NORD gratefully acknowledges German C. Steiner, MD, Clinical Professor of Pathology, NYU School of Medicine, New York, for assistance in the preparation of this report.
Dysplasia epiphysealis hemimelica (DEH), also known as Trevor disease, is a raredevelopmental bone disorder of childhood. It is characterized by an abnormal overgrowth of cartilage arising from the cartilage which is normally present in the terminal ends (epiphyses) of the long bones particularly of the lower limbs. The bones of the knee and ankle joints are most commonly affected, as well as part of the foot (tarsal bones). The upper limbs are very rarely involved. The cartilage overgrowth occurs either in the medial or lateral part of the bone (hemimelic) usually medial. DEH may affect a single bone (localized form), multiple bones in a single limb (classical form) or an entire limb (generalized) usually involving a leg from the pelvis to the foot. Approximately two-thirds of affected children have multiple lesions.
DEH was first described in the medical literature in 1926. Trevor recognized this condition in 1950. The name, dysplasia epiphysealis hemimelica first appeared in the medical literature in 1956.
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