Re: Scoliosis and kyphosis in CMT Disease

[Guest guest]

Scoliosis in CMT Disease

by Dr. Kamal Ibrahim MD, FRCS© Loyola University Medical Center,

Chicago

Scoliosis is a side to side or lateral curve of the spine. Sometimes

this side curve is very slight and can hardly be seen. In more severe

cases, however, the spine begins to look like a letter S or a long C.

The patient with true scoliosis may appear to slump or lean to one

side. This should not be confused with poor posture which corrects

easily when the patient simply stands up straight. When a patient

with scoliosis stands as straight as possible, the back is still not

straight.

In Charcot-Marie-Tooth disease, there is a muscle imbalance due to an

inherited condition affecting the nervous system. As a result of this

muscle imbalance, scoliosis could develop and continue to progress.

Very little is written in the literature about scoliosis and Charcot-

Marie-Tooth disease, therefore this report is a result of review of

the literature and my personal experience.

The incidence of scoliosis occurring along with Charcot-Marie-Tooth

disease is about 10 per cent. That is to say, out of 100 patientss,

10 may develop scoliosis. If the scoliosis occurs, it usually starts

late in the first decade of life or in teenagers. Half of those

patients with scoliosis and CMT will have a small to moderate degree

of scoliosis which usually does not progress and will not need any

significant treatment while the other half of the patients will have

scoliosis which will progress quickly and may end up needing surgical

correction. This course of the disease behaves somewhat similar to

scoliosis in normal teenagers.

Very little information is available in regard to scoliosis in adults

with CMT. From my experience, I believe that it will not occur in

adults de nouvelle, but usually it is a progression from adolescent

scoliosis which was not treated and continued to progress to a very

significant degree.

KYPHOSIS ( " Round Back " )

If the backward or rounded curve in the upper spine when viewed from

the side is too great (round shoulders or humped back, a condition

called kyphosis is present. When the inward or forward curve in the

lower back is too great the condition is called lordosis (swayback).

Many patients with scoliosis have kyphosis and lordosis as well.

Kyphosis or excessive round back, sometimes called Scheuermann's

disease, is a common problem. It has to be distinguished from poor

posture. It is best seen in the bending test. When the child with

kyphosis bends forward there will be a sharp bend or angulation of

the back instead of the expected smooth curve.

DIAGNOSIS

Children should be checked regularly every year, even by their

parents. The child should be asked to stand straight and the observer

should look at the child's back to notice any uneven shoulders or one

shoulder blade being more prominent, or the space between the body

and the arms are unequal, the hips may be uneven one appearing higher

or more prominent than the other, or if the child has some difficulty

in clothes fitting properly or having to adjust the hem of a skirt or

slacks. When a person with mild scoliosis bends at

the waist, the sideways curvature of the spine becomes more obvious

and would appear as if there is a hump in the back. That is to say

one side of the back is higher than the other side. If any of these

findings are observed by the parents, then the child should be

examined by a physician and X-rays should be taken and repeated every

year or more frequently if the scoliosis shows progression and is

getting worse.

TREATMENT

Treatment modalities in those patients and the result of treatment is

again similar to those of normal children who have scoliosis.

If the curve is very mild, then the child should be observed. If he

does not show any progression, then no treatment is needed. Exercises

have no rule in correcting scoliosis, although back exercises are

generally good for the well-being of CMT patients. If the curve shows

progression and it is of a moderate degree, then a brace can control

the progression until the child matures which is around 14 years for

girls and 16 years for boys. Brace treatment is successful to achieve

these results about 80 per cent of the time. There are many kinds of

braces used for scoliosis, but generally they are divided into two

groups, braces which go all the way to the neck and braces which stop

under the arm. Each type of brace is used according to the part of

the spine that has the scoliosis.

On the other hand if the scoliosis shows sudden and fast progression

toward a significant curve above 40 degrees, or if the brace

treatment is unsuccessful in controlling the scoliosis, then the

advised treatment is surgical correction of the scoliosis with fusion

of the part of the spine that is curving.

The technique that is used now for this surgery involves inserting

stainless steel rods in each side of the spine, hooked to the spine

with multiple hooks. This way the curve can be corrected to a great

extent and fused in that position so the scoliosis does not come

back. This surgery is now performed with a great degree of safety

using a state-of-the-art technique which is known by the name of

Cotrel-Dubousset internal fixation. With this new technique patients

usually leave the hospital after one week from surgery without any

cast, brace or external support with great results and a high success

rate. The same techniques of surgery can be used for adults with

scoliosis to correct their curve and prevent further deterioration of

the spine.

In patients older than 50 years with very severe scoliosis, this

surgery might be risky and full of complications. Their symptom,

which is usually back pain, can be relieved with a body brace to work

as an external support to decrease the range of motion of the back,

this way the pain could be relieved.

The complications of untreated, progressive scoliosis occur mainly in

late adulthood and are back pain and problems with heart and lung

functions.

SUMMARY

In summary, scoliosis in CMT should be detected early in children

during the first and second decade of life and watched carefully. If

it does progress, then treatment should be implied in form of bracing

or surgical correction and fusion. For adults who have curves beyond

surgical treatment, they can be helped with a body brace, and if they

are using a wheelchair, then some scoliosis modification in the back

of the chair would be advised to help provide more comfortable

seating and prevent excessive back pain so that those patients will

be able to deal with daily living activities.

[Guest guest]

Hi and others,

Here's something to think about/ask your Physical Therapist about - the

Traction Table.

My PTC/Gym has one and I had the opportunity to 'try it out'. It is electric,

quiet, no needles, no pain, no stress on the body. The motion of the table is

very gentle and slow. I asked our head PT if the Traction Table would help

someone with scoliosis or kyphosis and she said " yes " .

Here's a link to what the tables looks like - there are many kinds

http://www.source1medical.com/s.nl/sc.2/category.49/.f

Gretchen

[Guest guest]

Wow!

Thanks for this info. I knew there had to be other therapies. Thank you for your

research and inquiries. I'll check this out first thing tomorrow morning.

Best,

_____

From: [mailto: ] On Behalf Of

gfijig@...

Sent: Tuesday, September 02, 2008 4:34 PM

Subject: Re: Scoliosis and kyphosis in CMT Disease

Hi and others,

Here's something to think about/ask your Physical Therapist about - the

Traction Table.

My PTC/Gym has one and I had the opportunity to 'try it out'. It is

electric, quiet, no needles, no pain, no stress on the body. The motion of

the table is very gentle and slow. I asked our head PT if the Traction Table

would help someone with scoliosis or kyphosis and she said " yes " .

Here's a link to what the tables looks like - there are many kinds

http://www.source1m <http://www.source1medical.com/s.nl/sc.2/category.49/.f>

edical.com/s.nl/sc.2/category.49/.f

Gretchen