At Boston Children’s Hospital, the skilled surgeons in our Orthopedic Center have developed innovative surgical treatments for children with all degrees of severity of Madelung deformity.
What is Madelung deformity?
Madelung’s is a rare congenital condition in which the wrist grows abnormally. Part of radius, one of the bones of the forearm, stops growing early. The other forearm bone, the ulna, keeps growing and can dislocate, forming a bump. Other bones can be affected as well, and the hand ends up rotated and lower than the forearm. Movement of the hand and elbow are not affected.
Madelung deformity can occur:
- by itself (in isolation) without any pattern or genetic association
- in association with certain genetic syndromes, such as Leri-Weill mesomelic dwarfism (dyschondrosteosis) or Turner syndrome
Note: This website mainly discusses the condition in isolation, rather than as associated with a genetic syndrome.
How common is Madelung deformity?
Madelung’s is not a common condition. While exact numbers are not known, in one recent study it was found that among a sample of 1,476 patients with congenital hand and upper limb differences, Madelung’s accounted for only about 1.7 percent of cases.
How is Madelung’s diagnosed?
Madelung’s is usually diagnosed by physical examination when the deformity becomes apparent. This is usually when a child is between 8 and 14 years old, although the condition sometimes shows up earlier.
Your child’s doctor will take a thorough medical history and perform a careful physical examination. Typically, x-rays confirm the diagnosis. (For details visit our Testing and Diagnosis page).
How is Madelung’s treated?
If a child is pain-free with mild deformity, she will probably be treated with simple observation, rather than surgery. But if, the child or teen is experiencing pain, limited range of motionor other significant problems, surgery is likely to be indicated.
Surgical approaches can include corrective soft tissue and bony procedures. (For details, visit our Treatment page).
Will my child be OK? What’s her long-term outlook?
Most of the time, after surgery at Boston Children’s the child’s bones heals, the deformity corrected, the pain improved, and the appearance of the wrists improves. But recurrence of the condition may occur, especially in younger children. Boston Children’s doctors use several treatment strategies to help minimize the risk of recurrence. (For details, visit our Treatment page).
Researchers don’t really know what causes Madelung’s. Some researchers believe that the condition is due to an abnormal growth plate at the end of the radius and/or an abnormal ligament connecting the end of the radius to the small bones of the wrist.
Girls are more often affected than boys. Researchers believe that this is probably due to mutation in a gene (the SHOX homeobox gene) on the X chromosome.
Madelung’s can occur in association with certain genetic syndromes, such as Leri-Weill mesomelic dwarfism (dyschondrosteosis) or Turner syndrome.
Signs and symptoms
Signs and symptoms of your child’s condition can range from a just a slight protrusion of the lower end of the ulna to a complete dislocation of the wrist. Other indications can include:
- Limits to wrist range of motion (ROM)—can range from minor to major
- Pain—chronic or following activity involving the wrist joint
- Visible difference in the wrist