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
congenitalhand@wudosis.wustl.edu



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
congenitalhand@wudosis.wustl.edu

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
congenitalhand@wustl.edu

Saturday, October 14, 2017

The 5 Finger Hand

The 5 Finger Hand is a rare congenital hand difference which I have written about in this blog once before, HERE..  It is, as it sounds, the presence of 5 fingers, all in the same plane, instead of 4 fingers and a shorter (2 phalanx), palmarly placed thumb.   The challenges of the 5 Finger Hand include
- a lack of a full first web space which is so important for large object grasp
- a finger with its extra length instead of the thumb
- a notable appearance difference

These images show a 5 Finger Hand with a small extra thumb.

The 5 Finger Hand with a small extra thumb

The 5 Finger Hand with a small extra thumb
When I consider the 5 Finger Hand, two other diagnoses come to mind.  First is the mirror hand as I have written about HERE.  This diagnosis is clearly different but the extra finger and the general hand appearance and the surgical strategies for reconstruction all make me consider the two diagnoses in a similar fashion.  And second, the thumb in the plane of the hand.  I have previously shared my thoughts in this blog specifically around ulnar deficiency and the thumb in the plane of the hand/ fingers HERE.  We have also shared our experience in the medical literature HERE.

The treatment for the 5 Finger Hand is surgical reconstruction for functional reasons and also for appearance considerations.  We typically prefer a pollicization for our reconstruction.  Most commonly, when we discuss pollicization- as we have in these Pollicization links, we do so for treatment of radial deficiency and the hypoplastic thumb.  This is clearly a different surgical indication but the surgery itself is quite similar.  In the child demonstrated above with a 5 Finger Hand, we excised the small extra thumb and pollicized the 1st finger.

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



Friday, October 13, 2017

Hand Camp 2017

One of the highlights of my year is Hand Camp.  Each year (see previous posts), I have shared a brief post but for those of you without a Hand Camp experience, I will briefly describe.  This is a camp run by the Shriners Hospital team including the occupational therapists, recreational therapists, social workers and nurses.  Planning is a year long process and Camp Lakewood in Potosi, Missouri has been our camp site each year.  Approximately 15 families (including the child with a limb difference, parent(s), siblings) join with junior counselor, staff and team members to create an amazing weekend of learning, bonding, and growth.  Hand Camp allows kids to see and interact with others with birth differences that may be similar to their own.  It allows kids and families to interact with our amazing group of junior counselor who have successfully navigated childhood and adolescence and have such wonderful stories and experiences to share.  And it allows all to participate in great camp activities including horseback riding, archery, rock wall climbing, and so much more. 

There are so many reasons that I enjoy Hand Camp.  A few include:
- Seeing kids and families outside of the hospital environment
- Watching kids with notable hand differences accomplish so much (i.e, climbing the rock wall)
- Seeing kids and junior counselors interact
- Listening to the junior counselors as they share a lifetime of wisdom with parents of younger campers.  These lessons can be very emotional but the experience is incredible.
- Watching kids bond. 
Two amazing kids who met and bonded at Hand Camp.

A more spontaneous and 'natural picture'.  The girls share an a difference with ulnar deficiency of the left arm.

Hand Camp 2017 was a success.  Already looking forward to next year.

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

Saturday, August 26, 2017

Cleidocranial Dysplasia


Cleidocranial dysplasia is a rare birth condition most notable for the lack of collarbones (or small collarbones- clavicles).  While this is recognizable, other findings include abnormalities of the teeth as well as delaying closing of the fontanelles (skull bone deficits).  This is an autosomal dominant condition related to the RUNX2 gene but importantly, it is often the result of a new mutation.  More information on the gene can be found HERE.  Genetic testing can help the diagnosis of cleidocranial dysplasia but may not be necessary.  The identified gene is also known as the CBFA1 gene located on the short arm of chromosome 6.

A few other sites provide good summaries of cleidocranial dysplasia including the rarediseases site through the NIH, the NORD site, and OMIM. 

From an orthopedic surgeon standpoint, the lack of the clavicle or collarbones is the most notable and unusual finding.  This provides dramatic shoulder mobility but does not specifically cause problems with daily function.  Therefore, surgery or other intervention is not required.  And this is different than pseudoarthrosis of the clavicle- a condition on only one side (vs both sides with cleidocranial dysplasia) and one that may benefit from surgery. I have previously blogged on it HERE. 

The images of a patient with cleidocranial dysplasia without clavicles are dramatic- demonstrating the mobility of the shoulders seen below.

Patient with Cleidocranial dysplasia which is not immediately noticeable.

Patient with Cleidocranial dysplasia showing dramatic flexibility.

Patient with Cleidocranial dysplasia.

Patient with Cleidocranial dysplasia without obvious difference from behind.


Cleidocranial dysplasia x-ray demonstrating absent clavicles

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


Wednesday, August 23, 2017

Hypoplastic Thumb Reconstruction


Patients with a small thumb- a hypoplastic thumb- often have functional limitations due to limited muscle development and a lack of joint stability.  Therapy is always the first line of treatment but is often unsuccessful with this anomaly.  We therefore often proceed with surgical reconstruction.  I have previously blogged about the surgical choices and recently posted a video of function after surgery.  In this post, I wanted to share anoth brief video on what a successful surgical result look like, at the early time point of 2 months.  This patient was already thrilled with his thumb motion and strength and demonstrates excellent motion of the thumb.  As you watch the video, note the surgical scars (still maturing) as well as the lack of muscle around the thumb (defining the hypoplastic thumb).






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