The dyschondrosteoses of Nievergelt and Langer occur only in girls. The disorder consists of bilateral Madelung deformities, illustrated here. The radius is shortened and a synchondrosis may form with the ulna. These are very rare conditions with autosomal dominant transmission.
The Madelung deformity is caused by an absence of the ulnar portion of the radial growth plate of the radius. The radial side of the radial diaphysis is under formed as a result. Surgical treatment is by the Darrach procedure consisting of distal radial osteotomy with ulnar shortening.
REFERENCES
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Petrella R, Ludman MD, Rabinowitz JG, Gilbert F, Hirschhorn K. Mesoamerica dysplasia with absence of fibulae and hexadactyly: Nievergelt syndrome or new syndrome? Am J Med Genet. 1990 Sep;37(1):10-4.