Saturday, June 16, 2018

Families, Choices, and Untreated Triphalangeal Thumbs

One of the benefits to my taking care of kids with birth anomalies of the upper extremity is the opportunity to really come to know the patient and the family, often over many years.  This is magnified when more than one child is affected.  This is one of the best things about my practice (and one of the limitations of the other parts of my practice)- that is treating a patient and family over years.  I really enjoy watching kids grow, learning about their development, and understanding how their birth anomaly does or does not affect their lives.

Another interesting opportunity is talking to and understanding the experience of an affected parent.  This is critically important because the experience of the affected parent influences their goals and hopes for their child.  It also usually moderates their concerns.  What I mean by this is that most parents who are also affected understand that their child is likely to be highly functional and also likely to be well adjusted

Kids with birth anomalies never cease to amaze us and it takes time for 'new' or unaffected parents to realize just how well their child will do and how many expectations will be exceeded.  Affected parents simply better understand their child's status and have appropriate expectations for the future.  

One particular family has become particularly special to me as I have come to know the whole family and done so over many years.   Mom and the kids have triphalangeal thumbs together with an extra thumb.  Mom's triphalangeal thumbs were untreated (although the extra thumbs were removed when she was a child) and she understands the diagnosis and its implications very well.  We have performed surgery on her kids due, at least in part, to mom's understanding of how surgery would be helpful.

A few features to take note of.  
1) The thumbs are long.  This is due to the extra bone in each of the thumbs (triphalangeal- 3 phalanges instead of the normal thumb with 2 phalangers).  
2) The thumbs appear thin, almost like a finger.  
3) The thumbs do not rest in the normal position.  The thumbs are more in the plane of the fingers- some might call this a 5 fingered hand rather than a hand with 4 fingers and a thumb.

Adult with triphalangeal thumbs.

Adult with triphalangeal thumbs, palm side

Adult with triphalangeal thumbs, thumb flexion.  Note the length.

Adult with triphalangeal thumbs. Note that the thumbs are in the plane of the fingers.

Adult with triphalangeal thumbs, right
Adult with triphalangeal thumb, left

Nonetheless, the patient has very good function.  There are a few challenges with fine motor skills and pinch.  This video shows thumb motion and gives a sense of function.

A few relevant blog posts:

The bottom line is that patients with a triphalangeal thumb function well with or without surgery.  However, surgery does offer improved function and appearance as confirmed by this family and the choices they have made.

Charles A. Goldfarb, MD
My Bio at Washington University

Sunday, June 10, 2018

Three thumbs

Radial polydactyly (duplicated thumb) is an uncommon condition in the general population- I have blogged about kids with extra thumbs a number of times.  Those posts can be found HERE.  Extra thumbs come in a wide variety of patterns, some of which are captured in the Flatt Classification (previously called the Wassel Classification) as shown in the this picture from
Flatt Classification for radial polydactyly from

Yet, this classification does not capture the extra thumbs in all kids with radial polydactyly.  Many simply do not fit this pattern.  As a result, the Rotterdam (in the Netherlands) group added a more complex classification system which is much more comprehensive.  The publication abstract can be found HERE.  It includes more specific patterns for triphalangeal thumbs (longer thumbs, more like a finger), triplicate thumbs (three thumbs, rather than the more common 2 thumbs), as well as adjustments for stiffness, deviation, and small size). 

This is one example of a triplicate thumb.  These are uncommon (even compared to the uncommon radial polydactyly). 
Triplicate thumb

Triplicate thumb, another view

Final view of triplicate thumb

And here are a few x- rays which show the complexity.  This is a complex arrangement of bones and joints.
Triplicate thumb xrays

Additional xrays for triplicate thumb

The principles for treating the triplicate thumb are to create a stable, well aligned thumb.  The thumb's main role is to serve as a post and if stable and well- aligned, it can do just that.  Ideally, we would love to have motion at the joints but motion is not mandatory for a great thumb.  So, in reconstructing these three thumbs- the goal is to create the best single thumb.  This requires excision of some parts, straightening of some bones, stabilizing joints (with new ligaments), and careful treatment of the skin and soft tissues (cosmetic surgery).

Here are images at about 3 months after surgery for the triplicate thumb. The child is starting to use his new thumb and will, over time, use it more and more.  The thumb is stable.  It is reasonably straight although not perfect.  And he does have some motion.  It may be that he will benefit from additional surgery down the road.  If so, we try to perform that second surgery before kindergarden.  Such surgery is needed in approximately 1/3 typical radial polydactyly patients but in notably more triplicate thumb patients like this one.

Top view of thumb after reconstruction for triplicate thumb

Palm view after triplicate thumb reconstruction

Charles A. Goldfarb, MD
My Bio at Washington University

Sunday, April 15, 2018

Mission Trip

I wanted to share the experience of my first Mission Trip.  I went with the World Pediatric Project (WPP), a wonderful organization with which I have a long relationship.  I have cared for many of their patients in the US but have not traveled with them (or any other organization).  Their website, which can be accessed HERE, tells the organization's story.  Two teams traveled together, our upper extremity team (see pictures) and the lower extremity team. 
WPP Mission Trip to St. Vincent.  Our two teams at Milton Cato Memorial Hospital

The Upper Extremity Team with Tina, Valerie, and Brinkley in front and Dave and I in the back.

Dr Gordon, my Washington University partner at St Louis Childrens Hospital and St Louis Shriners Hospital, has led many teams to Mission Trips including a number to St Vincents.  He led this trip and the Lower Extremity Team. 

This map highlights the Southern Caribbean islands.

While many of the pictured islands are tourist destinations, access to medical care, especially complex orthopedic care, can be limited.  The WPP has a number of people in the Southern Caribbean on various islands to help increase awareness and facilitate the care of kids with challenging orthopedic issues.

For several months prior to the trip, I reviewed emails, images and videos of potential patients with two goals.  First, we want to have a reasonable understanding of whether we can help kids in advance and second, we want to understand what supplies may be helpful.  We brought most of the supplies that we would need and, thankfully, planning was successful in this regard.  I should also note that this trip was not just about surgery.  Valerie, an outstanding hand therapist, was a key part of the team treating kids that did not need surgery and providing splints to surgical and nonsurgical patients.

The two team saw more than 120 patients on Sunday and then operated the rest of the week.  The Upper Extremity Team saw 40 children on Sunday and performed 18 total surgeries on Monday, Tuesday, and Wednesday.  We treated children with cerebral palsy, arthrogryposis, birth anomalies, and brachial plexus palsy.  Each day was really busy but the local team (nurses, coordinators, hospital staff, etc) and our team worked so well together.

Clinic day on Sunday.  The waiting room was in the Courtyard of the Hospital.  We were able to evaluate 120+ children.

The Upper Extremity surgery team.

Part of our two surgery teams with one visiting surgeon from a nearby island.

The WPP local team who are one key to success.  Lucianne and Jackie are pictured here with Brinkley and myself.
Here are a few of the patients that we had a chance to evaluate and treat.
Sprengels Deformity

A growth arrest of the distal ulna

Birth brachial plexus palsy

This was a really great experience for all of us.  It was a privilege to take this trip and bring our experience to an area in which there is a need.  I would like to express my gratitude to the WPP and to the American Society for Surgery of the Hand and the Touching Hands Project (THP) which provided financial and other support for this trip!

Charles A. Goldfarb, MD
My Bio at Washington University

Saturday, February 17, 2018

Ulnar Deficiency Follow Up

Hand surgery is a great subspecialty.  I am able to interact and treat many patients and I am rewarded in seeing my patients get better.  However, there is a downside which is that I rarely follow patients long term.  Typically, I am able to see them, provide treatment (whether that be therapy, injection, or surgery) and watch them improve.  And then, typically, I only see them back if another issue arises.

The wonderful aspect of treating kids with birth anomalies is the fact that I am able to follow these children over many years.  We often follow kids through the end of their skeletal growth (i.e., through skeletal maturity).  I am able to develop relationships with the children and their families, understand the real impact of the birth anomaly on the patient, and help families adjust and adapt as needed over the years.

Many years ago, I posted on a child with ulnar deficiency with excellent function: Original Post.  She recently returned and let me know how excited she was to see herself on the blog.  I wanted to update her status with new pictures and x- rays from an 8 year old visit.   She has ulnar deficiency and continues to do well.  This was a routine follow up and there are no major issues.  She functions at a high level.  The elbow is positioned in some flexion, the finger motion is satisfactory and her outstanding shoulder motion helps to compensate for her ulnar deficiency.  This was another gratifying long term follow up visit with a patient who is doing great with ulnar deficiency.

8 year old with left ulnar deficiency and high function.

8 year old with left ulnar deficiency and high function.  She has outstanding shoulder motion.

8 year old with left ulnar deficiency and high function.  She has outstanding shoulder internal rotation motion.

8 year old with left ulnar deficiency and high function.  She can easily get her hand to her mouth but with notable shoulder motion help.

Type 2 Ulnar Deficiency and fusion of the radius to the humerus.

Type 2 Ulnar Deficiency and fusion of the radius to the humerus.
 Charles A. Goldfarb, MD
My Bio at Washington University

Sunday, January 28, 2018

Surgery for Camptodactyly

Camptodactyly is the bent (flexed) position of a finger.  I have previously blogged about camptodactyly HERE, but wanted to share my updated surgical strategy.

Surgery is considered for camptodactyly when therapy is not expected to be effective or if therapy has failed to lead to improvement.  It is absolutely my preference to always begin camptodactyly treatment with therapy.  That includes passive stretching as well as splinting.  Splints may include resting splints to stretch the finger (s) at night as well as splints that are spring loaded to wear for shorter periods of time ( LMB link).  For some patients, the initial deformity- that is the severity of the flexion position- is too much to allow effective splinting.  And, for others, splints simply do not make enough of a difference in straightening the finger(s).  In these cases, surgery is considered.  The primary goal of surgery for camptodactyly is to release the tight structures on the palm side of fingers- these structures are different for each patient but typically include skin, tendons, muscles, and possibly the joint (PIP joint).  Only rarely is it necessary to strengthen the tendons which straighten the finger.

A treatment algorithm has evolved based on the treatment of many children with camptodactyly.  While a variety of different anatomical structures have been related to camptodactyly, in our experience, four surgical steps are key to a satisfactory correction.

1) Address the lack of skin on the palm side of the joint.  There is almost always a lack of skin which means that once the other issues are corrected, there will be a skin deficit.  I do not believe skin grafts are the best treatment option and instead favor a rotation flap.  The use of skin from the side of the finger to rotate over the palm side of the joint has been very effective for our patients.  See pictures below. 

2) The tight flexor digitorum superficialis (FDS) tendon is a part of the bent posture of the finger.  The other finger flexor, the FDP is expected to be present and, therefore, we typically release the FDS tendon.  This removes one major contributor the finger flexion position.

3) The PIP joint itself is tight in the flexed position in patients with a more severe camptodactyly.  While a formal joint release (or volar plate release) is sometimes required, we have had success with joint manipulation as a means to straighten the joint.  This requires less surgical dissection and allows a faster procedure and, I believe, a faster recovery.

4) I try to avoid pinning the joint.  We have used kirshner wires to pin the joint temporarily in a straight position.  I have found benefit to avoiding the pins and simply casting/ splinting the joint for 2 weeks after surgery.

Here are a few pictures, before and after surgery, of a recent patient with all 4 fingers affected.  By definition, this patient has a distal arthrogryposis. 
4 finger camptodactyly

4 finger camptodactyly with a lack of skin on the palmar side.

After surgery for camptodactyly, the skin flaps are visible and the fingers straighten.

After surgery for camptodactyly, the skin flaps are visible and the fingers straighten.

After surgery for camptodactyly, the skin flaps are visible and the fingers straighten.
Charles A. Goldfarb, MD
My Bio at Washington University

Monday, January 1, 2018

Macrodactyly, Syndactyly, and Keloid

Macrodactyly is very rare, even at centers such as ours that evaluate and treat many kids with birth anomalies of the upper extremity.  Macrodactyly is a difficult condition to research as there is so much variability in presentation- fingers may be dramatically large, moderately large, or only slightly larger than the expected finger size.   Treatment varies depending on the age of the child and the severity of the macrodactyly.  This manuscript nicely summarizes treatment strategies.

Excellent review on macrodactyly treatment

Keloid is the prominent thickening of scar after trauma or a surgical incision.  It is more often seen in dark skinned patients and is common on the torso.  If a patient demonstrates keloid formation at the site of one scar/ surgery, the patient is more likely to demonstrate keloid formation at the next surgery site.  Treatment of macrodactyly carries a risk of keloid formation.  Syndactyly reconstruction in patients with enlarged digits likewise carries a risk of keloid formation.  The medication Methotrexate has been suggested as a means to treat keloid formation, typically at the time of a revision surgery.  Two manuscripts have reviewed this issue and the use of methotrexate:

Here are two examples of keloid formation after treatment of syndactyly patients.  In the first, there was a clear, dramatic macrodactyly of the middle finger with syndactyly to an unaffected ring finger.  The family requested reconstruction of the syndactyly.  Scarring is prominent.

Macrodactyly and syndactyly

Thickened scars on both fingers after syndactyly reconstruction and debulking of macrodactyly.

Thickened scars on both fingers after syndactyly reconstruction and debulking of macrodactyly.

In the second case, the digit enlargement is much more subtle.  But there is likely a degree of digit enlargement associated with prominent scarring after syndactyly reconstruction.

Syndactyly preoperative.
Prominent scarring after syndactyly reconstruction

A more detailed view of scarring after syndactyly reconstruction.
Revision treatment of both of these patients is an option.  At the time of revision surgery, methotrexate would be considered and a lengthy discussion with the family will be important regarding the risks and benefits of this medication.

Charles A. Goldfarb, MD
My Bio at Washington University

Travel and News

Happy 2018!  I hope it is a great one for you and your family.

Well, I already have one resolution for 2018- blog more consistently.  I am not meeting my goals and hope to do better in this new year.

A few things to share.

I had a wonderful trip to Japan as guest speaker for the Japanese Pediatric Orthopedic Society.  I spent 3 days in Tokyo at the meeting and exploring the city.  My hosts, pictured below, were wonderful and I thoroughly enjoyed the exchange of knowledge and culture.  I had the privilege of speaking on arthrogryposis.  I shared our philosophies for the care of the arthrogrypotic child including our assessment and treatment strategies.

My host and the President of the JPOA, Dr Takayama and his amazing wife.
Catching up with old friends, Hide and Emiko, who I see almost each year at our study group, CHASG.

An absolutely amazing dinner hosted by Professor Takayama!  Many courses of delicious food and even better conversation.  

There have been a few news stories that caught my eye in the later half of 2017.  Today, a major college football bowl day, is a good time to share one such story.  Troy Fumagalli is a talented tight end for the University of Wisconsin.  He is likely to be drafted as a high pick in the upcoming NFL Draft and he is reported to have "great hands".  He is also an amniotic constriction band patient who is missing the index finger on his left hand!  His early life story will sounds familiar to many of you.  His confidence and determination should inspire all of us.
Fumagalli link
Second Fumagalli link

One of the reasons that I love what I do is the interactions I have with amazing children with birth differences of the upper extremity and their families.  Their positivity and hope sends a strong message for me every day.

I am very much looking forward to the new year.  As with each preceding year, it will bring many opportunities to make a positive impact.  Exciting travels await as well!  Stay tuned!

Charles A. Goldfarb, MD
My Bio at Washington University