Sunday, January 13, 2013

Amazing People

I will try to share links to interesting people and news stories as I become aware.


Here is a new link.  I do disagree with the diagnosis (amniotic constriction band)- instead, based on what I can see, it looks like symbrachydactyly.

http://www.cnn.com/2013/03/15/tech/innovation/adrian-anantawan-violinist/index.html?iref=allsearch


Here is one such link to an amazing basketball player (near and dear to my heart).

http://www.yardbarker.com/high_school/articles/msn/high_schooler_zach_hodskins_is_a_basketball_sharpshooter_despite_having_just_one_hand_video/12426304


Here is another of a one- handed pianist.  Nicholas McCarthy.

http://www.bbc.co.uk/news/uk-england-surrey-19179499

There are other similarly amazing stories out there- demonstrating the power of the Internet!


Both seems to have symbrachydactyly or transverse arrest.  Inspiring to say the least.

Award

I hope the blog has been helpful for patients and families.  My goal is a weekly post and while I am not always successful at this target, I feel like I have been pretty good at regularly sharing my thoughts.  My efforts on this blog have made me realize how hard people work to regularly post on the web.  The blog has had more than 30,000 page views in approximately the last 12 months and the number of regular visitors seems to be increasing!  All good news.

Also, the blog has won an award!  We were recognized as one of the best blogs for surgery (all types) in 2012.  http://onlinesurgicaltechniciancourses.com/surgery-websites/  That award is the impetus behind the new logo on the main page.



Saturday, January 12, 2013

Clinodactyly



I have previously posted on clinodactyly but recently performed several surgeries so I though adding a few more pictures and thoughts might be helpful.  There are two types of clinodactyly: isolated and associated with a syndrome.  Isolated clinodactyly is common in the general population but is very well tolerated and usually ignored.  In fact, many people are not even aware that they have a clinodactyly, especially if it is mild.  It is most common in the small finger but can be seen in any digit.  When the pinky is involved, it can cause an appearance problem or it can be a functional problem.  It tends to be most noted in adolescence.  Functional difficulties are most commonly seen in those patients that play a musical instrument.  Here is a single example of an isolated case of small finger syndactyly in a flute player with functional limitations. The family and patient are happy.


Isolated small finger clinodactyly
Clinodactyly after correction.



Clinodactyly associated with a syndrome is most common in Aperts syndrome- also called acrocephalosyndactyly due to facial anomalies and syndactyly in the hand- and Rubenstein Taybi syndrome.  In both of these conditions, the clinodactyly affects the thumb and can interfere with function especially in the severe cases.  These cases are more severe than the isolated cases and therefore are treated at a younger age.  Here are 2 examples.

Clinodactyly in Rubenstein Taybi before surgery.  The after surgery x-ray and pictures are below.




A more severe clinodactyly in Rubenstein Taybi.
X-ray show the abnormal phalanx (rounded) and the deformity in clinodactyly.


Clinodactyly after correction.
Clinodactyly after correction.  The opening wedge osteotomy is visible (as a "hole") in the phalanx which allowed straightening of the digit.

Toe Transfers

There are 2 types of toe transfers for children born with hand deficiencies: vascularized complete toe transfer and non vascularized transfer of a toe phalanx (i.e., just the bone).  Vascularized toe transfers are considered for children with absent digits, typically in cases of symbrachydactyly (or transverse arrest) or amniotic constriction band.  One or two toes can be transferred to improve function.  This is obviously a quite involved surgery involving the removal of a toe with all of its bone, nerves, arteries, veins and tendons.  The digit is then moved to the hand with reconnection of all of these structures, using a microscope for the nerves and vessels.  It has a great potential to improve function, especially in a hand with no digits but this type of intervention is not for every family.  Simon Kay, in England, has written on this topic http://www.ncbi.nlm.nih.gov/pubmed/8982913 in a two part article.  Neil Ford Jones in California is also an advocate of this procedure and has shared his views in a review article http://www.ncbi.nlm.nih.gov/pubmed/17478259 as well as speaking on the topic.

Nonvascularized toe transfer is another option to provide length to the deficient congenital hand.  Surgeons who believe in this operation believe that one could remove the toe phalanx (one of the 3 bones in the toe), transfer it to the hand, and growth can be maintained.  I, along with others, have been less successful in achieving growth of this transferred bone.  Additionally, it has been shown that the earlier the transfer (ideally at less than 6 months of age), the more likely that growth will be achieved.  However, the problem with such an early surgery is that if growth does not occur, the bone that has been transferred is really small and unlikely to make a functional difference in a growing hand.

I believe that there is a role for this surgery with several considerations.  First, when I consider this operation, I do so when the child is several years of age.  That way I am transferring a toe phalanx that is of a reasonable size which can improve function even if it does not grow.  And second, I consider toe transfer together with lengthening of the digit.  http://congenitalhand.wustl.edu/2012/06/finger-lengthening.html  By transferring a toe, the amount of bone to work with and then lengthen is increased- I believe increasing the chance of a successful intervention.  Bill Seitz has written on this topic.  http://www.ncbi.nlm.nih.gov/pubmed/20353864

Finally, in my experience, families are not excited about harvesting bone from the feet.  Obviously it will leave a scar and sometimes there can be deformity.  I share this concern.  A recent paper from England documents that the toe deformity is greater than we previously appreciated.  http://www.ncbi.nlm.nih.gov/pubmed/22305432

In short, I will occasionally use nonvascularized toe transfer but it is not my first choice for reconstruction in most children.  When I do consider this option, external fixator lengthening is also considered a part of the procedure in most kids.  I believe that vascularized toe transfers also have a role for very specific children in certain families.

Below are several pictures from a recent child I evaluated as a second opinion.  Early nonvascularized toe transfer had been performed without much benefit and with toe deformity noted.


Hand appearance in child with symbrachydactyly after the addition of 3 toe phalanx bone years ago.  Functional pinch is still between the thumb and the pinky.

X-rays demonstrating small additional bones in toe transfers with symbrachydactyly.


Foot appearance after nonvascularized toe transfers from 3 toes.