Background: In polyostotic fibrous dysplasia, particularly in lesions of the proximal femur, pathological fracture and coxa vara deformity (including shepherd's crook deformity) are likely to develop and progress.
Patients and methods: In 7 femurs with shepherd's crook deformity (5 patients), we performed intramedullary nailing by using multiple osteotomies and two screws crossing the femoral neck.
Results: Restoration of the neck shaft angle of the femur was obtained from an average of 92 degrees prior to surgery to 129 degrees after surgery. During the minimum 2-year follow up, no loss of neck shaft angle of the femur and no refracture occurred. All patients were able to return to normal activities of daily living.
Interpretation: In polyostotic fibrous dysplasia, multiple osteotomies and intramedullary nailing with neck cross-pinning can be used to correct developed or progressing shepherd's crook deformity, and to prevent recurrence and refracture.