Presentation
Jumped off bed, presented with painful leg, limbing and not weight bearing.
Patient Data
Spiral fracture of the distal tibia. Multiple lucent bone lesions in the tibia and fibula. Expansion of the cortex, but no periosteal reaction or other aggressive features.
Follow up x-ray of the healing tibial fracture. The tibial and fibular lesions are clearly demonstrated on the x-ray.
A femoral x-ray shows femoral neck lucency.
Monomelic polyostotic T2 hyperintense medullary lesions in right femur, tibia and fibula. Heterogeneous signal. Narrow zone of transition. Expansion of the medullary cavity. No periosteal reaction or soft tissue mass.
No other bony or soft tissue abnormality. MRI brain (not included here) is normal.
CT guided biopsy of the fibular lesion after the tibial fracture had healed. Tissue sent to pathology.
Histopathology
Irregular shaped portions of woven trabecular bone, without obvious osteoblastic covering, lying within variably cellular fibrous tissue. The appearances are suggestive of fibrous dysplasia rather than LCH.
Case Discussion
The key to this case is identifying the lucent bone lesions on the first x-ray and triggering investigation.
Multiple bone lesions give us a differential that includes CRMO, LCH and polyostotic fibrous dysplasia.
- CRMO the multiplicity of lesions would be appropriate, but the distribution is wrong.
- LCH is a possibility, but the bone involvement is monomelic and there is no spinal abnormality
- fibrous dysplasia (polyostotic) seems the most likely diagnosis on the imaging and was confirmed at biopsy.
Once the diagnosis of polyostotic fibrous dysplasia is made, consideration about whether there is other multisystem involvement should trigger further investigations, especially endocrine to exclude McCune Albright.
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