General Considerations

• Arrest of epiphyseal growth of the ulnar (medial) and volar (anterior) portions of the distal radius
• Leads to shortening of the radius and relative overgrowth of the ulna
• Inherited as an autosomal dominant due to mutation on X chromosome and has variable penetrance
• Overwhelmingly occurs in females
• Rare congenital deformity that does not usually manifest until 10-14 years
• May also be seen as an acquired consequence of trauma to the growth plate (e.g. Salter V fracture)

Clinical Findings

• Onset of pain in one wrist and then the other
• Prominence of the distal ulnar head posteriorly
• Limitation of motion, especially extension and supination

Imaging Findings

• Bilateral in 50-66% of patients
• Exaggerated radial inclination
• “V-shaped” proximal carpal row
• Dorsal dislocation of the ulnar head

Differential Diagnosis

• Madelung's dyschondrosteosis
• Form of mesomelic dwarfism, especially if condition is bilateral or there  is a family history of short stature
• Short forearms
• Short tibia/fibula

Treatment

• Deformity will progress until distal radial epiphysis closes
• Conservative management if asymptomatic
• Operative correction for relief of pain and cosmetic improvement

Associations

• Bone dysplasias associated with Madelung deformity include
  • Multiple hereditary exostoses
• Ollier’s disease
• Achondroplasia
• Multiple epiphyseal dysplasias
• Mucopolysaccharidoses (Hurler and Morquio syndromes)

 

Named after: Otto Wilhelm Madelung, German surgeon, in 1878. [Discovery of x-rays by Wilhelm Roentgen occurred in 1895]  

 

 

Madelung Deformity. Frontal and lateral radiographs of both wrists show shortening of the ulnar portion of the distal radii (blue arrows) with exaggeration of the radial inclination and proximal migration of the proximal carpal row (black arrows) producing a V-shape between the radius and ulna. There is also dorsal dislocation of both ulnar heads (yellow arrows).
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eMedicine  Madelung Deformity  Lamberti, P; Light, T and Scholl, W.

Wheeless’ Textbook of Orthopaedics   Madelung’s Deformity

e-Hand  Madelung’s Deformity