Friday, May 25, 2012

Sprengel Deformity


Sprengel deformity is an uncommon condition in which the scapula (shoulder blade) on one side is higher in the neck than the other side.  Normally, during prenatal development, both scapula begin high in the neck and slowly descend toward their normal resting position in the upper back.  If, for unclear reasons, one scapula does not descend, a Sprengels deformity results causing two issues:

1) functional issues related to decreased motion of the arm.  Typically, the patient will have decreased forward flexion (raising the arm in front until straight above the head) and decreased abduction (moving the arm away from body in the plane of the body).
2) appearance issues related to the high scapula.  Essentially, it looks like a large bump in the neck.

When a Sprengel deformity is identified, associated conditions should be considered including Klippel Feil, scoliosis, cervical ribs, and torticollis.

Treatment depends on the severity of the condition- specifically how much the limited motion and abnormal appearance bother the patient/ family.   Therapy may be beneficial in improving motion but ultimately if a big enough problem, surgery is the best way to improve motion and appearance.

My preferred treatment is a modified Woodward procedure in which we removed the upper portion of the scapula (i.e., the prominence) and bring the scapula down to a more normal resting position.  The scapula is not normally sized and so we have to be careful not to overcorrect or bring the scapula down too far.  Depending on age, we may also perform an osteotomy (i.e., cutting) of the clavicle to reduce the risk of nerve injury during the procedure.   The preferred age of the surgery has gradually become younger and we prefer surgery between 3-8 years of age.  Younger patients may have a better outcome as judged by the improvement in motion.  Older patients can also have a very good outcome with improvement in motion and appearance.


Sprengel deformity at rest.  Notice elevated shoulder on left.
Sprengel deformity with limited arm elevation.

Xray of Sprengel Deformity.  Note bump in left shoulder compared to right.  That is the high shoulder blade.

9 comments:

  1. Looking at this X ray, it appears the patient might also have scoliosis. Is that the case?

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  2. As mentioned in the blog,there may be several different conditions associated with Sprengels. I agree that this patient does have a scoliosis.

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  3. Dr. Goldfarb, our child has a relatively severe sprengel's deformity. She is 2.5 years now and we are planning to have the surgery when she is 4. We live in Denver, Colorado. Is Children's Hospital in Colorado a good place to have this done or shall we go to somewhere else?

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    1. Minghua,

      Thank you for the question- I get similar questions on a regular basis. When deciding on a surgeon, there are a couple of general comments which might help.
      1) Are you completely comfortable with the surgeon? Has he/ she answered your questions and explained the process?
      2) Has the surgeon had a good experience with the procedure? While Sprengels is not common, the more cases that your surgeon has completed, the more likely your child is to have a good outcome and the less likely complications are.

      I hope that helps. Good luck.

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    2. Dr. Goldfarb, thank you very much for your quick response and helpful input. Our surgeon told us the age won't affect the surgery outcome and we can wait until 10 or 12 yrs to do it. but from your post, and other research articles that I have read, it seems age does matter. Could you comment on that? And you mentioned neural damage, is this a common complication? Thank you very much!

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    3. Minghua,

      There is some evidence to suggest that surgery at a younger age can be more successful in restoring motion. We certainly have been operating on younger patients over the last 10 years.

      Neural damage is not common and, especially in older kids, we often perform a clavicle osteotomy to decrease the chance of nerve issues.

      Good luck.

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    4. Thank you very much for your help!

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  4. Dear Dr Goldfarb,

    Your blog has been really insightful to me as a 25 year old that suffers from this condition. Having seen one specialist who has advised me that there is nothing further that can be done it is refreshing to read that older patients can still have the same outcome.

    Have you done this surgery on older patients around my age and were there any additional complications?

    Zahira

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    1. Zahira,
      Thank you for your question and kind comment. I do believe surgery could be considered in the older patient although results seem clearly better in the younger patient. I would not expect motion improvement to any significant degree although appearance could be improved.

      Good luck!

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