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
Email me: congenitalhand@wudosis.wustl.edu

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
Email me: congenitalhand@wudosis.wustl.edu








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
Email me: congenitalhand@wudosis.wustl.edu







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

Sunday, September 23, 2018

Radioulnar Synostosis

Radioulnar synostosis is the presence of a bony connection between the radius and ulna.  I have previously blogged a few times on it HERE.  While a synostosis can develop after a trauma, we typically discuss it when present from birth.  The synostosis prevents forearm rotation but does not affect elbow or wrist motion (these are typically normal).  There have been many attempts to 'cure' radioulnar synostosis to restore forearm rotation, all without full success.  In 2018, we typically treat radioulnar synostosis if the forearm is stuck in a position of significant palm down (pronation) or palm up (supination).  We also tend to consider surgery more frequently when both arms are involved.

Surgery for radioulnar synostosis tends to be an osteotomy or a cutting of the bone and rotating the forearm to a more functional position, close to the clapping position ('neutral').  Usually positioned in slightly palm down position due to the importance of keyboarding and tabletop activities.  I like this surgery as patients and families are happy with it.  BUT, surgery is not always necessary because patients have a great ability to compensate.  This video is a great demonstration of a patient with radioulnar synostosis who appears to be rotating the forearms but really is just rotating through the wrist joints.


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

Thursday, August 16, 2018

Unusual "Extra" Finger

I have posted many times on polydactyly- both on the thumb side and the pinky side- as can be seen HERE.  The following patient demonstrates an unusual presentation of what initial appears to be an extra finger, an ulnar polydactyly.  But, as a quick 'count' confirms, there are only 4 fingers with a thumb.


Ulnar deficiency with apparent polydactyly

Ulnar deficiency with apparent polydactyly, palmar view
The xrays are very helpful in understanding this patient's hand.  There is one thumb and four fingers BUT, there are only 4 metacarpal bones in the hand instead of the normal 5.  This is an ulnar deficiency of the hand as demonstrated by the lack of a fifth metacarpal.  The 4th metacarpal is wider than normal.  I have written about this uncommon form of ulnar deficiency HERE.  This is a scientific article sharing our experience.
Ulnar deficiency affecting the hand.

Occasionally the 4th metacarpal is wide enough to allow a surgery to split it to support both the ring finger and the pinky finger.  In this case, however, I did not feel that the metacarpal was wide enough to consider such a reconstruction.  

In addition, in this patient, the pinky finger was tethering or limiting the more normal ring finger, decreasing its motion and causing deformity.  The patient's family was initially hesitant to excise the pinky finger.  It is always difficult to make this decision despite the deformity.  With time, the limitations caused by the pinky finger to both function and appearance were appreciated and the family requested surgery to remove the finger.  Here are pictures and xrays only 6 weeks after surgery.  Function and appearance are excellent and the patient and family are both very pleased.  The function (motion and alignment) of the ring finger are much improved. 

After surgery for pinky excision in ulnar deficiency.  (The picture is a little hard to interpret because the index finger is bent).


Xray after pinky finger excision in ulnar deficiency.

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

Sunday, August 12, 2018

Function in Radial Deficiency

Radial longitudinal deficiency is both a functional challenge and an appearance issue.  Different surgeons have developed different strategies based on these challenges and the lack of a 'perfect' surgery.  My philosophy is to consider different surgeries, based on the child.  I will consider centralization (typically with a period of external fixator distraction prior to the centralization), a release with bilobed flap, or occasionally a lengthening of the radius.  And, sometimes, even with a deficient radius, surgery on the wrist may not be helpful.  The pollicization procedure is one of my favorites because it is so effective and so helpful.

This is a patient with a severe radial deficiency on the right (treated with external fixator, centralization, and pollicization).   Note the 90 degree deformity of the wrist.  The patient had a milder deficiency on the left (treated with pollicization).  This is an image before surgery on the right.
Severe radial deficiency with the wrist at about 90 degrees from the forearm. Note the short forearm and the lack of a thumb.


Here is an image of both hands after surgery on the left wrist (note how much straighter it is compared to the picture above) and the creation of both thumbs with pollicizations.




This first video gives some indication of the outcome of her pollicizations.  Remember- the pollicization helps in a few major ways including fine pinch (such as beads, in this video) and larger object grasp (second video).  The right hand and wrist were severely affected from birth and while she is doing well after surgery, she still has limitations.  You can see how well her left hand and new left thumb work (after pollicization).  However, on the right, the new thumb is not as strong or effective and she sometimes uses  the ring and small fingers (called prehensile function).  This may further change over time to favor use of the new thumb. 




This next video demonstrates several important findings.  First, the right side is weaker and she clearly, she prefers the left hand (which has only had the pollicization).  The left side in general (and the left thumb in particular) is stronger and more stable.  The right hand is helpful to her as is its pollicized digit BUT, when she really tries to use the right hand and the pollicized digit does not help, she switches to try the ring and small finger for grip (that is reflective of her prehensile grasp which never quite goes away).  


I hope these videos are helpful in understanding outcome with severe radial deficiency.

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

Saturday, July 21, 2018

Trigger Thumb and Fingers

Trigger thumb is one of the more common reasons for kids to present to my office.  While uncommon in the general population, for hand surgeons who treat children, trigger thumb is quite common.  And, thankfully, kids do quite well with this condition.  Some will improve on their own without surgical intervention and others require surgery and do well.   I have other posts on this topic which can be found HERE.  Most trigger thumbs in kids are locked (the thumb is stuck in a position of flexion).

Surgery is quite reliable for trigger thumb.  95% or more are cured with a straightforward, 5- minute surgery.  My personal protocol is a small, 1-cm incision closed with dissolving stitches.  We put numbing medicine in at the time of surgery and most kids never require pain medication.  The most common complication is a superficial infection treated with antibiotics by mouth.  Other complications are incredibly uncommon.

Unfortunately, there can be a less than perfect outcome.  This patient is a 5 year old female who was treated surgically for bilateral trigger thumbs at another hospital.  Unfortunately, her symptoms did not improve.  This video demonstrates that her thumb catches when she tries to bend it.  We performed a revision surgery to correct the residual catching.

Pediatric Trigger Thumb


Trigger finger is much less common compared to trigger thumb.  It can be helped with therapy and splinting but occasionally surgery is required.  This video shows the finger catching with bending.  While treating a trigger thumb in a child or adult requires a similar surgery, trigger finger surgery can be quite different.  A pediatric trigger finger surgery can be more complex and requires a step-wise approach to care.
Pediatric Trigger Finger Video demonstrating catching.




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

Sunday, July 15, 2018

Extra Thumb Reconstruction

I have posted a number of times on radial polydactyly- aka split thumb, extra thumb, etc- HERE.  This is a common birth anomaly and the decision for surgery is usually straightforward.  Sometimes surgery is also straightforward while other times the reconstruction can be quite challenging.  And, about one out of three patients with radial polydactyly will need a second surgery at some point down the road.

I wanted to briefly share images of one patient who recently came back for repeat assessment after reconstruction for a somewhat complex radial polydactyly reconstruction.  Here is one picture before surgery in the clinic.  Note that the inner thumb is larger, clearly the dominant thumb.

Radial polydactyly

Here are other pictures before surgery from top and bottom.
Radial polydactyly from the palm view.

Radial polydactyly from top view

In surgery, we removed the outer, smaller thumb and realigned the remaining thumb with a cutting of the bone.  We also created a new ligament to support the thumb.  The metal pin is left in place for about 6 weeks with a cast.

The thumb after reconstruction for radial polydactyly.

Another view after reconstruction for radial polydactyly

Here is the patient/ thumb about 6 months after surgery.  He has fully incorporated the thumb into daily use. The thumb is stable and has reasonable motion.  Importantly, despite a very successful surgery for radial polydactyly, when we compare the thumbs, the smaller size of the new thumb is clear.  That is why some of us prefer the term 'split thumb' which emphasizes that even after surgery, the thumb will be smaller.  However, the thumbs will typically look great and unless directly compared as in the pictures, this size difference does not affect function and does not dramatically affect appearance.
Smaller thumb after radial polydactyly reconstruction.

Thumb after reconstruction for radial polydactyly

I typically follow patients for a few years after surgery to assure no early problems develop and, of course, welcome families to come back anytime if issues are noted.  Overall, reconstruction for radial polydactyly is usually a very successful surgery providing a highly functional thumb which works well and looks near normal.

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



Saturday, June 16, 2018

Families, Choices, and Untreated Triphalangeal Thumbs

One of the benefits to my taking care of kids with birth anomalies of the upper extremity is the opportunity to really come to know the patient and the family, often over many years.  This is magnified when more than one child is affected.  This is one of the best things about my practice (and one of the limitations of the other parts of my practice)- that is treating a patient and family over years.  I really enjoy watching kids grow, learning about their development, and understanding how their birth anomaly does or does not affect their lives.

Another interesting opportunity is talking to and understanding the experience of an affected parent.  This is critically important because the experience of the affected parent influences their goals and hopes for their child.  It also usually moderates their concerns.  What I mean by this is that most parents who are also affected understand that their child is likely to be highly functional and also likely to be well adjusted

Kids with birth anomalies never cease to amaze us and it takes time for 'new' or unaffected parents to realize just how well their child will do and how many expectations will be exceeded.  Affected parents simply better understand their child's status and have appropriate expectations for the future.  

One particular family has become particularly special to me as I have come to know the whole family and done so over many years.   Mom and the kids have triphalangeal thumbs together with an extra thumb.  Mom's triphalangeal thumbs were untreated (although the extra thumbs were removed when she was a child) and she understands the diagnosis and its implications very well.  We have performed surgery on her kids due, at least in part, to mom's understanding of how surgery would be helpful.

A few features to take note of.  
1) The thumbs are long.  This is due to the extra bone in each of the thumbs (triphalangeal- 3 phalanges instead of the normal thumb with 2 phalangers).  
2) The thumbs appear thin, almost like a finger.  
3) The thumbs do not rest in the normal position.  The thumbs are more in the plane of the fingers- some might call this a 5 fingered hand rather than a hand with 4 fingers and a thumb.

Adult with triphalangeal thumbs.

Adult with triphalangeal thumbs, palm side

Adult with triphalangeal thumbs, thumb flexion.  Note the length.

Adult with triphalangeal thumbs. Note that the thumbs are in the plane of the fingers.

Adult with triphalangeal thumbs, right
Adult with triphalangeal thumb, left























Nonetheless, the patient has very good function.  There are a few challenges with fine motor skills and pinch.  This video shows thumb motion and gives a sense of function.






A few relevant blog posts:

The bottom line is that patients with a triphalangeal thumb function well with or without surgery.  However, surgery does offer improved function and appearance as confirmed by this family and the choices they have made.

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








Sunday, June 10, 2018

Three thumbs

Radial polydactyly (duplicated thumb) is an uncommon condition in the general population- I have blogged about kids with extra thumbs a number of times.  Those posts can be found HERE.  Extra thumbs come in a wide variety of patterns, some of which are captured in the Flatt Classification (previously called the Wassel Classification) as shown in the this picture from orthobullets.com.
Flatt Classification for radial polydactyly from orthobullets.com


Yet, this classification does not capture the extra thumbs in all kids with radial polydactyly.  Many simply do not fit this pattern.  As a result, the Rotterdam (in the Netherlands) group added a more complex classification system which is much more comprehensive.  The publication abstract can be found HERE.  It includes more specific patterns for triphalangeal thumbs (longer thumbs, more like a finger), triplicate thumbs (three thumbs, rather than the more common 2 thumbs), as well as adjustments for stiffness, deviation, and small size). 

This is one example of a triplicate thumb.  These are uncommon (even compared to the uncommon radial polydactyly). 
Triplicate thumb

Triplicate thumb, another view

Final view of triplicate thumb

And here are a few x- rays which show the complexity.  This is a complex arrangement of bones and joints.
Triplicate thumb xrays

Additional xrays for triplicate thumb

The principles for treating the triplicate thumb are to create a stable, well aligned thumb.  The thumb's main role is to serve as a post and if stable and well- aligned, it can do just that.  Ideally, we would love to have motion at the joints but motion is not mandatory for a great thumb.  So, in reconstructing these three thumbs- the goal is to create the best single thumb.  This requires excision of some parts, straightening of some bones, stabilizing joints (with new ligaments), and careful treatment of the skin and soft tissues (cosmetic surgery).

Here are images at about 3 months after surgery for the triplicate thumb. The child is starting to use his new thumb and will, over time, use it more and more.  The thumb is stable.  It is reasonably straight although not perfect.  And he does have some motion.  It may be that he will benefit from additional surgery down the road.  If so, we try to perform that second surgery before kindergarden.  Such surgery is needed in approximately 1/3 typical radial polydactyly patients but in notably more triplicate thumb patients like this one.

Top view of thumb after reconstruction for triplicate thumb

Palm view after triplicate thumb reconstruction



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