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November 1971

Picture of the Month

Author Affiliations

Boston; East Meadow, NY
From the Meadowbrook Hospital, East Meadow, NY.

Am J Dis Child. 1971;122(5):429-430. doi:10.1001/archpedi.1971.02110050099013

Denouement and Discussion 

Dyschondrosteosis (Leri-Weill Syndrome, Leri's Pleonosteosis) 

Manifestations  Dyschondrosteosis is an inherited bone dysplasia characterized by mild to moderate dwarfism (height below the 25th percentile), symmetrical shortening of the forearm in relation to the upper arm (mesomelia); posterior dislocation of the distal ulna which is easily reducible but cannot be maintained in position; painless limitation of motion of the elbow and wrist, shortening of the tibia in relationship to the femur; short, spadelike hands; broad thumbs, genu recurvatum; thickening of the ulnar and forearm fascia; skin nodules; mongoloid slant of the eyes; and vertebral abnormalities. The fork-like deformity of the forearm and the wrist, known as Madelung's deformity, is not restricted to this syndrome, but may be found as an isolated congenital deformity, associated with Turner's syndrome, or as a result of infection, trauma, or rickets. Although there are other findings, the three fundamental criteria for the diagnosis of

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