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