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
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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
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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
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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
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Tuesday, August 8, 2017

Thumb Reconstruction in Action

My favorite day of the month is our 'big' clinic at the Shriners where my partner and I see patients all day (no surgery).  It is such a pleasure to see new and followup patients with so many different rare conditions.  I learn at each of these clinics because patients show me (and sometimes tell me) what works, what doesn't, and why.  Needless to say, the residents and fellows learn so much as well- it is an unusual experience with rare conditions, great kids and great families.

Today, I had the pleasure of seeing one patient back, about 6 years after reconstruction of his left thumb.  He has hypoplastic thumbs, a topic on which I have posted about several times including HERE and HERE.  Actually both of his thumbs are small with limited muscle but his left side was the problem leading to surgery.  The right side has continued to do well despite a lack of full muscle development.
Underdeveloped thumb, left side had surgery for reconstruction with Huber Opposition.

The left side was reconstructed with a Huber opposition transfer in which a muscle (abductor digiti minimi) is transfer to the thumb.  This is one great option and it used to be my first choice to help the thumb move better.  We recently published our positive experience with this operation in The Journal of Hand Surgery.  These pictures are, to me telling.  The thumb is nicely resting in a position of function.  However, some have observed that it is hard for the patient to flatten the thumb after this procedure.  I have been using a somewhat different surgery over the last few years (the FDS tendon opposition transfer) with very good results also.

Pictures are helpful but video tells the real story.  Watch his 'new' skill.  He is quite talented and his thumbs work amazingly well.

Charles A. Goldfarb, MD
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Saturday, May 20, 2017

Hand Anomalies and Adoption

Adopted Kids and 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.

I am fortunate to see many families with newly adopted children.  It is one of the most enjoyable parts of my practice.  Sometimes I am able to assess the paperwork prior to the actual adoption to provide information for families seeking to better understand the challenges that adoption may bring. To meet these amazing families that have traveled across the world to adopt kids in need is such a pleasure.  These 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 (least common).

I admit that these kids and families are some of my favorite to treat.  The joy the families bring and the interest in learning more about their new child’s upper limb difference is powerful.  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 (possibly new brothers and sisters), etc.  While there may be a notable limb difference, we 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 inflict.  We do recognize that some families are eager to move forward but patience is typically our goal.

This is a newly adopted 4- year old child who was previously treated in China.  I believe that she had radial polydactyly (extra thumbs on each hand) and was treated with excision.  While she functions well, there is an appearance difference on both thumbs and some discomfort when bumped, especially on the left.  The right side is in a better condition- it is somewhat stiff with mild angulation.  Motion is limited on both sides but more so on the right (although there is no pain).

When the time is right, we will consider surgical reconstruction to better align the thumbs.  The right thumb, on top, is better aligned.  The left thumb (below) has the bump which represents a part of the extra thumb which was only partially excised in China.

Charles A. Goldfarb, MD
My Publication List

Media Links

I would like to share a few media links which I have enjoyed.  The first is from the New York Times- LINK- on the 3D printed prosthetic.  Interesting read and highlights some of the information we have also shared: My Blog's 3D printed information.

Additionally, the St Louis Post Dispatch recently shared a great story about one of our patients with a myoelectric 3D printed prosthetic.  We are still working out some minor kinks (related to myoelectric firing of the prosthesis) but she is doing great.  Check it out HERE.

Finally, the Shriners Hospital in St. Louis just opened a new lab, creating an amazing collaboration between the Shriners Hospitals, Washington University School of Medicine, and the Department of Orthopaedic Surgery.  We are fortunate to have Farshid Guilak at the Shriners to direct the lab and the research program.  The new lab is profiled HERE.  Farsh joins Michael Whyte who's fantastic bone research in kids has led to some groundbreaking discoveries on rare diseases and the entire Orthopaedic team.  We believe this multifaceted approach will change orthopaedic research and, through translation discoveries, change orthopaedic care for kids (and adults) over the years to come.

Charles A. Goldfarb, MD
My Publication List