Friday, July 31, 2015

Outcome after Surgery for an Extra Thumb

The condition of a child born with two thumbs has many, many names but most commonly, we call it radial polydactyly, thumb duplication, or split thumb.  While all are utilized, radial polydactyly is the medical term and split thumb is appropriate as both thumbs are smaller than the other thumb.  I have posted about radial polydactyly previously as can be seen HERE.

We have learned over the years that surgical reconstruction of the extra thumb is much more than removing one of the thumbs.  The remaining thumb requires stabilization and often straightening. This can be tricky and, I believe, is best accomplished by a surgeon with experience in treating these problems- really the more experience, the better.  Even then, sometimes a second surgery may be required as the child ages.  This has been discussed over the years but a nice long term follow- up study from Dallas, as detailed HERE, reports on 43 kids at an average follow- up of 17 years.  These late surgeries were for different reasons but a crooked thumb was often the issue.

The surgical goals for radial polydactyly are to
1) Remove the extra thumb
2) Maintain the 'better' thumb.  Typically that is the one closer to the hand but not always
3) Stabilize the thumb (i.e., make sure the ligaments are good)
4) Align the thumb (i.e., make sure the bones are straight)

We typically add a temporary metal pin to protect the surgery during the first 5 weeks when the child is casted.  Cast and pin are removed at the first visit after surgery.

Here is an example of the early outcome after reconstructive surgery for radial polydactyly.  This child was not as complex as some but still a challenge.  One thumb was clearly larger and it was also crooked, requiring straightening of the bone at the time of surgery.  Both the family and I are pleased at this point but we will follow the child over time to assure that the thumb stays straight and functional.

Radial polydactyly before surgery.  Note that the large thumb is somewhat crooked.  

Here is the side view of the thumb after surgery for radial polydactyly.  Not the subtle scar.

Top view of the thumb after radial polydactyly reconstruction.  The thumb is straight.

Charles A. Goldfarb, MD
My Bio at Washington University

Sunday, July 19, 2015

Multiple Hereditary Exostosis- Its Not Always about the X-rays

Multiple hereditary exostosis is an uncommon tumorous condition and is capable of causing significant functional limitations.  I have previously posted on MHE.  While multiple hereditary exostosis in the fingers can cause deformity and limitations of finger flexion (I need to post on this topic), we worry more about the forearm.  The problem is that if the two bones of the forearm don't grow at the same rate, the natural rotation of the forearm may be lost.

However, when the bones don't grow at the same rate, the best techniques for surgical correction in multiple hereditary exostosis is not clear.  Multiple techniques have been described including lengthening the short ulna, correcting angulation of the radius, or 'detethering' (separation) of the radius and ulna growth.  There is no question that these techniques each have a role and make conceptual sense.  And all may allow the x-rays to look better.  But none have shown to clearly improve motion in patients with multiple hereditary exostosis.

And, to make things more confusing- even patients with dramatic findings on x-ray can do very well functionally.  The case below in a 12 year old with multiple hereditary exostosis proves this point. She has no pain, great motion, and no interest in surgery.  Time will determine if her outstanding function continues.

Multiple hereditary exostosis.  Note the radial head dislocation on the left.

Multiple hereditary exostosis.  Great forearm supination.

Multiple hereditary exostosis.  Very good but not perfect forearm rotation on both sides.

Multiple hereditary exostosis.  Right radial head is out of position.

Multiple hereditary exostosis.  Right radial head is dislocated.

Left elbow with radial head dislocation (with great motion and function). In multiple hereditary exostosis.

Right elbow with radial head dislocation although not as notable as the left (with great motion and function). In multiple hereditary exostosis.

Charles A. Goldfarb, MD
My Bio at Washington University

Sunday, July 12, 2015

Pollicization- concerns immediately after surgery

Pollicization, or the creation of a thumb, is a fantastic procedure that creates a hand that is more functional and looks better.  It is certainly a specialized procedure in that most hand surgeons don't perform this surgery.  I do believe that this is a surgery that is best performed by surgeons that do at least several of these each year.  If this is the case, the surgeon is familiar with the procedure, the specific tricks and tips in performing the surgery, and understands what to worry about.  That is, the problems that can happen around the time of surgery and the problems that can appear months or years later.  I have blogged several times about pollicization- read here. 

Here is a recent surgery on pollicization.  This case is interesting to me because the child has a thumb- but it is a really small thumb that is completely unstable (i.e., floppy) and a thumb that she does not use.  In the US, most of congenital hand surgeons agree that this thumb is best treated with a pollicization (in Asia, some surgeons will stabilize this thumb).  However, this is a tough conversation for the family (it is easier to discuss a pollicization when there is no thumb).

Hypoplastic thumb Type 3B

Second view of a hypoplastic thumb, type 3B

Successful pollicization

Second view of successful pollicization
So this child did well in surgery and just after surgery.  The color of the thumb was good.  We carefully watch kids in the recovery room and also typically overnight in the hospital.  We want to make sure the thumb does not turn white (which means not enough blood flow in) or purple (which means that there is not enough blood flow out).  In this case, all looked good.

Mom called 3 days later to say that she was concerned about the color of the thumb and we asked her to bring the patient in to be seen.  The thumb did look slightly darker but not dramatically so.  We loosened the dressing and it looked somewhat better.  

Pollicization position and color of the thumb.

Slightly dark thumb after pollicization.

We elected to continue to carefully watch the patient.  Rarely, in the situation when thumb clearly does not look healthy, a return trip to the operating room is required.  In my experience, that has only very, very rarely been necessary and it has been immediately after pollicization.

Charles A. Goldfarb, MD
My Bio at Washington University

Saturday, June 20, 2015

Finger Masses

I see a number of kids with growths on the fingers.  These growth vary tremendously in size and expectations but these are technically tumors. The good news is that almost all of these finger masses are benign- meaning that they don't spread to other parts of the body.  They are still concerning to the family.  It is, therefore, key to have a good sense of what each mass might be so that can better understand the best treatment.

One such growth is infantile digital fibromatosis.  This is a benign growth- typically a bump on the finger or fingers.  It does not hurt.  Often, infantile digital fibromas are small as depicted in this picture from Medscape:

However, these growths can also be much bigger and concerning.  Here is one dramatic example of an infantile digital fibroma.

Infantile Digital Fibroma

Infantile Digital Fibroma

Here are a couple of links with more pictures of infantile digital fibroma:

The key step in evaluation of these bumps is be evaluated by a physician or surgeon with experience.  If the correct diagnosis of infantile digital fibroma is made, observation is recommended because surgery can lead to the bumps coming back bigger (recurrence).  In the case above, there was a biopsy done to make a diagnosis and the bumps did come back even bigger.  Surgery for infantile digital fibromatosis is not curative and is, therefore, typically avoided.  I must admit, however, that this is difficult to accept for the family AND for the surgeon.  Everyone wants this to be cured but unfortunately, there is no easy answer.  The good news is that over time with observation alone, many of patients with infantile digital fibromatosis will find that the lesions spontaneously get smaller or disappear.

Failla, et al nicely summarized the condition at this LINK.

Charles A. Goldfarb, MD
My Bio at Washington University

Sunday, May 31, 2015

Exciting Times!!

The opening of a new hospital or major facility is unusual in our modern healthcare system.  It requires a great deal of planning, coordination, and plans for growth.  That is why it is so incredibly uncommon and exciting that TWO new pediatric facilities are opening right now in St. Louis.  TWO, both in the first week of June, 2015!

Saint Louis Childrens Hospital is opening a large outpatient facility about 15 miles west of the main St Louis Childrens Hospital.  It is called the CSCC- Childrens Specialty Care Clinic.  It is beautifully done, outside and inside, is 3 stories high and is 140, 000 square feet in size.  It will be the home to specialty clinics (thus, the name) including orthopedics and hand surgery as well as 3 operating rooms.  There will also be some pediatrician offices housed in the building.  We are excited because it will make the St Louis Childrens Hospital team of doctors more accessible for more people.

View from highway of St Louis Childrens Specialty Care Clinic (CSCC).

A recent picture of mine of a the St Louis Childrens Specialty Care Clinic (CSCC) from the west side.

The new St Louis Shriners Hospital is opening Monday June 1st, 2015.  Originally, in 1924, the Shriners opened in St Louis on the Washington University Medical School campus.  40 years later, we moved to Frontenac and now we are moving back to the Medical School Campus.  The practice of medicine has changed a great deal and the needs of the new hospital are different.  The 90,000 square foot, 3 story hospital has far fewer inpatient beds, more space for research, and lots of great space for taking care of patients.
New St Louis Shriners Hospital

New St Louis Shriners Hospital

Entry of the new St Louis Shriners Hospital

While both facilities are beautiful, what really matters is that we now have even better space for our patients- St Louis Childrens Hospital, St Louis Specialty Care Clinic (CSCC) and St Louis Shriners Hospital.  I am lucky to have the chance to work in all 3 facilities and look forward starting this week!

Charles A. Goldfarb, MD
My Bio at Washington University

Saturday, May 30, 2015

Stiff Fingers

Symphalangism is one type of finger stiffness;  I previously written about it here.  I wanted to share a few more thoughts.  In addition to the classification noted in my other post, I often think of symphalangism in terms of whether or not the fingers are short.  Whether the fingers are short or not, symphalangism is quite rare.

Flatt and Wood reported on symphalangism and divided it into 3 types (a long time ago- 1975, in the journal Hand)
- Symphalangism with normal length fingers (i.e. True Symphalangism)
- Symphalangism with short fingers (symbrachydactyly)
- Symphalangism with another syndrome like Aperts

The clinical examination is notable for the lack of motion of one or more of the interphalangeal joints- typically the proximal interphalangeal joint.  It can be one finger or it can be many fingers. Importantly, the normal creases of the fingers are not there in symphalangism.

The arrow is pointing to the top of the PIP joint of the pointer (index) finger.  Note the normal creases which demonstrate that the finger has been moving.
Kids with symphalagism lack the normal joint development and therefore lack motion and therefore lack the creases.  Here is one child with symphalangism affecting both hands.
Symphalangism.  This is the child attempting to make a full fist.  The PIP joints do not flex (bend) much.

The other hand in Symphalangism.  This is the child attempting to make a full fist.  The PIP joints do not flex (bend) much.
Also note the lack of creases on the tops of the fingers.
Careful review of the x- rays show that the PIP joints have not developed in symphalangism.

Lateral view (side view) of the hand and fingers.  The PIP joints have not developed in symphalangism.  The arrow marks the PIP joint of the long finger (middle finger).
Charles A. Goldfarb, MD
My Bio at Washington University

Sunday, May 24, 2015

World Congenital Forum

Once every three years, an international group comes together for approximately 3 days to discuss all things relevant to the care of children born with musculoskeletal differences.  In 2012, it was here in the United States, Dallas Texas to be specific.  In 2015, the 10th World Symposium on Congenital Malformations of the Hand and Upper Limb was held in Rotterdam, The Netherlands.  It also included a precourse on cerebral palsy.  There were more than 45 invited speakers, 50 free paper presentations, 70 posters, and much, much conversation about malformations, treatments, and research.  We were hosted by Christianne van Nieuwenhoven and Steven Hovius, plastic surgeons with a wonderful congenital program at Erasmus University in Rotterdam.  And WOW! it was quite a meeting.

Our hosts.  We had a delightful small group tour on Saturday.  This picture is in front of a statue of Erasmus.

I had the opportunity to present some of our work and enjoyed the back and forth regarding this information from the widely experienced audience:
1) Data from our study of the prevalence of congenital differences based on the New York State Congenital Malformation Registry.
2) Syndactyly
3) Central Synpolydactyly (poster presentation)

As always, there was a great deal of information shared and I learned much from my colleagues from all over the world.

This is my registration badge- I wanted to share the awesome logo.
I could also show you many sites from the beautiful city of Rotterdam, but the bridge is it most notable piece of architecture (at least to me).
Rotterdam's Erasmus Bridge

Thank you again to our hosts, Steven and Christianne.

Charles A. Goldfarb, MD
My Bio at Washington University