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.

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.

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 http://www.ncbi.nlm.nih.gov/pubmed/1572919 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).  Tjuyuguchi http://www.ncbi.nlm.nih.gov/pubmed/4045133 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.

Pseudarthrosis of the Forearm

Pseudoarthrosis or, more commonly, pseudarthrosis literally translates to “false joint.”  The term Pseudarthrosis is used commonly in the situation of a fracture nonunion.  For example, if the shinbone, the tibia, does not heal after a fracture, a nonunion develops.  Eventually the nonunion will develop into a pseudarthrosis- related to motion between the bone ends.  There is fluid and a joint like appearance to this space.

While this can happen in children, more often (but still really rare) some children are born with a pseudarthrosis, a congenital anomaly.  We don’t know the reasons for these situations but these pseudoarthroses can occur in in specific locations such as the tibia.  The tibia is indeed the most common location for pseudarthrosis and about 50% of the time these patients have neurofibromatosis (NF)type I.  It can also occur in the clavicle although these cases are not usually related to NF- see my previous blog on this topic:   http://congenitalhand.wustl.edu/2012/11/clavicle-pseudoarthrosis.html

The forearm is a common site of Pseudarthrosis in NF.  This can involve the radius or, more commonly, the ulna bone.  Anatomically, the forearm and the lower leg both have two bones (radius and ulna in the forearm and tibia and fibula in leg).  The difficulty with a pseudoarthrosis in either location is the differential growth of the 2 bones.  When one bone doesn’t grow as the other grows, deformity develops and may be accompanied by dislocation (typically of the radial head).  We see similar issues in the condition of osteochondromatosis- also directly related to the differential growth.  Pain can be an issue but typically is not. 

Ulna pseudarthrosis.  A good portion of the ulna is missing.  See the forearm curve.

Ulna pseudarthrosis from side view.

Above is the ulna pseudoarthrosis and below is the radius pseudarthrosis.

Radius pseudarthrosis.  Not the curvature of the forearm.  The radius is very short compared to the ulna.

Another view of radius pseudarthrosis.

Treatment is appropriate to create a unified bone that will balance the forearm and prevent progressive deformity and angulation.  There are a variety of different approaches to achieve healing and improved alignment:

      1)  Cleaning out the nonunion site, filling the site with bone graft (typically from hip) and often bone morphogenic protein (BMP- an off the shelf bone stimulant).

2    2)  Free fibula vascularized bone transfer.  In this procedure, a wider area of the Pseudarthrosis site is removed and normal bone from the fibula is substituted into the void.  The beauty of this procedure is that the fibula bone is unaffected and can be brought to the forearm with a blood vessel to maximize healing.  The negative of this approach is related to problem of taking this bone from the leg.  While fortunately these complications are rare, they do include issues with muscle (FHL) function (affects toes), temporary sensory issues, pain, and ankle deformity.

·         3) External fixator deformity correction and bone grafting (often with BMP).  

Family Challenges

I just read a very interesting article in Money Magazine, May 2013 issue.   It is called "Paying for Finn" and is written by Jeff Howe.  The article details the challenges in caring for a special needs child.  A child such as Finn on the autism spectrum brings different challenges than most children with birth anomalies of the upper extremity but the article is interesting nonetheless. It got me thinking...

Some of the children I see- often those children with a syndrome or chromosomal difference- do have larger cognitive issues which affect their interaction with the world.  These problems are different than a child with autism but still can be a challenge.  The majority of the children do not have such issues.

Most of the children that I see and treat with upper extremity birth anomalies are behaviorally just like every other kid their age.  A 2- year old tantrum is the same in a child with radial deficiency as it is in a child without it.  At certain ages, however, upper extremity differences will be a challenge.  I am no psychologist (and am lucky to work with some very good ones) but I have learned of two periods of challenge for the child and family- kindergarden and 7th grade (I learned some of this at Texas Scottish Rite Hospital also). These are times of a growing self- awareness and a time when children are working to  understand who they are and where they fit in their social world.  In kindergarden, kids ask a lot of questions.  As long as the child with the difference (whatever that might be) has a response to questions from other kids, all will usually be well. Some kids say something like "God made me different" and some say "an alligator bit my hand off."  It really doesn't seem to matter what one says, just that the child has a response to the questions.

As a parent and a caregiver, I have learned that every child brings unique challenges and every person has "something".  The Something is some challenge, some difficulty which may or may not be apparent.  In kids with upper extremity birth differences, the difference is physical and is visible.  It may or may not slow the child down, it may or may not allow him to play basketball or play the piano or whatever.  This difference will affect the way the child interacts with the world mainly because it affects the way the world interacts with him or her.

The other time frame which is a challenge is the 7th grade.  Again, kids are figuring out their place in the world and comparing themselves with others.  There are no easy answers to questions from others at this point and the more comfortable and confident the child with the difference is, the more successful they will be in navigating this difficult time.  Support at home is clearly important in gaining the skills to get through the questions at this age.

I hope that this post generates some thoughts from others who may well have different opinions and different feelings.  I am sure I will learn from those thoughts...

Small thumbs

Small thumbs are a part of radial longitudinal deficiency- the official name is hypoplastic thumb meaning underdeveloped thumb.  Sometimes this is an isolated problem and sometimes it is associated with abnormalities of the forearm and wrist (radial longitudinal deficiency)- http://congenitalhand.wustl.edu/2011/07/radial-longitudinal-deficiency.html

A small thumb can be on one hand or both but most of the time the hands are not exactly the same (meaning one is worse than the other).  There are 4 main parts to the small thumb: size, stability, and muscle, and motion.
1) As the name implies, the small thumb is small.  It is short and can be thin.
2) It is also unstable at the MCP joint which is the joint (where motion happens) between the thumb and the hand.  The problem with instability at this location is that it affects how you use your hand.  An unstable thumb usually prevents good, strong pinch.
3) The muscles of the thumb are called the thenar eminence.  These muscles are used to position the thumb to allow best function- things like holding a soda can or pinching.  If these muscles are small or absent then the thumb-= the most important of the fingers- can't work correctly.  If the thumb is not working correctly, the patient will have less use of the entire hand.
4) Lastly, a small thumb may have limited motion.  This can be due to the limited muscles or it can be due to abnormal tendons from the forearm- tendons that can have abnormal connections.  The thumb may not bend or straighten well.

The good news about the small thumb is that surgery can be helpful to correct it.  Surgery can make it stronger and more stable and more mobile.  Surgery can never make the thumb normal but it can make a better thumb.  If a thumb is really small, sometimes the thumb may be best treated with pollicization, rather than reconstruction.  http://congenitalhand.wustl.edu/2011/10/pollicization-creating-new-thumb.html

If the thumb is reconstructed without pollicization, there are 3 basic steps.  1) Give the thumb more muscle. There are choices for this step but both work well (flexor tendon to ring finger or muscle from pinky side of hand).  2) Make the MCP joint stable  3) Deepen the space between the thumb and index finger, the first web space.  This makes the thumb longer and able to grab larger objects.

These pictures are unusual in that they are of an 11 year old with small thumb or hypoplastic thumbs on both sides.

Small thumbs on both sides.  The left hand (the one on the right side of the picture) is worse- smaller and with less muscle.

This picture is of the child with small thumbs trying to bring the thumbs up.  Neither side does it well.

Another view of the small thumbs trying to lift the thumbs.

Yet another view of small thumbs, attempting to lift.

X-ray of small thumb (the left side) showing a small thumb with unstable joint.

Great x-ray of small thumb, unstable.  Notice the bend in the thumb.

Last picture of small thumbs.  Notice the the left thumb (on the right) is smaller or more angulated than the other one.