Sunday, August 9, 2020

Tight First Webspace

 The space between the thumb and index (pointer) finger is crucial for function.  We say that the thumb accounts for 40% of hand function - much of that function is related to grasping large objects in this 'first webspace'.   The thumb-index webspace, or as it is also called- the first webspace, can be tight or contracted for two basic reasons- a limitation present at birth or a limitation related to trauma.  

Birth differences leading to first webspace contracture

I have previously shared thoughts on the first webspace in arthrogryposis a few times, here is one LINK.  The first webspace can also be tight in other birth differences such as hypoplastic thumb, as seen HERE.  I have also previously shared thoughts on different techniques to deepen various areas of tightness.  This might be a little technical but here is that post as well, see link.

Trauma leading to first webspace contracture

When there is trauma to the hand, tightness or contracture can develop as well.  This may be a deep abrasion or a burn.  The abrasion type injury can happen on a treadmill when a child's hand is injured by the spinning treadmill.  Burn injuries are more obvious and can happen in various ways including grasping a coal or log in a cooking fire, falling into a campfire, or grasping something hot such as a curling iron.  Ultimately, the skin will heal but will contract and lead to limitations in function and in motion.  This type of injury is very common in underdeveloped countries that depend on cooking fires and are less common in more developed countries but certainly can still happen.

Thankfully, we have good treatment options.  If there is a straight-line burn scar, the goal is to break up the scar band with a z-plasty.  This can be highly successful. With a more complex contracture, a different type of procedure is required to break up the thicker, typically broader band.  Often we use a flap of normal tissue to rotate into the area.  The first webspace is a classic area to have a contracture and the dorsal rotation flap can be very helpful.  In this procedure we take normal skin and advance it into the first webspace.  We may have to release deeper tight tissues as well and sometimes even the deeper muscles.  We divide or excise the poor skin and use the skin flap to cover the areas. Sometimes, advancing this normal skin is enough but sometimes skin grafts can be required also.

Here is one example.  My 'before' pictures are limited but I believe you get a sense of the contracture.  I have also already drawn my flap on the skin- this is normal tissue.  Usually we can bring the thumb out about 90 degrees from the index finger.  In this case, the patient is limited to about 45 degrees.

Tight first webspace after trauma

Then we have good after pictures, 4 weeks later.  Notice how deep and wide the first webspace is.  The thumb can be brought 90 degrees from the hand.  The thumb moves normally again and there is healthy, soft skin in this critical area.

First webspace after advancement flap.  Notice the motion of the thumb.

Nice view of first webspace after deepening 

This patient has done well and I expect will continue to do well in the years to come.

Charles A. Goldfarb, MD              


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Sunday, July 19, 2020

Pollicization Long Term

Hello all.  I want to share a good result in a challenging pollicization procedure.  This patient has radial longitudinal deficiency with a poor, very limited thumb (we call it type 4 hypoplastic thumb).  The decision was made to excise the thumb and create a new one with the index finger (e.g., the pollicization procedure).  We know that this is a great procedure- I have blogged about this many times as you can see at this link:

We also know that there are a couple of factors that affect the results after the pollicization procedure.      1. The severity of the radial longitudinal deficiency.  If highly severe and effecting the entire forearm- the results are likely to be worse.  
2. The quality of the index finger.  What we focus on is the motion- an index finger with good motion is likely to be a good thumb.  An index finger with limited motion will provide a thumb which is not quite a good.  Another factor is index finger alignment- when the index finger is crooked, it is likely to create a more limited thumb after pollicization.  But, the alignment can be carefully adjusted to improve over time.

We have a great series of pictures to share.  First, the pictures before surgery.  Note the really small thumb (we call it a pouce floutant, or Type 4 small thumb).  Also note how crooked the index finger is- this is clinodactyly and is very uncommon in this situation.
Hypoplastic thumb-  note how small and deficient it is.  Also see the deformity of the index finger.

Hypoplastic thumb with index finger deformity.  Patient is trying to make a fist and index finger does not bend well.  This makes an excellent outcome after pollicization more difficult. 

We performed the pollicization procedure around 2 years of age.  At the time, we also worked to straighten the new thumb by adjusting the growth plate.  The scar is a bit different from usual because we had to address the thumb growth plate.  See these pictures next.

After pollicization surgery.  Note that there still is deformity in the thumb as this will correct over time and with growth.  

After pollicization surgery.  Note that there still is deformity in the thumb as this will correct over time and with growth.  

After pollicization surgery.  Note that there still is deformity in the thumb as this will correct over time and with growth.  

We then saw him at 6 weeks after surgery- note how well he was already using the thumb.

Pinching 6 weeks after pollicization surgery

6 weeks after pollicization surgery

6 weeks after pollicization surgery.  There is still deformity but good alignment.  

6 weeks after pollicization surgery.  There is still deformity but good alignment. 

Here he is at 2 years after pollicization.  Again, I am pleased with the result and he is using his head regularly.

2 years after pollicization with excellent pinch.

2 years after pollicization at rest.

2 years after pollicization with improved deformity of the new thumb. 

Finally, here he is, 7 years after pollicization.  Here is a video showing his function.  He uses the thumb for everything.  While he uses the other hand as first choice (he is right hand dominant), the left hand and the thumb work great for him.

It will great to watch as he continues to progress.  This is an unusual pollicization but an excellent result despite different 'challenges'.

Charles A. Goldfarb, MD              

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Sunday, June 21, 2020

Radial polydactyly- progress and patient follow up

I have shared my thoughts on radial polydactyly (extra thumb) a number of times- those other posts can be found at  Radial polydactyly is a common diagnosis for those children with birth differences but each child and each extra thumb is also very different.  This makes comparing children difficult and results are limited in the medical literature.  I am excited to share two positive steps.  

The first is that our CoULD Registry recently completed an assessment of classifications.  First- what is CoULD?  This is a registry that we created to enroll and follow kids with birth differences of the upper extremity.  We have been enrolling kid for about 5 years and have over 3200 patients at 10 different sites.  Really exciting as these patients will teach us so much and help us better understand these birth differences.  Here is the website for more information:   

Working with different sites (Ann Van Heest in Minneapolis led this study) we evaluated hundreds of children with an extra thumb to better understand the classification systems available to us- this paper will be published soon and will be an important step forward!

The second exciting potential is that we applied for a grant to further understand the genetics of radial polydactyly.  While we won't hear about the grant for another 6 months, we are super excited and have a grant team in place to move this effort forward!

Now, I would like to share the 4 year outcome of a great patient with radial polydactyly.  This is type 4- meaning that the extra thumb comes from the MCP joint.  These thumbs are not terribly divergent (i.e., they are not going in the opposite directions) but the surgery still required a realignment and ligament construction to make it straight and functional.

Here are the before surgery pictures:

Radial polydactyly, type 4

Radial polydactyly, type 4 from palm side

Surgery entailed a carefully designed incision of the skin, removal of the extra thumb, reconstruction of the ligament, and bony alignment improvement.  We placed a temporary pin and casted for 6 weeks.  

Thumb polydactyly reconstruction with pin in place

Thumb polydactyly reconstruction with pin in place

Radial polydactyly xray in operating room with pin in place

The patient returned recently for the 4- year followup and he is doing great.  The thumb has very good but slightly decreased motion compared to the opposite side and good alignment.  There is no pain.  There is, as we expected, a size difference.  That is impossible to correct.  However, it is also very difficult to notice unless the thumbs are placed next to each other as we have done in these pictures.

Comparison of thumb size.  Note the slightly smaller right thumb

Thumb flexion (bend) is good but not quite the same as the normal left thumb after radial polydactyly reconstruction

Alignment after radial polydactyly reconstruction

Side view of thumb after radial polydactyly reconstruction

Every patient is different and this is a very good outcome.  We do watch as the patient grows to assure no changes to alignment or function.

Charles A. Goldfarb, MD              

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Sunday, April 19, 2020

The Elbow in Birth Differences of the Upper Extremity

The elbow is not commonly discussed around the topic of birth differences of the upper extremity.  We discuss fingers, wrist, and forearm most commonly.  The shoulder is also occasionally involved but much less so compared even to the elbow.  My goal in this blog post is to highlight some of the birth differences that may include elbow involvement.  This post is not meant to be a comprehensive discussion of each of these many topics (rather, see the many other posts on these topics).

The elbow is made up of the three joints:
1) the ulnohumeral joint which allows flexion and extension (bending and straightening).
2) the radiocapitellar joint which helps with forearm rotation.
3) the proximal radioulnar joint also helps with forearm rotation

These conditions can interrupt normal function of any one of these joints.

1) Radial longitudinal deficiency (RLD) does not truly affect the elbow as the ulnohumeral joint is normal.  However, when the radius is completely absent (type 4 or 5) it does affect the forearm (absent rotation).
RLD with absent radius bone.  The ulna is relatively normal (slightly short).  The hand and wrist are clearly affected.

2) Ulnar longitudinal deficiency (ULD) affects the elbow joint through the ulnohumeral joint.  Without a normal ulnohumeral joint, elbow flexion and extension may be limited.  In addition, there can be fusion (or joining) of the radius to the humerus which prevents any movement of the elbow joint at all.   And finally, depending on the exact issues, the radial head may be dislocated.

ULD showing a short ulna and a curved radius bone with a dislocation of the radial head.

3) Radial head dislocation occurs when the radial head does not line up with the capitellum.  While this can be related to trauma, often it is related to a birth difference.  Sometimes this has a minimal effect on function but sometimes forearm and elbow motion may be limited.

Patient with dislocation of the radial head (it is sitting too high in the picture).

4) Radioulnar synostosis is when the radius and ulna are fused together.  This limits rotation of the forearm.  Sometimes the radial head can be out of position as well (radial head dislocation).

Normal forearm and elbow
                                  Synostosis of the radius and ulna.  Note the bony bridge between the two forearm bones.

5) Arthrogryposis affects the muscles in the upper extremity and, due to muscle stiffness, can also affect the joint mobility.  The elbow is classically in a position of full extension (i.e., straight) which makes function difficult.

6) Transverse Deficiency/ symbrachydactyly occurs when there is an amputation at the proximal portion of the forearm.  There may or may not be nubbins on the end of the forearm.  The elbow may be normal but occasionally, and especially as kids get older, there can be grinding and discomfort in the elbow joint itself.

Forearm level amputation just past the elbow, symbrachydactyly with nubbins.

7) Ulnar dimelia (mirror hand) occurs when there are two ulna bones in the forearm and no radius bone.  There often are 7-8 fingers as well (with no thumb).  The two ulna bones means that there is no forearm rotation and elbow flexion and extension can be limited as well.

Ulnar dimelia with two ulna bones and no radius.

While there are many other birth differences that affect the elbow joint, these are the most common and the most notable.  Please refer to other posts for more details.

Charles A. Goldfarb, MD              

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Sunday, March 29, 2020

Congenital Differences- Timing of Intervention in the era of COVID-19

March 28, 2020

We are in an unprecedented time.  COVID-19, the coronavirus which began in Wuhan Province in China in late 2019, has impacted our world in many ways.  Medical centers in the US and Europe are in a time of crisis as they prepare for or are in the midst of the pandemic.  In the US, in large part but curiously not at all sites, elective surgery has been halted, indefinitely.  Generally, most centers (perhaps unless the center is in a 'hot zone') have a similar approach in that, time- sensitive surgeries may proceed as needed.  That means that emergency surgery (typically trauma) can be treated surgically but other surgeries are carefully considered and most are being delayed.  The logic behind these decision are around social distancing, keeping patients and healthcare providers safe, and preserving PPE (personal protective equipment).

How will these restrictions affect upper extremity surgery for congenital differences?  What happens if your child's surgery for a congenital difference is delayed?  Thankfully, in large part congenital difference surgery is not time sensitive.  Waiting is, in all likelihood, ok.  We consider a few factors when recommending surgery:

1) We consider surgery at an age when safe for the child.  The risks of early surgery are debatable and there has been much discussion about the impact of surgery at a young age.  There are concerns that anesthesia exposure may increase the risk of behavioral issues or conditions like attention deficit disorder.  We believe that relatively short surgeries are safe.  And single surgeries are likely safer than a number of surgeries. However, delaying surgery, especially in these times, might make sense if the condition does not require intervention as noted below.

Certainly, we avoid surgery in the very young child due to specific anesthesia risks related to age and size.  While surgery is safe at even 3 months of age, we try to avoid intervention until at least 6 months of age for surgeries with some urgency.  For more elective surgery, often 12-24 months, is our preference.  Again, we can wait longer in most conditions.

2) There are few conditions that may have urgency.
     a) a newborn with an arm or finger with compromised blood supply.  These are very rare and may be related to neonatal compartment syndrome (if identified early and still in evolution) or amniotic constriction band, tight around a finger or extremity.  If a condition such as one of these is identified, urgent surgery is considered.
     b) Syndactyly on a border digit.  That means ring- little finger syndactyly and, more importantly, thumb- index finger syndactyly. The idea is that the longer digit will be tethered and likely deviated in some way by the shorter digit.  We often consider surgery at approximately 6 months of age for children with these conditions.
     c) Some syndactyly surgeries related to amniotic band may benefit if all the fingers are tethered.  If the digits are freed early, a remarkable improvement can be seen.  Here is one example of tethered digits.

Amniotic constriction band with tethered digits

3) The timing of surgery as relates to school is another condition.  We aim to have surgeries complete prior to kindergarten.  While this is not always possible, it is helpful.  That way, school is not interrupted and the child may be more comfortable with and 'used to' the condition.  Function is often improved after surgery and this can help with advanced, age related activities such as scissor, etc.  Therefore, a surgery planned at two years of age can often safely be delayed until three years of age.

4) Other surgeries.  The timing of more typical congenital difference surgery is often based on surgeon preference and hospital protocols.  For example, there is little to guide us on the timing of 'typical' syndactyly' surgery although at least one paper has suggested that later surgery is better.  Most of us believe that surgery at 18 months is a good time.

Another example is radial longitudinal deficiency.  Surgery can be considered almost anytime although surgery at or after 18 months is our preference.  But, if a series of surgeries are planned, an early start may be helpful.  For example, a child might have an external fixator placed at 18 months of age, a centralization procedure at 24 months of age, and a pollicization procedure at 30 months of age.  Sometimes the pollicization can be accomplished at the time of centralization but we often separate.  AND, if both sides are affected, bilateral surgery can be considered but may be too much for the patient and the family. Tough decisions.

I hope this is helpful.  Post questions on specific surgeries and I can share my personal protocols and also share our feelings on risks (or safety) for delay.

Charles A. Goldfarb, MD              

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Saturday, March 14, 2020

Radial Deficiency Outcome

Radial deficiency (or radial longitudinal deficiency, RLD) is one of the most challenging conditions that I treat.  We classify patients with RLD based on their bone development but that does not capture the underlying muscle and joint abnormalities.  Reports on treatment including surgery have been mixed and that has led hospitals and doctors to have different recommendations on procedures to consider.  And I agree, there is no one correct answer and every patient is different.  Sometimes that is obvious (one side affected vs both sides) or completely missing bones vs partially missing bones.  Other times it is less obvious and related to muscle strength, finger motion, and joint deviation.  I have posted many times before on this topic as you can see HERE.  There are also numerous resources regarding radius deficiency on the internet but many are hospital sites promoting care and others are references to scientific papers.  We in St. Louis have an algorithm for treatment based on what surgeries and interventions that we have found successful.  This includes therapy and different forearm, wrist, and thumb surgeries based on various factors.

I have been fortunate to treat many great patients and families with RLD and other diagnoses.  This is one of those patients/ families who happens to have RLD.  It starts with the perfect sweatshirt!  The right side has an absent radius, limited finger motion, and a radially deviated and flexed wrist.  The left side has better alignment, normal finger motion and a nearly normal radius bone.  The difference in motion of the forearm, wrist, and fingers is striking.  Both sides have had wrist surgery and both sides have had a pollicization (creation of a thumb from the index finger- more information HERE).  The patient clearly favors her left arm but uses both hands together for some activities.

Here are some pictures of the patient with RLD including both arms and an x-ray. 
Patient with RLD, right side more affected than left.

Patient with RLD, right side more affected than left.
Good elbow motion on both sides.  More deformity on the right.

RLD on both sides but more severe on the right.

Here is a video demonstrating function.  Very good overall on the left and pretty good on the right. 

Charles A. Goldfarb, MD              
My Bio at Washington University     

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Saturday, March 7, 2020

Syndactyly: The Type Matters.

Syndactyly, simply put, means joined fingers.  However, there are a number of different types including:
- cutaneous (or simple)- only a skin connection
- Complex-  with a bony connection
- Complicated- with an associated syndrome
- Partial vs complete (whether part way or completely to tip of finger).
- Complex polysyndactyly.  That is, extra bones and bony connections.

The partial cutanous syndactyly type is the most common followed by cutaneous complete.

The treatment philosophy for each of these syndactyly types is similar but the details and outcomes can vary.  For example, we shared the results of treatment of only patient with complex syndactyly (bony connection) with this Manuscript.  There has been very little shared on this topic although a variety of papers on syndactyly include a few patients that are more complex.  Our goal in writing this manuscript was to focus on outcomes.  And we found that these patients did not do as well as other patient types and had rotational deformities and nail abnormalities at a rate which was higher than other syndactyly patients.

We also have written about complex polysyndactyly.  This type of syndactyly is even less common and we sought to provide some framework to classify these patients HERE. 

I have previously written about kids with syndactyly- those entries can be found HERE.

I want to briefly share images of a child with complex polysyndactyly.  This child has an extra bone and joined central fingers.  The thumb, index, and little finger are normal.  The middle and ring finger are joined with an extra bone between.
Central polysyndactyly. 

Central polysyndactyly.  Note the joined central bones and extra bones.

Palm side view of central polysyndactyly.

Syndactyly surgery aims to separate the middle and ring fingers, remove the extra bones, and reconstruct the skin to provide a long term good outcome.  We hope to avoid skin creep or scarring from causing problems with the skin- specific skin incisions are chosen for this purpose.  Finally, we want the appearance to be as close to 'normal' as possible.

In this case, we performed a syndactyly reconstruction with a skin graft substitute, a dermal substitute called hyalomatrix.  This avoids the need for skin grafting.  These pictures show a nice reconstruction near the hand (the commissure).  The sides of the nails (the lateral nail fold) are also not quite perfect despite efforts to perfectly reconstruct that skin (we have to create that tissue).  The scars are slightly prominent at 4 months after surgery but we expect that will improve with time.  Finally, the hand looks great from the palm view with nice web space and alignment.  The xrays also show a nice separation and bony appearance.

Hand after reconstruction of complex central polysyndactyly

Hand after reconstruction of complex central polysyndactyly

Xray after reconstruction of complex central polysyndactyly

Hand after reconstruction of complex central polysyndactyly

We will continue to follow this patient until growth is done.  This will help early identification of any skin tightness or creep of tissues which may occur.  But, function should be excellent and the appearance should continue to get better over time.

Charles A. Goldfarb, MD              
My Bio at Washington University     

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