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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Sunday, March 1, 2020

Two different thumb birth differences

The thumb is our most important digit.  In the adult, we estimate that it affects about 40% of hand function.  So, a thumb that is not fully or normally developed may really affect our abilities- whether that be strength, motion, dexterity, etc.  I have written a number of posts (See HERE) about the thumb but thought I would share a few pictures and xrays of two patients with different thumbs.

The first patient has an extra thumb.  These come in a number of different patterns.  In this case, the extra thumb is smaller and less functional than the normal thumb.  The more normal thumb is still smaller than the opposite side but has good structure and tendons. 

Thumb polydactyly (extra thumb).  The outer thumb is not normal.
Thumb polydactyly
Surgery entails removal of the extra, outer thumb and reconstruction of the main thumb.  While it is not completely normal, it should function at a high level.  The pin stays in 5-6 weeks with a cast.  Then the cast is removed, the pin is removed and therapy is begun.

Thumb polydactyly after reconstruction with excision of extra thumb, ligament reconstruction, and pinning.

Thumb polydactyly after reconstruction with excision of extra thumb, ligament reconstruction, and pinning.

The other case is an extra bone within the normal thumb.  The thumb is different than the fingers.  The fingers have 3 bones, three phalanges.  The thumb normally only has two.  A triphalangeal thumb is more like a finger with 3 bones.  Sometimes it otherwise seems like a thumb and sometimes it can rest like a finger.  With an extra bone, the thumb is longer and may be more deviated (the extra bone is often not normally shaped).  It also bends and functions differently.  We often (but not always) consider surgery for these patients. 
Triphalangeal thumb.  Note some deformity, angulation

Triphalangeal thumb x-ray. See the small bone in the middle of the two other bones.  

We removed the middle bone, reconstructed the supporting ligaments, and adjusted the tension on the tendons.  This patient should also do well with a strong, aligned thumb for the future.  Sometime motion takes time to recover but most patients have a highly functional thumb after this surgery.

Triphalangeal thumb after excision of the abnormal bone.

The patient's other side.  More deformity with the triphalangeal thumb due to the triangular shaped bone.

These are just two examples of birth differences of the thumb.  Both of these patients should do well over the long term with strong, stable, well aligned thumbs.

Charles A. Goldfarb, MD              
My Bio at Washington University     

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