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Madelung deformity

Madelung deformity is an epiphyseal growth plate disturbance characterised by dorsal and radial bowing of the radius with resultant cosmetic effects and decreased grip strength. It can be bilateral in 50-66% of patients. It often occurs as rare congenital deformity and does not usually manifest until 10-14 years. It may also be seen as an acquired consequence of trauma to the growth plate (e.g. Salter V fracture). The congenital form has an autosomal dominant inheritance with a variable penetrance. 

Demographics and clinical presentation

Most non-traumatic cases of Madelung deformity present with progressive deformity during late childhood or early adolescence 3. The abnormality is more common in females. 

Pathology

The common mechanism for all causes of Madelung deformity is due to partial closure, or failure of development of the ulnar side of the distal radial growth plate. There is an arrest of epiphyseal growth of the medial and volar (anterior) portions of the distal radius. This leads to shortening of the radius and relative overgrowth of the ulna. The underlying cause of this is unclear, with possibilities including 3:

  • vascular insufficiency
  • trauma
  • infection (osteomyelitis)
  • muscular disorders
Associations

Presentation is with deformity, decreased grip strength and often with pain in the wrist relating to ulnocarpal impaction 1,3.

Radiographic features

The deformity is characterised by:

  • dorsal and radial bowing of the radius
  • exaggerated palmar (up to 35°)  and ulnar tilt (up to 60°) of the radiocarpal articulation 3
  • failure of ossification of the ulnar side of the distal radial epiphysis
  • exaggerated radial inclination
  • decreased carpal angle below 118°; normal from 118° to 139°.
  • carpal subluxation in a palmar and ulnar direction
  • lunate is gradually forced to the apex of the V-shaped radioulnaocarpal joint
  • “V-shaped” proximal carpal row = herniated proximal carpal row.
  • dorsal subluxation of the distal ulnar and positive ulnar variance
  • wedging of the carpus between the radius and ulna

Treatment and prognosis

Many treatments have been proposed and tried, often with limited success. Conservative measures tend to be ineffective. Surgical options include 3:

  • radial epiphysiodesis
  • radial corrective osteotomy
  • radial physiolysis
  • ulnar epiphysiodesis
  • excision of the distal ulnar
  • ulnar shortening osteotomy

Etymology

The Madelung deformity was first described in 1878 by Otto Wilhelm Madelung, German surgeon (1846 - 1926) 4-5.

Madelung deformity should not be confused with Madelung-Launois-Bensaude syndrome (or Brodie syndrome II) which is characterised by lipomatous accumulation in the subcutaneous regions of the neck and upper thorax and upper limbs 6.

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