Abstract
Guillain Dupuytren was the first to describe in 1834 the condition that later became known as Madelung deformity. Otto Wilhelm Madelung (Fig. 14.1) was a German surgeon and the first physician to provide at the 7th German Surgical Congress in 1878 a comprehensive clinical description of the condition, which now carries his name. [1,2] He described the condition as spontaneous forward subluxation of the hand, which may be quite subtle and not become clinically evident until the adolescent growth spurt (Fig. 14.2). Madelung deformity is characterized by dysplasia of the volarulnar distal radial physis that leads to growth disturbance and excessive volar and ulnar tilt of the distal radial articular surface. A fibrous band or anlage can usually be found providing traction to the ulnar side of the articular surface. This results in premature closure of the dysplastic regions of the physis with formation of a bony bridge across of the physis of the radius. The carpus and hand in response endure palmar subluxation and the ulnar head becomes prominent over the dorsum of the wrist. Diastasis of the distal radius and ulna occurs with the carpus in particular the lunate, which migrates proximally between these two bones. An analogue connecting the lunate and radius has been described to be responsible for proximal lunate migration [3]. This disturbance in the anatomy and subluxed carpus gives the appearance of a dinner fork deformity of the wrist and hand (Fig. 14.3). The radius is usually short and has angular deformity. The radial angulation of the distal radius is excessive and in more severe cases leads to a V-shaped notch above the distal radioulnar joint. The ulna although short, does not have the same angular deformity as the radius and may be subluxed or dislocated dorsal [4–7].
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Rayan, G.M., Upton III, J. (2014). Madelung Deformity. In: Congenital Hand Anomalies and Associated Syndromes. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-54610-5_14
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DOI: https://doi.org/10.1007/978-3-642-54610-5_14
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