Madelung's deformity
Madelung's deformity
Classification & 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 |
| 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.
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
- ^ 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.
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Congenital malformations and deformations of musculoskeletal system (Q65-Q79, 754-756) |
| Limbs |
hip: Dislocation of hip/Hip dysplasia
feet (Club foot, Flat feet, Pes cavus)
head, face, spine and chest: skull, face and jaw (Dolichocephaly, Plagiocephaly) - Scoliosis - chest (Pectus excavatum, Pectus carinatum)
Polydactyly/Syndactyly (Webbed toes)
reduction deficits (Ectrodactyly, Amelia, Phocomelia)
upper limb (Cleidocranial dysostosis, Madelung's deformity, Sprengel's deformity)
knee (Genu valgum, Genu varum)
Arthrogryposis |
| Skull and face bones |
Craniosynostosis (Scaphocephaly) - Trigonocephaly - Oxycephaly - Crouzon syndrome - Hypertelorism - Macrocephaly - Treacher Collins syndrome - Platybasia |
| Spine and bony thorax |
Klippel-Feil syndrome - Spondylolisthesis - Cervical rib - Bifid rib |
| Osteochondrodysplasia |
growth of tubular bones and spine: Achondrogenesis - Thanatophoric dysplasia - Short rib-polydactyly syndrome - Chondrodysplasia punctata (Rhizomelic chondrodysplasia punctata, Conradi-Hünermann syndrome), Achondroplasia (Hypochondroplasia, Osteosclerosis congenita) - Ellis-van Creveld syndrome - Spondyloepiphyseal dysplasia congenita
Osteogenesis imperfecta - McCune-Albright syndrome - Osteopetrosis - Metaphyseal dysplasia - Hereditary multiple exostoses - Osteopoikilosis - Chondrodystrophy - Osteodystrophy |
| Other |
abdominal wall (Congenital diaphragmatic hernia, Omphalocele, Gastroschisis, Prune belly syndrome) - Ehlers-Danlos syndrome |
| See also non-congenital conditions (M, 710-739) |
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