Management

The management of Madelung's Deformity is dependent on the severity at presentation. Function may be surprisingly good and surgical intervention frequently is unwarranted. Later presentation is associated with a deformity, reduced range of motion due to carpal subluxation and pain. Pain, reduced range of motion and functional limitation are indications for surgery; cosmetic deformity is not.

The surgical treatment depends on the stage of the disease process:

• if skeletally immature, the abnormal physis is ablated on the ulnar side and a fat graft can be inserted
• if skeletally mature, the distal radius is exposed by a volar longitudinal incision, the anomalous volar ligament is released where it attaches to the lunate; the distal radius is osteotomised to achieve a more functional position
• distraction osteogenesis; the anomalous radiolunotriquetral ligament may prove problematic

There may be a role for early childhood radiography in the children of parents who have themselves had Madelung's Disease, Leri-Weill or Turners syndromes. Genetic testing is warranted if a syndrome is suspected.