Sunday, November 23, 2014

Inspiration

I am fortunate.  Each day I am inspired by the kids (and families) that I am able to treat. These children are born with differences and, therefore, they do not know any other way.  But they must learn to exist in the everyday world with functional and social challenges.  And succeed they do.  Each child has different tricks and tips, coping mechanisms and strategies- techniques built up over time and with family support.   That is why, each day, I am inspired.  And if I have the opportunity to make things a little easier for a child (with surgery or therapy or...), I am happy to help.  And, as suggested below, share some of the "light."

Edith Wharton said " There are two ways of spreading light: to be the candle or the mirror that reflects it."

What follows are a few spectacular examples of kids with birth differences succeeding in ways that amaze.

This first video is Adrian Anantawan, a violinist that I was lucky enough to see in person.

video


And another video of Adrian Anantawan from CNN.

video


Here is a one- handed, high level college basketball player.  Check it OUT.


Here is a wonderful example of basketball skills despite radial deficiency (thanks to Scott Kozin).


video


Lastly, here is an amazing example of the power of a positive outlook.  Brian Ndungu.







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




Sunday, November 16, 2014

Great Toe Polydactyly (extra toes)

It might seem odd that I am writing about extra toes.  Typically, hand surgeons don't take care of the foot but, in many situations, the hand surgeon is the perfect person to help take care of certain foot problems including extra toes, toe syndactyly (toes joined together), and even things like cleft foot. The reason I feel that the hand surgeon can help with certain foot problems is pretty clear- the foot issues are similar to the hand problems and the reconstruction techniques are very similar.  This not to say that there are plenty of great pediatric orthopedic surgeons who can care for toe and foot issues like these, because there certainly are.  

I have previously blogged about extra toes- find that here.   I hope to also post about toes on the outside of the foot- there are a bit easier to address and less critical for balance and walking.

It is key that the big toe be as sound as possible with reconstruction.  We want to create the right sized digit (i.e., one that won't cause problems wearing shoes) that has good motion and is stable.   To me, this is very similar to reconstructing the extra thumb as the goals are similar and the thumb and the great toe are critically important to hand and foot function.  The timing is debatable.  Some surgeons chose to operate before the child really starts walking as it makes the recovery easier while other wait longer as the toes get larger (and operate at 18-24 months).  I prefer to operate before walking but there are a number of factors to consider and discuss with each family.

Surgically, the reconstruction involves the following:
1) The skin.  We need to create a great toe that is not tensioned by the skin immediately or in the future (from scarring).
2) Removing the extra toes.  Ideally the smaller toe or the toe with greater challenges from a bony or ligament standpoint.  Sometimes, as in this case, the toes are relatively equal and this decision is not quite as straightforward but typically we remove the inner toe.
3) Stabilize the remaining toe to create good ligaments
4) Make sure the remaining toe is as straight as possible and is well set up for growth in the future.  There is always a chance that there will need to be a surgery later but hopefully it can be avoided if we get good balance with the first surgery.
5) Address the toenail.  This can be quite challenging with conjoined nails but the goal is a well balanced toe with good nail support.

I typically cast for about 5 weeks and often use a pin to stabilize the healing toe.  That pin is removed at the time of cast removal.  Here is a case example.  The x-rays are particularly interesting as there are delta phalanges on both sides- that is the bones are not the typical rectangular shape but are curved which can indicate an abnormal growth plate.  This may be corrected at the initial surgery.

Extra great toes with conjoined nails (synonychia is the technical term).  Note that there is some deviation of the toes.

Weight bearing with even more deviation noted in the extra great toes.

X- rays of the extra great toes.  Note that the feet are different.  
3 months after surgery.  The child is walking well and the family is very pleased.  
Great toes after reconstruction.  Excellent alignment has been achieved.  The nails are imperfect.
Great toe after reconstruction.  Right.






Great toe after reconstruction. Left.

Tuesday, November 11, 2014

Arthrogryposis: Arm position and elbow flexion

Kids with arthrogryposis are smart and are able to figure out how to accomplish many of life's tasks despite their functional limitations.  We, as surgeons, can sometimes help children with arthrogryposis by releasing and/ or repositioning tight joints.  We cannot make the arms or hands normal but little changes can make for big improvements.

It is always amazing for me to watch kids with arthrogryposis figure out the best way to accomplish a task.  Furthermore, different kids almost always figure out the same way to do these maneuvers. These tricks are not taught but are highly effective ways to get the job done.  Below is one example. Watch this child with arthrogryposis:
1) use his back to swing his arm into flexion and prop it onto the table
2) then use the table to further flex the elbow 
3) Finally he bends his neck forward to allow his hand to reach his mouth

And he makes it look so easy! I thank his family for allowing me to post this video.


Many children have stiff elbows.  Surgery to release the stiff elbow and allow elbow flexion (even without a muscle to power the elbow flexion) can make a big functional difference in a child's life.   One of the most impactful scientific articles on arthrogryposis treatment was on this Topic.  Straight elbows are tough as you simply cannot get your hand to your mouth!


The other interesting thing about this child with arthrogryposis is the fact that his arm is internally rotated.  Look at his right arm especially- the hand is pointed backwards because the shoulder is rotated in.  While he can adjust to this position somewhat, it can make grabbing and functioning a bit harder.  See our previous Post on this topic.  If you look again at the video above, when he swings his arm up onto the table, the hand is pointing backwards and he has to shift it around so that he can use it on the table.
Arthrogryposis with internal rotation of the arm.

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





Sunday, November 2, 2014

Early Outcome for Pollicization (creation of a thumb)



Pollicization is the name of the surgery which entails the creation of a new thumb, a "Pollex" (Latin for thumb).  For those of us who regularly care for children born with anomalies of the upper extremity, this is perhaps our favorite because it dramatically helps both appearance and function.  I have previously blogged about pollicization several times as summarized in this Post.  There is some good information on the Internet about pollicization.  My friends at the Texas Scottish Rite Hospital have a wonderful set of videos on pollicization.  The following is a trailer from their work with some very good information:
One of the interesting questions that we face as surgeons is regarding the timing of this surgery.  We want it to be early enough so that the child learns to incorporate the thumb into daily activities as soon as possible.  There is some thought that the surgery should be done very early (1 year of age) so that the brain is reprogrammed to understand that the index finger is now the thumb.  It is my personal belief that the brain has an understanding of the index finger as the most radial (or thumb sided digit) and so pushing for earlier surgery is not necessary.  The child will learn to best incorporate the thumb and it will become consciously and subconsciously clear that the new thumb will help the child function.   

Others wonder whether waiting until an older age might be helpful.  As the hand doubles in size between birth and 2 years of age and then nearly doubles again at maturity, a larger hand will be easier to address surgically.  And a larger hand will be safer to address as the key anatomical structures are bigger.  All of this is true but with surgical experience, the "risks" of surgery in the younger child are very manageable.  An older child, it is argued, will also better participate with therapy.  This is also true but, thankfully, not a great deal of therapy is needed as the thumb usually works well for the child and time/ play activities are usually the keys to a successful functional outcome.

I personally take what I consider is the middle ground an like to perform the pollicization between 18-24 months of age.  I feel that it is the best balance between the benefit of an early surgery (incorporation of the new thumb into life) and a later surgery (larger size and the child may be easier to work with after surgery).  

Here are a few pictures of a child we treated recently.  

Hand with absent thumb before surgery

Palm view of hand with absent thumb before surgery


We performed surgery to create the thumb using the index finger- the pollicization procedure.
After pollicization surgery.  Note the index finger has been reconstructed into a thumb.

After pollicization surgery.

Here is the child only 6 weeks after surgery.  Note that the child is using the thumb but not quite "normally."  He tends to use the two fingers first with the thumb as an assist.  Dad has noticed that he is progressively using the thumb more and more.  It will be fun to watch him learn to incorporate the thumb into all activities- his use pattern will change during the next 3-6 months.  It is tough to video kids this young, maybe on his next visit I can obtain and then share. 
Early after pollicization, using the thumb to help the next to finges.

Early after pollicization, using the thumb to help the next to fingers.
Another picture 6- weeks after pollicization.  He is using his left hand but differently from his normal right hand.  This will change over time.


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







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
congenitalhand@wudosis.wustl.edu





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
congenitalhand@wudosis.wustl.edu

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
congenitalhand@wudosis.wustl.edu