Wednesday, August 19, 2015

3D Progress

I have posted several times previously on our work with 3D Printed Prosthetics, the posts are compiled here.  Sydney is one of our patients who has been a big part of this process.  She recently received the latest prosthetic version, a 3D Printed, Myoelectric device and fabricated for about $110. Today, there was a great deal of press on Sydney and her new 'arm'.  Here are a few of the links:

KSDK link

CBS KMOV link

Fox2 Link


In addition, Sydney and I talked just after she got the new prosthetic.  She wasn't yet a pro at using the hand but still lots of interesting things to say.  Check it out


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

Tuesday, August 18, 2015

More on Amniotic Constriction Band

I recently posted on a young child with Amniotic Constriction Band Here.  I wanted to update that post with additional information.  Prior to coming to me, the patient had been treated in an urgent way by Dr Erin Greer who performed a z- plasty and release of the tight band.  This surgery was done to help the thumb survive as it looked concerning due to the tight band.  Needless to say, this surgery was successful and the thumb looks really good and clearly will survive.

Here are new pictures (not previously posted) just after birth with a thumb with Amniotic Constriction Band that is at risk.  See the tight band and the swollen thumb past the band.

Amniotic Constriction Band with tight band.

Amniotic Constriction Band with tight band.

Amniotic Constriction Band with tight band.

Here are some pictures soon after the procedure- notice how improved the thumb looks already.
Amniotic Constriction Band after band release

Amniotic Constriction Band after band release
Finally, here is one picture when the patient came to see me.  Look how much improved the thumb is with the additional time.  There is still evidence of the band but this is a thumb that will do well long term and really help with function!

Amniotic Constriction Band with tight band after release.

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

Sunday, August 16, 2015

Amniotic Constriction Band and the Hand

Amniotic Constriction Band is a common problem in our clinics.  Kids can have a diagnosis of amniotic constriction band and yet their hands can all look very different from one another as there are simply so many different presentations.  I have previously written about this topic here but I wanted to share a few more thoughts.

We typically think of 3 things when we make a diagnosis of amniotic constriction band.  Kids may have 1, 2, or 3 of these issues- that is what makes the diagnosis so different from one child to the next.  First, they can have constriction rings or indentations in the skin.  These can be mild or can go really deep.  If deep enough, the blood supply to the finger (or for that matter the whole arm if the ring is higher up in the arm) can be affected and can theoretically not survive.  These rings can be in the arms, hands, or legs.  

The next issue is amputation, or loss of an arm or more commonly a finger or part of a finger.  We think this is related to a deep, tight ring while the baby is still in the womb.  Rarely, a newborn can have a tight ring and we worry that the finger might not live unless we do surgery to save it (and that is what happened in this child's thumb- early surgery may have saved the thumb).

The final issue is amniotic constriction band is joining of the fingers together- the so- called syndactyly.  This is different from other types of syndactyly as we believe it is related to scarring after an amputation of a finger.  It can be two fingers or the whole hand.  This can be difficult to treat but early treatment can lead to really satisfying results and a great hand.

Here is a case for consideration showing all 3 types of problems in amniotic constriction band.

First is the left hand.  Note the constriction in the index finger.  This can be addressed with surgery which will decrease the band and allow more normal growth. The second picture has a side view showing another view of the band.  The rest of this hand looks pretty good.  The middle finger (or long finger) has been amputated and is about the length of the index finger.  Amputation of the long finger is most common.

Amniotic constriction band with a tight band of the index finger.

Amniotic constriction band with a tight band of the index finger, a side view.

The right hand is more complicated in this case of amniotic constriction band.  The thumb had urgent surgery after birth for a tight band and looks very good now.  There is also a reasonably well formed pinky.  The ring finger has a tight band that will be released.  But the other digits are joined together from scarring and are short due to amputations.  Early surgery may help create better, more functional fingers and hand.
Amniotic constriction band with a thumb band previously released.

Amniotic constriction band with a tight ring finger band, amputations of other digits.
Amniotic constriction band with several amputations.

Amniotic constriction band with a tight band of the ring finger.

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


Hand Surgery in Children

The timing and appropriateness of surgery of the hand and arm in children with  birth anomalies are difficult topics.  The problem for parents and doctors is that we don't truly know how well any particular child will adopt to his limitations and how much those limitations will affect his/ her life.  We all hope for kids with excellent function and 'normal' appearance and, therefore, families seek doctors and surgery to make their kid's hands 'normal'.  As a surgeon with experience, I see it as my job to share reasonable expectations about surgery (as well as risks and recovery).

As kids get older, they can participate in decision- making.  But the reality is that families often want 7 year old or 10 year old kids to really make the touch decisions about whether to have surgery and that is not likely to be helpful.  We want such kids to have a stake in the decision but they are not usually able to truly really make such difficult decisions.  Understanding the future is tough and having reasonable expectations about surgery (pain, recovery, etc) is really challenging.  In the case below of a 17 year old, his feelings and his life experience with his condition are vital to an appropriate decision for surgery (in this case family was interested and he was absolutely not).

These are issues that we face every day in clinic and every family approaches such decisions differently.  It is my job to help each family, in its different way, make the best decision.  Not easy (and sometimes talking through this is more challenging than the surgery itself).

Distal arthrogryposis is, in my practice, less common that amyoplasia or the more extensive disease. Because the disease is focused on the hands alone, patients tend to function at a higher level and have an easier time with activities in general.  I have posted twice on this topic, here and here.  Surgery is often considered as it can improve hand position and sometimes finger and thumb motion.  But such decisions are not easy for most families.

I recently met a 17 year old male who reminded me that function can be very good even with some deformity and some limitations in motion.  He was absolutely not interested in surgery as he felt he could perform all activities without too much difficulty.  He did admit to using two hands for activities that others would accomplish with only one hand.  He also stated that some tasks might take him a bit longer to complete compared to other kids.  But he was not interested in surgery.

Here are some pictures of his hand (both sides looked the same) to frame the discussion.  The fingers do not straighten fully, especially the ring and small fingers with camptodactyly.  The fingers do however make a great fist (which is not always the case in other types of arthrogryposis) and with some strength.  Finally, the thumb web space is only marginal, not great, which limits his ability to grab larger things.
Distal arthrogryposis with attempted finger straightening

Distal arthrogryposis with attempted finger straightening from palm view

Distal arthrogryposis with a full fist demonstrated

Obviously, we did not perform surgery on this happy 17 year old with imperfect hands.  His parents were somewhat interested in learning more about the options which included therapy to help straighten the fingers (vs surgery to release the tightness on the palm side) and possibly surgery to help the thumb web space.  But could do everything he wanted to do and was okay with accomplishing certain goals differently than his peers.


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

Wednesday, August 5, 2015

Thumb position and the 3 digit hand

I have posted on thumb rotation and if you search the blog, you will find a number of posts on ulnar longitudinal deficiency.  Scrolling through the pictures, a pattern begins to develop in these kids with ulnar longitudinal deficiency- that is, many of the kids have a well developed thumb and 2 very good fingers.

When we think about ulnar longitudinal deficiency, we think mainly about the forearm.  And we should as deficiency of the ulna with its associated tendon and muscle limitations are important.  But from a functional standpoint, as Dr Manske taught us so many years ago with this classic article, the hand and thumb specifically are very important.  Dr Manske focused on the thumb web but, as we later demonstrated in this article, the rotation of the thumb is important also.  Because if the thumb is in the plane of the fingers, some function of the thumb is lost and pinch is neither strong nor precise.

This child with ulnar longitudinal deficiency has a thumb and 2 fingers.  All are well developed.  But the position of the thumb is not great for function.  The family is considering a rotation of the thumb to allow improved function.

Ulnar longitudinal deficiency with a thumb and 2 fingers.  Note the position of the thumb.

Ulnar longitudinal deficiency with limited pinch.  No that the child is pinching with the side of the the thumb, not the 'meaty' pulp which gives the best strength and function
I posted these pictures and this brief discussion simply because I though the pictures were so powerful.  This is a challenging situation because the child functions well.  However, I believe that he can function better with more precise thumb function with surgery.

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

Friday, July 31, 2015

Outcome after Surgery for an Extra Thumb

The condition of a child born with two thumbs has many, many names but most commonly, we call it radial polydactyly, thumb duplication, or split thumb.  While all are utilized, radial polydactyly is the medical term and split thumb is appropriate as both thumbs are smaller than the other thumb.  I have posted about radial polydactyly previously as can be seen HERE.

We have learned over the years that surgical reconstruction of the extra thumb is much more than removing one of the thumbs.  The remaining thumb requires stabilization and often straightening. This can be tricky and, I believe, is best accomplished by a surgeon with experience in treating these problems- really the more experience, the better.  Even then, sometimes a second surgery may be required as the child ages.  This has been discussed over the years but a nice long term follow- up study from Dallas, as detailed HERE, reports on 43 kids at an average follow- up of 17 years.  These late surgeries were for different reasons but a crooked thumb was often the issue.

The surgical goals for radial polydactyly are to
1) Remove the extra thumb
2) Maintain the 'better' thumb.  Typically that is the one closer to the hand but not always
3) Stabilize the thumb (i.e., make sure the ligaments are good)
4) Align the thumb (i.e., make sure the bones are straight)

We typically add a temporary metal pin to protect the surgery during the first 5 weeks when the child is casted.  Cast and pin are removed at the first visit after surgery.

Here is an example of the early outcome after reconstructive surgery for radial polydactyly.  This child was not as complex as some but still a challenge.  One thumb was clearly larger and it was also crooked, requiring straightening of the bone at the time of surgery.  Both the family and I are pleased at this point but we will follow the child over time to assure that the thumb stays straight and functional.

Radial polydactyly before surgery.  Note that the large thumb is somewhat crooked.  

Here is the side view of the thumb after surgery for radial polydactyly.  Not the subtle scar.

Top view of the thumb after radial polydactyly reconstruction.  The thumb is straight.

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

Sunday, July 19, 2015

Multiple Hereditary Exostosis- Its Not Always about the X-rays

Multiple hereditary exostosis is an uncommon tumorous condition and is capable of causing significant functional limitations.  I have previously posted on MHE.  While multiple hereditary exostosis in the fingers can cause deformity and limitations of finger flexion (I need to post on this topic), we worry more about the forearm.  The problem is that if the two bones of the forearm don't grow at the same rate, the natural rotation of the forearm may be lost.

However, when the bones don't grow at the same rate, the best techniques for surgical correction in multiple hereditary exostosis is not clear.  Multiple techniques have been described including lengthening the short ulna, correcting angulation of the radius, or 'detethering' (separation) of the radius and ulna growth.  There is no question that these techniques each have a role and make conceptual sense.  And all may allow the x-rays to look better.  But none have shown to clearly improve motion in patients with multiple hereditary exostosis.

And, to make things more confusing- even patients with dramatic findings on x-ray can do very well functionally.  The case below in a 12 year old with multiple hereditary exostosis proves this point. She has no pain, great motion, and no interest in surgery.  Time will determine if her outstanding function continues.

Multiple hereditary exostosis.  Note the radial head dislocation on the left.

Multiple hereditary exostosis.  Great forearm supination.

Multiple hereditary exostosis.  Very good but not perfect forearm rotation on both sides.

Multiple hereditary exostosis.  Right radial head is out of position.

Multiple hereditary exostosis.  Right radial head is dislocated.



Left elbow with radial head dislocation (with great motion and function). In multiple hereditary exostosis.

Right elbow with radial head dislocation although not as notable as the left (with great motion and function). In multiple hereditary exostosis.

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