Saturday, February 27, 2021

 

Post on Congenital Scoliosis #4                                 2-27-2021

 

1.      How much will the congenital scoliosis change with growth?

a.       Plain radiographs and MRI may be able to predict spinal growth and the chance of developing a spinal deformity or its speed of progression (worsening) in some patients.  This is more likely if there is a single area of abnormal vertebral development.  If there is more than one location, then predicting spinal growth or the risk or progression of spine deformity becomes less and less precise.


Simple: Single Hemivertebra           Complex: Multiple hemivertebra and bars

 

2.      Will my child grow normally?

a.      Half a person’s body height comes from their legs, the other half from their spine.  If the child’s legs are normal, then the legs will grow normally, which is determined by their genetics.

b.      The other half of a person’s height comes from their spine and head. 

 


                                                    i.     In congenital scoliosis the vertebra are abnormally developed and have altered growth.  The vertebra affected will never grow normally.  At present, we cannot make them grow normally, with or without surgery.

                                                   ii.     It is typically difficult to prognosticate early in the child’s life the amount of spinal shortening or development of spinal deformity that will occur. 

3.      So, if the spine will never be “normal” height what will that mean for the child?

a.      Their maybe some impact on the appearance of the child’s proportions between the body and the legs.  The body may appear shorter and the legs longer, proportionally, when compared to other children.

b.      The most significant issue is this decreased growth is the lungs and smaller volume of the lungs.  The lungs will not have the same space to grow maximally.  The less vertical growth of the child, the less volume of the lungs will develop.  Smaller lung volumes as a child don’t typically cause much impact, but in an adult can cause issues with breathing, respiratory infections, shortness of breath, etc....


3d reconstructions of lungs in a patient with congenital scoliosis


4.      What can be done to maximize the growth of the spine and the lungs?

a.      This is where it is crucial to work with a pediatric spine deformity surgeon closely.

b.      It is important to identify the location of the congenital deformity and the type of congenital scoliosis, using plain radiographs, and sometime MRI.

c.      Routine, scheduled follow-up with spine radiographs is essential.  Changes in the spine usually are difficult to see with your eyes alone.  Radiographs are essential to follow to identify the development of spinal deformity (scoliosis, kyphosis or both), and its progression.

d.      If there is a low magnitude (small) of the spine deformity, and it doesn’t progress/worsen, then observation is typically recommended.  This is ideal.

e.      However, if there is a high magnitude (big) deformity and/or there is significant progression then an intervention/treatment is often the best option.

f.       It is important to remember that every child and spine deformity is unique.  The pediatric spine deformity surgeon must look at the entire child, not just the spine problem to optimize treatment recommendations.  This means some treatments and surgeries may be preferred in some children, but not others.

5.      So if the child has a large magnitude congenital scoliosis and/or it is significantly progressing, what are the treatment options?


Large magnitude curve

a.      In general, the main nonsurgical option is observation.  The prevailing opinion amongst pediatric spinal deformity surgeons is that bracing does not change the natural history of the congenital scoliosis.  I agree with this perspective.  However, sometimes casting and bracing may be an option to guide the growth of the “normal” part of the spine.


Casting of 3 year old with congenital scoliosis

b.      The other treatment option is surgery.

 

 

Next week’s blog post will discuss surgical options for congenital scoliosis.












Sunday, February 21, 2021

 

Post #3 on Congenital Scoliosis                                            2/21/2021

 

Why is congenital scoliosis different than idiopathic, syndromic, neuromuscular?

               As presented 2 blogs ago, congenital scoliosis is the group of spinal deformity in children that is due to the vertebra being abnormally developed.  The other three diagnostic categories usually have normal vertebra development, for the most part.  So, in the other three the growth of the vertebra are also much more “normal” in their shape and have the normal growth centers on each vertebra.

What are the “growth centers”

The vertebra basically grow from birth to adulthood in 2 ways: 1) the height of the vertebra increases due to the presence of growth plates on the top and bottom of each vertebra, and 2) the width increases by appositional growth, meaning it gradually widens without a growth plate.  This widening of the vertebra is the same as in other bones of the body, such as the femur, tibia, etc…

https://core.ac.uk/download/pdf/33505427.pdf

It is important to know, the main problem with vertebra which are abnormally shaped, as in congenital scoliosis, is their abnormal growth vertically, not horizontally. Though the abnormally shaped vertebra do cause some of the scoliosis, it is the abnormal vertical growth of those vertebra causes the majority of the problem, creating potentially severe, progressive scoliosis.

Severe congenital scoliosis in a 4 y/o male

 

So, why do the vertebra grow abnormally?  Simply put, there are an uneven number of growth plates (with varying levels of growth capacity) on one side of the spine vs. the other.  Over a segment of the spine, if there are 2 growth centers on the left side, and 5 on the right side, the spine will grow more on the right side than the left.  The speed of the asymmetric growth varies, based on each growth plate present and other vertebra anatomy.

In the figure below, there is a partially formed vertebra which is wedge shaped. 

 

Now each disc, the area in blue means that there are 2 growth centers in the vertebra, one on each side of the disc, top and bottom.  This means on the left side there are 4 discs and on the right side 3 discs.  If each disc means there are 8 growth centers on the left and 6 on the right.  Hence, there will be more growth potential on the left than the right.


In the next example, there is a bony bar across multiple vertebra on the right side.

 

Using the disc concept we just talked about, there are 4 discs on the left, and one on the right.  This means 8 growth centers on the left and only 2 on the right.

 

More on congenital scoliosis next week….be safe

Sunday, January 17, 2021

 

60 Post on Congenital Scoliosis                                1-17-2021          

 

Frequently-Asked Questions about congenital scoliosis

 

1.      Why does it happen? Unfortunately we don’t know

2.      When does it happen? In utero when the fetus is very, very small and is very early in its development.

3.      What other problems can be associated with congenital scoliosis?  There are three other organ systems which have high abnormality rates in patients with congenital scoliosis: heart, kidney and spinal cord.  The theory is that during development something happens at one time point during development that can cause the congenital scoliosis and the other heart, kidney and spinal cord problems.

4.      How often are these other problems present? In 2019 we published a study looking at this very problem.  We reported on 305 patients from Shriners Hospital, St. Louis and St. Louis Children’s Hospital over a 25-year time span who had congenital scoliosis.  Our patient population 54% had a heart anomaly, 43% had spinal cord anomaly and 39% had a urogenital (kidney, bladder, etc…) anomaly.

5.      How to we check for these heart, kidney and spinal cord problems? Many patients have already had the imaging studies to check for these problems.  If not, then we need to assess if additional imaging studies are necessary. 

6.      What are the imaging studies to look at the heart? For the heart we order an echocardiogram, a painless study which looks at the heart function and for any small holes in the heart or valve problems.




7.      What is the imaging study for the kidneys? Renal ultrasound

8.      What is the imaging study to study the spinal cord? MRI (magnetic resonance imaging) scan of the entire spinal cord, from the brain to the sacrum).


This child has a spinal cord syrinx, which is a fluid-filled dilation of the spinal cord, and a Chiari malformation.


9.      Do we always get all three of these studies, for every patient? Not always.  We are more likely to get these imaging studies in the younger patients, if they have not already been obtained.  In the older patient, say in adolescence, we may not get an echocardiogram or spinal cord MRI.  This is because if they have been followed for many years by their primary care physician (who looks at them annually for school physicals) and they are not having any symptoms related to the heart or spinal cord, the likelihood of finding something wrong is very, very low.

10  What happens if something abnormal is found on one of these three imaging studies? It depends.  We usually consult other specialists as to the need for further evaluation or follow-up (kidney, urologists; spinal cord, neurosurgeon; heart, cardiology).

      More on congenital scoliosis next week.....