Dysplasia epiphysealis hemimelica (Trevor's disease): 7 of our own cases and a review of the literature

Abstract

Background and purpose Dysplasia epiphysealis hemimelica is characterized by irregular overgrowth of cartilage in the epiphysis, usually affecting the knee and ankle. We treated 7 children by surgery between 1980 and 2005. After reporting one child case, we summarize our cases and the cases described in the literature. Method We discuss the diagnosis of this dysplasia, especially the role of radiography. We describe the suggested treatment, which could be surgical or non-surgical depending on the location and the symptoms. Results After reviewing 57 cases, we found that this dysplasia occurs twice as often in males as in females. The medial side of the epiphysis is affected twice as often as the lateral side. In two-thirds of the cases, more than one epiphysis was affected. If the location of the exostosis suggests that it might lead to joint deformity, early surgical excision is recommended. Interpretation Since there is often involvement of more than one epiphysis, we emphasize the importance of a skeletal survey once this dysplasia is diagnosed.