Sunday, March 10, 2019

Research Matters

The Paul R. Manske Award for Best Congenital Manuscript is named for my late partner who had such an important impact on the field.  This Award recognizes the most impactful paper related to kids born with hand or arm differences, anomalies, etc.  I am happy to say that our paper was selected for this award by the Pediatric Hand Study Group at the recent annual meeting in Denver:

Bae DS, Canizares MS, Miller PE, Waters PM, Goldfarb CA.  Functional Impact of Congenital Hand Differences: Early Results from the Congenital Upper Limb Differences (CoULD) Registry.  J Hand Surgery, 2018; 43(4):321-330

The paper, Abstract Here, shared the findings from our CoULD Registry regarding function in kids with these birth differences.  Simply put, we found that that these children did have lower upper extremity function scores but had better peer relationships and positive emotional states compared to the normal population.  Really quite amazing findings which are similar to at least one previous report by Ann Nachemson in 2011 which showed positive psychological well being in a smaller but similar group.  I previously blogged about this paper HERE.

This paper is the result of a great deal of hard work from the CoULD Group.  The idea is that we follow children with birth differences or anomalies over time to better understand the effect of time and treatment.  We follow the results of surgery, type of surgery, challenges with surgery, etc but also kids treated with therapy or simple observation.  This group was founded by Don Bae and I to address one of the great challenges in understanding of kids with birth differences- the lack of studies with a sufficient numbers of kids.  So, we began enrolling kids in 2014 and we have added 5 additional sites with a number of other sites set to join.  So far, we have enrolled more than 2,400 children with the 5 most common diagnoses being radial polydactyly, ulnar polydactyly, syndactyly, radioulnar synostosis, and symbrachydactyly.  Most of the 2,400 were malformations of the upper limb or hand plate.  There are now a number of research projects in the works and we are excited to see how these studies will change our understanding of birth anomalies and their effect on children!

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

Saturday, February 23, 2019

Finger Deformity- What happens late?


Clinodactyly, bent finger, may present at birth, in childhood, or with the rapid growth of adolescence.  If the bent finger is mild, it will not limit function and often may be treated with stretching or simply observed.  However, a more notable bent position becomes a functional issue with large object grasp and with simple things like typing.  Treatment begins with therapy including active and passive motion and splinting to decrease the deformity.  And therapy is often very helpful.  However, in some patients, therapy does not provide the desired correction.  Pain in childhood is rare.  However, with more severe camptodactyly, pain can develop over time and, occasionally, arthritis can develop as well.

If the finger position is causing problems, again, this would almost always be functional limitations but could include pain in the older child or adult, surgical intervention is considered.  However, the issue with surgery is that it doesn't always provide the improvement that we desire.  And, it can carry the risk of stiffness.  This bears more discussion.  With camptodactyly, patients can make a full fist- they just cannot completely straighten the finger. Flexion/ making a fist is a crucial function.  If surgery stiffens the finger and full flexion is lost, function can be worse.  While not the point of this post, to minimize the risk (we can't eliminate it) of a loss of flexion, we limit the immobilization after surgery, limit the use of pins, and start early therapy. 

So, with younger patients with camptodactyly, we attempt to remove the block to extension.  And we work hard to maintain full motion.  I have blogged about this previously HERE.  However, an older patient with longstanding camptodactyly may need a different approach.   If the joint degenerates and if arthritis develops, a surgical fusion of the joint in the best functional position makes sense.  This limits motion but puts the finger in the best possible position and removes pain.  And, this raises another controversy- the role of camptodactyly in the development of joint arthritis.  Some believe that the flexed joint position and limited motion increases the risk of joint arthritis.  They also, therefore, recommend early surgery to decrease this risk.  This is not necessarily my approach as I feel this is a decision for the family to make with my role in providing additional information.

Here is a patient without previous treatment and with long standing camptodactyly.  The markedly flexed position of the finger was limiting his function and there was pain.  To address both issues, we fused his joint (no further motion at the middle joint but normal motion at other joints) in a better position of function.  The surgery was successful in removing pain and helping function.  Again, this is a rare option for camptodactyly.

Limited finger extension with camptodactyly.  Long standing problem which has interfered with function.


Long standing camptodactyly with major joint changes and joint arthritis.  This is RARE.


Finger fusion in an adult for treatment of long standing camptodactyly with pain.  Note the improved position of the finger compared to prior to surgery.

Late, painful camptodactyly is rare, thankfully.  This rare case does illustrate important points including the possible development of an arthritis joint and the need for surgery.

We recently wrote a review article for the Journal of Hand Surgery on camptodactyly, focusing on treatment options in kids.  See the highlights in Recent Article


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


Sunday, February 3, 2019

Madelungs Deformity Surgical Technique

I have posted a few times on Madelung Deformity, as may be seen HERE.  While there have been a number of surgical techniques recommended over the years, I believe that the dome osteotomy of the distal radius is the best option.  That procedure was described well in this important MANUSCRIPT.

Below is a brief surgical technique video on the dome osteotomy for Madelung Deformity.   While this is not a truly graphic video (no notable blood loss), it does provide a look inside the forearm and shows how this procedure is performed with drills, wires, and chisels (osteotomes).  What I mean to say is that everyone may not want to watch this!

I use this surgery for Madelung Deformity when patients have pain in the central wrist or radial wrist (thumb size).  That is typically in younger patients (example, 12 year old female) and may be part of a surgery which includes excision of Vickers ligament and physiolysis.  However, in older patients with pain on the pinky side of the wrist (the ulnar side), this may not be the best option- instead, an ulnar shortening osteotomy might be the ideal surgery.




Thanks to Andrew Yee for his expertise in creating this video!


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

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
Email me: congenitalhand@wudosis.wustl.edu

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
Email me: congenitalhand@wudosis.wustl.edu

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