The topic to be discussed will be the orthopedic 

and rehabilitation aspects as well as 

the prevention of severe deformity in 

Children and adolescents with neuro muscular 

diseases.

When one thinks of Children with neuro 

muscular problems,

one tends to think only of the Children with pseudo 

hypertrophic muscular dystrophy as in this 

boy with the severe deformity and the 

relatively short life expectancy 

here,

probably little need be done for many of 

these Children.

But occasionally they can benefit from surgery 

and long legged bracing as shown in this 

child and is discussed in 

other lectures.

The topic.

However,

today revolves more around Children with 

other neuro muscular problems other than the suitor 

hypertrophic occasionally.

However,

even in the most severely involved child,

such as the feet shown here,

the child can benefit by heel cord 

length earnings or minimal re sections of the 

heel cord to allow the feet to come up into better 

position for Kaz missus as 

well as better shoeing.

Also at times,

even in the most severely involved child 

minor splitting can be used such as 

these light plastic splints which can 

allow the child a little freedom without 

encumbering them with heavy braces and the need 

for any extensive surgery.

However,

there are many foot deformities such as this 

child with shark oh Marie Tooth where the 

feet are in the various position.

This produces instability because of the 

small pressure points.

And also has shown from this photograph taken 

from beneath on with the child standing 

on a on a glass plate that 

the force is distributed on a small area of the 

foot leading to calluses and pressure 

sores.

And by relatively simple surgery of 

realigning the tendons.

And on occasion,

doing Arthur DCIS of the small bones of the feet,

the foot can be brought into much better position,

giving a much better appearance and also a 

much better functional result 

at times,

braces,

especially worn at night,

such as these high top shoes with 

single upright bar braces can keep the feet in the 

neutral position when the child is not 

walking and therefore prevent 

deformity from occurring.

And if it is recognized that there is a potential for a 

severe deformity,

sometimes this can be very beneficial in 

retarding the time when significant deformity 

is present and even at times preventing its 

occurrence,

Here is a young man with a neuro muscular 

problem that was not diagnosed.

He had a muscle biopsy and was 

allowed to stay in bed for a few days.

His mother also felt sorry for him after 

his surgery and allowed him to be relatively 

inactive for the next 3-4 weeks,

he developed a significant a 

quietness,

deformity of both feet which has 

necessitated his use of crutches,

which was never needed before.

And he has had a significant setback just from his 

treatment program.

It has felt that if a biopsy is to be done on 

a child that is active,

it should be done.

And the child back up on his feet in a shorter 

time as possible to prevent this side 

effect,

which is unnecessary,

branching off into the other causes of neuro 

muscular problems.

One of the more unusual things is congenital 

insensitivity to pain where the child 

cannot perceive pain and at times will 

bite their tongue and produce the deformity.

As shown here associated with 

this can be deformity of joints 

because the patient cannot properly 

perceive minor degrees of pain 

and can end up with gross deformities such as this 

boy with his right knee,

which from the back view shows that it's 

completely distorted and can 

only be controlled with a long leg brace 

because on X ray,

it has shown here that there's complete deformity of the 

joint.

The femur has slipped off the tibia and there's no 

satisfactory reconstructive way to 

repair this other than attempt to 

control it with external bracing.

At the same time,

these Children should be warned of the potential for this type of 

deformity.

And the family should be careful as possible to 

prevent serious or prevent them 

from doing things that will result in 

serious injury and gradual deformity 

and even at the minor signs of 

deformity,

swelling,

any type of heat and the joint,

it should be protected with some type of splitting to 

prevent this serious problem.

Another unusual condition has been the 

association with dislocated hips as is 

shown in this 2.5 year old child 

with the neurological condition of central 

core disease.

And if this is not recognized as a combination,

it may make the treatment of both conditions much 

more serious.

This child had a reduction of the hips 

by placing the lower extremities in 

traction and then reducing the hips back into 

the hip joint and holding the child in a cast for 

a period of time.

Following this,

it is showing that the hips are pretty much reduced 

back into the femoral heads are pretty much reduced 

back into the acetate but are not deeply 

seated into the into the joint.

As one would expect if this was a common,

usual type of congenital dislocation of the hip.

This was followed up by osteo autumn,

ease of the proximal femurs,

directing the forces into the A C tabula 

and a much more satisfactory 

result has,

has been obtained.

Here is the little girl after she is back up on her 

feet.

After having her hips reduced of 

interest.

Her brother came along a year and a half later with the 

same problem.

A problem was recognized earlier,

his hips were reduced at an earlier 

stage and it was done in a much simpler way 

and,

and there was not the need for the osteo economy.

At this time,

the family was worked up for the uh 

for the problem and showing that the father 

has the same type of condition of central core disease,

but to a much lesser degree and did not 

have the associated dislocation of the hips.

So that when one has a family history of the neuro 

muscular problem and has an associated 

dislocation of the hip problem.

The connection between central core disease and the 

dislocated hips should be 

evaluated.

Moving on to the problem of the spine,

which is very common in neuro muscular problems because 

many of these Children have weaknesses 

and therefore there is a risk of the spine can,

can go off into a deformed position and produce a 

scoliosis.

The orthopedic surgeon has had experience over the 

years with the neuro muscular problem of polio 

and has gained considerable experience in how to take care of 

problems which now can hopefully be transferred 

to the other more unusual and 

exotic types of neuro muscular diseases.

This was a boy with polio who was seen 

approximately 10 years ago.

His vital capacity was only 400 

CCS.

He was unable to walk more than a block 

because of the deformity of his chest,

not because of his basic polio,

but because off the weakness produced,

his chest had become deformed and had 

decreased his vital capacity because of the 

secondary pressure on the lungs 

By a process of traction,

followed by internal fixation with a Harrington 

Rod and a spinal fusion.

His spine was corrected to a considerable degree.

He was able to stand much straighter.

His vital capacity increased to over 

1200 CCS and he was able to walk 

many blocks.

In other words,

the correction or improvement rather of the 

scoliosis has allowed this patient 

a increased longevity,

not because of his basic polio,

but because of correction of the secondary 

deformity of deformity of the chest itself.

This is a young girl with Nima Lean 

Myopathy who was 16 years of age and had the 

diagnosis made some 6-8 years 

before being evaluated here 

over the interval,

she had been progressively shorter of 

breath and came to see us just 

because she was unable to walk any 

distance,

not because of her basic neuro muscular problem,

but because of shortness of breath.

And here she is still up on her feet and able to 

walk.

But her vital capacity is essentially down to 

zero.

This child or adolescent young lady 

rather died a few weeks after this 

because of the secondary deformities 

of her chest,

not from her basic mammalian myopathy 

itself.

It is true that the basic neuro 

muscular condition may have might in the long time,

have shortened her life to some extent.

But the secondary scoliosis is really 

the thing that caused her early demise.

And earlier stage stabilization of 

her spine might well have allowed her to be alive.

Today,

here is a curve showing 100 and 23 

degree curve at 15 years of age.

And this is producing not only cosmetic 

problem but is producing secondary 

cardiovascular pulmonary problems because of the 

severity of the problem itself.

The next child in line was a floppy 

baby first seen at a few years of age.

At that time,

an X ray was taken of her spine at two months of 

age,

which showed it to be essentially straight.

She was followed along and gradually got up on her 

feet.

And here's an X ray of her spine at 12 years of 

age,

lying down or Supine,

which shows only a 12 degree curve I 

think of interest and significance is the fact that on 

the same day by standing up,

the curve increases to 32 degrees,

which is much more significant than the Supine 

X rays.

And would suggest that as these Children are followed,

that they should be followed with standing or 

sitting X rays rather than depending on the 

Supine X ray alone,

which do not show any of the dynamics of the 

problem itself.

By 14,

the curve has gone up to 

1660° 

And by 15,

it has increased further.

At that time,

a external support was applied which 

has no influence,

no significant influence on the curve itself.

In fact,

at this stage,

an external support may be a detriment 

because with the deformity of the chest that already 

exists,

further pressure on the ribs may further 

decrease the pulmonary function.

She went ahead and at 16,

had a 77° curve.

And at this time was very short of breath.

She was still able to walk because of the 

strength.

She had adequate strength in her lower extremities to 

allow this but did not have the pulmonary 

function to sustain.

At that time,

she was placed in traction stretched out 

and at a spinal fusion was done 

in an effort to realign her spine.

However,

in spite of all efforts,

she was uh she did very 

poorly post operatively.

Her pulmonary function was in such poor shape 

that she went on and died a number of weeks thereafter.

This has been considered a surgical death,

but it's most likely that you would only have lived a number of 

months anyway.

And it is our hope that if these Children can be 

found earlier and something done at an earlier stage 

that this type of thing could be prevented 

as far as external devices are concerned.

The Milwaukee brace has been shown here with 

traction under the neck and,

and a pelvic girdle and pressure across the points of the 

curve have allowed good stability of the 

spine.

But more related to the Children with the 

idiopathic type of scoliosis are the ones where we do 

not really know the ideology and has had 

relatively little value in the severe 

paralytic because of the pressure against the ribs.

And often the difficulty that the patient already 

experiences in holding up his or her 

head away from the chin piece at 

times,

an Ortho plast or other types of material has 

been used to encircle the body.

But this has some difficulty because 

again,

because of the pressure against the ribs,

which I'll and the already decreased pulmonary 

function.

And again,

maybe a liability in the neuro muscular Children.

So that on the average,

very little in the way of external support will 

prevent the natural progression.

However,

if the child has a relatively mild neuro muscular 

problem,

This type of material can be worn as shown in this 

child with 11 years of age with a 

47° curve that being placed in 

a jacket under arm type of device,

the curve can be reduced down to 

20°. This is probably 

only at best a temporizing thing till the child 

is old enough to benefit from a 

spinal fusion.

But at times can be helpful but must be used 

with great caution because of the risk of pressing 

against the ribs and and decreasing pulmonary 

function.

Another patient with a 

severe left thor ical lumbar 

or curve that's down somewhat lower in the spine,

which shows that 14 degrees years of age,

that this curve has reached over 90 degrees.

This is not only cosmetically unappealing,

but since all the alignment of the 

spine is is since 

produces a misalignment of the spine.

It can produce problems in gate 

by preliminary traction 

was 

And followed up by internal fixation by 

16 in a year.

Post operatively,

she has a very satisfactory improvement in her 

overall overall deformity.

However,

in the short range,

something has been sacrificed in that before the 

surgery,

she was able to walk without crutches.

But here she is having to use crutches 

because she has developed somewhat further 

greater weakness during the time that she has been 

lying around at the time of her surgical 

procedure.

It therefore becomes very important 

that these Children are placed on a strenuous 

rehabilitation program before their surgery 

soon after their surgery.

And after their surgery,

also internal fixation as was done in this 

girl should be used for possible so that the 

patient can be back up on their feet as soon as 

possible after surgery and not stay in bed for 

many months.

So a combination of strenuous 

and vigorous physical therapy,

both at the time of hospitalization and on a 

vigorous home home program.

And also with allowing the patient to get back up on her 

feet and get going as soon as possible after the 

surgery has allowed very little in the way 

of loss of strength and and an 

ability through the surgical procedure.

But most of these have to be kept in mind.

And here's the same young lady having discarded her 

crutches and back and going again without her 

crutches,

a year to a year and a half after a 

procedure.

So here's her X ray before the operation 

and after the operation showing that her spine has been 

considerably better aligned and that 

it should prevent further progression and she should be 

the better for the procedure.

However,

it must be pointed out that the rod is only a 

stabilizing device and that the actual 

long term result is a result of a spinal 

fusion or bone that's been placed in along the 

length of the of the metal strut 

to provide intrinsic stability for the 

future.

Here's another young lady with a 64 degree curve by 

the time she is seven,

which by eight has gone up to 72 degrees 

and this would seem a much better time to,

To do some type of procedure rather than to wait till the 

child is older.

There's nothing magic in one particular age 

group that if the deformity is increasing and 

cannot be controlled,

it is better to stabilize it at this point.

And here she is at age 18,

some 8.5 years after her surgical 

procedure with a good stability of her spine.

Here she is on the left before her 

surgical procedure and on the right.

Some six to so years later,

this girl was basically a sitter and in the 

Kuperberg Wilander group and has done 

very well.

She's been able to go to school.

She's been able to maintain herself,

she's completely independent and other 

than walking has leads essentially a normal 

life and there's no doubt that to have 

stabilized.

Her spine has given this child a much better procedure for 

the future.

Here are two sisters,

the older sister starting out at two years and nine 

months with essentially straight spine.

By the time she has aged 10,

the curve is significantly increased.

An under armed jacket was attempted,

but with no stability.

And by the time she is 12,

the curve has gone up to 67 degrees.

At that time,

internal fixation was used here.

She is a year later and again,

three years later,

the curve has been essentially stabilized.

There's been no further progression in her curve And 

this represents not only an improvement in 

appearance but the chance should be minimal 

now that she will get into trouble with her cardiovascular 

pulmonary problem because of secondary deformity of 

her chest,

her little sister by age 

eight had a curve at 24°. And by the 

time she's nine,

this has gone up about 40°. It 

was felt that because of the progression in this girl that this was 

a much better time to do a procedure 

and a liner at 10 rather than allowing 

her to go on till the deformity is more severe.

This allows for a more complete correction of the deformity of 

the spine because the spine is more 

flexible.

It also allows a simpler surgical 

procedure.

Because if the deformity is very severe,

one must count on using 

internal traction 

preceding the internal fixation rather 

than just doing the 

the surgical procedure itself as was 

possible in the younger sister.

The uh internal fixation 

allows for good stability of the spine.

But it must be re emphasized that the long 

term effect is that of the spinal 

fusion that must accomplish must 

accompany the internal fixation and not 

depend on the on the internal fixation 

alone.

The next is a physician son who was 

diagnosed as having Kupferberg Wilander disease.

He had weakness since early childhood but 

had little if any increase in his weakness.

Over the past few years,

he had walked temporarily at 5-6 

years of age but had been in a wheelchair into his 

adolescence.

At this time,

he was having marked pain in his 

thor ical lumbar spine because as 

he sat,

he curved more to the side and the pain became 

significant to the point that he could not attend school 

and was on homebound instruction because of 

the fact that his neuro muscular problem was 

basically stable and because he was having 

significant pain,

it was felt that his spine should be stabilized and he 

had a spinal fusion and internal fixation.

This boy has held very nicely and it's 

2.5 years.

This the spine has stayed essentially in its 

same degrees of curvature.

He has been attending college and 

the spinal fusion has allowed this boy 

to re enter the,

uh,

the outside world rather than having to stay at 

home with,

with pain.

One other type of problem that's encountered is 

the post traumatic paraplegia.

This girl had a meningitis 

and essentially a transaction of record at the 

upper thoracic area.

As far as function is concerned as she sits,

she shows a moderate scoliosis to the left.

But as she lets go with her upper extremity,

she curves over to the side.

So not only is she paralyzed in her lower 

extremities,

but she must use her upper extremities to main stability of her 

spine.

And therefore,

this was considered an indication for 

internal fixation.

Here,

she is sitting showing an 80° curve which 

after internal fixation allows her to sit much 

more upright and she's been able to 

uh regain function which she did not 

have and free or upper extremities for other 

more important work.

And she is now employed as a secretary 

and is is quite satisfied with the result.

The other neuro muscular problem associated with 

scoliosis is the Riley Day Syndrome or 

disorder nemea where the child has 

many,

many problems.

But included in many of these are a significant 

scoliosis.

This was a girl of 16 with a 

78 degree scoliosis,

which at this point was not only pushing 

her to one side and making mobility 

rather difficult but also beginning to impair her 

cardiovascular pulmonary function.

In spite of all the difficulties which have been 

recognized with the card with the Visa 

motor system in the Riley Day Syndrome with 

good anesthesia.

This girl had internal fixation.

She got a satisfactory result except that her 

spine shifted somewhat to the left but not 

terribly severe.

And some eight years later,

she has gainfully employed as a librarian.

And although she's tipped slightly to the side,

it has stabilized her trunk and therefore 

cut down the risk of cardiovascular pulmonary 

problems for the future.

And therefore by should have 

prolonged her life and louder,

more freedom rather than wearing external devices as 

well.

A boy with Friedrichs,

a taxi who just barely getting about with 

100 and two degree curve,

getting into trouble with the alignment of the spine as 

well as the risks of pulmonary instability 

with preliminary traction.

This could be reduced down to 36°. 

He was then fixed internally here,

he is up and about soon after his surgery.

And although this did not allow him more freedom as 

far as getting about with his lower extremities,

it has stabilized his spine to prevent further 

progression of his curve.

And as allowed him to be 

a better citizen in other ways.

And here he is 2.5 years after his spinal 

fusion with good stability of his spine allowing 

him to be a much better sitter,

although it has not really changed his 

locomotive stability or use of his,

of his lower extremities.

One of the other unusual types are the 

so called Becker or unusual form of 

hypertrophic muscular dystrophy.

In this patient has was expected 

to not survive into the late teens 

because of the early changes.

However,

he rapidly or he stabilized.

And here he is when he attempts to sit 

has a curve of 100 and 14 degrees which 

essentially causes this patient to have to 

stay in,

in bed With the use of 

traction.

He was pulled curve was 

realigned to the level of 64°, which is 

still a considerable degree.

But with the use of external fixation at this 

point,

the patient has been given a second opportunity 

to set up to some degree and has 

made life more tolerable no 

longer probably but more tolerable,

but it would have been much better if the potential for 

this deformity had been recognized periodic x 

rays taken and that the sign that this was 

progressing to have used some type of external 

support or if it became apparent that the child was 

going to live for a long period of time to have 

done a spinal fusion as was shown in the 

previous Children.

One of the most recent things that have 

been allowed us to do more with the spine has been 

the the Dwyer procedure which is 

an anterior approach to the bodies of the vertebra 

themselves developed by Dr Dwyer from 

Australia.

This has had a particular indication 

in the Children with muscular 

with uh with spina 

bifida where there are no poster 

elements to do a proper spinal fusion.

In this case,

by using this series of screws and 

cables,

one can stabilize the spine from the anterior 

approach.

However,

this is a much more difficult procedure requires 

longer and it's probably not as 

indicated in the uh in the 

typical neuro muscular problem,

except in the spina bifida Children where the 

poster elements are missing or whether it's a very,

very severe lord.

Doses here shows the series of 

screws and and the cable connecting them in the front 

of the spine.

And this requires as was mentioned a much more 

elaborate approach to the spine than doing a simple 

posterior procedure.

Well,

in summary,

I would suggest that if continuous x 

rays are taken on these Children probably at six months 

to yearly intervals,

that one should not in the future.

See this type of deformity of a 38 degree 

curve at age 12 that goes up to 77 

degrees by the time the child is 16 and 

so compromises or cardiovascular 

pulmonary systems 

that she dies soon there afterwards.

The hope is that if it can be recognized even earlier 

than this,

that one can convert the child over to something that's 

not only more appealing from appearance point of 

view,

but also hopefully give them a better 

life while it 

exists.

I would however,

income in final summary state.

However,

that the need for this type of procedure is 

relatively little in the same time 

that 495 

Children have been operated on in our 

institution for idiopathic scoliosis.

92 have had procedures for congenital 

scoliosis.

That is where the bones have been formed incorrectly and 

allowed to and,

and causes a secondary deformity which is relatively 

rare during this period.

Only 20 Children have had the procedures that have 

outlined today for the spinal muscular atrophy,

the shark.

Oh Marie tooth of this oughta know Mia.

And it therefore,

although it's not common,

it should be considered,

but also would point out because of the severe 

problems encountered in these Children that it 

should be thought of as a team approach.

And hopefully the procedure carried out by a 

team that's well versed in the usual 

forms of treatment of scoliosis.

Thank you very much.