- 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.
Epiphyseal Metaphyseal Enchondromatosis.
A new clnical entity.
PG Gabos et al.
JBJS Vol 80-A.
No 6.
June 1998. p 782.
