Saturday, September 24, 2016

Hand Camp 2016

Once a year, Hand Camp is held in Pitosi, Missouri at Camp Lakewood.  This is a wonderful opportunity for 15 kids with hand and arm differences to come to camp with their entire family (parents, siblings).  The kids get a chance to meet and socialize with kids with similar brith differences but also work with junior counselors who are similarly affected.  This years' junior counselors were all once campers themselves- how exciting!  So the kids have a great chance to socialize and also engage in great activities.  The parents have the chance to meet other parents and learn from each other.  And lastly, there is the camp staff.  The staff is a mixture of nurses, recreational therapists, physical therapists, and hand therapists all spending time away from home and 'working' the camp.  But, the camp is a great experience for them as well- a chance to interact with kids and families away from the hospital and be reminded of how amazing these kids (and their families) can be!

A few pictures of the Hand Camp 2016 Attendees.

Camp Lakewood in Pitosi Missouri

The lake



Hand tracings of some of the kids.

The whole crew including campers, families, junior counselors, and staff at Hand Camp 2016.

Hand Camp 2016 campers and junior counselors.


The Hand Camp 2016 Staff.  All are so generous with time and spirit.

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

Tuesday, September 6, 2016

Choosing Your Child's Surgeon


Choosing a surgeon for your young child with a birth difference of the upper extremity can be challenging, stressful, and confusing.  There is no simple equation to simplify this process but there are a few factors which might help.

Bedside manner.   When a family chooses a surgeon for their child with a birth difference of the upper extremity, a long- term relationship with doctor and his/her team of nurses, assistants, and therapists often develops.   It is crucial that you get along well with the surgeon and his/ her team.  There is no need to put up with a surgeon with a poor bedside manner or a surgeon with whom you have a difficult time communicating.  It is imperative, therefore, that you meet as many times as necessary with the doctor and his/ her team to have your questions answered.  I see many families that have ‘interviewed’ different doctors across the country to find the best fit for their family and their child. This is expensive and time consuming, but it can be very helpful.

Surgeon Background.  I believe that children with congenital hand differences are best served in the care of pediatric hand surgeons.  This means hand surgeons with specific training and expertise in the care of kids and hand surgeons who spend a majority of their time caring for kids.  There are certainly other surgeons that can do a great job in caring for your child, such as hand surgeons that primarily treat adults or pediatric orthopedic surgeons who treat the whole child.  However, it is more difficult as a parent to understand the expertise of doctors in these other groups.  If you are considering such a surgeon, it will be more important that you question the specific experience of these doctors for your child’s specific problem.

A pediatric hand surgeon may be trained in orthopedic surgery or plastic surgery.  As long as they treat kids regularly and have a background and training in caring for kids, they are likely qualified to care for your child.  

I believe surgeon certification can be a helpful piece of information as I believe in the value of a board certified orthopedic or plastic surgeon.   In addition, society membership shows a commitment to continuing education and the physician community.  The two key societies for upper extremity birth differences are the American Society for Surgery of the Hand and the Pediatric Orthopedic Society of North America.  I believe membership in one or both societies is a helpful sign for families.

Surgeon Experience.  This is likely one of the most important factors to consider, but also one that may be the most difficult to assess.  As a parent, you are interested in the surgeon’s overall experience (i.e., volume of cases performed over a lifetime) but you are most interested in their experience with the specific surgery your child requires over the preceding 5 years.  You should ask that specific question.  In my opinion, if a surgeon doesn’t answer or gives a vague answer, this is a red flag suggesting limited experience.  For most uncommon birth difference procedures, if the answer is ‘a few’ or ‘1-2 each year’, it is inadequate to obtain and maintain expertise.   When possible, your child deserves a surgeon who specializes in the problem that he/ she has.  You should avoid a surgeon who only occasionally does the procedure.

Some surgeons are active in the academic world through teaching and writing about uncommon conditions such as upper extremity birth differences.  I believe it is important that all surgeons critically analyze the results of their patients from all surgeries.  I also believe that surgeons who publish their results as scientific papers are more likely to carefully look at patient outcomes and share those results in the medical literature.  Therefore, one other criteria for a parent to consider is publications by a surgeon on a particular topic.  A parent can search for such publications at Pubmed.  This will both provide information on the surgeon and also provide information on the medical problem.

 Other Parents’ Experience.  Families with a child similar to yours may be very helpful in choosing a surgeon.  Such families may have already been through the process of sifting through the limited information out there on different surgeons and certainly will have good information on the surgeon they have chosen for their child.  Most families in this situation are willing and even eager to share their experiences.  However, finding the right family can be tricky- you want a family that you can talk to and relate with.  You want a family whose child has a similar limb difference as your own child.  And ideally, you want a family in your geographical area.  It can be tough to identify such families but Internet resources including Facebook parent groups, parent blogs, online messaging boards, etc. can be helpful.  My blog offers some links which might be helpful.Doctor Rating Sites (Healthgrades, Vitals.com, etc.) provide some information on doctors and surgeons.  While these sites fill a void, the information is far from comprehensive.  These sites provide basic information on surgeons (medical school, training, insurance accepted, etc.) but perhaps most importantly, the sites allow patients to rate the doctor.  However, the ratings are most reflective of bedside manner, not technical skill or outcomes.  There are inevitably a few poor ratings for almost every surgeon with enough reviews.  In my opinion, as long as the majority of ratings and reviews are positive for a particular surgeon, a few poor scores should not rule him/ her out.  Likewise, all positive scores may suggest a pleasing bedside manner but such scores indicate little regarding knowledge, skill, and experience.

Hospital Rating Sites also provide useful information.  The criteria for hospital ratings are more objective than the doctor ratings as they are based on specific criteria such as readmissions, etc.  Hospitals are beginning to pay more attention to these criteria and many are working towards improvement- this is good for patients.  However, it is important to remember that there can be great surgeons at hospitals with lagging scores and poor surgeons at great hospitals. 

Doctor Rating Sites (Healthgrades, Vitals, etc.) provide some good information on doctors and surgeons.  While these sites fill a void, the information is far from comprehensive.  These sites provide basic information on surgeons (medical school, training, insurance accepted, etc.) but perhaps most importantly, the sites allow patients to rate the doctor.  However, the ratings are most reflective of bedside manner, not technical skill or outcomes.  There are inevitably a few poor ratings for almost every surgeon with enough reviews.  In my opinion, as long as the majority of ratings and reviews are positive for a particular surgeon, a few poor scores should not rule him/ her out.  Likewise, all positive scores may suggest a pleasing bedside manner but such scores indicate little regarding knowledge, skill, and experience.

      Choosing a surgeon for your child is a challenge.  I hope that this information can make the process a little bit easier.  Good luck!


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


Sunday, August 28, 2016

Making a More Functional Hand

I have written numerous times in this blog about the importance of improving function for kids born with hand differences.  Function can be made better through therapy, through splinting, or through surgery. Whenever possible, we want to improve appearance as well.  There have been several investigations that found that kids born with hand differences are typically well adjusted, found HERE,  HERE,  and in our investigation to be published soon.  However, it is also clear that improving appearance is important to kids and families.  In a perfect intervention, we would improve both function and appearance.

There may be a times, thankfully not often, when improving function may come at a cost of some worsening of appearance.  If this is a possibility, frank discussions should be held with the family and many will eagerly proceed with an intervention for improved function.

This case is one example.  The child has symbrachydactyly, the cleft type (classification is reviewed in this POST).  He does well overall but has trouble grasping with the hand.

Symbrachydactyly, cleft type, palm view

Symbrachydactyly, cleft type, top view

Symbrachydactyly, cleft type, top view with thumb held
The thumb is of reasonable quality but the pinky is not greatly developed.  When we think of how to improve function in these situation, we need to lengthen the digits to allow the thumb and pinky the ability to pinch or grab.  There are three basic options:
1. Sometimes, lengthening a digit will make sense as described HERE.  But there must be sufficient bone present before we can lengthen.  
2. Other times, transferring a toe or toes to the hand will be the best option to improve function.  While this is a significant surgery for the patient and the whole family, it can be the best option to provide pinch and grasp in kids with few options related to limited anatomy.
3.  Lastly, and perhaps counterintuitively, we can deepen the space between the thumb and pinky.  This does not actually lengthen the digits but it makes them seem and act longer because bigger objects can be grasped.  Again, this may be considered different than some interventions because we usually try to get rid of the cleft, but in this case we deepen it.
This is the same patient after cleft deepening.

Palm view after cleft deepening.

For this particular patient, the seemingly small amount of deepening will (and has) make a notable difference for function.  And as he gets older, lengthening the bones can still be considered.

Functional improvements are always our goal in the care of kids with hand differences.  In this case, a small surgery should really help.

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


Sunday, August 14, 2016

Skin Contracture and Z-plasty Correction

The field of hand surgery was developed around the time of World War II combining the fields of orthopedics (care of bones, joints, tendons, muscle), plastic surgery (care of skin, soft tissues, and nerves) and vascular surgery (care of blood vessels).  Given the very complex anatomy of the hand, wrist, and forearm, the concept of 'hand' surgeons trained with an anatomical focus in the upper extremity with expertise in the areas of orthopedics, plastic surgery, and vascular surgery makes a great deal of sense.  This 'new' field of hand surgery has grown dramatically in the last 60 years.

Skin tightness or contracture is a common finding in the care of children with birth differences.  The most serious skin deficit is syndactyly which, itself, varies in the degree.  Treatment of syndactyly has been a repeated topic of the blog, see posts HERE. 

Z- plasties are typically thought of as 'plastic' surgery procedures but in reality all hand surgeons, and especially those who treat kids, enjoy and utilize these procedures.

Tightness of the first webspace is seen in a number of different birth differences including the hypoplastic thumb, amniotic constriction band, simple or complex syndactyly, and symbrachydactyly.  Treatment varies based on the exact nature of the tight first web space, ranging from a large rotation flap (as described by Buck Gramcko) or z- plasties (skin lengthening through re- arrangement).

Z- plasties are used in any area of the body where there is a skin contracture.  The goal is simple: lengthen a tight band (no matter the cause).  There are different types of z- plasties including 2- flap, 4- flap, and 5- flap (or running man flap).  These flaps all lengthen the skin but with somewhat different benefits and challenges.  The simplest of these is the 2- flap z- plasty for the obvious reason that it only involved two flaps of skin.  These are typically designed at 60 degrees with each limb of equal length.  The four and five flap z-plasties are a bit more complex to design but those who favor these flaps believe they create a more natural appearance of the lengthening.  There is an excellent review of the basic 2 flap z- plasty HERE.

I will use different flaps including the 2 and the 5 flaps z- plasties most frequently.
Here is an example of the 2- flap z-plasty.
Tight first webspace in symbrachydactyly
First webspace after 2-flap z-plasty

Palm view of z- plasty after reconstruction.
This technique creates a nice deepening of the space which is critical to improving large object grasp. I enjoy this operation as it can dramatically improve function with a relatively straightforward surgery- few surgeries provide such a functional improvement.

Another technique is the 4- flap z-plasty.  While I don't want to get too technical, this flap can be created with either 90 or 120 degree basic flaps.  This figure provides an excellent explanation of the 120 degree 4- flap z- plasty.

This video from Bobby Chhabra shows this same concept in practice- Video Link.

 Finally, this image from the fastbleep.com website shows the 4 flap (both types) and 5 flap z- plasty.  The bottom image is the 5- flap z-plasty which is also called the "running man" which combines a 4- flap z-plasty and an advancement flap.

 I have used and will continue to each of these flaps.  Each can make a marked functional improvement for the patient.



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


Sunday, August 7, 2016

Extra Digits: functional and social considerations

Today, children born with extra fingers or toes (polydactyly) are typically treated with excision and reconstruction of the hand or foot.  There are multiple reasons for the surgical approach.

First, the extra digit does not provide a functional advantage. The digits are rarely fully formed or fully functional.  Second, the extra digits can cause problems with daily activities.  The digits can get in the way of the other fingers as there may be deviation of the extra digit.  And, in addition, the extra digit can tether or cause deviation of the larger, more normal digit.  So the extra digit likely does not help function and may actually make it worse.

The extra toes cause the additional problem of interfering with shoe wear.  The extra toes widen the foot and can change loading patterns with walking or running.  While this can be addressed with especially wide shoes, removal of the extra toe may be a more straightforward solution.  Extra fingers cause a similar although less problematic issue- glove wear.  Clearly, wearing gloves may be a challenge or may not be possible with an extra digit.  However, given that in most places, shoes are typically worn and gloves are more optional, this is less of a problem for most patients.

Polydactyly of the feet with deviation of the extra toes.


There are also social considerations with the extra digits.  The extra digits certainly look different and may be noticeable in public.  Interestingly, a missing pinky is typically less noticeable than an extra pinky.  There have been multiple studies on appearance and hand differences.  One such study, whose lead author was Ann Nachemson, found that children with milder birth differences of the hand, such as patients with extra digits, had worse social interaction scores compared to more severely affected kids and 'normal' kids- see citation HERE.   Another study showed that overall health related quality of life measures in children with limb reduction deficiencies were better than kids with other health conditions.  However, 'unexpected attention and perceived physical appearance' affected scores.  Citation.

Recently, there have been a few newsworthy reports regarding extra digits in the ancient world.  The first is a white paper by Richard D. Barnett on "Polydactylism in the Ancient World".  This PAPER  reviews some interesting findings on polydactyly from centuries ago including the importance of which side was affected.  

In addition, there are recent findings from Chaco Canyon, New Mexico from Pueblo culture.  This National Geographic ARTICLE  reviews the findings.  "The findings, published today in American Antiquity, indicate that the society did not view six-toed individuals as supernatural, but this form of polydactyly did grant them exalted status in life and in death.  'We found that people with six toes, especially, were common and seemed to be associated with important ritual structures and high-status objects like turquoise,' says Crown, who is also a past National Geographic grantee."

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

Sunday, July 17, 2016

Rebuilding the Mirror Hand


Mirror hand is an incredibly rare birth difference of the hand and upper extremity.  I have previously blogged about this diagnosis, specifically regarding how limbs form and how birth differences come to be- find the post HERE.  But I have not previously written about the reconstruction or rebuilding of the mirror hand.  And there is very little to be found on a Google search or in the medical literature.

The term mirror hand reflects the appearance of the hand and the duplication of the ulnar half (the non- thumb half) of the hand (such that it can look like a mirror image).  Typically there are 7 or 8 fingers without a thumb.  There can also be a duplication of the wrist bones and the forearm bones (in which case there are two ulna bones without a radius).  I will share more about the forearm and elbow in another post.

What follows is the case of Evan, a young boy with mirror hand.

Mirror hand with 7 fingers. 
Mirror hand with 7 fingers from the palm view.

Mirror hand with 7 fingers from the palm view.
When a child with mirror hand is examined, the use of the hand is important to understand.  The basic reconstruction/ rebuilding strategy is to pollicize (turn a finger into the thumb- see previous blogs on the pollicization HERE, recognizing that these are radial deficiency hands) one of the fingers- the question is- which finger is best to be the thumb?  Sometimes the strategy that makes the most sense is to use the fourth finger which means the hand will have, after reconstruction, 3 fingers and a thumb (which might seem crazy given the child started with 7 or 8 fingers).  In other cases, we can create a 4 finger and thumb hand in the mirror hand reconstruction.  The decision is based on quality of the fingers (size, mobility, and child's use).  This video is instructive as Evan uses the space between the 4th and 5th fingers for function which makes the concept of a 4 finger and thumb hand appropriate.

Once we have decided how to proceed with reconstruction, two procedures are performed.  A pollicization is the most challenging but we also perform a procedure to help the wrist straighten or extend.  The wrist tends to flex because of a lack of the wrist extensor muscles (which is explained by the muscles)- surgery can strengthen the wrist extensors.  We also temporarily pin the wrist to allow healing.  Here are a series of images after the surgery while still in the operating room.


Mirror hand post pollicization.

Mirror hand post pollicization.

Mirror hand post pollicization.

We were very pleased with the pollicization and wrist reconstruction procedure for Evan. The later pictures are even more helpful in understanding Evan's improving function.

Here is Evan shortly after surgery with his friend Owyn.  The kids share the diagnosis of mirror hand and the families have helped one another through the diagnosis and treatment at the St. Louis Shriners Hospital.

Evan and I shortly after surgery.  Note the tape on Evan's new thumb which helps for positioning soon after surgery.

Evan showing off his new thumb after mirror hand reconstruction, approximately 7 weeks after surgery.  Note the pinch with the new thumb.


In this video taken approximately 3 months after surgery, Evan is using the new thumb for pinching the large metal balls.  


In this final video, also taken 3 months after surgery, Evan uses the new thumb for smaller object pinch.


Evan and his family traveled a long distance to come to St. Louis for the care of his mirror hand and he has, thankfully, done wonderfully.  The function (and appearance) of his hand are much improved.  We will follow his elbow (specifically the flexion of the elbow) for possible later reconstruction.  Evan's mom agreed to allow me to post the images and videos in part to allow others with this rare condition to understand treatment options.

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