Skip to main content
SpringerLink
Log in

Madelung Deformity

  • Chapter
  • First Online:
Congenital Anomalies of the Upper Extremity

  • 1754 Accesses

Abstract

Madelung deformity is a complex entity that is not completely understood. It is a rare condition caused by asymmetric growth at the distal radial physis secondary to partial ulnar-sided arrest. A shortened radius that curves palmarly and ulnarly, a dorsally prominent ulnar head, and a triangular arrangement of the carpal bones characterize the deformity. It occurs more commonly in females than males and typically affects both wrists. The deformity may occur in isolation or as a part of a genetic syndrome. Due to varied inheritance patterns, a clear family history may be lacking. Few studies exist on the natural history of the disease, as well as long-term data on those who have had surgical intervention. Nonoperative management is recommended for asymptomatic individuals. Pain, stiffness, and aesthetic concerns have been cited as indications for surgical intervention. Vicker ligament release, physiolysis, radial osteotomies, and/or ulnar shortening procedures may effectively correct radiologic alignment and provide improvement in wrist appearance and pain.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 149.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 199.00
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Lecon GD. Orale de Clinique Chirurgicale Faites a l’Hotel-Dieu de Paris. 1834;4:197.

    Google Scholar 

  2. 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 

  3. Madelung OW. Die spontane subluxation de hand nach vorne. Verh Dtsch Ges Chir. 1878;7:259–76.

    Google Scholar 

  4. Leri A, Weill J. Une affection congenitale et symetrique du developpement osseux: la dyschondrosteose. Bull Mem Soc Med Hosp Paris. 1929;53:1491–4.

    Google Scholar 

  5. Roubicek M, Arriazu MC, Isaac G. SHOX deficiency phenotypes. J Clin Endocrinol Metab. 2003;88(8):4004.

    Article  CAS  PubMed  Google Scholar 

  6. Rao E, Weiss B, Fukami M, Rump A, Niesler B, Mertz A, et al. Pseudoautosomal deletions encompassing a novel homeobox gene cause growth failure in isiopathic short stature and Turner syndrome. Nat Genet. 1997;16(1):54–63.

    Article  CAS  PubMed  Google Scholar 

  7. Belin V, Cusin V, Viot G, Girlich D, Toutain A, Moncla A, et al. SHOX mutations in dyschondrosteosis (Leri-Weill syndrome). Nat Genet. 1998;19(1):67–9.

    Article  CAS  PubMed  Google Scholar 

  8. Shears DJ, Vassal HJ, Goodman FR, Palmer RW, Reardon W, Superti-Furga A, et al. Mutation and deletion of the pseudoautosomal gene SHOX cause Leri-Weill dyschondrosteosis. Nat Genet. 1998;19(1):70–3.

    Article  CAS  PubMed  Google Scholar 

  9. Flatt AE. The congenital hand anomalies. St. Louis: CV Mosby; 1977. p. 50.

    Google Scholar 

  10. Harley BJ, Carter PR, Ezaki M. Volar surgical correction of Madelung’s deformity. Tech Hand Upper Extremity Surg. 2002;6:30–5.

    Article  Google Scholar 

  11. Canale ST. Madelung deformity. In: Campbell’s operative orthopedics. 10th ed. St. Louis: Mosby; 2002. p. 76.

    Google Scholar 

  12. Waters PM, Bae DS. Pediatric hand and upper limb surgery: a practical guide. Philadelphia: Wolters Kluwer Health; 2012.

    Google Scholar 

  13. 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 

  14. McCarroll HR, James MA. Very distal radial osteotomy for Madelung’s deformity. Tech Hand Up Extrem Surg. 2010;14(2):85–93.

    Article  PubMed  Google Scholar 

  15. Munns C, Glass I. SHOX-related haploinsufficiency disorders. In: Pagon R, Bird T, Dolan C, editors. Gene reviews. Seattle: University of Washington; 2005. Updated 2008.

    Google Scholar 

  16. Ross JL, Scott Jr C, Marttila P, Kowal K, Nass A, Papenhausen P, et al. Phenotypes associated with SHOX deficiency. J Clin Endocrinol Metab. 2001;86(12):5674–80.

    Article  CAS  PubMed  Google Scholar 

  17. Ghatan AC, Hanel DP. Madelung deformity. J Am Acad Orthop Surg. 2013;21:372–82.

    Article  PubMed  Google Scholar 

  18. Sanchez J, Perera E, Jan de Beur S, Ding C, Dang A, Berkovitz GD, et al. Madelung-like deformity in pseudohypoparathyroidism type 1b. J Clin Endocrinol Metab. 2011;96(9):E1507–11.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  19. Rump P, Jongbloed JD, Sikkema- Raddatz B, Mundlos S, Klopocki E, van der Luijt RB. Madelung deformity in a girl with a novel and de novo mutation in the GNAS gene. Am J Med Genet A. 2011; 155A(10):2566–70.

    Article  PubMed  Google Scholar 

  20. Ioan DM, Maximilian C, Fryns JP. Madelung deformity as a pathognomonic feature of the onycho-osteodysplasia syndrome. Genet Couns. 1992;3(1):25–9.

    CAS  PubMed  Google Scholar 

  21. Anton JI, Reitz GB, Spiegel MB. Madelung’s Deformity. Ann Surg. 1938;108(3):411–39.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  22. De Smet L, Claessens A, Lefevre J, Beunen G. Gymnast wrist: An epidemiologic survey of ulnar variance and stress changes of the radial physis in elite female gymnasts. Am J Sports Med. 1994;22(6):846–50.

    Article  PubMed  Google Scholar 

  23. Fagg PS. Reverse Madelung’s deformity with nerve compression. J Hand Surg Br. 1988;13(1):23–7.

    Article  CAS  PubMed  Google Scholar 

  24. Ducloyer P, Leclercq C, Lisfranc R, Saffar P. Spontaneous ruptures of the extensor tendons of the fingers in Madelung’s deformity. J Hand Surg Br. 1991;16:329–33.

    Article  CAS  PubMed  Google Scholar 

  25. Jebson P, Blair WF. Bilateral spontaneous extensor tendon ruptures in Madelung’s deformity. J Hand Surg Am. 1992;17:277–80.

    Article  CAS  PubMed  Google Scholar 

  26. McCarroll HR, James MA, Newmeyer III WL, Molitor F, Manske PR. Madelung’s deformity: Qualitative assessment of x-ray deformity. J Hand Surg Am. 2005;30(6):1211–20.

    Article  PubMed  Google Scholar 

  27. McCarroll HR, James MA, Newmeyer III WL, Manske PR. Madelung’s deformity: Diagnostic thresholds of radiographic measurements. J Hand Surg Am. 2010;35A:807–12.

    Article  Google Scholar 

  28. Cook PA, Yu JS, Lubbers L, Coleman CR, Cook II AJ, Kean JR, et al. Madelung deformity in skeletally immature patients: morphologic assessment using radiography, CT, and MRI. J Comput Assist Tomogr. 1996;20(4):505–11.

    Article  CAS  PubMed  Google Scholar 

  29. Stehling C, Langer M, Nassenstein I, Bachmann R, Heindel W, Vieth V. High resolution 3.0 Tesla MR imaging findings in patients with bilateral Madelung’s deformity. Surg Radiol Anat. 2009;31(7):551–7.

    Article  PubMed  Google Scholar 

  30. Green DP, Hotchkiss RN, Pederson WC, Wolfe SW, editors. Deformities of the wrist and forearm. In: Green’s operative hand surgery, vol. 2, 5th ed. Philadelphia: Elsevier; 2005. p. 1484–91.

    Google Scholar 

  31. 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;31:1499–506.

    Article  PubMed  Google Scholar 

  32. Imai Y, Miyake J, Okada K, Murase T, Yoshikawa H, Moritomo H. Cylindrical corrective osteotomy for Madelung deformity using a computer simulation: Case report. J Hand Surg Am. 2013;38A: 1925–32.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Orthopaedic Surgery, Shriners Hospital for Children Northern California, 2425 Stockton Boulevard, Sacramento, CA, 95817, USA

    Sarah E. Sibbel M.D., Andrea S. Bauer M.D. & H. Relton McCarroll M.D.

Authors
  1. Sarah E. Sibbel M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Andrea S. Bauer M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. H. Relton McCarroll M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Andrea S. Bauer M.D. .

Editor information

Editors and Affiliations

  1. Fletcher Allen Health Care and Vermont Children’s Hospital, Departments of Surgery and Pediatrics, University of Vermont School of Medicine, Burlington, Vermont, USA

    Donald R. Laub Jr.

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer Science+Business Media New York

About this chapter

Cite this chapter

Sibbel, S.E., Bauer, A.S., McCarroll, H.R. (2015). Madelung Deformity. In: Laub Jr., D. (eds) Congenital Anomalies of the Upper Extremity. Springer, Boston, MA. https://doi.org/10.1007/978-1-4899-7504-1_24

Download citation

  • DOI: https://doi.org/10.1007/978-1-4899-7504-1_24

  • Published:

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4899-7503-4

  • Online ISBN: 978-1-4899-7504-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation