Friday, October 11, 2013

Severe Radial Longitudinal Deficiency

As with most medical conditions, different children will be affected at different severities.  These differences can be notable such that two kids labeled with the same congenital difference may look completely different.  While this obviously matters to the family, it also matters to the physicians because once we sort out a diagnosis (sometimes easy, sometimes not), we then have to give the best treatment recommendations.  Most of us have a preferred treatment based on a particular diagnosis.  For example, a believe in the concept of centralization for radial longitudinal deficiency.    http://congenitalhand.wustl.edu/2011/07/radial-longitudinal-deficiency.html
I also believe in precentralization distraction as a means to make the centralization procedure easier.  http://congenitalhand.wustl.edu/2012/05/fixator-for-radial-longitudinal.html   And I definitely believe in pollicization (making a thumb).  http://congenitalhand.wustl.edu/2013/09/more-thoughts-on-pollicization.html

However, there is no "one size fits all" solution for children with radial longitudinal deficiency.  Some mildly affected kids may need therapy only or a tendon transfer only to balance the wrist.  The thumbs may be reconstructed rather than treated with pollicization.  And centralization is not always the right choice.  For example, in kids with poor elbow motion (fortunately, this is rare in radial deficiency), centralizing the wrist can take the hand away from the mouth if the elbow doesn't move- bad idea.

I think this video is an amazing example of a child who makes us think about our normal treatment plans for radial longitudinal deficiency and what might be best for this particular child.   Watch the child function.  The wrists are markedly radially deviated but his hands are working straight ahead.  This is because the ring and small fingers are the digits that work best for him (amazing dexterity).  The thumb does not work at all and the index and long finger are limited.  When considering these facts, the typical treatment of centralization of the wrist and then pollicization of the index finger into a position of a thumb might not make sense.  This is because
1) Centralization takes the vital ring and small fingers out of the best position and makes them lie further away from where they are needed (i.e., more ulnar).
2) Pollicization outcomes depend on the quality of the index finger.  In this case, the index finger is not great and therefore when pollicized, it will not make a great thumb.

Therefore treatment for this child has to be carefully considered.  There are options but the best ones might be different from the typical "protocol."

Thank you to mom for allowing us to post this video.




2 comments:

  1. What are your opinions on centralization vs. ulnarization for pts. with RLD?

    ReplyDelete
  2. Mandybeth, thank you for your question. For those readers who are not familiar with the terminology, I will explain. And there is some lack of agreement on the terms. Centralization is the balancing of the wrist/ hand on the end of the ulna. It is the classically accepted procedure. Radialization was the overcorrection of the wrist/ hand on the end of the ulna with the idea that it better balances and decreases recurrence. This was described by one of the fathers on such surgery, Buck- Gramcko. Ulnarization furthers the correction of the hand/ wrist by placing it ulnar to the ulna bone and proximal to it as well (not trying to balance on the end of the ulna). I prefer the centralization procedure although it is not perfect and has a risk of recurrence. Ulnarization is not a well accepted technique amongst congenital hand surgeons perhaps because there are no published results. Additionally, it shortens the forearm/ hand segment. It does, however, achieve a good balance with a presumed low risk of recurrence.

    These are just my opinions. I hope they are helpful.

    ReplyDelete