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

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

Charles A. Goldfarb, MD
My Bio at Washington University

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

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

Monday, February 8, 2016

Separating joined fingers: A Case Demonstration of a New Strategy

Surgery to separate joined fingers has been largely unchanged for 40 years.  This post is meant to share case pictures and details on this new strategy. Syndactyly, or the abnormal joining of the fingers, is one of the most common birth anomalies of the hands and feet.  In our recent assessment of birth anomalies in NY State, the prevalence of syndactyly was approximately 1.3 in 10,000 live births (polydactyly was clearly the most common anomaly).

The treatment of syndactyly has been successful but with little change over the last 40 years.  I have previously blogged a few times on this topic HERE.  We understand the importance of the webspace (the 'commissure') and avoiding tension on the flaps. Skin grafts are often necessary in syndactyly reconstrution (although there are good graftless techniques- see previous posts).  I recently blogged HERE about a new technique that we have utilized- a skin graft substitute called hyaluronic acid scaffold.  It has been very successful in our hands and we hope to share our early term results soon.

This case demonstrates both the basic principles of syndactyly reconstruction and the use of the hyaluronic acid scaffold instead of skin grafts.  I will blog in the near future with some early postoperative results in another case.

Syndactyly of the middle and ring fingers.

Planned skin incision in syndactyly reconstruction.  Note the primary commissural flap and the zig zag flaps.

Syndactyly reconstruction from palmar approach.
Syndactyly reconstruction after suturing.
Syndactyly appearance from the palm after suturing.
Syndactyly after suturing with areas in need of coverage.

Syndactyly after suturing with areas in need of coverage.

Syndactyly reconstruction after placement of hyaluronic acid scaffolding (white)
Syndactyly reconstruction after placement of hyaluronic acid scaffolding (white)

Syndactyly reconstruction after placement of hyaluronic acid scaffolding (white)

Charles A. Goldfarb, MD
My Bio at Washington University

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