What can you tell us about Madelung deformity in children? We have a boy (age 12) who has just been diagnosed with this problem. Evidently, it is rare and even moreso in boys. We’ve just started our search on-line for information and hope you can help us.

Two orthopedic surgeons from the University of Washington (Seattle) Medical Center recently reviewed this wrist deformity and offered their opinions and preferred treatment. As you have discovered, it is, indeed, a rare wrist deformity affecting females four times more often than males. And as the name might suggest, it was named for the German physician who first described it back in 1878.

Both wrists are usually involved and although the condition is present at birth, it may not become evident until later in life after growth has taken place. Two distinct features of this problem include: 1) the presence of a ligament (Vickers ligament) that holds the lunate bone of the wrist to the bottom of the radius and 2) growth arrest at the growth plate (physis) where the radius joins the wrist. Vickers ligament is named after the physician who first identified this extra soft tissue structure. It is believed that the ligament may be the cause (or at least a contributing factor) to the deformity because of the pressure (compression) it places on the bones.

The deformity gives the wrist a pyramid shape. Bowing of the radius (bone in the forearm) is what gives the forearm the appearance of a deformity. Of course, what to do about it is a big question among the orthopedic community. There is quite a controversy over when to do surgery. The question comes up: should surgery be done to correct the appearance when there are no symptoms? If the patient does report pain and/or loss of motion, is surgery warranted if there is only mild loss of function? And what type of surgery should be done?

After describing various types of surgeries used by others (e.g., soft tissue releases, radial dome osteotomy and physiolysis, combined radial and ulnar osteotomies, isolated radial osteotomy, isolated ulnar osteotomy, arthroplasty), the surgeons from the University of Washington offered their preferred treatment. We thought you might be interested in their comments.

They suggested surgery should be done when there are limiting symptoms rather than just for cosmetic purposes. A growing child who has no symptoms should be watched and re-checked each year. Only when the deformity is getting worse, the wrist is unstable, and/or the wrist is jammed together should surgery be planned.

Type of surgery is still under debate and study. Factors that must be considered when planning a surgical procedure include age (whether the patient is still growing), the presence of a difference in bone length between the two bones of the forearm, and severity of the radial bowing. Three basic surgical approaches include: release/removal of the Vickers ligament with corrective osteotomy, wrist arthroplasty (joint replacement), or arthrodesis (wrist fusion).

Because there just isn’t enough evidence to guide management of this rare condition, more studies are needed to identify the best treatment approach. Finding successful nonsurgical ways to treat the problem is always preferred in the growing child. Early joint replacement is not advised because of the limited time the implant will last, thus requiring additional surgery later.

Surgery that does not improve wrist motion or relieve pain may not be the best way to treat Madelung deformity. Patient preferences and dissatisfaction with the appearance of the forearm and wrist are important considerations as well. Older adults who have experienced additional complications from this condition (e.g., tendon rupture, wrist subluxation or dislocation) may require surgical reconstruction of the wrist.