Possible Surgical Treatment for Radial-Ulnar Synostosis?

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Possible Surgical Treatment for Radial-Ulnar Synostosis?

(24 posts)

Started 1 year ago by lizzyc1974
Latest reply from Kim37

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lizzyc1974
Member

Dear Dr. Harley,

My 24 month old son has been diagnosed with Bilateral Congenital Radial-Ulnar Synostosis. He also has a bicuspid aortic valve and Chiari Malformation. We also have just found that my husband also has bicuspid aortic valve (he has not had an arm x-ray yet) and his grandmother had radial ulnar synostosis that was never "officially" diagnosed. No knowledge of any heart problems, but she is already deceased. There is obviously some genetic link here. We have seen a geneticist but have not found anything yet. Are there any syndromes you are familiar with that can encompass these problems? Possibly Holt-Oram?

My other question is regarding possible surgical treatment of radial ulnar synostosis. My son's arms are at about 85 degrees pronation. He is not able to supinate at all. In speaking with an orthopaedic surgeon, I was told that the main treatment option is derotational osteotomy. Is this an option you recommend? He is having some difficulty with use of silver ware, cups etc.

In my own research I came across another method that I am looking for more information on. Are you at all familiar with a technique to treat by Fuminori Kanaya involving a fat graft? The study was "Mobilization of a Congenital Proximal Radioulnar Synostosis with Use of a Free Vascularized Fascio-Fat Graft". I also found another study, by Dr. Neil Jones out of the Department of Orthopedic Surgery and the Division of Plastic and Reconstructive Surgery, University of California Los Angeles, "Treatment of radioulnar synostosis by radical excision and interposition of a radial forearm adipofascial flap". These techniques look like an interesting possibility and I am hoping to find a doctor who can give me more information about the success of these techniques and whether my son would possibly be a candidate for a surgery such as this.

Thanks for your help!!
Liz Holland

Posted 1 year ago #
Alison
Member

MY son now aged 5 was born with a tracheooesphageal fistula/oesphageal atresia,he was also found to have bilateral radialulnar synostosis.He had a derotational osteotomy in great ormond street hospital on his right arm.He is right handed, to position his wrist in slight supination which does allow him to carry out normal everyday activities.My mother in law also has bilateral radialulnar synostosis she only realised she had an actual diagnosis after my son had his xrays and then she was xrayed also,she has just lived with it.My older son has a congenital dislocation of his leftarm only discovered after his brother and my daughter has fibrous fusing of 4th finger of her left hand.My husband has not had his arms xrayed but the surgeon stated he did not have normal movement so there must be some genetic cause?Alison

Posted 1 year ago #
lizzyc1974
Member

Alison,

Were you happy with the outcome of your son's surgery? How bad were his arms prior to surgery, was he able to supinate at all? I am at a loss as to whether to consider a surgery or to let him learn to adapt to the condition. Did doctor's recommend you do the surgery? Both doctors I have seen so far suggest doing nothing, especially in today's computer oriented society. My thought is if there is something I can do to make life easier for him I need to consider it. Tough decisions. Have you had anyone's heart tested yet? We did so upon reading an article that radial ulnar synostosis is often accompanied by heart and kidney problems, like I said before both my son and husband have been found to have bicuspid aortic valve. You might want to check into this if you have not already.

As follow up to my post, I did speak to the doctor in California doing the fat graft surgery. In his opinion some rotation (fat graft) is better than none (derotational osteotomy) but the surgery has better results in those with traumatic RUS than congenital. No guarantees, I guess. I am going to be sending my son's films out for him to take a look at and see if this is something we may be a candidate for.

I have formed a yahoo group for those who would like to share information or support.
http://health.groups.yahoo.com/group/radial-ulnar/

Liz

Posted 1 year ago #
Wajid Jamil
Member

My daughter who is 8+ years has also Congenital Radial-Ulnar Synostosis. I searched net to find a cure for my daughter but it was suggested that surgery should be avoided.

I also took her to Orthopaedics but they advised not to go for operation as she can do a lot of her jobs.

I am not sure how much of degree she can rotate her hands. One of the Orthopaedic surgeon suggested to look for a place where such operations are done, so that if operation is done satisfactorily due to knowledge, expertise and practice.

I live in Karachi, Pakistan. I would like to know if anywhere in the world can it be cured by operation satisfacorily with good results.

Will appreciate feed back.

Regards to all.

Wajid Jamil

Posted 1 year ago #
Tracy1968
Member

Hi Wajid,

My 13yr old son has congenital proximal radio-ulnar synostosis, he is currently under an orthopedic doctor here in the uk.
He has told us that he will monitor him until he stops growing and then decided what action to take,they know of an operation but haven't had much results yet to see how effective it is.
Like you I am looking for answers if there is any.

Anybody out there with some info please get in touch.

Thanks Tracy

Posted 1 year ago #
harleyb
Moderator

Liz,

There is no reliable operation to reverse a congenital radioulnar synostosis. Your child developed this way for 9 months in utero - and no surgery can put back what was missing from this development. Any reports on this are highly experimental - and you would have to look very carefully into what you are getting your child into. The real problem is not cutting the bones apart or putting some tissue in between them - we do this for post-traumatic synostosis all the time. Rather it is trying to make a joint where one has never existed previously. We do not have anything for this currently.

Lack of supination in both forearms can be a problem, and one of the arms could be derotated later in life.

I am not a geneticist by any stretch. While your story sounds like there could be a genetic link, if you have seen a geneticist that is the best advice I can give.

Hope this helps.

Posted 1 year ago #
lizzyc1974
Member

Thanks for your input, Dr. Harley. I am really just searching for as much information as possible so I am well informed as to all the options. I'll continue my research. Currently, my son is doing quite well, and is not having any major problems with function yet. He is only 2, so we'll have to evaluate as he grows. What scares me are the posts I have read from those who are older who have lived with this and who are experiencing pain and arthritis from compensating by shoulder rotation, or are having difficulty in function and embarrassment in daily life. We have been told the derotation is best done prior to starting school (4 years). Will this surgery help to alleviate future arthritis/pain issues? Is there any benefit to doing the surgery at this age rather than later when he can decide for himself?

We have started in occupational therapy. This may be something others with young kids with this condition may want to look into. Owen enjoys it and it helps us to learn techniques and other ways of doing those daily life things that may be difficult for him. We get this therapy for free through our state's Early Steps Program. Most states have these programs so others may want to look into it for your children.

Interestingly, as to the genetic aspect, we have also found through x-ray that my husband has a very slight irregularity in his radial bones. His Mom had an echo and she has aortic regurgitation. She will also have an x-ray to check for irregularity. We tested for Holt-Oram, and he does not have it. Whatever it is it is is being passed autosomal dominant, but at this point we may never know exactly what the problem is or what the potential affects could be for future children.

Thanks for the advice!
Liz

Posted 1 year ago #
harleyb
Moderator

Liz,

Derotation surgery will not reduce the arthritis risk in the elbow long term, it only repositions the hand. Derotation surgery is best done once it is determined that your son is struggling with a daily task and could be helped with the derotation. The elbow arthritis risk long term is usually quite easily treated if and when it becomes a problem as a young adult. I've seen many patients with elbow arthritis, but I have not seen a patient with shoulder problems.

Hope this helps.

Posted 1 year ago #
jjeeaann
Member

I am 50 years old and was diagnosed with congenital radioulnar synostosis when I was in the third grade. When my parents were presented with the surgery options or leaving it alone, my parents chose not to go with the surgery. As they were told then, the surgery was so close to the elbow and I could end up not being able to bend my arms. Being a child, and the decision completely up to my parents, I say now as I have said all along, I am grateful they left it alone. It has NEVER caused me any real problems. So I hold my fork like a child, and so I can not cover my eyes using only one hand and I got excused from the paralel bars in gym. I can think of a million things that it did not effect. In my lifetime, I have only met about 5 or 6 people that even noticed unless they were told upfront. Oh, and yes my grandfather's twin sister had the same thing. My children do not. I will be watching my grandchildren when I get some.

Posted 1 year ago #
countrygirl
Member

I am 28 years old and was diagnosed with this when I was 5. The drs told my parents that if I wanted my arms fixed then i could wait till I was 13 when my bones were fully hardened. I chose to "live " without being able to turn my hands over like "normal" people. I get asked all the time what I did.... why cant u flip your hands over? Its not the fact that it is embarrasing. I am fully capable of doing 90% of the things that others do I just had to do them different. Up until about 7 years ago I was going to go thru life as being different and not have any type of surgery to correct my arms. I am like jjeeaann, I have to hold my silverware like a child... i basically have do do everything with the tops of my hands( you wouldnt believe the looks I get when I hold a plate or box... I can balance things on top of my hands better than people holding things with the palms. I started having Upper back pain about 6 or 7 yrs ago right in between my shoulder blades... Thought that I had pulled a muscle playin softball or I had picked up something wrong. Over the last few yrs I have been to Drs, physical therapist, chiropractors, had MRIS done of my back and X rays. Nothing showed anything was wrong with my spine. I also have had nerve tests on my elbows because they go numb whenI have them bent for a period of time( like when Im sleeping). They have confirmed that I do have some Nerve damage in my arms but nothing extreme. It was up until a few months ago I had a friend give me a "guest pass" to her chiropractor that is also involved with sport medicine. I was going to give him a try to see if he could help me. I was tired of being in pain. I am in retail management and have to lift and when i count money they say that i look like a chipmunk because i have to hold the money real close...because of my hands i cant hold money right...... After we talked and i explained my "situation" to him. He did some poking, proding, pushing, pulling. and I had to "demonstrate" how I have to do things to acommodate. He told me something that totally made since. Beacuse of the way that I have to do things I am twisting my shoulders to try to compensate not being able to flip my hands. he said that me doing that is pulling my back muscles which is causing my constant back pain. I have prayed about this since he turned on the Light bulb in my head and got me thinking. This man has told me the most logical thing that could be causing my pain. Although my arms do not hurt, this "condition" has caused me other problems. I have woke up in pain to the point that my husband had to help me out of bed and watch me cry because I was so tight i couldnt move. I have prayed about this and I am making an appt this week to see about having something done to correct my arms.I am tired of being in pain.... if anyone has been thru this surgery or has had any upper back pain I would like to know. It has been told to me that upper back pain is not common... I really think the the reason that some doctors have gaffed me off is that they think that I am making this up to get a "pill". I guess they think that at my age you are not having pain you just want a drug of somesort. I was once told by a Dr " well I dont know what is wrong.... just dont do anything that hurts !"...Sorry I am not out looking for a drug I am looking for relief!!!. if anyone has any input I would really like to know.

Posted 1 year ago #
TRICIA1973
Member

Country girl
I Really enjoyed reading your post about your condition. I am 35 years old and was finally given the correct diagnosis of congential radial ulnar synostosis. I have spent my life dealing with the difficulties of the drive thru, receiving change at the checkout, I have to say bathing my babies was particularly difficult. I had use the top of my hand under their head. I also hold my silverware and writing utensils like a small child. That made me smile to read other people have the same problems. I know no one else with my condition, no documented family memebers and my children don't have it. The first thing I did when they handed me my babies was see if they could turn their hands over. I can agree with you that in the last five years I have begin to experience back and shoulder pain which lead me to getting a diagnosis. I also can have numbness of the arm, my husband is very careful not to even lay next to my arms.
I have adjusted my life for every activity. I know I can never do a cartwheel,chin-ups, push-ups my arms are so weak I can not support my body weight. My left arm is severe, I'm not able to turn it at all. I often joke God put my left arm there has a decoration. I am not interested in seeking out surgery although my doctor feels physical therapy might benefit me. We just put on new door handles because it was to hard to turn the doorknob, love those push down handles. My best advice is to curb some of your activity to see what is causing the most pain. No heavy lifting, try not to turn your hands for awhile, sometimes I was rest my arms on a pillow. After a week or so the discomfort goes away. I always know when I have done somehting that is going to cause me pain. Once when I was 8 years old an uncle was playing with me and twisted my arm and I passed out from the pain. I enjoyed reading your posts good luck to you.

Posted 1 year ago #
countrygirl
Member

OMG Tricia1973 you are just like me!!!! Yep I feel like my Left arm is a decoration. I get wierd looks at the drive thru because I can turn my left arm over only backwards. That is the only way that i can accept the change(I had one guy at Chick fila ...he was like oh my goodness how do you do that.. That is awesome!!!) I crack up!!!. i have definatly gotten used to it over the years and learn how to compensate.. I am a frontend manager at a retail bulk grocery store. So unfortunaly there is heavy lifting. I get fussed at by everyone there bc they know that I have back issues... I tell Them I can lift anything as long as i can get ahold of it.. there are times that i come home with bruises on top of my left hand because of the way that I have to lift. I know i shouldnt but when you have to you have to!!. I have one employee that when she first started it was the middle of summer and i was counting money and she asked me if I was cold because of the way that I was holding that money lol.... My friends; kids when they were babies i could hold them, pick them up...true giving baths was interesting but I managed,I had to flip my engagement ring over so I wouldnt scratch thier heads. They were amazed at how i did certain things. When we are all together sometimes i am the butt of their jokes.....Like certain ways that i have to hold things.. Sometimes it gets old and i want to know why God made me different but I know that he doesnt give me anything that I cant handle.. I am so glad that their is another pewrson out there that knows how in the world I feel! That warms my heart. I hope that you try the therapy and I hope that it works for you... My mom and dad actually took me to the dr when I was 5 I still remember... My grandma was trying to hand me some M&Ms and they were getting me to turmn my hands over so i could hold them and I could not do it... the drs thought that my parents had picked me up by my arms and dislocated something.. not true.... My mom thinks now this is why i cried constantly as a baby. she said that nothing would soothe me and she thinks now that I was in pain????? Not sure on that one but is a theory I guess. I am hoping to talk with my Dr and see what options that I have... I am tired of hurting on a day to day basis. I have been to therapists to strengthen my back muscles but alot of the excersizes that they want me to do I cant because of my arms... Good luck to you Tricia1973 Hope to hear from you soon

Posted 1 year ago #
wuzzleme
Member

I was born with extreme radial-ulnar synostosis. I am now 43 yrs old and a doing well. Now that I am approaching middle aged, the condition has worsened. I am affected in both arms. My arms have a noticable "bow". I cannot completley straighten either arm. I have profound difficulty with rotation, flexation and supination. I have not allowed the condition to slow me down, though I do have great pain at times. I served in the United States Army as a combat medic. And work in nursing with the United States Department of Veterans Affairs. I sustained a severe unrelated shoulder injury a year ago that greatly aggrivated the condition. If anyone would like to converse through e-mail to discuss, I can be contacted at mark.swafford@va.gov..I personally would recommend against any surgury to correct the condition as I feel it my axacerbate the condition.

Posted 8 months ago #
wuzzleme
Member

Country girl...I know exactly where you are coming from. I have always lifted objects with the backs of my hands. Looks strange, but it works. My bowling game suffered some from this..how about you?

Posted 8 months ago #
wuzzleme
Member

country girl, how old are you..I am 43..been dealing with this my whole life. I have an appt in Nashville, Tennessee in Jan/2010 with Orth Specialist and see how much worse it ahs gotten..great pain these days...

Posted 8 months ago #

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