Sunday, February 16, 2020

Recurrent Syndactyly

Syndactyly is the abnormal connection between fingers- typically skin connection but sometimes bony connection (called complex).  I have written much about it HERE.  There is also information on the internet including some basic information on Wikipedia, NIH, and numerous hospital websites.  While syndactyly is one of the most common congenital upper extremity diagnoses, treatment is not perfect and 'failures' occur including, most commonly, recurrence of the skin with a process of 'creep' of the skin.  The other issue is a contracture or banding that limits finger straightening after surgery.  These problems typically are not present right after surgery but often appear years later.  What often happens is that the scarred skin does not grow or stretch with bone growth during childhood.  Certainly, that is a simple explanation and it is part of a more complex process.  In my experience, these are more common and worse with complex (bony) syndactyly but can occur with any type of syndactyly (partial, complete, etc).

We wonder about the risk of recurrence and surgeon experience.  I am definitely biased but I believe that recurrences and complications after surgery are less common when the treatment is provided by someone who treats these conditions on a regular basis.  We know that this is true for some diagnosis like joint replacement and hospital volumes- see HERE.  But, again, this is less clear for less common diagnoses like syndactyly.  Nonetheless, my advice for any parent is to make sure you are 1) comfortable with your surgeon's interactions and approach and 2) comfortable asking about the surgeon's experience with your child's diagnosis.  If the surgeon becomes difficult or doesn't like the question, that may mean that his/ her volume is not high.  This does not mean that the surgeon is not good, it just means that they do not perform this surgery commonly and I believe that this is a key factor for success.  The opposite is also true.  Just because a surgeon may perform an operation commonly, the surgeon may still have less than perfect outcomes.  So--- this is tough to sort out.

Here is an example of a recurrent syndactyly. There is web creep- the skin has crept out the fingers and a contracture or banding.  The right and left hands were both treated at an outside hospital.  Again, I am not suggesting anything was done incorrectly although the outcome on both sides is less than ideal.  I am showing before surgery and after surgery (hands are mixed).  There is creep of the skin, rotation of the fingers, and a band limiting finger motion.  There is also clinodactyly- a curvature of the small finger because the middle bone is not the typical rectangular shape.  This can be addressed at the time of surgery.

Recurrent syndactyly with creep of the skin, rotation of the fingers, and curvature of the little finger.
Recurrent syndactyly with creep of the skin, rotation of the fingers, and curvature of the little finger.  The banding is causing a contracture of the finger and rotating the finger.

This xrays shows the clinodactyly or curvature of the little finger.
Clinodactyly related to the abnormal middle phalanx on the little finger.

Surgery deepens the web space from the recurrent syndactyly, breaks up the banding, and interrupts the abnormal growth plate affecting the little finger middle phalanx.  This is done through rotation of the skin and skin flaps designed to accomplish these goals and prevent future problems.  These pictures are about one year out from surgery.  The finger alignment will continue to improve but there will always likely be some hint of rotation.  There is full finger motion (straightening was limited before).

Results after correction of recurrent syndactyly including deepening of the webspace, breaking up the band and addressing clinodactyly

Results after correction of recurrent syndactyly including deepening of the webspace, breaking up the band and addressing clinodactyly

Syndactyly surgery may be considered 'simple' surgery by some.  I do not think that it necessarily is.  Experience matters and designing the best flaps, using skin grafts when necessary, and addressing all issues are all important.  When necessary, revision surgery can really be helpful as shown here but, obviously, we would love to avoid the need for revision surgery in syndactyly whenever possible.

 Charles A. Goldfarb, MD              


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Thursday, February 6, 2020

Cleft Hand: Beyond the Basics

I have posted numerous times on cleft hand. (See other posts.)  In my world, this is a still an unusual diagnosis and can be challenging as each child is somewhat different than the next.  The early and late treatment of cleft hand, therefore, has to be considered for each child.  

Late treatment in cleft hand is usually related to one of a few issues.  The first is muscle weakness, typically related to the lack of muscle development in the cleft.  Another issue that we see in cleft hand include instability of the MCP joints (the joints that connect the hand to the fingers), especially the index finger and the ring finger.  In addition, we often see challenges with straightening the PIP joints (main knuckle in the finger)- this is due to a lack of muscle in the hand which should straighten the finger.  Thankfully, despite these three issues, patients with cleft hand usually are highly functional and without pain.  This example shows another issue- finger alignment.

Here is an example of a great child and family who I have followed since birth.  Early surgery improved function and appearance for both hands.  As he has gotten older and more active, he has been frustrated around function and specifically that his left hand index finger (pointer finger) is pulled away from the rest of the fingers.  He can bring it back towards the next fingers but only by pushing with the thumb.  

Cleft hand with abnormal positioning of the index finger

Cleft hand with abnormal positioning of the index finger
The finger is able to be brought to the midline but only with thumb pressure.

Cleft hand with abnormal positioning of the index finger

Cleft hand with abnormal positioning of the index finger but excellent motion.

This video is really interesting and shows this nicely.  Watch how he uses the thumb.

After much discussion over a year, we decided to cut the bones and realign the index finger as well as tighten the ligaments to better support the finger.  This type of ‘touch’ up surgery in cleft hand can be highly successful, but we always think and talk a lot about it before actually proceeding with the surgery because we would never want worsen function or appearance.  In this case, I am happy to show, surgery has been very helpful and has increased activity, dexterity and appearance.

Surgery like this is an outpatient procedure, meaning the patient comes in for surgery and can then go home the same day.  The index finger proximal phalanx was cut and realigned and held in place to heal with pins.  

Bony reconstruction in cleft hand.

Cleft hand surgery.

The ligament on the midline side of the index finger (the ulnar collateral ligament of the MCP joint) is tightened (we also pinned this joint during the healing process).  After about 6 weeks, the pins were removed, therapy begun, and activities progressed.  These pictures and video are at 3 months.

The pictures after surgery are also helpful.
Cleft hand after reconstruction showing improved alignment. The patient can keep the index finger in the midline.

Cleft hand after reconstruction showing improved alignment. The patient can keep the index finger in the midline.
Cleft hand with surgery on both sides.
Charles A. Goldfarb, MD              


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Saturday, January 25, 2020

Amniotic Constriction Band- how we can help function.

Amniotic constriction band is a common condition which typically involves more than one extremity.  While the belly and face can be involved in rare patients, the arms and legs are the classic sites.  Every child with amniotic band is different.  Totally.  Sometimes, physicians refer to it as a syndrome but that is not really accurate- it is a random pattern of constriction rings, amputations, and syndactylies (classically, what we call these fenestrated syndactyly- that is syndactyly with a hole between the fingers).  Because every child is different, treatment is developed for each child.  But treatment is also not always required, thankfully.  This is the very brief story of one amazing child who has done well and is so happy with his new thumb.

The goal in the treatment of a hand with notable finger loss is to assure (when possible) that the child has at least two fingers (ideally a finger and a thumb) for pinch.  This is, of course, even more important when both hands are involved and when one hand is completely absent.

This adopted patient presented with amniotic constriction band and a midforearm deficiency on the right and multiple finger amputations with a short thumb on the left.

Amniotic constriction band affecting both arms.  There is no hand on the right and a limited hand, as seen better below, on the left.

A child with amniotic constriction band before treatment.  Not the normal little finger, short ring finger, very short pointer and middle finger and very short thumb.

This x-ray confirms the amniotic constriction band diagnosis with a normal little finger, short ring finger, and very short thumb, pointer, and middle fingers (really only the hand bones- the metacarpals).

The patient was struggling grasp and pinch.  We met the family several times and elected to proceed with a lengthening procedure of the thumb metacarpal to improve pinch against the ring and small fingers.  This requires at least two surgeries
-       one surgery to ‘break’ the bone and apply the fixator
-       a second to remove the fixator and deepen the first web space to ease the pinch motion

-importantly for both child and family- this lengthening is done by turning a dial 2-4x each day and is completely painless.

I really like this operation but there can be challenges.  And I have learned from my partners Eric Gordon and Mark Miller (see their site) who lengthen and straighten larger bones. 

-       The bone does not always unite and this process take a long time
-       Careful weekly x-rays allow monitoring and adjustment of the ‘turn’ rate
-       Regular follow up to assure no infection or to treat pin tract infections (unfortunately, these are common).

This patient eventually healed but there was some deformity in the bone and we straightened it (a third simple surgery).  He and his family are very happy with his function.  We will, obviously, continue to follow the patient over time.  He will likely benefit from a second operation in a few years and maybe even a third as a teen.  But, in a child like this with a challenging presentation of amniotic constriction band, the surgeries will make a clear functional difference.

Patient after lengthening for amniotic constriction band.  Note the length of the new thumb.

Patient after lengthening for amniotic constriction band.  Note the length of the new thumb.  It is great to see him using the new thumb!

Patient after lengthening for amniotic constriction band.  Note the length of the new thumb.  He uses the new thumb to write.
Lengthened bone in amniotic constriction band.  Not the length of the thumb metacarpal compared to the xray above.

Charles A. Goldfarb, MD              


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Sunday, January 5, 2020

Ulnar cleft hand, part II

My most recent post, from Dec 5th, was on a rare type of cleft hand, the ulnar cleft handUlnar cleft hand is very different and even less common that the classic cleft hand which involves the central hand (classically with middle finger absence).  There have been a few manuscripts on the topic of ulnar cleft hand including (among others):

2014 Al- Qattan paper

2002 Tonkin paper

These papers demonstrate how truly rare ulnar cleft hand is.  The patients have a cleft between the ring and little fingers and may have deformity of the little finger or, more commonly, have a very small little finger which may be duplicated (i.e., extra little finger). 

This case, just like my previous post, is an example of ulnar cleft hand but is more similar to other cases in the literature.  Thankfully, the patient has done wonderfully,  Importantly, the family wished to remove the abnormal finger with unusual bones and to maintain both little fingers- their goal (as was mine) was to improved alignment for function and appearance.

Ulnar cleft hand preoperative
Ulnar cleft hand preoperative from palm side

Ulnar cleft hand before surgery.  Note abnormal collection of bones.

In these pictures from 2012, the thumb is normal as are the index and middle fingers.  However, there are three small digits on the ulnar side of the hand.  The central one was removed and the outer two were preserved at surgery and realigned through a bony cut.  

The next group of pictures if from 2019 show the appearance and motion now.

Ulnar cleft hand after surgery

Finger motion in ulnar cleft hand after surgery

Appearance from palm of ulnar cleft hand
These three pictures confirm an outstanding outcome for a challenging presentation of ulnar cleft hand with rotated and malaligned digits.  While the thumb, index and middle fingers are the key for function, the two small digits on the ulnar hand do not interfere with function and are utilized for activities.  This patient has excellent function and the family is thrilled with the appearance outcome.

Charles A. Goldfarb, MD              
My Bio at Washington University     

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