Treatment of Dysplasia Epiphysealis Hemimelica: A Systematic Review of Published Reports and a Report of Seven Patients

Abstract

Dysplasia epiphysealis hemimelica is a rare entity. It is characterized by cartilage overgrowth in the epiphyses and is considered to be an epiphyseal osteochondroma. It usually presents during childhood and can cause pain and functional limitations. The aim of the present study was to describe the entity through our cases and published data and to present the results of our suggested treatment. A systematic search was performed to retrieve and describe all studies on dysplasia epiphysealis hemimelica. Studies were included if sufficient information on the patient characteristics, lesion location, treatment, and outcomes were described. In addition, we report on 7 of our patients. In our review, 48 studies were found, of which 42 could be included. Owing to the large heterogeneity in the studies, no pooling of data was performed. The studies included 138 patients with 255 lesions. Approximately 27% were female patients. The age at presentation ranged from 3 months to 66 years; however, in most, the entity was discovered before 8 years of age. No apparent guidelines were presented concerning the treatment strategy; however, most investigators performed resection because of pain or limitations in the range of motion. Most lesions were located in the ankle or foot (139 of 255). In our own cases, 5 of 7 patients experienced pain, limited range of motion, or activity restriction. The mean follow-up period was 4 years. Dysplasia epiphysealis hemimelica is a rare entity, occurring predominantly in the lower extremity of in young male patients. Its aggressive growth warrants aggressive treatment in the case of pain or functional or range of motion limitations.