Answer BT010
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Ollier's disease
Synonyms: Multiple Enchodromatosis (Ollier's Disease)- Discussion: - originally was described by Ollier in late 1800's; - characterized by multiple enchondroma lesions primarily located w/ in the metaphyseal regions of tubular bones; - usually diagnosed in childhood; - pts w/ Ollier's dz have increased risk of a secondary chondrosarcoma developing later in life; - risk is about 25% by age 40 yrs; - Clinical Findings: - most pts have bilateral involvement, w/ predominance on one side; - enchondromas most frequently involve the short tubular bones of hands and feet as well as long bones of upper and lower extremities; - development of palpable masses, angular deformity & growth disturbances resulting in angular deformities & leg length discrepancies; - limb length inequality may be of sufficient severity to require surgery; - Radiographs: - in most cases, there will be diaphyseal lesions or metaphyseal lesions which do not cross the growth plate, at least, not until growth plate closure; - in some cases enchondromatosis will affect patients in the metaphyseal and epiphyseal regions; - when enchondromas cross the growth plate, severe limb length deformities and angular deformities will develop; The malignant potential of enchondromatosis Ollier's Disease. An assessment of angular deformity, shortening, and pathological fracture in twenty-one patients. Approximately 30% of patients with Ollier's disease will develop malignancies of their tumors. It is important to recognize these changes in order for prompt treatment of the lesion. |
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BRTS
