Friday, October 23, 2015

Radial Deficiency, Doing Fine

As a surgeon, I have a bias that surgery can help most of the time.  And I feel this one because I have seen it help in so many children.  Surgery can help function and it can help appearance- both are important.  And as a surgeon, I feel fortunate to be able to offer surgery to kids and families.

However, surgery is not the answer for every child and sometimes the best path is different. Sometimes it is therapy, sometimes watchful waiting, and sometimes support in other ways.  Kids with birth differences of the upper extremity almost universally adapt well, learn to function in their own way, and generally can accomplish the tasks that are necessary to go about their day.  It is my job to know when I can help but also know when to avoid the recommendation for surgery.  

And this can be really, really difficult.  Because I want to do something to help each child.  And families come to me (and surgeons like me everywhere) because, if something can be done, they want to do it for their child.  Some families push harder than others, but most want something done if it at all can help.  But we, as surgeons, need to be realistic and honest in our approach.

Below is a great kid with a great family who came to me for the first time at age 12.  He had been observed elsewhere and moved to St Louis and asked for our opinion and, if appropriate, surgery. Radial longitudinal deficiency comes in many different varieties with differing severity.  This is a relatively severe presentation.  The family of this child was hopeful that surgery could be done to help him function better and easier.  His arms are short and his elbow don't bend.  His fingers work reasonably well, especially the small finger and ring fingers.  But the stiff elbows make many tasks tough including eating without aids.  But, he does reasonably well and HE is not interested in surgery.

Severe radial longitudinal deficiency.  Note the bent wrist position and lack of elbow motion.  But the shoulder work well. The patient's face is blinded for privacy.

Severe radial longitudinal deficiency.  Note the bent wrist position and lack of elbow motion.  

Severe radial longitudinal deficiency.  Note the lack of elbow joint formation.
Severe radial longitudinal deficiency.  Note the lack of normal elbow joint formation.


The temptation for the surgeon is to straighten the wrists.  However, when the elbows don't bend, the angled position of the wrists actually can help.  Straightening the wrists would only take the hands further from the midline and further from the body.  And, unfortunately, restoring elbow motion is not really possible at this age and with his elbow development.  Therefore, we agreed with the patient and did not recommend surgery.  Not easy for me to say and not easy for the family to hear.  We will, of course, follow him regularly and if certain issues arise, we may have therapy or adaptive recommendations.  But, at least for now, no surgery.

Charles A. Goldfarb, MD
My Bio at Washington University
congenitalhand@wudosis.wustl.edu

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