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March 1958

Dysplasia Epiphysialis Hemimelica

AMA Am J Dis Child. 1958;95(3):276-281. doi:10.1001/archpedi.1958.02060050278008

The epiphysis is a most important component of the growing limb, and any disease process which primarily disturbs epiphyseal growth is significant, but fortunately such diseases are very rare. According to Fairbank,1 only three conditions are thus-far known in which the primary defect lies in the epiphysis: (1) dysplasia epiphysialis multiplex, (2) stippled epiphysis, and (3) dysplasia epiphysialis hemimelica. It is true that the epiphyses are involved in other diseases occasionally, such as the bone dystrophies, but in all these entities the epiphyseal involvement is only part of the general skeletal disturbance.

Historically, dysplasia epiphysialis hemimelica was first described by Mouchet and Belot2 in 1926, in a patient with ankle involvement, and the condition was named by them "tarsomegalie." Trevor3 subsequently collected 10 cases of similar nature involving the knee and/or ankle and suggested the diagnostic term "tarsoepiphysial aclasis." A single case was described in the French