Dyschondrostéose (Léri-Weill Syndrome): Congenital Short Forearms, Madelung-Type Wrist Deformities, and Moderate Dwarfism

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Abstract
Dyschondrosteose of Leri and Weill is a rare growth disorder characterized by extreme undergrowth of the forearms, Madelung-type wrist deformities, and an adult height of 4 ft., 8 to 10 in. A case with typical roentgen findings is reported in a 6-year-old male. Differential diagnosis, other than true Madelung deformity, includes pseudo-Madelung disturbances of peripheral growth, as in multiple exostoses, and related conditions include myositis ossificans progressiva, cleidocranial dysostosis, and Albright''s pseudohypoparathyroidism and pseudo-pseudohypoparathy-roidism. The mesomelic (midsegment) type of dwarfism and the abnormalities of the radius and ulna are discussed in the light of recent attempts at bone dysplasia classification.