Fibrous dysplasia around the elbow

Abstract

First named by Lichtenstein in 1938, fibrous dysplasia is a noninherited, skeletal developmental abnormality that commonly presents in adolescents and young adults. Normal marrow and cancellous bone are replaced and weakened by immature woven bone and a dense fibrotic stroma containing a disorganized matrix of bony trabecular spicules. Fibrous dysplasia accounts approximately for 5% to 7% of benign bone tumors. It may be monostotic, accounting for 70% to 80% of cases, or polyostotic. Fibrous dysplasia frequently occurs in the ribs, femur, tibia, skull, pelvis, spine and shoulder. Within the long bones, the lesions are predominately diaphyseal, with an epiphyseal lesion rarely occurring. Radiographically, they are classically described by their ‘‘ground glass’’ appearance because they are well circumscribed, radiolucent lesions with a hazy quality to them. These lesions compromise the structural integrity of the involved bone and may lead to a bowing deformity in weight-bearing bones, hence the ‘‘shepherds crook’’ deformity describing coxa vara in the femur. Surgical treatment of fibrous dysplasia is palliative, the main goals being to limit pain and deformity and prevent pathologic fracture. Fractures through a lesion often heal without difficulty but are susceptible to repeat fracture because of the dysplastic nature of the bony callus that forms. Indications for surgery include nonunion, progressive deformity, or persistent pain. Surgical options include curettage and bone grafting (either cortical or cancellous), open reduction and internal fixation (ORIF), and vascularized bone grafts.