Friday, January 24, 2014

The Hand in Arthrogryposis

Hand function is clearly vital for function.  As I have written about previously, the thumb is key for both fine manipulation (think picking up a coin) and large object grasp (soda can).  The fingers are key for grasp and strength in grasp.  http://congenitalhand.wustl.edu/2013/11/5-finger-hand-follow-up.html

Hand function in arthrogryposis can be a challenge.  First, in order to effectively use the hands, the arm has to be able to position the hand in an appropriate position.  We take that for granted but in the child with arthrogryposis, this is not so straightforward.  A stiff elbow and an internally rotated shoulder and a flexed wrist all make using and positioning the hand difficult.  So, before we specifically address the hand in children arthrogryposis, we have to correct the more proximal deformities.  This concept is especially important if there are severe hand issues because the more severe the issues, the more likely the child will need to use both hands to perform tasks (rather than being able to perform one handed tasks).  So, as I have previously written, we externally rotate the arms, we work to release the elbow, and we extend (or straighten the wrist).  


The wrist surgery probably should be discussed a bit more as it relates to finger function.  Many children with arthrogryposis have a flexed wrist position.  Sometimes this can be helpful for weight bearing as the child moves around but often, the flexed position only gets in the way.  To understand how difficult the bent wrist position can be, try this.   Bends your wrist down and try to make a fist with your fingers- it is really hard.  If we can bring the wrist up with surgery (splints rarely work), the fingers move better.

In my opinion, the finger are perhaps the most difficult aspect of the arthrogryposis upper extremity. There is often little we can do to make the fingers themselves work better.  However, if we can better position the arm and put the thumb and fingers in the best possible position, it can definitely help function.  The goals are to put the thumb in a position to allow larger object grasp and to remove it from a position that may block function of the fingers.  The thumb in arthrogryposis
If there is camptodactyly of the fingers, we straighten the fingers for the same reasons.  These surgeries are often a challenge as the skin may be insufficient (requiring skin grafts or flaps), the tendons may be short, and, as always, the muscles may be weak.  Here is a child with a markedly clasped thumb and with severe camptodactyly.

Here is the top of the hand in arthrogryposis with the ring and small fingers straighter.  You can't even see the thumb.

This is the palm sided view of the same arthrogrypotic hand.  Note the middle finger is markedly bent, as is the thumb.

Here we are trying to straighten those two digits, with little luck in this severe arthrogrypotic hand.

Here is an immediate after surgery picture.  We have lengthened tendons, moved skin, released joints and more.  We were very pleased with this early position in a difficult arthrogrypotic hand.


As always, please post questions if I can clarify further.  Thank you.

Charles Goldfarb, MD.

17 comments:

  1. My hands look very much like your second pic and I have never had surgery. I am 39 years old and can type 112 wpm and do anything anyone else can do. I had an orthopedist in Jax, Florida take pics of my hands because he wanted to show that AMC was not as debilitating as some thought. For me, NO surgery was the best decision my parents ever made. It allowed me to learn to adapt to the way my hands were and do everything everyone else did without the loss of use. While I do not use my middle fingers or my thumb on my left hand, I have NO limitations in use at all.

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    1. Lora, thank you for your thoughts. I am happy to hear how functional you are! Unfortunately, not all children with arthrogryposis are so lucky. Every patient with arthrogryposis is different, each has different abilities and challenges. At our institution, we tend to see children more severely affected and limited by arthrogryposis.

      Your point is very important- we try to assure that any surgery that we consider will address a specific functional limitation. This patient's issues related to poor finger motion in general but specifically a lack of extension of the thumb and the long finger. Both impeded function.

      Thanks again for your post.

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  2. What can be done for fingers (AMC) that are all stuck in extension - all stiff and weak?

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    1. Lorene, thank you for the question. Unfortunately, there is no easy answer. I personally believe that stiff, straight fingers are the most difficult aspect of care for the upper extremity in AMC. The fingers don't move because the muscles are underdeveloped and fibrotic. While in other conditions we may be able to move muscles or tendons or release tight muscles, in AMC, we have very few options. So, for me, therapy is the primary treatment. The earlier therapy is begun, the better.

      Thank you again.

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  3. My daughter has arthrogryposis of both hands one with just the middle finger in a flexed position as in photo #2 , the other hand has overlapping fingers. She is 3 months old so her fingers are very flexible, with splinting they have become quite looser, however I am concerned about her finger strength. She is able to grasp my finger but it is not very strong is this something that can become stronger with time/therapy.? Please let me know your thoughts

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    1. Thank you for your question. First, and most importantly, is the flexibility of the fingers. I would encourage you to continue to work very hard to keep and even gain more motion. Motion is, clearly, vital for function and now is the time when gains are possible. Strength is a function of motion and the quality of muscle (which is compromised). With time and therapy, there will likely be some gains in strength but certainly, there will be limits.

      Good luck.

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    2. Thank you so much for your advice! I have one more question, just recently her index finger has become stiff in a flexed open position in the hand with the bent digit #2. it was my understanding that arthogryposis does not get worsen as child grows. Could it have been injured? Therefore temporary? She has been seeing the OT every 2 weeks, the OT said we just need to massage it and it will be fine but I am not seeing any change. Please let me know your thoughts. I should note I work full- time so I am not sure how much she wears her braces at the babysitter. Is this a case of over bracing? Or worsening arthogryposis?

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    3. Nikki,

      I am not sure how to explain that. It would be very unusual for a finger that was previously mobile (or at least not stuck) to become stiff in a flexed position. But typically, we would recommend what you are already doing- stretching and splinting. Of course, other diagnoses are possible (although not terribly likely).
      Good luck.

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  4. My son has arthogryposis, his fingures on both of his hand are very flexible. His thumbs are my concern.on one hand his thumb is inverted just like the picture, and on his other hand not as bad, he can use his thumb a little. I have done therapy splints. My question is can something be done to make his thumbs a little bit more mobile? He has ajusted fine to his hands and does things in his own way. His main plrblemb is just the function of his thumbs.

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    1. Valerie,

      Thank you for the question. The fact that your son has good finger motion is incredibly important and it means that he will have very good function. If the thumb is "tight", there are indeed surgeries (as noted above) that can help either improve motion or allow a better positioning of the thumb. I hope that helps, good luck.

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  5. Do you have an update on how the fingers are working and look now that they are healed? My son is now 10 and is becoming very aware of his hands being different. His hands look almost exactly like the child in these photos.

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    1. Hello. I do not have any updated pictures but this patient (and family) are pleased with the finger and thumb position. Function is better with an easier time grasping large objects and manipulating objects as the two most important improvements.

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  6. Hello my name is Osbel, I live in Canada. I have a daughter in Cuba with arthogryposis but her problem is in shoulder, elbow and hands. I will like to know how can I try her sickness.she is just 2 years old. And in my country the doctors don't have much experience in arthogryposis. Thank you. I going to be glad with you guys If somebody help me with information about this. Thanks again.

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    1. Hello. I am not sure where you live but the Shriners Hospital in Montreal likely has a good experience with arthrogryposis (as other Shriners Hospitals in the USA). Otherwise, seek a Childrens Hospital with experience. Good luck.

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  7. My name is Robert im from Papua New Guinea me and my wife Noelene recently welcomed our fifth(5) child to this date 05/01/2016 the she is 6months old and has problems with her wrist and elbow causing her fingers to fold back to her palms especially the right hand, please advise on what we should do and where we should go to seek treatment or a specialist and where to have surgery

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    1. Robert,
      Congratulations on the birth of your 5th child. I am not sure of the diagnosis and some extra flexibility can be temporary in childhood. However, I agree that this seems like more than normal. While surgery may not be needed, a pediatric orthopedic surgeon or hand surgeon can give you more information. Good luck!

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    2. Thank you very much for the advise and great news I will advise you on my daughters progress
      Thanks
      Robert

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