Sunday, December 18, 2011

More thoughts on Pollicization

More thoughts on Pollicization

The decision to proceed with pollicization is not easy.  It is most straightforward when the thumb is completely absent and is most difficult when the child has any thumb, as families very reasonably would like to keep and improve the small thumb rather than excise it and pollicize the index finger.  However, the majority of congenital hand surgeons (especially in the United States) believe that a pollicized index finger will look and function better than a reconstructed small (hypoplastic) thumb.   Importantly, this is not to say that all small thumbs should be excised- in reality most can be reconstructed and stabilized but need to have a stable base (CMC joint).

This case demonstrates a Type 3b hypoplastic thumb with an unstable CMC joint.  Therefore, we did not feel that reconstruction made sense and we recommended pollicization.

Small, hypoplastic thumb, without function, prior to pollicization

This pictures were taken one week after pollicization.  The bruising and discoloration typically improves over the next 2 weeks.
Pollicization, the creation of a new thumb

Another picture of pollicization.  Note the new web space.
The bruising disappears within a couple of weeks after surgery.



Sunday, December 11, 2011

Radial Polydactyly



An extra thumb has been labeled many different ways: ‘radial polydactyly, thumb polydactyly, split thumb, and duplicated thumb’.  My preferred name is radial polydactyly as it tells you exactly the issue.  The term ‘duplicated thumb’ is not ideal as the thumbs are both smaller than normal but ‘split thumb’ is not perfect either because the thumbs are usually more than 50% of the size of the opposite thumb.

Radial polydactyly is typically both a functional problem and an appearance issue.  Surgery is recommended.  However, surgery is not simply removing the extra thumb- it is a reconstructive procedure that can range from a moderately complex procedure to a very complex procedure.  There is no ideal time for surgery but typically surgeons choose to operate between 1 and 2 years of age.  Sometimes the thumbs are merged to make the best, combined thumb and sometimes the bigger, more mobile thumb is reconstructed to make it stable and straight.  Surgery can involve soft tissues only but often also involves bone surgery.  A second procedure is required in some patients as they get older.

In this case, an older child had the smaller, outer thumb removed with a stabilization and reconstruction of the larger thumb.  



Before Surgery







After Surgery



Friday, December 9, 2011


Symbrachydactyly

Taking the word literally, “sym” means joined (like “syn”), “brachy” means short and “dactyly” means finger= short, joined fingers.  However, symbrachydactyly comes in many different forms.  In fact, some forms of symbrachydactyly don’t have developed fingers at all.  We can classify symbrachydactyly in 4 basic types- these are helpful for discussion.

 - Short finger type- fingers generally well formed but short, likely webbed.
2     - Cleft hand type- absence of central digits.  Nubbins may be in cleft.
3     - Monodactyly- Fingers are absent and only the thumb is present (and may not be normal).  Finger     nubbins may be present.
4     -  Peromelia- Finger and thumb are absent and only the wrist bones are present.  Finger nubbins may be present.

    In addition, symbrachydactyly can end in the forearm with amputation; finger nubbins may be present at amputation site.



This child has a cleft hand type of symbrachydactyly.  He was in clinic for evaluation of the index finger after stabilization.  Pinch has been improved by our surgery to stabilize the index finger DIP joint (near the nail).


Here is an example of the short finger type of symbrachydactyly.


Tuesday, November 29, 2011

Arthrogryposis


Patient with arthrogryposis after rotation of left arm but before surgery on right arm and before wrist surgery.
One of the many challenges in the child with arthrogryposis is internal rotation of the arms (arms rotated towards body).  This can make functional activities like bringing the hands together difficult.   It can be tough to even identify the front of the elbows- especially when the elbows don’t bend.

To address this internal rotation position, the humerus can be rotated with surgery (osteotomy or cutting of the bone) which can really improve alignment.  See the attached picture.  The left arm has been corrected and is resting in a more natural posture.  The right side remains markedly internally rotated! 

Tuesday, November 22, 2011

Ho Chi Minh City

I am sitting in the Tokyo airport awaiting my plane to fly back to the USA.  Had a great 4 days in Vietnam with an international group of congenital hand surgeons.  I was flattered to be asked to join the meeting.  I arrived a day early so had a chance to tour Ho Chi Minh City (formerly Saigon) and surrounding areas.  Fascinating history, interesting museums .


Anyway, learned much about the Vietnam War (or the American War as it is called there) and even more about the new Vietnam.  It is still a communist country but sure doesn't feel that way.  A great deal of entrepeneur spirit.  Crazy traffic (see video)- can be a challenge to cross the straight (and this wasn't even a busy street).

Friday, October 28, 2011

Pollicization- Creating a New Thumb


Pollicization is the hallmark congenital hand surgery.  It is performed for patients with absent or severely diminished thumbs.   The thumb is vital for overall hand function and in adults, it is estimated that the thumb provides 40% of hand function.  A normal or reconstructed thumb allows for grasp of large objects (think: soda can), a task that is impossible when using only the fingers.  It can also simplify tasks requiring small object manipulation (think: stringing beads), very challenging using only the fingers.   A thumb is also important for thumb appearance.  A hand with four fingers and no thumb is abnormal appearing and likely to be noticed whereas a hand with a thumb and 3- fingers is often overlooked and thought to be “normal.” 

Pollicization the creation of a new thumb

Key ability provided by pollicization is pinch against index finger.


Pollicization digit on the left hand compared to right hand.
While clearly different, the new thumb improves both function and appearance.
In this operation, the index finger is rotated into the position of the thumb.  It requires shortening the index finger, repositioning muscle and tendon, preserving the arteries (bring the blood in) and veins (bring blood out), and creating new joints.  It also requires repositioning the thumb in the best position for function and appearance.  We change the dressing one-week after surgery to assure satisfactory thumb position.  The thumb is held in its new position while healing occurs.  Beginning at week 3, we tape the thumb into a functional position, which helps healing in the best position while allowing function.

Tuesday, September 27, 2011

Ulnar Longitudinal Deficiency: The Basics

What is Ulnar Longitudinal Deficiency (dysplasia)?

Ulnar Longitudinal Deficiency (ULD) is a lack of formation of the pinky side of the upper extremity.  It usually affects the forearm but can affect the hand, forearm, and upper arm.  It can affect bone, muscle, tendon, nerves, and blood vessels.  The severity is different in each affected child.

What specific abnormalities are seen?
Hand:  The entire hand may be affected.  The thumb can be affected and the webspace between the thumb and the index finger is often narrowed.  The hand can have syndactyly between other digits.  In more severe cases, a variable number of digits may be absent.  There can abnormal bony connections between the bones of the wrist and/ or bones of the hand (typically, the metacarpals).  Forearm:  The ulna is most commonly short.  It can be absent.  The radius bone may be affected as well but usually is normal.  Sometimes the radius is fused to the humerus (radioulnar synostosis). 

What are other names for ULD?
The most common other name is ulnar clubhand.  It is also sometimes called ulnar deficiency for short.

How does ULD happen?
The arm forms between 4 and 8 weeks of gestation, sometimes before a mom even knows she is pregnant.  By 8 weeks the arm is fully formed although obviously really small.  If there is some insult to the developing arm, part of it may not form normally.  There are different types of insults- some are genetic and some may be caused by outside influences (“environmental factors”).

Are there are medical problems associated with ULD?
The short answer is no.  The only associated medical abnormalities are to the musculoskeletal system.  Specifically, there can be abnormal development of the fibula (at the ankle) or even more rarely other bones in the lower extremity.  

Monday, September 26, 2011

Las Vegas and Potosi- What do these 2 cities have in common?

Recently, I took two hand surgery related trips.  First, the American Society for Surgery of the Hand was in Las Vegas at the beginning of September this year.  Not my favorite city but certainly a good place for a conference.  This meeting is always very educational and provides a wonderful forum for discussion on general hand issues.  There was some reporting/ discussion of congenital and pediatric hand issues including scientific papers and several lectures.  One of the papers was ours on pediatric and adolescent cubital tunnel syndrome.  Another reported the changing nature of scaphoid fractures in children; current fracture patterns are similar to adults with scaphoid waist fractures most common.  A third reported the syndromic relationships with fully formed extra ulnar digits.  And finally, there was a large series on outcomes after carpal wedge osteotomy for the arthrogrypotic wrist.

My next stop was a little more rural.  Last weekend, September 16-18, was our 4th annual Hand Camp at Camp Lakewood in Potosi, Missouri.  The camp for families and children with severe upper extremity anomalies is about 1.5 hours from Saint Louis.  There are physical activities including wall climb, archery, arts and crafts, and horseback riding with all children participating in each activity.  There are group sessions to allow frank discussion among parents and families.  Additionally, the junior counselors, teens who have successfully navigated the difficult social and functional issues, share their tips with the families and campers.  My favorite part of camp is watching kids succeed at tasks that seem daunting (think archery for a child with one arm) and also the group sessions where emotions can run high and lessons abound from the sharing of experiences.  Can’t wait for next year!

Monday, August 1, 2011

Photo of Syndactyly Involving Four Fingers

Syndactyly involving all 4 fingers.  Much less common presentation.  Likely to have bony involvement.


Photo of the Classic Appearance of Syndactyly

Syndactyly between the long and ring fingers.  The most common appearance.  Skin involvement only.



Monday, July 25, 2011

Radial Longitudinal Deficiency

What is Radial Longitudinal Deficiency (dysplasia)?Radial longitudinal deficiency (RLD) is a lack of formation of the thumb side of the upper extremity. It can affect the hand only (usually the thumb) or, more commonly, the hand and forearm. It is a lack of bone, muscle, tendon, nerves, and blood vessels. The severity is different in each affected child.

What are other names for RLD?The most common other name is radial clubhand. It is also sometimes called radial deficiency for short.

How does RLD happen?The arm forms between 4 and 8 weeks of gestation, sometimes before a mom even knows she is pregnant. By 8 weeks the arm is fully formed although obviously really small. If there is some insult to the developing arm, part of it may not form normally. There are different types of insults- some are genetic and some may be caused by outside influences (“environmental factors”).

Are there are medical problems associated with RLD?There can be important other medical problems associated with RLD. Because the arm and the important internal organs such as the heart, intestines, blood cells, and kidney form at about the same time, one insult can affect all organs. Therefore, problems in these other areas need to be evaluated and often treated before the arm is treated.

Thursday, July 7, 2011

Frequently Asked Questions About Syndactyly

Dr. Goldfarb has the answers:

1.When should surgery be performed?There are no firm guidelines on the timing of surgery for syndactyly but some basic principles apply.
•Thumb- index finger syndactyly or ring- small finger syndactyly should be corrected early. Many surgeons will consider surgery between 3 and 6 months for these syndactylies.
•Index- long finger and long- ring finger syndactyly can be reconstructed in a more elective fashion. Many surgeons choose to operate between 12 and 16 months. The hand is bigger by that age (the hand doubles in size from birth to 2 years of age and nearly doubles once again by maturity), making reconstruction more straightforward. Some families object to waiting this long for surgery but I believe the benefits of waiting are real.

2. Why can’t the fingers simply be separated by cutting the skin between the fingers?• The skin surrounding 2 joined fingers is not enough to cover the fingers when they are separate (apparently they taught us that in high school math: circumference= pi (r)2).
• A straight line incision tends to scar in a band which tightens (contracts) over time.
• The skin at the base of the fingers (the commissure) is most likely to scar and must be reconstructed most carefully to look near normal and to prevent recurrent scarring or web creep.

3. How does the surgeon bring more skin to cover the newly separated fingers?• One technique is to apply skin grafts to fill in the holes left after the fingers are separated. The skin grafts are full thickness which means that the entire thickness is taken and then the donor site is sutured back together in a straight line. The graft can be taken from different areas including the groin and the front of the elbow (my preference for several reasons).
• The other technique is to slide skin from the top of the hand into the commissure (the new skin is the skin from the top of the hand). The technique avoids the need for skin graft and therefore is my preferred technique when possible. I like avoiding skin grafts.

4. What happens after surgery?• My preference (and that of many of my peers) is to place a bulky soft dressing on the hand and forearm (and maybe over the elbow). The child returns to clinic at 3-4 weeks for a dressing change and to initiate therapy.

5. What are the risks of surgery?• Most surgeries are uneventful and the child does well with a satisfactory appearance after surgery and excellent function.
• Infection is a risk with any surgery. Sometimes a low grade infection can threaten skin grafts.
• Skin grafts and flaps may not survive after surgery. This complication is very rare.
• Web creep is perhaps the most notable risk. Web creep is scarring in the commissure between the fingers that leads to a partial “recurrence” of syndactyly.
• About 1 out of 3 children will need a second surgery. For some types of syndactyly, that number is higher.

6. How many surgeries will be necessary?Usually, only one surgery is required for a syndactyly that affects only 2 fingers. However, about 1 out of every 3 patients will need another surgery- most commonly to deal with web creep.

7. What is different about complex syndactyly?Complex syndactyly is most commonly a bony connection between the fingers near the fingernails. The reconstruction process is similar to cutaneous (or “simple”) syndactyly but the fingers typically have a less satisfactory outcome. Even with appropriate surgery, the fingernails are often deformed and the fingers may be angled or rotated. Additional surgeries to straighten the fingers may be helpful in these children.

8. What is central polysyndactyly?This is complicated form of syndactyly. There is typically an extra portion of a digit between the long and ring fingers. This extra digit makes syndactyly reconstruction more challenging and increases the likelihood of additional surgeries. The separated fingers are more likely to be smaller than normal and may be angulated, rotated, or lack full motion.

9. What is Apert’s syndrome?This is a complex syndrome with abnormalities of the skull bone, facial appearance and hands/ feet. The syndactyly that these children have is quite complex and often requires multiple surgeries to correct. Skin graft is usually required.

10. What is oculodentodigital dysplasia?These children have a typical facial appearance including a narrow nose, small teeth, small eyes, syndactyly of the 3rd, 4th, and 5th digits, and may have neurological problems as well. Children with syndactyly of the 3rd, 4th, and 5th fingers may be evaluated for this diagnosis.


11. What is symbrachydactyly?The term means short, webbed fingers, at least that is the most common appearance. However, other children with a different appearance may also have symbrachydactyly (see future post- this gets complicated). Reconstruction of the typical syndactyly in these children is similar to the above discussion.

12. How is syndactyly in constriction band syndrome (or amniotic band syndrome) different from other syndactyly?These children develop normal fingers in the womb but scarring leads to abnormal connections, typically at the fingertip. Reconstruction is different that what has been described, sometimes more straightforward (see future post).

Friday, June 24, 2011

Syndactyly

What is SYNDACTYLY?
Syndactyly is the abnormal connection of 2 fingers. Most commonly, it is a webbing between 2 fingers- the middle and ring fingers. However, it can involve any two fingers or can involve the thumb and pointer finger. It can also involve more than 2 fingers. The webbing can involve the entire 2 fingers or just involve part of the fingers. It can be just skin or can involve the bone also.

Why does syndactyly happen?
Most types of syndactyly happen during the development of the hand and arm. The entire arm forms between 4 and 8 weeks of gestation- this means that by about 56 days after the baby is conceived, the hand is in its final shape and form (although a lot smaller!). During the final developmental phases nearing 40 days and after, the fingers are all webbed. Normally, the skin interconnections disappear before 56 days but, if the cell signaling doesn’t happen like it should, the skin connections (the webbing) stays and that is what we call “syndactyly.” In most cases, there is nothing a mother or father did to cause this abnormality and there was nothing that he or she could have done to prevent it.

Constriction band (amniotic band) syndrome is another possible cause of syndactyly. In this case, we believe the hand and individual fingers form normally but then there is a trauma/ scarring s by a constriction band leading to scarring and the syndactyly. Many times the hands in this kind of syndactyly may have short fingers due to fingertip amputations.

Symbrachydactyly is another type of syndactyly. In this case, the fingers are short and webbed. Kids with symbrachydactyly can be very different from each other- some have almost normal fingers, some have short and completely webbed fingers, and some have really no fingers at all.

Definitions:
Syndactyly: Fingers that are joined together, with skin and possibly bony connections.

Partial vs. Complete Syndactyly: Syndactyly can involve the complete fingers (all the way to the tip) or just part of the fingers (partial).

Cutaneous vs. Complex Syndactyly: Cutaneous (or “simple”) syndactyly implies a skin connection only whereas a complex syndactyly includes bony interconnections between the fingers.

Complicated Syndactyly: Syndactyly associated with a syndrome (usually a situation with other health issues).

Function and Appearance:Typically, children with syndactyly function well although the more fingers that are affected, the more difficult function may be. The primary functional problem in syndactyly is grabbing large objects or grabbing circular objects (because the fingers don’t spread apart as well as they normally would). The hand looks different with syndactyly. This may bother the child and may change how others relate to the child. In some kids, this may lead to stress and decreased self-esteem.

Surgery:The only way to correct syndactyly is through surgery. The timing of surgery depends on many different factors. When the syndactyly involves the thumb and pointer finger or the ring and small finger, we try to perform surgery early- often by 6 months of age. Other syndactylies can be treated when the child is older, often between 12 and 18 months of age.

Separating fingers is not as simple as cutting the skin connecting the fingers (there is not enough skin and the syndactyly will reform). Instead, the syndactyly must be reconstructed through a series of carefully designed flaps. New skin must be brought between the fingers- sometimes this requires skin grafts. We try to and can usually avoid skin grafts when we reconstruct syndactyly. Kids are usually placed in bulky soft dressings until 4 weeks after surgery and then they start therapy. Therapy helps keep the scars soft and also helps to regain motion.