Skip to main content

Madelung’s Deformity in Children

  • Living reference work entry
  • Latest version View entry history
  • First Online:
The Pediatric Upper Extremity

Abstract

The exact etiology of Madelung’s deformity is not known. In addition, the clinical and radiographic presentation can vary greatly between not only patients but also between forearms in the same patient. In severe cases, the premature closure of the volar-ulnar physis of the distal radius leads to the characteristic appearance of volar subluxation of the hand/carpus as well as a dorsally prominent ulna. In some cases, a genetic component is present in the form of Leri-Weill syndrome in which there is an abnormality in the short-stature homeobox gene (SHOX) (Benito-Sanz S, Thomas NS, Huber C, Am J Hum Genet 77:533–544, 2005). Symptoms can include pain, decreased wrist/forearm ROM, as well as cosmetic dissatisfaction with appearance.

It is important to assess and counsel every patient individually as not everyone will need surgical intervention. If the patient has no symptoms and the deformity is minimal, then careful observation with serial examinations and radiographs is warranted. If the deformity is more severe and symptoms are present, then operative intervention may be warranted. Depending on the age of the patient and deformity present, several types of operative treatment are available. The results following surgery for Madelung’s deformity have consistently been shown to be of benefit and should be considered for symptomatic patients with substantial deformity.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Institutional subscriptions

References

  • Arora AS, Chung KC. Otto W. Madelung and the recognition of Madelung’s deformity. J Hand Surg Am. 2006;31(2):177–82.

    Article  PubMed  Google Scholar 

  • Babu S, Turner J, Seewoonarain S, Chougule S. Madelung’s deformity of the wrist-current concepts and future directions. J Wrist Surg. 2019;8(3):176–9. https://doi.org/10.1055/s-0039-1685488. Epub 2019 Apr 22. PMID: 31192036; PMCID: PMC6546486.

    Article  PubMed  PubMed Central  Google Scholar 

  • Benito-Sanz S, Thomas NS, Huber C, Gorbenko del Blanco D, Aza-Carmona M, Crolla JA, et al. A novel class of Pseudoautosomal region 1 deletions downstream of SHOX is associated with Leri-Weill dyschondrosteosis. Am J Hum Genet. 2005;77(4):533–44.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Bruno RJ, Blank JE, Ruby LK, Cassidy C, Cohen G, Bergfield TG. Treatment of Madelung’s deformity in adults by ulna reduction osteotomy. J Hand Surg Am. 2003;28(3):421–6.

    Article  PubMed  Google Scholar 

  • Carvalho M, Cardoso PS, Alves C, Balaco I, Ling TP, Matos G. Madelung deformity – esthetic and functional outcomes from the surgical treatment with distal radial dome osteotomy and Vickers ligament section. Rev Bras Ortop (Sao Paulo). 2022;57(1):113–9.

    PubMed  Google Scholar 

  • Del Core M, Beckwith T, Phillips L, Ezaki M, Stutz C, Oishi SN. Long-term outcomes following Vickers ligament release and growth modulation for the treatment of Madelung deformity. J Pediatr Orthop. 2020;40(4):e306–e11.

    Article  PubMed  Google Scholar 

  • Glard Y, Gay A, Launay F, Guinard D, Legre R. Isolated wedge osteotomy of the ulna for mild Madelung’s deformity. J Hand Surg Am. 2007;32(7):1037–42.

    Article  PubMed  Google Scholar 

  • Grigelioniene G, Schoumans J, Neumeyer L, Ivarsson A, Eklof O, Enkvist O, et al. Analysis of short stature homeobox-containing gene (SHOX) and auxological phenotype in dyschondrosteosis and isolated Madelung deformity. Hum Genet. 2001;109(5):551–8.

    Article  CAS  PubMed  Google Scholar 

  • Harley BJ, Carter PR, Ezaki M. Volar surgical correction of Madelung’s deformity. Tech Hand Up Extrem Surg. 2002;6(1):30–5.

    Article  PubMed  Google Scholar 

  • Harley BJ, Brown C, Cummings K, Carter PR, Ezaki M. Volar ligament release and distal radius dome osteotomy for correction of Madelung’s deformity. J Hand Surg Am. 2006;31(9):1499–506.

    Article  PubMed  Google Scholar 

  • Hirschfeldova K, Solc R, Baxova A, Zapletalova J, Kebrdlova V, Gaillyova R, et al. SHOX gene defects and selected dysmorphic signs in patients of idiopathic short stature and Leri-Weill dyschondrosteosis. Gene. 2012;491(2):123–7.

    Article  CAS  PubMed  Google Scholar 

  • Leti Acciaro A, Garagnani L, Lando M, Lana D, Sartini S, Adani R. Modified dome osteotomy and anterior locking plate fixation for distal radius variant of Madelung deformity: a retrospective study. J Plast Surg Hand Surg. 2022;56(2):121–6.

    Article  PubMed  Google Scholar 

  • Madelung O. Die spontane Subluxation der Hand nach vorne. Verh Dtsch Ges Chir. 1878;7:259–76.

    Google Scholar 

  • McCarroll HR Jr, James MA, Newmeyer WL 3rd, Manske PR. Madelung’s deformity: diagnostic thresholds of radiographic measurements. J Hand Surg Am. 2010;35(5):807–12.

    Article  PubMed  Google Scholar 

  • Murphy MS, Linscheid RL, Dobyns JH, Peterson HA. Radial opening wedge osteotomy in Madelung’s deformity. J Hand Surg Am. 1996;21(6):1035–44.

    Article  CAS  PubMed  Google Scholar 

  • Nielsen JB. Madelung’s deformity. A follow-up study of 26 cases and a review of the literature. Acta Orthop Scand. 1977;48(4):379–84.

    Article  CAS  PubMed  Google Scholar 

  • Otte JE, Popp JE, Samora JB. Treatment of Madelung deformity with Vicker ligament release and radial physiolyses: a case series. J Hand Surg Am. 2019;44(2):158.e1–9.

    Article  PubMed  Google Scholar 

  • Ranawat CS, DeFiore J, Straub LR. Madelung’s deformity. An end-result study of surgical treatment. J Bone Joint Surg Am. 1975;57(6):772–5.

    Article  CAS  PubMed  Google Scholar 

  • Rappold GA, Fukami M, Niesler B, Schiller S, Zumkeller W, Bettendorf M, et al. Deletions of the homeobox gene SHOX (short stature homeobox) are an important cause of growth failure in children with short stature. J Clin Endocrinol Metab. 2002;87(3):1402–6.

    Article  CAS  PubMed  Google Scholar 

  • Steinman S, Oishi S, Mills J, Bush P, Wheeler L, Ezaki M. Volar ligament release and distal radial dome osteotomy for the correction of Madelung deformity: long-term follow-up. J Bone Joint Surg Am. 2013;95(13):1198–204.

    Article  PubMed  Google Scholar 

  • Sugiura H, Horii E, Koh S. Radial osteotomy with callus distraction for the correction of severe Madelung deformity. Hand (N Y). 2022:NP1- NP5.

    Google Scholar 

  • Vickers D, Nielsen G. Madelung deformity: surgical prophylaxis (physiolysis) during the late growth period by resection of the dyschondrosteosis lesion. J Hand Surg Br. 1992;17(4):401–7.

    Article  CAS  PubMed  Google Scholar 

  • Zebala LP, Manske PR, Goldfarb CA. Madelung’s deformity: a spectrum of presentation. J Hand Surg Am. 2007;32(9):1393–401.

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

The authors wish to recognize the work of previous author Lesley Wheeler, MD, and colleagues for their contributions to The Pediatric Upper Extremity and to this manuscript.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Scott N. Oishi .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2023 Springer Nature Switzerland AG

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Oishi, S.N., Sayavong, A., Ezaki, M. (2023). Madelung’s Deformity in Children. In: Abzug, J.M., Kozin, S., Zlotolow, D.A. (eds) The Pediatric Upper Extremity. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8758-6_81-2

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-8758-6_81-2

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-8758-6

  • Online ISBN: 978-1-4614-8758-6

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics

Chapter history

  1. Latest

    Madelung’s Deformity in Children
    Published:
    09 October 2023

    DOI: https://doi.org/10.1007/978-1-4614-8758-6_81-2

  2. Original

    Madelung’s Deformity
    Published:
    20 June 2014

    DOI: https://doi.org/10.1007/978-1-4614-8758-6_81-1