Follow Up Exam After Fracture

Jonathan Gordon, M.D., Ph.D.

July 29, 2004


Ollier's disease (OD) is named after the pioneering French orthopedic surgeon, Louis Ollier (1830-1900), who described the disease in 1898. OD is a sporadic, nonhereditary disorder of growing bone which predominantly affects tubular bones resulting in bowing, shortening and the growth of multiple enchondromas. Enchondromas are the second most common cartilage containing tumor (osteochondromas #1) and have the capacity to undergo malignant transformation. They classically are expansile, with endosteal scalloping and display glass-glass opacity owing to the content of chondroid matrix. OD typically presents in early childhood with deformity of the hands or feet which is predominantly unilateral. There may ultimately be gross unilateral limb deformities including shortened limbs as well as Madelung deformity. Most of the enchondromas will stabilize by puberty but can continue to enlarge throughout life. Patients with OD have a higher risk of fracture as well as malignant transformation into osteo-, fibro- or chondrosarcomas (up to 50%) relative to patients with a solitary enchondroma. Suspicion should be aroused when a patient presents with new pain at the site of a lesion in the absence of fracture. The differential for this case would include Maffucci syndrome which is composed of multiple enchondromas and soft tissue hemangiomas. It is radiographically differentiated from OD by the presence of soft tissue calcifications indicative of hemangiomas. These patients are at the added risk of transformation of hemangiomas into angiosarcomas.

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