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. 

Precautions
-       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              

email: congenitalhand@wustl.edu

Please CLICK HERE to support our research.  
Designate my name.  Thank you!

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     
email: congenitalhand@wustl.edu

Please CLICK HERE to support our research.  
Designate my name.  Thank you!



Thursday, December 5, 2019

Not the typical hand deficiency- ulnar cleft hand

A patient with a thumb and three normal fingers has a hand that functions at a near normal level and looks great.  The narrow hand caused by a lack of a single finger (as long as the others are well aligned and no significant gaps exist) is not easily detected by others- it looks good. 

The choice to remove an abnormal finger in a child is never easy.  All of us have a tendency to want to maintain all the fingers even if one is notable abnormal.  The issues of appearance and of function must be considered.  A finger that looks different that does not interfere with function is very different than a finger that interferes with function.  To me, a finger that interferes with function is one that should be reconstructed and if it cannot be reconstructed, it should be removed.

Sometimes families prefer that the child play a part in the decision- making on digit removal.  I understand this concept but families should understand that most children will need to be at least 10 years of age before that type of decision- making can realistically take place.

Another scenario is around eastern cultures that prioritize a hand with 5 of digits.  To those in Japan or China, the abnormal finger should be reconstructed due to the extreme priority of keeping all digits.  This is true even if the finger does interfere with function.

Consider this patient.  His abnormal finger was kept in place until he decided, as a teenager, that it was getting in the way.  It was caught on things and simply interfered with activities.  At first glance, this looks like a very abnormal extra digit.  In reality, this is a very rare form of cleft hand with a deep cleft between the ring and little finger and with an abnormal little finger  After much thought and discussion, the patient requested excision and has been very, very pleased with the functional improvement and appearance.

Patient with ulnar cleft hand with very abnormal 5th finger.  The thumb is not visible in this picture.

Another view of abnormal fifth finger in ulnar cleft hand.

Patient after removal of abnormal finger in ulnar cleft hand.

Patient with abnormal 5th finger removed in ulnar cleft hand.  The palm is narrow.

The hand is narrow and small through the palm.  The wrist is wider than the palm.  Nonetheless, the hand has been functioning better and certainly looks improved compared to function and appearance before the surgery.

So how does this happen?  During limb development (see HERE for review), there are 3 developmental axes.  The apical ectodermal ridge is the one that is a problem for this patient- disruption leads to the cleft.  Two important manuscripts in hand surgery review this issue:

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

Please CLICK HERE to support our research.  
Designate my name.  Thank you!



Saturday, October 26, 2019

Hand Camp 2019- St. Louis Shriners Hospital

I realize that I post about Hand Camp nearly every year.  Our St. Louis Shriners Hand Camp is really something special.  But I do so for good reason- it is just so impactful for kids, families, staff, and me!  Being outside the hospital setting allows a different kind of conversation and allows a different perspective for everyone.  Kids and families being with other kids and families with similar challenges is simply something that the hospital environment cannot reproduce.  Plus the activities at Hand Camp as shown below are fun, build confidence in the kids, and provide a similar sense of confidence for parents.  The parents come to really understand that their child truly will not have limits.  The group sessions allow honest conversation amongst parents and allows parents to ask our teen counselors questions- who better to provide a honest perspective than a teen or young adult who is living with similar challenges?

There a handful of Hand Camps throughout the US, some bigger than others but most with similar philosophies.  Most of these are hospital based and typically free for participants and families (ours certainly is).  Some are run through organizations:

Camp No Limits

Hands to Love

Nubability

Here are a few pictures and videos to inspire us!

Hand Camp nature hut with guinea pig.

Shriners Hand Camp team.

Hand Camp.  Dream Team.



Shriners Hand Camp climbing wall

Inspirational video of the climbing wall.




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

Please CLICK HERE to support our research.  
Designate my name.  Thank you!

Sunday, October 20, 2019

Nerve Injury after Trauma

The blog is mostly about kids born with differences in the arms (and occasionally legs).  However, my practice also includes kids with sports injuries and other traumas.  I will occasionally post interesting findings on these topics as well.  This is a child with a nerve that is not working correctly after an elbow fracture.  Specifically, this child sustained a fracture of the humerus (supracondylar humerus fracture) as shown here.



This patient was treated with a closed reduction (bones put back in place without making a skin incision) and pins were placed to keep the bones stable as healing started. 

Supracondylar humerus fracture after fixation


It is important to understand the patient's physical examination prior to surgery- this patient had a median nerve palsy.  That is, the median nerve was stretched and not functioning perfectly.  There were signs of the muscles/ tendons not working and signs the feeling in the fingers was not normal. 

This is not expected to improve during surgery and typically the nerve recovers over months after the surgery.  We typically watch and wait as the nerve recovers. 

This patient is now 8 weeks after surgery.  The bone is healed.  Elbow motion is almost normal.  BUT, the median nerve is not working perfectly- specifically, the FPL and FDP to index fingers are not strong as she demonstrates in this amazing video.




Also, this picture helps with an understanding of the change in feeling- see the skin changes on the index finger.  The other helpful finding on examination is the lack of sweat on the index finger- without the nerve working well, the finger is dry.

Skin changes with median nerve palsy

To be clear- this patient has a median nerve palsy (bruising).  This injury affect muscle and feeling.  Sometimes, there can be a AIN (anterior interosseous nerve) palsy which only affects the muscles (specifically, the FPL, the FDP to index and middle, and the pronator quadratus).  Patients with an AIN palsy have normal feeling.  Or, if the whole median nerve is affected, there are changes to the muscle and to the nerve that affect feeling. Anatomically, the AIN is part of the median nerve at the elbow and then, typically around 5 cam past the joint, the median nerve branches the AIN as a separate nerve.  The AIN is susceptible to injury because it sits in the back of the nerve, closest to the bone and, in this case, closest to the fracture.

At this point, we will watch and expect the nerve to get better over the next few months.  Very rarely, the nerve will not perfectly recover and further assessment with nerve studies can be helpful to understand the nerve's recovery.



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

Please CLICK HERE to support our research.  
Designate my name.  Thank you!

Saturday, October 12, 2019

Activity Specific Prosthetics

This is a brief post on activity- specific prosthetics.  There has been much controversy on the timing and appropriate use of prosthetics in kids.  I have previously blogged about this several times as can be seen HERE.  I really appreciate activity specific prosthetics.  The child is engaged in the process based on a demonstrated interest and the specially designed and fitted prosthetic makes a difference.  This idea, compared to the 'old- fashioned' idea that every kid is fitted with a general prosthetic at 6 months of age, just makes so much more sense!

This is a great example of a really helpful tennis specific prosthetic.  It allows an ability to more easily play tennis including serving.
Activity specific prosthetic.  This one is designed for tennis to allow serving.  

Here are a few other activity specific prosthetics.  I took these pictures in the lab and the prosthetic is not typical (harness included) but demonstrate the point- easily changeable prosthetics that have a specific function.  These are durable, functional, and appreciated by kids and families.



Voluntary closing prosthetic.  

Activity specific prosthetic.  Associated with harness (not typical).  This one can be used for weight lifting, etc.

Activity specific prosthetic.  This one is designed for riding a bicycle.  

Activity specific prosthetic.  This one is designed for... yes- lacrosse. 

Activity specific prosthetic.  This one is designed for volleyball. 

Activity specific prosthetic.  This one is designed for volleyball (other view)

I will close by saying- search the internet.  There is a great deal of information and pictures out there on what is possible.  Many companies will work with you for sports and activities.


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

Please CLICK HERE to support our research.  
Designate my name.  Thank you!




Sunday, September 29, 2019

Untreated Adult Hypoplastic (Small) Thumb

On occasion, an adult comes to my clinic for evaluation of one issue and a previously unrecognized issue is identified.  This type of presentation can come in different varieties and, due to my particular practice type, often we find a birth anomaly that has not been recognized or treated.  This patient is one very interesting example.  She has a left hypoplastic thumb.  There is an appearance difference and a functional difference yet it has not caused major problems for her.  Her right thumb and hand are dominant and the left hand and thumb are used for assist (as we all function).  Her thumb is weaker and more lax and has decreased motion.  But none of these problems were completely troubling to her and she certainly is not interested in treatment at this point. 

From the hand surgeon perspective, she has a classic small thumb on the right (technically a hypoplastic thumb- Type IIIA).  It is thin (and seems long but that is really only because it is thin).  She has small muscles at the base of the thumb (thenar muscles), a decreased web space (between the index and thumb), laxity at her MCP joint, and decreased thumb motion.  In other words, she checks all the boxes for a classic hypoplastic thumb.

These videos demonstrate many of these classic findings for hypoplastic thumb and hopefully are helpful to better demonstrate the limitations of her left thumb.  While I see many children with this condition, it is a little bit more tricky to capture the presentation on video!








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

Please CLICK HERE to support our research.  
Designate my name.  Thank you!