Madelung's deformity

Madelung's deformity

Infobox_Disease
Name = PAGENAME


Caption = Madelung deformation, a type of bone malformation associated with both SHOX and SHOXY genes mutations.
DiseasesDB = 32115
ICD10 = ICD10|Q|74|0|q|65
ICD9 = ICD9|755.54
ICDO =
OMIM = 127300
MedlinePlus =
eMedicineSubj = orthoped
eMedicineTopic = 513
MeshID =

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.

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.

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. [WhoNamedIt|synd|759] [O. W. Madelung. Die spontane Subluxation der Hand nach Vorne. Verhandlungen der deutschen Gesellschaft für Chirurgie, Berlin, 1878, 7: 259-276.]

ee also

* Léri-Weill dyschondrosteosis

References


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