The exact etiology of Madelung’s deformity Madelung deformity is not known. In addition, the clinical and radiographic presentation can vary greatly between not only patients but also between forearms in the same patient. In severe cases, the premature closure of the volar-ulnar physis of the distal radius leads to the characteristic appearance of volar subluxation of the hand/carpus as well as a dorsally prominent ulna. In some cases, a genetic component is present in the form of Leri-Weill syndrome in which there is an abnormality in the short stature homeobox gene (SHOX) (Benito-Sanz et al. Am J Hum Genet 77:533–44, 2005). Symptoms can include pain, decreased wrist/forearm ROM, as well as cosmetic dissatisfaction with appearance. It is important to assess and counsel every patient individually as not everyone will need surgical intervention. If the patient has no symptoms and the deformity is minimal, then careful observation with serial examinations and radiographs is warranted. If the deformity is more severe and symptoms are present, then operative intervention may be warranted. Depending on the age of the patient and deformity present, several types of operative treatment are available. The results following surgery for Madelung’s deformity have consistently been shown to be of benefit and should be considered for symptomatic patients with substantial deformity.
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