Monday, October 27, 2014

Shark Tank

Proud to post about my wife and her business, Myself Belts.  Talia and her sister created a patented belt that allows kids and adults to fasten a belt with one hand!  The belt has been really helpful for many of my patients.

Check out her Website!

The exciting news is that Talia and Myself Belts will be on TV this Friday night.  On Shark Tank!

Charles A. Goldfarb, MD
My Bio at Washington University

Wednesday, October 22, 2014

Small finger polydactyly, extra fingers

Extra fingers can be on the thumb side of the hand, the central part of the hand, or the small finger (pinky) side of the hand.  The location of the extra digit is important because it relates the risk of other conditions such as syndromes or other abnormalities.  Small finger polydactyly is the most common location and most patients have a very small extra digit, or nubbin.  However, others can have fully formed extra digits which can either be straight or 'crooked."  African Americans are most commonly affected with the extra small finger and there is a strong autosomal dominant hereditary pattern.  Rarely are there any other health problems in these patients.  Caucasians are more commonly affected with thumb sided polydactyly.  When Caucasians have small finger polydactyly, the physician needs to be aware of the syndromes that can be associated.  A genetic assessment may be recommended.

Wikipedia has a nice summary of polydactyly.  There are a number of syndromes which are considered in these patients including Greig cephalopolysyndactyly syndrome, Meckel syndrome, Ellis van Creveld syndrome, McKusick Kaufman syndrome, and Bardet Biedle syndrome.  

Ellis van Creveld syndrome is perhaps the most discussed of these associated syndromes and is quite uncommon.  As always, there is great information on the NIH Genetics Homepage.  It is also known as chondroectrodermal dysplasia.  Other important clinic findings aside from the polydactyly include: short stature, abnormal fingernails, and heart defects (in some patients).   We know a fair amount about the genetics. It is an autosomal recessive disorder meaning the affected child received the abnormal gene from each parent and mutation is in the EVC gene (or EVC2 gene).  It is more commonly seen in the Amish population in Pennsylvania and also in Western Australia.

Below are clinical pictures and a few x- rays demonstrating the appearance of the hand.
Ellis van Creveld syndrome with 6 digits on the left hand.  Slightly crooked ring and small fingers, the site of the polydactyly.

Palmar view of Ellis van Creveld syndrome with polydactyly.

X-ray of polydactyly in Ellis van Creveld syndrome.

The right hand in a patient with Ellis van Creveld syndrome with 6 digit polydactyly.  Note the deformity of the ring and small fingers and extra digit.

An x-ray of the right hand in a patient with Ellis van Creveld syndrome with 6 digit polydactyly.  Note the deformity of the ring and small fingers and extra digit.

Charles A. Goldfarb, MD
My Bio at Washington University

Monday, October 13, 2014

Ulnar Deficiency and Syndactyly

Ulnar deficiency (or ulnar longitudinal deficiency) is much less common that radial deficiency, possibly only 1 case of ulnar deficiency per 10 of radial deficiency.  And it is much different in the problems that it may cause.  The forearm abnormality can include a deficient or absent ulna and flexor muscles BUT, it is much better tolerated than radial deficiency.  There is less deformity and more wrist stability.  And the shoulder can help compensate more easily for the deformity.

The hand abnormality in ulnar deficiency can include missing digits (so- called ectrodactyly) and syndactyly (abnormal joining of the fingers).  I have previously blogged about ulnar deficiency- multiple examples are seen on this page.  Also, I have written on multiple occasions about syndactyly here.

The following case demonstrates many important points in the understanding and treatment of ulnar deficiency and syndactyly.

Ulnar deficiency and syndactyly

Palmar view of ulnar deficiency and syndactyly

This child has a relatively normal forearm (slightly short ulna only) without deviation of the wrist.  He has great wrist motion along with good elbow motion and forearm rotation.   He has a great thumb, a full thumb web space, and  two really good fingers.  However, the fingers are joined together (syndactyly).  To further promote hand function with independence of the fingers, the family and I elected to separate the fingers.

What follows are clinical pictures of the planned skin incisions for syndactyly reconstruction and the separate digits.
Syndactyly flaps from top of hand

Syndactyly flaps from palm side.

The flaps are elevated and sutured into place.  We use absorbable sutures so nothing has to be removed and place a bulky dressing for 3 weeks.
Syndactyly reconstruction from top of hand.

Syndactyly reconstruction from palm view.

We performed this surgery as we believe it will take a really good hand in a child with ulnar deficiency and syndactyly and make it functionally better.

Charles A. Goldfarb, MD
My Bio at Washington University

Sunday, September 28, 2014

St Louis Shriners Hospital Does Great Things for Kids

I am very fortunate to work at the St Louis Shriners Hospital.  The first Shriners Hospital opened in 1922 in Shreveport, Louisiana and more soon followed to care for children with polio.  The St Louis Shriners Hospital opened in 1924 on the main campus of Barnes Hospital and moved to the current location on Lindbergh in 1963.  Now, 90 years after opening the original facility, the hospital is about to move back to the Barnes Hospital campus with a beautiful new facility.  We care for children with orthopedic challenges of all varieties including those with birth anomalies, trauma, scoliosis, and many other conditions.

After 90 years in St Louis, the Hospital and its staff continue to do amazing things for kids.  Here is a great video highlighting one such special patient who I know well.

Hope for Jude Video

Another amazing program which our team hosts each year is Hand Camp.  I have previously blogged about hand camp Hand Camp 2013 and Hand Camp 2012 but this camp has been an anticipated part of our year since 2008.  Each year dedicated therapists, nurses, and staff meet at Camp Lakewood in Pitosi Missouri for a weekend of fun, education, and enlightenment.  The junior counselors at Hand Camp share their experiences and guide the kids (and families) all weekend.  It is definitely one of my favorite times of the year; I am able to interact with families outside the hospital and watch them learn and share with other kids and families struggling with similar issues.

Amazingly educational, grounding, and fun.  Here are a couple of pictures from Hand Camp 2014!
The amazing junior counselors at Hand Camp 2014.  
Kids and Counselors at Hand Camp 2014

Two campers just before Hand Camp 2014
Hand Camp 2014.  

Friday, September 5, 2014

Patient TV Interview Regarding 3D Printed Prosthesis

Check out this great interview with a wonderful patient/ family regarding our 3D printed prosthesis. 
Go Sydney!

Interview with Sydney and family

Charles A. Goldfarb, MD
My Bio at Washington University

Tuesday, August 26, 2014

Central Polysyndactyly

Polydactyly means that more than the normal 5 digits.  Typically patients have either an extra thumb or an extra pinky.
A patient with radial polydactyly (extra thumb).
Syndactyly is the abnormal joining of the fingers.  It is most common between the middle digits but can affect any of the fingers.
Syndactyly of the fingers.
While these conditions are uncommon, each is one of the most common birth (i.e., congenital) anomalies of the upper extremity.  And both typically do well with surgery.  I have previously posted several times on both syndactyly and polydactyly.

Central synpolydactyly is, in contradistinction, very uncommon.  In this condition, there is both syndactyly of the central digits and an extra digit in the middle of the other fingers.   It comes in variety of different formats- I have shown a few of these different types below.

Central synpolydactyly is, however, a very difficult problem to treat.  Even with successful excision of the extra digit and separation of the digits, the outcome is still a challenge.  Specifically, even after a "successful" surgery, finger motion is often limited and there may be finger deformity.  Yet, surgery makes sense because it does improve both function and appearance.
Left hand, central polysyndactyly.

Right hand, central polysyndactyly.

Right and left hands, central polysyndactyly.  Note the differences between the right and left sides.  There is a "bizarre" collection of bones rather than the typical straight fingers.

Happy reading.

Charles A. Goldfarb, MD
My Bio at Washington University

Friday, August 15, 2014

Scarring after Syndactyly

Syndactyly, or the fusion of digits, is a condition present at birth and is related to the failure of the fingers to separate in utero.  I have previously blogged about this condition, as seen here.  There are different surgical techniques to correct syndactyly and generally good results are obtained, no matter the technique.  

Scarring is one concern with any surgery for syndactyly.  There are several problems with scarring. First, if scar bands form, the finger may contract and motion may be limited.  This can cause a functional problem, most commonly, a limitation in finger straightening.  One way to lower this risk is zig- zag incisions.  Another problem with scarring is what we call creep.  Creep is the slow process of the skin "growing" towards the fingers- causing a loss of the deep webspace.  One way to think of this is that the syndactyly is regrowing.  The literature is not clear on the percentage of children with syndactyly that develop creep after surgery, but it is likely less than 1 in 5 patients.  The 3rd issue regarding scarring after syndactyly surgery is the appearance.  We hope and strive for a complete correction of syndactyly without prominent scars and usually we can accomplish that goal.  However, thick scars and dark skin grafts are a problem that no one likes to see.  We try to avoid skin grafts to avoid the appearance below but sometimes grafts are necessary.  We have found better results with skin grafts taken from the front of the elbow compared to the groin.

Most children with syndactyly reconstruction do wonderfully.  Surgery can create new webspaces between the fingers that look very similar to the other webspaces/ fingers.   It is, therefore, a surgery that we enjoy.  However, the risks of scarring, as noted above are concerning and we work to minimize the risk of those problems.  The surgical techniques and surgeon experience both can help lower the odds of problems with scarring.

Scarring after syndactyly surgery.  Note the prominence in the space between the thumb and index finger.

Scar bands limiting finger extension after syndactyly surgery.  Note also the darkened skin grafts.  
Another view of scarring after syndactyly surgery.  See the lack of finger extension from scars.
The following patient has symbrachydactyly (short, webbed fingers) and has had surgery for the syndactyly.  While he and his family remain pleased, there was creep between the index and ring fingers.

Patient after surgery for symbrachydactyly.  Doing very well but scars are notable.

Limitation noted with creep affecting the space between the index and long fingers after symbrachydactyly surgery.

Charles A. Goldfarb, MD
My Bio at Washington University