Out Of the Garden: When Scoliosis Is Not Just Scoliosis

Earlier this month, I had the privilege of presenting a talk at the Chiari and Syringomyelia Foundation regarding scoliosis other than the adolescent idiopathic variety.  It was fun and liberating to talk about all the unusual causes of the disorder, something that I rarely get to do since the idiopathic variety accounts for 90% or so of scoliosis cases.  Researching and considering the topic was also eye-opening, because it reminded me of the fact that complex causes of spinal curvature may not be as uncommon as we suppose and so-called Garden variety scoliosis may be far more complex than we imagine.

The talk is archived at this address: : http://csfinfo.org/videos/physician-lecture-videos/carolinas-chapter-physician-lectures/

Please enjoy, and as always, I welcome your comments.

CLUBFOOT AND GLOBAL MOTOR DEVELOPMENT: SOME PRACTICAL CONSIDERATIONS

Along the lines of my Tummy Time essay comes this reflection on the treatment of clubfoot.  As I note, our understanding of clubfoot is very much under evolution.  I have tried to discuss the larger picture of childhood development with clubfoot rather than specifics of clubfoot treatment.  Please let me know your take on this and all of my posts.  ~KPM

The foot is undeniably the pedestal for human balance and ambulation.  Humans depend on the complex interaction of the many joints and ligament structures in the foot to hold up their weight, propel them forward, stop their momentum and cushion their landings.  The emphasis on proper foot function is so strong that it has fostered a billion dollar footwear and orthotics industry that governs what we wear (and don’t wear) and how we play and live.

Because of this overarching significance, great care is placed in early childhood in getting the foot to grow as “normal” as possible.  This care is especially seen in the congenital deformities of the foot, such as talipes equinovarus, also known as clubfoot.

The understanding of clubfoot continues to evolve.  Most experts no longer think that clubfoot is simply a positional abnormality from months of in utero molding.  Although a small subset may be due to extrinsic issues like multiparous conditions, breech positioning and fibroid uterus, the vast majority of clubfeet are now recognized to be a true disruption of the neural axis into the foot, probably arising from the brainstem or even the brain itself.  In other words, clubfoot is a true neuromuscular condition.

The treatment of clubfoot is evolving as well.  Not so many decades ago, the emphasis of early treatment was on loosening the ligaments up so that a child could be more easily repositioned during surgery.  Mixed long-term functional results from surgery as well as the need for non-surgical options in developing countries led to the advent of rigorous casting and bracing protocols, sometimes lasting for many months or longer.  Although there is no clear consensus that the casting techniques are the final word in treatment, nevertheless most children with clubfoot now have an extended course of casting and/or bracing.

 

The issue at hand is that clubfoot itself may pose challenges to a child’s development, even after the foot is realigned.  The treatments themselves are time-consuming and by definition happen during the period of utmost importance in the early motor development of a child.  The casts may span the first six to nine months of life, when a child is typically starting to grapple with crawling and other forms of locomotion and beginning to assay the challenge of upright stance and walking.

It is important to give special attention the motor development in these children throughout the treatment process.  There is a tendency to keep the children lying on their back throughout the treatment to “protect the casts”, but this is both unnecessary and potentially harmful. Parents need to understand not only that milestones may be somewhat delayed by the treatment, but also that they should not be passive about the child’s positioning and function during the treatment.  It is essential, despite casting to allow as much of the child’s general function to develop as possible.

There are a number of strategies which I have incorporated into my treatment protocols which hopefully will minimize the developmental effect of the casting.  First and foremost, I have tried to engage the parents in the child’s positioning as much as possible.  A lot of the early postural development need not be lost to the casting regimen.  Since the casts are typically placed with the knees bent, the all-important curving forward of the body and limbs into what is termed the flexor position need not be disturbed.  Also, the child will still be able to explore side positioning and rolling, although the parents may have to be more active in helping the child position and in clearing the casts over the other leg during rolling.

Since positioning on the baby’s stomach (prone-lying) is so important for the development of arm and hip/ pelvis position and function, I try to place the casts so that there is some active movement available in the knees.  The child should be able to bring the legs under while in the prone position and push off with the arms and hips to get the full benefit of the ground reactive force.   

Another important point is to make full use of whatever time out of the casts might be available.  In point of fact, since it is not the casting, but the interval stretching before casting that is the actual treatment, I find that these timeouts are actually very important in the regimen.  I try to encourage the parents to take full advantage of the time away from immobilization to have the child rolling and prone and to do as much facilitating as possible in the brief respites.  In the early stages of the casting, I will use a material that the parents can remove the day before the next stretching/ casting treatment.  This not only allows the parents to bathe the child thoroughly but also give an extended period of attention to motor stimulation.  I have also talked to parents about specific techniques like brushing to stimulate the child’s awareness of the feet during the periods out of the casts.  These same techniques can also be done while the child is the cast since the toes and forefoot are exposed.

An interesting technique I have incorporated recently has been cast application in a bouncy seat.  Not only does this help the child to relax during the cast application.  It also allows the child to maintain a flexor posture better than placement of the cast in the fully flat position typically used.  My hope is that the child will accommodate to the cast as part of the more natural flexor positioning.  Early results in terms of acceptance of the cast by the child and maintenance of leg function have been encouraging.

In summary, the most important point is to not allow the casting regimen to dominate the child’s function.  The parents can and should be instructed to be as active in the child’s development as they would be if the child were not casted.  And special exercises and activities should be used to take advantage of every moment that the child is freely mobile.

The Hip and Tummy Time

(Although this blog has long been devoted to pediatric spine topics, I feel like I reserve the right to branch out a little and touch on some general pediatric orthopaedic areas and maybe even on some issues in society pertaining to medicine and the care of kids.

Along these lines, I offer this essay which I wrote in regards to an early motor development program that I am helping develop.  Please let me know if the topic is of interest.  There a many more essays like this one in this feverish brain of mine. -  KPM)

There is an ancient Greek fable about a dreaded creature called the Sphinx who used to waylay travelers with a riddle.  Answer the riddle (as none did) and you could pass safely on your path.  Fail to answer and you were torn apart by the ravenous beast.  The riddle, made simple by the repetition of the years, was “What walks on four legs in the morning, two at midday and three at night?”  The answer or course is a human – crawling in infancy, walking tall in midlife and using a cane in dotage.

What makes this riddle true is the remarkable changes that occur in the structure of the child to go from a fetal ball to a walking youth and nowhere are they more evident than at the hip joint.  The hip is the only joint in the body to actually change its whole structure, positon and anatomy as it develops, and it is these changes that allow humans to eventually stand and walk on two feet in a way that no other creature can.

One of the major changes that the hip undergoes between birth and walking is the alignment and rotation.  When first born, a child has hips that are very flat with respect to the pelvis (the technical term is retroverted).  This allows the hip and legs to retract out of the way without undue stress as the child is delivered through the maternal pelvis and the birth canal.  But it means that, at first, the child cannot bring the hips in front.  We see that in the newborn alignment of the hips with the legs splayed out in what we call the frog position, bent up and rolled out.  A newborn cannot (and should not) be able to bring the hips in to the midline and care should be made in the very early days of life not to pull the legs together to swaddle since it drives the hips into an unnatural position.

Over the course of the first few months of life, the hips begin to roll forward into what is called anteversion.  Ultimately, this lets the child bring the hips straight in front and gives them the stability to start bearing weight and even to push the body forward into crawling.  Ultimately the anteversion provides the child with a secure, forward directed center of balance that will allow upright stance and walking.  Finally, as the child gets more secure and the balance and ambulation muscles develop fully, the posture becomes more upright and the hips assume their adult position.

As with all bone development, the hip realignment cannot happen in a vacuum.  Bone shape follows function, so the bone will shape and position according how it is used.  In children with neuromuscular disease who never walk or crawl, the hips stay retroverted.  If the muscle forces don’t balance during early walking (as in some types of cerebral palsy) then the hips end up too anteverted by late childhood and adulthood.

Which brings us to Tummy Time.  There was a trend through the 1980’s and early 90’s to avoid stomach lying as much as possible.  Genuine fears about protecting the child’s airway led to this well-meaning but ultimately misguided conservatism.  The prevailing feeling was that children ultimately roll over and crawl, so we will protect them until they figure out how to do those actions safely on their own.  Added to the advent of car-seats and other important safety measures, we saw a generation of kids who were essentially told to figure it out on their own.

Many did, but we did see an increase in delayed walking and in some cases the loss of important milestones, like rolling, arm push up and other actions which help develop normal patterns of gait and balance.  Although it is still early to tell, we seem to be seeing an increase in early joint problems in this protected generation.

We seem to have finally figured out that prone lying, or Tummy Time, is safe and vitally important to the development of upright function.  It promotes the awareness of weight bearing and transfer.  It leads directly to experimentation with arm and leg movements which facilitate crawl.  And ultimately, it is from the prone position that humans learn to lift up and become upright walkers.

As for the all-important hip joint, which seems to govern all of propulsion, Tummy Time provides the ground and reactive forces necessary to start the process of realignment of the joint.  The baby feels the ground below the pelvis and reacts with muscular contraction.  The muscle function starts to reshape the hips.  As the hips reshape, pulled inward and forward by the muscle function, they become stronger and better positioned to lift the body and to start forward propulsion.  And ultimately, these functional changes lead to increased strength and balance which lift the child upward into stance and walking.

The Sphinx would be so proud!

"VALUE" IN HOLLYWOOD - Notes from the Pediatric Orthopaedic Society 2014 (part 1)

I recently traveled to Hollywood for the annual meeting of the Pediatric Orthopaedic Society of North America (POSNA).  I am always bemused by LA and especially Hollywood, packed as it is with an odd mix of tourists and denizens, the former trying to snap photos of Marilyn Monroe’s footprints, the latter trying to shill lurid stories about the stars’ dwellings and demises.

The meeting was held in the same facility that hosts the Oscars, and as expected, they did their best to schmaltz it up, complete with a Red Carpet Banquet, which I missed.  It would have been fun to see what some of my colleagues came up with as Hollywood formal.

On the serious side, amidst the usual presentations about hip reconstruction and clubfoot (to operate or not, that is always the question), there were some curious trends that I noticed in the overall thought process of the group, mostly change for the better although some of it remains to be seen.  The watch cry for the whole meeting was “Value”, which is the newest in a long line of buzzwords in the medical community.  In past the issue has always been to define the word in a practical manner so that it can be measured, duplicated (and I guess hopefully rewarded).  The working definition that interlaced a lot of the conversation was the quasi-mathematical expression [Value=Outcome/Cost].  So simple – plug in the numbers and you will have the secret value score.  The problem remains as always: what are the numbers to plug in?

Cost, one would think, is an easy variable to define.  All things have a finite price after all, and there must be some way to find and report that price.  But as we all know, medical economics defies even the simplest logic.  In fact any medical occurrence has multiple prices.  There is the price for the actual treatment (brace or implant or what have you), the price for the treating doctor’s visit, the price for any hospital service, the price for anesthesia, and so on.  About the only one of those that is easy to obtain is the charge of the treating physician, and even that is complicated by the fact that the actual number may have little or nothing to do with the actual fee that a patient or an insurer pays.  Don’t even try looking for hospital or implant costs – that way lies madness.

What about outcome?  By definition, this is a non-numerical factor which wiser heads than mine have been trying to arithmetize for decades.  As such, there are complex surveys and instruments which are supposed to come up with a useable score to plug into this kind of formula.  Although some of these may work in some settings (the Harris Hip Score is elegant for simple arthritis of the hip but not so good for hip impingement), these tools are bulky and hard to implement.

The emphasis at POSNA was on bolstering awareness of the need to understand cost and outcome, so that a value can be derived.  The insurers and the government conflate value with cost alone, but the mere cutting of costs may not enhance the value of anything.  Ultimately, the goal is to bolster the value to such a degree that it overrides any effect that the cost may have.

An excellent point made was that value must be determined in communication with the patient and family, not just by some measurements on an x-ray.  Even if a hip implant lasts for 25 years, it is still not valuable if the patient is in constant pain for every day of that span.

So, finally and at long last, surgeons (and I presume all doctors) will be forced to speak to their patients before, during and after their care.  They will need to go beyond the superficial questions (“Any pain?”) and delve into complex interactions like “How has this affected your life?”, “Did the surgery match your expectations?” or the all-important “Are you satisfied with your care?”  Although these factors will be hard to quantify, they are the true measurements of value.

THOUGHTS FROM THE EAST - My visit to Korea

Last week, I had the estimable pleasure of being one of the invited speakers at a symposium on Patient Care in Spinal Surgery at SoonChunHyang University Medical Center in Seoul, Korea.  The faculty, led by Dr. B-J Shin, was made up of some of the leading orthopaedic spine experts in that country.  Despite the obvious language barrier (the talks, besides mine, were exclusively in Korean), I was able to learn a tremendous amount, not only about the art of treating spine patients but also about the nature and the character of my colleagues in this delightful and mysterious country.

This was my first visit to Asia and from first to last I found my hosts charming and welcoming.  They all seemed delighted to try out their English speaking skills, which to my great fortune were uniformly excellent.  The curious nature of the medical field is that many of the words used in talks are not translated into the native tongue, so despite the balance of the talks being in Korean, many of the words and phrases were understandable.  Combined with the tone of voice, the frequent laughter and the body language, I was able to understand the gist of almost all the lectures, although there were some in which I wish I could have gleaned the details.

The symposium ran from preoperative evaluation to postoperative and chronic pain management.  There were a number of talks on blood loss and transfusion, which is one of my special areas of interest.  I presented my work on Blood Conservation In Scoliosis Surgery which fit in very well with the overall scope of the conference.  The audience asked me a number of insightful questions and the whole interchange made it clear that my message had been delivered.  In fact, the talk was well enough appreciated that I was 'hi-jacked' by Dr. K-Y Ha, another prominent spine professor, to give Grand Rounds the next morning at another University Hospital, Seoul St. Mary's for the orthopaedic residents.  Despite being Saturday morning and my talk again being in the non-native language, I only put two of the residents to sleep, which is an excellent ratio.

After a whirlwind city tour of the magnificent and exotic city - think New York skyscrapers mixed with Buddhist shrines and ancient palaces - I spent the final day in the city observing the surgery of Korea's preeminent deformity specialist, Dr. C-S Lee.  I was impressed by his flair and elegance as well as the fascinating process that had gone into the development of his technique.  As with all my other hosts, he was friendly and welcoming and genuinely interested in my thoughts on everything from his screw placement and curve reduction to the skating of Yu Na Kim (I agree with him and his whole country that she was cheated out of Olympic Gold, but that is a conversation for another time).

Dr. C-S Lee and me

I was thoroughly enchanted by my visit to Korea.  I was treated with fantastic courtesy and amazing hospitality.  But more importantly, I was impressed by the dedication and the skill of the spine community in that country.  I hope that I can continue to interchange ideas and to learn from them and I certainly hope that my future includes another visit.

BRACING FOR SCOLIOSIS: PROBLEM SOLVED?

For years, one of the great controversies in the treatment of scoliosis has been the use of bracing.  Despite almost a hundred years of (mixed) experience, the scoliosis world divided itself into three camps.  On one side, were the Ardent Bracers – “Give me enough Plastazote and I will conquer any curve!”  The rival camp, the Nihilists, would rejoin, “It’ll never work!” like the character in the child’s cartoon.  Somewhere in the middle was a pragmatic group which would use the braces with modest expectations, sometimes because they knew that a brace must work in some settings or no one would have invented in the first place and sometimes because they had nothing better to do.

In October 2013, a landmark article was published in the prestigious New England Journal of Medicine titled “Effects of Bracing in Adolescents with Idiopathic Scoliosis” with the lead author Dr. Stuart Weinstein from University of Iowa.  The article reported on the findings of a multicenter trial (the BrAIST trial) involving both randomized and preference groups of braced vs. observed patients.  72% of the braced group did not progress to surgical curves by maturity vs. 42% in the observed cohort.  The trial was in fact so successful that the group applied the research equivalent of the Mercy Rule – they stopped the trial well before its scheduled time because the numbers were so one-sided.

This is a landmark for several reasons.  For generations, the holy grail of bracing literature was how to do a suitably ‘controlled’ study with the devices.  After all, you can’t fool a patient into thinking they are wearing a brace when they are not (and vice versa).  And you can’t hide the brace from the doctor.  Combining the randomizing with a preference group would seem to balance out some of the inherent bias in any brace study.  Although I believe that there is still an ‘apples vs. oranges’ effect inherent in the fact that every curve has its own personality, the relatively large groups involved would tend to even this out (although I am surprised that the authors could only come up with 242 patients for this study – why not add another few centers and get up to four- or five hundred?).  So here at last is the first recognizably scientific paper on bracing, and it is in favor of the devices.

 

The favorable outcome for bracing is in itself landmark in a surprising way.  Dr. Weinstein, while not a true Nihilist has been known in the community to be skeptical to say the least about braces.  I was involved in discussions with him and Lori Dolan, PhD at a national meeting not long ago, and they seemed convinced that the study would go quite the opposite way, citing the difficulties in getting kids to wear the braces and the somewhat suspect effectiveness as a reason why bracing is not cost effective.  The numbers in this study must be truly convincing if Dr. W has come around to the pro-brace side.

The third and most astonishing thing about this study is that it was published in NEJM.  The Journal as a rule is the bastion of Internal Medicine and Public health studies.  It is unusual to say the least for them to publish a surgical paper at all.  The last time they even acknowledged the existence of orthopaedics was to publish a very negative article concerning the use of a specific knee arthroscopic technique.  I’m not sure they even know how to spell ‘pediatric orthopaedics.’  For them to feature a work on a mechanical treatment in Pedi Ortho is as remarkable as when the Red Sox first won the World Series.  But there it is, in proverbial black and white.  Which means that the paper must be remarkably rigorous from a statistical point of view (and it seems to be) and it must be of really significant clinical importance.

So does Dr. W’s paper solve the question?  Are the Nihilists now going to throw down their standards and take up Boston braces with a hearty “Huzzah?”  Probably not.  There are still important questions about which curves do best with bracing, when to start (age and magnitude of curve) and when to stop. The paper rather tantalizing brings up the amount of time in the brace as an important variable but falls short of any clear recommendation.  What is the effect of genetics of the curve? Will the ‘successful outcomes’ in this study hold onto their success or will they progress even after maturity?

Most importantly, what kind of brace is best?  I will discuss the types of bracing a future post, but there are rigid and flexible, daytime and nighttime types.  Until the community can come to a real consensus as to who to brace, how long to brace and what brace to use, the controversy will rage on and on.