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

Sunday, December 3, 2017

Adoption and Radial Polydactyly

In 2015 (the most recent year with statistics available), there were 5647 children adopted to parents in the USA.  About half of those were from China.  See link HERE.

I am fortunate to see many families with newly adopted children.  In addition, I often have the chance to assess x-rays and clinical information for families before their trip overseas.  My review is often helpful for families as I can often provide a 'translation' of the medical information and realistic expectations regarding their child’s function, special needs, and need for additional surgery or therapy. 

Adopted children are brought to see me for one of three reasons:
1) To assess an upper extremity difference that has not been treated
2) To assess an upper extremity difference that has been treated, perhaps well and perhaps in a less than ideal way
3) To generally assess the child.

These kids and families are some of my favorite to treat.  The joy that the families bring and their interest in learning more about their new child’s upper limb difference is powerful.  I also find that parents of an adopted child have a very realistic and practical approach to their child's challenges.  There are added complexities in treating newly adopted kids including the social aspect of the adoption.  The adopted child takes time (variable) to adjust to the new home environment, their new family including new brothers and sisters, etc.  While there may be a notable limb difference, we typically do not rush to treat the patient.  We might institute therapy or splinting but our primary goal is to give the families as much time as needed to limit any additional ‘trauma’ that surgery might bring.  We do recognize that some families are eager to move forward but patience is typically our goal. 

The pictures below are of a newly adopted 4- year old child who was born and treated in China.  It appears that she was born with radial polydactyly (extra thumbs on each hand) and these extra thumbs were treated with excision alone.  While she functions reasonably well, there is an appearance difference on each side.  The left thumb has a big ‘bump’ which can be sore with pressure.  The right side is somewhat stiff and is angled.  Motion is limited on both sides but more so on the right.

Intervention is completely based on the desires of the family. Typically, we give the family and child time (as noted above) to adjust and truly assess how much the deformity is affecting function or quality of life.

Right thumb polydactyly after treatment overseas.

X-rays of right thumb polydactyly after treatment overseas.
Left thumb polydactyly after treatment overseas. Note the bump on the outside of the thumb.

X-rays of left thumb polydactyly after treatment overseas.  See the bump representing the site of the extra thumb removal.

We are embarking on a new study to assess the differences in families of children with birth differences of the upper extremities to help better understand how perceptions vary. We will share our results when we have completely analyzed the data.

 Charles A. Goldfarb, MD
My Bio at Washington University

Saturday, November 11, 2017

Syndactyly Revision Surgery

I enjoy performing surgery to correct syndactyly because it is a reliable procedure with high patient, family, and physician satisfaction.  But, as with any surgery, there are complications and adverse events that can happen in the short term or at longer follow- up.  The risk of complications related to syndactyly surgery is very low.  It includes risks of:

- nerve injury,
- loss of blood supply to the finger,
- infection

Each of these risks is very low especially if the surgery is performed by an experienced surgeon who can take steps to minimize each of these risks.  In our experience, even the risk of infection is lower than the risk in most surgeries.

More challenging in syndactyly reconstruction is the risk of adverse events or less the perfect outcomes.  I have previously posted on some of these in several posts including HERE.  These include:

- thickened scar
- discolored skin grafts
- web creep (the slow advancement of the skin between the fingers after the surgery)
- scar bands limiting finger extension
- fingernail abnormalities

Each of these is a risk no matter the experience of the surgeon.  The characteristics of the patient's syndactyly will affect the outcome of the surgery.  For example, a complex syndactyly (with bone connections) is known to be more difficult to correct and has a greater likelihood of fingernail abnormalities and web creep.  However, the experienced surgeon can take steps to limit each of these risks.  For example, it has been shown that avoiding a transverse incision for the commissural flap will limited skin tension and therefore limit web creep (Citation here). 

This child had complex syndactyly (bony connection and therefore more challenging to reconstruct) which was treated with reconstruction.  It affected the ring/ small finger of both hands.  There is a strong family history but each member of the family was affected differently.  He has had previous reconstruction with a graftless technique on the right and a 'standard' technique on the left with skin grafts (taken from the antecubital crease of the elbow).  While he is generally happy, the patient and his family do not like the creep on the right and the scar band which has developed- this band limits ring finger straightening.  Today, I rarely perform the graftless technique (dorsal commissural flap) utilized on his right hand, for two reasons.  First, I believe in the use of hyalomatrix as previously posted HERE.  And second, in some patients the flap migrates back dorsally and becomes noticeable as demonstrated in these pictures. 

Graftless syndactyly reconstruction.  Note the flap scar which has migrated.

Syndactyly reconstruction using skin grafts.

Palmar view showing a nice deep web on both sides after syndactyly reconstruction.

The right hand syndactyly, treated with a graftless technique, has developed a scar contracture with web creep.

This patient is scheduled for revision syndactyly surgery for the right hand.  We will deepen the web and use a z-plasty to 'break up" the scar band limiting the ring finger straightening.  We expect a very good long term outcome.

Charles A. Goldfarb, MD
My Bio at Washington University

Saturday, October 28, 2017

First Pitch World Series

Hailey Dawson has been in the news over the last few months as she seeks to throw the first pitch in all the Major League Ballparks.  Well, she took a big step tonight on the biggest stage- the World Series!  World Series Story.   What a great story which helps to raise awareness of birth differences of hand and upper extremity and 3D printed prosthetics.

Hailey has symbrachydactyly associated with Polands syndrome.  Symbrachydactyly means short, webbed fingers but it can be very different from patient to patient.  It appears that she has a reasonably well developed thumb and otherwise small digits (monodactyly type).  I have posted a number of times about symbrachydactyly, as can be found HERE.  Included in those links is some information on Poland syndrome as well.  Poland syndrome is the lack of development of a portion of the chest muscle, the pectoralis major muscle.

While I am confident that Haley functions quite well with her normal left hand and a very functional right hand, the 3D printed prosthetic can offer function, appearance, and social benefits as noted in these posts, from my blog.  These 3D printed prosthetics are inexpensive, relatively easy to make, easy to use (depending on something called the tenodesis effect in Hailey's case) and are available in a variety of styles and colors.  There are many examples of these on the web and it seems the UNLV is helping Hailey and her family.  A recent Review Article summarizes the state of 3D printed prosthetics for the upper extremity and cites our experience here in St Louis.  We continue to refine the powered 3D printed hand (so- called myoelectric hand) with the support of the St. Louis Childrens Hospital Foundation.  Here are two of more recent 3D printed prosthetic hands.

3D Printed hand in one of our younger patients

3D printed myoelectric hand developed in St. Louis

Go Hailey!  

Charles A. Goldfarb, MD
My Publication List