Sunday, June 26, 2016

Thumb Deformity

The thumb is vital for high level hand function.  The concept of the opposable thumb- the thumb that can meet the fingers for pinch and other high level function- has been said to separate man from at least some of our primate 'relatives'.  In the growing child, the thumb is key for exploring the world and, most notably at school age, for fine motor manipulation with pens, pencils, scissors, and other tools.

As a surgeon, our job is to assess thumb function and thumb alignment and, when necessary, offer intervention to assure each child can obtain maximal function.  For some children, therapy can help with strength and help the child and family understand how to best function.  For others, surgery can provide better alignment, rotation, or stability in an effort to allow the thumb ideal interaction with the fingers and with the world.  Thumb stability is key to function but a lack of stability is not something that kids complain about (or know how to complain about).  But pinch is affected and the surgeon should look for this issue as it is correctable with good results.

Thumb stability is most commonly compromised in two situations.  First, the underdeveloped or hypoplastic thumb.  I have previously blogged about the hypoplastic thumb HERE, among other blog posts.  A typical hypoplastic thumb is small, has poor muscle and has an unstable joint.  Surgery can address the instability and the lack of muscle support.
Hypoplastic thumb.  Not the rotated position of the smaller thumb.

And second, thumb polydactyly, or extra thumb, can be associated with instability.  The most common scenario of instability in thumb polydactyly is after removal of the extra thumb.  That is, the patient has surgery to remove the extra thumb and a lack of stability or a zig-zag deformity becomes a problem several years later.

Triphalangeal thumb, or a thumb with three bones rather than two, is often associated with polydactyly (extra thumb).  Early surgery may be offered to remove the extra thumb and often the third bone in the primary thumb is initially watched to confirm whether or not that bone will cause problems.  I have previously blogged about the problematic triphalangeal thumb HERE.

This is another example.  This child had an extra thumb removed and there is deformity of the remaining thumb which is affecting function including pinch.  There is a lack of pinch strength related to both a lack of stability and the deformity.  In addition, the fact that the thumb has an extra joint (an extra area to bend) can change the way the thumb works.  Adults have shared that this extra joint is not a good thing for function.
Triphalangeal thumb.  The thumb is small and crooked compared to the normal thumb.

Another view of the deformity of the triphalangeal thumb.

Palm view of the triphalangeal thumb with visible scar from previous extra thumb excision.

Triphalangeal thumb with small middle bone (middle phalanx)

Triphalangeal thumb with small middle bone (middle phalanx).  The angulation of the thumb is clear.
Thumb stability is key to function.  This child will benefit from stability and correction of deformity.


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

Thursday, June 16, 2016

Bent Finger: Surgery for Camptodactyly

I have blogged several times on camptodactyly as it is a common finger anomaly and I have received regular requests for information on the topic.  Those posts can be found HERE and HERE and HERE and HERE.    The popularity of the topic camptodactyly can be seen with the large number of comments and questions on the last link.

The medical literature tends to assess outcomes and causes of camptodactyly- neither of which is easy to do.  There are many reported causes including unusual tendons, tight tendons and an imbalance between tendons that bend (too strong) and straighten the finger.  There is also much information about associated conditions and syndromes (arthrogryposis, for example).  A Google search on camptodactyly returns 86, 500 results with many of the first page results from hospitals, Wikipedia, and other information sites.

My general philosophy in treating camptodactyly is therapy first which includes splinting and stretching.  Most kids will respond to therapy and, even if not perfect, the finger becomes fully functional and surgery is avoided.  Some kids, unfortunately either fail therapy (therapy is attempted but not successful) or can never really try it because the deformity is too severe to allow effective splinting.  Those kids may benefit from surgery although families should know that surgery is unlikely to provide a fully mobile finger.

I wanted to share early outcomes of one child with camptodactyly.  He had an isolated ring finger flexion deformity without associated syndrome or other anomalies.
Camptodactyly, bent finger deformity, resting posture.

Camptodactyly, bent finger deformity, resting posture.

Camptodactyly, bent finger deformity, resting posture.  Attempted extension demonstrates a nearly 90 degree contracture.

This child was sent to therapy but ultimately, the degree of deformity contributed to a failure of splinting and stretching.  Surgical release of the tight structures in this camptodactyly was performed with straightening of the finger.  In addition, once such fingers are released, there is often a skin deficit.  We have been using a rotation flap to cover this deficit and avoid bigger flaps or skin grafts.

Camptodactyly 1 week after surgery.  The pin is still in place (removed that day).  Note the triangular flap which was rotated to cover the deficit.

Camptodactyly 5 weeks after surgery.  Note how the patient can straighten the finger and the flap is healed nicely.

Camptodactyly 5 weeks after surgery.  Flexion of the finger continues to improve.

This patient is doing well and both bending and straightening the finger should improve with time and therapy.  The lack of full flexion (bending) as demonstrated above does demonstrate a key point in the treatment of kids with camptodactyly- we need to be careful to avoid loosing finger flexion and work on flexion and extension (straightening) after surgery.

Camptodactyly is a common birth difference of the upper extremity which thankfully often responds to splinting and surgery can be avoided.  While surgery is never a perfect solution, it can be very helpful in the right children.

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









Monday, June 6, 2016

Clasped Thumb.

Clasped thumb is an uncommon diagnosis in which the thumb is held in the palm.  While there are several potential reasons for this positioning, in many cases it is simply due to a delay in muscle development of the thumb extensors.  Over time, those muscles (EPB and EPL) can (but may not) strengthen sufficiently to allow normal function.  Our job during the interval between diagnosis and recovery is to keep the thumb flexible so that if the muscles do develop, the thumb can straighten.  This mainly entails therapy to straighten the thumb to stretch it and sometimes we use splints to do the same.  Occasionally, a soft Benik Splint can help place the thumb in a better position to allow function.

Often clasped thumb affects both sides but the recovery may not be equal.  In the patient below, the right side was worse from early childhood.

Clasped thumb with the fingers straightened.

Over time, in this patient, the left side recovery but the right side did not.
Continued clasped thumb in a 2 year old.

This video demonstrates that the left thumb has recovered nicely but the right still has notable limitations.  We will continue to discuss surgery while giving this clasped thumb a bit longer to recover on its own.


I have previously blogged about congenital clasped thumb- see HERE.  I hope this video sheds light on the diagnosis.  

A couple of further thoughts on congenital clasped thumb.
1)  Surgery can be helpful.  If the patient fails to gain the strength to straighten the thumb, surgery can improve function.  Typically we rearrange tendons to add another muscle unit to help the straightening process.  We do want to allow sufficient time for the thumb to gain strength and recover on its own, but this does not always occur.
2) Generally the diagnosis of clasped thumb is clear but not always.  Other diagnoses must be considered including an association with syndromes or even things like cerebral palsy (CP).  CP can have a thumb in palm deformity but it is related to tight muscles (spastic muscles) rather than weak extensor muscles.

Clasped thumb is a diagnosis that can have a very favorable outcome with or without surgery.  




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

Saturday, May 28, 2016

Finger Flexion Deformity

I have previously posted several times on camptodactyly, a condition with a stiff, bent finger.  Those posts can be viewed HERE.  Camptodactyly is relatively common compared to other birth differences of the hand and upper extremity as it can be seen in isolation (i.e., not associated with any other conditions) or in association with cleft hand, ulnar deficiency, arthrogryposis, or other syndromes.  It can involve one finger, or several.

Thankfully, many kids with camptodactyly do well with stretching and without surgery as the bent finger position does not affect function.  Surgery is reserved for kids with significant bend of the finger that has failed therapy with splinting and interferes with function.

One reader posted about her young child with a flexible camptodactyly.  That is, a bent posture of the PIP joint that can be passively straightened (i.e., by mom) but is not able to be straightened actively by the child.  In my experience, this is far less common than the more typical, stiff or fixed, camptodactyly.  Here are a few photos of a 13 month old child (similar in age to the child mentioned above).
Flexed position of the PIP joint of the patient's right index finger.  This has the appearance of camptodactyly.

In this picture, I am demonstrating that the finger can be fully straightened at the PIP joint- thus not a typical camptodactyly.

Live action shot (sorry, a bit blurry) demonstrating that the child cannot straighten the finger on his own.

This is similar to a clasped thumb, a condition in which the child's thumb is in the palm and the child cannot straighten it.  I have previously blogged about it HERE.  As in clasped thumb, we will give the child above time to develop muscle strength to straighten the thumb.  In clasped thumb, the strength issue is generally thought to be the EPB muscle (forearm based) whereas in the child above, a weakness of the intrinsic muscles of the hand are to blame.  Either way, with time, we hope that the muscles will develop and the straightening power will appear.  Our job, while we wait, is to assure that the finger (or thumb) do not get stiff in the the bent position.

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

Monday, May 23, 2016

Amazing Function in Arthrogryposis

I have blogged many times on arthrogryposis as can be seen HERE.  Our goal as upper extremity (hand) surgeons is to use therapy and surgery to improve function.  The real purpose of our interventions is to allow independent function such as eating, writing, toileting, etc.  There are a series of surgeries which we have found helpful in athrogryposis although each child is considered based on what he/ she is able to accomplish and what he/ she has trouble doing.  For example, we believe the external rotation osteotomy of the humerus can be incredibly helpful to allow the use of both hands together- an incredibly important ability when the hands have limited function.  But every child does certainly not need this surgery.  Another example is providing the ability for the elbows to bend.  This is perhaps the most important intervention we can provide for most kids as it allows the child to bring his/ her hand to the mouth.  This can open a whole new world of independence!

But what happens when there is severely limited hand and upper extremity function?  Severe limitations can challenge the idea that surgery can make a real, day to day difference in abilities. We can better position the arms and hands and fingers but if movement and strength in the hands and arms is so limited, these improvements may not help much, or even not at all.  What then?

Well, the inborn abilities of kids to figure it out helps... a lot.  Kids will figure out how to make the best use of the arms and hands. And we can still help with that process but, as always, in a way guided but what the child is able to accomplish.

And sometimes we have to get out of the child's way.  This is not always easy for us as physicians, for parents, or for teachers.  Below is a great example of an amazing child who has found that his feet are FAR more functional that his hands.  He eats with his feet (using forks and spoons) and performs many of life's activities with his feet.  School can be a challenge in these situations and we work with schools and teachers to make sure they empower the child to learn and interact in the best way possible.  Socially, this is not easy for the parent or the teacher (at least initially, it is not a problem for the child).  Over time, kids may become more self conscious about eating and writing with their feet and look for help in improving hand and upper extremity function.  Again, every child is different and will be 'helped' in different ways.

A few video examples (mom gave permission for us to share):






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


Saturday, May 7, 2016

Meetings

I have been away from this blog for too long.  I have been traveling a bit and want to share some information about the meetings I have attended.  I hope and expect to get back to regular blogging this week!

POSNA Annual Meeting 2016

POSNA is the Pediatric Orthopaedic Society of North America.  This organization supports surgeons and other providers who provide care for children with orthopedic challenges, whether that be issues from birth, trauma, infection, etc.

Their website shares:

The Pediatric Orthopaedic Society of North America (POSNA) is a not-for-profit professional organization of over 1200 surgeons, physicians, and allied health members passionately dedicated to advancing musculoskeletal care for children and adolescents through education, research, quality, safety and value initiatives, advocacy, and global outreach to children in underserved areas.
Our Mission: To improve the care of children with musculoskeletal disorders through education, research, and advocacy.

I am a member of POSNA and find it to be an incredibly collegial and educational organization with great people dedicated to the children for whom they provide care.  The hand and upper extremity offerings (lectures, discussions, networking) are growing as well.  I have participated the last 3 years and this year, along with my co- author Lindley Wall-Stivers, shared our work on outcomes with birth anomalies of the pediatric upper extremity.  

My next stop was the PHSG meeting in Toronto, Canada.  PHSG- Pediatric Hand Study Group- is a group of surgeons and therapists with a special interest in caring for kids with birth differences of the upper extremity.  The group has been around since 1998, became more formalized in 2002, and has grown year by year.  While focused on true birth differences of the upper extremity, the group also has notable expertise in birth brachial plexus palsy and cerebral palsy.  Much of the published literature in all of these areas comes from the members of this group!

Our meeting was hosted by Toronto Sick Kids Hospital, a famous institution providing outstanding care for kids for many years.  What an amazing event with great lectures, discussion, and collegiality. Definitely one of my favorite meetings of the year!
Sick Kids Hospital, site of 2016 PHSG meeting
Dr Wall- Stivers and I shared our work in two areas.  First, we discussed the St Louis experience with the humerus external rotation osteotomy for arthrogryposis.  I have blogged about this previously as can be seen HERE and HERE..  We believe this operation both improves function and appearance for kids.  
And second, we shared our experience with Hyalomatrix for syndactyly reconstruction.  Again, I have written about our experience HERE.  While it is still early in our use of this skin graft substitute, we are excited about its potential.  There was a great deal of interest in the audience about both of these surgical procedures.

Finally, I was privileged to share with the group the winner of the 2016 Manske Award (for a scientific publication in 2015):
Ann E Van Heest, Anita Bagley, Fred Molitor, and Michelle A. James.  Tendon Transfer Surgery in Upper- Extremity Cerebral Palsy Is More Effective Than Botulinum Toxin Injections or Regular, Ongoing Therapy.  J Bone Joint Surgery, 2015; 97: 529- 36.

This manuscript reports the one year outcomes of children upper extremity cerebral palsy treated with tendon transfer surgery, botulinum toxin injection, or continuing therapy.   The surgical group showed significantly greater improvement in the Shriners Hospital Upper Extremity Evaluation (SHUEE), with notably improved wrist extension and supination. 

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




Sunday, April 10, 2016

Finger at Risk- Amniotic Constriction Band

I have previously posted on Amniotic Constriction Band.  I post again on the topic as it is common and sometimes early surgery can be helpful.

This child was born with an abnormality of the hand including a markedly swollen finger.  There are several important findings in these 2 pictures.
Amniotic constriction band with swollen finger.

Amniotic constriction band with swollen finger.
First, there is a well developed thumb and pinky finger.  That means that this child will have excellent function.  Almost all of life's activities can be accomplished with a thumb and one other digit.  This includes writing, typing, grasping large objects, and manipulating small objects.  Clearly, the lack of the central 3 digits will impact dexterity and slow the performance of some tasks.  A child born with this hand will learn how to accomplish tasks and will be more functional, for example, that an adult with a trauma causing the loss of the same digits.

If we look closely at the two pictures, there is a dark cord which can be seen just past the swollen digit.  It is a very small amniotic constriction band.  This band is wrapped around the finger and, when noticed, it can be removed.  These bands are not seen in most patients with amniotic constriction band, but one theory on amniotic constriction band holds that the bands may be the cause of the swelling or even amputation of fingers.  Unfortunately, removing the band does not mean that the finger will survive but it may increase the odds.  Sometimes, there is also an area of scarring which may limit blood flow and release of such an area may also increase the likelihood of survival of the finger.

In this patient, the band was removed and an area of scar tissue released in the hoops of allowing better blood flow.  Unfortunately, fingers with this degree of swelling probably will not survive.  Thankfully, as noted above, this child will have excellent function.

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