Saturday, December 1, 2012

The Wrist in Arthrogryposis

Arthrogryposis is different in every child.  While there may be a classic appearance- internal rotation of the arms, elbow straight (extended), wrists flexed, and limited finger and thumb motion- the variability between kids makes treatment a challenge.  I have previously commented on arthrogryposis and general treatment concepts (my own thoughts guided by my experience and the experience of others): http://congenitalhand.wustl.edu/2012/06/arthrogryposis-general-thoughts.html

The wrist is almost always flexed.  The position of wrist flexion is different for each child and the effect of the flexion is also different for each child.  However, anyone who flexes their own wrist realizes how much that affects the ability to move the fingers.  When the wrist is flexed, it is impossible to make a tight fist. Other factors compound this difficulty.  First, obviously, the muscles are limited in arthrogryposis- this also affects finger motion.  And second, the functional limitations of arthrogryposis make two- handed activities important.  The flexed wrist position makes it difficult to use both hands together.

Therefore, the concept of straightening the wrists makes sense for a number of reasons.  First, the straighter wrist position allows the best finger function.  A change in wrist position cannot overcome the limitations of the muscles in arthrogryposis- but it can help the child function as best as possible.
Second, a straighter wrist allows the hands to work together.  Because of the overall limitations in function, using both hands together is vital, made even more important in kids severely affected.

Technically, the surgery of closing wedge osteotomy (osteotomy= cutting the bone) of the wrist removes a wedge of bone from the top of the wrist bones (the dorsal carpus).  By removing a triangular wedge of bone, the wrist is brought into a straighter position.  Additionally, the radiocarpal joint (one of the places which allows wrist motion) is preserved, thus allowing wrist motion.  So this is NOT a wrist fusion.  Other parts of the procedure may include tendon release (if tight on the palm side of the wrist) and also tightening or centralization of the tendons on top of the wrist.  Patients and families have been very happy with this surgery.  It improves function and also improves the appearance of the wrist.

Arthrogrypotic wrist before surgery

Arthrogrypotic wrist after osteotomy
Arthrogrypotic wrist after surgery.  Note small incision.  Pins are visible and can be removed at approximately 6 weeks.



The Power of a Positive Outlook

It is amazing how much my patients teach me every day.  The power of positive thinking and the power of a positive outlook mean so much.  This newspaper piece highlights this attitude.

http://www.ajc.com/news/ap/florida/sixth-graders-turns-tables-on-bullying/nTBgZ/


Tuesday, November 20, 2012

Unusual Combination

We have come a long way in understanding congenital hand and upper extremity abnormalities.  Dr Swanson, more than 40 years ago, published a classification system that was adopted by most of us who treat patients with birth anomalies.  This classification system was based purely on appearance of the extremity but allowed us to communicate better as well as advancing the field through research.  Recently, Drs Oberg, Tonkin and Manske suggested another classification system still based on appearance but a bit more refined and improved through the last 40 years of research.  Eventually we will have a system based not on appearance (phenotype) but based on the genetics (genotype).  We have a ways to go.

Our research has allowed us to better understand the origin of many anomalies, but certainly not all.  The more we learn, the more we can give good advice to families on 1) associated conditions to look for in their child http://congenitalhand.wustl.edu/2012/04/diagnosis-and-evaluation.html 2) the risk to future children and 3) the risk to the affected child's future children.  We also want to give rest to mothers' (and fathers) fears that they caused the problem: in almost all cases, this is simply not the case.  The more I learn about how limb forms, the more amazed I am that it ever happens "correctly."  There are so many signals and responses and molecules and proteins- simply amazing.

Combination of cleft hand and forearm synostosis

another X-ray of cleft hand and synostosis


I present only one example in this post.  This is a very rare combination of a cleft hand http://congenitalhand.wustl.edu/2012/01/central-deficiency.html and a forearm synostosis http://congenitalhand.wustl.edu/search/label/Synostosisin an otherwise healthy child.  The child has been evaluated by genetics on several occasions without identification of any know syndrome or abnormality.  The cleft hand appears as a thumb and pinky only.  The other hand is normal.  The forearm does not rotate as bone unites the proximal portion of the radius and ulna.  Why do these 2 anomalies happen together?  It is unclear.  The cleft hand usually only affects the hand plate.  The forearm synostosis often happens in isolation.  I believe it is safe to say that the two anomalies happened together for 1 of 2 reasons.  First, it may simply have been timing.  The forearm bones may have been separating at the same time the central digits were forming- one insult at that critical time caused both.  Alternatively, a key signal may not have happened affecting both areas and thus causing both problems.  At this point, the error is not clear and we know formation is complex. http://congenitalhand.wustl.edu/2012/10/limb-formation.html One day it might be.  I should also point out that these unusual combinations often teach us a great deal because it is unlikely to be coincidence.  Unlikely to be lightning striking twice in one unfortunate arm.

Wednesday, November 14, 2012

Carpenter Syndrome

Carpenter Syndrome is one rare type of acrocephalopolysyndactyly, Type 2.  It is closely related to acrocephalosyndactyly, with the most notable type of this group of disorders being Apert syndrome.  These syndrome all have craniosynostosis (premature fusion of the suture lines of the skull) and syndactyly, with or without polydactyly as well.  Carpenter syndrome has been further described at the Online Mendalian Inheritance in Man- http://www.omim.org/entry/201000  and http://www.carpentersyndrome.com/links.html

I have not cared for a child with Carpenter Syndrome but have had the recent pleasure of meeting and caring for a delightful young adult.  This patient had previous surgeries to address the polydactyly and syndactyly with primary current complaints of deformity of the fingers and the thumb and a narrow first web space of the thumb.  The finger deformities affected the long and ring fingers, the site of the previous syndactyly (likely bony or complex syndactyly), previously treated at a young age.

Narrow first web space in Carpenter Syndrome.

Finger appearance in Carpenter Syndrome appearance after syndactyly reconstruction in the past.  Note especially the deformity of the ring finger.  The long finger is also angled but more difficult to see in this picture.

Carpenter Syndrome xrays.  Not that the middle phalanx bones are not rectangular- leading to deformity.

The thumb in Carpenter Syndrome.

At the patient and family's request, surgery was done to 1) straighten the long and ring fingers (clinodactyly deformity) 2) straighten the thumb (fusion of the thumb IP joint) and 3 deepen the thumb- index web space.  Surgery was successful in meeting these goals.  Below are pictures immediately after surgery.
Clinodactyly correction in Carpenters syndrome.  The finger alignment is markedly improved but there is still some deviation in the long finger.


Lateral xray (side view) showing improved alignment after clinodactyly correction in Carpenters Syndrome

Clinical photograph after surgery focusing on the deepened web space.

View from palm after Carpenter's syndrome correction.

Demonstration of straighter fingers after clinodactyly correction in Carpenters Syndrome.


Friday, November 2, 2012

Clavicle Pseudoarthrosis


Clavicle pseudoarthrosis (meaning fake joint) is a rare condition.  It occurs when the normal growth centers of the clavicle do not join (fuse) during development.  It is almost always right sided, a fact which is may be related to pressure from the subclavian artery.  Clavicle pseudoarthrosis is generally painless but it causes a large prominence in the center aspect of the clavicle- it can be functionally limiting.
Clavicle pseudoarthrosis.  Note the prominence on the left side compared to right.
Another view of clavicle pseudoarthrosis
The x- ray in clavicle pseudoarthrosis shows a "joint" or separation in the midportion of the clavicle.

Two x- rays showing clavicle pseudoarthrosis


Treatment for clavicle pseudoarthrosis is based purely on symptoms.  If the bump does not interfere with function (complaints may be related to backpack straps or sports), then observation is reasonable.  If the bump is causing a problem or if painful, operative intervention can be considered.  Surgery removes the interval between the bone ends (i.e., the fake joint) and the bone ends are approximated and held in place with a plate and screws. The bone typically heals uneventfully.  Pain and deformity can reliably be treated.  Complications with surgery are rare.

X- ray demonstrating clavicle plating in pseudoarthrosis

After reconstruction for clavicle pseudoarthrosis
Another view after reconstruction for clavicle pseudoarthrosis



Tuesday, October 30, 2012

Central Deficiency with 2-3 fingers

Central deficiency (cleft hand) is classically as I have shown in previous blogs.  http://congenitalhand.wustl.edu/2012/05/cleft-hand-surgery.html We have previously published our experience with the classic central deficiency, that is the hand with a limited thumb- index web space, 3 fingers (index, ring, and small finger), and a large cleft http://www.ncbi.nlm.nih.gov/pubmed/18984341

However, there are many different varieties of cleft hand, as previously classified by Dr Ogino in Japan and Dr. Manske here in St. Louis.  Dr Ogino's classification is based on how many digits are missing and Dr Manske's classification, http://www.ncbi.nlm.nih.gov/pubmed/7594304  is based on the thumb web space- which is important for function.  If the thumb- index web space is limited, the overall hand function will be markedly limited.  The child will struggle to grab large objects but also will find it difficult to manipulate smaller ones.

With less common types of central deficiency, surgery is sometimes completely avoided or may be minimized.  Consider these 2 examples.


1)
A conjoined thumb with pinky finger in cleft hand.  Note the small web space.

Cleft hand with difficulty grasping large objects.  The thumb is not positioned to best grasp or pinched (i.e., not opposed to fingers)

Cleft hand xray.

We will likely help this child's function with 2 procedures, one to slightly deepen the web space to allow larger object grasp and a second to rotate and reposition the thumb in a position to better pinch and grasp against the pinky finger.

2) Consider the next child with a very similar hand.  A merged thumb and a single other digit.  However, the difference is that the cleft is deeper allowing the child to grasp larger objects and the thumb is opposed.  Look how the child can grasp with the thumb against the finger with a pen and with cheerios.  Surgery is unlikely to help.  Additionally, one might consider narrowing the thumb but I would argue against that procedure as I think it would hurt function and would be unlikely to help appearance.

A second child with similar cleft hand.

Cleft hand with a well- opposed thumb easily manipulating a pen.

Also able to easily manipulate a cheerio.  Note the position of the thumb against small finger.



Wednesday, October 24, 2012

Hemihypertrophy

Hemihypertrophy is a difficult diagnosis due to its rarity and the variety of associated conditions.  Basically, the diagnosis means that one extremity (or one side of the body) is enlarged compared to the other side.  As little as a 5% difference can be diagnostic but usually families seek care when the difference is more notable.  It can be dramatic.  As a hand and upper extremity surgeon, I typically see kids with one arm or one part of the hand which is larger than the other.  There is some information on the web which is useful including this website: http://hemisupport.com  The most commonly associated conditions are listed below (and there are others as well); not all patients will have a syndrome but we do assess for the following conditions.  I have only briefly summarized these conditions with links for more information.

Beckwith- Wiedemman syndrome is one possible diagnosis and its diagnosis is important to make because of possible associated tumors.  http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002168/

Proteus syndrome is an overgrowth condition affecting skin, bones, soft tissues, and other organs.  It may be asymmetrical.  This condition is exceedingly rare (1 in 1,000,000) and is related to a genetic abnormality (not inherited).  http://ghr.nlm.nih.gov/condition/proteus-syndrome

Klippel- Trenaunay Weber Syndrome.  KTW is characterized by port wine stains on the skin, extra bone and soft tissue growth, and varicose veins.  Classically associated with vascular malformations.   http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001205/

Neurofibromatosis is a condition with numerous nerve tumors which may grow rapidly.  Type 1 is more common and is associated with cafe au lait spots on the skin.  http://www.ninds.nih.gov/disorders/neurofibromatosis/neurofibromatosis.htm

 The orthopaedic surgeon or pediatrician often makes the diagnosis of hemihypertrophy but a geneticist is usually involved for full evaluation of the possible associations.  My role in hemihypertrophy is to provide information, refer families to geneticists and other providers, help make the appropriate diagnosis, and arrange referral or testing to rule out the rarely associated tumors (such as Wilms tumor).  Surgery for hemihypertrophy can sometimes be helpful to control growth (by limiting the growth plates) or to debulk enlarged areas.  Sometimes amputations are the best course of action.  Surgery is a difficult decision as our options are not ideal and the effectiveness of surgery is also limited.

One example of hemihypertrophy


Sunday, October 21, 2012

Too much about me

I have been asked on several occasions by patients (or prospective patients) to provide more information on the blog about my professional career.  Enclosed, please find a variety of links including local and regional publications and videos.  I have included links to several books and articles.

First, my basic bio from Washington University
http://www.ortho.wustl.edu/content/Patient-Care/2637/Find-a-Physician/Listing-of-Faculty/Charles-Goldfarb-MD/Bio.aspx


Others:
1) Great Story on a Child born in Central America with 3 arms
http://www.ksdk.com/video/97712837001/0/Child-born-with-three-arms-to-have-more-surgery-

2) Peer Reviewed Publications as of October 6, 2012
http://www.ncbi.nlm.nih.gov/pubmed?term=goldfarb%20ca

3) Links to Published Books
- Hand and Upper Extremity Therapy: Congenital, Pediatric and Adolescent Patients.  Saint Louis Protocols.
http://www.amazon.com/Charles-A.-Goldfarb-MD/e/B0069RCATM

- A Pocketbook Manual of Hand and Upper Extremity Surgery: Primus Manus
http://www.amazon.com/Pocketbook-Manual-Upper-Extremity-Anatomy/dp/1608314669

- Tendon Transfer Surgery of the Upper Extremity: A Master Skills Publication
http://www.assh.org/Professionals/ProdsSvcs/Store/Pages/MS_Tendon_Transfer_ebook.aspx

4) St Louis Medical News
http://saintlouismedicalnews.com/content/physician-spotlight-charles-goldfarb-md

5) Ladue News
http://www.laduenews.com/living/health-wellness/washington-university-orthopedics/article_9aa0bbcc-0337-11e2-8116-001a4bcf6878.html

6) St Louis Children's Hospital Trauma Services Interview
http://www.stlouischildrens.org/media-center/video-library/physician-profile-charles-goldfarb-md?page=2

7) St Louis Children's Hospital Doctors Digest on Trauma
http://www.stlouischildrens.org/media-center/video-library/physician-profile-charles-goldfarb-md?page=2

8) Best Doctors in America, 2007-2012
http://www.bestdoctors.com/us/What-We-Do/For-Physicians/Best-Doctors-in-America-List.aspx

9) American Society for Surgery of the Hand (ASSH) Board of Directors (Council)
http://www.assh.org/Professionals/AboutASSH/GovernanceAdministration/Council/Pages/default.aspx


Severe Ulnar Deficiency

Ulnar deficiency, as described in a previous post has characteristic findings at the elbow, forearm, and hand.  http://congenitalhand.wustl.edu/2011/09/ulnar-longitudinal-deficiency-basics.html

Most classifications describe 4 types of ulnar deficiency (or ulnar longitudinal deficiency- ULD).  These classifications only address the bony deficiency (and not the important other issues such as muscles, tendons, nerves, etc).  While there are a number of classifications, the basic description is as follows:

Type I is a slightly short ulna
Type II is a notably short ulna
Type III is an absent ulna
Type IV is a completely absent ulna with fusion of the radius and the humerus.

We have helped to extend the classification in both directions.  When the ulna is normal and there are only abnormalities of the hand (such as metacarpal synostosis, absent digits, etc) , this can be described as a Type 0 ulnar deficiencyhttp://www.ncbi.nlm.nih.gov/pubmed/16344190

On the other end of the spectrum, some kids have a severely shortened extremity.  Traditionally, these children have been classified as having a phocomelia.  Phocomelia (which will be the topic of another post) technically implies a segmental deficiency in which, for example the hand is attached to the arm (i.e., humerus) without a forearm.  However, in another investigation we were able to demonstrate that most kids with a diagnosis of phocomelia actually have a diagnosis of either radial deficiency or ulnar deficiency: http://www.ncbi.nlm.nih.gov/pubmed?term=ezaki%20goldfarb%20manske

The importance of making the correct diagnosis cannot be understated, most importantly for 2 reasons: the genetics related to the diagnosis and the associated conditions.  The genetics of a particular diagnosis will allow for information regarding the risks that the parents should understand for having another child with the same diagnosis and also the risk that the patient will someday have a child with the same diagnosis.  The other issue is associated conditions.  Some diagnoses carry a risk for associated conditions such as a heart or kidney problem whereas others do not.  Therefore, making the correct diagnosis allows the ideal medical evaluation of the patient.

Here is an example of severe ulnar deficiency, or Type 5 ulnar deficiency.  Note the short extremity and the three conjoined fingers.

Ulnar deficiency on right

Another view of ulnar deficiency

Note the 3 fingers with syndactyly in ulnar deficiency

Radiograph of ulnar deficiency.  There is one bone which has a split (kind of like a wishbone).  There is no separate radius and ulna.

Saturday, October 6, 2012

Severe Symbrachydactyly and Nubbins

Symbrachydactyly, as noted previously, http://congenitalhand.wustl.edu/2011/12/normal.html  means "short, webbed fingers."  The use of this label is clearly appropriate for some children who (brace yourself for this truth) have short and webbed fingers (i.e., a syndactyly).  The term symbrachydactyly has further meaning as it has implications for etiology (i.e., cause).

I believe symbrachydactyly occurs due to difficulties with the AER and the underlying mesoderm during development- please see previous description of limb development http://congenitalhand.wustl.edu/2012/10/limb-formation.html  If the AER is lost, limb outgrowth stops and there is a deficient limb.  The theory is that early loss (i.e., at 4-5 weeks of gestation) leads to a really short limb and later loss (i.e., at 6-7 weeks of gestation) leads to more subtle symbrachydactyly.  The following children would be considered to have a severe symbrachyactyly- at least the way I consider it.  We have shared our thoughts and experiences with this diagnosis in the Journal of Hand Surgery: http://www.ncbi.nlm.nih.gov/pubmed/17996776

Symbrachydactyly
Nubbins of symbrachydactyly


















Another example of severe symbrachydactyly
The "nubbins" on the end of the arm likely represent some remnant from the cells from development (from the apical ectodermal ridge).  The nubbins can be surprisingly well formed and often have fingernails.  Some families preserve and value the nubbins while others feel that the nubbins get in the way and can be difficult to keep clean.  When families request, the nubbins can be easily excised with a small surgery.  The nubbins certainly do not grow and removal, therefore, is reasonable because function of the nubbins will not get better with age.  I leave these decisions to the family.

Others believe severe symbrachydactyly really should be called a transverse arrest or congenital amputation of the limb.  I don't mind those terms but I happen to believe this is most likely a development issue (a malformation)- meaning that something went wrong during limb development, not after.

The above examples differ from less severe symbrachydactyly:
Short finger type of symbrachydactyly- short, webbed fingers.

Another short finger symbrachydactyly



Limb Formation

I have been remiss in that I have not given an overview of limb formation.  Mind you, this is  a topic that is constantly changing- we learn more each year about the process, the proteins, the timing, etc.  So this is a hand surgeon's perspective in 2012.  There are others with a much better understanding of limb development- people who continue to add to our understanding.  I have enjoyed getting to know Kerby Oberg from Loma Linda, one of those people.  He is a vital part of a congenital hand surgery study group as well.  http://www.llu.edu/medicine/basic-sciences/faculty/anatomy/oberg-kerby.page

The upper limb forms from about 28 days of gestation to about 56 days of gestation- that's right from 4 to 8 weeks of gestation. Many women may not even be aware of pregnancy at that time.  By the 8 week point, the arm and hand are in an amazingly, fully formed state.  There are fingers, a thumb, and joint with a big- picture normal appearance.

So, how do we get there?

The limbs start to form based on signaling mechanisms, genes, and proteins (and everything is about genes, signaling and proteins).  TBX-5 and TBX- 4 are genes that help "turn- on" upper and lower limb formation respectively.  The upper limb is about 2 days ahead of the lower limb.  HOX genes play a role as well.  There are three axes that guide limb development: proximal/ distal, anterior/ posterior, and dorsal/ ventral.

If you imagine that the developing limb is a blob of cells, the center of the blob is mesodermal cells and the outer layer is ectodermal cells.  The complex interaction between these cells guides development of the arm.  The outer layer is called apical ectodermal ridge (AER for short) and it continually interacts with the inner cells of the mesoderm.  Fibroblast growth factors (FGFs) are the key to this interaction.  But importantly, the AER guides limb growth or lengthening.  If you take away the AER, the limb stops growing.

The other key area is on the back side (post axial) of the limb and is termed the zone of polarizing activity (ZPA).  The ZPA interacts with the AER and the developing limb to help determine the development of the different parts- specifically thumb side of limb (radial side) versus pinky side of limb (ulnar side).  This is based on a morphogen called sonic hedgehog (which can be abbreviated Shh).  (lab humor= the name 'sonic hedgehog').  This protein affects development of the fingers, thumb, etc.  Some really amazing experiments showed that if you take the ZPA (or even the sonic hedgehog) and add it to the other side of the developing limb, you can develop a "mirror" limb as more fingers develop.  This is described beautifully in one of my favorite articles: http://genepath.med.harvard.edu/~tabin/Pdfs/Riddle.pdf

This is a mirror hand with 8 fingers.  We believe this happens due to problems or duplication of the ZPA.
And the ZPA interacts with the AER constantly as the limb develops.  Check out the simple drawing (from 12 years ago!) which emphasizes this interaction:

Complex interaction between ZPA and AER, simplified.


http://www.nature.com/nature/journal/v408/n6810/fig_tab/408313a0_F5.html



Finally, Wnt 7a and Lmx-1 help determine the top and bottom of the limb and fingers (ie nail side of finger vs palm side).

I will refer back to this blog on limb development as it relates to anomalies.


Saturday, September 29, 2012

"Simple" Extra Digits

An extra finger on the pinky side of the hand is one of the most common birth anomalies of the hand.  The official name is ulnar polydactyly or postaxial polydactyly.  It is typically seen in African Americans and is usually passed on as an autosomal dominant trait.  OMIM has numerous details: http://omim.org/entry/174200?search=postaxial%20polydactyly&highlight=postaxial%20polydactyly

In caucasians, it is unusual and may be associated with a syndrome, such as Ellis Van Creveld,  among others.  http://omim.org/entry/225500?search=postaxial%20polydactyly&highlight=postaxial%20polydactyly

We can help rule out an associated syndrome.

Small extra digit, a postaxial polydactyly.



Another view of the extra digit prior to surgical excision
Hand surgeons such as myself often do not see these patients with small extra digits because they may be treated in the newborn nursery.  In these situations, the extra digit is "tied off" such that the blood supply is blocked and the finger necroses (turns black) and eventually falls off.  The process is typically non painful and usually is a successful treatment.  The small side effect with this treatment is that there will be a skin lesion at the site of the previous attachment, essentially what appears to be a wart.
Small "wart" after "tie off" procedure for ulnar polydactyly

Another example of skin lesion after a "tie off" procedure for ulnar polydactyly



For that reason, when I am able to see patients with postaxial polydactyly in the office, I offer two treatment options: the office based "tie- off" procedure and a formal surgery.  The surgery is quite small and offers an appearance benefit.  It is a very different surgery than reconstruction of other extra digits, such as radial polydactyly.  http://congenitalhand.wustl.edu/2012/05/difficult-extra-thumbs.html

Either way, most patients with this anomaly do wonderfully.

Thursday, September 27, 2012

September 2012 Travels

It has been too long since I last blogged.  Several catch- up blogs are on the way.  In the meanwhile, I have been to several interesting events in the last month.

First, we had our annual St Louis Shriners Hospital Hand Camp.   There were approximately 18 children with their families, 10 junior counselors, and a variety of therapists and volunteers from the hospital.  This 3- day camp at a rural lodge is an amazing experience for all involved.  The camp participants, those with notable hand deformities, are able to interact with others like themselves, experience new activities such as archery and wall climbing, and learn from the junior counselors.  My favorite part has become the group discussions- one with parents alone and another with parents and the junior counselors.  The exchange of information and advice is amazing!  Here are a few pictures and one video from the weekend.

Eli, a long time patient and friend, demonstrating how a to use a bow with radial deficiency 


The following video shows the same two campers with below elbow amputations (symbrachydactyly) successfully climbing an impressive wall (written and appreciated by someone who struggled up the same wall).  Check it out and look carefully at the climbing technique!


The other event was the annual meeting of the American Society for Surgery of the Hand, in September in Chicago.  As always, the meeting was a great chance for hand surgeons from across the world to congregate and learn from one another.  I had the opportunity to help lead (with Don Bae from Boston) a 4.5 hour Precourse on Congenital Hand Anomalies.  We had a great panel of speakers from the United States as well as Australia (Dr Tonkin) and South Korea (Dr Baek).  The exchange of information was amazing.  The only disappointment was that our room was not large enough to seat the entire audience!

http://www.assh.org/AnnualMeeting/AnnualMeeting2012/program/precourses/Pages/Pre10.aspx


Saturday, July 28, 2012

Symbrachydactyly Web Deepening

Web space deepening is a straightforward surgical technique to provide a deeper space between fingers.  There are several variations of the technique of web space deepening, typically based on the number of skin flaps that are utilized.  These techniques include 2- flap, 4- flap, 5- flap and other variants; precision is required for all.  A variety of congenital anomalies may be surgically addressed with a web space deepening, including a small thumb (hypoplastic thumb) and certain types of syndactyly.

Symbrachydactyly (short, webbed fingers) may present in a variety of different ways.  I have provided some basic information about symbrachydactyly in a previous blog, http://congenitalhand.wustl.edu/search/label/Symbrachydactyly , and one treatment technique- lengthening- in another blog, http://congenitalhand.wustl.edu/2012/06/finger-lengthening.html .

What follows is a description of how a web space deepening can be helpful in symbrachydactyly.  In the first case, the short finger type, function is very limited.  Surgery will not restore normal or even near normal function but our goals should be limited.  Because the opposite hand is almost always normal, the goals for surgery on the affected hand in symbrachydactyly are limited.  If we can even slightly improve function, we can make the hand a better helper hand.  We can help the hand hold bigger objects.  In this case, we used a dorsal flap to resurface the web space between the thumb and the index finger.
Symbrachydactyly, short finger type, after surgery to deepen web space between thumb and index finger.

The second case is more unusual.  This is an example of the cleft type of symbrachydactyly.  This condition is different from a true cleft hand (central deficiency) which is discussed in previous postings:   http://congenitalhand.wustl.edu/2012/05/cleft-hand-surgery.html  and
http://congenitalhand.wustl.edu/2012/01/central-deficiency.html .  In this case, the patient and family complained about his inability to grasp large objects between the thumb and other digits.  After a discussion of the options, the family wanted to deepen the cleft.  While this might make the hand difference more apparent to others (i.e., make it more of a cosmetic issue), both the family and me felt that the functional improvement was worth this risk.
Symbrachydactyly, cleft type before surgery.

Symbrachydactyly, cleft type, after surgery from palm side

Symbrachydactyly, cleft type, after surgery from top of hand


Other treatment techniques for symbrachydactyly must be considered on a patient-by- patient basis.

Sunday, July 8, 2012

Recurrent Radial Longitudinal Deficiency

The best treatment for the forearm/ wrist deformity in Radial Longitudinal Deficiency remains uncertain as I have previously outlined in a previous blog posting http://congenitalhand.wustl.edu/2012/05/form-and-function-in-radial-deficiency.html .  The problem is that the anatomical deficiencies in Radial Longitudinal Deficiency limit our ability to sufficiently correct the underlying deformity.  There is a lack of muscles to extend (bend back) the wrist and a lack of bony support for the thumb side of the wrist and forearm.  The most common surgery to address this issue is a centralization (or radialization) procedure.  The idea is that the tight structures on the thumb side (Radial) of the wrist are released, the ECU tendon is advanced (tightened) to help balance the wrist, and the wrist and hand are placed on the end of the ulna (i.e., centralized).  We like the concept of the surgery as it lengthens the forearm, improves appearance and likely helps function overall.  Obviously, however, if there is severe recurrence, we need to reconsider it.

For some kids with Radial Longitudinal Deficiency, the surgery succeeds in balancing the wrist and the alignment is maintained but for others, the deformity recurs.  And by deformity, typically we mean both radial deviation (wrist and hand angle towards thumb) and flexion (bending down) of the wrist.  There have been many different techniques utilized to try to prevent recurrence of deformity.  These attempts include using a toe (with joint) including its blood supply to provide thumb sided support to the wrist.  This broadens the platform for the wrist and may help prevent recurrence.  However, this procedure is technically complicated and requires the sacrifice a toe without certain improvement in the long term.

An additional technique to minimize recurrence in Radial Longitudinal Deficiency is to use an external fixator prior to centralization.  This stretches the soft tissues and makes the centralization more straightforward and balanced and seems to prevent recurrence.  We have discussed this previously at http://congenitalhand.wustl.edu/2012/05/fixator-for-radial-longitudinal.html .

Ultimately, in those kids that do have severe recurrence after centralization, a fusion of the wrist can be a very effective procedure.  Usually this procedure has to be delayed until at least age 12 when there is enough bone for the fusion.  While the procedure sounds scary, it has many positives (by improving the position) and very few negatives (related to a loss of some motion- motion which is limited anyway).  We like this procedure very much in certain kids.

Here are some clinical pictures showing recurrent deformity in an 8 year old with radial longitudinal deficiency.

Radial Longitudinal Deficiency which has recurred.

Close up of recurrent Radial Longitudinal Deficiency.  Note the marked radial deviation of at least 70 degrees.

Radial Longitudinal Deficiency with recurrence with flexion of the wrist.

Radial Longitudinal Deficiency, recurrent.  Note that the index finger has not been pollicized as there is very poor motion of the index finger.

More information is always available through our website at http://ortho.wustl.edu/content/Patient-Care/3220/SERVICES/Hand-Wrist/Congenital-Hand-Disorders.aspx