Madelung's deformity

Source: Wikipedia, the free encyclopedia.
Madelung's deformity
Madelung deformation, a type of bone malformation associated with both SHOX and SHOXY genes mutations.
SpecialtyMedical genetics Edit this on Wikidata

Madelung's deformity is usually characterized by malformed

Léri-Weill dyschondrosteosis
. It can be bilateral (in both wrists) or just in the one wrist. It has only been recognized within the past hundred years. Named after Otto Wilhelm Madelung (1846–1926), a German surgeon, who described it in detail, it was noted by others. Guillaume Dupuytren mentioned it in 1834, Auguste Nélaton in 1847, and Joseph-François Malgaigne in 1855.

Signs and symptoms

It is a

radio-ulnar joint with marked ulnar deviation of the hand. Severe deformities are associated with congenital absence or hypoplasia of the radius.[citation needed
]

The male:female rate of this disorder is 1:4. The incidence is unknown, and there is no described racial predominance. Even though Madelung's Deformity is considered a

congenital disorder, symptoms sometimes aren't seen until adulthood. In most cases, symptoms find their onset during midchildhood. At this age, the relatively slower growth of the ulnar and palmar part of the radius, leads to an increasingly progressive deformity. Pain and deformity are the main symptoms patients present with.[1] Typical clinical presentation consists of a short forearm, anterior-ulnar bow of the radius and a forward subluxation of the hand on the forearm. As mentioned before, the severity of the disorder varies greatly, which also leads to a spectrum of presentation.[citation needed
]

Genetics

Homozygous SHOX gene mutations result in Langer mesomelic dysplasia.[2]

Pathogenesis

Madelung deformity of the wrist is caused by a growth disturbance in the inferior

mesomelic dwarfism and a mutation on the X chromosome. Attempts can be made to treat the deformity surgically by addressing the deforming bone and fibrous bands called "Vickers ligament". This is an abnormal ligament formed between the Lunate bone of the wrist and the radius and is found in 91% of cases of Madelung's deformity.[citation needed
]

Diagnosis

Diagnosis is normally confirmed by X-rays.[citation needed]

Treatment

Non-surgical

First options for treatment are conservative, using hot or cold packs, rest and

brace can be used to keep the deviated arm straight. When none of the conservative treatments work surgical intervention is designated.[citation needed
]

Surgical

Pediatrics

Physiolysis

Purpose of the treatment is the removal of the

Palmaris longus and leaves the Median nerve and Radial artery protected. Then the Pronator quadratus muscle is found and detached from the radius. Here a cut into the bone will find the abnormal epiphysis. When the epiphysis is clearly defined more bone is removed so the radius is in its normal position and prevents a new bone bar from forming. This is the end of the physiolisis. This is always combined with a Vickers Ligament release.[3]

Dome osteotomy

Purpose of this treatment option is to straighten the abnormal radius. To do this, an 8 cm incision is made from the wrist crease at the palmair radial side. The approach is made passing the Flexor carpi radialis with detachment of the Pronator quadratus muscle from the radius. Now the Vickers ligament release is done. After this the periosteum is elevated and a crescent-shaped osteotomy, concave at the end, is marked on the bone. Now the radius is cut dome shaped and straightened. The distal end of the radius stays attached to the ulna. The dome shape of the osteotomy allows adequate bony contact for stability and a subperiosteal void for rapid healing.[4]

Vickers Ligament Release

This ligament causes the wrist to deform even more. The purpose of this release is to release the tension and leave the wrist straight in further growth. In both physiolysis and dome osteotomy there should be a clear view of the abnormal.[3]

Adults

Ulna reduction

Adults with Madelung’s deformity may suffer from ulnar-sided wrist pain. Madelung's Deformity is usually treated by treating the distal radial deformity. However, if patients have a positive ulnar variance and focal wrist pathology, it’s possible to treat with an isolated ulnar-shortening osteotomy. In these patients the radial deformity is not treated.[5]

The ulna is approached from the subcutaneous border. A plate is attached to the distal end of the ulna, to plan the osteotomy. An oblique segment is removed from the ulna, after which the distal radial-ulnar joint is freed, making sure structures stay attached to the styloid process. After this, the freed distal end is reattached to the proximal ulna with the formerly mentioned plate.[6]

Total DRUJ replacement

An alternative treatment for patients with ulnar-sided wristpain is a total replacement of the distal radial-ulnar joint. There are many surgical treatments of the condition, but most of these only improve the alignment and function of the

radiocarpal joint. A persistent problem in these treatments has been the stiff DRUJ. However, a prosthesis helps in managing the pain, and might also improve the range of motion of the wrist.[7]

The procedure consists of making a hockey-stick shaped incision along the ulnar border. This incision is made between the fifth and sixth dorsal compartment. Being careful not to harm any essential structures, like the

medullary canal of the ulna, allowing centralization for a cannulated drill bit. A poly-ethylene ball, which will serve as the prosthesis, is then placed over the distal peg. After confirming full range of motion, the skin will be closed.[8]

Dome Osteotomy

In case of Madelung's Deformity in conjunction with radial pain, a dome osteotomy may be conducted. For more information about this procedure, please refer to the treatment of Madelung's Deformity in children.[citation needed]

Eponym

It is named for Otto Wilhelm Madelung.[9][10]

References

  1. ISBN 978-1608313907
    .
  2. PMID 16175500
    .
  3. ^
    S2CID 25118540
    .
  4. PMID 17095381
    .
  5. PMID 12772098
    .
  6. ^ "Reconstruction: Ulnar shortening osteotomy for distal radius fracture malunion".
  7. PMID 19306049
    .
  8. PMID 18984351
    .
  9. Who Named It?
  10. ^ O. W. Madelung. Die spontane Subluxation der Hand nach Vorne. Verhandlungen der deutschen Gesellschaft für Chirurgie, Berlin, 1878, 7: 259–276.

External links

Retrieved from "https://en.wikipedia.org/w/index.php?title=Madelung%27s_deformity&oldid=1164961320"