Thursday, January 19, 2017

The Arthrogryposis Thumb

I have previously written many times about arthrogryposis and several years ago focused on the thumb- see Link.  As with every part of arthrogryposis, each child is affected differently.  But the thumb is affected, the position of the thumb can really make a big difference.  Consider these points:

1) The thumb normally provides about 40% of hand function.  So, in a child with arthrogryposis and a thumb which is not functioning well- overall hand function will be affected.  The thumb web space is key for holding big objects such as a soda can and, if the thumb is tight, that action may not be possible or may require using both hands together.
2) Not only will a poor thumb limit hand function, it can be doubly bad by also interfering with function by getting in the way.  Kids with more notable arthrogryposis often use both hands together (bimanual activities).  If the thumb is in the palm and can't be moved, using the two hands together may be challenging.

As I have previously written: Link to arthrogryposis posts, there are several surgeries which we in St. Louis like to use to help function including, elbow release, humerus rotational osteotomy, wrist closing wedge osteotomy, and thumb first web space release.

The tight thumb in arthrogryposis is likely due to several factors including limited muscle development and tight skin.  The thumb rests across the palm and straightening it can be tough.  To treat kids so affected, we can rotate skin from the index finger across the thumb web space while releasing tight muscle in the palm.  Sometimes we add a tendon transfer for thumb positioning.  We often hold the thumb in a better position with a metal pin for a few weeks while the soft tissues heal.   Here are a few pictures showing a range of presentations for kids with arthrogryposis and a tight thumb.

Somewhat less severe but still notable tight thumb in arthrogryposis.  Passive motion, demonstrated, was much better than active motion or the patient's ability to pull the thumb out of the hand.

Severe hand involvement in arthrogrypsosis

Severe hand involvement in arthrogrypsosis

If therapy fails to improve thumb position and thumb mobility, surgery can be considered.  Surgery improves thumb passive motion and hopefully also allows the child the ability to actively move the thumb to a better position for function.  Consider these pictures after surgery:

The thumb in arthrogryposis several weeks after surgery with healing of the flap and a markedly improved thumb position.

The thumb in arthrogryposis several weeks after surgery with healing of the flap and a markedly improved thumb position.

 The bottom line is the kids with arthrogryposis can be helped with function through therapy and through surgery.  Treatment of the thumb has become more commonly performed and we (provider and family) have been happy with the results.


Charles A. Goldfarb, MD
My Publication List
congenitalhand@wudosis.wustl.edu

Saturday, December 31, 2016

Surgery for Macrodactyly

Macrodactyly is an uncommon birth condition of the upper extremity.  I have posted several previous times on macrodactyly:

Macrodactyly Post
Macrodactyly Post 2

Macrodactyly means 'large finger' and sometimes is referred to as local gigantism.  My other posts have discussed different facts about the diagnosis including why this may happen but here I would like to discuss three potential surgeries for macrodactyly.

1. Local control of size and growth.  It is not uncommon that this surgery is performed multiple times on a young child.  The idea is to debulk the finger (primarily by removing extra fat and skin) but also potentially closing growth plate early.  The appeal of this surgery is that it is straightforward and seemingly less of a major step.  But there is a real negative- the potential for the need to repeat this surgery multiple times on a growing child.  This is an important consideration which must be considered.

2.  Ray resection.  If there is one large digit in macrodactyly, excision of that digit may be the best option.  This might make sense for a few reasons including the fact that the digit likely does not function well (often stiff) and is a cosmetic concern.  It is typically the middle finger that is large although the thumb and index finger may be involved.  But, when the middle finger is the one that is primarily involved, the functional and appearance concerns can be made notably better with this surgery.  The surgeon must consider whether to:
- only excise the middle finger (often called the ray resection which means removal of the finger and the appropriate metacarpal bone of the hand)
- excise the middle finger and move the index finger into its position.  The benefit of this surgery is that it makes a more normal appearing hand and removes the gap between the index and ring fingers.

Here is a recent surgical case in an adult who had grown frustrated with his large middle finger which did not help him functionally.  
Macrodactyly primarily involving middle finger.


Macrodactyly primarily involving middle finger.
Macrodactyly after ray resection with a nice appearance outcome.
Macrodactyly after ray resection and carpal tunnel release

3. Carpal tunnel release.  Macrodactyly is often associated with an enlarged median nerve and enlarged nerves to the fingers (digital nerves).  In both kids and adults with macrodactyly, this can lead to carpal tunnel syndrome including pain, numbness, and tingling.  A relatively straightforward carpal tunnel release surgery can relieve these symptoms.

Here is another recent macrodactyly case in which the patient was not concerned about the appearance of the hand and felt that function was satisfactory.  The thumb and index finger were most affected.  We therefore only performed a carpal tunnel release for the symptoms of pain and tingling.

Macrodactyly involving the thumb and index finger primarily.

Macrodactyly involving the thumb and index finger primarily.

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

Saturday, December 3, 2016

Simple Surgery for Symbrachydactyly

I have posted on several times on symbrachydactyly, with one post HERE.  Each child with symbrachydactyly, and really any birth difference of the upper extremity, is unique. No matter their bony and soft tissue deficit, their family situation and functional needs must also be considered.  So even if two kids look similar, I believe that musculoskeletal appearance is only part of the story- many other issues are considered.

This child has symbrachydactyly with a single digit (monodactyly type).  The thumb tip is flexed and he cannot straighten it.  His other extremity is normal.  Overall, he functions well.  The question that we discussed with the family is whether anything could be done to further improve his function.  Note the small palm which is typical in symbrachydactyly.

Symbrachydactyly with a single digit.

Symbrachydactyly with a single digit- side view.

Symbrachydactyly with a single digit- palm view.

Our team had discussions with the family and observed his function with activities.  Given that the thumb would not extend, together we all decided to position the thumb in extension with a stiffening of the joint (like a fusion but given the bony immaturity, technically not a fusion).  This more extended position should help with activities and will allow the patient to better use the hand for function.

Symbrachydactyly post surgery top view.

Symbrachydactyly post surgery- side view.

The pins will be in place for about one month and then he will use a splint temporary.  We look forward to this straightforward surgery improving function.

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


Sunday, November 20, 2016

Clasped Thumb Follow Up

I have posted several times on clasped thumb, a rare condition in which the muscles that straighten the thumb are slow to develop and the thumb rests across the thumb.  It must be differentiated from several other diagnoses included trigger thumb and spastic thumb (in which the tight muscles pull the thumb down).  Here are links to the other two posts: Post 1 and Post 2.

In, this post, I wanted to briefly share the early results after surgical treatment for clasped thumb.  Surgery is usually not required.  In most cases, support in the form of a soft splint or hard splint allow the thumb muscles time to develop.  However, for the rare child, those muscles don't develop and we perform a tendon transfer- where we move a muscle/ tendon from one position to the thumb to improve the strength of extension.  Good results are expected and, at early follow- up, here at 3 months, good results have been obtained.  Remember, it is not easy to capture pictures of a very active 2 year old but I believe we can see the results!

Patient doing well after clasped thumb surgery

Excellent thumb straightening 3 months after clasped thumb surgery.


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

Saturday, November 19, 2016

Patient Pollicization Testimonial

Gracie is an 11- year old who had a pollicization 3 months ago for her hypoplastic (small) thumb. Compared to the typical patient treated with a pollicization, Gracie is unusual because she was much older at the time of her surgery.  Gracie's age at the time of surgery does bring a few challenges mainly because she has been using her four finger hand with her 'small' thumb for her entire 11 years. However, she came to realize the challenges of life without a highly functional thumb- her thumb did not function .  The thumb is key for large object grasp such as grasping a soda can which is impossible with the fingers for most of us.  Additionally, the thumb allows fine pinch- also very difficult with the fingers.  Gracie and her family elected to proceed with the surgery.  Below find pictures before and immediately after surgery.

Hypoplastic thumb before surgery
Hypoplastic thumb before surgery

New thumb immediately after surgery
New thumb immediately after surgery

New thumb immediately after surgery

I recently saw Gracie in the office 3- months after her surgery.  She is an impressive and dynamic 11 year old and agreed to share her thoughts on video.  She is very excited about the results of the surgery.  Her thumb is working great and will continue to get better.  I wanted to capture Gracie on video as her words may help parents considering this surgery for their child.



I hope these pictures and, more importantly, this video are both helpful for families considering the pollicization procedure!

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

Surgery Ecosystem

There are many factors for families to consider as they choose a surgeon and a hospital.  There is no perfect path to making these choices but some factors that I believe are important include:

1) Surgeon.  I have previously blogged about choosing-your-childs-surgeon  and this is a key factor for consideration.   I believe that the surgeon must be committed to the care of kids' upper extremities, be involved in the community of doctors who care for these types of patients, and be committed to advancing the field.  I believe surgeons who only occasionally treat kids are not ideally suited to take care of your child with a birth difference of the upper extremity.

2) Hospital.  Utilizing a children's hospital that takes care of kids 100% of the time is an important criteria, as everything the hospital offers is specifically for children.  The anesthesia is more routine, the nurses more familiar, the whole process easier.  This does not mean that you can't get good care in a hospital that treats all aged patients, it just means that if you have a choice, pick a hospital that has specific expertise in pediatric and adolescent patients.

3) Therapy.  We tend to focus on surgery as the most important step in treatment.  And it often is critical, but in many conditions, therapy can be effective instead of surgery and therapy is almost always utilized after surgery.  Therapy matters and therapist experience matters.  You want a team taking care of your child that has experience in your child's specific condition. Splints, exercises, and wound/scar care all effect outcome.

4) Communication.  I strongly believe that when a physician takes care of kids, they should provide increased access for families.  While this can mean the opportunity for thorough office visits, it also means availability to communicate via phone or email.  I understand that decisions about surgery can be challenging for families, and having all questions answered and feeling comfortable help to make the decision easier.  While not every doctor will share his/ her email or cell phone, many will.  Consider this in your decision.

5) Research.  While taking care of the patient is and should be the primary 'job' of any doctor or surgeon, research is another consideration.  Some doctors focus on patient care, but others take care of patients AND teach and conduct research.  Research has the potential to change our understanding of birth differences, change our treatments, and improve outcomes.  Research is ultimately the way we can positively affect all kids born with upper extremity differences.  I feel this is a critically important goal.  It is important for families to understand that research can be done in a way that does affect their child's care in any way as most research is simply observational (we watch and understand how certain treatments affect outcome).  Participating in research offers the hope of a future with increased knowledge of best practices for kids with birth differences of the upper extremity.

There are many factors for families to consider as they pick a team to care for their child.  I hope my brief discussion of a few of these factors is helpful.

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





Friday, October 28, 2016

Buenos Aires IFSSH Meeting

It was my pleasure to attend the 50th anniversary meeting of the IFSSH- the International Federation of Societies for Surgery of the Hand.  This international organization meets every three years (previous meetings in South Korea and India, next meeting in Germany) with a weeklong exchange and dialog on hand surgery.  I was pleased to have the opportunity to be a part of a great symposium as well as share our work in 3 scientific presentations.



Steve Moran organized a great congenital hand symposium including Scott Oishi (USA), Michael Tonkin (Australia), Goo Hyun Baek (South Korea), Miguel Hernandez (Mexico), and Neil Jones (USA) discussing late complications after a variety of congenital procedures.  I was pleased to participate and discuss late complications after syndactyly reconstruction.  I shared our experience in St Louis including the early positive findings with hyalomatrix.  Great symposium!

We also shared our experience in scientific presentations for
1) humerus rotational osteotomy for internal rotation posture in arthrogryposis
2) hyalomatrix early outcomes with syndactyly reconstruction
3) Elbow release procedures

Buenos Aires is an amazing city which I was briefly able to tour. A few of the sites:

One of the many beautiful churches in Buenos Aires

The widest avenue in the world with the Obelisk in the distance

One of the few "Thinker" statues by Rodin, in Buenos Aires.

In short, while my trip was brief, it was both a great cultural experience and scientific experience!


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