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


Monday, April 4, 2016

3D Printed Prosthetic Design

The 3D printed prosthetic( 3DPP) has captured the attention and the imagination of both the general public and the parents of kids with birth and acquired limb loss.  In my opinion, the public envisions the 3D printed prosthetic (3DPP) as a futuristic solution which will eventually become completely life- like.  The parent sees it as a practical, lower cost solution but shares the general hope for the potential of this new technology.  I have shared my thoughts on several occasions: My 3D Posts

A simple Google search generates 500,000+ hits for 3D printed prosthetic (3DPP) but interestingly a search of the medical literature demonstrates less than 5 manuscripts.  So while there is a huge interest in 3D printed prosthetic (3DPP) options, those in the medical field have not necessarily led the way in this revolution.  I hope that this can change and those in medicine can help to refine and improve the 3D printed prosthetic (3DPP) options.

 There are many factors which play into the popularity of the concept of the 3D printed prosthetic (3DPP)- I will share my perceptions.

1) Prior to now, a prosthetic was something that could only be created by a trained prosthetist.  It was labor- intensive and time consuming.  It could be quite expensive.  The 3D printed prosthetic (3DPP) can be created by almost anyone with a knowledge of computers and 3D printers.  It is inexpensive.

2) One of the major limitations up to now was the fact that the growing child required many prostheses through growth.  This required many trips to the doctor and the prosthetist.  It also makes the expensive prosthetic even more expensive as many are required through growth.  This has, therefore, limited the types of prosthetics available to kids.  No one would create a $50,000 dollar prosthetic for a child who would outgrow it in 2 years.  The 3D printed prosthetic (3DPP) changes that paradigm as printing is fast and easy and cheap.

3) Until recently, the goal of a prosthetic was one of two things.  It could be lifelike and as normal appearing as possible OR it could be functional.  But in a low cost device, both together are simply not possible.  Parents and patients would make a choice for one or the other but could not obtain the best of both worlds- both function and appearance.  In adults, only the very expensive myoelectric prosthetic can satisfy both goals.

The 3D printed prosthetic (3DPP) revolution has brought a very different point of view on appearance.  Since the earliest prothetic was printed, these prosthetics have looked different and do not attempt to look like a 'normal' hand.  The very different and colorful appearance has become part of the appeal for kids- the prosthetics give a distinctive look.  Kids are making the prosthetic a part of their identity- a part of who the whole child is.  A recent article summarizes this changing concept: NPR story.  To me, this is a revolutionary change and critically important in supporting the 3D printed prosthetic (3DPP) effort.

I am very excited about the future of prosthetics for kids.  We at the Shriners Hospital in St Louis together with Washington University are working on 3D printed prosthetics (3DPP) and providing myoelectic options.  The other organization making great progress and offering hope to many kids is http://enablingthefuture.org/.





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


Tuesday, February 23, 2016

Surgery for Olliers Disease

Multiple enchondromatosis is also known as Olliers Disease.  In this disease, at least 2 cartilage tumors known as enchondromas grow in different bones.  Initially, we tend to watch the growths, but sometimes surgery is necessary.  I have previously blogged about Olliers as part of a post on MACRODACTYLY.  There are number of sites that provide good, basic information on Olliers Disease including WikipediaNORD, and OMIM.  The NORD site is perhaps the best.

The enchondromas in Olliers Disease are typically benign, meaning that they don't spread and are not malignant.  There is a risk, however, of transformation to "bad" cartilage tumors such as chondrosarcoma.  This is the reason that we keep a close eye on these tumors.  A related disease, Maffucci Syndrome, also has enchondromas but includes blood vessel growths called hemangiomas.  This can have a higher rate of malignancy.

The indications for surgery include pain, deformity (such as angling of the bone), or a break in the bone.  The reason problems develop is that the cartilage tumors can expand the bone and make it weak- this weakness can lead to a fracture.  In addition, the growing tumor can affect the growth plate leading to angulation of the bone.  It is interesting that adults can also develop these tumors but in these situation in adults, only 1 enchondroma appears.  

It takes several years at least for evidence of the enchondromas to appear in the child.  Sometimes the first sign is a broken bone through the weak area and sometimes the first sign is angulation of the bone.  If we believe that a child has Olliers, we perform a skeletal survey- that is we xray multiple different body areas.  Typically Olliers only affects one side of the body.

Here is one child with pain and deformity related to the enchondroma growths.  There are 3 growths in this child, 1 in the thumb and 2 in the index finger.
Olliers Disease.  Note the swelling of the index finger.  That is the expanded bone.


Another view of Olliers Disease in the hand.

Olliers Disease.  Note the disease in 2 bones of the index finger and 1 bone of the thumb.

In this child, due to pain and enlargement, some deformity, and decreased motion, we performed surgery to remove the cartilage tumor.  We filled the "holes" with bone graft.  This should heal over time.
Enchondroma as removed from the index finger.  This is soft cartilage growth.

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


Monday, February 15, 2016

Camptodactyly- bent finger- treatment with splinting

Camptodactyly is the Greek word for bent finger- specifically flexed so that straightening the finger at the middle joint is not possible.  I have blogged about camptodactyly several times before- read those posts HERE and HERE. 

Therapy is the first line treatment for camptodactyly.  And it usually works.  But therapy has its limits and if the finger is too far bent, therapy can not obtain the leverage for splinting.  Typically, fingers bent less than 60 degrees are treated with splinting at least to start.

Here are a few pictures of a 13 yo child with camptodactyly presenting to me for the first time.

Camptodactyly.  The ring finger on both hands is most effected.

Camptodactyly.  The ring finger is most effected.

Camptodactyly demonstrating an ability to make a fist. This ability is preserved.


There are different splinting techniques but I wanted to share our hand- based splint for camptodactyly of the central digits.  The fingers are held in a bent position (through the MP joint) which relaxes tight structures in the fingers such as the tendons.  The tan straps then put pressure on the tight PIP joints as a straightening force.

Camptodactyly hand based splint.  

Camptodactyly hand based splint.  

Camptodactyly hand based splint.  

Camptodactyly hand based splint.  


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