Madelung Deformity
May 8, 2008 by Dr Arun Pal Singh
Filed under Pediatric Disorders
Madelung’s deformity is a congenital abnormality of the wrist caused by a growth disturbance that retards development of the ulnar and volar portions of the distal radial physis.
The eponym gives credit to Madelung, who described this entity. Carpus curvus, radius curvus, and progressive subluxation of the wrist, manus valgus, and manus furca are other terms used for this condition.
The primary deformity is bowing of the distal end of the radius, which in the most typical form curves in a volar diorection while the ulna continues to grow in a straight line. The distal ends of the radius and ulna are at different levels in the lateral plane. That of the ulna has maintained its original normal position, while that of the radius has curved down to a volar level.
It is the distal end of the radius that is displaced. Becaue of its curvature and growth disturbance, the radius has become short while the ulna has ocntineus to grow normally and has become relatively longer.
Etiology
There are four categories of Madelung deformity
- Posttraumatic: Following trauma that disrupts growth of the distal radial ulnar-volar physis
- Dysplastic: Associated with bone dysplasias like multiple hereditary osteochondromatosis, Ollier disease, achondroplasia, multiple epiphysial dysplasias, and the mucopolysaccharidoses . This type can also be seen secondary to sickle-cell disease, infection, tumor, and rickets.
The most important dysplasia associated with MD, however, is Leri-Weill dyschondrosteosis. - Chromosomal : As in Turner syndrome
- Idiopathic – Where no cause or association can be found
The exact nature of the pathologic process that causes the disturbance in the growth of the distal radial physis is unknown.
The asymmetrical growth disturbance is similar to that seen in Blount’s disease or tibia vara. Madelung’s deformity is a hereditary disorder, transmitted as an autosomal dominant trait with incomplete penetrance.
Sporadic forms do occur.
It is more common in the female; involvement is frequently bilateral.
Normally, the distal articular surface of the radius is tilted 5 degrees toward its volar surface and 25 degrees toward the ulna, with its dorsal surface and radial margin convex and its volar surface and ulnar border concave.
There are two types of Madelung’s deformity.
- Typical, or regular
- Atypical, or reverse.
In the typical form, the distal articular surface of the radius may tilt toward its palmar surface as much as 80 degrees and ulnarward as much as 90 degrees. In the normal wrist, the proximal row of the carpal bones is arranged in an arc, with its proximal surface forming a convex dome.
In Madelung’s deformity, this dome becomes peaked, its apex resting on the lunate bone. The radius and ulna are separated, with the peak of the carpal bones wedged into the interosseous space. The entire carpus is shifted toward the ulnar and volar side of the wrist. Coalition of carpal bones may be present.
In reverse, or atypical, Madelung’s deformity (which is rare), the distal end of the radius is tilted dorsally, reversing the plane of the distal end of the articular surface with a shift of the carpus toward the dorsal side. The distal end of the ulna then appears to be displaced volarly instead of dorsally.
Genetics
Primary chromosomal association with Madelung deformity has been observed in patients with Turner syndrome (Patients having only one X chromosome). Within families affected by a short stature dysplasia, a mutation has been found in short stature homeobox-containing gene, SHOX, present on X chromosome.
But families with this mutation and individuals with Turner syndrome and families with a history of MD have been shown to exhibit a variable expression of MD and dyschondrosteosis. This raises a possibility of a modifier gene on another area of the X chromosome or on an autosomal gene may be involved.
Clinical Features
Deformity of the wrist is the initial presenting complaint; it usually becomes obvious in late childhood or early adolescence, between the ages of 8 and 12 years.
In typical Madelung’s deformity the distal end of the ulna remains in its normal anatomic position and grows distally, causing a visible prominence on the dorsal and ulnar aspects of the wrist. Normally, the radial styloid process is long and is located 1 cm distal to the ulnar styloid. In Madelung’s deformity, the radius is shortened at the wrist; the radial styloid process may be on the same horizontal line as the ulnar styloid or may reach a point proximal to it.
The range of motion of the wrist is limited, especially in dorsal extension and ulnar deviation. Because of the diasthesis between the distal radius and ulna and the displacement of the carpus between the two separated bones of the forearm, pronation and supination of the forearm are also limited; as a rule, supination is definitely decreased, and pronation is impaired to a slight degree.
In reverse Madelung’s deformity palmar flexion of the wrist is decreased, while dorsiflexion is increased. Range of rotatio in of the forearm, especially pronation, is decreased. When it is minimal, madelung’s deformity may be asymptomatic. In moderate or severe deformity, however, pain develops insidiously at the wrist.
Initially it is minimal, disappearing on rest. With progression of the deformity and impingement of the displaced carpus on the distal ulna, the pain increases. Volar displacement of the carpus may cause discomfort in the region of the median nerve and flexor tendons. Weakness of the wrist may result from progressive instability of the joint.
Diagnosis
Characteristic radiographic findings include dorsal and radial curvature of the distal radius; exaggerated palmar and ulnar tilt of the distal articular surface of the radius; pyramiding of the carpal bones; greater length of the ulna as compared with the radius; wide interosseous space; and assumption of a relatively dorsal position by the ulnar head, which appears to be enlarged.
In the differential diagnosis, one should consider the possibility of trauma (dislocation of the distal radioulnar joint), rickets, inflammatory conditions of the wrist such as rheumatoid arthritis, and infection involving the ulnar half of the distal radial physis.
Treatment
Treatment is primarily directed toward the relief of pain and the restoration of function, with cosmetic improvement as a secondary consideration.
The majority of patients with Madelung’s deformity do not require surgical treatment.
Conservative measures consist of curtailing physical activities that may cause forced dorsiflexion of the wrist and wearing a plastic wrist splint to provide support and relieve symptoms.
Surgical treatment is directed toward shortening the ulna, correcting the bowing deformity of the distal radius by wedge osteotomy, stabilizing the carpus, and preventing recurrence of deformity by controlling the asymmetrical growth of the distal radius.
The ulna can be shortened by Milch’s cuff resection in children or Darrach’s resection.
Deformity correction is achieved by either a closing wedge or an opening wedge osteotomy of the distal radius at its metaphyseal-diaphyseal junction.
Fusion of the radial half of the distal radial physis will prevent recurrence of deformity.
Related posts:
- Congenital High Scapula-Sprengel Deformity
- Tibia Vara – Xray Showing Varus Deformity In Both Tibia
- Leri Weill Dyschondrosteosis
- Congenital Radioulnar Synostosis
- Congenital Dislocation of The Radial Head
Thank you for this article. I’ve had Madelung Deformity all my life and continue to live with it. Of course, at the time I was diagnosed, I didn’t know all the questions I should ask as I was only a preteen. Mine would appear to be a sporatic instance, or very removed as we have not known anyone in either side of the family with this occurrence.
If you would like any photographs to include in your website/article, let me know. I’ll be happy to send some.
Regards,
Heather
Dr Arun Pal Singh Reply:
July 1st, 2009 at 2:10 am
@Heather Knorr,
Thanks Heather. Good to have you here. Would you like to post some tips on how you manage your day to day activities.That would be helpful to future readers.
Thanks for your offer. It would be great if you could send some.
I have another question. Can this deformity, and the conditions that cause it, also influence improper rib development?
Dr Arun Pal Singh Reply:
July 1st, 2009 at 2:14 am
@Heather Knorr,
Though a direct association is not known (to me) but because it is a developmental condtion , it cannot be ruled out.
I also have had madelungs all my life. I was wondering if lipomas are also related to madelungs bone deformitys? I have had a few lipomas and no doctors around my area have a clue what madelungs is. But i found this web site that said symmetric lipomatosis was madelungs and causes lipomas? thanks for your thoughts! Im having many problems and cant find the link.Thanks again!
I also have madelungs deformity. But mine cause me alot of wrist and elbow pain and I go in for shots every few months to help ease the pain. I had surgery once on my left to have one of the bones shortened. It worked for about 15 years. Now pain is coming back along with pain in my elbows. One surgeon told me the only other hope out there is to just fuse the joint altogether. But that is a very, very last resort. I think I will be doing these shots for years to come. Any other advise out there would be great. I am 44. Thank You
Dr Arun Pal Singh Reply:
September 6th, 2009 at 11:02 am
@Lori,
Could you mail me your xray of both wrists please.
I also wanted to ad is there a possibility of having leri-weil syndrome since I am short at 4′11. My forearms are short and my forearms are also diagnosed with being deformed and dislocated at the elbow joints as well as my wrists. Is the test for this easy and should my doctor look into it. Thanks
Lori
Yes I could, Where would I mail them too?
Lori
Dr Arun Pal Singh Reply:
September 10th, 2009 at 1:53 am
contact [at] boneandspine dot com
I wrote to them for address. please let me know what you think when you get them. Thanks.
Lori
Dr Arun Pal Singh Reply:
September 15th, 2009 at 3:35 pm
@Lori,
I mailed you back. You can reach me at the same address.
I have a FIVE yr old daughter with madelund deformity,
discover today.Xray in hospital.
She is not in pain @ this time and I wondered if anyone could give me so advise, on how to help her to deal with her conditon ??
many thanks
She may not experience any pain with this problem for many years to come. I have it as well and did not get any pain until late teens to early twenties. I never knew I had it until I was in my 30’s. Some people will get pain sooner than others. The older you get and more your wrists are over used the more pain and problems you get.. I have noticed that ice helps and Ibuprophen helps a great deal. As I got older and pains got worse I started getting shots in my wrists to help with pain. I did have surgery once in my mid 20’s. That helped for about 12 yrs or so. There is surgery that can be done to help her now since she is still young and also meds I have heard of to help the bone grow longer. You will need an orthopedic that specializes in madelungs deformity. They are hard to find. Most ortho’s don’t know how to properly treat this condition. Hope this helps.
Lori
Faithen – i have madelungs deformity, i recommend seeking a specialist who KNOWS about it and has treated it before, i also reccomend yearly check ups with them to monitor her growth. I would push for preventative surgery as soon as symptoms like restricted movement, and pain set it. Early surgery can correct the wrist as she grows (its unplesant but not major surgery if you get it early). As long as you monitor it and get a hand and wrist surgeon who knows what theyre talking about she shouldnt get major deformity.
A question for the doctor – noone else in my family has this. Im female, what are the chances of my children having madelungs?
Dr Arun Pal Singh Reply:
October 2nd, 2009 at 1:52 am
@Caroline,
Yours seems a sporadic mutation. I am not aware if it can be calculated how much chance is there if your children would get this condition.
Al I can say is that there is a possibility but it cannot be quantified. And i do not think that there is a marker available to know it before birth of of the child.
may be someone from genetics is able to tell it in better way.
If you have any update on this, please let me know.
I did not experience pain untill I was 23 while doing a desk job.That was also when I was diagnosed. I have found that when ever I feel the twinge coming on I now the pain is coming and I rest my wrist immediately. It’s good that you know early It is best to start now teaching her how not to over use her wrist. Don’t carrry anything heavy, take breaks when she is writing or typing. don’t open jars or use can openers (use electric ones or have someone else do it ) as she gets older use drills, not screw drivers, when driving turn hand over hand instead of driving with one hand and twisting your wrist around. It seems that the twisting, turning, pulling and repetitive motion along with heavy weight is what really tears at the joint wearing it down. You’ll find that she won’t have a lot of strength in her wrist and as she gets older they well get weaker. Gymastics is probably not a good idea. I don’t know if she is into sports but certain things may increase the chances of getting pain earlier. I know my wrist hurt a little after bowling. Her deformity might not be as severe and she may have littlle to no pain, but certainly if giving up sports is not an answer for her some of the other sugestions won’t hurt to keep in routine. As I got older I learned what activities aggravated my wrist more than others and I believe, for me, that avoiding those or doing them in limitatation has helped tremendously to control pain I now hardly experience any. Although my Dr. said my deformity is severe he also said I had great range of motion so my case may also not be as severe as others.
I have Leri Weill Dyschondostreosis, and the Madelung deformity (Wrists, elbows and ankles-knees).
I found out I have this condition properly when I was 13. An unfourtunate unrelated injury made my parents more aware. I had been seeing a Pediatrition since I was 3 or 4 due to my short stature, and after this injury, the Madelung deformity became more pronounced.
I suffered until I was 16 with severe pain and aching in my right wrist, and the movement I had slowly decreased. After 5 years of some kind of peace, I’m not experiencing the same problems with my left wrist, only the pain is worse.
I desperately want to communicate wiith other people who have this condition, I am now greatly concerned about the future, as the Doctor I have just started seeing mentioned Arthritis to me, and my mother has just been diagnosed with Osteoporosis.
Does anyone know of any websites, forums or community websites based on these condtions?
Many thanks
-Laura
This sounds crazy as I have lived with the condition all my life till now. I dont know how to describe it exactly but I have restricted pronation of my elbow /wrist joints,which unables me to turn my palms upwards. Would it be possible to correct the movement somehow?
Sincerely
Gill
depending on the age of the child there is also hormonal shots that can be given to help in the growth of the bones in the arms. That may be something to look into as well. I have heard of these in different discussions. I too did not find out until my early twenties. I am now in mid 40’s and have so much pain in my wrists and elbows are getting affected from pain traveling up arm and losing strength in both arms and hands. I am sure my jobs over the years have not helped any. I got shots in both wrists every few months to ease pain now. Just went the other day for more shots. But, the hormone shots are something to look into along with maybe a surgery to help.
Team Inspire is rare disorder or disease site, Where people get suport from other people with the same conditions. It’s free to join and you can talk to others with the same condition or people who are going through similar strugles.
inspire [dot] com
The only way you can correct the movement after you’ve finished growing is to have a reconstruction, for me that would mean taking bone off my hip and have major reconstructive surgery. Id personally rather the restricted movement, but then ive found ways to work around mine.
For Laura, sounds like reconstruction wouldnt be a bad idea if you have that kind of severe pain. And yea, im pretty sure as i get older ill loose strength in my right hand and ill have issues with arthritis, ill worry about that if or when that happens though. There is a facebook group i saw the other day of people, about 20 or so, from round the world with madelungs, you might want to look at that. Thats pretty much the only group ive seen though.
Caroline, I did not know that there was another site as well. Is that the regular “facebook” that people sign up for?
I found out I had Madelungs Deformity when I was having troubles playing volleyball in highschool. I was also bowlegged when I was 2 years old and had special shoes to fix it. I am now 29 years old and I live with pain in my wrists every day. My family doctor wants to send me for nerve end testing for carpol tunnel and I’m not sure she knows much about the deformity. I have heard that people with madelungs can get tendon ruptures in their wrist and I am wondering if this testing is going to put me in more pain then I face every day. Can someone please help me with this?
Dr Arun Pal Singh Reply:
October 21st, 2009 at 2:36 pm
@jEN,
Do you have symptoms of carpal tunnel syndrome?
the nerve conductive study will be of no use to you. I had one done to when the doctors really did not know much. It will come back neg. The pain is from the bones and not nerves. Jen you can e-mail me at proud_mom3 [at] yahoo.com I will be willing to talk to you about what you are going through and help with ideas. I have been dealing with this for 20 yrs now. Take care.
hey Lori
yea its on facebook. to join it you need to join facebook if you havent already, just set up a basic profile, and search ‘madelungs deformity’ in the groups section and join that group.
I never had problems with either of my wrists or hands. In September 2004 my hand got caught in a machine and dislocated my distal ulnar bone. I have fought with workmans comp over this since then. When i first started going to doctors they said all i did was sprain my wrist. They are now saying it is madelungs deformity. I have loss of feeling in 2 fingers, limited range of motion, cannot grip things, and have severe chronic pain most of the time. I cant do my job anymore and doctors recommend i change profession. I am a 36 year old divorced mother of two and feel like crawling in a hole and giving up. My bones have separated 10mm and the bones in my hand are separating and falling. Does anyone have advice for me?
Dr Arun Pal Singh Reply:
November 1st, 2009 at 3:16 pm
@Amy in TN.,
Could you send me your xray and photograph of your limb including hand.
Also
Which fingers are numb?
What activities of hand are restricted?
Madelung deformity following trauma is not seen in adults.
Did you have this before trauma?
What do you mean by bones separating and falling?
My 12-year old daughter just found out that she has Bilateral Madelung Deformity. She is double-jointed in both elbows and shoulders. For the past year or so, we visually noticed that her wrists were getting worse especially when she would do her stretches/warmups during her trampoline/gymnastics classes her wrists would start to feel a burning sensation.
Since July, we’ve visited our family doctor, then a paedictrician, then an occupational therapist, and just last week we visited a specialist who has now referred her to one of his colleagues in Montreal. It seems that is a rare deformity.
If she requires surgery, my question is from what I read in the above postings is, if surgery only works for so many years, why not get it done again? I’m glad I found this website and the postings above….
Surgery does help, and your right, it only helps for so long. My surgery lasted about 20 yrs though. Its the over use of the hands and wrists as you get older. They really can’t do another surgery cause they remove part of the bone. But there is pain shots they can give that help after that and maybe wrist fusion later in life. So sorry your daughter is going through this. I also have this. I am now 44. She will have good days and bad. Just have her not do heavy things with her hands and wrists. It does help. Also, twising motions aggrivate it as well. Gymnastics was very hard for me to do as a child. The more I tried to do the more pain I got. Keep in touch. I have posted my e-mail address up further in this chat. Please read the article Leir Weill’s it helps with info.
No i never had any problems until after the trauma. The fingers that go numb are my pinky, middle, and finger inbetween. I am limited to things i can do. I can’t grip steering wheel of car to drive, can’t pick up gallon of milk to pour, can’t hold my drinking cup at times, have trouble opening jars because i have lost strength in my grip, can’t play ball with my kids because my hand can not take the blow from the toss. It’s hard for me to do dishes’ sometimes even shampooing my hair is a task.
As far as the bones in my hand they told me they were seperating. My first diagnosis was that i just had a sprained wrist. Then they say i have a dislocation of the distal ulnar bone. At first it was only 5mm gap between the bones and as of almost 9 months ago it has separated to 10mm. Now they are saying all it is is madelung syndrome. This is all workers compensation and i have been fighting them since 2004. But as of september the doctor says it isnt work related.
Dr Arun Pal Singh Reply:
November 12th, 2009 at 1:08 pm
@Amy in TN.,
It would help if you could mail a picture of your xray and if possible pictures of your hands and wrists.
Is there any inability to extend your 4th and 5th fingers or to use then way you want.
What is meant by separation of the bones of hand.
Numbness is almost always due to some nerve involvement.
Why do you have it. Did you talk to your doctor?
Have you taken another opinion on this matter?
My son, age 20, was diagnosed with Madelung’s at age 13. He had surgery on both wrists at that time. He does have wrist pain, as well as knee and shoulder pain. He also had learning disabilities in school, and I believe I read somewhere that learning disabilities sometimes presents with Madelung’s. Here is my concern: should I have my other children tested as well? My other son is tall, long arms and limbs, but has always had pain in his ankles, hips especially, and shoulders. My daughter has long arms and limbs, and also has complained of pain in her ankles. She appears to have a “wide” ankle, it almost appears to have an extra bone, as does my other son. I am so curious about this syndrome, and the future implications for my son, as well as our other children. Also, I am concerned about our son with MD and his future treatment, as the physicians in our area had never heard of it prior to his diagnosis.
Dr Arun Pal Singh Reply:
December 1st, 2009 at 9:00 am
@Carrie,
I can understand your concern. Th only way to be sure is to get your children examined. MD has genetic component if you think a doubt has crept into your mind the only way to confirm is examination and investigation by specialist.
It would also help if you read more about the disease and its course. You might not understand many terms
in the beginning but as you keep learning things would start making sense.
Knowledge may not alter course of the disease but it makes you more confident.
There are comments from people from various age groups and you can get a fair idea about how people carry on in spite of disease.
The deformity and disability varies from one individual to another and the treatment also varies from one person to other.
All the best.
Please join our group, Madelung’s Wrist Deformity at:
groups[dot]yahoo[dot]com/group/Madelungs
Ed. Note – Carrie, The link structure has been altered as we do not allow active outgoing links in comments untill editors deem them absolutely necessary.
All the best for your efforts.
I have madelungs deformity i was told i had it last year. i am 14 years old and i am 5 ft 3 . i really am not quite sure what it is so would you be able just to briefly explain what it is to me simply cos i dont really get all the technical stuff. the doctors wont do surgery but i am worried about how it will effect my life. id also really like to get in touch with some people who have it too to discuss preferably my age tho because everyone seems to be older and to have lived through there life with it. HELP! Thanks =]
Dr Arun Pal Singh Reply:
January 1st, 2010 at 4:17 pm
@Molly S,
I would suggest you to go through what people have written here. If there is any specific query, please let me know.
@ Molly S: My daughter Selina doesn’t mind if you contact her about it, she’s turning 13 this week. You can reach her at selinafavaro[at]hotmail [dot]com anytime. You both have something in common.
Ed. Email address modified to prevent spam.
@ Nancy F. Thanks
I Have Emailed Her
Molly S. Selina didn’t receive your email yet maybe again..Nancy
Nancy F
I Emailed Her Again If Not This Is My Email – molzirules [at] hotmail [dot] com
I have 2 daughters one almost 17yrs old and one just turned 10yrs old. My older daughter has madelungs and just found out that the 10yr old has it also. The Dr. said that was rare also. Would that be true? My 10yr old has to get some genetic testing to see if she has a syndrome, she is small for her age and if need be she may need to take some growth hormones. Which I was told this is the time to start. She was a breached baby at 5 lbs 12 1/2 ounces 18 inches long about 4 to 5 days before due date. Born with a cleft lip only. Which I don’t think has anything to do with it. Are we on the right path with our 10yr old?
Dr Arun Pal Singh Reply:
January 22nd, 2010 at 10:40 pm
@sharon,
families with MAdelung deformity are know but yes, sporadic form occuring in two siblings is kind of rare.
Your younger daughter has short stature and Madelung deformity ( you did not mention if it was bilateral), do rule out Leri Weill Dyschondrosteosis if it is bilateral.
You can read more about that here
http://boneandspine.com/pediatric-disorders/leri-weill-dyschondrosteosis/
I’ve just been diagnosed by my daughters pediatrician. I saw doctors about my arms growing up but was never given a name for it until now. I had pain in my wrists and elbows between about 14 and 22. I’m now 32 and occasionally get pain but usually manage to avoid it by knowing what i can and can’t do.
I find if i need to lift things I’m better off lifting with my arms and hands in a vertical position. I’m more likely to get pain if I carry something with my hands horizontal and palms facing upwards. My elbow’s would sometimes lock when I was a student and sitting particularly long exams (over 2 hours).
The doctor I saw in my teens told me he wouldn’t operate on my wrists because the bones from my hands going into my wrist were also deformed so if they corrected one thing it would likely upset something else in there and possibly lock my fingers. This was about 18 years ago though and I’m sure a lot has changed since then.
Sharon, I also have madelungs in both wrists. Went to genetics Doctor and was tested and came back positive for Leir Weills. Your daughters should be tested for this as well, it is somewhat common for people with madelungs deformity. I had surgery in my left wrist years ago, and possible have to have it for my right. Now I am having sever problems with Tennis Elbow in both arms, due to the demormity of my forearms. I will be having surgery to fix this soon. Some people never have a problem with the madelungs and some have severe problems and pain. The best thing is to dont overuse the wrists alot and make things easier, like can openers and such. It will help them later in life. Let me know if I can help with any ideas or anything. I have dealt with this for most of my life through all kinds of things. There is also a good talk group on facebook, just type in madelungs deformity under search. Myself and a few others are in there that can help you and your children in the right path.
Lori