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DYSPLASIA EPIPHYSEALIS HEMIMELICA: EARLY SURGICAL TREATMENT



Abstract

Dysplasia epiphysealis hemimelica is a rare congenital abnormality of enchondral ossification of one or more epiphyses, thus determining an asymmetrical growth in that bone district. We are here considering 2 cases. The first case regards a 4-month-old female baby with an irreducible flexion of the right knee. The X-rays highlighted an area of different density with irregular contours and multiple ossifications; the MRI showed a bone lesion with osteocartilaginous structure in contiguity with the medial condyle; during the operation a osteocartilaginous formation partially fused with the medial condyle was observed. The second case regards a 10-month-old male baby, with a medial malleolus swelling. The X-ray highlighted an area of different density with irregular contours and multiple ossifications, medial to the epiphyseal nucleus of the talus; the MRI showed an osteocartilaginous lesion in contiguity with the talus; during the operation an osteo-cartilaginous formation fused with the talus was observed. The histological findings confirmed in both cases the presence of cartilaginous and bone tissue in absence of pathological lesions.

Being a lesion of malformative origin, the early removal of the abnormal ossification nuclei will permit to avoid the secondary lesions described in literature, like asymmetrical growth of the limb and functional limitation.

Correspondence should be addressed to Ariella Neustadt at Studio EGA, Professional Congress Organisers, Viale Tiziano, 19 - 00196, Rome - Italy.