OBSOLETE: Madelung deformity, bilateral

Madelung deformity, bilateral (Orphanet code 295223) is an obsolete clinical entity entry that was previously used to describe the bilateral presentation of Madelung deformity. Madelung deformity is a skeletal condition affecting the wrists, characterized by abnormal growth of the distal radius (the bone on the thumb side of the forearm near the wrist). In this condition, the growth plate (physis) of the distal radius closes prematurely on its ulnar and volar (inner and palm-side) aspects, leading to a characteristic bowing of the radius, dorsal prominence of the distal ulna, and a triangular configuration of the carpal (wrist) bones. When bilateral, both wrists are affected. The deformity results in limited wrist motion, pain, reduced grip strength, and cosmetic concerns. Madelung deformity is most commonly associated with Léri-Weill dyschondrosteosis, caused by mutations or deletions involving the SHOX gene. It can also occur as an isolated finding or in association with other skeletal dysplasias such as Turner syndrome or multiple exostoses. This Orphanet entry has been marked as obsolete, meaning the concept has been retired or merged into other existing disease classifications. Patients with bilateral Madelung deformity are now typically classified under the broader entries for Madelung deformity or the underlying condition (e.g., Léri-Weill dyschondrosteosis). Treatment options include conservative management with pain relief and activity modification, or surgical interventions such as physiolysis (release of the abnormal growth plate tether), corrective osteotomy of the radius, or ulnar shortening procedures in more severe cases.

Common questions about OBSOLETE: Madelung deformity, bilateral