Blog Topic: Severe, complex congenital scoliosis cases using Shilla procedures

Over the last 6 Blog posts congenital scoliosis has been exhaustively presented and explained.  Most cases of congenital scoliosis are mild and do not require surgery.  Of those that do need surgery there are several well-accepted techniques for improvement and control of the scoliosis: distraction constructs (traditional growing rods, magnetically-controlled growing rods, VEPTR), resection with short/long fusion, fusion without resection, and resection with Shilla procedure.

This post will finish the postings on congenital scoliosis.

The last case which will be posted is a severe, complex type congenital scoliosis.  The major problem for this young patient (4 years old) who has two adjacent hemivertebra which is at the junction of the thoracic spine (chest) and the lumbar spine. These two hemivertebra cause an acute, severe deformity.

 

The management of this problem involved resection of both of the hemivertebra and fusion over the severely involved section of the spine.  Though there is instrumentation which goes high and low in the spine, these screws are Shilla screws, so they are not fused and will slide up and down the spine as the patient grows.

If there are questions about congenital scoliosis or any other post, please let me know…I am happy to answer all inquiries.

 

Blog Topic: Surgical Treatment of Congenital Scoliosis

 

As previously mentioned congenital scoliosis comes in a wide range of types, locations, and complexity. 

This means surgeons caring for this group of patients needs to have multiple surgical treatment options to optimally care for this potentially challenging deformities, from simple hemivertebra resections to complex vertebral column resections and Shilla constructs. The below three cases demonstrate the progression from simple to complex, both in terms of the deformity and the required surgery.

 

Simple: 7 year old male with a single hemivertebra treated with resection and short fusion

Moderate: 13 year old female with previously operated (outside hospital) on lumbosacral hemivertebra with continued worsening of deformity.  Underwent hemivertebra resection and longer fusion.

 

 

Complex: 8 year old male with Prune Belly syndrome.  Treated with vertebral column resection (VCR) at T10, and Shilla construct T4-L4.

 

On the more complicated end of the congenital scoliosis spectrum (i.e. mixed-type), the simple resection and short fusion may not be the best option.  This is due to the possible presence of adjacent areas of involvement which may induce increasing curves above or below the surgical site.  In the past if this was a concern after surgery, the patient may be placed into a brace, which often is less than ideal as these patients may need to wear a brace for many, many years. 

 

Shilla treatment of congenital scoliosis

The “complex” case above was treated with a VCR and a Shilla procedure.  However performing a Shilla procedure in less severe cases can be optimal.  The below case is a 4 year old male with two areas of congenital deformity (one higher and 31.5 degrees, and one lower and 46.8 degrees). 

CASE 1

 

Because of the upper deformity was congenital and more stiff (see below slide), a simple hemivertebra resection will not be able to control the upper curve.

So, at the time of surgery the left T10 hemivertebra (the lower curve) underwent a VCR (complete removal of the vertebra), and then Shilla T4-L4

The arrow on the below slide shows the closure of the gap when the T10 hemivertebra was resected.

Here is the 6 months after surgery follow-up.  His body shift to the left is significantly improved.  The only levels of fusion are within the white box (below).  The remaining vertebra above and below this white boxed area will be able to grow vertically along the Shilla rods.

The next Blog post will demonstrate complex congenital scoliosis cases using Shilla procedures

 

Blog Topic: Surgical Treatment of Congenital Scoliosis

 

When is surgery necessary?

               This is not a simple answer, mainly because there is a wide spectrum of deformities, from number of areas involved, type of problems, location, magnitude, progression, and age of patient, to name a few of the considerations.  Each patient is unique, so the decision of when surgery is a reasonable option is also unique.  Small magnitude, non-progressive deformities usually don’t require surgery, whereas large magnitude, progressive deformities more typically require surgery.

Small deformity                    Large deformity

No surgery needed                 Surgery needed

 

 

The basic concepts of surgical treatment of congenital scoliosis:

1.      Delay surgery as long as possible to permit vertical growth of the spine.  This can minimize the number of surgeries needed by the end of growth.  In addition, we like to avoid surgery, if possible, until after 4-5 years of age, at a minimum.

2.      Identify the areas of concerns and quantify the problem

3.      Only address the problem area(s).  This means to resect, fuse, and control the problem area(s) only.

4.      Fuse as few vertebra as necessary: this again permits more vertical growth

What are accepted treatments for congenital scoliosis?

1.      Distraction constructs or Growing Rods, both traditional and magnetically-controlled

               Distraction constructs are used occasionally in congenital scoliosis.  Very young patients, multiple problem areas, unknown progression can be factors in which a distraction construct may be used as a temporary method of treatment.

 

2.      Short fusion

12 year old male                      5 years after hemivertebra resection and fusion

1 hemivertebra

 

               A short fusion can work very well when there is a single area of problem.  In the above case the hemivertebra was resected, the defect was closed down…..and fused, which prevents this area from growing crooked.

 

 

Diagram 1                                Diagram 2

Diagram 3

Diagram 1 demonstrates an isolated, single hemivertebra (triangle).  Resection (diagram 2) creates a triangular defect which is then closed down, straightening the deformity (diagram 3).  For this to be successful treatment the spine above and below has to be fairly straight.

 

3.      Short fusion and growth guidance (Shilla)

We will present this surgical treatment next week……..