Introduction and Definition
Madelung deformity is a developmental deformity of the distal radius and wrist caused by abnormal growth of the volar-ulnar portion of the distal radial physis. The deformity produces progressive malalignment of the distal radius, carpus and distal radioulnar joint, usually becoming evident during late childhood or adolescence. It is typically bilateral in syndromic disease but may be unilateral in isolated cases.
The condition should be distinguished from an acquired “Madelung-like” deformity caused by trauma, infection, metabolic disease or premature physeal closure. In true developmental Madelung deformity, the abnormality is related to disordered physeal growth and tethering of the volar-ulnar distal radius. The deformity may be painless and discovered incidentally, or may cause pain, limited forearm rotation, cosmetic prominence of the ulna and impaired wrist function.
Pathology and Pathophysiology
Growth-plate abnormality
The primary lesion is relative failure of growth at the volar-ulnar part of the distal radial physis. The exact mechanism is not completely established, but abnormal physeal orientation and tethering by the volar radiocarpal structures are important. The anomalous volar radiocarpal ligament, commonly termed the Vickers ligament, extends from the distal radius towards the lunate and acts as a tether across the abnormal physis. It is…