The term "Madelung's deformity" refers to a condition of the
upper extremity characterized by abnormal growth and development of
the end of the radius, one of two bones of the forearm.
Due to abnormal growth, the end of the radius typically grows
palmarly and radially (i.e. in the direction of the palm and of the
thumb). Because the other bone of the forearm -- the ulna -- is not
affected and continues to grow, the end of the ulna becomes quite
prominent on the dorsum, or back, of the wrist.
What causes Madelung's deformity?
The exact etiology (cause) of Madelung's is unknown. Most
cases occur sporadically, without any defined inheritance pattern or
known genetic association. Some believe that Madelung's deformity 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 (the so-called "Vicker's ligament").
Furthermore, there are some cases in which prior trauma or injury to
the growth plate at the end of the radius may cause a deformity
similar to Madelung's.
How common is Madelung's deformity?
The exact incidence of Madelung's in unknown. Typically,
females are more commonly affected than males. Usually the changes
are bilateral, seen in both wrists. Though the deforming forces may
be present from birth, often patients are asymptomatic and there are
no noticeable changes in the appearance of the wrist until patients
are in their early teenage years. Madelung's deformity may also
occur in the setting of associated syndromes, such as Leri-Weill
dyschondrosteosis and nail-patella syndrome.
How is Madelung's deformity diagnosed?
Madelung's deformity is diagnosed by treating physicians
after a thorough medical history and careful physical examination.
X-rays are used to confirm the diagnosis and to identify the extent
of bony involvement. Due to the association with other clinical
syndromes, genetic testing may be performed.
How is Madelung's deformity treated?
Fortunately, many patients with Madelung's deformity have no
pain or limitations in activities of daily life. In these
situations, no treatment is necessary, though serial examinations
and X-rays may be recommended to monitor the progression, if any, of
the deformity.
In patients with pain, functional limitations, or progressive
deformity, surgical treatment may be recommended. In general, the
goals of surgical treatment are to reposition and stabilize wrist to
allow for painless function and prevention of recurrent deformity.
The type of surgery is dependent upon the patient's age, degree of
deformity, functional limitations, and general health.
Treatment options include releasing the soft-tissue or bony tethers
on the radius ("physiolysis"), cutting and realigning the end of the
radius bone ("corrective ostetomy"), ulnar shortening osteotomy , or
even removing the prominent end of the ulna bone ("the Darrach
procedure"). Furthermore, wrist arthroscopy may also be performed in
some patients to evaluate and treat cartilage injury within the
wrist joint.
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