Presentation
Left hip pain.
Patient Data
Coxa varus angulation of the left femoral bone indicates shepherd crook deformity with an expansile lytic lesion involving the femoral neck with ground glass matrix.
A pathologic horizontal fracture in the proximal left femoral shaft is detected. The left femoral head is located inside the acetabular fossa.
Multiple sclerotic lines are seen in the right femoral neck and right iliac wing due to arrest lines in response to previous treatment with bisphosphonates.
Case Discussion
All mentioned features are consistent with Fibrous dysplasia.
Fibrous dysplasia is a benign condition seen in young individuals characterised by replacement of bone by ground glass matrix with associated cystic changes.
This case had typical clinical and imaging features of McCune-Albright syndrome (MAS), also known as McCune-Albright-Sternberg syndrome, a genetic disorder characterised by the triad:
endocrinopathy: precocious puberty
polyostotic fibrous dysplasia: more severe than in sporadic cases
cutaneous pigmentation: coast of Maine 'café au lait' spots
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