Displasia Epifisária Hemimélica

apresentação de um caso com 12 anos de evolução

Abstract

Trevor Disease or Dysplasia epiphysealis hemimelica (DEH) is a rare skeletal developmental disorder characterized by asym metricovergrowth of cartilage in the epiphyses. From the histological point of view, it is considered to be an osteochondroma of the epiphysis. Most of the time, the onset of the symptoms occurs in the childhood. Males are 3 times more affected than females. The most common symptom is a painless bony mass around the ankle or the knee, followed by swelling, restricted range of motion and deformity. Imaging diagnosis is based on plain radiographs, CT scans and MRI. The treatment depends on the location, amount of involvement of the epiphysis, deformities and symptoms. Asymptomatic patients do not need treatment. When no deformities were identified simple excision of the mass is the treatment of choice. lf the mass causes asymmetry of the epiphyses the resection of the mass must be combined with osteotomies. We present a case of Trevor Disease in a 12 years old girl, treated by ressection of the talar bony mass and the talo navicular arth rodesis. After 12 years of follow-up patient still asymptomatic and no recurrence was detected.