Dysplasia epiphysealis hemimelica: radiographic and magnetic resonance imaging features and clinical outcome of complete and incomplete resection

Abstract

Objectives: The objectives of this communication were to discuss radiographic and magnetic resonance (MR) imaging manifestations and clinical outcome after complete and incomplete resection of the mass of dysplasia epiphysealis hemimelica (DEH).

Materials and methods: Clinical records, radiographs, and MR images of eight patients with DEH were retrospectively examined. Six patients were treated by complete excision of the lesional mass, and two patients were treated by incomplete resection at our University Hospitals during the period from 1980 to 2006.

Results: We found that, unlike in osteochondroma, DEH was radiographically not clearly separable from the underlying or host bone with preserved cortical bone and marrow continuity. The finding in the talus distinguished DEH from (osteochondroma-like) parosteal osteosarcoma, in which a radiolucent demarcation line clearly separated the tumor from the host bone. The DEH mass had a well-defined low to intermediate signal intensity on T1-weighted images and an intermediate to high signal intensity on T2-weighted images, with irregularity of the articular surface. Simple excision was performed in all patients. The excision was complete in six patients and incomplete in two patients whose lesions was juxta-articular in the ankle and articular in the knee, respectively. The residual mass slowly absorbed and vanished, resulting in mild flaring of the affected portion of the epiphysis. No local recurrence or complication was seen in any of the eight patients.

Conclusions: Although the radiographic signs of DEH are characteristic, (osteochondroma-like) parosteal osteosarcoma should be differentiated from DEH when there is a radiolucent separation line between the mass and host bone in the talus. Simple excision was effective in the management of DEH if the deformity was not complicated. Incompletely excised masses resolved and vanished with time.