Recurrent Radial Longitudinal Deficiency

The best treatment for the forearm/ wrist deformity in Radial Longitudinal Deficiency remains uncertain as I have previously outlined in a previous blog posting http://congenitalhand.wustl.edu/2012/05/form-and-function-in-radial-deficiency.html . The problem is that the anatomical deficiencies in Radial Longitudinal Deficiency limit our ability to sufficiently correct the underlying deformity. There is a lack of muscles to extend (bend back) the wrist and a lack of bony support for the thumb side of the wrist and forearm. The most common surgery to address this issue is a centralization (or radialization) procedure. The idea is that the tight structures on the thumb side (Radial) of the wrist are released, the ECU tendon is advanced (tightened) to help balance the wrist, and the wrist and hand are placed on the end of the ulna (i.e., centralized). We like the concept of the surgery as it lengthens the forearm, improves appearance and likely helps function overall. Obviously, however, if there is severe recurrence, we need to reconsider it.

For some kids with Radial Longitudinal Deficiency, the surgery succeeds in balancing the wrist and the alignment is maintained but for others, the deformity recurs. And by deformity, typically we mean both radial deviation (wrist and hand angle towards thumb) and flexion (bending down) of the wrist. There have been many different techniques utilized to try to prevent recurrence of deformity. These attempts include using a toe (with joint) including its blood supply to provide thumb sided support to the wrist. This broadens the platform for the wrist and may help prevent recurrence. However, this procedure is technically complicated and requires the sacrifice a toe without certain improvement in the long term.

An additional technique to minimize recurrence in Radial Longitudinal Deficiency is to use an external fixator prior to centralization. This stretches the soft tissues and makes the centralization more straightforward and balanced and seems to prevent recurrence. We have discussed this previously at http://congenitalhand.wustl.edu/2012/05/fixator-for-radial-longitudinal.html .

Ultimately, in those kids that do have severe recurrence after centralization, a fusion of the wrist can be a very effective procedure. Usually this procedure has to be delayed until at least age 12 when there is enough bone for the fusion. While the procedure sounds scary, it has many positives (by improving the position) and very few negatives (related to a loss of some motion- motion which is limited anyway). We like this procedure very much in certain kids.

Here are some clinical pictures showing recurrent deformity in an 8 year old with radial longitudinal deficiency.