nail hit wrong. It grows bent like a tree tethered by something.

Do only things we can see get involved? Obviously, no. Think of all the stuff not seen. Visual impairments, swallowing defects, salivary glands spewing given over amplified nerve signals (no different than a muscle doing what it is told and told badly). So where do we draw lines? Fix everything?

First we make a distinction between findings and problems. Not every finding needs an intervention. Those that are causing trouble - present tense - are easy enough.  You don’t need Latin names to know something is interfering with living comfortably. But some need the trained person who knows what the odds are that something that seems OK will not remain OK and what will happen needs to be preempted.

In the chest area, we can easily measure trouble. People do not use much of their breathing capacity most of the time. But be chased by a crazy dog and you know how important it is to have reserve (speed helps too). But we also need that reserve to grow older. Bad habits or tough work environments can wheedle away at our lung capacity and we don’t know it until its is gone. You have seen the old folks with nasal prongs and an O₂ canister. Less capacity than just reserve gone.

A thoracic curve of 80 degrees has - under the best of circumstances - eliminated all reserve breathing capacity. Exertion is impossible.

But that’s not all. Remember that single sided lung volume loss? If that side is stiff (shaped like that it will be) then the reserve on the other side gets us into trouble. The ease of breathing one, including its reserve, side leaves the other side under ventilated. Even though enough oxygen comes from the intact side, the poorly dynamic side pools secretions and becomes repeatedly infected. Still waters turn green. The side that can’t cough or even partake in a yawn - infects. That kills more and more lung.

Simple blood gas measurements and such can detail the woes of the chest. But the abdominal changes are far more subtle. It isn’t until AFTER the bad curves are fixed that families realize just how bad eating was. Those finiky eaters who reflux despite everything, who in fact were acting obstructed, suddenly thrive on three large meals a day. The skin & bones kid gains twenty pounds that doesn’t show anywhere. Bowel timing programs actually work.

Also the bowel distention of poorly motile intestines causes increased dystonic posturing in some of the kids. So bodily mal-postures are worsened for no visible reason.

Sometimes the kids are simply too ‘spent’ (depleted, cachectic) to survive large surgery. Then we have to get them ready by temporizing and letting the medical doctors do their work.

Although almost any type of scoliosis can get to be like this, it is far more probable and rapid when the cause is neuromuscular. In that category is a sweep of particular diagnoses. But they share a disturbance of the brain or spinal cord as the aggravating underlying pathology. The largest “group”, cerebral palsy is itself a collection of many different neurological insults which more or less (often less) is limited to those in which the neurologic pathology is not worsening. Academically, that’s the definition. In practice, a diagnosis without a home gets put here until somebody sorts out otherwise.

This next young lady was in danger of respiratory arrest:

To try to pull a curve as this straight is impossible and dangerous. Something has to be removed to allow a collapse into correction. So from the “front” (a side incision), the spine is exposed in the abdomen and in the chest by pulling all the organs aside and taking the diaphragm off the chest wall and spine. And yes, we put all that back again. From the front, the very large true discs (not just the gooey centers) get entirely removed. Some bone inducing stuff gets put in the open space where discs have been removed and then the spaced are scrunched down as the connecting metal gets contoured as needed. This is showing one of the ways to connect metal to the spine (from the front). This curve was brought down to just about 15 degrees. Not bad. because of skin problems on the back side, the anterior surgery was the only surgery. Typically we like to reinforce from the back side as well. In any event, this one did fine as is and has been stable for years now.

This next youngster had a Baclofen pump and a 117 degree spinal curve causing all sorts of woe and difficulty in care. Treating the curve and maintaining the pump,  all in one session was the way it was handled:

By handling the worst part from the front, a comprehensive correction from the back was made possible. Anterior surgery allows extreme corrections over short spans. Posterior surgery handles overall balance and strength of repair but alone cannot equal what anterior surgery allows. Sometimes it is best to add instrumentation from the front, especially when posterior anatomy may be wanting of sufficient structure. Alternately, releasing from the front can allow more modern posterior instruments to be the only implants. There are so many factors. Experience is vital.