Wednesday, June 19, 2019

Severe Camptodactyly

Severe camptodactyly can be a challenge as it will limit function and may cause pain.  This is less common in childhood and more common for the adult with untreated camptodactyly.  I have previously shared thoughts on camptodactyly HERE.

Treatment of camptodactyly in the child begins with therapy.  Depending on the nature of the camptodactyly, this may be curative or may provide sufficient improvement to avoid the need for surgery.  Rhee, et al (including senior author Baek) showed how effective therapy can be, especially in the younger population MORE INFO HERE.  In those patients that remain with functional challenges after failing 3-6 months of therapy, surgery for camptodactyly can be helpful.  We have shared our approach in the Journal of Hand Surgery HERE..  We do not regularly perform a tendon transfer to power extension (straightening) as we fear the finger may be stiff and straight which is a bigger functional challenge than the finger that will not completely straighten. 

The concern with untreated, severe camptodactyly is the development of arthritis at the PIP with permanent stiffness and pain,  That, indeed, is how our patient presented.  He had isolated camptodactyly of the ring and small finger.  The fingers interfered with function given their severe flexion posture.  He did not have other medical conditions and requested surgical intervention.  Unfortunately, as might seem obvious, if there is arthritis, the option of providing additional motion through releases does not make sense.  Therefore we elected to proceed with fusion of the PIP joints in a better functional position with the idea that this would address the pain as well as improve function.
Xrays in severe camptodactyly

Severe camptodactyly of the ring and little fingers

Severe camptodactyly but with maintained flexion of the fingers.





















This patient underwent succesful fusion surgery.


PIP joint fusion for severe camptodactyly


I do not routinely push for surgery in camptodactyly.  I give families that option if therapy fails.  I explain why I think it will be helpful for function (and appearance).  But I never consider it mandatory.  This patient, and patients like him, illustrate the danger of untreated camptodactyly- that is- worsening posture which interferes with function and potentially arthritis at an early age which causes pain.  These risks should be made clear to those families hesitant to consider surgery for camptodactyly (when the patient has failed therapy).

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

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Saturday, June 8, 2019

Distal Arthrogryposis- thoughts and techniques for treatment

Distal arthrogryposis is defined as multiple contractures affecting the hands and feet.  This can include the thumb, fingers (camptodactyly) or clubfoot.  Our understanding of these conditions continues to expand and these sites each provide great information:
Rare Disease site
Genetics site
OMIM


While distal arthrogryposis may be lumped as a diagnosis, the diagnosis can also be subdivided into different types as the links above clarify including Type 1, Type 2B, Freeman Sheldon, and more.  Each type of distal arthrogryposis is somewhat different in presentation, genetics (including identified genes and inheritance pattern), and more.  The severity of involvement of the fingers, thumb, etc vary notably from type to type and patient to patient.  This is what make arthrogryposis patients so difficult to treat and so difficult to predict outcome from treatment.

I have previously posted several times on camptodactyly which means bent finger. 
Post 1
Post 2 and others as well.


The finger is bent through the PIP joint (first joint actually in the finger) and typically in arthrogryposis, can't be straightened. Therapy can absolutely be helpful and is always recommended as the first step.  But surgery might be helpful for function (especially if the bent finger is getting in the way of grasp).  Additionally, it has been suggested that when the finger is left untreated, arthritis can develop and joint deformity can become an issue.

The thumb is often a bigger functional issue due to its flexed position.  It makes large object grasp difficult and can also get in the way.  The function and position of the thumb is always important BUT becomes even more important in kids with severe finger limitations.  Those kids depend on a good thumb for most of their hand function.  I have previously posted on the thumb in arthrogryposis here:
Post 1
Post 2 and others as well.

This child is about 5 years of age.  The pictures do not present his challenges well (I am trying to take more video but...).  His middle finger is bent 90 degrees and stuck.  His thumb is very tight at the MCP joint and his a complex skin contracture as well.



Distal arthrogryposis with tight thumb and middle finger



Distal arthrogryposis.  First web space (thumb space) is tight!

Distal arthrogryposis with tight middle finger (can't be straightened) and tight thumb.

Given the functional limits caused by the distal arthrogryposis and primarily the thumb and middle finger, the family elected to proceed with surgery.  Therapy had provided some, but not enough improvement.  The thumb was flexed and could not be straightened.  The space between the thumb and pointer finger was very tight.  The middle finger was bent and could not be straightened.  

I have posted on different techniques which overlap with treatment here.  I recently posted on our treatment of camptodactylyCamptodactyly Post- and I treated this patient in a similar way.  I have also previously posted on the thumb.  However, in this case, I used a somewhat different flap to resurface and deepen the first web space.   This is a modification of the traditional Buck Gramcko flap for first web space reconstruction.  Abdel- Ghani has written about this and shared his positive results:



I also stiffened (a procedure called a chondrodesis) the thumb MCP joint to help keep the thumb in a better position.  I expect this child will do very well.

Distal arthrogryposis treated thumb and middle finger reconstruction

Distal arthrogryposis treated thumb and middle finger reconstruction


Distal arthrogryposis treated thumb and middle finger reconstruction

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

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