Friday, January 18, 2019

A New Thumb: Early Pollicization Outcome

Pollicization is one of my favorite surgeries for kids born with hand differences.  This child is several months out from the pollicization procedure.  He is using the new thumb as demonstrated in this video and the family (and the surgeon:)) are all very pleased with the appearance of the hand and thumb.  While kids begin use of the new thumb at different time intervals after surgery, this child has incorporated the pollicization rapidly and with great function!

Charles A. Goldfarb, MD
My Bio at Washington University
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Madelungs Deformity- not always a problem

Madelungs Deformity is a common condition in my practice although uncommon in general.  I have previously blogged about it a few times- you can read those posts HERE.  In general, I see patients in my office with Madelungs who have symptoms including pain with activities, limited motion, and wrist deformity.  Most commonly, we see patients in their early teenage years.  Every patient is somewhat different in these complaints and, therefore, a careful conversation with patient and family is vital.  Madelungs Deformity is also genetic and associated with the SHOX gene.  An excellent (somewhat technical) discussion can be found at this NIH site.  Because of the genetics, it can run in families, typically passed from mother to daughter.  It can also be associated with Leri Weill Dyschondrosteosis (a mouthful) which includes Madelungs, short stature and short forearms and thighs.    In those patients with a family condition (ie genetics), we sometimes see patients before there is deformity and before there may even be clear Madelungs on x-ray.  We try to give every family the best advice but these discussions can be tricky as every teenager and adult has different symptoms and so, we can' t predict what will happen.  What I mean is that some patients have notable Madelungs Deformity but no or minimal pain while others are limited by their deformity.  This variability makes it tough for patients and families when we discuss surgical options that have the chance to minimize or prevent the development of the Madelungs Deformity.

Here are my thoughts.
1) Most patient have disease on both sides.  When I treated one side, almost every single patient comes back for surgery on the other side which, to me, implies satisfaction with surgery.
2) The surgery for established Madelungs Deformity cuts and repositions the bone and attempts to re- establish growth plate lengthening.  The surgery for those with early or mild Madelungs (or even those patients predicted to develop Madelungs because of the genetics) is different.  In these cases, we try to release a tether that may contribute or cause the development of Madelungs.
3) Surgery for older patients (adults) is typically related to pain on the ulnar side of the wrist (the pinky side) and for those patients, we shorten the over- long ulna.  This surgery has good success.

Below is an excellent example of one patient with Madelungs Deformity without pain or significant limitations.  She does have mild decreased wrist and forearm motion but is able to participate in all activities as desired.  She presented to my office only after a routine wrist x-ray detected the abnormality and she was referred.  Our discussion included all of the above.  She has notable deformity but only on one side.  Surgery can certainly help the deformity and, I believe, will help decrease the chance of future problems.  But, this is a tough family decision because she does not have current symptoms.  The family understand the issue and will consider how they would like to proceed.

Madelungs Deformity, only side affected.  It is likely associated with a syndrome including short stature and short forearms.

Madelungs Deformity on the left side only.  Not the deformity on the left and the straight wrist on the right.

Madelungs Deformity on the left side only.  Not the deformity on the left and the straight wrist on the right.

X-rays of typical Madelungs Deformity

Charles A. Goldfarb, MD
My Bio at Washington University
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Saturday, December 15, 2018

Myoelectric Prosthetic Training for Kids

It seems clear to me that 3D printed myoelectric prostheses are the future for upper extremity prosthetics in all age group but especially for kids.  These prosthetics can be customized, are developed/ fabricated quickly, can be replaced for a low price, and have so much potential.  I am hoping and expecting real progress over the next five years.  We have been working on this concept for several years, initially supported by biomedical engineering students (BME 401) at Washington University.  My previous blog entries noted HERE show some of the work we have done.  This work also led to a scientific publication HERE.

I have thoroughly enjoyed another opportunity to mentor senior biomedical engineering students at Washington University.  I say mentored which suggests that I was the teacher but, in reality, I am pretty sure they taught me much more than I taught them.  So, this Fall, I worked with Ilan Palte and Stephen Yoffie on their project to address a specific problem we have seen in kids.  Such a pleasure as these two super bright pre-medical students developed with and carried this project. 

The idea is based on this identified need.  When younger patients try to use a myoelectric prosthetic, it may be difficult for them to develop the controlled muscle firing needed for best use of the prosthetic.  Basically, the prosthetic requires them to use and hone muscles which honestly just have not done much for them prior to this 'need'.  The biomedical engineering project has been to develop a tool, in this case a game, to teach anyone better control of muscle firing and muscle control as well as increase muscle endurance.  In 2018, a game best captures the attention and imagination of most, especially kids.  So with a little biomedical engineering know-how which includes 3D printing and programming skill, I wanted to share are two teasers on what has been developed (early).  We hope and plan for this project to continue with refinement over time into something really easy and fun to use.  But the first go around has shown real potential!  There are three different games to help kids train muscles for most effective functional use of 3D printed myoelectric prostheses.  Two of the games are shown below (a bit hard to see- sorry).

Charles A. Goldfarb, MD
My Bio at Washington University
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Saturday, November 24, 2018

Severe Radial Deficiency, Before and After

I have posted numerous times on radial longitudinal deficiency.  The birth anomaly is common in my practice and I have been fortunate to meet many great kids and families with radial deficiency.  A few posts can be seen HERE.

Early in my career, as influenced by my training, most patients with radial deficiency were treated with a centralization procedure.  This procedure can be effective in straightening the deviated wrist but comes with a risk of the complication of shortening of an already shortened forearm (due to pressure on the growth plate of the ulna bone).  Due to this concern, there is now less consensus in treatment and, at least in our practice, each child is considered individually and may be treated with a number of techniques including: observation, centralization, centralization with precentralization distraction, release with bilobed flap, and fusion.  These decisions are affected by severity of the radial deficiency, the function of the fingers, the presence of a thumb, whether both arms are affected, amongst other factors.

I wanted to share somewhat early results in patient with severe radial deficiency, affected bilaterally.  She had limited use of her hands due to positioning and we elected to proceed with distraction and then centralization.  We used a ringed fixator (vs a uniplanar fixator) as the ringer fixator has so much more 'power' to correct and to do so in multiple planes.  Then we centralized the wrist.  The next step will be to create a thumb.  Finally, we will address the other side.

Radial deficiency on the right.  Note the absent radius and marked curvature of the ulna together with the deviated wrist.

Radial deficiency on the left.  Note the absent radius and marked curvature of the ulna together with the deviated wrist.

Clinical pictures of radial deficiency, severe.  Note the absent thumbs on both sides and markedly deviated wrist.

The patient was treated with an external fixator as depicted here.
External fixator in place during distraction before centralization

After centralization, radial deficiency wrist with temporary pin in place.  Note the straight forearm/ wrist.
After centralization, radial deficiency wrist with temporary pin in place. If you look at the middle aspect of the ulna, there is evidence of healing bone where we cut and realigned the ulna.

The patient is now several months after centralization.  Note the difference between the two sides.
The right side with radial deficiency is untreated, the left has undergone centralization.

The right side with radial deficiency is untreated, the left has undergone centralization.

There is much left to offer this child and her family but we are all pleased with the initial step.  Radial deficiency remains a tough challenge but there are good options to improve function and appearance.

Charles A. Goldfarb, MD
My Bio at Washington University
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Tuesday, November 20, 2018

Thumb Web Space in Amniotic Band

Amniotic Constriction Band is a common condition which affects each child differently.  It can lead to syndactyly (joining of fingers), amputations, or deep bands which limit function.  It often affects more than one extremity (ie, hands and feet) making efforts to maximize function really important.  I have posted many times on amniotic band- those posts can be found HERE. 

The thumb web space (also know as thumb- index web space or first web space) is vital for function.  A wide, deep space allows large object grasp.  For example, we often hold a soda can in the first web space whereas we cannot hold it between the fingers. 

Normal large object grasp

If we do not have a nice thumb web space, we often need to use two hands to grab larger objects.  The web space is also limited if the fingers are short- this makes the web space more shallow.

In patients with a tight first web space, surgery often makes sense to maximize the depth and width of this space.  This improves function in a very clear way- making it a very satisfying surgery for patient, family, and surgeon! This can happen in various conditions of the child's hand including arthrogryposis, cerebral palsy, hand burns, small thumbs, and amniotic constriction band.  There are various ways to address this surgically but the basic idea is to deepen the space and thus widen the gap between the thumb and index finger.  This usually requires a flap of skin to be rotated and sometimes a skin graft.

These pictures are of a child with amniotic constriction band.  Note the shortened fingers and narrow first web space.

Amniotic constriction band with tight first web space and short digits

Amniotic constriction band with tight first web space and short digits

After discussions with the family on the option of surgery to deepen the web, the patient was taken to the operating room for rotation flap from the top of the hand to deepen the space.  It has been dramatically helpful even at 3 months after surgery.  He is using the hand more and can more easily grab objects.  Note the flap and large space.

Amniotic constriction band with tight first web space with flap deepening.

Amniotic constriction band with tight first web space with flap deepening.

This patient will be closely watched over the next few years.  The other option we have to increase function is to lengthen the thumb and potentially the index finger.  This serves to deepen the web also.  I have previously written about lengthening HERE.

Charles A. Goldfarb, MD
My Bio at Washington University
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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
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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
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