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Madelung's deformity



Madelung's deformity
Classification & external resources
Madelung deformation, a type of bone malformation associated with both SHOX and SHOXY genes mutations.
ICD-10 Q74.0
ICD-9 755.54
DiseasesDB 32115
eMedicine orthoped/513 

Madelung's deformity is usually characterized by malformed wrists and wrist bones and short stature and is often associated with Léri-Weill dyschondrosteosis. It has only been recognized within the past hundred years, and occurs due to a genetically recessive gene.

Contents

Presentation

It is a congenital subluxation or dislocation of the ulna's lower end, due to malformation of the bones. Sometimes, minor abnormalities of other bone structures, often caused by disease or injury, such as a fracture of the lower end of the radius with upward displacement of the lower fragment. The deformity varies in degree from a slight protrusion of the lower end of the ulna, to complete dislocation of the inferior radio-ulnar joint with marked radial deviation of the hand. Severe deformities are associated with congenital absence or hypoplasia of the radius.

Notable cases

Famed rock 'n' roll guitarist Jimi Hendrix is said to have had the deformity[citation needed].

Treatment

The operation consists of excision of the lower end of the ulna; in severe cases of marked radial deviation, it may be necessary also to fuse the radius to the carpus in order to gain correction.

Eponym

It is named for Otto Wilhelm Madelung.[1][2]

See also

References

  1. ^ synd/759 at Who Named It
  2. ^ O. W. Madelung. Die spontane Subluxation der Hand nach Vorne. Verhandlungen der deutschen Gesellschaft für Chirurgie, Berlin, 1878, 7: 259-276.
 
This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Madelung's_deformity". A list of authors is available in Wikipedia.
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