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