Poly-ostotic 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
Gender: Female
Modality: X-ray

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

There is bony expansion, remodelling and ground glass matrix involving b/l 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, remodelling and bowing of b/l femurs giving characteristic Shepherd's crook deformity.

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

Case Discussion

Features consistent of polyostotic fibrous dysplasia.

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

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

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Case Information

rID: 22797
Case created: 24th Apr 2013
Last edited: 6th May 2017
Inclusion in quiz mode: Included

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