Polyostotic fibrous dysplasia

Case contributed by Dr Iqbal Naseem

Presentation

A young girl presented with generalized weakness and progressive bowing of bilateral legs.

Patient Data

Age: 9 years
Gender: Female
X-ray

Radiographs of bilateral hands with wrist joints, Pelvis with b/l hip joints including proximal femurs and bilateral legs were taken.

There is bony expansion, remodeling and ground glass matrix involving bilateral second metacarpal bones. 

Femoral neck deformity, widening and altered neck angles are seen on pelvis radiograph. Bilateral proximal femurs and bony pelvis shows ground glass bony matrix, remodeling and bowing of bilateral femurs giving characteristic Shepherd's crook deformity.

Legs radiographs show ground glass bony matrix and bowing involving bilateral tibia and fibula. 

Case Discussion

Features consistent of polyostotic fibrous dysplasia.

Fibrous dysplasia is a benign condition seen in young individuals characterized by replacement of bone by ground glass matrix with associated cystic changes. It can be mono or polyostotic disease.

Associations of polyostotic form include McCune-Albright syndrome (+Hormonal changes like precocious puberty and cafe au laits spots) and Mazabraud syndrome (+ Muscular myxomas).

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