Tuesday, July 16, 2013

Madelungs Deformity

Madelungs Deformity is a malformation of the distal radius which creates a deformity of the wrist.  It typically presents in adolescent females and often is bilateral.  Madelungs may be painful and may limit motion of the forearm and wrist.  There is a known genetic pathway for patients with a SHOX gene abnormality.   There genetics are also related to Leri Weill dyschondrosteosis which combines Madelungs with short stature and short forearm segments. We have written about the x- ray appearance of Madelungs and the different x- ray types: http://www.ncbi.nlm.nih.gov/pubmed/17996774.  Also, Relton McCarroll really advanced our understanding of Madelungs with descriptions and measurement techniques for x- rays: http://www.ncbi.nlm.nih.gov/pubmed/16344178

We believe that Madelungs deformity is caused by a growth plate abnormality of the distal radius.  Also, there can be a large ligament (Vickers ligament) which some believe contributes to the deformity. Patients typically present in the early adolescent years (10-14 years) and if conservative care does not help, surgery can be considered. If symptoms are on the thumb side of the wrist, we often consider an osteotomy (cutting) of the radius bone in a very specific way- a dome osteotomy.  Drs Carter and Ezaki pioneered this treatment for Madelungs and describe it in this technique article: http://journals.lww.com/techhandsurg/Fulltext/2002/03000/Volar_Surgical_Correction_of_Madelung_s_Deformity.6.aspx

The same group has published their results twice, short term and more than 10 years after surgery.  Both reports detail the success of the procedure for many patients.  The second article is from July of 2013- hot off the press!
http://www.ncbi.nlm.nih.gov/pubmed/17095381
http://jbjs.org/article.aspx?articleID=1698451

If the pain is related to the pinky side of the wrist, sometimes we only address the ulna bone and shorten it.

I recently had the chance to see a patient back more than one year after a dome osteotomy of the radius for Madelungs.  Her pain and her motion were both better and she and her mom were happy.  However, it is important to note that even if surgery goes very well, the wrist will not be "normal."  Additionally, later surgery can be required to deal with pain on the pinky side of the wrist.
Front view of Madelungs.  There is a great deal of deformity of the wrist
Side view of Madelungs with curving radius and split between the radius and ulna
White metal pins after surgery to hold the bone in place for healing in Madelungs Dome Osteotomy.



Appearance after surgery for Madelungs.  The wrists now look similar.


The wrist does not look "normal" after surgery for Madelungs but looks much more typical.
Side view after surgery for Madelungs.  Note that the radius and ulna line up much better now.

2 comments:

  1. Hello Dr. Goldfarb. Thank you for posting this. I am 40 years old, and I have relatively severe Madelung's in my left wrist, and is most likely a part of Leri-Weill dyschondrosteosis (I have Brachydactyly, and a lot of other goofy bone stuff and I'm pretty short). I was diagnosed with MD when I was in adolescence, and at that time a minimal procedure (removal of growth plate) was completed. I've learned to love my imperfections--(It's quite a party trick to show how one arm is shorter than the other...) Also, it seems as though I'm in much less pain than others with this condition. Ironically, I'm starting to learn about it more as I have been working with a personal fitness trainer. We've both been trying to figure out how far to push my wrist as sometimes it gets achy after any sort of stress. Anyway, I've realized how rare this condition is, and I really appreciate your blog.

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    1. Thank you for your comments. Pain with Madelungs (or for that matter, pain with many orthopedic conditions) is highly variable. The fact that you do not have pain now is likely a good predictor that you will not develop serious pain later. Most of the time when I see adults in the clinic for pain related to Madelungs, the patients are in their 20's. And I think you and your trainer are correct in carefully assessing pain with activities- it is probably best to avoid overdoing it. Good luck!

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