Clinical Example: Madelung's deformity

Madelung's deformity is a dysplasia of the radius resulting in an exaggerated radial inclination, a short forearm, dorsal dislocation of the ulnar head and a "V" shaped proximal carpal row. It may be painful and may result in extensor tendon rupture. Congenital Madelungs may be due to an abnormal fibrous band tethering sigmoid notch of the radius proximally to the ulna. Traumatic Madelung's may follow partial growth arrest of the distal radius. Treatment options differ in the mature and immature skeleton.

 
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Case 1. Typical Xray findings of congenital Madelungs.
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Case 2. Minimal Madelungs picture following pediatric distal radius fracture.
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Case 3. Adult bilateral congenital Madelung's deformity with pain and extensor tendinitis, treated with the Sauve Kapandji (Lauenstein) procedure: segmental distal ulnar ostectomy and distal radioulnar joint fusion. 

The right side, preop: 
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Right side, postop.
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And the left side, preop: 
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And postop: Here, an inadequate ulnar gap led to heterotopic bone formation which almost resulted in a pseudarthrosis. 
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The important technical for this procedure in Madelung's points are: 
  • Allow adequate gap between the ends of the ulna to prevent a pseudarthrosis. Law of pesky osteogenesis: if the bone gap is 2 to 15millimeters, it won't bridge if you want it to, but it will if you don't want it to.
  • In Madelung's, you can't just bang a screw across the DRUJ - the sigmoid notch of the radius is a sharp knife edge rather than a concavity,and you must expose and excise this to get a solid bone-to-bone contact. 
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