Wednesday, October 10, 2018

Untreated Cleft Hand

Cleft hand is one of the most notable birth anomalies of the upper extremities.  The appearance is distinctive although function can be really well maintained.  One of the founding fathers of the discipline of hand surgery, Sterling Bunnell, labeled cleft hand, "a functional triumph and a social disaster".

There are other names for cleft hand.  The geneticists call it split hand (often along with split foot).  EEC is a syndrome with cleft hand as a part- ectrodactyly (missing digit) and ectrodermal dysplasia.  It can also be associated with cleft lip and palate.  It may be genetic or random and it may affect one hand, both hands and, especially in genetic conditions, the feet.   We continue to learn more about cleft hand from a genetic standpoint.

There are two classification systems on cleft hand.  My former partner and mentor, Paul Manske, classified cleft hand based on the quality of the thumb web space.  This is relevant due to the need to reconstruct this web space if too tight.  Dr Ogino, a friend who advanced our understanding of cleft hand through lab and patient research, classified patients on the basis of the number of missing digits.  Together, these classification systems really help our understanding of each patient and help us plan treatment.

Families with other members affected may have a different outlook and approach to the evaluation and treatment of cleft hand.  If left alone, children can function well using the cleft for large object grasp no matter the size of the first (thumb- index) webspace.  This picture and video are of a child without functional limitation in a family with others with cleft hand.  The family wishes to avoid surgery for now.

As a surgeon, I know what surgery can offer: improved appearance and a better thumb grasp with enlargement of the first web space.  However, I also understand why every family may not chose surgery.  My role, as I see it, is to share my experience and help guide each family to the best decision for them.  Most of my families would chose surgery for these cleft hands, but not all.

Cleft hand with large cleft and tight first web space bilaterally.




This video shows these same cleft hands with dramatic instability of the index finger MCP joints.  The videos also demonstrate the limitations of the first web space with limited space available for thumb function.  The instability can become an issue with strength and grasp although surgical reconstruction can be helpful.  There can also be instability of the ring finger MCP joints.

Cleft hand is a complex and striking disorder.  Surgery can absolutely be beneficial for the child, for their function, and for their appearance but every decision is family- centered.

Charles A. Goldfarb, MD
My Bio at Washington University
Email me: congenitalhand@wudosis.wustl.edu

Sunday, September 23, 2018

Radioulnar Synostosis

Radioulnar synostosis is the presence of a bony connection between the radius and ulna.  I have previously blogged a few times on it HERE.  While a synostosis can develop after a trauma, we typically discuss it when present from birth.  The synostosis prevents forearm rotation but does not affect elbow or wrist motion (these are typically normal).  There have been many attempts to 'cure' radioulnar synostosis to restore forearm rotation, all without full success.  In 2018, we typically treat radioulnar synostosis if the forearm is stuck in a position of significant palm down (pronation) or palm up (supination).  We also tend to consider surgery more frequently when both arms are involved.

Surgery for radioulnar synostosis tends to be an osteotomy or a cutting of the bone and rotating the forearm to a more functional position, close to the clapping position ('neutral').  Usually positioned in slightly palm down position due to the importance of keyboarding and tabletop activities.  I like this surgery as patients and families are happy with it.  BUT, surgery is not always necessary because patients have a great ability to compensate.  This video is a great demonstration of a patient with radioulnar synostosis who appears to be rotating the forearms but really is just rotating through the wrist joints.


Charles A. Goldfarb, MD
My Bio at Washington University
congenitalhand@wudosis.wustl.edu

Thursday, August 16, 2018

Unusual "Extra" Finger

I have posted many times on polydactyly- both on the thumb side and the pinky side- as can be seen HERE.  The following patient demonstrates an unusual presentation of what initial appears to be an extra finger, an ulnar polydactyly.  But, as a quick 'count' confirms, there are only 4 fingers with a thumb.


Ulnar deficiency with apparent polydactyly

Ulnar deficiency with apparent polydactyly, palmar view
The xrays are very helpful in understanding this patient's hand.  There is one thumb and four fingers BUT, there are only 4 metacarpal bones in the hand instead of the normal 5.  This is an ulnar deficiency of the hand as demonstrated by the lack of a fifth metacarpal.  The 4th metacarpal is wider than normal.  I have written about this uncommon form of ulnar deficiency HERE.  This is a scientific article sharing our experience.
Ulnar deficiency affecting the hand.

Occasionally the 4th metacarpal is wide enough to allow a surgery to split it to support both the ring finger and the pinky finger.  In this case, however, I did not feel that the metacarpal was wide enough to consider such a reconstruction.  

In addition, in this patient, the pinky finger was tethering or limiting the more normal ring finger, decreasing its motion and causing deformity.  The patient's family was initially hesitant to excise the pinky finger.  It is always difficult to make this decision despite the deformity.  With time, the limitations caused by the pinky finger to both function and appearance were appreciated and the family requested surgery to remove the finger.  Here are pictures and xrays only 6 weeks after surgery.  Function and appearance are excellent and the patient and family are both very pleased.  The function (motion and alignment) of the ring finger are much improved. 

After surgery for pinky excision in ulnar deficiency.  (The picture is a little hard to interpret because the index finger is bent).


Xray after pinky finger excision in ulnar deficiency.

Charles A. Goldfarb, MD
My Bio at Washington University
congenitalhand@wudosis.wustl.edu

Sunday, August 12, 2018

Function in Radial Deficiency

Radial longitudinal deficiency is both a functional challenge and an appearance issue.  Different surgeons have developed different strategies based on these challenges and the lack of a 'perfect' surgery.  My philosophy is to consider different surgeries, based on the child.  I will consider centralization (typically with a period of external fixator distraction prior to the centralization), a release with bilobed flap, or occasionally a lengthening of the radius.  And, sometimes, even with a deficient radius, surgery on the wrist may not be helpful.  The pollicization procedure is one of my favorites because it is so effective and so helpful.

This is a patient with a severe radial deficiency on the right (treated with external fixator, centralization, and pollicization).   Note the 90 degree deformity of the wrist.  The patient had a milder deficiency on the left (treated with pollicization).  This is an image before surgery on the right.
Severe radial deficiency with the wrist at about 90 degrees from the forearm. Note the short forearm and the lack of a thumb.


Here is an image of both hands after surgery on the left wrist (note how much straighter it is compared to the picture above) and the creation of both thumbs with pollicizations.




This first video gives some indication of the outcome of her pollicizations.  Remember- the pollicization helps in a few major ways including fine pinch (such as beads, in this video) and larger object grasp (second video).  The right hand and wrist were severely affected from birth and while she is doing well after surgery, she still has limitations.  You can see how well her left hand and new left thumb work (after pollicization).  However, on the right, the new thumb is not as strong or effective and she sometimes uses  the ring and small fingers (called prehensile function).  This may further change over time to favor use of the new thumb. 




This next video demonstrates several important findings.  First, the right side is weaker and she clearly, she prefers the left hand (which has only had the pollicization).  The left side in general (and the left thumb in particular) is stronger and more stable.  The right hand is helpful to her as is its pollicized digit BUT, when she really tries to use the right hand and the pollicized digit does not help, she switches to try the ring and small finger for grip (that is reflective of her prehensile grasp which never quite goes away).  


I hope these videos are helpful in understanding outcome with severe radial deficiency.

Charles A. Goldfarb, MD
My Bio at Washington University
congenitalhand@wudosis.wustl.edu

Saturday, July 21, 2018

Trigger Thumb and Fingers

Trigger thumb is one of the more common reasons for kids to present to my office.  While uncommon in the general population, for hand surgeons who treat children, trigger thumb is quite common.  And, thankfully, kids do quite well with this condition.  Some will improve on their own without surgical intervention and others require surgery and do well.   I have other posts on this topic which can be found HERE.  Most trigger thumbs in kids are locked (the thumb is stuck in a position of flexion).

Surgery is quite reliable for trigger thumb.  95% or more are cured with a straightforward, 5- minute surgery.  My personal protocol is a small, 1-cm incision closed with dissolving stitches.  We put numbing medicine in at the time of surgery and most kids never require pain medication.  The most common complication is a superficial infection treated with antibiotics by mouth.  Other complications are incredibly uncommon.

Unfortunately, there can be a less than perfect outcome.  This patient is a 5 year old female who was treated surgically for bilateral trigger thumbs at another hospital.  Unfortunately, her symptoms did not improve.  This video demonstrates that her thumb catches when she tries to bend it.  We performed a revision surgery to correct the residual catching.

Pediatric Trigger Thumb


Trigger finger is much less common compared to trigger thumb.  It can be helped with therapy and splinting but occasionally surgery is required.  This video shows the finger catching with bending.  While treating a trigger thumb in a child or adult requires a similar surgery, trigger finger surgery can be quite different.  A pediatric trigger finger surgery can be more complex and requires a step-wise approach to care.
Pediatric Trigger Finger Video demonstrating catching.




Charles A. Goldfarb, MD
My Bio at Washington University
congenitalhand@wudosis.wustl.edu

Sunday, July 15, 2018

Extra Thumb Reconstruction

I have posted a number of times on radial polydactyly- aka split thumb, extra thumb, etc- HERE.  This is a common birth anomaly and the decision for surgery is usually straightforward.  Sometimes surgery is also straightforward while other times the reconstruction can be quite challenging.  And, about one out of three patients with radial polydactyly will need a second surgery at some point down the road.

I wanted to briefly share images of one patient who recently came back for repeat assessment after reconstruction for a somewhat complex radial polydactyly reconstruction.  Here is one picture before surgery in the clinic.  Note that the inner thumb is larger, clearly the dominant thumb.

Radial polydactyly

Here are other pictures before surgery from top and bottom.
Radial polydactyly from the palm view.

Radial polydactyly from top view

In surgery, we removed the outer, smaller thumb and realigned the remaining thumb with a cutting of the bone.  We also created a new ligament to support the thumb.  The metal pin is left in place for about 6 weeks with a cast.

The thumb after reconstruction for radial polydactyly.

Another view after reconstruction for radial polydactyly

Here is the patient/ thumb about 6 months after surgery.  He has fully incorporated the thumb into daily use. The thumb is stable and has reasonable motion.  Importantly, despite a very successful surgery for radial polydactyly, when we compare the thumbs, the smaller size of the new thumb is clear.  That is why some of us prefer the term 'split thumb' which emphasizes that even after surgery, the thumb will be smaller.  However, the thumbs will typically look great and unless directly compared as in the pictures, this size difference does not affect function and does not dramatically affect appearance.
Smaller thumb after radial polydactyly reconstruction.

Thumb after reconstruction for radial polydactyly

I typically follow patients for a few years after surgery to assure no early problems develop and, of course, welcome families to come back anytime if issues are noted.  Overall, reconstruction for radial polydactyly is usually a very successful surgery providing a highly functional thumb which works well and looks near normal.

Charles A. Goldfarb, MD
My Bio at Washington University
congenitalhand@wudosis.wustl.edu



Saturday, June 16, 2018

Families, Choices, and Untreated Triphalangeal Thumbs

One of the benefits to my taking care of kids with birth anomalies of the upper extremity is the opportunity to really come to know the patient and the family, often over many years.  This is magnified when more than one child is affected.  This is one of the best things about my practice (and one of the limitations of the other parts of my practice)- that is treating a patient and family over years.  I really enjoy watching kids grow, learning about their development, and understanding how their birth anomaly does or does not affect their lives.

Another interesting opportunity is talking to and understanding the experience of an affected parent.  This is critically important because the experience of the affected parent influences their goals and hopes for their child.  It also usually moderates their concerns.  What I mean by this is that most parents who are also affected understand that their child is likely to be highly functional and also likely to be well adjusted

Kids with birth anomalies never cease to amaze us and it takes time for 'new' or unaffected parents to realize just how well their child will do and how many expectations will be exceeded.  Affected parents simply better understand their child's status and have appropriate expectations for the future.  

One particular family has become particularly special to me as I have come to know the whole family and done so over many years.   Mom and the kids have triphalangeal thumbs together with an extra thumb.  Mom's triphalangeal thumbs were untreated (although the extra thumbs were removed when she was a child) and she understands the diagnosis and its implications very well.  We have performed surgery on her kids due, at least in part, to mom's understanding of how surgery would be helpful.

A few features to take note of.  
1) The thumbs are long.  This is due to the extra bone in each of the thumbs (triphalangeal- 3 phalanges instead of the normal thumb with 2 phalangers).  
2) The thumbs appear thin, almost like a finger.  
3) The thumbs do not rest in the normal position.  The thumbs are more in the plane of the fingers- some might call this a 5 fingered hand rather than a hand with 4 fingers and a thumb.

Adult with triphalangeal thumbs.

Adult with triphalangeal thumbs, palm side

Adult with triphalangeal thumbs, thumb flexion.  Note the length.

Adult with triphalangeal thumbs. Note that the thumbs are in the plane of the fingers.

Adult with triphalangeal thumbs, right
Adult with triphalangeal thumb, left























Nonetheless, the patient has very good function.  There are a few challenges with fine motor skills and pinch.  This video shows thumb motion and gives a sense of function.






A few relevant blog posts:

The bottom line is that patients with a triphalangeal thumb function well with or without surgery.  However, surgery does offer improved function and appearance as confirmed by this family and the choices they have made.

Charles A. Goldfarb, MD
My Bio at Washington University
congenitalhand@wudosis.wustl.edu