Tuesday, May 28, 2013

Toe Transfers

Symbrachydactyly may present in different forms although the thumb is typically well formed.  Reconstructive technique are varied and can include free toe transfers as I have previously written
http://congenitalhand.wustl.edu/2013/01/toe-transfers.html .

I recently saw a symbrachydactyly patient back for followup 10 years after initial treatment.  We had, after a lengthy family discussion in 2003, treated the 9 month old patient with a nonvascularized toe phalanx transfer.  There are a few reports in the literature detailing positive outcomes with growth of the transferred bone (although more recent reports are not so positive).  I no longer have great confidence in this procedure because the transferred bone does not seem  grow after transfer to the hand.  Nonetheless, 10 years ago, we elected to perform this procedure to give the child a post to pinch against with the thumb.  Even today, I do believe this procedure should be considered but perhaps at a slightly older age when the bone need not grow significantly to be helpful.

Symbrachydactyly x- rays before surgery.  Note that thumb is well formed but all other digits are short.  We felt that providing a bit of length to the index finger would help with function.

Symbrachydactyly x-rays just after transfer of a nonvascularized toe phalanx.  The length of the index finger looks great.
Symbrachydactyly x- rays 1 year after surgery.  Note that index finger is no longer as long as previously with absorption of the index finger added bone.








10 years after free toe transfer.  Note that the transferred bone is  no longer visible.
Appearance of the hand at 10 year follow- up.

Palm side view of hand 10 years after surgery.



Patient making a fist 10 years after free toe transfer.

Free toe transfer donor site 10 years after surgery.  The patient and family do not really like the appearance of the foot.

Saturday, May 18, 2013

POSNA

POSNA is the Pediatric Orthopaedic Society of North America.  It is a wonderful organization of orthopaedic surgeons who care for children with orthopaedic birth, acquired, and traumatic injuries.  It was founded around 1970 and has grown over the last 40 years.  I recently became a member of POSNA and attended my first meeting in Toronto this month- I thoroughly enjoyed it.  Peters Waters of Boston- recent President of POSNA- has, among others I would think, tried to make POSNA the home of different super subspecialty groups like pediatric hand surgeons and scoliosis surgeons and pediatric foot surgeons.
It is an interesting idea as there are a small number of us across the country who make the pediatric upper extremity our area of specialty.  While we have our own meetings (historically based on a group of Shriners Hospital surgeons but now a wider group), POSNA has emphasized the upper extremity and created a specialty day on upper extremity topics.  This is a new concept especially for someone like me who has traditionally thought of myself as a hand and upper extremity surgeon first (who happens to take care of a lot of kids).  I am a more active member of the ASSH (American Society for Surgery of the Hand); we certainly run courses and lecture on the pediatric upper extremity at ASSH meeting also.

Anyway, I enjoyed the meeting because of the opportunity to meet new people and experience a new format for a large meeting.  I was able to moderate a general session with upper extremity scientific papers and also run a "specialty day" session with Don Bae (Peter Water's partner in Boston at the Childrens' Hospital).  Good presentations on trauma and birth anomalies of the upper extremity.

Cleft Hand Long Term Follow- up

Cleft hand, central deficiency, is an uncommon birth anomaly of the hand.  I have previously posted a few times on this interesting difference including one about surgery http://congenitalhand.wustl.edu/2012/05/cleft-hand-surgery.html
and another about cleft hand in general http://congenitalhand.wustl.edu/2012/01/central-deficiency.html and a few others as well.  I thought a long- term follow- up sequence of pictures might be interesting to share.

We are learning more about all of the birth anomalies of the hand and upper extremity.  There is good evidence to suggest that cleft hand (also called central deficiency) is a hand difference which is part of a spectrum with central syndactyly and synpolodactyly.  We will continue to learn more about the origin of this hand difference and with this knowledge will come different treatment options.  I do not see a lot of changes in our basic treatment plan for many years but I am excited about our continuing progress and understanding.

Currently, it is our goal to make the cleft hand function better and look better.  The thumb- index web space often needs to be expanded and the cleft needs to be narrowed.  Those are the clear goals.  Additionally if there is a syndactyly between the ring and small fingers, that is corrected as well.  Finally, there can be laxity or instability of the index finger MCP joint (see picture below) and sometimes the PIP joint of the ring finger can be limited in motion (not in this patient).

The case below is an excellent example of a severe cleft hand and an outstanding clinical and functional outcome.  As the patient said to me (12 year old), "no one notices my hand."  I must admit that not all cleft hand reconstructions look as good as this hand.  However, if we apply the basic principles of reconstruction, generally good outcomes (both function and appearance) can be achieved.

Pre surgery for cleft hand.  Notice the cleft, missing long finger and abnormal hand appearance.

Another pre surgery view of cleft hand with a direct view of  cleft and finger rotation.

More than 10 years after surgery for cleft hand.  Notice how well the fingers are aligned.
Palm side view of cleft hand after surgery.

In cleft hand the index finger MCP joint (connecting finger to palm) can be lax.  This demonstrates that I can move his finger a great deal but this is not a regular problem for him.
The patient making a fist in cleft hand.  See that the fingers are not perfect but motion is generally excellent.  Interestingly, strength is also very good, almost as good as the other side.

The thumb- index web space is key to outcome in cleft hand. In this case we took skin from the central cleft and rotated it into the area that needed skin providing a great outcome.


Syndactyly reconstruction which looks very good in cleft hand.
X-ray showing good outcome with well- aligned fingers in cleft hand.





Wednesday, May 15, 2013

Radioulnar Synostosis, revisited

Radioulnar synostosis has been previously discussed in this blog  http://congenitalhand.wustl.edu/2012/06/radioulnar-synostosis.html

In that post, I discussed that in most cases, surgery was not necessary because kids typically function very well. However, sometimes that is not the case.  Most kids with radioulnar synostosis are diagnosed around age 7.  Sometimes it is picked up earlier but around age 7, activities increase and kids with synostosis may realize limitations.  A recent patient is a good example of problems that can exist with radioulnar synostosis.  This patient presented to us at age 12.  She had absolutely no pain but both forearms were palm down (fully pronated to use the therapy language).  She was in great position to type but not to play sports, not to hold a tray, and not to get change or hold M&Ms.

                        
             Resting position of radioulnar synostosis for this patient.  She is fully pronation or palm down.



In this picture of radioulnar synostosis, the patient is trying to turn her palms up.   If you look carefully, her forearms are still palm down.
These pictures are great examples of the difficulties that can exist.  The bottom picture shows that patients can use the wrist to change the forearm/ hand position (kids are really limber and this proves it).  However, despite this increased wrist motion, the forearm still doesn't move and function is still limited.

The x-rays are educational as well.
Radioulnar synostosis x-ray.  A single x-ray can be confusing as this one is.  The diagnosis is not clear.

The diagnosis of radioulnar synostosis is now clear.  The radius and ulna are one bone near the elbow.  Additionally, the radial head is dislocated.  In some children the dislocated radial head can cause problems, but not in this child.

The treatment options are simple.  Obvservation versus surgery to reposition the forearm in a better position to allow function.  An osteotomy, or cutting of the bone, is the way to take the forearm from a palm down position and place it in a position between palm up and palm down- neutral rotation (clapping hands position).  Once repositioned, shoulder movement can help compensate and allow the patient to function in all positions.  In 2013, despite many advances in medicine, we do not have a way to restore motion.

Saturday, May 4, 2013

Congenital Clasped Thumb


Congenital clasped thumb describes a condition present at birth (but potentially not recognized until 3-4 months of age or even later) in which the thumb is flexed into the palm.  Congenital clasped thumb may affect one or both thumbs but more commonly affects both.  Most newborns keep the thumb positioned in the palm for the first months of life and, therefore, the diagnosis may not be clear.  With age and an interest in grasping objects in the world, the clasped thumb becomes more apparent.  

The basic problem in congenital clasped thumb is that there is a muscular imbalance: the muscles/ tendons that bend (flex) the thumb are stronger than those that straighten (extend) the thumb.  The flexed position of the thumb is most often related to weakness of the key muscles that extend the thumb- extensor pollicis brevis and/ or the extensor pollicis longus.  Other possibilities include shortening of the flexor tendons (flexor pollicis longus) or weakness of the abductor tendons (in my mind, the least likely cause).  The space between the thumb and the index finger may also be tight (first webspace contracture).  It is unclear if this is a cause of the problem or a secondary effect of the muscle imbalance. 

Patient is attempting to grab pen with thumb. Note flexed thumb in congenital clasped thumb (difficult  to see).

Another view of congenital clasped thumb- note flexed position of thumb.


McCarroll and Manske in this article classified these congenital clasped thumbs as either supple or fixed (complex).   The citation was primarily in reference to the treatment of Freeman Sheldon Syndrome (windswept hand). Tsuyuguchi in this article included 3 types, supple, fixed, and associated with arthrogryposis.   Multiple other diagnoses can be considered but 2 are most common: locked trigger thumb and cerebral palsy (spasticity).  Both of these diagnoses are usually clear. 
  
Treatment for most children is passive stretching of the thumb: bringing it out of the palm and holding it in an extended position.  Splinting in a position of extension can also be helpful.  Hard splints are one option but can be uncomfortable and therefore a challenge in the young child.  Benik splints, soft neoprene splints, are an excellent choice as they provide some stretch while being comfortable even for the young child with clasped thumb.

If the thumb is not better by age 3, surgery is considered to provide better balance to the thumb.  Sometimes we widen the thumb- index web space with a skin rearrangement (z- plasty) or, in situations in which the web is very tight, a flap of skin may be moved into the web.  However, the key concept is to release or lengthen the tight muscles on the palm side of the thumb and strengthening the extensor tendons, often with a tendon transfer.  Rarely, the thumb is so flexed that it is fused (stiffened) in a straighter position.  Clearly, the type and severity of congenital clasped thumb affects the treatment but patients do well with surgery.  More importantly, most patients can avoid surgery with appropriate therapy to include stretching and splinting.