Madelung's Deformity: Difference between revisions

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== Pathogenesis  ==
== Pathogenesis  ==
Premature growth plate arrest at the medial volar aspect of the distal radius causes Madelung deformity. Repetitive traumatic pressure may result in Madelung-like deformity. Mutation or absence of the short stature homeobox (SHOX) gene is thought to be the cause of congenital madelung deformity.
Premature growth plate arrest at the medial volar aspect of the distal radius causes Madelung deformity. Repetitive traumatic pressure may result in Madelung-like deformity. Mutation or absence of the short stature homeobox (SHOX) gene is thought to be the cause of congenital Madelung deformity.


Congenital madelung deformity can occur as a part of Leri-Weill dyschondrosteosis (LWD) or Turner syndrome.<ref>Tranmer A, Laub Jr D. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4993032/ Madelung deformity]. ''Eplasty''. 2016;16.</ref>
Congenital Madelung deformity can occur as a part of Leri-Weill dyschondrosteosis (LWD) or Turner syndrome.<ref>Tranmer A, Laub Jr D. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4993032/ Madelung deformity]. ''Eplasty''. 2016;16.</ref>


== Diagnosis ==
== Diagnosis ==
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* Turner syndrome
* Turner syndrome
* Nail-patella syndrome
* Nail-patella syndrome
* Hereditary, multiple exostosis
* Hereditary, multiple exostoses
* Ollier’s disease
* Ollier’s disease
* [[Achondroplasia]]
* [[Achondroplasia]]
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==== '''Conservative Management''' ====
==== '''Conservative Management''' ====
Conservative management of Madelung’s deformity may be helpful for skeletally mature patients with following measures:
Conservative management of Madelung’s deformity may be helpful for skeletally mature patients with the following measures:
* physiotherapy for wrist strengthening and to reduce pain<ref>Limb Lengthening and Reconstruction Society. Madelung Deformity. Available from: https://llrs.org/information-for-patients/specific-conditions/madelung/ [Last accessed on 27 December 2020]
</ref>
* oral analgesics
* oral analgesics
* activity restriction
* activity restriction

Revision as of 07:05, 27 December 2020

Introduction[edit | edit source]

Madelung’s deformity is a condition of the wrist characterized by a shortened distal radius with volar–ulnar curvature and a dorsally prominent distal ulna.[1]

Madelung's deformity

A Vickers’ ligament is the feature to differentiate Madelung’s deformity from Madelung-like deformities.[2]

Epidemiology[edit | edit source]

Predominantly adolescent females are affected by a ratio of 4:1. The deformity is bilateral most often.[3]

Clinical Features[edit | edit source]

  • Initially asymptomatic, progressive clinical deformity
  • Pain at wrist
  • Loss of wrist extension
  • Compromised forearm rotation
  • “Bayonet” deformity- prominent distal ulna as a result of dorsal subluxation[4]
  • Girls are more often affected than boys
  • Presents between the ages of 6 and 13 years[5]

Pathogenesis[edit | edit source]

Premature growth plate arrest at the medial volar aspect of the distal radius causes Madelung deformity. Repetitive traumatic pressure may result in Madelung-like deformity. Mutation or absence of the short stature homeobox (SHOX) gene is thought to be the cause of congenital Madelung deformity.

Congenital Madelung deformity can occur as a part of Leri-Weill dyschondrosteosis (LWD) or Turner syndrome.[6]

Diagnosis[edit | edit source]

Key Features from X-Ray[edit | edit source]

  • increased dorsal and radial convexity of the distal radius
  • increased volar and ulnar tilt of the distal radial articular surface
  • widened interosseous space
  • relative dorsal position of the ulnar head
  • pyramiding of the carpus[3]

MRI[edit | edit source]

MRI is done on the patients who need the surgical release of Vickers’ ligament to prevent deformity progression.[5]

Differential Diagnosis[edit | edit source]

  • Turner syndrome
  • Nail-patella syndrome
  • Hereditary, multiple exostoses
  • Ollier’s disease
  • Achondroplasia
  • Multiple epiphyseal dysplasias
  • Mucopolysaccharidoses (Hurler and Morquio syndrome)[7]

Treatment[edit | edit source]

Conservative Management[edit | edit source]

Conservative management of Madelung’s deformity may be helpful for skeletally mature patients with the following measures:

  • physiotherapy for wrist strengthening and to reduce pain[8]
  • oral analgesics
  • activity restriction
  • volar splint[9]

Surgical Management[edit | edit source]

  • Radial or ulnar osteotomy
  • Vickers’ ligament resection
  • Ulnar epiphysiodesis[10]

References[edit | edit source]

  1. Dubey A, Fajardo M, Green S, Lee SK. Madelung’s deformity: a review. Journal of Hand Surgery (European Volume). 2010 Mar;35(3):174-81.
  2. Prasad N, Venkatesh M. Madelung Deformity of the Wrist: A Classic Presentation. International Journal of Contemporary Medicine Surgery and Radiology. 2020;5:C4-5
  3. 3.0 3.1 Thomson C, Hawkes D, Nixon M. Madelung’s Deformity: Diagnosis, Classification and Treatment. Journal of Orthoplastic Surgery. 2020 Apr 17;4(1):1-1.
  4. Babu S, Turner J, Seewoonarain S, Chougule S. Madelung's Deformity of the Wrist—Current Concepts and Future Directions. Journal of wrist surgery. 2019 Jun;8(03):176-9.
  5. 5.0 5.1 Knutsen EJ, Goldfarb CA. Madelung's deformity. Hand. 2014 Sep;9(3):289-91.
  6. Tranmer A, Laub Jr D. Madelung deformity. Eplasty. 2016;16.
  7. Kakarla S. Madelung and pseudo Madelung deformities-Pictorial essay. Journal of Medical and Scientific Research. 2019;7:1-6
  8. Limb Lengthening and Reconstruction Society. Madelung Deformity. Available from: https://llrs.org/information-for-patients/specific-conditions/madelung/ [Last accessed on 27 December 2020]
  9. Shahi P, Sudan A, Sehgal A, Meher D, Meena U. Madelung Deformity of the Wrist Managed Conservatively. Cureus. 2020 May;12(5).
  10. Bebing M, de Courtivron B, Pannier S, Journeau P, Fitoussi F, Morin C, Violas P. Madelung's deformity in children: Clinical and radiological results from a French national multicentre retrospective study. Orthopaedics & Traumatology: Surgery & Research. 2020 Nov 1;106(7):1339-43.