Madelung's deformity: a spectrum of presentation.

PURPOSE: To evaluate the hypotheses that all Madelung's deformity subjects have dyschondrosteosis (defined as short stature and mesomelia, in addition to Madelung's deformity) and to evaluate the concept that Madelung's deformity may affect the entire radius.
METHODS: A radiographic and medical records review was performed for 26 subjects (46 extremities) with Madelung's deformity. The radiographs were assessed for radius and ulna length, sagittal radial bow, severity of the Madelung deformity, and radiocapitellar joint space. The sagittal radial bow and the radiocapitellar joint space were used to classify subjects according to whether the Madelung deformity was limited to the distal radius or involved the entire radius.
RESULTS: Thirty-one extremities in 18 subjects were classified as having a distal radius Madelung deformity and 15 extremities in 8 subjects were classified as having an entire radius Madelung deformity. The radius and ulna length and subject height were significantly decreased compared with age- and height-matched normal values in both groups; the entire radius group was more severely affected. In addition, the entire radius group had more severe deformities with respect to lunate subsidence and ulnar tilt. All of the entire radius subjects and 9 of 14 of the distal radius subjects had dyschondrosteosis.
CONCLUSIONS: Madelung's deformity presents as a spectrum. It may affect the entire radius or it may affect only the distal radius. Extremities with involvement of the entire radius have a shorter radius and ulna, decreased height, and a more severe deformity than extremities with involvement of only the distal radius. Additionally, most subjects with Madelung's deformity have dyschondrosteosis.