What is Madelung's Deformity?
Madelung's Deformity is an extremely rare congenital disorder of the musculoskeletal system in which there is abnormal growth of the wrists when the child is born. In Madelung's Deformity, a part of the radius which is one of the bones of the forearm stops growing, while the other forearm bone which is the ulna keeps on growing and at one point dislocates and forms a bump or a deformity. This is what is called as Madelung's Deformity. The other bones in the hand and forearm may also be affected and the end result of it is that the hands ends up rotated and lower than the forearm. Madelung's Deformity does not affect the movement of the hand or the elbow but the deformity is quite visible and may not look pleasant to the eye.
In some cases, Madelung's Deformity occurs without any genetic association while in majority of cases it occurs in association with certain genetic disorders like the Turner Syndrome or Leri-Weill mesomelic dwarfism. Statistics suggest that only about 1.5% of patients with hand and upper limb deformities have Madelung's Deformity.
Treatment for Madelung's Deformity is basically two fold which is observation and surgical. Observation is reserved for those patients in which the deformity is very mild and the patient is experiencing very mild or no symptoms at all and the patient has no problems with using the wrist for daily activities. Surgery is reserved for those patients in which the deformity is quite large and the patient is experiencing symptoms severe enough to preclude him or her to perform any activities which involve use of the wrist and the patient is experiencing severe pain with any motion of the wrist due to Madelung's Deformity.
What are the Causes of Madelung's Deformity?
Madelung's Deformity does not have an exact cause but researchers are of the opinion that this deformity may be caused due to an abnormal growth plate at the end of the radius while some researchers are of the opinion that this deformity is caused due to an abnormal ligament which connects the radius to the small bones of the wrist.
Madelung's Deformity affects girls more than boys. It is believed that this is due to mutation in the gene on the X chromosome found in girls. Madelung's Deformity is also shown to occur in association with certain genetic disorder like dyschondrosteosis or Turner syndrome.
What are the Symptoms of Madelung's Deformity?
The signs and symptoms of Madelung's Deformity can range from just a slight deformity in the form of a protrusion at the wrist to complete dislocation of the wrist with a visible bump. Some of the other symptoms that may point towards Madelung's Deformity are:
- Limited range of motion of the wrist is one of the symptoms of Madelung's Deformity which may be less in mild cases and more in extreme cases of Madelung's Deformity
- Presence of severe chronic pain especially after any sort of activity involving the affected wrist
- A visible difference of the affected wrist from the contralateral normal side.
How is Madelung's Deformity Diagnosed?
Madelung's Deformity is usually diagnosed when the child is between the ages of 9-15 years when the deformity becomes more apparent. A simple physical examination of the wrist is conclusive enough for the doctor to give it a diagnosis of Madelung's Deformity, although in some cases the deformity may show up at a much younger age as well.
If there is a suspicion of Madelung's Deformity, the doctor will order radiological studies in the form of x-rays to look for any abnormalities in the structure of the wrist bones. A genetic study may also be done to rule out any genetic cause for the patient's deformity. The doctor may also perform tests to rule out other disease conditions like sickle cell trait which has symptoms similar to that of Madelung's Deformity. Once all these possibilities are ruled out the doctor can confirmatively diagnose the patient with Madelung's Deformity.
How is Madelung's Deformity Treated?
In cases where the Madelung Deformity is very mild and the patient is symptom free the treatment of choice is just observation to see if the deformity grows any bigger. In cases where the patient is experiencing severe pain with activity and the pain is interfering with the normal activities of the patient then surgery to correct the deformity is the route to go. The surgical approaches towards treating Madelung Deformity can be corrective soft tissue and bony procedures. The main aim of the surgery is to reposition the wrist and make it more stable so as to prevent it from future dislocations. Corrective measures will also allow the patient to move the wrist without pain and able to do activities. It will also help prevent the deformity from worsening.
The type of surgery required by the patient to correct Madelung's Deformity depends on the age of the patient, the extent of the deformity, the symptoms experienced and how disabling they are, and overall general health of the patient. Some of the procedures done to correct Madelung's Deformity are:
Physiolysis: This is a procedure in which the growth plate abnormality in the radius is corrected
Corrective Osteotomy: This procedure involves cutting and realigning the end of the radius so that the wrist looks more well aligned and well positioned
Ulnar Shortening Osteotomy: This is a procedure in which the ulna bone is shortened to the level of the wrist and thus correcting Madelung Deformity.
Darrach Procedure: This is a procedure in which the protruding part of the ulna is totally removed thus correcting the Madelung Deformity.
What is the Prognosis of Madelung's Deformity?
In majority of the cases of Madelung's Deformity, postsurgery once the deformity is corrected the patient can lead a normal life and does not face any problems with using the wrist. The appearance of the wrist also looks almost normal to the unaffected contralateral side. In retrospect, there have been cases of recurrence of this deformity in children but the percentage is very less. Hence with corrective surgery one can completely get rid of Madelung's Deformity.