Saturday, October 27, 2018

Severe Radial Deficiency- No slowing this kid down!


The absolute best part of my job is meeting so many great kids and great families.  What other job allows daily inspiration, with each day being a bit different with a new story, a new set of expectations exceeded, and many, many smiles.

This is the abbreviated story of one amazing child and family with a great attitude and desire to participate in anything and everything.  I will start with a few pictures and then a short video.  The patient has severe, bilateral radial longitudinal deficiency, a common blog post as can be seen through this LINK. 

Radial Deficiency, left hand.   Marked wrist deviation.  There is prominence of the ulna head.

Radial deficiency, left hand.  Note the floppy thumb and straight index finger.

Radial deficiency, right hand.  Marked wrist deviation.  There is prominence of the ulna head.

Right radial deficiency again with floppy thumb.


Radial deficiency xray.  Not wrist deformity/ absent ulna.
Radial deficiency xray.  Not wrist deformity/ absent ulna.




















And here is a video of the radial deficiency patient stringing beads which requires both large object grasp and fine manipulation- both of which can be compromised in radial deficiency. Every patient is different based on wrist motion, thumb presence/ motion, and finger motion.  This patient has marked wrist limitations and deviation, a thumb that does not help, and stiffness of the index and middle fingers.  Function in this patient with radial deficiency is largely from the ring and small fingers.





After lengthy discussions, we planned surgery to improve function without a significant risk of compromise.  This patient was treated with a bilobed flap to improve straightening of the wrist and improve mobility without stiffening the wrist.  This procedure also allows maintenance of finger function in a good position.  By allowing some radial deviation, the ring and small fingers (the key ones for his function) stay well aligned.

Bilobed flap for radial deficiency.  These 3 pictures show the planned flaps.

Bilobed flap for radial deficiency.  These 3 pictures show the planned flaps.

Bilobed flap for radial deficiency.  These 3 pictures show the planned flaps.

After bilobed flap with temporary metal pin.  Not improved alignment of the radial deficiency.

Improved alignment after bilobed flap with flaps rotated and excellent coverage for radial deficiency.

Another view of bilobed flap for radial deficiency.
We are very happy with his outcome.  Here are some videos demonstrating no boundaries and impressive skill!!




                                                    Baseball.  No problem!




                                                   Golf.  Again, no problem!!





                                                   Getting a hit!  No problem.



                                                   And tennis.  Look out!


Talent recognizing talent.  This is so awesome and does not get old!  Watch his throwing motion.

I have a great job.  I get to interact with amazing kids and families.  Just take a look.  I look forward to everything this kid will tackle in life and will always bet on him to succeed.

Charles A. Goldfarb, MD
My Bio at Washington University
Email me: congenitalhand@wudosis.wustl.edu


Wednesday, October 10, 2018

Untreated Cleft Hand

Cleft hand is one of the most notable birth anomalies of the upper extremities.  The appearance is distinctive although function can be really well maintained.  One of the founding fathers of the discipline of hand surgery, Sterling Bunnell, labeled cleft hand, "a functional triumph and a social disaster".

There are other names for cleft hand.  The geneticists call it split hand (often along with split foot).  EEC is a syndrome with cleft hand as a part- ectrodactyly (missing digit) and ectrodermal dysplasia.  It can also be associated with cleft lip and palate.  It may be genetic or random and it may affect one hand, both hands and, especially in genetic conditions, the feet.   We continue to learn more about cleft hand from a genetic standpoint.

There are two classification systems on cleft hand.  My former partner and mentor, Paul Manske, classified cleft hand based on the quality of the thumb web space.  This is relevant due to the need to reconstruct this web space if too tight.  Dr Ogino, a friend who advanced our understanding of cleft hand through lab and patient research, classified patients on the basis of the number of missing digits.  Together, these classification systems really help our understanding of each patient and help us plan treatment.

Families with other members affected may have a different outlook and approach to the evaluation and treatment of cleft hand.  If left alone, children can function well using the cleft for large object grasp no matter the size of the first (thumb- index) webspace.  This picture and video are of a child without functional limitation in a family with others with cleft hand.  The family wishes to avoid surgery for now.

As a surgeon, I know what surgery can offer: improved appearance and a better thumb grasp with enlargement of the first web space.  However, I also understand why every family may not chose surgery.  My role, as I see it, is to share my experience and help guide each family to the best decision for them.  Most of my families would chose surgery for these cleft hands, but not all.

Cleft hand with large cleft and tight first web space bilaterally.




This video shows these same cleft hands with dramatic instability of the index finger MCP joints.  The videos also demonstrate the limitations of the first web space with limited space available for thumb function.  The instability can become an issue with strength and grasp although surgical reconstruction can be helpful.  There can also be instability of the ring finger MCP joints.

Cleft hand is a complex and striking disorder.  Surgery can absolutely be beneficial for the child, for their function, and for their appearance but every decision is family- centered.

Charles A. Goldfarb, MD
My Bio at Washington University
Email me: congenitalhand@wudosis.wustl.edu