Dysplasia epiphysealis hemimelica: a huge articular mass with unpredictable surgical results

Abstract

Dysplasia epiphysealis hemimelica or Trevor's disease is a rare disorder of localised osteochondral overgrowth affecting the epiphysis of extremities. This paper reports a 12-year-old boy presenting with a large bony mass at the left ankle diagnosed as dysplasia epiphysealis hemimelica. The articular surface of the ankle joint of the patient was evaluated with preoperative and postoperative MRIs. The 2-year postoperative MRI showed early osteoarthritis of the ankle, therefore demonstrating the importance of early excision avoiding more complex resections of intra-articular lesions.

Figure 1

Left ankle shows a large mass restricting the motion of the ankle joint.

Figure 2

Plain radiographs of the left ankle show a huge lobulated mass with multiple focal calcifications on the anterolateral and posterior side of the ankle.

Figure 3

Three-dimensional CT of the left ankle demonstrates a huge multilobulated mass protruding from the talus and obstructing the greater part of the ankle joint.

Figure 4

MRI of the left ankle. T2-weighted images demonstrate a heterogeneous mass arising from the talus and occupying the greater space of the joint.

Figure 5

Histopathological appearance demonstrates cartilaginous cap covering the medullary bone which looks similar to osteochondroma.

Figure 6

Excised mass shows cartilaginous caps covering the bones.

Figure 7

MRI of left ankle at 2-year postoperative exam. T1-weighted images demonstrate premature arthritis. Note: small articular defect at the distal tibia and the dome of the talus.