Multiple Enchodromatosis (Ollier's Disease)

- Discussion:
    - originally was described by Ollier in late 1800s;
    - 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.

Epiphyseal Metaphyseal Enchondromatosis.  A new clinical entity.

Ollier Disease: Pathogenesis, Diagnosis, and Management



Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Tuesday, June 30, 2015 2:52 pm