Monday, July 25, 2011

Radial Longitudinal Deficiency

What is Radial Longitudinal Deficiency (dysplasia)?Radial longitudinal deficiency (RLD) is a lack of formation of the thumb side of the upper extremity. It can affect the hand only (usually the thumb) or, more commonly, the hand and forearm. It is a lack of bone, muscle, tendon, nerves, and blood vessels. The severity is different in each affected child.

What are other names for RLD?The most common other name is radial clubhand. It is also sometimes called radial deficiency for short.

How does RLD happen?The arm forms between 4 and 8 weeks of gestation, sometimes before a mom even knows she is pregnant. By 8 weeks the arm is fully formed although obviously really small. If there is some insult to the developing arm, part of it may not form normally. There are different types of insults- some are genetic and some may be caused by outside influences (“environmental factors”).

Are there are medical problems associated with RLD?There can be important other medical problems associated with RLD. Because the arm and the important internal organs such as the heart, intestines, blood cells, and kidney form at about the same time, one insult can affect all organs. Therefore, problems in these other areas need to be evaluated and often treated before the arm is treated.

4 comments:

  1. Dear Dr Goldfarb
    If you could take a moment of your tike and say somethijg regarding the centralizatioj and ulnarization procedure in radial club hand procedure. So far my understanding is that the ulnarization procedure has far less reacuring risks with rdl. And that the appearance is much better due to the fact that the ulna bone is lengthened and straightend in the proces. Were as to the centralization process has a high reacurriblng rate and the ulna is not lenghtened nor straightened. If you could comment on this . thank you

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  2. Alen,
    There is limited data on outcome for ulnarization. Those of us who treat children with radial deficiency are somewhat split between favoring centralization or ulnarization. Neither procedure actually lengthens the ulna but by placing hand on end of ulna, the arm is effectively lengthened. Both straighten the extremity. Recurrence is a concern with centralization. The use of an external fixator prior to centralization seems to decrease that risk (my current approach= external fixator followed by centralization). Thank you for question.

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  3. Hello Dr my ultrasound has just revealed to me that my baby boy won't have the second part of this lower right arm. Do you know any cause of this ? & what are the treatments or surgery to be performed to lengthen or add that missing piece and besides physical therepy and use of prosetic?.

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    Replies
    1. Esabella,
      First, congratulations on your boy. Unfortunately, I need a little more information to provide more information or direct you better. Do you mean the whole forearm and hand are absent? Or is one forearm bone (such as the radius or ulna) absent? Is the hand ok? 4 fingers and a thumb? Obviously, you may not be able to answer these questions but these questions will help me.

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