Ulnar deficiency, considerations for surgery

I have previously written several times about ulnar deficiency.  I would like to provide some additional thoughts on function, limitations, and possible ways that a surgeon can help.  First, I want to state again that most kids with ulnar deficiency do not need surgery.  Function is typically good although there can be some notable limitations.

This first child functions well with her ulnar deficiency.  No doubt.  And surgery is not always appropriate.  However, I think it is my job to consider options to help her function better.  I explain some of these below.

Excellent shoulder function in ulnar deficiency

Ulnar deficiency.  Note short arm

Side view of ulnar deficiency

These pictures demonstrate that the shortness of the arm can be an issue.  Obviously, the affected arm moves well but you can imagine that 2- handed activities can be a challenge.  The following x-ray demonstrate that the radius is fused to the humerus and there is a short ulna.

Ulnar deficiency with radiohumerus synostosis.

Very rarely, if there is a well developed proximal ulna (in this case the ulna is really small), the surgeon can try to separate the humerus and the radius to allow some (although limited) elbow motion.  This has been an option only a few times despite many cases at our hospital.  If this is an option, it is done at a young age.  Another option is to lengthen the radius (or humerus) to make the arm longer.  This is not a procedure I will offer commonly in these children but it can be considered.

Ulnar deficiency hand to mouth

Ulnar deficiency resting position

Ulnar deficiency, hand on side

Other thoughts.  First, the hand appears to rest on the flank.  More bluntly, the hand/ arm can appear to be on backwards.  However, as the pictures demonstrate, the shoulder works great and the child can put her arm wherever she wants or needs to.  So, we can cut and rotate the forearm bone to make the position of the arm look better, it doesn't usually help function in ulnar deficiency and so we rarely perform this surgery.

The most common surgery in children with ulnar deficiency is hand surgery.  These surgeries typically address angulation, syndactyly- http://congenitalhand.wustl.edu/2011/07/frequently-asked-questions-about.html, or positioning of the digits.   The child pictured above had 3 fingers but only 2 metacarpals (the hand bones on which the fingers rest).  It will be difficult for a surgery to improve her function but rotating one of the digits into more of a thumb position might be helpful and will be considered as she gets older.

This is a different child with a syndactyly of the two digits in ulnar deficiency.  Separation of the digits will make a big functional difference for the child.

Ulnar deficiency and 2 fingered hand with syndactyly

Palm view of syndactyly in ulnar deficiency

After reconstruction.  Note separated fingers and skin donor site at elbow for grafts.

Syndactyly reconstruction in ulnar deficiency

Extra Digits

Polydactyly, or extra digits, is likely the most common type of birth abnormality affecting the hands.  Extra digits come in many different varieties, from complex extra digits affecting the thumb to small nubbins affecting the pinky.  In many cases of extra digits affecting the pinky, a simple surgery or simple suture around the extra digit can address the issue.  However, sometimes a more significant treatment is necessary.  Here are the hands and feet of a single patient with unusual presentations of extra digits.  I believe this patient serves to highlight the differences in presentation.

Unusual polydactyly of the small finger.

X-rays of unusual polydactyly

 This polydactyly did arise from the joint with an extra articulation for the extra digit.  The joint was stabilized at the time of surgery.

A slightly more typical polydactyly

X- ray of more typical polydactyly.

This polydactyly on the left is more commonly seen than the polydactyly on the right hand and is more straightforward to treat.

Toe polydactyly

Toe polydactyly. Split metacarpal.

The other side with another variation of toe polydactyly.

X-ray demonstrating the y-shaped metacarpal.

These toe polydactylies are more difficult to treat than the polydactylies affecting the fingers.  The width of the foot can cause issues with shoe wear.  Both feet required a bony correction and stabilization.

Toe Syndactyly

Syndactyly, or abnormal joining of the digits, is most commonly considered to involve the hands.  This is most likely for two reasons: hands matter more than feet to appearance and hands with syndactyly are more affected functionally than are feet with syndactyly.  In most cases, syndactyly of the toes affects the 2nd and 3rd toes, and often is an incomplete syndactyly.  However, occasionally a more significant syndactyly can exist in the feet.

Wikipedia, http://en.wikipedia.org/wiki/Webbed_toes notes several celebrities including Dan Aykroyd and Ashton Kutcher have toe syndactyly.

While I consider surgery for most cases of syndactyly of the hands, I feel that most cases of syndactyly of the feet can be treated without surgery. Again, the reason for this philosophy is that toe syndactyly is less problematic for appearance and function.   Additionally, I have a greater concern for complications such as infection after toe syndactyly correction.

Nonetheless, in certain situations, toe syndactyly may be considered for surgical correction.  For me, the most common reason to consider surgery is syndactyly affecting the great toe and second toe.  This may affect gait and shoe wear.   There are several medical publications on the topic of toe syndactyly including a recent technique description with excellent outcomes: http://www.ncbi.nlm.nih.gov/pubmed/20708986

Here I present a recent case of toe syndactyly with excellent early outcome.

Toe syndactyly affected great toe and second toe.  The gap between the 2nd and 3rd toes also was a problem.

Another view of toe syndactyly

Surgical correction of toe syndactyly.  The skin graft was harvested from the gap (which was narrowed).

Final view of toe syndactyly corrected.