Presentation
Limb length discrepancy. Shorter right lower limb.
Patient Data
Diffuse well-defined lytic lesions with rings and arcs matrix calcification and predominance in the right side of the skeleton.
Most notable lesions:
proximal and distal metaphyses of the right femur
proximal metaphysis of the right tibia
small tubular bones of the right foot
Case Discussion
Enchondromatosis refers to the group of skeletal disorders presenting with multiple enchondromas.
Enchondromas are somewhat common benign cartilaginous tumors. They are usually seen in the small tubular bones of the hands and feet, but they can also be found in large tubular bones.
Typically, enchondromas will have a distinct radiographic depending on whether they are present in a small or large tubular bone.
In large tubular bones, such as in this case, they usually present with a chondroid matrix and no aggressive features such as endosteal scalloping, periosteal reaction, or cortical disruption.
If aggressive features are present, low-grade chondrosarcoma is an important differential diagnosis.
There are many subtypes of enchondromatosis, of which Ollier disease is the most common. The main characteristic of Ollier disease is the unilateral predominance.
In the present case, there is clear right-sided predominance.
Due to the metaphyseal disturbance, patients with unilateral enchondromatosis usually develop limb length discrepancy during skeletal growth.
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