Polyostotic fibrous dysplasia

Case contributed by Domenico Nicoletti
Diagnosis almost certain

Presentation

Hip pain.

Patient Data

Age: 20 years
Gender: Male
x-ray

Left coxa varus angulation of the proximal femur (shepherd crook deformity) with expansion of all the femoral shaft, pelvic ring and left iliac bone.

Expansion of iliac bone, pelvic ring and left femur with ground glass bony matrix and cyst formation.

mri

Fibrous matrix with cystic lesions and low-intensity signal on T1 and T2 sequences. Modest hip joint effusion on the left.

Case Discussion

Fibrous bone dysplasia is a congenital non-hereditary benign skeletal disease in which the bone is replaced by a fibrous-like tissue with early osteogenesis. Fibrous dysplasia can be mono- or polyostotic and may be associated with bone pain and fragility, evolving to fractures. In some patients or in some bone sites, lesions are hypertrophic and may cause neurological complications secondary to compression. 

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