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 et al. Am J Hum Genet 77:533–44, 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.
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References
Arora AS, Chung KC, Otto W. Madelung and the recognition of Madelung’s deformity. J Hand Surg Am. 2006;31:177–82.
Benito-Sanz S, Thomas NS, Huber C, Gorbenkodel Blanco G, 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;77:533–44.
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;28A:421–26.
Glard Y, Gay A, Launay F, Guinard D, Legré R. Isolated wedge osteotomy of the ulna for mild Madelung’s deformity. J Hand Surg Br. 2007;32A:1037–42.
Grigelioniene G, Schoumans J, Neumeyer L, Ivarsson SA, Eklöf 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;109:551–58.
Harley BJ, Carter PR, Ezaki M. Volar surgical correction of Madelung’s deformity. Tech Hand Up Extrem Surg. 2002;6:30–5.
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.
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 Léri Weill dyschondrosteosis. Gene. 2012;491:123–27.
Madelung OW. Die spontane subluxation de hand nachvorne. Verh Dtsch Ges Chir. 1878;7:259–76.
McCarroll HR, James M, Newmeyer W, Manske PR. Madelung’s deformity: diagnostic thresholds of radiographic measurements. J Hand Surg Am. 2010;35A:807–12.
Murphy MS, Linscheid RL, Dobyns JH, Peterson HA. Radial opening wedge osteotomy in Madelung’s deformity. J Hand Surg Am. 1996;21:1035–44.
Nielsen JB. Madelung’s deformity. A follow-up study of 26 cases and a review of the literature. Acta Orthop Scand. 1977;48:379–84.
Ranawat CS, DeFiore J, Straub LR. Madelung’s deformity: an end-result study of surgical treatment. J Bone Joint Surg. 1975;57A:772–75.
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;87:1402–06.
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;95:1198–204.
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;17B:401–07.
Zebala LP, Manske PR, Goldfarb CA. Madelung’s deformity: a spectrum of presentation. J Hand Surg. 2007;32A:1393–401.
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Oishi, S.N., Wheeler, L., Ezaki, M. (2014). Madelung’s Deformity. In: Abzug, J., Kozin, S., Zlotolow, D. (eds) The Pediatric Upper Extremity. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8758-6_81-1
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DOI: https://doi.org/10.1007/978-1-4614-8758-6_81-1
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Madelung’s Deformity in Children- Published:
- 09 October 2023
DOI: https://doi.org/10.1007/978-1-4614-8758-6_81-2
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Madelung’s Deformity- Published:
- 20 June 2014
DOI: https://doi.org/10.1007/978-1-4614-8758-6_81-1