Fibrous dysplasia - shepherd crook deformity

Case contributed by Mostafa Elfeky
Diagnosis probable

Presentation

Abnormal gait

Patient Data

Age: 20 years
Gender: Female

Coxa varus angulation of the proximal femur (shepherd crook deformity) with expansion of all the femoral shaft and right iliac bone.

The right iliac bone, right femur neck and proximal femur shaft as well as the inferior pubic ramus show multiple lucent areas and mild bone expansion some shows sclerotic rim. Preserved bone cortex with no defiant pathological fracture could be seen.

Multiple intramedullary, geographic osseus lesions are seen involving the entire right femoral neck & shaft, right iliac bone and the examined part of the right tibia, the largest lesion is at the right femoral neck, seen mildly expansile with associated coxa vara. All lesions showed heterogeneous matrix with lucent and ground glass density regions. Sclerotic margins with narrow zone of transition. No pathological fractures. No periosteal reaction. No extra-osseus component.

Case Discussion

Picture of polyostotic fibrous dysplasia affecting the right lower limb and right side of the pelvis. Digital radiograph shows typical findings of ground-glass opacity and shepherd's crook deformity of the proximal femur.

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