Wednesday, January 27, 2016

Not the Typical Thumb and Wrist

Radial Longitudinal Deficiency (RLD) has a wide range of presentations from a small, unstable thumb to major bone and soft tissue problems of the entire upper extremity.  And yet, despite this wide range, most kids have a somewhat predictable appearance within a couple of different categories.  I have shared my thoughts on these variations HERE, numerous posts included.

We have a large group of patients that we follow with RLD and, therefore, we see some kids that fall outside that 'typical' group (for this very unusual problem).  This is one such child.

Left wrist in radial longitudinal deficiency.  Note the flexed and deviated position of the wrist.  Also, there are only 4 fingers.

Right wrist in radial longitudinal deficiency.  While the wrist is slightly deviated, the major issue is the thumb.

His right forearm is relatively normal but his hand is not.  He has an unusual type of hypoplastic or underdeveloped thumb.  The web space between the thumb and index finger is very small.  This is a key factor for function including grasping large objects.  He was treated surgically to widen the space, stabilize the thumb, and provide a new muscle for thumb strength.
Hypoplastic thumb with very small web space.
Hypoplastic thumb with very small web space, view from palm.
Large dorsal flap designed to maximally widen the web space in the hypoplastic thumb.

View of the hypoplastic thumb immediately after surgery 
Another view of the hypoplastic thumb after reconstruction.
The left upper extremity was a bit more of a challenge as the forearm was affected- a type IV radial longitudinal deficiency- and the hand was affected as well.  The thumb was also affected in a very unusual way- the index finger and the thumb were fused.

Type 4 Radial Longitudinal Deficiency with absent radius and unusual, fused thumb and index finger.

The first step for us was to address the forearm.  The patient did not have a radius or any of the radial sided muscles.  We therefore chose a centralization procedure (again, see previous posts HERE) with a fixator to stretch the tight structures.
Here is the fixator just before removal in radial longitudinal deficiency.  Note how straight the wrist has become.

Radial longitudinal deficiency after fixator removal.

Radial longitudinal deficiency after fixator removal and after centralization.  Note the straight wrist. 
Here is the wrist position about 6 months later.  Note that the wrist remains straight.  Also note, the position of the thumb which is a real challenge.  It has little movement.
Radial longitudinal deficiency after centralization.  The thumb remains a challenge.
The extra thumb bones were removed to increase the chance of useful thumb motion and the thumb was repositioned.

Here are our most recent pictures showing hands and thumbs that are in an improved and more functional position.  The family is pleased with function and appearance.

                                    Patient appearance after right thumb reconstruction and left wrist and thumb reconstruction                                                   for radial longitudinal deficiency.

                 The patient does use the thumb even though it remains stiff.  Thumb function after reconstruction for radial longitudinal deficiency and thumb deficiency.
While this is an unusual type of radial longitudinal deficiency, the basic principles of our care program are applied.  We work with the family to develop a plan to maximize function and minimize the number of surgeries required.  We will follow the patient over many years to observe function and use therapy as necessary if functional limitations are demonstrated.  We do not expect he will need additional surgery, but we will follow him to observe his development and limitations.

Charles A. Goldfarb, MD

My Bio at Washington University

Tuesday, January 19, 2016

Prosthetics: 3D Printed vs Task Designed

The Shriners Hospitals have long been known for Prosthetics.  Each hospital in the system has expertise for both upper and lower extremity prosthetics.  As I have mentioned in previous blog POSTS, there is a great deal of new, exciting progress in the field.  We continue to work here at the Shriners Hospital in St Louis and St Louis Childrens Hospitals together with Washington University on 3D printed prosthetics.  Two recent news clips highlight these efforts:

St Louis Today Link

Outlook Magazine

However, it is important to remember that not all prosthetics need to be high tech to be very effective.  We learned from Dr Michelle James and a group of Shriners surgeons (as seen HERE in this great article from 2006) that many kids abandon their upper extremity prosthetic as they may not be helpful for quality of life or function.  As I have previously discussed, this has led to a change in philosophy in the time to best fitting of the prosthetic.  Now we often wait until a child has a specific need or request.

Here is one great, very specialized example of a specific request: a task- designed prosthetic.  This child has bilateral cleft hand with a single digit on both sides.  He functions really, really well using both hands together for many activities.  But one task that was important to him and his family- but was a challenge- was fishing.  Therefore, a special prosthetic was designed which helps him accomplish this goal.  He is very pleased with the prosthetic.
Prosthetic specially designed for fishing!

Charles A. Goldfarb, MD
My Bio at Washington University

Monday, January 11, 2016

More on Amniotic Constriction Band

I have posted several time previously on Amniotic Constriction Band as can be found HERE.  There is also reasonable information on the web as can be found at Wikipedia and NORD and HERE. However, I do believe that it is helpful to share case examples.

Here is a 6 month old with Amniotic Constriction Band that was urgently treated for a tight thumb band and then sent to us for further assessment.  Pregnancy was uneventful and ultrasound did not identify the anomaly.  I want to emphasize again that this is really not a syndrome (which implies other findings) but is a diagnosis on its own.  It can effect any extremity or really any part of the body.  The right hand is more severely effected.  The thumb was treated emergently at another hospital due to a concern about survival.  This is a rare but real concern- the constriction band can be tight enough to cut off circulation.  There is a great pinky finger but the other digits are limited and there is the large excess tissue on the ring finger.  The left hand is less severely involved with a band on the index finger and a short middle finger.

Right hand with amniotic constriction band.  

Right hand with amniotic constriction band.  

Left hand with amniotic constriction band.  

This patient was taken to the operating room to remove the band on the left index finger and address the thumb on the right.  In addition, we removed the large bulbous tissue on the right ring finger.
Right thumb after reconstruction for amniotic constriction band.  Sutures are still dissolving.

Right ring finger after mass excision in amniotic constriction band.

Left index finger after reconstruction for amniotic constriction band.
This patient should do wonderfully from both an appearance and functional perspective.  The skin will continue to remodel.  The digits are sufficient for most functional activity.

Charles A. Goldfarb, MD
My Bio at Washington University

Sunday, January 3, 2016

Cleft Hand Reconstruction

I have posted a number of times on cleft hand, or central deficiency as can be found HERE.  To me, cleft hand is one of the most interesting diagnoses as kids with it may look and function very differently from each other despite have the same diagnosis.  Some of my other posts highlight these differences between kids with cleft hand.  This highlights the importance in understanding both the functional and the appearance differences for each child.

This case is that of a child who is a bit older- at 12 years- with less dramatic complaints compared to some kids but with clear functional difficulties.  There is a syndactyly involving the ring and small finger which limits his ability to wear gloves and maximally spread the fingers and the ring finger has limited flexion (bending) and some angulation.
Cleft hand with limited ring finger motion and syndactyly

Cleft hand palm view with limited ring finger motion and syndactyly

x-ray of cleft hand
This cleft hand is very interesting to me.  The pictures of the hand and the x-ray are informative.
  1. The cleft itself (ie, the gap between the index finger and the ring finger) is not a problem for this child.  Sometimes this gap is much larger and, therefore, more of an issue whether for appearance or function.  
  2. The thumb- index webspace is not an issue in the patient.  It is often the single biggest problem in cleft hand as it limits function if it is narrow.
  3. The xray clearly shows the extra bone from the missing long finger is joined to the ring finger.  This is the proximal phalanx of the long finger (middle finger).  In this child, that bone is causing some difficulties with ring finger motion and also deviation.  
  4. Finally, it is not uncommon to find syndactyly between the ring and small finger.  However, patients and families do not always find this to be a problem (in this case, it was).
As mentioned above, the primary issue for this child was the ring finger, tethered on the thumb side by the extra bone and on the pinky side by the syndactyly.  The family requested reconstruction.  

We removed a portion of the extra bone and deepened the web space between the ring and small finger.  Certainly, we could have done a more extensive surgery but, as for most cases, we focus on accomplishing our surgical goals in an appropriately limited way.  Early results are promising.  

Cleft hand after reconstruction.

Cleft hand after reconstruction.
Charles A. Goldfarb, MD
My Bio at Washington University

A New Year filled with Hope

Happy New Year!

My hope for 2016 is much progress towards our understanding of birth differences of the upper extremity. 

My primary resolution is to continue our research in St Louis at Washington University, St. Louis Childrens Hospital, and the Shriners Hospital for Children.  We have a number of exciting projects underway including:
  1. The CoULD (Congenital Upper Limb Difference) Database.  This is a partnership with Boston Childrens Hospital (and in 2017, at least 2 additional institutions) to identify and tracking children with birth differences over time.
  2. Projects examining treatment outcomes for
    1. Hypoplastic thumb
    2. Arthrogryposis
    3. Other congenital differences
  3. Several projects examining outcome instruments for kids (PROMIS, PODCI, etc)

My other resolution is to post to this blog more regularly.  My goal is once a week and I hope to better keep to this pace in 2016.

Finally, I am intrigued by the potential of 3D printing as it can benefit kids with upper extremity differences.  We have one publication in print on this topic which is described HERE.  We have another manuscript which has been submitted for publication describing some notable advances. While there is a great deal in the popular press on the topic, there is very little in the literature.  One additional manuscript can be found HERE.  

I have become somewhat more knowledgable on the Magic Arms effort.  This is a 3D printed device (called Magic Arms or the WREX) which can help kids overcome gravity for functional improvement. 

The organization attempting to increase our understanding and the availability of these devices is MAGIC ARMS- their website is worth a look and is also a new Link for my blog.

Happy New Year.

Charles A. Goldfarb, MD
My Bio at Washington University