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Madelung’s Deformity

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Congenital Anomalies of the Upper Extremity

Abstract

Madelung’s deformity is an uncommon congenital wrist condition characterized by premature closure of the volar-ulnar aspect of the distal radius physis, volar carpal subluxation, and distal ulna prominence. It is classified as a malformation of the radio-ulnar axis involving the entire upper limb, according to the Oberg-Manske-Tonkin Classification. It accounts for less than 2% of congenital upper extremity differences. Madelung’s deformity is most commonly idiopathic, but a Madelung-like deformity may result from trauma, infection, multiple hereditary exostoses (MHE), and Ollier’s disease. Additionally, it is associated with skeletal dysplasias involving mutations of the short stature homeobox (SHOX) gene. Madelung’s deformity predominantly affects females and becomes clinically apparent during adolescence. Affected individuals may present with wrist pain, restricted range of motion of the wrist and forearm, decreased grip strength, and function difficulties, as well as aesthetic concerns. Several surgical options have been described for children with Madelung’s deformity, depending on their age and degree of deformity, and include physiolysis, soft tissue release, and osteotomies, with promising outcomes.

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References

  1. Goldfarb CA, Ezaki M, Wall LB, Lam WL, Oberg KC. The Oberg-Manske-Tonkin (OMT) classification of congenital upper extremities: update for 2020. J Hand Surg. 2020;45(6):542–7.

    Article  Google Scholar 

  2. Flatt A. The care of congenital hand anomalies. St. Louis: Mosby; 1994.

    Google Scholar 

  3. Lecon GD. Leçons Orales De Clinique Chirurgicale: Faites A L’hotel-dieu De Paris, Germer Baillière; 1839, vol. 4; 1834. p. 197.

    Google Scholar 

  4. Mostofi SB. Who’s who in orthopedics. London: Springer; 2005. p. 89–92, 214, 217–219.

    Google Scholar 

  5. Malgaigne JF, Baillière JB. Traité des fractures et des luxations. Paris: chez JB. Baillière; 1855, Vol. 2. Paris; 1855. p. 259–76.

    Google Scholar 

  6. Madelung O. Die Spontane Subluxation de Hand Nach Vorne. Verh Dtsch Ges Chir. 1878;7:259–76.

    Google Scholar 

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

    Article  Google Scholar 

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

    Article  CAS  Google Scholar 

  9. 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  Google Scholar 

  10. Munns CF, Glass IA, LaBrom R, Hayes M, Flanagan S, Berry M, et al. Histopathological analysis of Leri-Weill dyschondrosteosis: disordered growth plate. Hand Surg Int J Devoted Hand Up Limb Surg Relat Res J Asia-Pac Fed Soc Surg Hand. 2001;6(1):13–23.

    CAS  Google Scholar 

  11. Seki A, Jinno T, Suzuki E, Takayama S, Ogata T, Fukami M. Skeletal deformity associated with SHOX deficiency. Clin Pediatr Endocrinol. 2014;23(3):65–72.

    Article  Google Scholar 

  12. Dawe C, Wynne-Davies R, Fulford GE. Clinical variation in dyschondrosteosis. A report on 13 individuals in 8 families. J Bone Joint Surg Br. 1982;64(3):377–81.

    Article  CAS  Google Scholar 

  13. 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.

    Google Scholar 

  14. Lippe B. Turner Syndrome. Endocrinol Metab Clin N Am. 1991;20(1):121–52.

    Article  CAS  Google Scholar 

  15. Zinn AR, Wei F, Zhang L, Elder FF, Scott CI, Marttila P, et al. Complete SHOX deficiency causes Langer mesomelic dysplasia. Am J Med Genet. 2002;110(2):158–63.

    Article  Google Scholar 

  16. Clement-Jones M, Schiller S, Rao E, Blaschke RJ, Zuniga A, Zeller R, et al. The short stature homeobox gene SHOX is involved in skeletal abnormalities in turner syndrome. Hum Mol Genet. 2000;9(5):695–702.

    Article  CAS  Google Scholar 

  17. Ghatan AC, Hanel DP. Madelung deformity. J Am Acad Orthop Surg. 2013;21(6):372–82.

    PubMed  Google Scholar 

  18. Rump P, Jongbloed JDH, 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  Google Scholar 

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

    CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

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

    Google Scholar 

  22. Ducloyer P, Leclercq C, Lisfranc R, Saffar P. Spontaneous ruptures of the extensor tendons of the fingers in Madelung’s deformity. J Hand Surg Edinb Scotl. 1991;16(3):329–33.

    Article  CAS  Google Scholar 

  23. Jebson PJ, Blair WF. Bilateral spontaneous extensor tendon ruptures in Madelung’s deformity. J Hand Surg. 1992;17(2):277–80.

    Article  CAS  Google Scholar 

  24. Shahcheraghi GH, Peyman M, Mozafarian K. Madelung deformity and extensor tendon rupture. Am J Orthop Belle Mead NJ. 2015;44(7):E242–4.

    PubMed  Google Scholar 

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

    Article  Google Scholar 

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

    Article  Google Scholar 

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

    Article  Google Scholar 

  28. Peymani A, Dobbe JGG, Streekstra GJ, McCarroll HR, Strackee SD. Quantitative three-dimensional assessment of Madelung deformity. J Hand Surg Eur Vol. 2019;44(10):1041–8.

    Article  Google Scholar 

  29. Oishi S, Wheeler L, Ezaki M. Madelung’s deformity. In: The pediatric upper extremity. 1st ed. New York: Springer Reference Abzug JM, Kozin S, Zlotolow DA eds. 2015;1763–72.

    Google Scholar 

  30. Kozin SH, Zlotolow DA. Madelung Deformity. J Hand Surg. Abzug JM, Kozin S, Zlotolow DA eds. 2015;40(10):2090–8.

    Google Scholar 

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

    Article  Google Scholar 

  32. 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):e306–11.

    Article  Google Scholar 

  33. 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. 2006;31(9):1499–506.

    Article  Google Scholar 

  34. 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  Google Scholar 

  35. 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. 2003;28(3):421–6.

    Article  Google Scholar 

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

    Article  Google Scholar 

  37. Bauer AS, Storelli DAR, Sibbel SE, McCarroll HR, Lattanza LL. Preoperative computer simulation and patient-specific guides are safe and effective to correct forearm deformity in children. J Pediatr Orthop. 2017;37(7):504–10.

    Article  Google Scholar 

  38. 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. 2013 Oct;38(10):1925–32.

    Article  Google Scholar 

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Correspondence to Michelle A. James .

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Manske, M.C., James, M.A., McCarroll, H.R. (2021). Madelung’s Deformity. In: Laub Jr., D.R. (eds) Congenital Anomalies of the Upper Extremity. Springer, Cham. https://doi.org/10.1007/978-3-030-64159-7_25

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  • DOI: https://doi.org/10.1007/978-3-030-64159-7_25

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