Findings: Radiographies of both wrists show increased ulnar and palmar tilting of the distal radius articular surface, bowing of the distal radius, and relative overgrowth of the distal ulna with dorsal subluxation. There are triangulation of the distal radial epiphysis, and wedging of the carpus between the deformed radius and protruding ulna, with the lunate at the apex of the wedge.
Diagnosis: Madelung’s deformity
Discussion: Madelung’s deformity, first described in 1855 by Malgaigne and 1878 by Madelung, is caused by premature closure of the physis of the distal radius in its volar ulnar segment. Madelung’s deformity may be idiopathic, but genetic studies have demonstrated that it may be transmitted as an autosomal dominant trait with incomplete penetrance as well. A mesomelic variety of dwarfism known as dyschondrosteosis is associated with Madelung’s deformity, however, the relationship between Madelung’s deformity and dyschondrosteosis is not completely understood. The isolated variety of Madelung’s deformity is more commonly bilateral than unilateral, and more frequent in female.
Radiographic and clinical features of Madelung’s deformity have been well described: an ulnar and dorsal curvature of the distal radius, increased inclination of the distal radius joint surface, triangulation of the carpus with proximal and volar migration of the lunate, and a prominent dorsal subluxation of the ulnar head. Clinical manifestations usually become evident in adolescents or young adults.
References:
1. Cook PA, Yu JS, Wiand W, et al. Madelung deformity in skeletally immature patients: morphologic assessment using radiography, CT, and MRI. J Comput Assist Tomogr. 1996;20:505-511
2. Zebala LP, Manske PR, Goldfarb CA. Madelung's deformity: a spectrum of presentation. J Hand Surg Am. 2007;32:1393-1401
3. McCarroll HR Jr, James MA, Newmeyer WL 3rd, et al. Madelung's deformity: quantitative assessment of x-ray deformity. J Hand Surg Am. 2005;30:1211-1220 1.
4. Tuder D, Frome B, Green DP. Radiographic spectrum of severity in Madelung's deformity. J Hand Surg Am. 2008;33:900-904 |