Madelung's deformity
From Wikipedia, the free encyclopedia
Madelung's deformity Classification and external resources |
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Madelung deformation, a type of bone malformation associated with both SHOX and SHOXY genes mutations. | |
ICD-10 | Q74.0 |
ICD-9 | 755.54 |
OMIM | 127300 |
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.
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[edit] 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.
[edit] 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.
[edit] Eponym
It is named for Otto Wilhelm Madelung.[1][2]
[edit] See also
[edit] References
- ^ synd/759 at Who Named It
- ^ O. W. Madelung. Die spontane Subluxation der Hand nach Vorne. Verhandlungen der deutschen Gesellschaft für Chirurgie, Berlin, 1878, 7: 259-276.