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Madelung disease, or deformity (MD) is a predominantly bilateral wrist anomaly characterized by shortened and bowed radii and long ulnae leading to dorsal dislocation of the distal ulna and limited mobility of the wrist and elbow. The exact prevalence is unknown. MD is clinically recognizable in mid- to late-childhood or early adolescence (between 8 and 12 years of age) with a unilateral or bilateral presentation, and is more frequently observed in females. Patients present with a decreased range of motion and a limitation of supination, dorsiflexion, and radial deviation. Pronation and flexion are usually normal. Patients experience increasing deformity and pain in the wrist. MD arises from disorganized growth of the radial epiphysis leading to radial bowing, premature physeal fusion, and delayed development of the ulnar and volar portions of the distal radial epiphysis. These changes result in a volar- and ulnar-tilted distal radial articular surface, volar translation of the hand and wrist, dorsal dislocation of the ulna, and wedged carpal bones. The etiology of MD can be classified into post-traumatic, dysplastic, genetic or idiopathic causes. The unilateral presentation, although rare, is normally associated with a post-traumatic cause, resulting from injuries to the radial epiphysis or excessive repetitive loading of an immature joint of the upper extremities. Dysplastic causes include multiple exostoses syndrome or nail-patella syndrome (see these terms). Bilateral MD is a major clinical characteristic of Léri-Weill dyschondrosteosis and is an occasional feature of Turner syndrome (see these terms). MD can also occur in isolation or as part of other genetic syndromes such as dyschondrosteosis-nephritis and type C brachydactyly (see these terms). Environmental causes (trauma and infection) have also been reported. Diagnosis may be suspected on the basis of clinical examination and confirmed by X-ray of the lower arm/wrist. Wrist splints, supports and ergonomic devices may reduce the wrist discomfort associated with MD. In some cases, surgical intervention is required in mid-to-late childhood. This consists of physiolysis of the ulnar aspect of the distal radius and excision of the Vickers ligament, which may decrease pain and restore wrist function. Range of motion is usually only minimally improved and varies in different operative series. Pain may worsen with age due to arthritis in the joint. |